Say What?

New Eyes

2012-01-27T11:24:00.000-08:00

We picked up Nolan's new glasses today. This pair has the cable arms that we love so dearly, and fit his little face a lot better than the online glasses. They're Disney frames, so Nolan is quite excited to have "Mickey Mouse" glasses. There used to be tiny Mickeys on the end of original arms, but the Mickey Mouse emblems had to come off to put the cable arms on.

We have kept the glasses purchased online for a "back-up" pair, but hopefully we won't need them!

Fixed Ears

2012-01-25T09:59:00.000-08:00

Nolan's new ear hooks came in the mail yesterday - I was rather relieved to put the new hook on, as the other (broken) hook was rather loose. When the little guy had his tantrum last week, he had snapped through the threads - while the hearing aid was still usable, I was in constant fear that it would fall apart at school and that we'd lose a hearing aid! The new hook is nice and secure.

I called the pediatric surgeon's office yesterday to make sure all of Nolan's records had arrived. I knew his scans from the ENT were already there (they were what prompted the surgeon's phone call two weeks ago), but I wanted to make sure the pH probe, biopsy, and pathology report had arrived from the GI doctor's office. Everything has made its way to his office, so we should hear back on "The Plan" soon. The next step will probably involve another consultation with the doctor prior to surgery (if surgery proceeds) and will definitely involve another gastric emptying scan. In the meantime, I continue to bake and eat pastry. I hope things get settled before another week passes, because I am starting to measure the waiting time in pounds gained.

Nolan Has Vacation Plans

2012-01-23T10:47:00.000-08:00

Nolan came home from school the other day, bubbling over with excitement.

"Mom, we need to go to LONDON!"

Since that isn't a phrase expected out of the mouth of a four year old, I just stared and him and repeated, "London?"

"It's a CITY! A big city!"

I was surprised he knew about London, since we haven't exactly studied world geography yet. I asked him what he knew about London.

"It has bridges, Mom. I need to see the London Bridges. They're falling down. In the RIVER!"

Oh.

London Bridge Is Falling Down.

"Nolan, did you know that Mommy and Daddy have been to London?"

Eyes wide, he said, "Did they take a key and lock you up?"

I took out our photo albums and explained that the river in London is called the Thames, and that the bridges are not currently falling down. I also explained that they aren't locking up any "fair ladies" at the moment.

A bridge in London - not falling down

He seemed rather disappointed. Apparently, he considers watching bridges crash into rivers a ripping good time. Especially if a few ladies happen to get locked up in jail at the same time.

I have a feeling that England will be on the Future Vacation Plan list for Nolan. The Tower of London sounds right up his alley!

The Waiting Place

2012-01-19T09:35:00.000-08:00

This is one of my least favorite places to be.

Waiting.

Waiting for records to be mailed.

Waiting for the phone to ring.

Waiting for decisions to be made.

I send the consent form to the GI doctor on Saturday, so they should be receiving the form in the mail today (if they haven't received it already). Hopefully they'll fax Nolan's records to the surgeon quickly - I want to know what the GI team at Golisano Children's thinks, and what we need to do next.

I don't like the aimless feeling of not knowing what is going to happen next: I cope better when we have a solid plan in place.

In the meantime, Nolan is doing great. He's eating like a champ - on "off" days, he eats mostly yogurt and grapes, but he makes up for it on good days. His nose is filling up with the yellow-green stuff again, but he isn't too congested. He has had a few dizzy spells over the past few days, and I'm not quite sure what to make of those. My best guess is something vestibular, but who knows? He claims the earth is spinning and falls down or staggers a bit. I'm keeping an eye on those - he seems fine otherwise, so hopefully the "spinny" will just disappear and we won't have to worry about it anymore.

Now I'm off to eat some chocolate bars, because chocolate is a very healthy way of dealing with stress.

The Trouble with Online Glasses

2012-01-17T09:24:00.000-08:00

I tried ordering glasses online for Nolan, to save a little money and to get them quickly over the Christmas holidays. With his amblyopia, I want to make sure that we stay on top of prescription changes so that his right eye continues to improve.

Unfortunately, ordering online is really hard with kids. Most sites don't allow you to sort by specific frame size, and what an online site claims is a "small" frame is really a frame that might fit a ten year old. There aren't many frames for small preschoolers online.

I found some "Badger" frames at $39 Eyeglasses and bought them because they were one of the only pairs with a 120mm temple length. The glasses arrived and they were simply too big. The temples were much longer than 120mm (closer to 125mm) and they just didn't fit our little guy's face. This isn't really the fault of the online retailer - small preschoolers can be hard to fit.

I called our local optician's shop the other day and went in to look for glasses that would fit Nolan. He's so small that only ONE pair fit his small face - a pair they had in a drawer underneath all the display glasses. He almost wears an infant sized frame. His new glasses (a Disney frame, with tiny gold Mickey Mouse emblems on the temples) will be ready in a week or two.

We also found that his PD (pupilary distance) had changed in the past six months - not a huge change, but it went from 50mm to 51mm. Getting a proper PD measurement is critical to fitting glasses, so I'm glad I took him in. I tried measuring it myself, but if you have ever tried to do this with a four year old child, you know it is impossible.

In the meantime, the optician adjusted Nolan's online eyeglasses to help them fit better. They're staying up much better since the adjustment. They're still slightly too big (you can see how the lenses are a bit too wide for his face), but at least he's looking through the lenses again.

I learned my lesson - I won't buy online again (for Nolan) unless it is just for sunglasses. In the meantime, we're very grateful that our local optician bent the temples around his ears and fixed the nose pad to keep his current glasses on his face!

Argh. Seriously, Nolan?

2012-01-16T07:59:00.000-08:00

The other day, Nolan had a temper tantrum. When Nolan has a tantrum these days, he usually removes his glasses and hearing aids, throwing them across the room. This is actually an improvement from the days when he would remove his glasses, hearing aids, shoes and socks for a fit.

Unfortunately, this last tantrum involved pulling his hearing aids off and then snapping them in half. The clear, hard plastic part that screws into the hearing aid body is the part that snapped (this is called the tone hook).

Fortunately, I don't think tone hooks are very expensive.

Still? I'd really appreciate it if the little guy would refrain from breaking his equipment during tantrums.

Here's to hoping they can simply send a new one in the mail!

On Motility Issues

2012-01-13T10:44:00.000-08:00

When we had our consultation with the pediatric surgeon, we brought up the fact that Nolan had shown delayed gastric emptying on an earlier test (called a scintiscan). The surgeon reassured us that the majority of kids with reflux appear to have delayed gastric emptying because the food goes up as well as down, resulting a a delayed time. Once the fundoplication is performed, the food can only go down and motility "improves." This was a reassuring thought and we figured the delayed emptying was simply an artifact on the test because of his reflux.

When Dr. Wonderful called me at home the other night, he told me to forget everything he told me about motility. The aforementioned is true for kids with regular, straightforward severe reflux. It is not true for Nolan.

Motility issues have brought everything to a grinding halt.

From the few tests that have been performed, it is apparent that Nolan has two motility issues (one definite, the other possible):

1) Delayed Gastric Emptying, or Gastroparesis
2) Esophageal Motility Problems

Nolan's scintiscan showed 0 episodes of reflux, but significant delayed gastric emptying. In this case, there was no reflux to create a "false positive" result. He truly has delayed gastric emptying, and it is possibly the underlying cause to the ridiculous levels of reflux.

Nolan's 24 hour impedance probe (from when he was 2 years old) showed a slow "bolus transit time" in his esophagus. This means that food took too long to get from his mouth to his stomach.

So, why are motility problems important?

If Nolan had a Nissen Fundoplication without addressing the motility, several things could go awry. His stomach would not empty fast enough, and with the top of the stomach "tied off," he could get painfully bloated. The potential esophageal motility issues are more worrisome - with a Nissen, a child could actually lose the ability to swallow food if there are esophageal motility problems.

We are going to have a repeat gastric emptying scintiscan done at Golisano Children's Hospital. If he still shows gastroparesis, then a pyloroplasty will be performed alongside the fundoplication. A pyloroplasty is the same surgery as performed on children with pyloric stenosis - it is a cut into the bottom sphincter of the stomach to allow food to empty faster. This comes with its own set of risks and benefits, which is why they don't perform this routinely with Nissen Fundoplications - it is reserved for kids with gastric emptying problems. A g-tube is always used for kids with delayed emptying with a fundoplication, to allow venting of stomach contents or air, in the event that the stomach can't empty itself fast enough.

There isn't much to be done about esophageal motility problems. This is usually an absolute contra-indication to a Nissen fundoplication. There is a test for esophageal motility, but it is quite invasive (the test is called esophageal manometry).

Anyhow, there are a few decent articles on motility problems in children. I'm linking them here for my own reference, but also to help others understand the difference between "typical reflux" and "reflux with motility issues."

http://www.articles.complexchild.com/00005.pdf: A great, plain-English description of various motility problems in children.

http://www.articles.complexchild.com/00028.pdf: A great article about the difference between "regular" reflux and reflux with motility issues.

http://www.nature.com/gimo/contents/pt1/full/gimo46.html: An article about the difference between LPR and GERD types of reflux (Nolan has LPR reflux)

Article on gastroparesis: http://www.usagiedu.com/articles/gastrop/gastrop.pdf

And then there is this little gem, about understanding the surgical treatment of reflux in the presence of delayed gastric emptying: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1422000/

"the existence of delayed gastric emptying should not be a contraindication for a Nissen fundoplication. Second, those who do operations other than a Nissen fundoplication for the treatment of reflux may wish to consider a fundoplication in the group of patients found to have abnormal emptying, as this operation is the only one that clearly improves gastric emptying. Third, in patients with delayed emptying the surgeon should not advocate a pyloroplasty, but simply a fundoplication. This recommendation, while indirectly supported by the results of this study, stems from a number of other observations. First, pyloroplasty increases duodenogastric reflux, which may damage the gastric mucosa or worsen esophagitis if the cardia remains incompetent. Second, pyloroplasty without vagotomy is ineffective in the treatment of idiopathic or diabetic gastroparesis regardless of its effects on gastric emptying. Lastly, this study and the preponderance of evidence from other studies suggest that there is a good chance that gastric emptying will be normalized after a Nissen fundoplication. If that does not occur, there is always time to reassess the situation and devise a new strategy to deal with the problem."

Phone Call

2012-01-11T15:38:00.000-08:00

Another picture from the Strong Museum of Play

Nolan's surgeon just called. I've never had a doctor do that before.

The crux of the conversation? Nolan is weird.

Not the child, of course, but the medical history and test results.

He was looking at the upper GI bariums, which were normal (barring one episode of reflux). And the gastric emptying scintiscan, which is normal for reflux but shows delayed gastric emptying. And the two pharyngeal pH probes, which were done while Nolan was on medication.

He doesn't have the original esophageal pH probe or the biopsy report.

And so we have some issues.

Since the scintiscan shows delayed gastric emptying in the absence of reflux, a pyloroplasty would be performed along with a Nissen - it is likely his problems are primarily motility related.

He's also concerned about some cellular changes that are casually mentioned in one report. If true, he would need serial monitoring.

And the pharyngeal pH probes are rather cutting edge- since they aren't generally used in the pediatric population, he's putting those aside for the moment. Nolan obviously has airway reflux - heck, he CHOKES on the stuff, but the pharyngeal probes were done on medication and he'd rather have a traditional pH probe.

I will fax Nolan's original pH probe study to the surgeon tomorrow. He'll have to wait a bit longer for the esophageal biopsy results and the pathology report, because I don't have physical copies of those. I have to wait until we get the consent form from the GI doc and then I'll mail it back - once I do that, the GI doc will fax the medical reports directly to the surgeon.

So we aren't scheduling surgery yet. Things aren't so straightforward as the surgeon originally thought, and he's concerned there is some bigger, underlying issue with the progressive nature of the reflux and airway. His exact words were,

"These kids aren't always what they seem. We're learning more about them all the time and sometimes there's something bigger."

Great. I seriously don't want something "bigger."

On the other hand, what an amazing, wonderful, incredible doctor. Taking time at 6:00pm to call a parent to get clarification on medical reports that he is ACTUALLY reading. Trying to figure out the mystery that is Nolan.

Since the regular esophageal pH probe was done when Nolan was 2 years old (along with the gastric emptying scan), he really wants them to be repeated. Which would mean pH probe #4 for the little guy (this time an esophageal one, like the original probe) and GES #2. He would also like another endoscopy/biopsy to look at the little guy's esophagus.

But he's going to talk to the GI team at Strong Memorial first, to get a consensus. And he wants to review the other records.

Looks like things are going to be topsy-turvy for a while.

More Nexium Battles

2012-01-11T11:15:00.000-08:00

Our insurance policy changed at the beginning of the year - not a big deal in the scheme of things, but we knew there would be a few snafus, particularly with so many providers and a medication that requires pre-authorization.

To head off problems with the little guy's medications, I called in his Nexium prescription early. Unfortunately, it was too early, so I had to wait to call it in. I tried again a week later, and it was still "too early." At this point, we only had 6 packets of Nexium left (3 days' worth), so I became a little concerned.

I called the next day and Rite Aid miraculously had Nolan's prescription in-house. I figured it was the result of some system error on their end, but at least his medication was available for pick-up. The pharmacy technician rang up the medication packets, and the total came to $900.

I looked at her and said, "Um. No."

She looked at the documentation stapled to the medication and it said, "individual not covered on plan."

I handed her our insurance card and said, "He's covered. We've used this card several times over the past few weeks and he is listed as a dependent."

Rite Aid had me wait for about 40 minutes as they called the insurance company. Our insurance company told Rite Aid they were too busy to take the call. Rite Aid gave up.

Which meant, of course, that my child didn't have the medication he needed.

I called Dennis at work and he looked at our insurance information online - Nolan was most certainly listed. Of course, our insurance company is having a huge battle with something known as competence: the "start date" listed on our cards was in error (the cards stated 02/01/12 as the effective date, when the real effective date was 01/01/12). The insurance company also had Matthew's name written down as "Motther." I suspect they have outsourced some of their labor. Still, the problem wasn't on the insurance company's end - it was on Rite Aid's.

In any case, I finally managed to get 3 packets of medication from Rite Aid, enough to cover Nolan for one more day.

I called this morning to see if they had sorted everything out, and the pharmacy tech said,

"We don't handle insurance issues. You have to call your insurance company."

I took a deep breath. It was a good thing I was at home and on the phone, or I would have started screaming. Mentally, I thought: "You imbecile! It is your problem, not the insurance company's! I can't fix your system, which is obviously malfunctioning!" Since this wasn't very nice, I didn't say it out loud.

I managed to say, "Talk to the pharmacist. See where the medication is. Then we might not have a problem, OK?"

She checked with the pharmacist and the medication is ready, all for the normal Co-Pay. They had obviously fixed their "system error" that was causing Nolan to appear as if he wasn't covered by insurance.

While we're stuck with our insurance companies and the battles that entails, we are certainly NOT stuck with a particular pharmacy. As soon as I feel the energy level, I'm switching to a different one. We certainly don't need added hassles to the regular difficulties in life!

At least Nolan will get his medication tonight. Sheesh.

Rochester: Pediatric Surgeon Consultation

2012-01-10T13:26:00.000-08:00

At the Museum of Play

We drove up to Rochester on Sunday, a day before Nolan's pediatric surgeon appointment. Rochester has a few kid-oriented museums, so we went to the science museum on Sunday and the Strong Museum of Play on Monday morning. Nolan and Matt had a blast, and we really enjoyed making time for some fun amidst the stress of the surgeon appointment.

The appointment was at Golisano Children's Hospital, part of Strong Memorial Hospital. Every time I go through Nolan's history, I realize how long it is. And I always forget something - though I was fortunate to have Dennis with me this time to help fill in the gaps. I do have his history typed up in a nice word file, but somehow managed to forget it on this trip (argh).

The surgeon did ask us why it had taken so long to get a referral for a fundoplication - with Nolan's symptoms, airway involvement, and severity, he should have had a Nissen quite some time ago. We had to explain that we (the parents) were the cause of the delay - his ENT wanted to refer him for surgery quite some time ago, but we wanted to exhaust every other avenue prior to doing this surgery (which, frankly, scares the bejeebies out of us).

The surgeon explained the procedure and how the fundoplication works - the fundus of the stomach is wrapped around the esophagus, and when food enters the stomach it fills the wrapped fundus, exerting pressure on the esophagus to keep anything from going UP.

We discussed our concerns, namely the idea that the surgery couldn't be "undone" if there were problems (he told us this was not true - while it is generally not "undone" because the person really needs it, a Nissen wrap can be taken down if necessary).

We also discussed things like gas bloat, wretching, and other scary, unpleasant side effects that can come with a fundoplication. Nolan is at risk for gas bloat, for a couple of different reasons:

1) He uses a C-Pap to breathe at night. This forces air down his throat, and sometimes that can get forced into the stomach.
2) He has delayed gastric emptying

Since Nolan is "complicated" and a little on the small side, it is common to insert a gastrostomy tube (g-tube) when the fundoplication is performed. If his emptying becomes a problem after surgery or he gets horrible gas bloat, we will be able to vent his stomach to release the trapped air (or stomach contents, in the case of delayed emptying). The g-tube can also be used to deliver medication and nutrition, if needed.

The type of gastrostomy tube that will be placed is called a "Mic-Key." A lot of people simply refer to these as a "button" or a "mickey." It is a small, skin level device that won't interfere with the little guy's daily activities, and if he doesn't need it we can have it removed.

The surgery (for the fundoplication) is done laprascopically, though sometimes the surgeon has to convert to an "open" procedure. From my basic understanding, this has a lot to do with visibility and how the liver covers the operating site.

The surgery requires a 5-7 day stay in the hospital following the laprascopic procedure, and a 7-10 day stay for an open procedure (we're really, really hoping the surgery can be completed laprascopically). While he's in the hospital, I will be trained on g-tube care and how to replace it if it should fall out accidentally. This is a big issue because our local hospital doesn't treat kids and lacks the necessary supplies - and a tube that falls out has to be replaced immediately.

If Nolan doesn't need the tube for venting and is eating well, the tube will be removed about 3 months after the operation. Removal is simple - you simply deflate the retention balloon, pull it out, and stick a bandaid on it - the wound heals in about 2 days.

There is also a restricted diet for a while and the recovery does take some time, but once everything is settled he should be free from reflux-related problems.

In the meantime, we are having his GI medical records sent to Strong Memorial. Since Nolan is "medically complex," the surgeon wants to be absolutely sure that there are no big contra-indications to the procedure. Once he has all of the records and verifies that the procedure is safe, the surgery date will be scheduled. We signed the consent form in his office, so we are simply waiting for the records to get sent and reviewed.

Ear Candy

2012-01-07T13:32:00.000-08:00

We picked up Nolan's new earmolds on Friday - red and white swirls. He calls them his "candy cane" hearing aids!

Easy Dentist Appointment

2012-01-04T19:15:00.000-08:00

The picture is from yesterday, but since today was basically a repeat of yesterday, I'm posting it.

The dental hygienist cleaned the tooth and we were able to get away with NO drilling. A huge relief to me (and to Nolan, too, though he doesn't know it). She dried the tooth, put the sealant over the soft spot, and sent us on our way.

For 10 minutes and $25, this was probably the easiest and cheapest dental appointment we've ever had!

Since we don't ever want to go a day without a doctor appointment, Matt's Molluscum decided to get very infected. The poor kiddo has boils all over his legs where the Molluscum lesions were. I'm calling the pediatrician tomorrow to get him in, because one looks extremely angry and infected. If you don't know what Molluscum is, be thankful. It's a pox-related virus that preschoolers get (and the incidence is increasing). They measure the infection time in years and the pox lesions spread during that time. Nolan got the virus from his brother, but fortunately hasn't had any of his lesions get infected. Both of my boys have eczema, and apparently the two don't mix well.

Nolan will be thrilled to be the one NOT going to the doctor's office, I'm sure. Judging from Matt's reaction to the dental X-ray machine, I suspect Matt will not be very cooperative for the pediatrician. Ugh.

Trip to the Dentist: Reflux Strikes Again

2012-01-03T15:54:00.000-08:00

I took both kids to the dentist today, and Nolan chose to go first. He climbed into the chair and let the hygienist clean and count his teeth. He is amazingly good at the dentist, probably because having teeth cleaned is a walk through the park compared to some of the things he goes through at the ENT's office. The dentist came in for a look, and both discussed a "soft spot" on his enamel that looks like acid damage.

"Does he have acid reflux?" they asked me.

Boy, does he ever. Words couldn't convey how severe his reflux is, so I just nodded my head.

I'm actually surprised that he's avoided dental issues with the level of reflux he has. Fortunately (for dental reasons) the airway reflux is less damaging to teeth. Still, his very breath has acid droplets in it and that eventually causes damage. He's also a tooth grinder, so his teeth are wearing away in the back. Lovely.

I get to take him back in tomorrow for his first "filling." They're actually billing it as a "sealant" because that is cheaper - we don't have dental insurance and the drilling will be minimal. They may be able to get away with no drilling, and simply seal over the soft spot. I am fervently hoping they won't need to drill. Fortunately, we caught the small area of decay early before it had a chance to do any real damage.

Of course, Matt was next. He was NOT so easy. They wanted to get his first set of dental X-rays, and Matt refused. This was as close to the machine as he would allow himself to get.

He would back off, talking constantly. He wanted to know where the wires were. How the machine worked. Why he needed X-rays. Why he had teeth.

He balked at the machine for a full forty minutes. Forty. Minutes.

I would have given up long before those hygienists did. I'm pretty sure they're angels or something. They finally got Matt to bite the yellow stick and hold still for 15 seconds. We got the X-rays. His mouth is beautiful - no cavities! His adult front teeth are right there and should start to pop through soon. He has something they called "buckling," which I had as a kid, too. His adult teeth are coming in BEHIND his baby teeth. Mine did the same thing - I walked around with a double row of teeth for a while. It was kind of cool in the "I have shark teeth" sort of way.

They may have to pull his baby teeth if they don't loosen on their own - hopefully that won't be necessary. Because after the trauma of the dreaded X-Ray machine, I don't think any of us would live through a Pulling of the Teeth.

I think I owe those two hygienists chocolate. Or dinner out. Or a vacation to Hawaii. They put up with a lot today.

Nolan goes back in at 2:00 tomorrow afternoon. Matt, fortunately, doesn't have to go back in for another six months.

Great Christmas Vacation

2012-01-02T17:22:00.000-08:00

Grandpas are fun

After a remarkably mild November and December, snow has finally descended upon Western NY. We had a wonderful (rainy) Christmas with Dennis's parents, and the boys were glad to have time off from school.

Nolan has been doing quite well lately - no real nausea and only the occasional cough/gag over the past couple of weeks. His nose is running with the sinusitis that accompanies the LPR reflux, though - I don't think we've had more than 2 days free of sinusitis since last September. He has managed to wear his C-Pap all night long for two nights in a row - a record!

His ears are clear, and his hearing actually seems to be better than it has been in a long time. I know his audiogram is probably the same, but he's responding to sounds unaided, and he hasn't done that in a long time. I sometimes wonder if the reflux causes inflammation or other issues with his hearing, particularly with the sinuses involved.

Both boys go back to school tomorrow, and we will get back to our normal schedules. Nolan has his earmold pick-up scheduled for Friday, and then we meet with the pediatric surgeon to discuss the possible fundoplication on Monday the 9th. The travel won't be bad as long as the snow stays away - the drive to Rochester is 3 hours long and the threat of lake effect snow plays a number on my nerves. Hopefully Mother Nature will cooperate and give us a few days off from the onslaught of winter!

Here's to a Happy New Year filled with healthy and growing children!

So Incredibly Busy - Taking a Blog-cation

2011-12-21T09:51:00.000-08:00

Nolan is doing fairly well, though his stomach is acting up quite a bit. It might have something to do with the insane amount of excitement that surrounds Christmas.

He did sleep two full nights with his C-Pap, however, which is wonderful. We're picking up my in-laws today and will be celebrating for the remainder of the holiday break. I'm taking a blog-cation until Christmas is over. In the meantime, here is a really cute rendition of the Christmas Story:

Annual Eye Exam

2011-12-14T11:27:00.000-08:00

Still lacking stereoscopic vision - but the glasses are cool!

Nolan had a dilated eye exam yesterday. Things are looking pretty good! As far as the amblyopia goes, there is still a line's difference between the two eyes - one eye is correcting to 20/25 and the other is correcting to 20/30. That is a really good improvement for Mr. Lazy Eye!

The exam itself shows a slight prescription change. His right eye became 0.5 diopter more nearsighted while his left eye became 0.25 diopter more farsighted. I am very glad we caught the amblyopia through last year's vision screening program, because his eyes are becoming more divergent as far as the prescription goes. This is known as "anisometropia" - when it develops in adults, it is not a big deal. In children, however, it causes blindness in one eye (unless treated).

A little sensitive to light with dilated eyes!

We are able to hold off on patching indefinitely, which is great news. I need to order new glasses to fit the stronger prescription, and he has to continue wearing them during all waking hours (or else the amblyopia will worsen).

The best news is that we don't have to return for a full six months - and the next exam will be free from eye drops!

Pediatric Surgeon Consultation Appointment Made

2011-12-13T11:20:00.000-08:00

It is crisp and sunny outside - I am VERY thankful that this winter is starting off on a mild note. I certainly hope it stays this way, at least through the beginning of January!

I made an appointment for Nolan's pediatric surgery consultation. We have an appointment for January 9th at Strong Memorial Hospital to discuss the potential Nissen Fundoplication. Rochester is a good three hours from our house, so we'll probably go up the day before and stay the night. The science museum looks like a fun side-trip, so we're thinking of taking both boys to the museum on the Sunday before the big appointment.

I am not sure what 'happens' at the consultation appointment, but I suspect we'll review his history and go over prior test results. It is possible that a few tests will be ordered (a gastric emptying scan and possibly esophageal manometry), and we will be able to ask our questions. I found a great article on Complex Child that suggests 10 questions every parent should ask before proceeding with a fundoplication:

1. Does the child have motility problems?

This is the biggest question we can't quite answer. Kids with motility problems do worse with fundoplications than kids who have no motility issues. Another gastric emptying scan and esophageal manometry will be able to help answer this question. Nolan had one GES over two years ago, which showed "borderline" gastroparesis (27% emptying time after one hour). The problem is that there is no national standard for pediatric gastric emptying times. In some clinics, Nolan's time would be considered "normal." In others, it would be considered flat-out delayed. We need to re-examine this angle.

2. Have all medical treatments been tried?

Yes. Nolan has been on adult dosages of a proton pump inhibitor and an acid blocker, and the reflux persists. When on certain antibiotics (which should improve potential motility problems), the reflux worsens or remains the same. He has failed medical therapy for reflux.

3. Has my child had the appropriate testing to rule out other conditions that mimic reflux?

Yes. There are many other conditions that mimic reflux (like cyclic vomiting syndrome, eosinophilic esophagitis, etc.). Other than the potential gastroparesis, Nolan has had all of the recommended and required tests:

a) A pH probe to demonstrate the presence of acid reflux, even on high doses of anti-reflux meds (this is the gold standard for diagnosing reflux).
b) Upper endoscopy with biopsies to rule out eosinophilic esophagitis. Nolan's upper endoscopy was performed two years ago, showed reflux damage, ectopic gastric mucosa, and blunted villi in the esophageal tract. Eosinophilic esophagitis was not present.
c) Gastric Emptying Scan. Yes. The gastric emptying scan was performed over two years ago and showed borderline delayed gastric emptying. This is a question that remains, which fits in with the first question regarding motility problems. He has never had manometry performed.
d) Upper GI Barium to rule out anatomical problems: Yes. Nolan has had two upper GI series performed, both of which showed normal anatomy. There is no malrotated intestines or hiatal hernia present.

4. Does my child have a history of vomiting, gagging, or retching?

Possibly. Nolan occasionally goes through vomiting cycles, similar to what is seen in kids with delayed gastric emptying. He often gags and/or chokes on reflux. He does not retch. Again, this ties into possible motility issues that will have to be investigated prior to consenting to a fundoplication. Since Nolan's type of reflux is not typical GERD but is LPR (extra-esophageal), motility medications will probably not help his situation greatly - both sphincters malfunction and the reflux ends up in his airway. In this case, ruling out motility issues will simply help us decide if the fundoplication will cause more harm than good, though when the lungs are affected, a fundoplication is nearly always indicated.

5. Does my child aspirate his secretions or food?

No. Nolan does not have aspiration problems with his saliva or with food. Kids who aspirate their own secretions have a harder time following a fundoplication - and a fundoplication cannot "fix" this problem since the aspiration is of secretions above the level of the reflux. This is not an issue with Nolan, fortunately.

6. Does my child have a history of neuro-irritability, visceral hyperalgesia, or chronic abdominal pain?

No. Nolan is often nauseated, but doesn't seem to be in acute pain very often. This is often seen in children who have neurological impairments, and a fundoplication may worsen abdominal pain in these children. This doesn't apply to Nolan.

7. Has a GJ Tube been tried?

This question was written primarily for children who are receiving a fundoplication for reflux associated with failure-to-thrive issues. In these children, a G-tube is often already present, and the child is not feeding orally. For these kids, a GJ tube can bypass the stomach entirely and prevent reflux, with fewer side effects than a fundoplication, particularly for children with neurological issues.

In Nolan's case, this is non-applicable. While on the low end of the weight charts, he is not failure-to-thrive. His reflux causes apnea and airway issues, but we have the weight issue under control. He eats orally and does not have a gastrostomy tube, so trying a GJ tube is inappropriate in his case.

8. Does my child have a history of esophageal atresia?

No. Kids with esophageal atresia often perform poorly with fundoplications. This is non-applicable to Nolan.

9. Does my child have dysphagia or feeding problems?

Not really. When in the middle of a bad reflux flare, Nolan will avoid solid food. He was dysphagic as a toddler (difficulty swallowing) due to the reflux, but this has improved greatly with medication. In general, he doesn't have feeding difficulties. There are still questions about his esophageal motility, which ties in to the first question on this form.

10. Is my child under two years of age?

No. Kids under the age of two often "outgrow" reflux. Children over the age of 3 or 4 are nearly all severe, chronic, life-long refluxers. Children under the age of two should not have a fundoplication unless the reflux is life-threatening (apnea, airway, or severe failure-to-thrive are present). At the age of 4 years, 4 months, Nolan's reflux has only gotten worse with time. He will not outgrow the reflux, and faces lifelong high-dose medical therapy, which has proven to be ineffective for him.

The questions provided by this article are great, because it helps us to narrow down the relevant questions prior to consenting to the surgery. Really, the biggest outstanding question is regarding motility issues - we need to get more definitive information on this problem, particularly since Nolan had a questionable gastric emptying scan and a longer-than-normal bolus transit time on his last pH probe.

Well Child Visit/Some Good Days/Phone Calls to Make

2011-12-12T06:15:00.000-08:00

Last Tuesday, Nolan had his four-year-old well child visit.Yes, he turned four in August... but insurance mandates that physicals be 365 days apart, so they have been slipping farther and farther away from his actual birthday!

The little guy is doing well. The vital stats:

Age: 4 years, 4 months
Height: 39.5"
Weight: 32 pounds
Clothing Size: 3T

He's meeting all developmental targets, and at this appointment his nose and ears were clear. His lungs were a little "wet" sounding, but he has been coughing a lot with reflux lately. His weight percentile is down a bit from last year. At his peak, we had managed to get him to the 24th percentile. Now he's in the 14th - still great, but obviously we keep a sharp eye on his weight and supplement with DuoCal in yogurt when he stops eating solid foods. His height is great - 22nd percentile.

The rest of last week was wonderful. His reflux seemed to settle a bit, and he had a clear nose and ears. We don't often see him "clear" anymore, and the difference in energy level between a healthy Nolan and a refluxing Nolan is astonishing. When he has energy, he doesn't know what to do with it and is absolutely HYPER.

Saturday night, he complained of a headache. He was in a lot of distress, so I put him to bed with Tylenol, and left the C-Pap off. With a full-face mask, we don't take any risks if he might get sick in the night.

He woke up in the morning and was fine, but coughing and gagging a lot. We're now measuring the "good days" in shorter and shorter time increments. Reflux, welcome back.

We went to the Christmas Tree Farm and Nolan was extremely difficult the entire time. His energy was gone and he wanted nothing to do with the process.

And then there is the sinusitis. His nose is running like a leaky hose again. At least his ears are clear.

He almost never naps, and he slept on the way home. We were hoping he'd get a good nap in, but he woke as soon as we got home from the tree farm.

Mr. Cranky spent the rest of the day watching cartoons. We left the C-Pap off again last night, so that we don't exacerbate the reflux (in Nolan's case, the blowing air just forces the infected material in his sinuses into his lungs and ears). Hopefully this will be a short bout and he'll be "clear" again soon.

In the meantime, I have to call the pediatric surgeon today to set up an appointment. I just hope that we get an appointment on a day without snow - I have no desire to drive to Rochester in a blizzard!

ENT Visit/Sinus CT Results/Fundoplication Referral

2011-12-07T12:01:00.001-08:00

Why does the flurry of doctor appointments always seem to come over the winter months?

On Monday, we went to see Nolan's ENT to discuss his sinus CT results. We had been given a heads-up that the CT results showed sinusitis, so that wasn't really a surprise. His sphenoid and maxillary sinuses show evidence of chronic sinusitis, a result of the ever-present acid reflux that inflames the area. The mucus can't drain, so it becomes infected. Since the sinusitis is caused by the reflux, we can't really treat the sinus disease until we get the reflux under control.

We also discussed Nolan's ridiculous ear infections. We now know why the antibiotic drops can't cure the infections. The child has stomach acid coming out of his ears - there is no amount of antibiotic therapy that is going to work in that scenario. You can clear the resulting infection, but the inflammation and drainage are going to remain.

Of course, this means that our attempts to treat the symptoms of Nolan's acid reflux are failing. We can treat the apnea with a C-Pap. That is, when Nolan can tolerate the C-Pap mask and isn't too nauseated at night. We have removed his tonsils and adenoids, trimmed his lingual tonsils, had his larynx trimmed and his epiglottis tacked up. We have had four sets of ear tubes inserted, and supplement his diet so that he can maintain his weight, even when he won't eat. We have him on adult dosages of two different anti-reflux drugs. And none of it is really working.

And then there is the nausea and stomach pain. We have a four year old child who begs us for more medication. Who asks for a bowl to hold onto at bedtime. Who says, "I feel sick" several times each day. Who gags and coughs from reflux on a regular basis.

With his lungs, airway, ears, sinuses, and nutrition affected, avoiding a fundoplication is no longer feasible. He has been referred to Strong Memorial Hospital in Rochester for a Nissen Fundoplication evaluation. We don't have any appointments yet, so I have no idea when the ball will get rolling. I don't know what the evaluation process involves, or what the overall success rates are for a child with Nolan's particular form of acid reflux.

We do have a bazillion questions for the surgeon, and I am busy formulating a list.

On the plus side, the ENT did call over the audiogram from his hearing test on Friday, and his hearing is definitely stable. We'll take all the good news we can get around here!

Stable

2011-12-04T12:19:00.001-08:00

No more pictures, Mom!

Nolan has been having a lot of trouble hearing lately, so we were concerned about his test on Friday. As it turns out, his hearing is stable - changes of 5dB here and there, but the audiologist was happy to report a similar audiogram to the one he had in July.

I suppose his difficulty in hearing simply comes from the deep end of a moderately severe hearing loss - he still can't hear his father (even aided) unless Dennis repeats words over and over again. His distance hearing is abysmal - he can hear me if I'm within 6 feet (aided) but outside of that he is lost. Frustrating, but something we'll just have to live with. Thank goodness for the FM system, because he wouldn't hear a thing in school without it.

I should get the report sometime next week. I didn't actually see the audiogram, so I want to see if the "5dB" was a 5dB loss across the board, or if there were 5dB variations up and down. It makes a difference, because Nolan has lost hearing very slowly: 5dB + 5dB + 5dB adds up over time. If the 5dB is up and down, then I won't be concerned - that's just normal variation.

He does have granulation tissue on his left eardrum - not that surprising considering the recent infection. Otherwise, his tubes are open. We took new impressions for earmolds, since the right earmold had a small rip. He hasn't actually outgrown them (the last set were made before we went to John Tracy, over a year-and-a-half ago). With all the infections, however, I wanted new earmolds. I don't know if the old ones are harboring any infection, but why take the chance? We'll pick up his new molds on January 6 - for the first time, we didn't get blue-and-white swirls. Nolan wanted something different!

We're off to the ENT tomorrow to discuss the "abnormal" sinus CT scan results. I also have to ask about additional medication for the reflux, since Nolan is frequently nauseous throughout the day and begs for relief. I'm going to ask if he can have children's Tums (on top of his high-dose Nexium and Zantac).

In the meantime, we're happy for hearing stability. We take good news where we can get it!

Finally, Dry Ears

2011-12-01T17:59:00.001-08:00

Watching the Grinch Who Stole Christmas

Nolan's ear dried up on Wednesday. His hearing still seemed "off" in that ear (he should still hear unaided in that ear if you are within three feet or so and speaking loudly). His hearing seems to have improved today, though, which is wonderful. He has a hearing test slated for tomorrow afternoon, and we really wanted clear ears and a return to his standard level of hearing. His hearing is pretty crummy anyway, but we'd rather see his normal moderately-severe level than the severe-profound levels that an infection incurs.

The Teacher of the Deaf (TOD) for the elementary school called and asked about budgetary requests for next year. Nolan already has his FM system, so I couldn't really think of anything to add to his IEP. She did put in some extra money for batteries, since the FM system drains batteries fairly quickly. The only "snag" that we might hit is if we change hearing aids and need different receivers for the FM system, but I am pretty sure the receivers we have will work with nearly all hearing aid brands. Plus, the next set of aids will probably be Phonak, which happens to be the same brand as his FM system - they should function seamlessly.

I did have a laugh when she said that Nolan could just use a speaker soundfield system if his ears are infected. The school's only other child with a hearing loss has a very mild loss in the high frequencies. If Nolan has an ear infection and is unaided, he can't hear anything. Period. He drops down past the 90dB range and is functionally deaf - there is nothing to help that situation! Even when his ears are clear, he can only understand words projected at a 90dB level (unaided) - his last test showed 88% accuracy with words at 90dB. Unless she's going to set up a speaker to blast out rock-concert levels of sound, it won't work. I have a feeling the school will need a bit of an education to deal with his level of loss - he has to use his personal FM system at all times in the classroom. Nothing else will allow him to hear in noise (and if he's unaided, he simply can't hear conversational speech at all).

In any case, hopefully Nolan's hearing test tomorrow will show stability. I'm worried about that right ear, which has been having difficulty for the past two months. It was really a problem when the little guy (temporarily) lost the rest of his hearing in the left ear - let's just say that we have been using a lot of sign language in the classroom over the past 2 weeks, because he simply couldn't hear a thing. His right ear was also the first to drop the high frequencies back in 2008 (the left ear fluctuated up and down and then finally followed suit in 2010). The right ear seems to precede the left ear when we do see changes in hearing, so I will be greatly relieved if that right ear is nice and stable!

Still Leaky

2011-11-25T17:48:00.001-08:00

Nolan's Ciprodex drops were supposed to be used for seven days - unfortunately, the time period has come and gone and his ear is still infected. Last night at dinner, there was some very obvious nastiness coming out of his left ear.We went back to the pediatrician today, and the doctor decided to place him on Floxin drops for a week to see what happens. I'm not exactly thrilled, since we've "been there, done that" before and he often ends up with a very persistent ear infection. We'll go with the drops, though - maybe it will help his ear clear out. If it doesn't we see the ENT on December 5, anyway.It looks like his left ear will be without sound for a minimum of two weeks. We can't put that aid back in until the infection is entirely gone. In the meantime, he's been going without his ears after bath time to prevent moisture build-up from the earmolds. This leads to a lot of evenings where Nolan declares that our TV set is not working.

"Mom, turn it on!"

Sigh...

He is doing "OK" with the right hearing aid in place, at least when I am speaking. He can't even detect Dennis's speech with it. We have a lot of translation going on in our house right now, where I have to repeat everything Daddy says.

Dennis: "Nolan, you are going to the doctor today with Mommy."
Nolan: "What?"
Dennis: "You are going to the doctor with Mommy."
Nolan: "What?"
Me: Daddy said that Mommy is taking you to the doctor."
Nolan: "Oh, WHY?"
Dennis: "Because your ear is sick."
Nolan: "What?"
Dennis: "Your ear is sick."
Nolan: "WHAT?"
Dennis: "Exactly."

I did get a call into his audiologist. She is going to try to squeeze him in for December 2, which would be great because our insurance changes on January 1. The new insurance policy only allows one hearing test per year, so we're trying to move January's scheduled test into December so we don't exhaust our "allowance" at the beginning of the year. Nolan is still on an every-six-months schedule because his hearing has never been stable over the course of a year.

In any case, it would be really nice if the ear drops would clear out the infection before his hearing test (if we manage to get in on December 2). An infection drops his hearing level by a lot, so we need his ear clear of fluid by next Friday. In the meantime, we are enjoying our Thanksgiving break. We took the kids to see The Muppets, and Nolan told me that he really likes The Muffins. We love having this time off from work, school, and other obligations - the quiet mornings and playtime with the kids are wonderful!

Happy Thanksgiving, 2011

2011-11-23T11:47:00.001-08:00

The Dragon Slayer

The day before Thanksgiving is divine. No school, cold weather, and a lot of free time for the boys to play. They have spent all morning playing with Legos and their Imaginext toys, while I have been cleaning and making cranberry sauce.

We are thankful for so much this year. We have health insurance. We have two beautiful boys who are spirited and happy. We have a house. A dog. Access to good medical care. Clean water to drink. Heat. A strong, cohesive family. Friends. Enough food to eat. Access to a high quality school to educate our children.

There are so many things to be thankful for this year. Signing off for turkey day -

Happy Thanksgiving, everyone!

Party Time (and sinus CT results)

2011-11-22T17:51:00.001-08:00

I was able to volunteer in Nolan's class today for his Thanksgiving feast - it was a lot of fun! Nolan was a Pilgrim, and the children sat down with all of their friends for some "friendship soup" (everyone brought a can of alphabet soup to cook together, with the extra cans donated to a food bank).

The little guy's ear drops have started causing agony again - he is one of those kids who has extreme pain with the application of the Ciprodex drops. I have no idea why - I thought we were going to be fine this round, but now that a lot of the fluid/infection has started to dry up, the drops are actually penetrating his tubes and reaching his middle ear space.. and causing pain. I hate to see him in that level of pain - you know it is bad when a child asks for a shot instead of a few ear drops! Unfortunately, we can never get the same doctor twice at our pediatric group, so no one has any idea about this problem. And convincing a new doc that your kid has intractable pain with the application of ear drops is impossible. The paradigm is: "patent tubes = ear drops." I have to talk to our ENT about it, because the little guy has too many infections each year to go through this every time.

Nolan is still without an ear, and unfortunately the infected ear is his "good ear." While both ears were the same on his audiogram last July, we know the right ear has been having some trouble lately - and the left ear is infected. He is coping fairly well, though he is getting none of the Ling sounds correct. He can discriminate (or figure out) whole words, though, so he's struggling along. We have been using sign language to communicate since he can't hear well with the one ear - our sign isn't very good, but at least we can ask him if he needs to go to the bathroom or clarify basic instructions that he can't hear. To top it all off, his right aid keeps getting impacted with "gunk," which causes it to quit working. His last ear infection lasted for 6 weeks, and that is a long time to go without adequate hearing. Hopefully this one will heal up faster.

Our little pilgrim's sinus CT report came back, and the nurse practitioner at our ENT's office called with the results. They show sinus disease (chronic sinus infections, likely from the reflux), so we will discuss that at our appointment on December 5. I am very glad they gave us a heads-up, because I hate getting bombarded with new information and having to make decisions on the same day. We can review the information and do some research before making any surgical or medical decisions.

Our medical insurance is changing, too, and the cost of Nolan's hearing tests will increase. The new insurance will only pay for one per year, and Nolan has been having them more often due to decreasing hearing levels. He is due for a test in January, but I am going to call our audiologist to see if we can get that bumped up to December, before the new insurance takes effect. Of course, with the chronic ear infections, it will probably be a moot point - we'll probably have to repeat the test in January, anyway. I hope and pray the ear infection(s) dry up and his tubes stay clear so that we can get an accurate reading before the end of the year.

Ear Drops and Flu Shot Drama

2011-11-19T05:33:00.001-08:00

'Tis the season.. for germs.

Our trip to the doctor went well yesterday. His left ear is definitely infected, so we decided to go with Ciprodex drops to see if they would work to cure the infection. The good news is that the left tube is still in place - while it had been clogged, the infection pushed its way through the clog and he still has a functional tube. Usually, an ear infection with a clogged tube just perforates the eardrum and wreaks havoc on his ear!

While we were there, he also got a flu shot. He was not very happy about that decision - he screamed bloody murder. On our way out of the building, he yelled at each person in the pediatrician's office: "I don't want a SHOT!" I had to stop by Rite Aid to pick up the ear drops, and he really hammed it up - he couldn't walk, he claimed, and he hopped on one leg. I would have believed him, but he kept forgetting which leg had gotten the shot, so he alternated the leg he was hopping on. Still, he let everyone know that he was the Boy Who Had Been Maimed By the Flu Shot.

Nolan has been going without hearing aids because of the infection (the right ear looked a little "wet," so we have been leaving that one out as well). It has been a wee bit difficult without the aids. Like an idiot, I decided to take the boys to the mall to exchange something, and Nolan was rather obnoxious.He ran around the mall and communication was extremely difficult. We do use some "survival sign language" with him, but my hands were filled with bags and I couldn't even sign to him to stop running around like a maniac. Any four year old might go crazy in a crowded Bath and Body Works shop, but it is especially frustrating when communication is limited. We ended up abandoning the trip and heading home. His right ear is looking fairly decent this morning, so I will probably put that aid back in today and hope for the best, infection wise. Those earmolds trap moisture and really exacerbate infection issues for Nolan!

I threw both boys into the bath last night and let them play with their toys - a few minutes later, I hear horrendous screams coming from the tub. They had been playing Lion King, and Nolan squirted half a bottle of shampoo into Matthew's eyes (in the movie, Scar the lion throws embers into Simba's eyes). Poor Matthew was in pain, and I finally got him sorted and feeling better. Nolan knew he was in trouble, so he threw himself to the floor and started flailing around yelling, "Oh, the shot made me do it! The shot made me do it!"

Nice try, kid.

Hopefully today will be better with both behavior and hearing!

Rats (Another Ear Infection)

2011-11-18T06:14:00.001-08:00

Nolan kept complaining that his ear was whistling the other day. I checked the hearing aid several times, but I couldn't hear any feedback. I did a listening check, and everything sounded A-OK.

Then, last night, he started screaming in pain. After a little while, he said, "I feel better now," and a thick stream of horribleness started pouring out of his ear. Another ear infection, another eardrum rupture.

Off to the pediatrician in the morning. Nolan insists that his ear is "all better" and that he doesn't need to see the doctor. I disagree...

Not sure if they'll try drops this time or an oral antibiotic. We prefer the oral route, since the drops cause so much pain. Also, I'm pretty sure the tube in that ear is gone, so the drops might not be as effective.

Ugh.

A Nice, Easy Appointment

2011-11-16T10:55:00.001-08:00

Nolan's ENT ordered a CT scan of his sinuses since they have been infected for the past two years (another consequence of airway reflux). We headed up to Buffalo Children's today, with Nolan complaining heavily about missing school.

It took a lot of convincing to get him to lie still on the table - once he saw the papoose board, he started to wig out. No surprise there, since that apparatus has always been connected to painful procedures in his experience - the technician walked him through the procedure with his hearing aids and glasses on, and then I took his "ears" and "eyes" off for the actual procedure. The little guy did great!

Listening to the radiology technician before the actual scan.

The entire scan probably took 2 minutes - sinuses are faster than imaging the inner ear, and I was extremely thankful that he can lie still and cooperate. Kids who can't lie still have to be sedated for the procedure, and that adds a lot of stress to a simple test! Nolan did beautifully and I put his hearing aids back on as soon as I was allowed back into the room. It took longer for the radiology department to burn the CD of his CT scan images than it took to actually perform the test!

We left Buffalo, waved goodbye to Canada (across the Niagara River) and stopped by the McDonald's on I-90 for lunch. Nolan loves watching the trucks pass under the bridge, and a nice man stopped by and showed him how to pump his arm to get the trucks to blow their horns. Nolan thought this was really fun - I had to drag him away to get lunch!

McDonald's is a post-doctor treat for the little guy, and he ate his apples with gusto. He wasn't very interested in the chicken nuggets, but was very happy to have some one-on-one time with Mommy.

I have the CD with the images of Nolan's sinuses - it is sort of weird to see your kid's sinuses on an X-Ray!

I still have to make the follow-up appointment to discuss the results (and to get the ENT to look at Nolan's ears - they've been bothering him, probably due to wax). Hopefully we'll get a week or two off before we trudge back up to Buffalo. I don't mind the trips, but I am less fond of traveling when the snowy weather hits!

Growing (Sleep Neurologist Update)

2011-11-15T12:17:00.001-08:00

Nolan had an appointment with the sleep neurologist today. It was all good news!

Age: 4 years, 3 months

Average C-Pap Compliance: 5 Hours/night

Height: 40"

Weight: 33 pounds!

The little guy is refluxes constantly, but we have managed to keep his weight up by letting him snack incessantly (he eats a tiny amount all day long). The C-Pap is increasing the quality and duration of sleep, which allows for the production of growth hormone. We should see continued weight and height increases as he continues with the therapy. We don't have to return to the sleep neuro for another three months.

I love these types of appointments!

Tennis Boy

2011-11-12T11:41:00.001-08:00

Nolan started his "Tot Tennis" class this morning. He was so excited to start the program that he was literally dancing as he waited for class to start. He's definitely the smallest kid in the class, but he is having a blast. The program is really perfect for the smallest kids: they learn some skills and do a lot of running and play various ball-throwing games.

I went to his parent-teacher conference at the elementary school yesterday, and he is doing really great in class. Academically, he's doing very, very well. Motor-skill wise, he is lagging a bit. We're continuing to monitor and he may need OT at some point. On the other hand, a lot of the coordination and fine motor skills could simply be developmental (there's a pretty big range of normal at the age of four). We'll see how he progresses as the year goes on!

Nolan's hearing is definitely not quite up to par. He is asking "what?" fairly frequently and is still missing a few Ling sounds during listening checks. His right ear seems to be particularly troublesome - he was holding a large jingle bell to his ear and was shaking it, and started crying because his bell was broken.

Unfortunately, the bell is perfectly fine... his right ear, not so much. He has a CT scan on Wednesday for his sinuses (suspected damage caused by acid reflux), and then we will schedule a follow-up with the ENT. I'll ask her to take a look at his tubes and his ears at that appointment, just to be sure there isn't some sort of infection brewing. I know his left ear has a clogged tube, so we may end up back in the OR before Christmas to replace those tubes.

We also have a sleep center appointment next week, so we'll be fairly busy with trips to Buffalo. Before the crazy week of travel and appointments starts, we are enjoying the snow (yes, snow) and a quiet weekend at home.

The Case of the Missing FM

2011-11-04T12:16:00.000-07:00

We've had a rather busy week - Nolan's gut seems to be doing well, though his missing "s" sounds haven't returned. No sign of an ear infection is present, so I suspect we'll see another drop in those high frequencies when his hearing is tested again.

On Wednesday, Nolan came home and I had a few errands to run. No big deal, but I didn't check his backpack until 4:00pm, when I discovered that his FM transmitter and its case were missing. After some questioning, I discovered Nolan had had a substitute teacher for the day.

I figured it had been left in the classroom - the only problem would be the following day, when the battery in the unit would be discharged. I was also a wee bit concerned that Nolan had taken the unit out of his backpack and tossed it somewhere... you never know what to expect with a four year old!

Fortunately, his teacher was back to school on Thursday and we quickly located the FM transmitter and case. The Phonak Inspiro only takes about 10 minutes to fully charge, and I had the unit set up and running by the start of class. I have a feeling we'll be playing "hide and seek" with the FM system every time there is a substitute, so I'll be sure to check his backpack immediately after school each day. It wouldn't have helped much in this case, though, because the unit was actually located on top of an 8-foot high cabinet.. I would never have found it while searching after school!

Nolan has been quite full of stories lately, too. He told me that it was "G" week and the teacher surprised them by bringing in Golden Retrievers. According to Nolan, there were three puppies and a mommy dog in a large crate, and the children got to take turns holding and petting the puppies.

I told his teacher how awesome she was for bringing in puppies for the letter of the week, and she simply said, "Puppies?"

Hmm... as it turns out, there were no puppies, but we do have a four year old boy with quite the imagination! Perhaps he's just trying to plant some ideas for when "G" week rolls around!

Happy Halloween!

2011-10-31T13:45:00.000-07:00

The kids had a great time watching their pumpkins get scooped out last night. Nolan wouldn't touch the pumpkin guts for love nor money.

Both kids had Halloween parties at school today. Nolan is Batman and Matt is Jango Fett (from Star Wars, if you aren't up on your intergalactic characters).

Batman with some friends.

Jango Fett on Parade.

We'll see how Trick-or-Treating goes tonight. Nolan's gut appears to be heading toward shut-down territory. He wouldn't eat lunch and he had an explosive "event" at his afternoon X-Cel preschool class, which involved a quasi-bath in the school sink and a LOT of Clorox wipes. It wasn't pretty folks.

He seems fine otherwise - oftentimes his gut rebels and he just gets right back to playing. We'll go on our candy quest later this evening and hopefully his intestinal tract will behave.

Please Be a Cold, Please Be a Cold...

2011-10-28T12:35:00.000-07:00

Nolan has been missing the /s/ sound for a while (we haven't gotten a detection of /s/ in the Ling sounds in about 2 months), but the past couple of days have brought a lot of sound confusions and "WHAT YOU SAY?"

Here was today's Ling sound check (hearing aid microphone only, no FM system, at a distance of six feet):

He does slightly better with his FM system (he only confuses /ooo/ for /mmm/ and omits /sss/ with the FM system on - it eliminates distance as an issue, since my mouth is within 8" of the microphone).Still, the loss of /sh/ and the confusion of the other sounds is fairly new. I hope we get them back!

What a Week!

2011-10-26T10:24:00.000-07:00

Nolan is doing great. His energy levels remain high (sometimes a little too high) and his reflux has been fairly manageable the past week or so. His school bus brings home a very happy boy, who simply loves all of the activities his teacher comes up with. Last week was "fire week" and they did the letter "F" and fire safety drills. He also made the really cute dalmatian hat in the picture. This week is pumpkin week - they have been determining if pumpkins will float (they will), carving pumpkins, and learning about the life cycle of pumpkins.

I, on the other hand, have been having a really... "interesting" week.

On Monday, just before Nolan's school bus arrived, I let the dog inside. I went upstairs to clean up the kitchen, then walked downstairs. Something squished. And then something crunched. Looking down, I saw what could only be described as The Massacre of The Squirrels. Somehow Casey had smuggled his prey inside, and it was now all over the living room.

To prevent the trauma of a young preschool aged boy, I had to dispose of said squirrel parts quickly. We had no paper towels (and paper towels were not up to this job), so I ran for the shovel. And carpet cleaner. Fortunately, I managed to fling the bulk of the squirrel into the woods before Nolan's bus arrived. Unfortunately, I have bad aim and the squirrel ended up hanging from a tree. When Nolan came home, I fed him lunch upstairs and rushed him off to his X-Cel class, to prevent him from seeing squirrel-parts-in-a-tree and squirrel-remnants-on-the-carpet.

I was extremely thankful that he was safely ensconced in preschool as I worked to clean up the heavily stained carpet. It was horrid. After shampooing the carpet twice, I figured I would hit the main level of our house. Might as well, since I already had the equipment out.

As I shampooed the upper level, I realized the dirty water container was full. I unlatched it from the machine, and carried it to the kitchen. That's when the lid fell off, causing the contents of the bucket to spill all over the tile, carpet, and walls. There's nothing like squirrel water splattered all over the house to make the day a little brighter! Since it was time to pick up Nolan, I simply threw bath towels over the whole mess and ran to the preschool.

Once home, I picked up Matt from his bus and then picked up Dennis from work (we were down to one car). Dinner was at Friendly's that night, because Squirrel Water = dinner out. It's a simple equation.

Tuesday morning brought a flood of activity to the house - literally. We woke up to a very flooded basement - about a foot of water was standing in the room. The water line to our ice maker had become detached in the night, and the water pump continually pumped the water onto the tile floor (which dripped right down into the basement). Those things can produce an amazing amount of water. The majority of Tuesday was spent with a shop vac and a sump pump. On the plus side, this water did not contain any traces of squirrel.

The rest was spent in the Walmart vision center - we took Nolan's bent frames in to get them straightened out, but the lab tech broke them when he tried to straighten them. Nolan walked out of the store with a brand new pair of glasses, after we convinced them that they really needed to be replaced immediately since he has amblyopia and we don't want to risk the vision in his right eye.

Wednesday has been sedate so far. No squirrel massacres, no flooded house, and all vision/hearing equipment is intact.

The week can only get better!

Loving School

2011-10-21T06:16:00.000-07:00

Nolan has been doing wonderfully this week. Other than an incident at 4:00am when the C-Pap machine went crashing to the floor (inspiring a brief panic over a possibly-broken machine), the week has been entirely uneventful and Nolan has been full of energy.

He adores school. Absolutely adores it. Every morning, he takes off running for the school bus with a smile on his face, and he comes home every day with stories of his adventures. Here's a sampling:

"I played in sand today. I didn't play with C---- because he wasn't there. I don't like L------ because she hits me. She goes in time out. I like Loghan because she is nice to me. We like to play."

"We paint today. We make a fire truck."

"There is a book in my backpack. Everybody make it. B---- likes pink apples. I like green apples."

I absolutely love these days. Matt is enjoying school, too. I did have a bit of a panic attack/mama bear moment when this came home in his backpack:

The bright red "Not Acceptable!" caused deep concern. There was no explanation for the bad mark, and since Matt is only five years old and can't read "long" words like acceptable, the note had to be meant for the parents. I wasn't sure if Matt's coloring in the circled objects was the issue, since the written directions simply say to circle the like objects. I felt it was a little harsh to criticize a five year old child for some extra doodling.

Then I wrote to the teacher and got the rest of the story.

Apparently, the verbal instructions were to circle the similar objects and then color the pictures with their best work. Matt decided to take the slacker route and scribbled quickly to get the assignment over with.

The teacher had discussed "not doing his best work" and they both decided the work was "not acceptable" prior to writing it on the paper. Matt has been doing his "best work" ever since, so this was a good lesson for him. I do wish I had had the explanation before the mama bear claws came out, though!

We are dealing with a bit of a conundrum with Matt. He's very "able" in the academic realm. By "able," I mean he came to me the other day and said, "Mom, 3 + 3 + 4 is TEN!" His next sentence was, "I think 9 plus 10 is nineteen - is that right?" His emotional and maturity level is at the kindergarten stage, but he needs more academic stimulation. Fortunately, his teacher is willing to provide it. He was able to do the "circle the like objects" thing at the age of two, so these worksheets are more "busy work" for him than anything else. He really needs first grade level math worksheets, so we're working on getting him more appropriate work.

Matt absolutely loves his class, and particularly loves gym. I had forgotten about games like Steal the Bacon. It is so fun to hear him talk about these things. I wish I could have foreseen this talkative, bright boy when he was so speech delayed at the age of three. It would have relieved a lot of worry!

Back to "Normal"

2011-10-17T13:22:00.000-07:00

Nolan's most recent bout of reflux seems to have disappeared. Over the weekend, we had two great C-Pap nights, and Nolan managed to keep his mask on until 6:00am (Saturday) and 8:15am (Sunday). Last night he didn't make it past midnight, but that's how it rolls with C-Pap therapy and tiny children. We take the good nights when we can get them!

Sadly, we didn't make it to Zoo Boo, because it was raining, 45 degrees, and the wind was gusting at 50mph. We'll try again this weekend: the temperatures are supposed to be lower, but it won't be raining.

The weather did cooperate for Nolan's field trip today. His "X-Cel" (private preschool) class took a trip to the Jamestown Audubon Society. The kids found maple, oak, and apple leaves. They also found chestnuts and made rubbings of tree bark and leaves. It was a wonderful afternoon (and I was thankful for the 100th time for his trusty FM system)!

Here's to a happy week ahead!

Hanging in There

2011-10-14T07:55:00.000-07:00

Nolan is doing surprisingly well, considering the small amount of food he's taken in over the past few days. His appetite has dropped, but he is still doing pretty well at school. He did tell his teacher that his tummy wasn't working, and he has developed a wet cough. I think we have a combination of reflux-flare up and cold.

In any case, the C-Pap has been put on hiatus yet again. Nolan keeps ripping it off, and we're a little more cautious with it during a reflux flare up. If he vomited into the full-face mask, it could be disastrous. He had pulled it off 4 times by 10:00pm last night, so we let him sleep without it, for fear that his stomach would rebel in the middle of the night.

He did eat half a waffle this morning, so hopefully we're seeing the other side of this episode. This hasn't been anywhere near the awfulness of the August situation, thank goodness. He has some energy and is still eating a little - the returning appetite is a good sign.

I'm still waiting for insurance approval for the CT scan of his sinuses. I haven't called the ENT to question the status of this one, because I'm really unsure of what we will do with the information. Is it worth putting him through surgery to fix the sinus issues when the reflux will simply wreak havoc again?

In any case, I'm hoping to see the return of a healthy appetite and more energy for the little guy. We have Zoo Boo this weekend (trick-or-treating at a local zoo) and we might want to find a few more letterboxes if the weather cooperates.

It's Baa--aaack!

2011-10-10T18:31:00.000-07:00

Nolan started showing decreased energy on Saturday. No big deal, but we kept an eye on it. Sure enough, the gagging and the coughing followed.

Then the reflux laryngitis appeared, making the little guy sound as if he smokes three packs per day.

I thought (and hoped) that the nasal C-Pap mask contributed to the last reflux flare-up in August. I was wrong.

It's back, and it is back in full force.

You can hear it in his voice. The hoarseness is caused by acid damage to his voice box.

He spent much of today crying and in bouts of pain. His entire gastrointestinal system is rebelling. He kept holding his hands in front of his mouth, and he told me repeatedly that his stomach wasn't working.

I hate this. I hate what it does to him.

Hopefully this will be a short flare-up. He is still eating well, so that is a big plus. Right now, it is just the pain, cough, gagging, and hoarseness that seem to be causing problems. With the laryngomalacia, he is wheezing and has loud stridor at the moment, too.

The pain isn't constant, which is good. He'll be going along just fine, and then an attack will hit him and immobilize him. We went letterboxing today (a pastime which involves a very short walk to find a hand-carved stamp - this one was hidden by a local train station), and he did fine for part of it. Then the coughing and gagging started, so we headed home.

I can only hope that this flare-up is brief, and that he'll be back to himself very soon.

We see the ENT again in November, and we need to ask her what the long-term damage to his vocal cords is going to be. He has worked really hard to listen and speak, and it frustrates me that the reflux is taking away his sweet little voice.

Holy Growth, Batman!

2011-10-02T06:40:00.000-07:00

Nolan's usage of the C-Pap is increasing - his "short" nights with the machine end at about 2:00am (he wakes up irritable). His "long" nights last until 7:00am (or later) - which means he is sometimes getting 11 hours of clear breathing.

The results are incredible. We have never seen Nolan with so much energy and life. The fatigue issues have disappeared, and he has energy through the entire day.

Sometimes he has TOO much energy

He also has rosy cheeks, and a happier attitude. He doesn't cry all. day. long. He giggles, plays tag, and can actually walk through the store without having to be carried.

At this rate, he is going to grow like never before. His little body is finally able to produce the right amount of growth hormone, and he is getting the proper amount of REM sleep (and oxygen) at night. I can't state how much I love this.

He does have a chronically runny nose and some chest congestion, but the trade off in energy is well worth it. Grow, Nolan, Grow!

As a side note, we did get his microarray results about a week ago. I was waiting for the official written report in the mail, because over the phone they told us that it was "normal, but with copy number variations (duplications) of unknown clinical significance." The report came in the mail, and it simply says, "normal." So essentially, his chromosomes look fine and aren't the cause of his problems - though this doesn't rule out a point mutation or other genetic issue below the chromosomal level. And those duplications may prove to be "problematic" in the future (i.e. they may find another child with the same physical issues and the same duplications, which would give the duplications clinical significance).

Since Nolan is healthy at the moment, we don't care about the "cause" of the problems as much - we feel like we have found our solution to his apnea and fatigue in the C-Pap therapy. Hopefully nothing new will pop up - and if it does, we'll just take it as it comes.

In the meantime, we are celebrating and loving life with our energetic little boy!

Another Great Week at School

2011-09-30T10:31:00.000-07:00

The letter "C" was the focus this week, and Nolan had a blast. His Universal Pre-K class is very creative with projects: the kids made cats from cups, glued cotton to a big letter "C," and sang C songs (The Color Train and Color Farm).

The longer-term theme is apples - so the class read about Johnny Appleseed and made a Johnny Appleseed puppet. They made applesauce in class and tasted some cider*, along with creating apple prints from halved apples. Nolan comes home exuberant from all these activities. He just loves them!

Next week is going to be even more fun: the class is going on a field trip to a local cider mill. Nolan is so excited he can hardly wait!

*While Nolan is normally not allowed apples due to dietary restrictions, we allow him to partake in classroom activities with these foods - we just reduce the volume of cider, etc.

In Which Nolan Gets to Skip All the Screening Tests

2011-09-29T12:22:00.000-07:00

The beginning of school has brought a full load of screening tests for every child in Universal Pre-K. First came the speech screening. I noted that he was already receiving services for an articulation delay secondary to his hearing loss. They went ahead and talked to him briefly, and simply noted "we understand he is already receiving services - he is a talkative little guy!" Basically, he got to opt out of the screening process and play with toys.

Next week is the vision screening by the Lion's Club. Since we found his vision issues last year, he gets to opt out of this one, too.

I'm waiting for the hearing screening form to come home - we should really just get a big form at the beginning of the year that says, "Um, yup - Nolan has everything you are going to be screening for. Screening tests not needed, thank you!"

Nolan doesn't mind skipping these screening tests - I suppose the one upshot of having speech/hearing/vision issues is that you get a little more time to play at the Lego table while your friends are getting tested!

September ENT Visit

2011-09-26T12:49:00.000-07:00

I took Nolan in for his routine ENT visit. Of course, with Nolan, there is almost never anything "routine" since he has a plethora of ENT problems.

Fundoplication vs. C-Pap

We discussed putting off the fundoplication indefinitely, though I realize we may have to revisit the issue again in the future. Our ENT feels that Nolan's "awful August" was directly caused by the C-Pap - he started C-Pap therapy in August and became very, very ill with retching, choking, and exacerbated reflux symptoms. Apparently, they've seen this reaction in the other kiddos with severe acid reflux. On the other hand, Nolan is doing extremely well this month, and seems to be tolerating the full-face mask much better than the nasal masks. Either he has adjusted or we're just having a great month. Time will tell, but I have every finger and toe crossed that the full-face mask is simply negating some of the issues of the nasal mask (the nasal mask forced air directly into his nose, whereas the full-face mask simply pressurizes the air around his entire face).

Balance Issues

Nolan was quite wibbly-wobbly in the office and fell a couple of times. We've seen this at home a lot, but it is the first time he's done it in a doctor's office. We've been attributing it to his sometimes wonky gait or to his amblyopia (both of which can cause "clumsiness"), but she thinks he has something vestibular going on. It would make sense since he has had a progressive hearing loss, but we'll see. She wants to do some sort of test in a spinning chair, but I don't really see the point. If it is vestibular, it isn't like we can do anything about it. She does want him to avoid contact sports that might result in a blow to the head because of the balance and hearing issues - which is strange, because he doesn't have enlarged vestibular aqueducts (these are the precautions usually given to kids with that "issue" who still have residual hearing). I'm letting it go - he's a little clumsy now and then, but it isn't the end of the world (and it isn't really a "fixable" thing, anyway).

Sinus Issues

His sinuses are horrible, of course, but they have been horrible for the past three years. With his form of acid reflux, the acid shoots past the esophagus and hits the nasal passages and airways. She ordered a CT scan of his sinus cavities to see how much damage has been done: cue episode 547 of "Reflux Sucks." Since he is a C-Pap user, he may require intervention with his sinus cavities, since they are chronically infected and he can no longer breathe through his nose. Another thing that I'm not too worried about, but we'll see what the CT scan shows.

The Dreaded Earwax

He had wax in his left ear, so we had to take a walk to the procedure room and use the Papoose Board of Doom. Since he has long-term tubes in his ears, we can't use DeBrox or any of the other over-the-counter wax removal products. We're stuck with the ENT's metal earwax removal device, which Nolan loathes. Actually, loathes might be too kind of a word. He completely flipped out once we went into the procedure room - he remembers this room and hates it with an unparalleled passion. I can't say as I blame the kid.

A Reward for a Difficult Day at the Doctor's Office

After the wax removal, Nolan's ear hurt too much to put the hearing aid back in place. He was rather unhappy with all of us, so I tried to cheer him up with a trip to McDonald's. We don't go very often, but I looked up a McDonald's with a great indoor playground, which happens to be right next to the ENT's office. They had eating booths that looked like trains and a massive indoor playground. Nolan was happy by the time we left (thank goodness).

We will have the CT scan performed (outpatient, no sedation required) and return in early November to discuss the results with the ENT.

At least October is nearly doctor free!

Mr. Hungry

2011-09-23T07:39:00.000-07:00

Whatever caused Nolan's problems in August seems to be gone, and I am SO very happy that his tummy seems to be working again. He has been absolutely ravenous lately, which is so wonderful I could literally do a happy dance.

The sleep neurologist said that one of the ways we could tell if the C-Pap was working was by Nolan's growth. He should experience a big growth spurt over the next few months. Judging by his appetite this week, I'd say we may be seeing one sooner rather than later. With the full-face mask, Nolan is actually starting to get some benefit from the C-Pap machine. We can still hear stridor (that comes with the laryngomalacia territory), but I think he is actually getting some quality sleep for the first time in his young life. Last night, he kept the mask on until 4:00am. This means he got 8 complete hours of sleep. For the first time, ever.

Yesterday, he ate breakfast, lunch, dinner, a string cheese, a pear, and 8 cookies.*

Grow, Nolan, grow!

In other news, still no word on the microarray results. This doesn't really mean anything one way or another - some cells just take longer to grow to obtain results. I did leave a message with genetics today to see if they'll call me back with some news (any news).

Nolan has an ENT appointment on Monday, and we are going to put off discussion of a fundoplication indefinitely. Despite our initial reservations and the horrible month of August, Nolan seems to be doing just fine on C-Pap and I can't see any reason to have a permanent, irreversible surgery performed on him when less invasive methods are working.

Here's to a happy, hungry kid!

*He wasn't exactly given permission to eat eight cookies. They were within reach and Nolan figured it was better to ask for forgiveness than ask for permission. I'm still in shock that he could down 8 of them!

Still Waiting

2011-09-19T12:40:00.000-07:00

Nolan's micro-array (oligonucleotide array) results should be coming back soon. We don't expect to "find" anything on this test, but there is still some anxiety during the waiting period. I have no idea why I am anxious - the results will likely be ambiguous. A micro-array is only useful when a deletion or duplication is actually found, with known significance. Micro-arrays don't detect point mutations or frameshift mutations (small changes to actual genes): they only detect the addition or removal of chromosomal segments. The chances of Nolan having a deletion or duplication are pretty low.

Anyhow, the possible results (and their ramifications) are:

Normal Microarray: No detected deletions or duplications. This means that we will know nothing more about the cause of the various medical issues. They could still be genetic, but not detectable on this particular test. On the other hand, we will have ruled out a lot of other syndromes. Essentially, we'll be in the same place we are now - watching and waiting to make sure nothing new pops up, and not knowing what to expect.

Variation of Unknown Significance: They may find a deletion or duplication which is not currently catalogued. Like Tigger, "he's the only one!" Some deletions or duplications may be harmless - to determine the likelihood of this scenario, we would be tested alongside Nolan if this situation occurs. If we also carry the deletion or duplication, it is likely harmless (because we don't have the health issues). If we don't carry the deletion or duplication, then it is more likely to be the cause of his issues. The geneticist would have to research all the genes affected in the region of duplication or deletion. This finding is a real gray area.

Known Deletion/Duplication Found: A known, catalogued deletion or duplication is found. If this occurs, then we will have a name and a cause for everything. This is unlikely, because Nolan doesn't have many of the features of known microdeletion syndromes. On the other hand, there is wide variance and there are a LOT of microdeletions/microduplications known to cause multiple health issues.

The geneticist said it would take a week to 2 weeks to get the results back. Tomorrow is the 2 week mark, so every time the phone rings, we are on pins and needles.

New C-Pap Mask and a Happy September

2011-09-16T08:18:00.000-07:00

We finally managed to get the full-face mask for Nolan to try out. While the Sleep Neurologist wasn't keen on the full-face mask option (many children swallow air with these masks), we thought it was worth a shot. If Nolan started swallowing air, the effects would be obvious pretty quickly - he would develop a distended belly, belch, or get sick. The other night, we put the new mask on and it was wonderful.

There was no air blowing into his eyes, to pressure marks around his flat nasal bridge, and the whole thing was fairly comfortable. This mask is also great when he is congested and needs to breathe through his mouth. The only negative is that he can't scratch is face, or wipe away tears if he needs to (he often has melt downs at bedtime, and having to put on a C-Pap mask at night certainly doesn't help with the evening tantrums).

Still, this mask is the best of the lot so far. He insisted on a nasal mask last night, and we let him have it since he is not congested. He made it to about 2:00am, but with multiple air leaks around his eyes. We're going to give the full-face mask another shot tonight.

I have to admit, I am much relieved that Nolan is doing better this month. August was simply awful. His reflux was at an insane level, he was cranky every single day, and the congestion/gagging/choking were terrible. This month seems to be MUCH better. His reflux is back down to its 'normal' level (if having severe reflux is ever normal) and he is eating, playing, and laughing again. He comes off the school bus with a bounce and is always excited to talk about his day. Today was show and tell, and thankfully we did not have to go in search of a Giant Frog this morning. He took in an airplane and wore his flight jacket to school.

Today is his first day of a Creative Arts class that he will be taking on Fridays. He'll be drawing with charcoal and learning about black-and-white art. I love having a happy boy who is thriving alongside his peers!

Sleep Center Appointment

2011-09-13T15:56:00.000-07:00

Nolan is quite the nature boy these days. This is mildly surprising, since he used to have a very strong fear of plants. He has been spending quite a bit of time on the edge of the woods, searching for newts and frogs - though he's a bit nervous about venturing deeper into the forest. That suits me just fine - a little healthy caution is never a bad thing!

He came home from school this morning with one "dead" hearing aid - his battery died at preschool. I asked him why he didn't tell his teacher (she has extra batteries), and he simply said, "I don't care if I can't hear with one ear." Sigh....

I'm going to start replacing his batteries every five days to prevent the batteries from dying on him during class. They used to last about 8 days (without the FM system running). With the FM system in school, the batteries last about six days. I figure if I change them every five days, then we should be able to avoid any problems during the school day.

I also found out that Nolan is changing his hearing aid program. He came home from preschool the other day and it was set to the regular hearing aid program (not FM). I told him to leave his program buttons alone, and he said, "I don't want to listen to my teacher all the time."

I think I have found his super power - he really does have the power of selective hearing! Stinker.

We went to the sleep center today, and he had his actual appointment. His lungs sound a little junky, but nothing too serious. The back of his throat looks good, and his weight is fine. I was relieved when the sleep center doc told us that 4-6 hours per night was absolutely fine for C-Pap use. Nolan may never get in a full night's sleep with the C-Pap, but most growth hormone is secreted during the first 4 hours of sleep. If he gets "quality" sleep during those first four hours, then that is "good enough." He is still very fatigued and has a hoarse voice- that is what we will live with since he has such severe reflux.

I did ask about getting a full-face mask, and he recommended against using one. Apparently, a lot of little kids are "aerophagic" (seriously, I learn a new word every day with this kid). If a full-face mask is used, Nolan could swallow air all night long and end up vomiting into the mask. Or he could just end up belching a lot with a distended stomach. On the other hand, he can't really use the nasal mask when he is congested. And with the airway reflux, he is congested all. the. time.

The respiratory therapist called me and said she does have a sample of the full-face mask in stock. We could try it - if he is "aerophagic," then we'll know right away. If not, then it may be a good solution for him. I'm happy they have one in stock - it lets us try before we buy. I'll pick it up tomorrow when both boys are at school.

Next week is entirely doctor free, so we are going to enjoy the down-time!

September the Eleventh

2011-09-11T16:30:00.000-07:00

Ten years ago, I woke up to a news announcement on my alarm clock. I was thoroughly confused: why was Peter Jennings talking about the Twin Towers? My mind couldn't make sense of it, but my first thought was they were discussing the 1993 bombing of the towers, and it must be some sort of anniversary. As I woke up, I realized this was not an anniversary of the 1993 bombing, but something entirely (and horribly) new.

I switched the TV on to watch the first tower fall. The shock was intense - and I called family as I got ready for work. As it turns out, my dad happened to be on a plane that day. And we didn't know where he was. At the time, we only knew of the three planes, and the worry was sickening as we learned of a fourth, unidentified plane that had been hijacked. A while later, we learned that my father's plane was grounded in Las Vegas - I was relieved that he was safe, but profoundly sad for all those who lost their lives that day. I spent much of my day at work going from the conference room where someone had rigged up a TV for news coverage, to the lab where we were making a product.

Ten years later, I remember that day vividly. The silence of the skies, with no airplanes in sight. Waiting for my dad to make his way home from Las Vegas (via rental car). Watching the news reports roll in with more and more horrible news.

My boys are far too young to have any knowledge of that day, and I envy them their innocence. When they learn about that day in school, it will be an event that "happened a long time ago." For now, I am glad that they live in a secure and free country, with access to healthcare, education, and the safety to express their opinions and beliefs freely.

We went to Nolan's preschool open house today. We are sending him to a second preschool, on Monday and Friday afternoons. This particular preschool has a wonderful program on Mondays called the X-Cel program, aimed at higher academic standards. Since Nolan's TOD is working with him on basic rhyming and reading concepts, this class will reinforce those ideas. On Fridays, he will attend the Creative Arts class - they do things like draw with charcoal, make mosaics, and paint on real canvasses. He starts this program tomorrow, and I am sure he will get a lot out of it. Matthew also attended this preschool, and it really helped him develop some artistic skills (and exposed him to some higher academic concepts like even and odd numbers/sight words/weights and measurements).

I am very glad that this September the Eleventh involved preschools, happy children, and nothing eventful in the news. My heart goes out to those who mourn and grieve friends and family lost on that fateful day ten years ago.

Updates and Some Good News

2011-09-09T15:42:00.000-07:00

It has been a rather long week. Matt started going to school full-time this week, which means he boards the bus at 8:15am and gets home at 4:10pm. His days are very long, and he is only five years old, which means he is one tired little boy by the time bedtime arrives! Nolan's days are definitely more manageable (8:15am-11:35am), but we've had several appointments this week.

Monday was supposed to be his sleep clinic appointment (yes, the person scheduled it for Labor Day). Unfortunately, the entire office was out from September 5-September 13 and failed to notify us of the cancellation. I tried to call to confirm the appointment prior to making the drive up to Dunkirk, but their office phone simply rang (no one had heard of an answering machine, I suppose). We ended up driving to Dunkirk and finding a note pinned to the window stating the office was closed for the entire week. There's nothing like spending 2 hours in the car for no reason! We're going back to the sleep clinic on Tuesday the 13th.

Tuesday was the Cleveland Clinic appointment, which went well and we obtained some direction regarding the fundoplication decision. On Wednesday, Nolan started his weekly visits with a Teacher of the Deaf (TOD) to help with identifying any gaps in his langauge development. Nolan is able to rhyme and is working on "A" level reading worksheets (a kindergarten level), so we aren't really too worried about his progress. He does have some random vocabulary gaps, though, so we are working to fill those in.

Thursday was the ophthalmology appointment in Erie - we weren't sure if we would have to patch or not. With his falling issues lately, I was concerned that his vision might not be improving well enough. As it turns out, he is doing VERY well with his glasses. He is not correcting to 20/20 yet, but the improvement is continuing and he is gaining a line of vision at every appointment. As long as he continues this trend, we don't have to patch. This is great news!

I also mentioned a connective tissue disorder that had been casually mentioned by the geneticist. I wanted to make absolutely sure that Nolan's eyes didn't carry the "vitreous changes" that occur with that disorder, and we reviewed his records. His eye jelly looks great, so there is no way he could have that particular issue. To absolutely rule it out, they are going to do a full eye exam at his next appointment in December. It is good to absolutely rule it out, because that particular disorder can result in blindness if certain preventive measures aren't taken. I am relieved to rule this one out, but also concerned at the continuing development of new issues over time. Sometimes, it would be nice to have a name (and a prognosis) for all the apparently random developments.

It is now Friday, and the boys are decompressing after a long week at school. Nolan's classroom is doing a caterpillar unit, and hearing him say "chrysalis" is about the cutest thing on the planet. They have been sequencing the caterpillar life cycle, reading the Very Hungry Caterpillar with props and puppets, and they had a music class today, where Nolan's favorite Slipper Fish song was sung. Show and Tell was also today: he decided to bring a Giant Frog. There was some stress this morning, as finding a Giant Frog at the last minute is a rather difficult task. We finally settled on a large stuffed animal given to him by his beloved SLP last Christmas. He talked about his frog and told the class that it can "hop hop hop." He absolutely loves Pre-K. His teacher is absolutely fabulous.

Next week is much less busy, with school and only one doctor appointment. Thank goodness!

Update From Our Cleveland Clinic Trip: Genetics

2011-09-06T16:35:00.000-07:00

We are back from the Cleveland Clinic - an absolutely amazing medical center. Nolan was amazed by the fountains and the landscaping everywhere - there's a reason they call this place "Medical Disneyland." The top-notch doctors make this place truly wonderful.

We saw the geneticist and the genetic counselor, who looked at Nolan's (extensive) medical history and at Nolan himself. I will say up front: there is no "Eureka! We found it!" moment coming up here - so this will be a long post detailing the general thoughts and impressions from the team at the Clinic.

First off, Nolan is definitely medically complex. There are a lot of things going on, obviously, and some things can be considered "birth defects" (the posterior urethral valves and the hearing loss) and some can be considered progressive (the acid reflux with increasing intensity, the laryngomalacia, the hearing loss, the vision). Some things are entirely new to us. Both the geneticist and the pediatric resident heard a distinctive heart murmur.

Nolan's echocardiogram a year ago was declared "normal." No one has ever detected a murmur before. It is likely he has developed a new issue, but the geneticist is requesting his echocardiogram from last year to confirm that this is a new development. If it is a murmur, the location of the murmur suggests that it is a mitral valve prolapse. This is generally a fairly benign murmur that doesn't need surgical correction, but it will need monitoring. We'll see where this one goes.

Hanging out in genetics

The geneticist went over Nolan's issues. Some "separate" issues are really linked together, because one issue causes the other. The truly separate issues are:

1. Hearing Loss

2. Myopia, Astigmatism, and Anisometropic Amblyopia (vision)

3. Severe reflux -Laryngomalacia - Obstructive Sleep Apnea - Chronic ear infections/upper respiratory infections

4. Posterior Urethral Valves

5. Heart Murmur, probably mitral valve prolapse (new)

6. Hyperextensible joints with mild hypotonia

Obviously, this many issues isn't exactly coincidence. On the other hand, there is no comprehensive diagnosis that fits his issues. The geneticist did feel that he should have a genetic microarray done, to rule out minor chromosomal deletions and additions that can cause issues for some kids.

She did warn us that the test will probably come back as "normal," however, because the majority of chromosomal rearrangements and deletions cause cognitive and developmental issues. Nolan has no cognitive issues, which makes a chromosomal problem less likely. We do need to run the test, though, because other things cannot be considered until this test is done and his chromosomes have been checked out.

The microarray was drawn this afternoon and we should have the results in about 2 weeks.

She did say that Nolan was doing well developmentally, so while he is medically complex, he is also very "normal." Which is wonderful to hear... though we do wish there was a way to treat the medical problems he does have (and stop new ones from cropping up)! Essentially, we will have to monitor him on an annual basis and see if new issues keep cropping up.

Heading out: happy to get to the car!

As for the reflux, we have no idea why it continues to increase in severity. We asked her opinion on the fundoplication, and there really isn't a right or wrong answer. If his lungs are definitely affected, we should do it. If it is just the apnea, we should probably stick with C-Pap and try to make it work. If he starts having difficulty maintaining weight, we should do it. Since he has re-gained his weight back to 32.4 pounds (woot!), we might consider holding off.

She did tell us to consider the following very carefully: with Nolan's history, we should be prepared for a fundoplication to completely fail in its goal to eliminate apnea. While it may work, there is no guarantee that it will be successful in controlling the apnea. We could very well do a fundoplication, and then still need to use a C-Pap for Nolan. And we will always have to use anti-reflux meds for him. So really, if the reflux starts attacking his lungs, voice, or ability to grow... we should do the fundoplication. If the reflux is just causing the apnea, we would be better off controlling that with the C-Pap. It will be an ever-evolving judgment call: with progressively worse reflux, our decision to hold off on the fundoplication may have to be reversed as new complications arise.

She also said that his fatigue and stomach pain is an inescapable component of his condition. He has apnea - he is going to be tired until it is well controlled. Controlling apnea with severe reflux is difficult, so he will likely struggle with fatigue. While a little depressing, it is realistic: he has a chronic condition that we will have to cope with, because it is unlikely that it can be "fixed."

His stomach pain comes with the acid-reflux territory. His gut doesn't work right, and he's going to hurt a lot. Again, he will have to develop coping mechanisms for this, and we'll have to try to keep on top of the anti-reflux meds as we have always done. A fundoplication will not fix the mysterious stomach pain (which is likely due to delayed gastric emptying).

Her input was valuable. For now, we will probably hold off on the fundoplication and work harder to make the C-Pap work for Nolan. But our decision may change, based on what the reflux does to his little body.

As for now, we just wait for 2 more weeks to see what the microarray shows.

Such a Turkey

2011-09-04T16:05:00.000-07:00

Today has been a long day. One of those days. Nolan has been pushing nearly every limit known to man. All. Day. Long.

Fortunately, bedtime is coming very soon. Hopefully tomorrow will be a more cooperative day!

I may not get much of a chance to update in the coming week, because we have a few appointments in different cities (and three different states). Tomorrow (on Labor Day), we have an appointment with the sleep clinic in the fabulous city of Dunkirk, NY. This is the closest doctor to our house (45 minutes away), but I am a tad concerned that they scheduled the appointment on a holiday. I will definitely call before we take the drive up there!

On Tuesday, we have our appointment with the geneticist at the Cleveland Clinic (in Cleveland, of course). I'm not sure what to expect from the appointment, but we are hoping for some direction, at least. And for some insight into some of Nolan's fatigue and stomach issues, which constantly plague us.

Fatigue, Nolan-Style

Wednesday is free of doctors, but Nolan has his speech-language-pathologist in his classroom and will return to school for the first time in four days. He also has his Teacher of the Deaf (TOD) coming in the afternoon.

Thursday is a visit to the Pediatric Ophthalmologist (PO) in Erie, PA. This is the appointment that will dictate whether or not we have to patch Nolan's good eye.

On Friday, the kids *just* have school and I think I may take a long nap.

Thank goodness for weekends!

Fall Down Go Boom

2011-09-03T14:50:00.000-07:00

Nolan decided to take a trip into the TV stand on Friday. I could tell the gouge was deep enough to require attention, so I bundled him off to the pediatrician. Fortunately, the cut was on the forehead and they could glue it back together.

I am SO very glad for modern medical inventions. The glue was SO much less stressful than stitches: no shots, no need to come back to have the stitches removed. A few passes with a tube of medical-grade super glue, and our boy was patched up quite nicely. The cut looks MUCH smaller now that it is glued together (it was about 1/4" wide when we went in), and it should heal nicely with only a small scar.

The glue should come off in about 7 days, and he'll be good to go. He still wasn't a happy camper about the whole thing, but I was just grateful we didn't have to hold him down for stitches!

Nolan has been falling a lot lately, and complaining that his glasses are blurry. This is mildly concerning to me since:

1) His glasses are clean.
2) His vision went from normal to amblyopia in the space of 1 year. He has a history of rapidly changing vision... so it isn't out of the range of possibility that he's having issues because of his eyes.

We have an ophthalmology appointment on Thursday, so hopefully we can rule vision out as a cause to his recent clumsiness. Hopefully it is just "normal" incoordination, though!

Of course, Nolan is forever coming up with new ideas, and his latest is the glasses-as-money-holder. He's been walking around with coins stashed in his glasses ("for the library parking meters"). Fortunately, we've been able to convince him that his pockets are a better location for his spare change!

An excellent place to store spare coins

Honestly, It Couldn't Get Any Better

2011-09-02T08:59:00.000-07:00

I am so very glad we live in our specific school district. The Universal Pre-K program at our school is simply fantastic. Nolan's teacher is beyond fantastic. Things are just going beautifully at school.

Nolan's teacher sends home a letter at the end of each week, detailing what they learned and what they are going to learn the following week. This is vital information for us - and for Nolan's Teacher of the Deaf (TOD) and Speech Language Pathologist (SLP) - because it allows us to pre-teach some material and vocabulary.

This week, Nolan's class:

Read stories about starting school
Sang familiar songs (like Twinkle Twinkle and the Wheels on the Bus)
Met the classroom puppet (dubbed Lola the Lamb)
Talked about the calendar and weather at circle time
Explored the classroom and played at centers
Practiced walking in a line
Practiced fire drill procedures.

Honestly, this is so wonderful. The kids are familiarizing themselves with the routine, and reinforcing the concepts by helping Lola the Lamb prepare for Pre-Kindergarten. They are practicing fire drill routines so that ~~no one freaks out and screams like a banshee~~ everyone knows what to do when the alarm goes off.

His teacher is using his FM system and that is working out VERY well. I have been setting the program on Nolan's hearing aids and connecting the receivers, then placing the FM unit in its case in Nolan's back pack (microphone on mute). The teacher just has to pull it out and un-mute the microphone, and voila - the FM is "on." This does mean that Nolan's program slightly mutes sound on the bus, but he can still hear his friends if they are in close proximity. It is the best trade-off at this time, since he doesn't have a TOD to set the system up every morning at school.

Next week they are studying Monarch caterpillars.

Nolan will miss Tuesday's class because of our trip to the Cleveland Clinic, but he'll get back into the swing of things very quickly on Wednesday.

Matt is also feeling better today. He broke out in a sweat last night and the fever disappeared. He seemed right as rain this morning, so I sent him off to school. He should get off his bus in about 45 minutes - I love seeing my boys after their exciting days at school!

School is Fun

2011-09-01T17:32:00.000-07:00

In Nolan's words, this is how his first day went:

"I sat on the rug. There was a story about doggies in the school. We sang Twinkle Twinkle Star. We didn't get to play outside. R----* is in my class! I played with the space toy."

It was a great day, and his teacher even called from home later in the afternoon to ask a few questions and to see how his day had gone. Did I mention how awesome Nolan's teacher is? She is beyond amazing. Nolan did have some stomach pain today, but sat on the rug and got through it.

Matthew also started school today. My big boy is in Kindergarten. Next week he will begin attending school from 8:45am - 3:15pm (he will board the bus at 8:15am and won't be home until 4:00pm). He's going to be a little tired while adjusting to the longer schedule.

And my little guy, who couldn't speak until he was three, was full of words about his day:

"We got to sit on the floor and listen to two stories, then we went to see the specials. We got to see the gym, but we weren't allowed to go in because the door was shut. We also got to see the art room, and it is right next door to our classroom! And then we got to see the music room and the cafeteria! They called it a TOUR! And, Mom? We got to play. Not outside or in the big room, but we got to play. And I saw K---* standing in a straight line. And then we got on the bus and A-----* was on the bus and now I'm home!"

Both boys are absolutely exhausted and fast asleep. Tomorrow is another half-day for Matt, which is good because he is really tired! He was also running 100.7 tonight... I'm hoping that is from being so tired and not from an impending virus!

*Names omitted for privacy.

The First Day of School

2011-09-01T06:16:00.001-07:00

I thought I was handling the whole going-to-big-boy-school thing really well. Until 3:00am, when lightning flashed and thunder crashed. I woke up with a start, and thought:

OMG. It is going to rain. I didn't think about rain. Nolan's hearing aids will get wet. A hat? Do we have a hat that will fit? OMG what am I going to do? I'll drive him. No... wait... UMBRELLAS! Yes. He can take an umbrella!

You can see that my mind does not function well in the middle of the night.

Of course, my next thought was,

Do they let them take umbrellas to school? Can they take them on the bus, or would they consider them a "weapon" or "projectile?" What if he can't take an umbrella? They didn't mention umbrellas at the parent information meeting and I FORGOT TO ASK ABOUT UMBRELLAS!!

I am pretty sure I was up from 3:00am until about 6:00am, worrying about whether or not it would be all right to send Nolan to school with an umbrella. Then it was time to get up. My brain grew rational once more. I gave Nolan his meds, fed the boys, got them dressed, set up the FM system and cleaned his glasses, and took them outside for first-day-of-school pictures.

Ready for Pre-K

Then I drove down to the bus stop (still threatening rain) and we waited for the bus - with umbrellas. The boys boarded the bus, and are probably just finishing up with circle time as I write this.

Matt has a half day today, so he'll be home shortly after Nolan's "Turtle" bus drops him off.

VERY excited about the bus!

Waiting... WITH umbrellas

In the mean time, I am going to resume my frantic house cleaning as a way to stave off tears.

I can't wait for the bus to bring my babies back home.

Please bring my babies back home SOON!

Wordless Wednesday: The Practice Bus Ride

2011-08-31T18:05:00.000-07:00

C-Pap Headaches

2011-08-30T05:37:00.000-07:00

Using the C-Pap machine with Nolan is a real chore. The poor little guy has reflux so badly that the machine can't get through all the congestion in his nose, and the air blowing against his already-inflamed nasal passages causes him great discomfort. Listening to him cry in pain until he goes to sleep is simply awful.

And then he gets the bronchitis-sounding cough in the morning, and a very hoarse voice. All this from only using the C-Pap machine for 3-4 hours every night. I just don't think this is going to work for him.

We're persisting until we can see the Sleep Clinic neurologist on the 5th, because Nolan desaturates without the C-Pap. Of course, he is so congested that we doubt it is doing any good, but I need the doctor to see the congestion and hear his lungs. Docs tend to "get it" better when they can observe the actual effects, rather than simply being told about it by the parents.

The sleep issues have me worried, because Nolan starts school on Thursday. His teacher has been forewarned, thank goodness. Nolan frequently sleeps until 8:30am or later - and before anyone says, "WOW, I wish my kid did that!" keep in mind that Nolan desaturates constantly, and has "arousals" every minute of sleep. He wakes up, at least partially, at least 60 times per hour. This is why he sleeps until 8:30, and is still cranky as a bear.

When school starts, I have to get him up at 6:30am, so that he can have his Nexium at least 30 minutes before breakfast. Then I have to get him to eat (for Nolan, this usually takes 45 minutes), get the Zantac into him, set up his FM system, and get him to the bus stop in a fairly decent mood. I'm pretty sure the other things will be no problem, but the decent mood part is going to be tough.

I suppose we'll see what happens once school starts.

Letter to the Teacher

2011-08-29T07:13:00.000-07:00

I was struggling with how to present information about Nolan's myriad issues without completely ~~freaking the teacher out~~ overwhelming the staff. I found a wonderful "Letter to the Teacher" on Life with the 'Tars, and copied the format.

Here is Nolan's "Letter to the Teacher" for this school year:

Hi! My name is Nolan. I'm so glad to be in your class this year! My mom helped me write this letter to tell you a little bit about me.

I love going to school and playing with new friends! I really love stories and painting, and I know I am going to have a great year!

****

I don’t hear very well, and both of my ears have a moderately-severe hearing loss. I wear two hearing aids to help me hear better – I compensate for my hearing loss very well, but the presence of background noise makes hearing more difficult. Because of this, I also use an FM System in noisy situations: this system brings your voice directly to my ears. I hear better when people face me, and I do not hear as well when people are behind me or speaking from a large distance.

****

I wear glasses to help me see clearly. Both eyes have significant astigmatism, and my right eye is also nearsighted. I have amblyopia in my right eye, and cannot see very well out of that eye, even with glasses. Because of this, I sometimes have difficulties with depth perception (and may bump into things or appear “clumsy.” My eye doctor says I might need to start wearing an eye patch over my stronger eye – if that happens, I will have some difficulty seeing while patched. We are hopeful that the glasses will be enough to help me see!

****

Sometimes, I will get a bit “droopy” and complain that I am “out of energy” or “tired.” Sometimes I will complain that my legs hurt or that my tummy is sick. My mom and dad are not sure why I do this – usually a snack or some gentle encouragement will get me back into the swing of things. I usually do this in the afternoon, so hopefully this will not happen during my school day this year.

****

I don’t breathe very well at night because I have a floppy airway (laryngomalacia) which causes sleep apnea. Because of this, I use a C-Pap machine at night. The doctors are not sure if this will work, as it causes me to become very congested and sometimes causes my lungs to sound very congested. I am not sick, but I will frequently have a congested nose and rattling lungs. Sometimes I wake up very cranky in the morning, because I have had a very bad night’s sleep. We are currently looking into some other options to find a way to treat my breathing problems, but this is expected to remain a problem for the foreseeable future.

****

My stomach doesn’t work quite right. I have very severe acid reflux. The acid reflux is the reason I have had four sets of ear tubes, chronic congestion, and why I developed the floppy airway. I generally do not vomit, but I will occasionally gag and choke on reflux. When this happens, I often cough and my eyes turn red and water. These episodes are brief and I will be fine as soon as the reflux clears. I take very high doses of proton pump inhibitor medication, in addition to an acid blocker, and none of these medications are able to control the reflux.

I also have delayed gastric emptying, which varies in intensity. This means my stomach does not digest food properly, and empties too slow. On very bad days, my stomach hurts badly and I will not eat any food. Sometimes I will vomit the food I have eaten the day before, completely undigested. Most of the time, however, my stomach works well enough and I eat just fine. If I complain about severe stomach pain, this is the cause of the trouble!

****

I'm so excited to be in school this year and I know I will have lots of fun in your class!

Your new student,

Nolan

Four

2011-08-28T19:57:00.000-07:00

We spent the weekend forgetting IEP's, C-Pap, and all the other things that seem to be pressing down on us lately.

On Saturday, Nolan turned four. We went to a local amusement park, and had an absolute blast. I am fairly certain this child contains little of my gene pool, because he LOVES thrill rides. The child has no sense of fear!

We spent the day riding the biggest thrills a child of 40 inches could ride - and consoling our little man when he could not ride amusements with names like "Deathflayer." My stomach has not quite recovered from his love of thrills. He has opened presents, and eaten birthday cake. I still can't believe my baby is four.

We left off the C-Pap for three full days, and his voice has somewhat recovered and his lungs sound clearer. To really determine if the C-Pap machine is causing the issues with his lungs, we are starting the therapy up again tonight. Hopefully the lung issues were coincidental...

I have a meeting with Nolan's teacher tomorrow morning, and school starts on Thursday. Pre-Kindergarten, here he comes!

Preparing for School

2011-08-25T17:15:00.000-07:00

School starts next Thursday. There are the normal preparations to make, like purchasing school supplies, going to open houses, and choosing the perfect lunch box.

Then there are the extra preparations - the ones that involve educating and advocating for a little guy who can't explain his extra needs to the adults around him. I called Nolan's future teacher earlier in the week to set up a meeting. I need to show her his equipment and to give her the general lowdown on the little guy. She called me back very quickly. And gave me her home phone number.

We chatted briefly, and I set up a meeting for this coming meeting. Her final words?

"What can I do to help him succeed?"

The best words ever spoken to a parent. She is a wonderful teacher.

I am trying to come up with information that is pertinent, but not overwhelming. I'm working on a simple "Nolan Information Sheet" to explain the relevant issues (vision, hearing, fatigue, choking and gagging). Some of the issues may not come into play very often - he only chokes on food occasionally, though the gagging is occurring at increasing intervals.

This teacher is going to be wonderful for Nolan. She loves art. She loves children. She has experience with special education and with the mainstream. She is amazing.

I know Nolan is going to have a great year in her classroom. I am very excited to see what this year brings!

Would You Like Some Butter with that Donut?

2011-08-23T05:46:00.000-07:00

Nolan started eating again last night. He ate half his dinner, a string cheese, a banana, and ice cream. This morning, he ate a bowl of dry cereal and two cinnamon rolls. He's trying to make up for lost time, apparently. We're letting him eat whatever he wants - he needs to gain back the three pounds he lost over the past month.

I am very relieved that his digestive system seems to have returned from its rather long vacation. Hopefully his energy level will return with his stomach: the past month has been filled with days of "noodle-boy," where Nolan loses all tone and turns into a puddle on the floor. Sometimes, he falls asleep.

Mostly, he just whines and cries that he is "out of energy" or "tired." The chronic fatigue is worrisome to me, but I have a feeling it is tied into his lack of eating. He's still adjusting to the C-Pap, too - we're making it to about 2:30am most nights. Then he wakes up crying because it is hurting his nose. Still, it is progress!

This is the last full week of summer vacation before school starts. We have a slip-and-slide party planned, a play date at the creek, and lots of other fun activities planned. I hope the little guy's energy level returns so that he can really enjoy all the splashing and playing with the other kids!

Enjoying the Rest of Summer

2011-08-20T06:37:00.000-07:00

Nolan with a beefsteak tomato

I received Nolan's "Hello" letter from his new teacher in the mail. There are less than two full weeks of summer left - the time of playing in the warm sunshine is quickly coming to an end!

Since the week after school starts brings an onslaught of doctor appointments (sleep clinic, ophthalmology, and the Cleveland Clinic in a four-day span of time), we are making the most of our summer days. We went to a local pool yesterday, we're going to playgrounds this weekend, and next week will be spent slip-and-sliding, playing, and otherwise participating in non-educational fun. Summers are entirely too short.

As a side note, the new CPap mask is marvelous, but Nolan still won't keep it on all night. He wakes up crying, because the air is hurting his nose. Or the air is leaking into his eyes. In the middle of the night, we are bleary eyed and exhausted, trying to fix the air leaks and the hurt nose. Part of the problem is the chronic congestion Nolan gets from the reflux. Part of the problem is a flat nasal bridge. Part of the problem is trying to stick a confining mask onto a tiny child, who cannot really understand the implications of not wearing it (the only other way to fix the apnea is a fundoplication followed by a revision supraglottoplasty - something we are trying to avoid).

In the meantime, we are stumbling around like zombies from lack of sleep. We've never really gotten a full night's worth of sleep since Nolan was born, but at least we learned to sleep through him crawling around our bed at 2:00am. With the CPap mask in place, we repeatedly get up and go into his room to adjust it.

I am on the hunt for a full-face C-Pap mask for kids. That may solve his congestion issue, because the air pressure could be delivered through his nose and mouth while he sleeps. The other option is to put him in a bassinet next to our bed, so that we can take care of him without having to get up multiple times during the night.

Anyone who has been-there-done-that, what have you done to keep the mask on your child through the night? We need some tips!

Much, Much Better

2011-08-19T07:01:00.000-07:00

The pediatric C-Pap mask I ordered off the internet arrived yesterday, and I was thrilled to find it was a nice, tiny size. The nasal portion fits over his nose and doesn't overlap onto his mouth. The head net is the right size for a small four year old, and the hose is on a swivel so that it doesn't bother Nolan as much in his sleep (the other mask had the hose in a fixed position on his forehead). Nolan kept this mask on for most of the night - he knocked it off when he got an air leak near his eyes. Still, he is getting much more time with this mask than the other one.

I know the need for a CPap at the age of three or four is pretty rare, but if anyone needs a good mask, the Respironics Child Lite mask is the one to go with. We got ours from Cpap.com for $99.
The only "negative" is that this mask makes a little more noise than the others (the other masks are silent), but I'll take some noise if the mask actually fits!

The next step will be to get Nolan to keep the mask on throughout the entire night. That actually seems like a possible feat, since we have a mask that actually fits the little guy.

Why Our Only Pediatric C-Pap Mask Doesn't Work

2011-08-18T10:23:00.000-07:00

The respiratory therapist has only given us one pediatric mask - this is the only one that truly fits Nolan.

Unfortunately, the mask is poorly designed: it only has two straps to hold it onto the face, so it frequently falls down. I frequently find Nolan in this position:

Obviously, having the head-net and mask wrapped around his neck is not the best idea in the world. For other C-Pap parents, this is the SleepNet MiniMe Petite CPap mask. It might work well for a non-mobile child, but I would otherwise avoid it. The retention system is terrible, and the designers did not consider the face and characteristics of a young child when creating this mask.

Every 10 minutes or so, I find him in this position and have to readjust him. When it is positioned correctly, it looks like this:

You can see there isn't much to hold it in place - just the two lower straps that fit under his ears. He can't get more than about an hour or two with the C-Pap working, because the mask just won't stay in place.

The pediatric mask I ordered online came in the mail today. It is smaller and has four straps to hold it in place. Obviously, our respiratory therapist is trying to pass off petite adult masks to save on overhead costs (she already has adult masks in stock and doesn't want to order a new mask in for Nolan). She tried to pass off the adult sized "EasyLife" mask as the "Child Lite" mask I had informed her of - unfortunately, she didn't realize that I could read (the side of the mask is labeled with the name and size, which indicate that it is NOT the mask we requested).

Thank goodness I was smart enough to invest the $100 and purchase our own mask from the online site. If you want something done right, you have to ~~forge your credentials and~~ do it yourself.

We are going to try the mask I ordered tonight.

We can't really judge the effectiveness of C-Pap therapy until we can get a solid night in with the machine!

Histories, DuoCal, and C-Paps - Oh, My!

2011-08-17T09:31:00.000-07:00

Since our pediatrician didn't send all of Nolan's relevant medical records to the Cleveland Clinic, we requested specific records from his ENT and GI doctors. Yesterday, I received a bill in the mail.

Nolan's ENT charges $0.75 per page to fax medical records to other specialists. Nolan has 50 pages of relevant ENT records, so the bill comes to about $40 for faxing records to the Cleveland Clinic. I only wish it wasn't so difficult to get his medical records in the first place, because then we could have sent the records on our own and saved about $40. I'm not going to fight it - we only have one shot at getting this genetics evaluation done, so I mailed off the check yesterday. Hopefully the ENT's office will get them faxed in time.

Nolan's appetite has largely returned, so we are dropping DuoCal into everything we can. Fortunately, he loves macaroni and cheese, so hopefully we can get our little 29-pounder back to his baseline.

In the meantime, my frustration level is about 10,000 on a scale of 1 to 10. Our respiratory therapist is worse than useless. She had one C-Pap mask that is designed for children - the Mini Me mask. Unfortunately, the headgear and design of this mask is poor - it is only attached by two straps, so it frequently falls down and off his face during the night. This leaves the head-net and tubing around his neck - clearly, this cannot be very safe.

I looked online and found another mask designed for children, and I sent the respiratory therapist the link. She called us back in and handed me a mask - not the one from the web link I had sent her. The one she handed to me was an adult petite mask - it is too large for Nolan. She also gave us petite adult nasal pillows (these insert into the nose) with an adult head-net. It just doesn't fit the little guy.

The mini-me mask (left), adult nasal mask, and adult nasal pillows

I am beyond frustrated with this process. The respiratory therapist is either lazy or stupid: adult masks do not fit 29 pound four year old children. On top of this, the home healthcare company charges $275 for these masks that don't fit. I ordered a pediatric mask off the internet - it was $99 with free shipping. Hopefully the company will send the correct mask, and we will have a solution for our little guy.

Nolan has been asking for his "mask" at night - and he has bonded with the Mini Me mask. If only this mask would stay on for more than 30 minutes at a time, it would work out well. The overall design of the mask retention system is the real problem.

Hopefully the "Child Lite" mask that I ordered will arrive soon. Once we find something that stays on his face through the night, we'll be able to really tell if the C-Pap is working or not!

Back to "Normal"

2011-08-15T14:50:00.000-07:00

I'm not sure what "normal" is, but Nolan is definitely back to his level of normal. He ate breakfast, lunch, and dinner today. He also ate popcorn and a piece of cheese for a snack. I am sighing a HUGE sigh of relief that he is able to eat again - there is nothing that turns me into a crazy-worry-wort mommy more than a little one who completely stops eating.

We're going to try the C-Pap again tonight.

Sometimes, I feel like a glutton for punishment. Hopefully Nolan will re-adjust to the mask and will fall asleep without any tears or fuss tonight.

Breakfast, Brunch, and Brinner

2011-08-14T18:01:00.001-07:00

Nolan's appetite picked up a little today. He ate dry cereal and yogurt at breakfast. He didn't seem very hungry for lunch, but we gave him french toast sticks and he ate a few of those - and then he had waffles for dinner. At this point, if he'll eat it, he can have it.

You know those people who always say, "Don't worry - a child won't starve himself?"

Yeah.

Turns out they're wrong.

Before I had kids, I had a list of things we would never do. We would never let the kids sleep in our bed. We would never make a separate meal for them. Lists and lists of "we would nevers."

Then we had a child who would, in fact, starve himself. A child who really does stop breathing at night. A child who not only lacks the sensation of hunger at certain times, but feels full to the point of nausea during these episodes.

He's down to 29 pounds (from a high of 32 pounds - he lost almost 10% of his body weight), but his appetite is coming back. If he wants waffles for dinner, he gets waffles for dinner.

We decided to hold off on C-Pap for one more night, to get him back onto a normal diet tomorrow. If he can eat some real food and keep it down, we'll start C-Pap again Monday night. He had more energy this evening, so things seem to be on the uptick.

In the meantime, I'm stocking up on french toast sticks.

Taking a Break

2011-08-14T05:49:00.000-07:00

Friday was such a miserable day. Nolan wouldn't eat anything, though he seemed to be fine in every other way. His nose is congested, but it is always congested with the combination of reflux and the C-Pap machine. Friday, Nolan spent the entire second half of the day like this:

We decided to stop C-Pap therapy for the weekend to let him recover to his "normal" level of activity and eating. He ate a waffle on Saturday morning. Two bites of a grilled cheese sandwich at lunch. 3 bites of macaroni and cheese at dinner. We took him out for ice cream, hoping that would entice him to eat: no dice. He ate about 3 bites of ice cream before his stomach was "full." He did eat some kiwi fruit when we got home - he always prefers wet foods when the reflux/DGE gets really bad.

Apparently, his digestive system has decided to go on vacation.

He seems to be eating a bit more this morning. He has had dry cereal and wants some bread. Mornings are his best time for eating when he gets like this (his tummy has all night to empty), so we're trying to load him up with high calorie foods.

We haven't seen the delayed emptying really cause a problem since he was about 2 years old. For some reason, it seems to have made a return this week. Nolan seems a bit more perky this morning, so hopefully that is an indicator that things are speeding up in tummy-land.

We'll start C-Pap again tonight, to see if the C-Pap is causing things to get worse. If Nolan takes a plunge into the vomiting/congested world of not being able to eat, we'll have to take a longer hiatus from C-Pap therapy. I don't really see how the C-Pap could increase Nolan's motility issues, though - I really do think the flare of his issues is just coincidental with the start of C-Pap.

Now I'm off to go dump some DuoCal into a jar of yogurt. Hopefully he'll eat it!

Is This Week Over Yet?

2011-08-12T11:29:00.000-07:00

Nolan has been having terrible flares in his reflux lately - last Friday he gagged and choked for two solid hours, and his eating really decreased. This week, we've had a lot of tummy pain and reduced appetite. Today, Nolan hasn't eaten anything.

Which is why I was surprised when he "baptized" the car with yesterday's lunch.

The car seat cover is now in the washing machine, Nolan has had a bath, and the car has been cleaned of the nastiness that was yesterday's lunch.

I'm so tired I can't even think straight. Nolan is fine, except for the whole not-eating tummy-hurting business.

This week can be over at any time.

There are a couple of bright spots.

I found a better mask for Nolan made by Philips Respironics: it is made for children in the 15 pound-40 pound range. Nolan is exactly 30 pounds, so he should fit into the mask. It also has a forehead strap to help keep the mask secured. It is also only $99, so I bought one. I emailed the respiratory therapist (who has no idea what to do with a child as young as Nolan), so she bought one, too. Hers will be here faster, so we'll use it until ours arrives. Then we'll have her get a different mask for us to try out. That way we'll have two masks to rotate.
Nolan's ear isn't leaking anymore. At least that is one thing we don't have to worry about this week!

Nolan is accepting the mask at bedtime, but he won't keep it on. There isn't much we can do about that, but wait patiently to see if he'll accept it over time. Arm restraints are another option, and we may have to resort to that if nothing else works - the "restraints" are also known as "no-no's," and are really just braces that keep kids from bending their arms at the elbows. This would keep him from forcibly removing his mask. I really don't want to have to do that, however. For one thing, he needs to be able to remove it if he is congested. Also, he vomits occasionally. So we can't have him throwing up into a C-Pap mask.

I am so, so glad we have our trip to the Cleveland Clinic scheduled for September 6. We really need some objective guidance on what to do with the little guy!

Exhausted

2011-08-11T06:53:00.000-07:00

We started CPap therapy on Monday night. Nolan whimpered a few times while putting the mask and hose on, but was surprisingly accepting of the whole situation. We read our bedtime stories, then he continued to look at some books until he fell asleep. He was conked out by 9:00pm, and I was relieved that it went so easily.

He woke up at 11:00pm, crying with the blowing air shooting into his eyes (he had dislodged the mask). He was fussy about the whole set-up by that point, and we figured 2 hours for the first night was good enough. We took off the CPap and let him sleep.

Then came Tuesday.

Terrible, terrible Tuesday.

Horrific Tuesday.

A too-big mask hangs over Nolan's upper lip.

He wasn't in a great mood on Tuesday. Maybe because he had slept poorly the night before. Maybe because his stomach has been acting up more than usual. Maybe because he's three-almost-four, and he just felt like being cranky.

Anyhow, he was not going along with the CPap plan on Tuesday night. We put the mask on at 8:00pm. He screamed. He hollered. He cried.

We tried loosening the mask when he cried that it was hurting him.

We tried adjusting the straps when he cried that it was "too tight."

We tried tightening the mask when air leaked out of the top of the mask.

It didn't matter, he just continued to cry. This was a night that I was so, so grateful to have a caring spouse. We took shifts in the boys' room, trying to get Nolan to calm down and trying to get the perfect fit on the mask.

When Dennis was upstairs and I was downstairs, Nolan would scream, "Mommy, HELP ME! HELP ME! Mommy, come here!"

It was awful. I was sobbing.

Is CPap worth it? Will it even work? Will he even accept it? Is this causing more trauma and torture than a surgical fix for his apnea and reflux? Would the "surgical fix" even fix the problem? What are we going to do?

I pulled myself together and traded shifts with Dennis. I put on my Calm Tough Mom face and sat next to the hysterical Nolan.

"Nolan, crying isn't helping. I can't understand what you are saying. If you want Mommy to stay in your room, you have to stop crying. I will read you a book, but you have to stop crying."

I doubted that would work with a three-almost-four year old sent over the edge of sanity by a scary air blowing mask. Surprisingly, it worked.

Maybe because it was 11:00pm and he was really tired.
Maybe because he was tired of fighting.
Maybe because he is three-almost-four and trusts his Mommy.

His hysterics calmed and he fell asleep. He stayed asleep until 2:00am. When he woke up crying, we took the mask off so he could sleep through the rest of the night.

Last night was Wednesday. We put the mask on and there were no hysterics. We read Ferdinand the Bull and the Research Guide on Pirates (to the Magic Tree House series). Nolan fell asleep as I was finishing the biography of Captain Kidd.

The lack of tears and drama/trauma were a welcome relief. Unfortunately, he kept knocking off the mask - every 15 minutes or so, the mask would be hanging loose from his face. There is no alarm when he knocks it off, so I stayed up until midnight to continually replace it, then went to bed. He woke at 1:30am with the mask dangling around his neck, crying.

We got about 2 hours of CPap therapy last night.

The respiratory therapist said the company that makes the toddler-sized masks has discontinued the "small" mask. Nolan currently has a medium sized "mini me" mask and headgear (this fits most preschoolers). Fortunately, the head gear does come in a smaller size. We don't have many options with regard to the mask: we could go to a full-face mask, but the danger of Nolan throwing up in the night into a full-faced mask is very real. We wouldn't want him to suffocate if he got ill in the night: if we try a full face mask, we will need an apnea monitor and alarm. Another option is to try arm restraints. I'm not a fan of that last option.

We have a month to try different configurations, but there aren't a lot of different options available for a child as small as Nolan. The popular "kidsta" masks found online are for kids over the age of 7: if Nolan can't fit into the medium "toddler" mask, there is no way the masks for kids "over the age of 7" will fit.

Anyhow, tonight is Thursday. We will soldier on and try the therapy again tonight. Hopefully, there will be no tears. Hopefully, he will leave the mask on in his sleep.

Hopefully, this will work.

The Cleveland Clinic: A Cut Above

2011-08-09T06:39:00.001-07:00

I was a little worried when I noticed that only about a quarter of Nolan's medical records had been faxed to the Cleveland Clinic. Knowing that we only get one shot at this, I was concerned that the partial records would make for an useless appointment.

I sent in medical records request letters to our ENT, Pediatric Ophthalmologist, and GI doctor, so that some of the missing records would find their way to the Clinic.

Yesterday, the phone rang. It was the genetic counselor at Cleveland Clinic - she noticed that some of the records were missing from the pediatrician's office.

"There is a record mentioning an echocardiogram, but there is no echo. There is a record mentioning an MRI scan, but there is no MRI scan."

"Are there any endoscopy biopsy results? Labwork? Ophthalmology reports?"

"No. There is no mention at all of his vision problems."

"OK, I'll get on that."

There were 54 pages sent, probably about 1/4 of Nolan's medical records. I'm working on getting the rest sent directly from the specialists. The records sent to the Clinic were missing his genetic testing work from 2008 (just Connexin 26, Connexin 30, and mito A1555G, which were all negative for mutations). The records were missing all mention of vision issues, reflux test results, biopsy results, labwork, etc.

The rest of the records should slowly start filtering in to the Clinic this week. I will fax the rest of the missing records (early ABR hearing tests, genetic test results, etc) tomorrow.

I am SO impressed that the Clinic noticed the missing records and called me about them. I honestly don't think anyone has ever sat down and reviewed all of Nolan's medical records before.

We may never have a comprehensive diagnosis, but I am impressed with the attention to detail and the professionalism at the Cleveland Clinic. I feel like I can trust their judgment - they have really looked at his entire case and have seen kids like him before.

I really can't wait for our appointment next month. We may not get answers, but maybe we'll get get pointed in the right direction with our care of Nolan's most pressing medical concerns. Sometimes, getting pointed in the right direction is the most important thing.

C-Pap for Kids: Not Much Fun

2011-08-08T12:06:00.000-07:00

Mr. Bear tries out the C-Pap

I took a little jaunt down to the Home Healthcare Company today - the respiratory therapist there set us up with Nolan's C-Pap machine.

She was rather flummoxed when she saw how tiny Nolan is: he is almost four, but he has that blasted flat nasal bridge and is on the small side for his age. There is a mask for toddlers, but the nasal mask is a bit too big. She is going to do some research online to see if she can find anything smaller (she can't - I've already looked). She did mention using a high-flow nasal cannula: this is what they do for premature infants, since they are too small for the typical C-Pap masks. We're not sure if this would work with an almost-four year old child's breathing method, though (infants breath entirely through their nose - a four year old is more likely to open his mouth and lose the pressure splinting open the airway).

Insurance will cover 50% of the machine. The rest will be paid by us, on a monthly basis. C-Pap machines are sold on a rent-to-own platform. Our first bill will be the highest, since that includes the disposable items like the hose, mask, filters, etc. Insurance will cover the rest. Maybe.

The "maybe" comes in the form of a little blue chip that sits inside the machine.

This chip is our police officer. It records exactly how long Nolan uses the machine each night. If he doesn't use it for a minimum of 4 hours each night, the insurance company will refuse to pay for the machine. Then we'll get stuck with the entire bill.

The monitoring chip was the brainchild of insurance companies, who got tired of adult patients requesting a C-Pap and then not using it because it is uncomfortable. Of course, children are a different scenario entirely. Due to other health concerns in young children, frequent congestion or colds may make wearing the C-Pap difficult at times. Let's face it: it is pretty rare for a 4 year old to require C-Pap, and most kids who do require therapy have complex medical issues.

So if Nolan gets sick on the C-Pap and can't wear it for a few nights, insurance won't cover any of the bill. Mr. Blue Chip says so.

He starts using the C-Pap tonight. We'll see how he does. He's been refluxing like crazy since Friday, and has a rattling cough. He may get too sick on C-Pap to make the entire enterprise feasible. In which case we can just stop the rent-to-own deal and we'll only be out $500.

Of course, if C-Pap doesn't work, we're left with a fundoplication and revision supraglottoplasty as our only options.

Despite the hassle and money involved, I really hope this works. We need our little one to grow!

Getting Things Ready for Cleveland Clinic

2011-08-05T08:09:00.000-07:00

Our pediatrician has faxed off the necessary medical records to Cleveland Clinic - it is a great relief to have this done. I picked up my copy of the faxed documents, and realized several records were missing. They sent:

A letter from his GI noting adequate control of the reflux (this was sent a year ago).
A letter from his ENT showing poor control of his reflux, and showing the diagnosis of laryngomalacia.
One audiogram.
A report from Nolan's posterior urethral valve surgery.
One sleep study report.
His normal EEG findings from the staring spells a long time ago.

They are missing all the labwork (showing the slightly elevated AST levels), his endoscopy biopsy results (showing ectopic gastric mucosa and flattened villi), his MRI's and CT scans, all ophthamology reports, and all of his pH probe results. Not to mention the four sets of tubes from chronic middle ear infections, the history of being failure-to-thrive (especially since he is dropping on the charts again), and the surgical failures to control apnea (tonsillectomy and supraglottoplasty). And then there is the mixed hearing loss which is slowly progressing over time.

I made a few phone calls to specialists this morning: I called the GI to get his biopsy record, lab work results, and pH probe results faxed over. I called the ENT and the audiologist to have the same thing done. I still need to call the ophthalmologist.

I have several release forms to fill out and send back to the specialists - it takes quite a lot of work to get everything organized. I wouldn't be so worried about the whole thing, but our insurance company has approved only ONE visit with the geneticist, and won't cover any lab work or follow-up visits. Because of this, we need to have all the ducks in a row. We only get one shot at this - we need to make it count.

Hopefully all of the records will be into the Cleveland Clinic in short order.

In Which I am Very Stealthy

2011-08-03T15:42:00.000-07:00

Matt and Nolan at Library Story Hour.

This blasted ear infection is driving me crazy. It isn't exactly clearing up, and the ear drops are such torture for Nolan. I discovered a way to get the ear drops into him without any upset, though. I wait until he's deeply asleep, then I sneak into his room, put the drops near his ear canal, tip his pillow, and then hold my breath for 2 minutes. He fusses for just a second, then goes right back to sleep.

I have excellent ninja skills.

This tactic does not work in the early morning, however. When I try giving him ear drops at 6:00am, I end up with a howling, angry boy. Apparently, my ninja skills do not work during daytime hours.

Anyhow, his ear is still leaking some fluid and looks kind of nasty through the otoscope. I'm not a doctor, but I'm pretty sure "oozing" means "still infected." I'll give it until Friday (one week), then I'm either calling the pediatrician back or calling the ENT's office. I don't want a repeat of last year's 8 week long epic ear infection.

Ugh.

Ear Drops of Doom

2011-07-31T06:10:00.000-07:00

Hiking in Chautauqua Gorge

I managed to get Nolan into the pediatrician on Friday evening, and his right ear is definitely infected. The nurse practitioner also weighed him and he's down to 30.5 pounds (he had been at a hefty 32 pounds a few months ago). They prescribed Ofloxacin (antibiotic) ear drops, and we went on our merry way.

I dread ear infections. The pain of the infection never seems to bother Nolan much. We don't even know he has an infection until we see blood and "goop" pouring out of his ear. The most horrible part of his ear infections is the treatment: the dreaded ear drops.

I know, I know. Ear drops don't sound all that bad. Especially compared to the other things Nolan has been through. For Nolan, however, ear drops are equated to some form of medieval torture. We have to pin him down when we add them to his ear, then rock him as he screams for the next 20 minutes. The medication hurts when it goes through the tube into the middle ear. Then there is the dripping feeling of the medication sliding back out of the ear, which Nolan cannot stand.

The good news is that his T-tubes are still firmly ensconced in his eardrum. With the standard ventilation tubes, this infection would have pushed the tube out and we would have been headed back into surgery to replace a tube. The T-tubes are much sturdier, and have withstood the infection process.

We only have to do the drops for seven days, so hopefully the time will pass quickly. Also, I hope we don't get a repeat of last year's eight-week-long ear infection drama.

Our August is currently (mostly) doctor-free. I would really like to keep it that way.

Dancing with Insurance Again

2011-07-29T12:30:00.000-07:00

The sleep neurologist's office called to let me know that the CPap information had been sent to the Home Healthcare Company (HHC). I gave the HHC a call to determine what we needed to do next (if anything): apparently, we just have to sit tight and see if our insurance company will pay for a CPap machine. The insurance company will likely require an authorization, then they'll decide if they will chip in for the machine or not. You know, because breathing is totally a "lifestyle choice."*

Like hearing aids (and many other "durable medical equipment"), CPap machines are often not covered. Fortunately, we'll "only" be out about $1,000 if our insurance company refuses to pay for the machine. We'll see what happens.

In the meantime, Nolan is sporting his first ear infection since getting T-Tubes. He brought me a clogged hearing aid and a leaky ear: it isn't pretty, folks. He has gone nearly 8 months without an infection, which is pretty record-setting in our world. Hopefully a round of Ciprodex drops will clear this one up (oh, the joys)!

*Hearing aids are often not covered because wearing them is considered a "lifestyle choice" among adults. Unfortunately, children learning to listen and speak are adversely affected by the refusal of insurance companies to cover hearing instruments.

Two Ears Again

2011-07-26T17:51:00.000-07:00

Nolan last week, when he was missing a hearing aid.

Today was a little busy: Vacation Bible School followed by a trip to Nolan's audiologist in Buffalo. A wonderful friend volunteered to take Matt on a little "field trip" to a local swimming pool with her kids while Nolan and I traipsed up to the hearing center. This was a wonderful turn of events: Matt didn't have to suffer through another four hours in the car, and I only had one child to attend to on the trip. Some friends are simply awesome!

We got his hearing aid back, and the report does show that there was some moisture damage. It is now repaired, and we have a warranty for 12 months on the aid. That is $250 well spent: much cheaper than purchasing a new hearing aid.

Of course, there was a bit of drama on the way home. Nolan ripped out the recently repaired hearing aid and declared that it was dead. I reached back for it, and sure enough, there was not a squeal of feedback to be heard. I turned it on and off a few times to no avail. I checked the filter on top of the microphone, taking it off and replacing it. No luck. I couldn't believe it!

I tried taking off the earmold, and the hearing aid squealed to life. To my great relief, Nolan's right ear had simply developed an impressive amount of hearing aid clogging wax. A simple solution, thank goodness, and something we'll have to check for since that ear has been unfettered by an earmold for about a month.

We won't have to see audiology until January or so, when Nolan will have another hearing test done. He's still on an every-six-month schedule since his hearing loss has fluctuated and changed over time (albeit at a very slow pace). Hopefully he'll be stable and we can move onto an every-twelve-months schedule.

C-Pap Titration

2011-07-22T13:33:00.001-07:00

Nolan and I headed up to Buffalo Children's last night for his fourth sleep study: this time a C-Pap (Continuous Positive Airway Pressure) machine would be titrated during the study.

Our favorite sleep study technician was only in for the evening, so another technician did most of Nolan's hook-up and study. Still, the respiratory therapist extraordinaire prepared several "prizes" for Nolan's departure the following morning, and set him up with some fabulous Lightning McQueen sleeping accessories.

The hook-up started shortly thereafter. The amount of wires that is involved is pretty impressive: a partial EEG array, 2 EKG leads, 4 leads on the legs to detect movement, leads on the chin and near the eyes to detect an open mouth and eye movement.

Nolan was absolutely brilliant during the hook-up of the leads. This is his fourth time, and he has an experience book that helps him understand what will happen.

The nasal cannula always makes him cry for a moment. He really hates any "tubie" up his nose, but he recovered fairly quickly from that part of the procedure.

Unfortunately, the mask to the C-Pap would prove more difficult for him. He started sobbing when they placed the mask over his nose, even with no air flowing. Once they started the air flow (at the minimum pressure of "3"), he absolutely flipped out. Fortunately, the respiratory therapist had a brilliant idea and snagged a mask for me: I was able to wear the mask and help Nolan through the process of getting it on. We even hooked up the air to my mask, so that Nolan could see that it didn't hurt and wasn't really scary at all.

This worked for a little while, anyway. The airflow does feel strange, and along with the feeling of the mask, was a bit much for an almost-four-year-old boy. There was a bit of crying and a lot of fitful sleep in the early evening.

They titrate the C-Pap machine by increasing the air pressure until the apneas and hypopneas disappear. Because the respiratory therapist cannot give me any information, I have no idea what Nolan's final pressure was. I do know the air was blowing quite a bit more forcefully by the end of the study.

The little guy barely slept at all until 4:00am. We had to replace his nasal cannula twice, which required removing the C-Pap mask and then reattaching everything. At some point, I realized he had wet the bed, which required removing and reattaching the leg leads as I changed him (note to self: future studies will be done in a Pull-Up).

He was mighty glad to get everything off in the morning, and we headed home with his trademark goopy head and "powers." I should get a call from the Home Healthcare Company (HHC) within the next week or so: they will arrange a meeting to pick up his machine and masks. I'll find out the pressure level settings at that point, since the machine will have to be set and ramped up to the correct pressure.

I have my fingers crossed and prayers said that the C-Pap will work to stop the apnea!

Information from the Cleveland Clinic

2011-07-13T17:07:00.001-07:00

The Cleveland Clinic sent off an information packet very quickly: I received it in the mail at the end of last week. It includes a map and a timeline of our appointment on September 6 (we meet with the genetic counselor first, then with the geneticist). They also included a fax number for Nolan's medical records - I talked to our pediatrician's office and they will probably send them on Saturday. Nolan's medical records are so extensive it will tie up the fax machine for a long time, so they don't want to do it during business hours.

Since our insurance only approved a solitary appointment with the geneticist at the Cleveland Clinic, further visits (and/or any tests) would have to be approved separately. I spend a significant amount of time battling our insurance company - I have a feeling that is not likely to change any time soon. I suppose we'll cross that bridge when we come to it.

We don't have much else going on, other than Nolan's C-Pap titration on the 21st of July. He's had a lot of tummy issues (reflux and some lower GI issues) lately, so hopefully those will settle a bit before the sleep study next week.

July 2011 Audiogram

2011-07-11T07:33:00.003-07:00

Sailing was a success - we went out on the lake and had a great time. Nolan's hearing aids stayed at home, but he did fairly well on the boat. We explained that he had to sit tight on the boat (we don't have a motor, so we use the sail as our sole means of power). If you have never sailed, it requires a bit of activity when tacking (going back and forth) - the occupants have to switch sides so that the person in control of the main sheet can control the sail. Fortunately, neither boy weighs enough to affect the balance of the boat - they were able to sit tight while Dennis and I darted back and forth across the boat. The wind did die down, unfortunately, which meant we had to drop sail and row back to the dock (the lake looked like a sheet of glass - never a good thing in a sail boat)!

I did get Nolan's most recent audiogram in the mail. His bone conduction is still slightly better than his air conduction, but there is no chance that his loss is conductive. His best bone conduction scores were at 40dB at 500Hz (this means the sensorineural portion of his loss is moderate). His actual hearing level is moderate severe, with his hearing at 55-65dB in all frequencies.

His (unaided) speech detection thresholds are 45dB for the right ear, and 50dB for the left ear (this is the level that he can detect speech is present).

They also performed a word recognition task: Nolan was able to identify 88% of the words in his right ear when the speech was presented at 85dB volume. He was able to identify 92% of words in his left ear when the speech was presented at 90dB volume. This testing is done using Nu-CHIPS, a word recognition hearing test designed for preschoolers. Basically, about 40dB of volume is added to the child's speech awareness thresholds, and words are presented at that level.

Nolan's word recognition is pretty good at the 85-90dB presentation level. Remember, of course, that 85-90dB is MUCH louder than typical conversation: he can't even detect speech is present at normal conversational volumes. Essentially, he can understand words without his aids when you bellow at him. That residual hearing is useful for sailing and other water sports, however, when he doesn't have his aids in!

His audiogram looks identical to the one in June. He can't hear the sounds above the line (unaided), he can hear everything below the line. Both ears are identical in hearing level.

New Language This Weekend

2011-07-09T17:22:00.000-07:00

Tack

Jibe
Batten
Jib
Block
Sheet
Boom
Clew
Coaming
Shrouds

Sail

Now that the kids are old enough to follow directions, we're taking the boat out again. Nolan is bound to learn a lot of new language (we're still hunting for a batten)!

Upper GI Results

2011-07-08T07:09:00.000-07:00

After playing a bit of phone tag, I finally got the results to Nolan's Upper GI Barium study. As expected, his anatomy is entirely normal. Since barium studies are not intended to diagnose or evaluate reflux (scintiscans and pH probes are far better, and only a pH probe can diagnose the severity of an acid reflux disorder), we didn't expect to see any reflux on the X-rays. Nolan's first upper GI barium study was entirely normal, but they did manage to catch a reflux event during the most recent test.

After talking to the nurse practitioner with our ENT clinic, I found that they do Upper GI studies routinely prior to fundoplications. The pediatric surgeon needs to verify normal anatomy (or abnormal anatomy) prior to embarking upon the procedure. In addition, certain conditions (like hiatal hernias) are repaired via a fundoplication.

If (and that is a big IF) we decided to proceed with a fundoplication, the first studies are completed: we won't have to go through all these tests once we visit the pediatric surgeon (if we didn't do them now, we would have to visit the surgeon, then do the tests, and then return to the surgeon again).

In the meantime, Nolan is coughing and gagging a lot - his blasted reflux seems to get consistently worse rather than better. We can hear his stridor increasing, and he complains about getting "throw-up" in his mouth. Reflux can flair up randomly, so I'm hopeful that this recent flair will dissipate soon. Ugh.

The First Black Eye

2011-07-07T08:43:00.000-07:00

Soccer Ball + Glasses = Ouch.

Appointment Made for the Cleveland Clinic

2011-07-06T08:11:00.001-07:00

Pure summer fun.

I called the financial services department at the Cleveland Clinic to inform them we had received a letter of approval from our insurance company. The financial services person had to call the insurance company to verify the authorization, so I hung up and figured it would take a week or so to get a call back to schedule an appointment.

Holy Guacamole, the Cleveland Clinic is fast. Within half an hour, the phone was ringing and the genetics department was on the line. They're scheduling into September now, so Nolan's appointment will be on September 6.

Apparently, the Cleveland Clinic is approximately the size of a small continent - they are sending an information packet and map system so that we can find our way through the campus (there are over 20 buildings on the main campus). They're also sending fax information and history forms, so that Nolan's pediatrician can fax the relevant information to the geneticist.

That's one major item crossed off the "to-do" list, and I am very pleased that we snagged our second-opinion appointment prior to the follow-up with the ENT in late September (when we will revisit the idea of a fundoplication referral).

Hearing Test, Redux

2011-07-05T16:27:00.000-07:00

Nolan and Casey the Snuggle Puppy

Nolan had a confirmatory hearing test today. His results look identical to the test last month, so his audiologist increased the amplification in his aids to suit the new audiogram. This is actually a bit overdue, since we saw those high frequencies drop off in the left ear last November - but that test was performed at Buffalo Children's, and they can't adjust hearing aids. Nolan's own audiologist hadn't seen that drop before, so she wanted to confirm before increasing the amplification.

Nolan's right hearing aid, which had been totally dead, sprang to life as soon as we pulled into the audiologist's parking lot. It is a lot like taking a car to the mechanic, and having it sound perfectly normal as soon as the professional looks it over. We still sent it in for repairs, since it is eating through a battery per day (he normally only goes through one per week). We'll go back in two weeks to pick it up - hopefully we won't have any more problems with it!

After the hearing test, we took a side trip to Buffalo Children's hospital to see if Matthew had left his hand-held video game in radiology during Nolan's Upper GI scan. Sure enough, it was sitting behind the radiology desk. I was so glad it was there! I think I am going to start labeling all of the "travel" toys - it is not-so-fun to run up to Buffalo when something expensive gets left behind!

We're all tired after the Independence Day weekend, which we spent with friends. The boys swam in a pond, played in a pool, roasted marshmallows, played with sparklers, and watched fireworks. After all the fun activities, we're ready for a few quiet days at home. Fortunately, Nolan's C-Pap titration was rescheduled for the 21st of July, so we have an entire week of relaxation ahead of us.

Approved

2011-07-02T16:59:00.000-07:00

The relief I feel is enormous. I just received word that a visit to see Dr. M at the Cleveland Clinic has been approved. Our insurance company will not cover any tests or procedures there, but they will allow us to get a second opinion with a geneticist.

I'll call on Tuesday to make an appointment. We should get in by mid August to early September.

I am so thankful to get the second opinion - we really need reassurance and advice before we think about doing any more surgeries.

Now I can relax and get back to worrying about the dead hearing aid!

Feedback, I Miss You

2011-06-30T12:46:00.000-07:00

~~Picking~~ Eating Strawberries

Nolan's right hearing aid has not revived itself after several cycles in the Dry and Store. I heard a very faint squeal yesterday morning, but nothing since that point in time. I have a feeling it is completely and utterly dead.

We see his audiologist on July 5 for a confirmatory hearing test, so hopefully she'll have some insight into what has happened to the aid. I never thought I'd say it, but I would kill to hear just a little bit of feedback from that aid right about now. The thought of having to replace his hearing aids makes me kind of queasy - those puppies are not cheap.

In other news, we are still fighting the insurance company for approval to get a second opinion with genetics. Our pediatrician informed me that they are giving her a lot of grief. They reviewed his symptoms and told her that having an overall diagnosis wouldn't change his treatment plan.

Therein lies the rub: if we don't know what is the cause of the various systemic problems, we cannot treat Nolan appropriately. Certain diagnoses do change the treatment plan, since some things are contraindicated for certain conditions. In addition, what if Nolan has some central problem that is treatable? It would kill me to miss a condition which could be treated by an enzyme replacement therapy or other methods. The truth is, you cannot appropriately address the issues in a medically complex child when you don't know what the underlying cause of the problems is.

A fundoplication is contraindicated in many children with neuromuscular disorders, for instance, because motility problems cause pretty horrible side effects in some kids.

On the flip side, if the geneticist tells us that there is no underlying condition, then it is more likely that Nolan will outgrow some of his problems. There is absolutely no way I would have a fundoplication performed on Nolan if there is a chance that he will simply outgrow the reflux.

Hopefully we'll get the approval from the insurance company soon.

Jumping Through Hoops and Some Technical Difficulties

2011-06-28T12:50:00.000-07:00

Nolan is the king of pouting. Some days, he has good reason.

The insurance company responded to our pediatrician's pre-authorization letter requesting a genetics consult at the Cleveland Clinic. They said that the authorization will be denied because Nolan has already had genetic testing, and our pediatrician needs to explain why the former genetic testing was insufficient.

So we move on to round 2 of getting approval to get a second opinion.

For the record, Nolan has only had genetic testing done for Connexin 26, Connexin 30, and the mitochondrial A1555G mutation: this is part of a 3-gene "Non-Syndromic Deafness" panel that is standard for nearly all children with hearing loss. His blood was taken at Buffalo Children's and sent to Boston for testing. Nolan was negative for any mutations in those three genes.

All of the "extra" medical issues have occurred since that point in time (he was only four months old during our genetics consult at WCHOB).

Our insurance company claims that Nolan was seen at Boston Children's Hospital, constituting a second opinion. This proves my theory that the insurance company employee reviewing the request cannot read.

The genetics report by Boston Children's is just the analytical data from the blood sample sent to them by WCHOB. We have never been to Boston, we have never gotten a second opinion, and the genetic test drawn by WCHOB and sent to Boston for testing was negative. So we know he doesn't have the most common gene responsible for non-syndromic deafness. This, coupled with the onset of new symptoms (including a machine necessary to keep him breathing at night, vision problems, muscle tone issues, etc.) should be enough to warrant a second opinion from a geneticist.

Hopefully the rebuttal letter our pediatrician is penning will be sufficient to grant us access to Cleveland Clinic. We're not seeking tons of expensive testing: we're simply seeking a second opinion from a clinical geneticist who works for a hospital which has a unit specializing in genetics relating to hearing loss.

In other news, Nolan's right hearing aid died on us today. It had been giving us a low "hum" and eating batteries. We normally go through one set of batteries per week with Nolan, and we started going through a battery per day. Besides the expense of buying a bazillion Size 13 batteries, there was the more pressing concern that Nolan's hearing aid might be ready to kick the bucket. It won't turn on at all today, so he is currently sporting only one "ear" and saying, "WHAT YOU SAY?" a lot. We see his audiologist on the 5th for a repeat hearing test, anyway, so hopefully she'll be able to help us find a solution. I just hope that solution is not buying new hearing aids.

Easy Peasy

2011-06-27T16:07:00.001-07:00

Nolan's first upper GI barium test was a little bit of an ordeal - he wouldn't drink it, so they had to administer the barium via NG tube, and then we had to wait around for a while to make sure the barium cleared his system. Plus, the radiology waiting room was way overbooked, so there was nowhere to sit or stand while waiting for the test.

This time was a complete 180 from the first test. There was no-one in radiology except for us (and one other family completing an upper GI), and we were called back at exactly 9:30am for the test. They did one "pilot" X-ray of Nolan's empty stomach, and then called him back for the barium test. While I can't stay in the room during the actual test, we had talked about the procedure over the weekend.

Nolan agreed to drink the barium while standing up (the table rotates to accommodate this), and was absolutely brilliant about the whole thing. He didn't seem to mind the barium much - his Zantac tastes much worse, and as long as he didn't have to get a "tubie," he thought drinking the barium was just dandy.

The test was so fast I could hardly believe it - within 15 minutes they said they were done and sent us on our merry way. We pulled out of the parking garage by 10:30am, and headed to the Buffalo Zoo. We spent a lovely afternoon as a family, looking at the elephants and gorillas.

The report will be at the ENT's office by tomorrow afternoon. It is most likely normal: his first scan was also completely normal, even though he had extremely severe reflux. For some reason, Nolan's reflux is off-the-charts severe on the probes, but never seems to show up on the scintiscans or the upper GI series.

At least it is one more appointment done and over with. The rest of this week slotted with lots of outdoor play and soccer games!

We Love Wonderful Summer Weekends

2011-06-26T14:26:00.000-07:00

The weather hasn't been that wonderful in our neck of the woods. The temperature is above freezing, however, so we're not complaining. We have spent most of this past weekend fishing on our local lake: Nolan and Matt both caught lots of little sunfish, perch, and even a small bass!

Tomorrow we head back up to Buffalo Children's for Nolan's upper GI series: he can't eat after midnight, and we have to keep him happy for the 2 hour ride up to Children's. Hopefully he'll drink the barium this time - last time he refused and they had to thread an NG tube (a tube that goes up the nose and into the stomach) to administer the barium. We've talked about it a bit, and Nolan agrees that he'll drink the white "medicine" tomorrow. He hates "tubies," so there is a good chance that he'll cooperate this time.

If he gets out of there on time, we'll get to go to the zoo afterward. I'm not promising that yet, though, because sometimes they take quite a few "pictures" after the initial study (i.e. I'm not sure if they're doing a small bowel follow-through tomorrow).

It'll make for a long day, but at least he can eat as soon as the X-rays are completed. Hopefully we'll be home in time to go swimming and play outside a little tomorrow afternoon!

Welcome to C-Pap

2011-06-22T09:40:00.001-07:00

Nolan, sans equipment

We took a trip to the sleep neurologist yesterday. The sleep center has an office in Dunkirk, which is (thankfully) only about an hour away from our hometown.

The sleep neurologist told us several things, which are all very comforting. The first is that Nolan's sleep apnea is now at a level that is not dangerous to his heart. If we didn't treat it at all (and it remained at the moderate level), his heart wouldn't be harmed at all. His growth and mental development is another issue, though, so we do have to treat it. Without getting the right amount of sleep (and oxygen) at night, his growth will slow and he will struggle with fatigue and the ability to focus in school. Interestingly, the incidence of sleep apnea is quite high in children diagnosed with ADHD.

The other wonderful bit of news is that there is a possibility that Nolan will grow out of the apnea. It has slowly been getting better since it was first diagnosed: whether that was due to the surgeries or due to growth is unknown. We can't know for sure, but there is the potential that as his airway grows, the laryngomalacia will have less of an impact on his ability to breathe at night.

This is an important bit of information, because we do not want to embark on a permanent procedure (the Nissen Fundoplication) for a problem that may be temporary.

Our biggest concern with the C-Pap was the fact that Nolan has midface hypoplasia (this causes his flat nasal bridge). A C-Pap mask places pressure on the face, and can cause the mid-face to retrude as the child grows: this is an issue with any child on C-Pap, but is a bigger issue in a child who already has a retracted mid-face area. The sleep neurologist wasn't concerned about this at all, because we can use several different masks with different pressure points, and alternate them every few days.

The other concern is the potential for the C-Pap to open his airways and allow the acid reflux to penetrate his lungs. This will effectively determine if C-Pap is a viable intervention for Nolan's apnea. If he starts developing aspiration pneumonia or nodules on his vocal cords, we'll have to stop C-Pap therapy. On the other hand, sometimes C-Pap improves night-time apnea because it removes the obstruction (an obstruction in the airway creates a negative pressure in the chest, which effectively sucks the reflux up the esophagus). Since Nolan's reflux is worse during the day than the night, it is unlikely that the C-Pap will eliminate the reflux. Still, the thought that it might actually help with the night-time reflux is encouraging.

The only way to know if the C-Pap will shoot his reflux into his lungs or help prevent the reflux from occurring is to go through a C-Pap trial. Nolan is scheduled for a C-Pap titration on July 7.

The C-Pap titration is basically a sleep study, but Nolan wears a C-Pap mask. When he goes to sleep, the sleep technician adjusts the pressure of the C-Pap machine until the apneas disappear. If he fails C-Pap (a possibility because of his hypotonia), they'll discontinue the C-Pap and try a BiPap. A BiPap lowers the air pressure to make exhalation easier - it is recommended in kids with hypotonia, but the insurance company won't cover it unless C-Pap is attempted first.

The Home Healthcare Company (HHC) will contact us after the sleep study to set up Nolan's machine. I really hate adding another piece of equipment to the dear boy's regime. It is certainly better than doing an irreversible surgical procedure that may cause terrible side effects, though, so we are all for giving it a try. The best thing is that a C-Pap is entirely reversible: if Nolan actually does outgrow the apnea, then we can simply stop using the C-Pap with no permanent side effects.

The neurologist measured and weighed Nolan before we left. He measured in at 39", which is wonderful. He is back on his height percentile line (he had been slipping down the percentiles, but a sudden growth spurt has brought him back to where he belongs). The bad news is that he weighed in at 31 pounds: he has lost a pound. This places his weight-for-stature somewhere between the 5th-10th percentiles (hey, he's on the charts, so I'm not complaining)! It appears our dear boy cannot grow up and out at the same time.

Hopefully the C-Pap will make growing a little easier for him.

Sometimes I Love Living in a Small Town

2011-06-19T16:58:00.000-07:00

Every boy needs a dog.

I ran into our pediatrician at the grocery store tonight (I also ran into three other families we know). Living in a small town can be wonderful sometimes - our pediatrician stopped by to see how Nolan was doing and to tell me that they faxed the letter off to the insurance company. She thinks we're doing the right thing by requesting a second opinion with a geneticist (and for putting off the fundoplication until we have proof that it is absolutely necessary).

She did confirm that it is not a minor surgery, but a rather major procedure. And that it is generally only performed for children who have life-threatening reflux and require a feeding tube. Since we're concerned about motility, there is the possibility of adding pro-motility agents to Nolan's medication regime prior to embarking on a permanent surgical procedure that has significant side effects.

Since the apnea is the biggest issue with Nolan's reflux (potential damage to his vocal cords is another problem with the airway reflux), it is not unreasonable to attempt a C-Pap or BiPap (as his muscle tone allows) to try and correct the apnea. In a nutshell, let's try some less invasive and less risky interventions before proceeding with a major, irreversible procedure. A basic principal: First Do No Harm.

The letter is with the insurance company and hopefully the approval to see Cleveland Clinic genetics will come through post-haste.

We see the sleep neurologist on Tuesday and I have a few questions for him, including:

Is the apnea likely to be caused by the reflux, or is it more likely to be caused by a physical obstruction/low muscle tone/recurrent laryngomalacia?
Is a C-Pap absolutely contraindicated for kids with silent reflux?
How likely is Nolan to grow out of the apnea issues (since his airway will increase in size as he grows)?

If the sleep neuro believes that a C-Pap (or BiPap) is a viable method for controlling the apnea, then we will likely be referred to a pulmonologist for more sleep studies and C-Pap titration. C-Pap isn't without its own set of issues (pressure on a growing face, a real issue for a kiddo like Nolan who has midface hypoplasia), but they are much less of a concern than the issues that come along with a fundoplication. Plus, if the C-Pap doesn't work (or he outgrows the need for it), it is entirely reversible. A Nissen Fundoplication is not.

We'll see what the sleep neurologist thinks on Tuesday.

A Bit of a Break

2011-06-17T06:25:00.000-07:00

I took the boys on a road trip to Buffalo Children's yesterday, for Nolan's annual renal ultrasound. They've always been normal in the past, so we were happy to see another set of normal, healthy kidneys. After the ultrasound, there was a bit of a wait to get into the clinic at 1:00 to discuss the results and future plan for Nolan. Killing time in a children's hospital that lacks playing areas for outpatient testing is not an easy task - I managed to sneak back into the radiology waiting room and let the boys watch TV for about an hour, then we headed to the cafeteria for lunch.

WCHOB is a great hospital for inpatient stays or for day surgery patients, but it lacks amenities for kids undergoing outpatient testing. For the longest time, they didn't even have changing tables in the restrooms (thank goodness, they seem to have gotten a clue and added a few of them). In our early days there, I would have to change Nolan on the dirty tile floor of the bathroom.

The cafeteria, for instance, only offers 0.5 liter bottles of soda and juice: there is no juice box or milk carton to be seen. There aren't really any meal choices for kids, either, outside of pizza/fried chicken strips/onion rings. I sat the kids down with their lunches (definitely not reflux friendly) and vowed to BRING our lunches next time. Nolan, of course, immediately spilled his Hawaiian Punch all over the table and himself. Neither boy ate very much, and it was difficult to kill time with a wet and sticky three year old.

We headed up to the clinic a little early, since I have experience with this system. Our appointment was at 1:00pm, and I knew that everyone had an appointment for 1:00pm. Basically, they open up the window and it is a mad dash to sign your name to the list - the child is called based on the position they hold on the sign-in sheet. We joined two other families that were long-term patients: we were all in the know with regard to the madhouse that the urology clinic can be. We heard the shutter roll up, and we made our mad dash to sign in on the sheet. I felt a *little* guilty for not informing the new parents of this crazy system, but I wasn't waiting 3 hours to get in like last year.

We were the second family called in (I could hear the other parents in the waiting room beginning to murmur "wait, your appointment is at one o'clock? So is ours!"). Dr. W. brought in a resident and explained Nolan's case - he's apparently pretty famous since posterior urethral valves are fairly rare (1/8000 births). He's also famous because we caught his valves before they did any damage: since it was an "accidental" finding, we spared his bladder and kidneys. Usually, late-presenting boys are found when they go into kidney failure.

I was hoping we'd be discharged from urology this year, but apparently Nolan will be a lifelong patient. He probably won't have the negative consequences of having PUV's, but there is no guarantee that we won't see problems emerge as he grows. Since he is doing so amazingly well, however, we don't have to come back for two full years. Yippee! I am glad to catch a break with this system.

I also talked to the urologist about Matt's situation. Matt has been on the DDAVP for about 3-4 weeks, and we haven't seen much of an improvement. For about 2-3 days, we thought we had a dry Matt during the day, but he is just as wet as ever. The urologist explained that the DDAVP is not supposed to be used for daytime wetting. Since it is taken at night, the drug is entirely out of the system by the time the child wakes in the morning (it only lasts about 8 hours). We'll finish out this week on the drug, then request an appointment with Dr. W. for Matthew to find out why he can't achieve daytime continence. Fortunately, the investigation consists of an X-Ray and an ultrasound, which are easy, non-invasive tests.

Starting Soccer

2011-06-14T05:16:00.000-07:00

Soccer is awesome.

We missed Nolan's first soccer practice because we returned late from his ophthalmologist appointment. He was rather upset about this, but cheered up considerably on Saturday: he would get to play soccer at last!

We was even more ecstatic when he received a jersey. The Under-4 league has jerseys already, and the Under-6 league does not - this meant that he constantly told Matt: "look at my shirt, Matt. I have a soccer shirt! You don't have a soccer shirt!" This began to wear on Matthew after a while (he should get his uniform by the end of this month).

I brought the FM system to the field with Nolan. I hadn't indicated any "special needs" on his soccer enrollment form - so it was probably a bit of a surprise to his coach. To be honest, I had simply forgotten to write it down. We're so used to his hearing aids, that we don't think about it much. I stuck the mic on her shirt with a brief "don't worry, he does just fine" and walked away.

And Nolan did great. He ran with the ball, he listened to the instructions, and he played the games. He was slower than a lot of the other kids (he's never going to catch the other preschoolers in duck-duck-goose), but that is mostly because he is smaller than most of the other kids. Those short little legs have to work harder to keep up!

Nolan: the little guy on the left.

He is extremely excited to play the rest of the season. He wouldn't take his shirt off when we got home, and was very proud of being "Number Seven." Soccer makes him happy, and that makes me happy.

The Letter is Being Faxed

2011-06-13T10:25:00.000-07:00

Our pediatrician's office is in the process of drafting a letter to our insurance company, in the hopes that they will authorize our trip to the Cleveland Clinic. They asked me for a general synopsis of Nolan's medical history and our reasons for wanting to go to the Cleveland Clinic, so I sent them the sheet of paper I take to each new doctor (his medical history is now long enough that I can't spit it all out in a few seconds). Here is what that paper looks like:

Nolan's Medical History

Gastrointestinal: Severe GERD unresponsive to medical therapy, mild delayed gastric emptying, slow bolus transit time noted during first impedance probe.

Urology: Posterior urethral valves, ablated in June 2009.

ENT: Obstructive sleep apnea unresponsive to surgical measures (tonsillectomy and supraglottoplasty), chronic otitis media (four sets of PE tubes by age 3) congenital bilateral hearing loss (moderately severe mixed hearing loss), severe laryngomalacia. Wears 2 hearing aids.

Neurology: History of severe central sleep apnea (resolved), mild hypotonia.

Vision: Anisometropic amblyopia (myopic in the right eye); astigmatism in both eyes. Wears glasses.

CURRENT MEDICATIONS:

Nexium, 20mg, 2x per day. Zantac 30mg, 2x per day.

PRIOR HOSPITALIZATIONS and tests:

October 24, 2007: ABR diagnosing mild/moderate bilateral hearing loss.

February 2008: Genetics evaluation: Negative for Connexin 26, Connexin 30, and mitochondrial A1555G mutations (non-syndromic hearing loss panel).

March 18, 2008: sedated CT scan of the inner ear (normal).

March 27, 2008: PE tubes placed (set #1).

August 11, 2008: Sedated MRI of the inner ear (normal).

December 2008: Second set of PE tubes placed.

May 2009: Gastric emptying scintiscan (normal for reflux/mild delayed gastric emptying)

June 1, 2009: Cystoscopy and ablation of posterior urethral valves.

July 9, 2009: Upper GI barium (normal).

August 2009: Endoscopy, 24 hour pH/impedence probe (inpatient: severe GERD detected).

January 20, 2010: Sleep study #1: severe central apnea and severe obstructive apnea.

February 10, 2010: Sleep-deprived EEG (20 minute EEG) for staring spells (normal).

March 23, 2010: Sedated MRI for central sleep apnea (normal).

April 13, 2010: Tonsillectomy, Adenoidectomy, PE Tube Set #3 (inpatient PICU monitoring).

August 2010: Sleep study #2: Severe obstructive sleep apnea, mild central sleep apnea.

October 12, 2010: PE tube set #4 (long term “T” tubes placed), Bronchoscopy: Severe Laryngomalacia diagnosed.

November 10, 2010: 24 hour Pharyngeal pH probe: reflux persistent despite PPI treatment. Zantac added to medication regime to stop reflux.

December 3, 2010: Echocardiogram and EKG (normal).

December 2010: 24 hour Pharyngeal pH probe: some reflux still noted with PPI and H2 blocker treatment.

December 14, 2010: Supraglottoplasty and lingual tonsillectomy (inpatient, PICU).

May 3, 2011: Sleep study #3. Central sleep apnea resolved. Moderate obstructive sleep apnea, persistent despite tonsillectomy/adenoidectomy and supraglottoplasty. Fundoplication recommended by ENT. Bi-Pap or C-Pap evaluation recommended by sleep neurologist. Upper GI barium scheduled for 06/27/11; Sleep neuro exam scheduled for 06/21/11; trying to get into Cleveland Clinic for a second opinion from a pediatric geneticist.

Our reasoning for choosing the Cleveland Clinic for the genetics evaluation site was written as follows:

"Due to the repeated surgical procedures, sleep apnea, chronic ear infections, chronic sinusitis, severe GERD (unresponsive to medical therapy), delayed gastric emptying, hypotonia, urology, vision and congenital hearing issues, we are concerned that there may be a single cause underlying the various symptoms that Nolan has experienced. Prior to consenting to additional surgical measures or procedures (recommended fundoplication; C-Pap), we would like to ensure that no genetic syndrome underlies his various issues. The Cleveland Clinic has a renowned genetics program, including a hearing-loss genetics subunit, which is unrivaled in the area. If there is a syndrome or other “issue” at play, we feel confident the Cleveland Clinic will be able to discover the cause – if there is no underlying cause, then we will feel confident with their assessment that his medical history is caused by separate (unrelated) incidents."

Hopefully the insurance company's doctor will look at his history and approve another genetics consultation without giving us a lot of hassle. Our pediatrician's office has been wonderful and is supportive of us getting a second opinion.

The letter should be faxed to the insurance company today, and we should have an authorization approval within 30 days: once the authorization comes through, the Cleveland Clinic will schedule our appointment with the geneticist. This will likely occur in August or September, as it takes a while to get into the Clinic.

In the meantime, our biggest worry is Nolan's fatigue and general "droopiness." This is due to his hypotonia (we think) - the hypotonia affects his upper body and arms the worst; his feet and legs seem generally unaffected. He still has the head lag, though his development hasn't been delayed by it. Still, the effect can be striking in the afternoons. He fatigues while walking, and gets very droopy while sitting or standing.

Nolan, a little droopy in the afternoon

There is a part of me that wonders if his muscle tone issues underlie most of his other problems. The GI tract is made of smooth muscle, so the slower motility and the reflux would be affected by a muscle tone problem. His obstructive apnea could be due to low tone, in addition to reflux and the persistent laryngomalacia. For kids with late-onset laryngomalacia and muscle tone issues, the airway collapse is due to an intrinsic weakness in the system.

We're extremely hopeful that the geneticist at the Cleveland Clinic will be able to piece this together - and if she can't, that she will be able to reassure us that these issues are not connected and that nothing new will pop up to surprise us in the future.

I Want My Sister!

2011-06-11T06:03:00.000-07:00

Nolan, his FM system, and the Tilt-O-Whirl.

I want was driving to Dennis's softball game, when Nolan suddenly shouted from the backseat:

"I want my SISTER!"

Considering that Nolan doesn't have a sister, this was a rather unusual request.

After some considerable questioning, we determined that his "sister" was not a girl, was not a friend, and was not a human child.

He finally clarified,

"I want my sister so I can hear!"

Oh. He wanted his system. His FM system. Wow - that's a first!

The new settings on his FM system are fabulous. I do have to listen to the aids to verify they're set to the right program, but once it is set, he loves it. I didn't realize how often I was tapping him on the shoulder to get him to face me, or shouting over the front seat of the car to the back seat. He used to repeat the same questions endlessly in the car, because he couldn't hear the answer until we screamed it to him. With the FM system, that issue has disappeared.

He wore it to the zoo, he wore it to his last day of preschool, and he wore it to the amusement park yesterday morning. It is simply brilliant. He can hear everyone else, but he can hear me at a slightly higher volume than everyone else. He can also hear me over distance, which is amazing. Normally he can't hear when he gets about 10 feet away in an outdoor environment, even with aids on. With the FM system, distance is not an issue. It was awesome to call to him as he was running the wrong direction in the crowded amusement park, and have him hear me calling to him to stop running.

We plan on using it at his first soccer practice this morning. Hopefully the rain will hold off long enough to let the kids play soccer!

Newest Audiogram

2011-06-09T11:38:00.000-07:00

I received the report from Nolan's most recent hearing test in the mail today. The results look remarkably similar to the test results from Buffalo Children's last November, but they are still a change from the last hearing test at our normal audiologist's office (particularly for the left ear). The low frequencies are still a mixed loss, but everything above 1000Hz is sensorineural. His bone conduction scores have dropped by about the same amount that the air conduction scores have dropped, which indicates that the small new loss is sensorineural (inner ear loss) in nature.

For those who aren't familiar with audiograms, Nolan can't hear anything above the plotted lines when his hearing aids are off. Basically, he can't hear any speech at normal conversational levels with his aids off, though he can hear things like rock music, lawn mowers, and other loud things. With his hearing aids on, he gets most of what lies in the middle frequencies, but still misses out on the high and low frequencies (hearing aid adjustment issues).

Thankfully, the worst hearing is at 65dB (I thought I had seen a 70dB mark on the audiogram in the office, but he is still just moderately severe in the low frequencies).

His speech reception thresholds changed from 40dB in each ear to 45dB (right ear) and 50dB (left ear). His hearing aids are still set for a mild loss in the high frequencies, which explains why John Tracy was showing under-amplification in those frequencies. He has lost a bit in the low frequencies, too - he seems to lose a bit at one frequency (or in one ear), and the rest follow suit shortly thereafter. Fortunately, it is a very slow loss - it has taken almost four years to go from mild/moderate to moderately severe.

We will return on July 5 to confirm the level of loss and (finally) adjust his hearing aids to match the loss.

An Eye Appointment, In Pictures

2011-06-08T17:05:00.000-07:00

Eating lunch in the car.

Heat makes Nolan wilt.

Looking over downtown Erie, PA

Matt plays with Legos during Nolan's appointment.

Dr. S's Office is like Disneyland.

Playing in the clubhouse.

Waiting to see the orthoptist.

Can you see the one that pops out? No, I can't!

All done - we got lollipops and we're on our way!

Nolan's right eye is still amblyopic, but the vision has improved a bit. He now corrects to 20/40 with glasses, which is pretty good for a kid with amblyopia! We are going to give the glasses another three months and re-check his vision in September. On the plus side, it gives his eye a little more time to improve. On the negative side, if we have to patch in September, he might have to wear a patch to school. Hopefully his eye will improve enough to avoid patching altogether!

Audiology: Check (Sort Of)

2011-06-07T18:35:00.000-07:00

Hanging out at the Buffalo Zoo

The drive to Buffalo is long, but fortunately we have ~~invested in hand-held gaming systems~~ reared travel-hardy children. Nolan was a little recalcitrant about going into the audiology booth, swinging his legs and beginning to throw a fit. I used blatant bribery (a trip to the zoo) to get him to cooperate. I'm not above zombifying my kids with electronic entertainment or offering bribes anymore. I think I have "arrived" as a mother.

He cooperated nicely. I think the last full hearing test done at his regular audiologist was over a year ago. Their last record still shows a rising configuration in his left ear, which made his loss moderately severe rising to mild. Our testing at Buffalo Children's last November showed a flat moderately-severe loss, so I wasn't surprised when our current audiologist showed the same thing. I did see one point hit the 70dB mark, which makes me a little anxious. That's a little close to the "S" word (severe) for my liking. Thankfully that was only one frequency.

Before increasing the amplification in his hearing aids, they want to confirm with another hearing test in a month.

Oh, goody.

The strange thing is that his air-bone gap has largely disappeared. This means that his loss is purely sensorineural again - either he's been throwing false air-bone gaps (it happens), or he has a truly fluctuating/progressive hearing loss that proceeds in a very weird fashion (the drops appear conductive at first, but solidify into a sensorineural loss). Weird.

I suppose time will tell. Hopefully he'll stabilize where he's at.

Tomorrow we are going to Erie (a slightly shorter drive, but still an hour each way) to see the ophthalmologist. To patch or not to patch, that is the question!

This is otterly amazing!

A Busy Week Ahead

2011-06-06T08:04:00.000-07:00

Nolan's preschool field trip to the Fire Station

This week is a little busier than most:

Monday: Matt's preschool, Nolan's speech therapy, and Matt's second preschool.

Tuesday: Drive to Buffalo for a hearing test, and to adjust Nolan's FM system. Matt's first soccer practice.

Wednesday: Matt's preschool, then drive to Erie for an ophthalmologist appointment (we get to find out if Nolan gets to rock the fashion world by sporting a patch and going pirate-style). Then Nolan has a soccer practice, and Matt has swimming lessons.

Thursday: Nolan's last day of preschool, and his "birthday" during class. I have a mothers of preschoolers steering meeting, and Matt has preschool.

Friday: Matt has an end-of-year picnic for pre-K at a local amusement park, so we will be spending the day having a ridiculous amount of fun in the sun!

I love the summer months, but June does get a little insane with the end-of-school parties/events, and then we have a bazillion doctor appointments for Nolan. He sees urology on the 16th (this is routine, and we are hopeful he will be discharged from this specialist). The sleep neurologist is scheduled for the 21st, and the upper GI is scheduled for the 27th. At least one trip a week to Buffalo Children's is on tap for the remainder of June: if only the drive were shorter!

The good news is that preschool ends on the 24th of June (for Matt), so July should ease up a bit with the schedule. I plan on spending a lot of time at the local parks, municipal swimming pools, and on "field trips" to the beach!

Getting Authorization

2011-05-31T09:35:00.000-07:00

Oh my goodness: it is finally WARM outside!

I finally managed to get hold of the financial services department at Cleveland Clinic. Our insurance company won't approve a visit to their genetics department until Nolan's primary care doctor writes an authorization to the insurance company (providing all medical records). We're in the process of getting this squared away.

We haven't met our deductible yet, so we will pay out of pocket for most of the costs. Still, it would be nice to have our insurance company cover whatever exceeds our deductible. Since the Cleveland Clinic is a world-class facility, we have great confidence in their opinion. If they say that there is nothing underlying Nolan's hearing loss/severe reflux/fatigue/urology issues/apnea, then we can relax and do the fundoplication surgery.

It can take 30 days for insurance to approve the visit once our pediatrician sends in the authorization, then we will have to book an appointment. At this rate, it is likely we won't get to the Cleveland Clinic until August or September. We could just go to our local children's hospital, but we don't have the same confidence there. We've been told that two separate surgeries would correct Nolan's apnea issues, and neither one has "fixed" his problems. Anyone could see why we're a little dubious about a third surgery, intended to "fix" the apnea. And why we are concerned that something major has been missed, since normal almost-four year olds haven't had so many surgeries (four sets of ear tubes, tonsillectomy and adenoidectomy, posterior urethral valve ablation, supraglottoplasty, upper endoscopy, bronchoscopy... and now recommended for a fundoplication). Typical four year olds also don't wear hearing aids and glasses, and need a machine to help them breathe at night.

I deeply hope our insurance will approve the visit. It will either let us rest assured that nothing is seriously wrong with our boy, or will shed some light on why we have been spending so much time in various doctor's offices.

Mr. Personality

2011-05-29T18:26:00.001-07:00

Nolan is full of hilarity these days. He has the most awesome facial expressions, and has come up with some pretty interesting sayings over the past few days.

"Mom, I need to frost my teeth."

That one didn't take long to figure out - though he may be the first child in the history of the world to love flossing so very much.

"Mom, I want bourbon."

This one took a little longer to figure out. I was pretty sure he wasn't asking for whiskey. I can only imagine what the neighbors think when he spouts off something like this. As if it isn't bad enough that we walk down the street with a leash tied to his bike (to slow him down on the steep hill) - now our kid is asking for bourbon.

After a round of questioning, we determined that bourbon was not a drink. It was also not candy, or food. Exasperated, he shouted, "I want bourbon! I want to pway at the part!"

Oh. Bergman. The local playground. Of course!

I have a feeling we'll be getting interesting notes home from his teacher next year...