Wednesday, December 21, 2011

So Incredibly Busy - Taking a Blog-cation

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:



Wednesday, December 14, 2011

Annual Eye Exam

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!


Tuesday, December 13, 2011

Pediatric Surgeon Consultation Appointment Made


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.



Monday, December 12, 2011

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


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!


Wednesday, December 7, 2011

ENT Visit/Sinus CT Results/Fundoplication Referral


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!

Sunday, December 4, 2011

Stable

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!


Thursday, December 1, 2011

Finally, Dry Ears

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!


Friday, November 25, 2011

Still Leaky

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!

Wednesday, November 23, 2011

Happy Thanksgiving, 2011

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!

Tuesday, November 22, 2011

Party Time (and sinus CT results)


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.


Saturday, November 19, 2011

Ear Drops and Flu Shot Drama

'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!

Friday, November 18, 2011

Rats (Another Ear Infection)

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.

Wednesday, November 16, 2011

A Nice, Easy Appointment

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!

Tuesday, November 15, 2011

Growing (Sleep Neurologist Update)


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!


Saturday, November 12, 2011

Tennis Boy


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.




Friday, November 4, 2011

The Case of the Missing FM


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!

Monday, October 31, 2011

Happy Halloween!


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.

Friday, October 28, 2011

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

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!

Wednesday, October 26, 2011

What a Week!


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!

Friday, October 21, 2011

Loving School


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!

Monday, October 17, 2011

Back to "Normal"


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!

Friday, October 14, 2011

Hanging in There


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.


Monday, October 10, 2011

It's Baa--aaack!

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.



Sunday, October 2, 2011

Holy Growth, Batman!


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!

Friday, September 30, 2011

Another Great Week at School


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. 

Thursday, September 29, 2011

In Which Nolan Gets to Skip All the Screening Tests


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!

Monday, September 26, 2011

September ENT Visit

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!

Friday, September 23, 2011

Mr. Hungry


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!

Monday, September 19, 2011

Still Waiting


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.

Friday, September 16, 2011

New C-Pap Mask and a Happy September

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!

Tuesday, September 13, 2011

Sleep Center Appointment


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!

Sunday, September 11, 2011

September the Eleventh


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.

Friday, September 9, 2011

Updates and Some Good News






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!

Tuesday, September 6, 2011

Update From Our Cleveland Clinic Trip: Genetics


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.