Matt's Sleep Study Results (Post Supraglottoplasty)

Matt's follow-up appointment wasn't until October 28 with the ENT - I figured if there was any "negative" news, we'd find out soon enough.

The ENT's office called and said his apnea is unchanged despite doing a tonsillectomy and adenoidectomy and a supraglottoplasty. They re-booked his appointment for September 22 to re-evaluate his case.

I don't have an official report yet, but the short summary is this:

The bad news: Matt still has moderate/severe sleep apnea.
The good news: I didn't throw out the C-Pap machine!

At least Nolan's apnea is under control for the time being, so we can focus on getting Matt's under control.

Nolan's Sleep Study Results

Nolan's sleep study was an exhausting, sleepless ordeal in late June. He woke frequently and cried. His legs bothered him a lot, and it was obvious from the sleep study report that he has difficulty with periodic leg movements and frequent arousals. He also has moments of bradycardia where his heart rate drops well below 40 beats per minute - this was observed in the PICU when he had his supraglottoplasty as well. He is fine during the day, so it is probably fine - but we will bring it up with his pediatric rheumatologist when we see her in September (along with the leg issues). Dysautonomia occurs with children who have Ehlers Danlos, so it is possible he isn't regulating his heart rate well. Better safe than sorry where the heart is concerned!

On the other hand, we received some fantastic, out-of-this-world news. His second supraglottoplasty/epiglottopexy actually worked. His apnea is now in the mild range for the pediatric age group. This means that we do not have to use C-Pap for the time being. He will have another sleep study next year, as he is high risk for the apnea to return with his connective tissue disorder. We are absolutely thrilled that we do not have to cart around a C-Pap machine and he is getting the oxygen he needs to grow.

So, goodbye C-Pap machine. We won't miss you.

ENT Visit and Culture Results

Nolan had his follow-up with the ENT today. This appointment was supposed to be a post-op for his revision supraglottoplasty, but turned into a pre-op since his supraglottoplasty was thwarted by a "massive airway infection."

I put "massive airway infection" in quotes for reasons that will become apparent in a minute.

In any case, it was a 2 hour drive to the office, an hour and a half wait time, and finally we were called back to see the doctor. Nolan was weighed and measured, and we waited.

The ENT came in and reviewed his records, and Nolan looked at Where's Waldo? I asked if his culture results had come in, and his ENT looked at his records. The culture results weren't there.

The nurse was sent in search of them, and she returned with the report.

The cultures were negative. Nolan never had a massive airway infection.

We are not sure what the large amounts of pus in his airways was from. It wasn't bacterial in nature.

The reason he never had any symptoms of infection (no fever, no cough, no congestion) was because he never had an infection to begin with.

So our poor little boy was put out for surgery, scoped, and then told he would have to go through this all over again when there wasn't any infection in his airway at all.

I asked the ENT what all the thick white pus was, if it wasn't an airway infection. She shrugged her shoulders and said, "Maybe it was just thick mucus. At least we know now."

Lovely.

In any case, he is remaining on antibiotics long-term, since we don't know what all the junk in his airway and lungs was. He's also staying off C-Pap for the foreseeable future. This means we will have a zombie-boy for the next few weeks, as his energy levels dip considerably when off C-Pap.

The repeated surgery attempt will be on April 9.

So, Been-There-Done-That parents... what in the world would show copious amounts of "pus" on a bronchoscopy that isn't caused by a bacterial infection?

In Which We Finally Get All the Medications

Nolan was prescribed Augmentin (a really common antibiotic) and Flonase compounded with Bactroban. Our ENT sent the prescriptions to Rite Aid, the pharmacy we've been using. They've been notoriously horrible at filling Nolan's prescriptions, and I keep forgetting to change pharmacies (he's only on Zantac now, so it hasn't been a pressing issue).

When we arrived home from the hospital, there was a message on our answering machine. Rite Aid called and said they don't have any Augmentin. They also couldn't compound the Flonase with Bactroban. They are extremely useless as a pharmacy. They called every Rite Aid store in town, and exactly none had Augmentin.

They finally found a store that had a short supply, so we traveled all the way down to the store on North Main Street to pick up the short supply. When we got there, we discovered we needed to have a label that had been printed out at the Rite Aid near our house to get the medicine. My husband asked when the antibiotic would be ready, and the pharmacy technician snottily retorted, "When will you have the label ready?"

Awake since 3:00 am, having gone through a long surgery day (that didn't turn into an actual surgery), we were not appreciative of the jerk behind the counter.

We returned to our main pharmacy and picked up the label. We returned to the Rite Aid across town. We got the miniscule amount of Augmentin and headed home. We were instructed to return to the pharmacy across town to get the rest of the medicine the following day.

Of course, when we returned, they didn't have the medication. They had sent it to our "regular" Rite Aid. We drove all the way across town and picked up the rest of Nolan's Augmentin. We also picked up the Flonase with Bactroban from Pharmacy Innovations, a wonderful little compounding pharmacy that was quite helpful and kind. Also, they had the medication we needed, which is always a plus when dealing with pharmacies.

In any case, Nolan is now on his Augmentin, Flonase/Bactroban (which he hates), and his regular Zantac.

He still has no signs of an airway infection. He does have a very rare, occasional cough. That is the only outward sign that has developed - nothing in proportion to the amount of pus in his airways. He also says that his right ear hurts (oh please, oh please, do not turn into an ear infection)!

Before he has another prescription filled, I need to switch pharmacies. I've been told that CVS and Wegman's are both fantastic. I am done with Rite Aid. It has been headache after headache with that store - and it would be nice to have a pharmacy that actually stocks common medications!

Revision Supraglottoplasty Attempt: Houston, We Have a Problem

Preparing for Nolan's surgery was complicated, but we were extremely blessed to have friends who offered to take care of Matthew while we were at Buffalo Children's. I booked a dog kennel stay for Casey. We had all of our ducks in a row, and were awake at 3:30 am for the trip to Buffalo.

We checked in at 6:00 am, and headed up to the 9th floor. Nolan was exhausted from the lack of sleep and was also feeling the effects of not having his overnight feed. He sat in a chair next to Dennis and didn't do much of anything until our name was called.

Our name was called and Nolan had his pre-op physical. His temperature, blood pressure and lungs sounded fine. He was weighed and measured, and we were sent to a different waiting room to wait for the same-day surgery nurse who would take his history. Nolan was very excited to find the new waiting room had a wii gaming console.

The same-day surgery nurse called me into another room to take Nolan's history. Since Nolan's more recent surgeries have been done in Rochester, those records weren't in their system. When she opened his file, she noted that none of his records were in his system. Apparently, Buffalo Children's didn't start putting records into a computer file until very, very recently. I went over as much of his history as I could remember, and the nurse had to leave to ask a fellow nurse how to spell "gastroparesis." She then took a look at Nolan and remarked on how healthy he was and how it was hard to believe he had so much medical history since he didn't look like he had growth problems and he looked so "normal."

I started thinking of her as Nurse Helpful. Nurse Helpful was quite convinced that there is nothing wrong with Nolan (he is of normal size and weight now, and has no growth issues due to the tube feeding).

In any case, the Amazing Health and Vitality of Nolan will come into play later in this saga.

We were taken downstairs to the 2nd floor, and our ENT and the anesthesiologist came in to see Nolan. Everyone listened to his lungs and heart and we discussed how he would be a direct admit to the PICU. He would be on a ventilator and intubated until Thursday. His records were taken to the OR and the nurse took him from our arms and walked him down the hall. He screamed and reached out for us the entire way. "MOMMY, MOMMY!!!!"

Having gone through this something like 12 times in the past, it never gets easier. Never.

The surgery should take about an hour, so Dennis and I went to get some coffee and something to eat. We feel a bit like seasoned pros at this, and know that the next meal may be a long time coming. We ate, and about 20 minutes later, we went back to the OR waiting room.

This is when we ran into Nolan's ENT.

"Oh, there you are," she said.

"Wow. That was really fast!" we said.

"It was fast because I can't do the surgery."

Houston, we have a problem.

She took us into a conference room and we learned that Nolan has a massive infection throughout his entire airway. I'm not talking about a minor upper respiratory infection. I'm talking about huge amounts of pus from his sinuses all the way down into his lungs. She showed us pictures and a video of his airway.

Remember Nurse Helpful? At this point I wanted to take those pictures and video and show them to her. How healthy does this look, lady?

He had no symptoms of a raging infection. None. He still doesn't have any symptoms of a severe infection. He has no fever, is playing, and is eating "normally" (normally for Nolan, anyway - certainly not normal for a typical child of his age).

Since they couldn't do the surgery, Nolan would be released and we will try again on April 9. I went to the recovery area to see my little boy, who had to go through the trauma of separation, general anesthesia, and IV placement... for nothing.

I am deeply grateful to his ENT doctor for taking the care to scope his airway before doing the surgery, because cutting into his airway and then intubating him with this sort of infection would cause significant danger to him. She is very cautious with airway surgeries in any case, and with Nolan's level of infection there is no way we could do any sort of surgery.

She cultured the infectious "debris" in his airway and we should have the results in a few days. Surgery has been rescheduled for April 9.

In the meantime, he has been placed on Augmentin for 21 days. His "post-op" appointment on March 18 will become his "pre-op" appointment. He'll be placed on another round of antibiotics at that time. Basically, Nolan will be on non-stop antibiotic therapy until his surgery date.

She also ordered a compound of Flonase with Bactroban - this is a nasal solution and we are supposed to rinse his sinuses with it to kill whatever bacteria is lurking there.

We left the hospital and went home. We played "run around the town to find a pharmacy with Augmentin," which is its own blog post. We picked up Matt and the dog from the dog kennel. The dog kennel owner was fantastic and didn't charge us for Casey's overnight stay (if anyone wants a recommendation for a good dog kennel in the Southern Tier/Jamestown area - give me a shout. Sadie Creek is excellent and the owner was amazing throughout our crazy surgery day). I booked Casey into the kennel for April 8, when we get to try this again.

Nolan is home from school today, and I am having extra cups of coffee. He still doesn't appear sick, despite the raging infection we know is in his airway. I'm interested to see what the culture shows.

ENT Appointment, Part 2: Revision Supraglottoplasty Scheduled

Nolan's g-tube and overnight feeds have done amazing things for him. He is now 41 pounds, 43" tall, and in the 37th percentile for weight. He no longer has growth failure, is stronger, healthier, and sturdier.

His C-Pap, however, offers variable performance for him. We sometimes hear stridor over the mask, which means the machine can't quite keep his oxygen consistently high. We reviewed the video from Nolan's sleep laryngoscopy, which shows the floppy tissue collapsing over his voice box.

Our ENT believes the first supraglottoplasty failed due to uncontrolled acid reflux. Now that Nolan has a fundoplication, the acid should be under control and a second supraglottoplasty has a higher chance of success. To maximize the chances for success, Nolan will be intubated and ventilated (while under sedation) in the Pediatric ICU for 48 hours.

Surgery has been scheduled for February 26th.

Next Tuesday.

There has been a whirlwind of activity since the appointment on Monday. We do not have local family, so we are extremely blessed to have amazing friends who will take care of Matthew while Nolan is in the hospital. I have called and booked a dog kennel for Casey. I have called the anesthesiology group to verify they participate with our insurance company (they do). I have taken care of the up-front deposit required for surgery, and moved a vet appointment and canceled Nolan's sleep neurologist appointment.

I am hopeful that Nolan will be released on Friday morning (March 1), but everything depends on how Nolan does after he is extubated and on room air.

If the surgery is successful, we can ditch the C-Pap. It would be really, really wonderful to ditch the C-Pap.

Nolan will have a follow-up (post-op) appointment on March 18, just 3 days before Matt's surgery.

Preliminary Sleep Study Results

The phone rang last week, and caller ID flashed "Home Healthcare Company" on the display screen. I figured the HHC was calling about insurance problems, or Nolan's next formula delivery. When I answered the phone, it was the other home healthcare company - the one that deals with Nolan's respiratory support.

We needed to increase his C-Pap pressure, so the respiratory therapist walked me through the process. I don't have Nolan's full report yet, but this is an indicator that his apnea is getting worse over time. Lovely.

Since I suspected Matt had sleep apnea, he had a sleep study, too. The pediatrician ordered this study, so I have access to the full report a little earlier than Nolan's. The pediatrician's office called me because he does have mild obstructive apnea, and he also has central sleep apnea. This sent them all a-twitter, but Nolan also has central apnea, so I'm not really phased by it.

For those who don't know what the difference between central and obstructive apnea is:

Obstructive Apnea: The child is trying to breathe, but something is blocking the airflow. This could be tonsils, adenoids, a floppy airway, or low muscle tone.

Central Apnea: The child's brain forgets to tell the child to breathe - no attempt is made to take a breath. This is usually neurological in nature.

In any case, Matt has both types of sleep apnea - just like his brother. He isn't nearly as severe as Nolan is with the obstructive, but they will probably want to take his tonsils and adenoids. The pediatrician is going to send me Matt's sleep study report in the mail, as I have more of a clue of what it means than they do.

Their initial inclination was to send Matt to a neurologist and a cardiologist for a full work-up. This would be fine, but if he is like his brother... there is nothing awry with his brain. I already have ENT appointments set up for both boys on February 18, so I will let the ENT sort it out and determine which tests to order. As our ENT explained, sometimes kids with longstanding obstructive apnea develop central apneas because the respiratory center of the brain "damaged" by the constant lack of oxygen during sleep.

I am really irritated that they didn't do CO2 monitoring at the sleep center we went to - it is likely that both boys will need follow-up studies, so I'll book those at Buffalo Children's to make sure we get a complete polysomnogram (PSG - sleep study) performed.

So, how did I suspect Matt had apnea? The signs he had were:

• Breathing through the mouth at night (and often during the day).
• Gasping in his sleep.
• Occasional snoring.
• Tired and weepy by the afternoon hours/frequent melt-downs.

Nolan has slightly different signs, and they are typical for malacia kids:

• Extending his head back to keep his floppy airway open.
• Loud stridor (sounds similar to snoring to the untrained ear).
• Audible stops in breathing.
• Grayish skin around his mouth (cyanosis) during sleep.

So now we have two kids with sleep apnea. I really, really need to start playing the lottery.

Sleep Study - Times Two

Our family camp-out at the sleep center is over, and I am glad it is behind us. We arrived at 8:30 pm and started the hook-up. Matthew was completely freaked out by the entire idea, so we hooked Nolan up first.

Sleep Studies - not much fun

Almost all hooked up.

Matt finally agreed to get hooked up, once he saw that the "stickers" really didn't hurt.

Watching TV during the hook-up.

We got Nolan settled into bed and let him play the tablet while Matt was getting hooked up.

Look, Mom, I'm a UNICORN!

This is the first time we've gone to one of the sleep center's satellite sites, and I wasn't impressed. They don't do CO2 monitoring and never notified us of that fact. This isn't a big deal for Matt, but Nolan has known severe apnea and should have his carbon dioxide levels monitored (particularly since they've increased over time). We'll see what our ENT says about this, in any case. They also had no pediatric pulse-oximeters, so nothing really fit Nolan's small fingers. I told the respiratory tech to put it on his toe (the respiratory tech has obviously not dealt with many pediatric patients and had never heard of this before). This solution worked, thank goodness.

Wired and Tired.

Nolan was quite uncomfortable in the night, and had his leg pains. These are not really "growing pains," as he occasionally gets them during the day and cannot walk. They also only affect one leg. I have to see if I recorded which leg was affected last time, but his right leg was killing him last night. He would arch out of the bed and become extremely tensed. I asked him if it hurt, and he would scream, "my leg, my leg!" He wanted me to pull on his leg, and when I did he wanted me to pull harder. I was afraid to hurt him, so eventually stopped.

I did catch it on video, as the pain would wake him from his sleep and he would cry out. I am going to show it to our ENT to see what she thinks. The pain isn't frequent, but is very severe and happens during the day as well as the night. I'm not sure if it is more of a cramp or a "pins and needles" sensation.



Fortunately he finally fell asleep, but woke frequently to pull at his C-Pap mask and at his leg. It was a really, really long night - I was relieved when the lights came on at 5:00 am and we could go home. We stopped by Tim Horton's for coffee and doughnuts. Both kids fell asleep the second we got home. The sleep study results should be available in 2 weeks.

Still Here!

We are still here, despite the lack of blogging activity lately. This time of year is always insanely busy, and our activities in real life have precluded my ability to keep up with this blog.

I have also reached the maximum photo limit for this blog. I'll have to start a new one if I want to upload any additional pictures. Nolan looks exceptionally cute lately - you'll just have to trust my written word on it! *Update - I broke down and paid for the extra storage. See? He's really cute.

As for updates, Nolan's eyes are doing really well. His amblyopia continues to improve and we are happy to have a six month reprieve from the pediatric ophthalmologist's office. I do need to get his new prescription filled, so we will do that next week.

We also had his follow-up appointment via the ENT. Things are going well with regard to his ears. He's been infection-free for a month, which is fantastic. We will have to discuss the possibility of another airway surgery in the spring - we have another appointment in February to make the decision. In the meantime, he's going to have another sleep study/C-Pap titration in January. Since Matt often snores, we're having a sleep study done for him at the same time. Killing two birds with one stone.

We are also going to get aided testing via a different audiologist, since our current audiologist simply refuses to do aided testing with hearing aids. It's beyond ridiculous, so hopefully this new audiology group will perform that testing on a regular basis. They can't do cochlear implants, so if we decide to go that route, we will need to find someone else. Our area isn't the best for medical or audiology care.

His stomach issues are still frustrating - he's doing really well with the overnight feeds, but we aren't seeing any progress with his daytime eating. We'll take it as it comes - as long as the overnight feeds are enough to maintain growth, we're golden. Our ENT did mention a gastric pacemaker as an option if his stomach function ever declined in the future.

We've been having a lot of issues with our insurance company and getting replacement Mic-Key buttons. Our insurance company only allows a new button to be issued every 91 days. Unfortunately, not every Mic-Key lasts a full 90 days. Nolan had a balloon burst on one in September. We replaced it and he had a good one from September - December, when we had to replace it due to clogging and sheer age (the balloon developed a slow leak). Our insurance company told us we couldn't get a new back-up until January. Fortunately the Home Healthcare Company sent one in our monthly shipment (there was a lot of anxious whining on my part to get them to send one). We're three hours from URMC and we don't really have a lot of options if it falls out and we don't have a back-up. Our insurance company will get to pay for surgery instead of a button if it happens.

I am REALLY glad the HHC sent us a backup button, because Matthew started screaming, "Nolan's button is GONE and his tummy is bloody!" Yep. Another Mic-Key failure. The balloon burst on this one, too. The same lot number as the one that burst in September. I am so frustrated. I was very glad to have the backup, and we managed to get it into his stoma (with a bit of a struggle - the stoma starts to close up very quickly).

I'm starting to wish we had gone with a Bard button. It has to be changed in the OR, but at least it lasts a year or so!

I have to start thinking about getting a new blog - if I do, I'll link to it from a new post. Pictures are sometimes necessary!

ENT Visit (Routine)

Waiting at the ENT

Nolan had an ENT visit today - just a routine follow-up. Normally a "routine" appointment would simply take a few minutes, but a lot has transpired in the past four months or so. We discussed the following:

The Nissen Fundoplication: everyone is in agreement that the surgery has been a success. "Success" does not mean that the reflux is entirely gone, but that the symptoms are mostly controlled. He has only had one ear infection and one sinus infection in four months - for Nolan, this is a major win. 

The G-Tube: due to continuing stridor and the use of C-Pap, the g-tube will remain in place for as long as Nolan has the need for mechanical ventilation at night.

The stridor: I showed our ENT a video of Nolan's stridor and she agreed that it is most likely due to the laryngomalacia. He's been through a lot lately, but she wants to do another scope in the fall or winter. She would like to try to cure the stridor surgically, but we aren't so sure about that route since past attempts have failed. 

Diarrhea: We aren't sure if the diarrhea is due to the fundoplication, the medication, or something else (read: intestinal issues or enteropathy). It comes and goes, and isn't as severe as it was a month or so ago. His ENT agreed to a trial wean from the Nexium (he must stay on the Zantac). This is rather convenient, as he has been off Nexium for about a week due to yet another insurance denial. If the diarrhea ceases, we'll know that is the cause. Since he's been off the Nexium for about a week, I doubt it is the medication (he had an episode last night). I also doubt it is due to the fundoplication, since it was present even before the surgery. We'll take it as it comes.

Weight: Despite some stellar days with eating, Nolan's weight hovers at 33 pounds. He's between the 5-10% for weight. The non-stellar days with eating keep him from gaining well. This is another watch-and-wait thing. He's still on the charts - little, but still on the charts. Yet another reason for the g-tube to stay put - if we remove it, we could very well be having one put in again in a few months as he falls off the charts (unless he starts to gain).

Falling episodes: It is hard to do formal vestibular testing on a young child, but the consensus is that Nolan's falls are vestibular in nature. There isn't much to do about this particular situation, since the attacks are random in nature.

Gross motor skills: Nolan is on "alert" with gross and fine motor skills through the school, since he has some muscle weakness. He can run just fine, but his throwing skills, jumping, and balance are not age-appropriate (as measured by the DIAL preschool scale). Not really the ENT's domain, but we discussed it. He'll probably end up with physical therapy or occupational therapy sometime during kindergarten. Another thing we'll take as it comes.

Fatigue: we aren't sure if this is due to stomach pain/dysfunction or if he has true fatigue. Watching and waiting on this one.

Progressive hearing loss: I brought up Nolan's new hearing level. This is one time I wish he did have fluid or a sinus infection to explain the additional loss. Sadly, this is simply a drop in his sensorineural hearing level. There is nothing to do for it but watch, wait, and increase amplification. I asked our ENT if most kids with progressive losses just level off at some point. She smiled and shook her head sadly, but then said, "Well, sometimes. We have no way to tell what is going to happen." Considering that Nolan started life with a normal hearing level and present OAE's in the high frequencies and now has a 70dB loss in that ear, things aren't looking promising. It could take a long time for his other ear to drop or for either ear to progress beyond the help of hearing aids, but we are probably going to see his hearing slowly slip away.

We'll return to the ENT on October 15 for another routine appointment. An endoscopy will probably be ordered to look at his esophagus and larynx at that time. We'll also be able to discuss his hearing loss, since I should have the audiograms from the July 6 test and the October 8 test by that time.

Random Update

Nolan is doing pretty fabulously this month. No signs of reflux, which is fantastic, and he's been pretty happy. We aren't using the g-tube to vent quite as frequently, which means his stomach is finally settling into a more normal "routine" following the fundoplication.

He is still struggling with weight gain. He's down to 32 pounds, which is hitting the 5% mark on the growth charts. His eating is hit-or-miss, and he's been struggling with chronic diarrhea. I'm not sure what is causing the diarrhea, but I will have to talk to his pediatrician about it, because it certainly isn't helping on the weight front. He still has stridor, of course - we can hear it when he doesn't use the C-Pap at night (he was sick last week and didn't use it for a few nights):



 Despite the stridor and weight gain issues, however, he is fairly healthy. He definitely has a rosy glow to his cheeks and the fundoplication has been helpful to him. He doesn't choke as often and his little nose is clear - and he has been free of ear infections since the surgery.

I filled out his kindergarten registration packet on Thursday and sent it back to his school. Nolan is behind on his immunizations because he had the flu shot 2 weeks before his four year old well-child visit. They didn't want to interrupt the immunity forming from the flu shot, so his four-year-old jabs were put on hold. Then he had the surgery, got a few nasty viruses, and we were on vacation. I called the pediatrician last week to get him in for the shots, since he'll be five in a couple of months and he'll need a whole new round of immunizations.

We're off to enjoy a fabulously warm Memorial Day weekend - we take the sun when we can get it in these parts!

It's pool time!

Swinging

The past couple of weeks have been really good for Nolan. His overall strength has always been on the low side, and he was still grabbing his crayons in an odd fist-grip as of two weeks ago. Within the past week, though, we've seen a few major gains in his coordination and strength. He was able to hold his crayon correctly the other day and maintain the grip for a few minutes. On Saturday, he pumped his legs and powered his own swing for the very first time.

With Matt, we took these little milestones for granted. With Nolan, however, each and every milestone is greeted with elation - and a sigh of relief. While he scored a ZERO on his motor skills evaluation during the first half of the year, he is gaining strength and catching up to his peers. I think his body simply couldn't gain strength when he was so ill with reflux all the time.

The little guy had his first tee ball game on Saturday. Matthew had to miss the game due to a conflicting piano lesson, so Nolan went with Daddy to the game. I was really happy that Dennis took a few pictures - there is nothing cuter than a little boy in a baseball uniform.

While the surgery stopped the reflux (at least, we think it stopped the reflux), the little guy still has stridor. He is also developing a disgusting, clogged up nose. It makes his stridor sound rather "wet."



Thankfully, his C-Pap does work to keep the resulting apnea in check. He wasn't using it on this particular night because the water had gone "off" rather quickly in the heat, and we got home from Buffalo too late to wash and dry the tubing. It was interesting, because his stridor alternated between sounding like a wet snore (in the video above) and the regular, higher-pitched inspiratory stridor that we normally hear. He'll probably have the stridor for a rather long time - the noise itself isn't dangerous, but the cessation in breathing can cause growth and developmental problems over the long term. I have a feeling the C-Pap machine won't be going anywhere anytime soon.

 I may not write as much over the next week or so - the little guy's transition from preschool special education to general (school-aged) special education is coming up and we have a meeting to prepare for!

Vacationing with Nolan

Nolan retched again last night, three separate times. I'm not sure what caused the retching events, because he had been fine all day Saturday. He also woke up with no difficulty and asked for a cheese sandwich for breakfast. Go figure. He seems relatively back to normal, whatever that is.

I am working on getting our vacation pictures posted on the other blog, if you want to read about our trip to Myrtle Beach. I will, however, write about the "medical" aspect of the vacation on this blog. We've always tried to keep the medical issues separate from the boy, which is why I maintain a regular "family" blog and this one, which helps me organize the various appointments and medical randomness that is Nolan. The vacation was absolutely wonderful, though Nolan had some issues with fatigue on the trip. He simply doesn't walk very far before he breaks down and has to be carried. Sometimes this involves a mammoth tantrum, other times he just sits down. We didn't bring the stroller on this trip, which was a mistake we won't repeat.

Trying to get Nolan to walk. Heading into collapse mode.

We also became more comfortable with venting Nolan in public. He asked to be vented daily, and he can be rather insistent when his tummy is bloated. We vented him in the parking lot of a McDonald's, in an Olive Garden, and at Margaritaville. At Margaritaville, Nolan shouted, "VENT ME, MOMMY!" and then went under the table to dig out the extension set from my purse.

Under the table at Margaritaville.

He required frequent venting on vacation. I'm not sure if it was because of all the swimming (gulping air), the food, or just a random coincidence, but the little guy tended to get rather bloated.

 Bloated tummy.

Venting air.

I am very glad we went on a beach vacation rather than a more taxing location. He did wonderfully at the beach and the pool, where he could just huddle up with a towel and take a break when needed.

Taking an energy break on the beach.

We learned several things on this vacation:

1) Having a condo was a necessity. We needed the kitchen, the fridge, the ability to store food for Nolan (so that he could eat on his own schedule) and the laundry facilities. I am so grateful to my brother and sister-in-law for giving us the condo unit for Christmas. Best. Gift. Ever.

2) Traveling by car made things easier. Nolan's equipment now takes up a suitcase of its own: the C-Pap machine, the hearing aid gear (including Dry and Store), the extra g-tube button and supplies, etc. Being able to stop whenever we needed to made the trip easier. We could eat, vent Nolan's tube, or do any other necessary action on our own timetable.

3) Nolan really fatigues. We don't notice it as much around the house or even when he's at school, but he just doesn't have the energy to consistently be on the "go." He requires a lot of rests, and a stroller would have come in handy on more than one day of this trip. We never walked far, but any time we went to a shopping center or walked for more than five minutes, he had to be carried. We do notice this in our local mall, but it was more evident on vacation.

Hopefully the little guy will get stronger over time, but the fatigue issues have been with him for a rather long time. He will learn how to cope with his fatigue better as he gets older, at least - I can hope that the raging tantrums will reduce as he gains a few more coping skills!

Sleep Neurologist Follow Up

Feeling puny, but lollipops are always good.

Nolan hasn't been feeling so wonderful lately - not really "sick," but running a low grade temperature and he has had a headache all day today. Thank goodness for children's tylenol!

He had a routine sleep neurologist appointment in Fredonia, so we made the little jaunt to the doctor's office, paid our $40 copay, and were told to continue C-Pap therapy and we'll see them again in six months.

The sleep neurologist also told us that we must take his C-Pap machine to the hospital with us to ensure there are no problems with the machine and the fundoplication (since the swallowed air might become trapped and create issues). This is one of the big reasons Nolan is having a Mic-Key button placed at the time of surgery - any trapped air can be vented if it becomes a problem.

Nolan did get weighed (34 pounds) and measured (41 inches) before we left. His next appointment will be in late August, just after his fifth birthday.

I was glad to hit the road and head toward home, particularly since a nice little snowstorm cropped up on Route 60.

Relieved to be home and out of the snow.

With the funky weather and the viruses running rampant through the schools, I may keep Nolan home for the second half of next week, just to keep him from getting ill before surgery.

Thank goodness Spring is only a month away!

Hilarity

Oh my goodness, this kid is going to kill me with laughter. He insisted on wearing goggles to bed last night.

He's awake in that photo, just staring at me. He said, "That is good." Then he waved me out of the room.

Whatever he lacks in physical health, he sure gained in personality!

Great Christmas Vacation

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!

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!

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!

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.

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!