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.

Still Here - Playing Catch-Up and Matt's Supraglottoplasty

I have been absent from the bloggy world. I have a good excuse. We had four - count them, four - surgeries this past spring. Two for Matthew and two for Nolan.

First was Nolan's attempted supraglottoplasty in February, which couldn't be completed because he had funny, white exudate all throughout his airway. He had to be woken up from general anesthesia and we were sent home. The cultures were normal, so this mystery was never explained.

Second was Matt's tonsillectomy and adenoidectomy in March. An outpatient surgery with 8 long days of recovery.

Third was Nolan's supraglottoplasty and 3 day stay in the PICU in April. He was intubated on a ventilator for 2 days.

Fourth was Matthew's supraglottoplasty and overnight stay in the PICU for observation on June 25, 2013.

Honestly, I plead exhaustion. It was simply a ridiculously exhausting spring.

Matthew's surgery went very well. Nolan's genetic syndrome (Ehlers Danlos) wreaks havoc on his tiny body, and it is much more evident when comparing the boys' surgery recoveries. Nolan was in a lot of pain and had a lot of trouble swallowing and required ventilation assistance after surgery. Matthew cried for an hour after surgery, then demanded French toast and a cheeseburger.

Unfortunately, he was only allowed clear liquids for the rest of the day. The morning, however, brought French toast and an ecstatic Matt. "Mommy, this is the goodest breakfast ever!"

Matt's recovery was ridiculously easy. He didn't have to take any pain medication after the first day in the hospital. We went home 24 hours after the operation. 48 hours after his surgery, he was playing in his baseball team's championship game. They got second place.

Seriously. This is Matthew 48 hours after surgery. His recovery was insanely easy. It was nice to have things go so smoothly!

We have a sleep study on August 9, 2013 (this coming Friday) to determine if he still has sleep apnea. I certainly hope his apnea is gone!

Doing Better!

New prescription sunglasses!

Nolan is almost completely recovered from his supraglottoplasty. He isn't on pain medication anymore, and we have stopped his steroids. I have to say, I am glad to be down to two medications again!

He is still choking on thin liquids, but that problem should resolve with time. He is able to eat most foods again, and we experienced one of the "good" side effects of steroids - they make people hungry. While Nolan still can't tolerate large volumes of food, he was eating constantly when on the steroid. I was very excited and thought that perhaps we could keep him on steroids to maintain the hunger - and then found out that steroids cause things like diabetes and liver issues and can't be continued at high doses for a long period of time. Still, it was great to see him hungry for the first time in...forever.

The little guy has been outside to play a bit, but does tire out easily. He rode his Green Machine for a few minutes yesterday, but couldn't pedal it on our driveway and started to scoot it around with his feet before heading inside for a rest. We also went to Matthew's first baseball practice last night, and Nolan did play on the playground for a few minutes.

It was nice to see an actual smile! He was completely wiped out by the time we got home, so he didn't eat dinner and had some serious bloating issues. He had trouble tolerating his feed last night, but seems fine again this morning. Even though he seems largely recovered, he doesn't quite have the stamina to do his normal activities.

Still, he will be ready to go back to school on Monday. We are all looking forward to the return of routine!

Revision Supraglottoplasty: Finally Home

Nolan's surgery went ahead as planned last Tuesday - this was a relief since the last one was cancelled due to strange "exudate" in his airways. Of course, the cancelled surgery still cost us more than $500 - just another bill to add to the growing pile!

In any case, we checked into Buffalo Children's at 6:00 am on April 9. Nolan was a bit wiped out, but was happy to play the wii in the pre-surgery waiting area.

In the middle of his game, he suddenly sat down and looked quite ill. He said he needed to throw up. We told the nurse and they brought us to a room with a gurney, and called his ENT.

Nolan's stomach is an up-and-down thing.. we've always wondered about hypoglycemia since he vomits more frequently on an empty stomach. They brought us down to the second floor pre-op area, and we waited in the chairs. As Nolan puked his guts up, the anesthesiologist visited with us. The nurse also came and took some blood for a blood glucose reading. His blood sugar was 60, which is at the lower limit of normal for a child. He doesn't have hypoglycemia, so the vomiting is due to some other factor.

In any case, the anesthesiologist saw Nolan's state and told me that I could come into the operating room to be with him while they put him to sleep. I have no idea why they didn't do this for the previous thirteen procedures, but boy howdy - it was so much easier. I walked him back into the OR and held his hand as he drifted off - there was no crying and no hysterics.

After 2 hours, we talked to the ENT for a post-op report. His epiglottis (flap that comes down when you swallow to protect your airway) was flopping over his voice box again, so they tacked that back up. This procedure (tacking the epiglottis to the base of the tongue) is called an epiglottopexy. She also lasered out the floppy tissue (called aryepiglottic folds) that flopped over his voice box. In addition to lasering, she sutured the area to (hopefully) prevent the tissue from collapsing again.

She also told us he has a condition called glossoptosis (gloss-op-toe-sis), which is when the jaw is too small to accommodate the tongue. His tongue falls to the back of his mouth and also obstructs his breathing. If this condition worsens over time, he may need a procedure to fix the problem. Hopefully he won't require help with this issue.

The PICU charge nurse came to retrieve us from the waiting room and brought us to Nolan's bedside. He was heavily sedated and on a ventilator.

Occasionally, he would wake and try to rip the tube from his throat. He was quite wakeful despite heavy sedation - he ended up requiring Fentanyl, Ativan, Versed, and Precedex to maintain a restful state. He did have a fever for the first two days in the PICU, despite the Tylenol they were giving him.

It was a long two days, though at least Nolan was resting comfortably by the second day. His PICU nurse was with him for three days and it was wonderful - his nurse was really amazing and took great care of our little boy. Finally, Thursday arrived and we were able to extubate him.

It took quite some time for the Versed and Remi-Fentanyl to wear off, and our first attempt to take the tube out failed (his O2 sats kept dropping so they left it in for a while). Finally, he woke up enough for the PICU staff to remove the tube. Parents who have gone through this know how heartbreaking it is. Nolan was awake and thrashing, terrified and not really understanding why he couldn't speak. Tears poured silently down his face... the doctors needed Nolan to respond to commands before they could pull the tube. Nolan couldn't do this. There were just silent tears and terror. Finally he mouthed the words, "TAKE THE TUBE OUT." His silent "scream" let the team know he was conscious enough to breathe on his own. They took the tube out.

Once the tube was out, he immediately went back to sleep. His oxygen saturation was great, and we would monitor him for another 24 hours in the PICU. If he could stay awake, eat, and drink, we would be discharged the following day.

Nolan slept all day and did not eat or drink anything. I did not want to stay over the weekend in the hospital. The medical staff wanted to be sure he could drink without choking before sending us home. Fortunately, we talked with the residents and Nolan's ENT - since Nolan has a g-tube, they would allow us to go home despite the lack of eating or drinking.

The following night was really rough. Nolan was very wakeful, but his voice hadn't come back. He would sit up and mouth words, trying to communicate. He couldn't swallow his own saliva, and needed to spit into a towel frequently. Since he was only there for monitoring, his PICU nurse was moved to another patient across the ward and I was the one "on duty" throughout the night.

Friday morning, Nolan woke and was in a great deal of pain - his legs were really bothering him. He gets a strange leg pain every once in a while - not exactly cramps, but he will yell at us to crush his toes, pull on his leg, and pull his feet off. I have no idea what causes this, but I was glad that other medical personnel saw this! Nolan felt very poorly, and we were discharged at 11:00 to go home. I was beyond tired, and grateful to get home.

The leg pain continued for the rest of that day, and was actually more difficult than anything else. He writhed on the floor yelling, "PULL MY TOES OFF!!!" There was really nothing I could do to help him - fortunately the episode passed after a few hours and he was fine later that evening.

Nolan can't swallow liquids well, so he coughs and splutters frequently with drinks. We are putting liquids through his tube. Fortunately, he can eat some foods. Cheese puffs, pancakes, and other dissolvable foods work well. He tried a bite of pizza, but ended up spitting it out. He can't do any "hard" foods like apples or chewy bread. He's still spitting out his saliva - but this should get better over the next few days.

His low-grade fever has made a bit of a comeback, but each day is better than the last. We have even seen a smile or two over the past day or so. I'm not sure when he can return to school. He is far too ill at the moment to do anything, and the choking problem has to be resolved before he can return safely to his classroom. It will probably be another couple of weeks before he's well enough to return to regular activities.

A small smile.

Ready for Surgery Tomorrow

Our second attempt at Nolan's revision supraglottoplasty is tomorrow. His ear infection seems to be under control (still on the Ciprodex and antibiotics), though his stomach has been acting up. Lots of fluke vomiting attacks and hurting tummy moments - he's had a day or two of "good eating," but is otherwise not doing well on that front. Hopefully his stomach will be in good spirits tomorrow morning.

We have the dog at the kennel, Matthew is with our friends for a couple of days, and we're heading to bed so we can be up at 3:30 am tomorrow. Hopefully his airway will be clear and the surgery will proceed without a hitch.

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.

Three PE Tubes and a Floppy Airway

We are home, thank goodness. Even though the placement of tubes is always a quick procedure, the day is still long. Nolan, of course, had to throw a few curve balls into the mix to create a slightly longer surgery than anticipated.

Firstly, he couldn't receive his overnight feed before surgery. He was as floppy as a rag-doll before surgery.

Then he started to retch and vomited some bile in the pre-op area. The child doesn't do well when he has to go for long stretches without food. Fortunately, they took him back to the OR at around 11:00am. An hour later, the ENT came to talk to me.

His right ear was infected and when the new tube was placed, the ENT could see that the fluid was still behind the eardrum. She made a second incision to try to suction the infected fluid out of the middle ear space, but the area behind his eardrum isn't communicating - for some reason, there are two separate sections of retained fluid. To solve the problem, she placed a second tube into his right ear. Now both sections are able to drain, and hopefully we'll achieve a dry ear. She also replaced the tube in his left ear. He is now sporting three tympanostomy tubes - I'm not sure how common that is, but at least his right ear will be able to heal from the chronic infection.

The sleep laryngoscopy showed severe laryngomalacia. His epiglottis was tacked up in the last operation, and it still looks good. The epiglottopexy worked and it no longer flops over his voice box/airway. The arytenoid folds, however, are large and floppy again, and completely occlude his airway. I got to watch a video, and they look just as bad as they did before his first supraglottoplasty.

We return to the ENT on November 26 to discuss our options. He will not outgrow the laryngomalacia, and we're not even sure if another surgery would work. The ENT has only had one other child who relapsed after a supraglottoplasty in the history of her career. There isn't a lot of history to predict what will happen if we attempt another surgery for Nolan's airway. Besides surgery, the other option is to keep him on mechanical ventilation at night for the rest of his life.

The ENT also wants to send us to another ENT, who is familiar with cochlear implants. With Nolan's right ear sitting at 75dB, she wants us to become familiar with the technology and at least say "hello" to the surgeon. His left ear is still well out of candidacy range, but tends to follow the right ear over the course of time. It is currently sitting at 60dB rising to 50dB, but if it drops then he will have two ears in the severe range.

We have a lot to research and consider before his follow-up appointment. In the meantime, I am going to take a nap. Or drink some coffee.

Sigh....

C-Pap for Kids: Not Much Fun

Mr. Bear tries out the C-Pap

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

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

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

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

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

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

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

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

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

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

Busy, Busy, Busy

This week has been fairly busy. Not in a "lots of crazy medical appointments" sort of way, but in the normal preschool-program-playground-visiting loveliness that comes with May.

Nolan did break the nose pad on his glasses, so we took a trip to Walmart to get the nose pad replaced. His glasses have a saddle-nose bridge piece, which does help the glasses to stay up on his face.


Unfortunately, the glasses still sit pretty low and he spends most of his day looking over the top of the glasses. With amblyopia, this is a very bad thing: he needs to look through the lenses to get his brain to recognize the presence of the weak eye. We return to the ophthalmologist in June, and I have a suspicion Nolan will be going Pirate-style with an eye patch this summer.


Matt has decided to give us a run for our money, too. He is, overall, very healthy. Unfortunately, he has never been dry during the day. Now, at five-and-a-half, this is becoming an issue. We go through an incredible amount of laundry, and obviously we don't want him to start kindergarten with an embarrassing continence issue. We see the pediatrician next week to address this: the first course of action is to try a drug called DDAVP to see if that helps him become dry during the day. We may also have to do some tests, since Nolan has a urology problem that can be seen in brothers (the posterior urethral valves). It is highly unlikely that Matt has the issue, since it only occurs in 1/8000 male births, but the consequences can be devastating if it isn't corrected. I'm really hoping the DDAVP is all he needs, because then all we need to do is "watch and wait" for his bladder to mature.

I'm also still waiting on Nolan's sleep study results. They are probably already in at the Pediatrician's office, but I will wait until next week's appointment to pick them up. In the meantime, we're just praying that the apnea is gone.

Approved

Growing: Nolan is now 38" tall!

After our insurance sent the denial letter for Nolan's Nexium, our ENT's office sent another request to have the medication approved. While there is a possibility that the Prilosec will work adequately to control the reflux, the effects of it not working would be disastrous.

We received the approval letter in the mail today.

If the Prilosec worked, life would have been OK. If it failed, we would have been faced with more airway surgery and more complications down the road. In addition, the Prilosec package insert says that the 20mg pills does not have the same bioavailability when emptied into applesauce (a requirement for children too young to swallow pills): "PRILOSEC Delayed-Release Capsule 20 mg was not bioequivalent when administered with and without applesauce. When administered with applesauce, a mean 25% reduction in Cmax was observed without a significant change in AUC for PRILOSEC Delayed-Release Capsule 20 mg."

The Nexium comes in a 20mg oral suspension, which does not affect the bioavailability of the drug at that strength.

I am greatly relieved that the insurance company approved the Nexium. Nolan's acid reflux affects his airway, which makes monkeying with the effectiveness of his medication quite scary.

We can take a deep sigh of relief and know that his medication approval is secure for the time being.

Denied (Again)

Nolan's ENT increased his Nexium dosage a month ago, because his weight has been increasing and he requires a higher dose to maintain effective suppression of his severe GERD. Nolan was on 10mg (twice daily) Nexium, which was originally approved through December 2011. Of course, the increase in dosage gave the insurance company yet another opportunity to deny drug coverage - we got the letter in the mail today denying Nolan's medication.

This means, of course, that Nolan is currently not on ANY proton pump inhibitor (PPI) medication. For a child with reflux so severe that it causes failure to thrive and his airway to collapse, this is probably not a good idea. Of course, the insurance company must know more about Nolan's condition than his various specialists (that sentence needs a sarcasm font).

As a recap:

1. Nolan was diagnosed with off-the-chart severe acid reflux. He was failure to thrive, had dysphagia, and was prescribed Nexium at a 10mg, once per day dosage.
2. Some improvement was noted, but Nolan began having trouble eating again. Obstructive apnea and central apnea was noted during a sleep study. The Nexium was increased to 10mg, twice daily.
   
3. Nolan developed laryngomalacia and severe obstructive sleep apnea. A follow-up pharyngeal pH probe demonstrated his 10mg Nexium was insufficient to control the reflux. 30mg of Zantac (2x per day) was added to his medication regiment. A follow-up pH probe demonstrated that his reflux was barely controlled (he still had at least one break-through reflux episode on the Nexium and Zantac).
4. A supraglottoplasty was performed to correct the severe laryngomalacia (caused by GERD). At Nolan's follow-up appointment to the surgery, his ENT increased his Nexium dosage to 20mg (twice daily) to completely suppress the acid reflux.
   

Our insurance company denied the claim, and he is not on his Nexium at the present time. This means that his pharynx is currently being assaulted by acid reflux, greatly increasing the likelihood of a recurrence of his laryngomalacia and obstructive apnea.

The Zantac is not sufficient to control his reflux (it is an H2 blocker, a "lesser" drug than the PPI's). The insurance company states that we have not:

1. Tried an H2 blocker with his Nexium.
2. Tried Nexium once daily.
3. Tried Prilosec (omeprazole, 20mg) twice daily.

Numbers 1 and 2 are clearly wrong. Nolan cannot take Prilosec because it comes in a pill form. He is three years old. Also, Nexium is very slightly stronger than Prilosec, and even the Nexium can't control his reflux without an additional H2 blocker.

And so, my little boy will continue to suffer from severe reflux, and cannot get the medication prescribed to him by his physician, because an insurance company determined it is too expensive.

The odd thing is, the insurance company is going to end up paying more when Nolan ends up in the PICU again with a revision supraglottoplasty.

Nolan has a sleep study on May 3, and is not on his medication. I don't expect the results to be pretty.

Recovered

At first, I thought the supraglottoplasty/lingual tonsillectomy was going to be easier than the regular tonsillectomy/adenoidectomy surgery. Nolan wasn't as swollen, and the pain wasn't quite as acute in the early days.

What I didn't count on was the pain increasing on day 8 and then lingering for A Very Long Time. I think we finally managed to ditch the Tylenol with Codeine on Day 15. Even now, almost three weeks from the surgery date, Nolan occasionally will hold saliva in his mouth and complain, "My throat hurts!"

Those moments are rare, though, and he is generally recovered. He was supposed to take his antibiotic until Tuesday, but extreme stomach pain and diarrhea made us discontinue it a few days early. His doctor is out of the office over the holiday, so I'll have to let them know about the problems with the antibiotic on Monday. I really hate stopping antibiotics early, but bleeding diaper rash and severe diarrhea are enough to make me stop giving him the medication.

We've been busy over Christmas break- playing in the snow, visiting a new Children's Museum in our city, and generally enjoying the slower pace of life. My Kindle (Christmas present) arrived just before New Years Day, and I have been sucked into reading the many free e-books available online (as well as a few purchased books).

Tomorrow, we get back to the rat race that is therapy, preschool, and ice hockey. Though Nolan is quite happy to get back to School on Tuesday, I am sad that our little break is over!

Mr. Cranky

Christmas was a great deal of fun, though Mr. Cranky is having a hard time coping with the festivities. He's still having some pain and difficulty swallowing his saliva at times. He spent much of Christmas in a bit of a funk.

On the other hand, he was breathing silently last night, and slept until 9:30am today. He has developed a hoarse voice, so it is possible he is developing another upper respiratory infection. He won't take his medication voluntarily, and we've had more than one incident of Nexium and/or antibiotic being spat out on the carpet. I've weaned the Tylenol with Codeine down to about 1 dose per day, and he seems to be handling that fairly well- I think the crankiness is due to a general "icky" feeling rather than acute pain.

We are keeping things quiet and staying at home, letting him recover his energy and hopefully get past this bout of crankiness. Hopefully he will be 100% recovered before returning to school on January 4.

I, for one, will be very grateful when 2011 arrives!

Christmas Eve

Nolan seems to be improving a bit on the pain front, which is very good news. He is still on the Tylenol with Codeine, but only takes it twice per day. We can tell when he needs a dose, because he stops swallowing and the drool starts pouring out of his mouth. He also cries and lets us know in a very loud voice, "MY THROAT HURTS!" Those communication skills we have worked on for three long years are coming in very handy lately.

We had friends over tonight, and he played outside in the snow for a while before fatigue set in (Nolan is not a fan of the great outdoors in general and wants to be carried- not fun in knee-deep snow).

The boys resisted bedtime, but Matt finally gave up the fight when he realized Santa would not come until all small children were fast asleep. Nolan, however, continues to fight the sleep fight. Playing Santa with an insomniac three year old in the house takes incredible ninja skills.

Somehow Mommy Santa managed to get the presents under the tree without Nolan noticing from his bedroom upstairs. His being hard of hearing works to my advantage sometimes- I'll take the benefits where I can get them.

I really can't wait to see the boys' expressions tomorrow morning. Three and Five are full of magic- and this year is truly magical!

Merry Christmas!

(To my friends who celebrated Hanukkah this year, I hope you had a joyous time as well).

Dear Codeine, I Love You

Nolan talking to, but not eating, a snowman cupcake

The ENT really wanted Nolan to discontinue the Tylenol with Codeine. I stopped the medication yesterday, and Nolan was rather cranky.

This morning, he woke up crying. Then screaming, whining, and general mayhem ensued. He wouldn't eat breakfast, he wouldn't eat lunch. He also wouldn't drink anything, or even swallow his own saliva. By noon, his mouth was held open and drool was pouring out.

I put him back on the Tylenol with Codeine. Regular Tylenol isn't cutting it, and the child is in so much pain he can't swallow. Within an hour of the medication, he was eating, chatting, and smiling again.

I'm going to give the medication another few days before trying to wean him off the pain medication again- the poor little guy needs a break!

Post-Op Appointment

Nolan had his post-op appointment for the supraglottoplasty today. He has discontinued the oral steroid and the pain medication, and will continue the antibiotic and his reflux medications. The weaning from the pain medication has left him rather cranky, but he seems to be fine most of the time.

I wasn't expecting the impromptu nasal endoscopy (a scope to verify his recovery is going well). This required more cotton with lidocaine up the nose, the papoose board, and a flexible scope going up his nose and down his throat. With the number of times the little guy has been held down for similar procedures, he's going to develop a complex about things going up his nose!

The endoscopy showed lots of scabs where the laser burned tissue. This is expected, but it made me sad to see all the burn marks. The poor little guy has to be in so much pain when he swallows! The scope also showed that the laryngomalacia is completely gone- his epiglottis is no longer flopping on top of his voice box when he tries to breathe.

His ENT gave him the all-clear to resume all activities, and wanted to see him back in another 3-4 weeks for a repeat nasal endoscopy. The office staff made his next appointment for March 28, however, so I have a feeling I might have misheard the doctor. He also has a follow-up sleep study scheduled for May, and we will verify that the obstructive sleep apnea has disappeared.

The important thing is to keep on top of his acid reflux (the extra-esophageal or LPR reflux, specifically), so the laryngomalacia and other airway difficulties do not recur. For now, the Zantac and Nexium are working to prevent further damage- I suspect there will be more pH probes in the little guy's future.

We did give Dr. B a little present- to thank her for figuring out much of Nolan's medical craziness. We painted a pot with Nolan's handprints and I wrote, "Thank you for helping me grow" on the rim. It seems a silly gift, but how do you thank someone for saving your kid from future heart failure? I suppose a plant will have to do- words can't express how grateful we truly are.

Our "thank you" present.

Despite being given the all-clear to resume all activities, I am keeping Nolan home from preschool tomorrow.* For one thing, it seems a bit absurd to send him to school for one day before Christmas vacation begins. In addition, I don't want to expose him to unnecessary germs while he's still recovering (he has already developed yet another upper respiratory infection, without exposure to preschool).

The last reason is that the Week of Craziness has officially begun. Today, we made the plant gift for the ENT, ran up to Buffalo and Back, Matt went to hockey, and I made a bazillion snowmen cupcakes.

An army of sugary goodness.

Tomorrow morning, Matt has his birthday celebration at his pre-K class. We will probably scarf down some lunch in the stairwell, then run over to his "X-Cel" and Creative Arts class Christmas program- then I will make a horde of Christmas cookies for his Pre-K cookie exchange. Wednesday involves his Pre-K Christmas party and cookie exchange. By Thursday, we are going to be happy to have a break from the programs/activities/parties.

I hope everyone has a Merry Christmas (or had a Happy Hanukkah or other celebration)!

*Our school district doesn't go on winter break until December 23, so we have a busy week ahead!