Getting Authorization

Oh my goodness: it is finally WARM outside!

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

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

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

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

Mr. Personality

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

"Mom, I need to frost my teeth."

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

"Mom, I want bourbon."

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

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

Oh. Bergman. The local playground. Of course!

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

We Have the Best School District, Ever

Nolan wields The Force at his IEP meeting

Some people live in school districts where it is an absolute nightmare to obtain services. Our school district is the opposite of that scenario - the special education department is an absolute joy to work with.

Nolan's IEP meeting went smoothly, and we obtained some push-in services from his SLP. We also obtained direct services from a teacher-of-the-deaf (TOD) once per week. For the first time, we are adding some real sign language goals to his IEP. Swimming lessons have proved that Nolan cannot hear in the water, which presents a very real communication issue. He is in that odd in-between hearing level state: he can hear (sans aids) in a quiet environment for a distance of about 5 feet. In noise, he hears almost nothing. Since we can't waterproof his hearing aids, we need an alternate communication method - so we have put in a goal for understanding and producing 75 "survival" signs.

Obviously, Nolan continues to be very verbal, and he has an auditory learning style. His remaining goals are for using the FM system in the classroom, filling in those odd language gaps (which have been noted by his SLP and TOD), and eliminating some articulation errors.

Since Nolan has a few other health issues, those are also mentioned on the IEP. He won't be eating in the cafeteria in pre-K, since it is a half-day program. Nonetheless, they included the dietary restrictions into his IEP. His medical information is also included, since he fatigues easily. Nolan looks very healthy, but he will completely slump to the ground and have to be carried at times - particularly in the afternoon. This is just something we are monitoring - it could be related to his apneas at night, or it could be something else altogether. He did have one of his fatigue-slumps during the meeting - he slid off his chair and his tears began. He ended up on Dennis's lap, and a cookie made him feel better.

We had almost finished the meeting, when the Special Education Coordinator watched Nolan holding a crayon. We know he has some low muscle tone, particularly in his hands. This hasn't caused any delays yet, but she was concerned with his grip (fisting the crayon). He passed an OT evaluation last year, but the team at the meeting wasn't really convinced - so they wrote monitoring for fine motor delays into the IEP.

We got what we asked for (hearing and language services), and some things we didn't think to ask for (fine motor monitoring/evaluation). The school district team really, really cares about their children - this is probably why we have the highest elementary school test scores in the region. The staff goes out of their way to ensure that every child has the best opportunity to learn and succeed.

I am so excited for Nolan to start Pre-K next year - we know he is going to excel in our amazing school district!

What is a Fundo-Majigger, Anyway?

The surgery recommended to aid in controlling Nolan's acid reflux is called a fundoplication. Essentially, the surgeon takes the top part of the stomach (the fundus) and wraps it around the esophagus. This tightens the lower esophageal sphincter, and helps to prevent reflux. It is a remarkable cure for reflux for some children.

So why would we question its necessity?

For some children, the quality of life is worse after fundoplication. Depending on how tight the wrap is, kids may not be able to burp or vomit. This can lead to a painful condition called gas bloat, particularly for children who do not have a g-tube. Retching is another side effect that can occur - some children get the dry heaves after the procedure, and it is extremely debilitating. Kids have to go on a liquid diet for a period after the surgery, then they are advanced to a pureed diet - after several weeks, carefully chewed solid food is allowed. Because the lower esophageal sphincter is tight, some children may choke or be unable to swallow larger lumps of food.

Most of the kids who have negative side effects are those with pre-existing motility issues: the peristaltic action in the stomach or esophagus is reduced.

We know Nolan has borderline/mild delayed gastric emptying. There is some question as to the accuracy of the test he had performed, since his gastric emptying scintiscan was not performed with one of the foods with a known digestion time. In addition, the definition for pediatric delayed gastric emptying varies widely by clinic. Nolan had about 25% emptying after one hour - in our area, that is considered mild. In others, that would be considered flat-out delayed emptying.

His first impedance probe also showed a slowed bolus transit time for his esophagus. This means that food doesn't pass efficiently from his mouth to his stomach, and a fundoplication could cause this situation to worsen in intensity. Here is a story of a child who had a fundoplication with undiagnosed motility problems, and she had several complications post-fundoplication.

There are very few motility centers in the United States. There is one in Boston, and one in Cincinatti. Prior to consenting to a fundoplication, we must have motility testing performed to verify that Nolan's reflux is "uncomplicated." For many children, the motility disorder is the cause of the reflux - and motility medications can be added to increase the peristaltic contractions. This helps to reduce the amount of reflux occuring.

Kids who have one fundoplication performed are at risk for the wrap slipping and causing a hiatal hernia, which requires another fundoplication - the procedure is irreversible, and the potential for more surgeries down the line remains.

Added to the motility concerns is the possibility that Nolan's hypopneas are caused by his low muscle tone, and not because of the reflux. Children with low muscle tone (hypotonia) are prone to obstructive apnea. In this case, a BiPap would be the best solution for his apnea. This is why we have booked an appointment with his sleep neurologist, to discuss his opinion on Nolan's tone and the potential efficacy of a BiPap machine.

If we finish our investigation and find that 1) Nolan has no underlying condition, 2) there is not a complicating motility problem, and 3) the only way to help the apnea is to do a fundoplication, then we will move forward with the procedure. Until then, we have a lot of research to do to ensure the best decisions are made for Nolan.

Sleep Study #3: Report

We had the official sleep study report faxed to us, because we wanted to see what the sleep neurologist's recommendations were. Since our pediatrician's office wouldn't give us the report, and our ENT didn't discuss the recommendations from the sleep neurologist, we wanted to review the report to see what the neurologist would recommend.

the report is like any other sleep study report. This is the best sleep study he has ever had, but there are still significant issues. A brief rundown of the actual data:

Minimum O2 concentration during sleep: 87%
Average O2 concentration during sleep: 98%
Number of obstructive hypopneas: 57
Number of arousals during sleep: 125
Respiratory Disturbance Index (REM sleep): 20.1
Respiratory Disturbance Index (non-REM): 7.6

For most people, that's a lot of mumbo-jumbo. A normal Respiratory Disturbance Index for the pediatric population is <1.5: Nolan is obviously above this threshold during any sleep stage. He woke up 125 times during the study. The good news is that despite occasional oxygen desaturations, his average oxygen level is great.

He isn't retaining CO2 yet, which is important. Children who retain carbon dioxide effectively poison their systems with the retained gas - Nolan's CO2 levels were 42mm Hg on average, which is in the normal range.

The sleep neurologist's recommendations read:

"Despite improvement compared to previous study (8-9-10), these findings indicate the ongoing presence of obstructive sleep apnea (for the pediatric age range normal RDI <1.5) consisting mainly of hypopneas with associated disruption in sleep architecture and oxyhemoglobin desaturation, especially in REM sleep (RDI: 8.6, and minimum oxyhemoglobin concentration 87%). If clinically indicated, evaluation by ENT for persistent airway abnormality should be considered. If this has already been performed, a return to the lab for nasal C-Pap or BiPap (given history of hypotonia) titration could also be considered. Clinical correlation is suggested."

I called the sleep neurology center and made an appointment for June 21. I want their take on his situation, and advice on doing a C-Pap or BiPap with the presence of persistent reflux.

While I was at it, I called the Cleveland Clinic and started the process to get into the genomics division at that center. Our ENT feels strongly that Nolan does not have a syndrome. I am not sure whether there is an underlying disorder causing his issues or not, but we are not consenting to any more surgery until we can verify that there is no underlying neuromuscular, genetic, or metabolic problem. The Cleveland Clinic has to get financial approval from our insurance company, so we sit with baited breath waiting to hear back from financial services and the genomics department. If our insurance approves the appointment, we could get in as early as late July or August. If nothing else, getting an "all-clear" from a fresh set of eyes will make us feel better and make better decisions for Nolan.

In Which the Third Time is Not the Charm

I was pretty sure Nolan had failed his sleep study, so I had Dennis come with me to the doctor's appointment. That turned out to be a good idea, since my instincts were correct: the supraglottoplasty has been entirely unsuccessful. His obstructive apnea remains, at about the same AHI (apnea-hypopnea index) as before the surgery.

In our quest to quell the apnea, nothing has worked. The tonsillectomy and adenoidectomy were useless - a procedure that cures obstructive apnea in over 90% of children with the condition. The supraglottoplasty was unsuccessful, which cures obstructive apnea in over 95% of children with laryngomalacia-induced airway problems.

The reason for the failure? Reflux. You have to say it like a dirty word to get the gist of how I feel about it - it has caused so many problems for Nolan, and nothing seems to work to counteract it.

We can hear the stridor returning. The apnea is obviously still there. Whether the reflux is causing the apnea because of a relapse of the laryngomalacia, or causing Nolan to obstruct on the reflux itself, is unclear. We do have evidence that he is failing his pH probes on the Zantac and Nexium, and the respiratory issues remain.

The ENT cannot do anything else for him, since the reflux will destroy any effort to surgically correct the apnea again. She recommended a procedure called a fundoplication, which I'll cover in another post. Trust me, there is no "fun" in fundoplication.

The surgery is irreversible and can be associated with gas bloat, retching, and a need to do more fundoplication procedures in the future. Most kids still have to take anti-reflux medication after the fundoplication is performed.

On the other hand, some children have great success with the procedure, and become totally free of apnea. Unfortunately, there is no way to tell if Nolan will be a success story or if he will have problems after the procedure is completed.

A C-Pap is not recommended for children with uncontrolled, severe reflux because the machine holds the airways open. Since Nolan's airway is likely contracting (laryngospasm) to prevent the reflux from entering his lungs, a machine that forces his airways open will simply provide a clear passageway for the acid to enter his lungs. Essentially, we'd be trading sleep apnea for aspiration pneumonia. I would like to get a second opinion on the matter, however, so I have made an appointment with the sleep neurologist for June 21.

We have decided to hold off for now. Nolan does have apnea, and the apnea will have to be addressed at some point in time: it can cause everything from learning issues, to CO2 retention, to cor pulmonale. For now, Nolan is not retaining CO2 and is growing, even if he is not sleeping well. We want to give him a few months on the high-dose Nexium (20mg, 2x per day) to see if it helps. If he is not clinically improved in September, then we will revisit the fundoplication idea and see a general pediatric surgeon.

In the meantime, we have requested another visit to genetics. Our ENT feels that Nolan does not have a syndrome. There are a lot of reasons for this: primary among them is that Nolan has normal cognition and no major problems with his heart or other metabolic processes. On the other hand, we are concerned that our not-quite-four year old child has had so many surgical procedures. We have a child with a congenital mixed hearing loss, urology issues, mild tone issues, laryngomalacia, severe extra-esophageal reflux disease, amblyopia/astigmatism, and chronic sinusitis/ear infections, and sleep apnea. Granted, many of these issues could be attributed to the reflux (the ear infections, sleep apnea, laryngomalacia, and sinusitis). The others, however, seem rather atypical. I don't know of many four year olds who wear hearing aids, are recommended for fundoplication, have airway issues, wear glasses, and have urology issues. Certainly, it is possible that Nolan got hit by lightning several times in a row - it just doesn't seem very likely.

I am going to make an appointment with Cleveland Clinic genetics to rule out any genetic or metabolic syndrome. Before we even consider a fundoplication, I need to make sure that there are going to be no more "surprises" that could complicate things.

In the meantime, our ENT wants another upper GI barium swallow test. I'm not quite sure why this is: when Nolan had his first upper GI barium swallow, the results were entirely normal. It isn't a very sensitive test for reflux (the gold standard is an impedance probe, or for LPR reflux, a pharyngeal probe). We know he has severe reflux, and I doubt his anatomy has changed during the past two years - though perhaps the test will show whether he is aspirating his reflux (barium would show up in the lungs in this instance).

I am waiting for the sleep study report to get mailed to us (the ENT's office printer was not functional, so I am still waiting for my child's medical records). For us to effectively advocate for Nolan (and to make the best medical decisions), we need access to the full report and the recommendations included by the sleep neurologist.

It looks like this summer is going to be rather busy...

Another Trip to Buffalo

Nolan, panning for "gold"

Nolan's post-sleep study ENT appointment is today, and I have been on pins and needles regarding the outcome of the study. I really, really wish the pediatrician had released the report to me, so that we could review the results prior to the appointment. On the one hand, if he has passed the sleep study, then we could have been relieved of stress over a week ago. If he has failed it, then we could prepare appropriate questions prior to considering our options. Doctors have a lovely tendency to spring the results on parents, then shove surgical papers at them before the information has been fully digested.

Anyhow, Dennis and I are both going to this appointment, just in case he did fail the test. If he has apnea, we will have to make a decision about doing a revision supraglottoplasty. In addition, if his laryngomalacia has returned, then we know his reflux isn't under control with the medication.

I'm trying not to think about it too much, since we simply don't know the results yet - if he passed the sleep study, then all is well and we can relax. In fact, if he passes this one, I think I will throw a party. Everyone is invited!

Matt is still on the DDAVP, without any success at the 0.2mg/day dosage. We increase the dosage to two pills/night (0.4mg/day) this week. I am hopeful that we will see some improvement, so that we can avoid a trip to the pediatric urologist. Buffalo is a great city, but I'd like to go there for a reason other than doctor appointments, thank you very much.

We did get some nice, sunny weather this weekend and the boys were invited to a birthday party in Dry Gulch - a pretend mining town in our area. They had a blast, digging up gems and seashells. Matt won an agate slice during a game, which made his day. I think we have two budding geologists on our hands!

A Mish-Mash of Updates

• Nolan's IEP meeting is on the 25th. This should go pretty smoothly, since his language is generally age-appropriate and we're going to maintain his services. Once we get his new goals into place, we should be set for another year.


• I finally managed to get an appointment to adjust the settings on Nolan's FM system: it is currently set to 100% FM, which is not great for the classroom setting (he can't hear his peers with it on). Since his ENT wants a hearing test, he'll have an audiological evaluation and FM System adjustment on June 7.


• Matt obtained his prescription for DDAVP (Desmopressin). We had the option to try this drug prior to starting on invasive testing through a urologist. If the DDAVP works, then we know that the little guy's bladder just needs to mature. If it doesn't, then we will have to see a urologist. We will start with 1 pill/day for week one, and if there is no improvement we will go to 2 pills/day for week 2. The last step is 3 pills/day during week three - if it doesn't work at that dosage, then we have to go to the urologist for more testing.


• The Nurse Practitioner has seen the sleep study results for Nolan, but won't review them with me. She said she would really prefer Dr. Brodsky to review the results with us, so I won't see the sleep study report until Monday. I hate having to wait until the main appointment to see the written report, because it is easier to formulate questions after I've had time to digest the information in the report. If he passed it, then I wish our pediatrician's office would just tell me - the extra stress of waiting isn't really appreciated. If he failed it, then several more procedures will likely be recommended - and it would be nice to not get blind-sided by the information (and have to make decisions) at the appointment on Monday.


• I made a vision appointment for Matt - I don't suspect any issues, but he starts kindergarten next year, and I thought it would be a good idea. Also, siblings have a higher rate of developing amblyopia than the general population, so it is probably a good idea to make sure both eyes are in good working order.

The weather has been truly awful: this entire spring, we have seen exactly 7 days of sunshine. We are supposed to see the sun and hit *gasp* SEVENTY degrees on Saturday. Otherwise, it has been rain every. single. day. With temperatures in the 40's. I will be glad when the weather warms up and we can get outside to play again!

On Language Holes

Nolan has excellent language. He's probably ahead of his age level in general language skill level, and his vocabulary is at a pretty high level. Sometimes, though, his "swiss cheese language" shows: he will not know a common word, even when he knows many others. Here is a list of words - guess which ones Nolan doesn't know?

Equivalent
Swing
Parallel
Broom
Boot
Fence
Gate
Interesting
Amazing
Motorcycle
Belt
Watch

There are some pretty high level vocabulary words on the list - ones that you wouldn't expect a typical three year old to understand. There are also some basic nouns on the list, that any three (almost four) year old would know. Here is the list again, with the words Nolan doesn't know highlighted in red:

Equivalent
Swing
Parallel
Broom
Boot
Fence
Gate
Interesting
Amazing
Motorcycle
Belt
Watch

Yep - the kid knows words like "equivalent," but has no idea what a broom is called. In fact, when he saw our broom the other day, he called it a Cleaning Thing. He later modified it to a Cleaning Stick, until we supplied the correct word and we worked on it for a while. He also had no idea what a mop was called (The Other Cleaning Thing). We caught this particular language hole during a speech session, when we saw a witch riding on a Cleaning Thing broom.

Typically hearing children will know the name for an object long before they can describe its function. Kids who miss incidental learning, however, will sometimes miss out on the basic vocabulary. He picks up some things, and misses others - in a completely unpredictable pattern. We always keep an eye out for language holes so we can address them, but they are hard to find until you run across some every-day object that Nolan calls a "thing."

I find it ironic that he has an SAT level vocabulary (which might lead some to think - "why is this kid getting language services?") but is missing some basic words.

In other news, we're still waiting on the sleep study results. The pediatrician has them, but won't let me see them - I have to wait until the 23rd to talk with the ENT. This leads me to believe he has failed the test, since the pediatrician's office will generally call with a delighted, "Don't worry, Mommy - the results are good!" when he passes something. Oh, well - only a week more until we know if we're in for a whole new spate of surgeries and procedures.

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.

Nolan's Urgent Message

"Mom! Mom! My glasses are DIRTY!"

Yes, son. That would happen when you color on them with crayon.

Sigh...

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.

Hashing Out IEP Goals

Spring is here, and certain parents know what that means: IEP season has arrived. For those that don't know what an "IEP" is, it is an "individualized educational plan" written for children who need educational supports or other specialized education.

For Nolan, the IEP is put in place because of his hearing loss. This is an interesting dynamic, because Nolan's language tests as age-appropriate, and he has no delays. If he were older, we would consider placing him on a 504 plan (this provides physical accommodations for children with physical handicaps).

Nolan is, however, only three years old. We want him to have a "running start" into kindergarten, to strengthen pre-literacy skills, and to have coping skills for situations where his hearing will inevitably be impacted by background noise.

His IEP meeting is on May 25, and we have to hash out some goals for the little guy. There are some areas which need some work, so we have come up with the following goals:

• Nolan will initiate peer interactions. Currently, Nolan will only initiate interactions with adults. In the classroom, he will only partake in parallel play unless aided by another adult. He will never initiate social contact with another child. He's come a l-o-n-g way, but socialization is very important, so we will place this goal into his IEP.
• Proper use of pronouns. Everyone is a "he" or a "him," which makes sense. Our household is almost entirely male! For some reason, Nolan has also reverted back to referring to himself in the third person nearly all of the time. I'm not quite sure where "my," "me," and "I" have gone!
• Articulation. Most of Nolan's articulation errors are age-appropriate (like fronting: using a /t/ for /k/and his use of /w/ for /l/), but he has some related to his hearing loss (confusing M and N). Most of the time, he is intelligible to a stranger, like any other child his age. Occasionally, the articulation errors compound in a single word. "Nilt" is "milk" - a stranger may not understand this word out of context.

Anyhow, I am looking for goals appropriate for a child turning five years old. The IEP will be in place next year (when Nolan is four- going- on- five years old), so I need great goal ideas for a child getting ready to enter kindergarten! Hearing loss mamas out there, do you have any great pre-literacy goals/auditory goals/self-advocacy goals? We are trying to keep the number of goals down on the overall IEP (easier to chart and track), but ideas are most welcome from those of you who have been-there-done-that!

Sleep Study #3

Chilling on the couch after a long night!

Last night went really well, all things considered. Nolan watched his portable DVD player all the way up to Buffalo, and seemed to be in a rather good mood. We checked into the 9th floor sleep study unit - Nolan recognized the floor as the same-day surgery unit, and was upset because we didn't go to the playroom.

Our regular sleep study technician is no longer working there, which was a bit of a bummer since he knows us by name. The new technician was really wonderful, though, and had Nolan attach all the wires and leads. He fussed for a minute about the nasal cannula, but didn't seem to care after a few more minutes. We had 'lights out' by 9:00pm, so the hook-up went really fast!

He was probably out by 9:45pm, and I could hear him breathing with his typical stridor. It wasn't as "heavy" as it is sometimes, but it was definitely present. This doesn't necessarily mean anything, because kids with laryngomalacia will often continue to have stridor, even if the apnea is gone. I did watch the CO2 monitor for a while and saw some spikes to 55mm Hg along with a reduction in respiratory rate (normal is under 45mm Hg) - this might be indicative of some hypopneas or apneas. The alarm went off on the CO2 monitor in the morning, due to condensation in his nasal cannula. The sleep study was almost over, so the technician disconnected the in-room unit so we wouldn't have to hear the constant alarming. We won't know until we get the sleep study back in two weeks.

The sleep study technician was optimistic - "He's been through enough! Let's pass this one, little guy!" At 4:45am (wake-up time), she noted, "He did really well. He slept on his back a lot last night!" She's not allowed to tell us how the sleep study went.

Of course, now I'm trying to read meaning into her statement. "He did really well!" could mean that his apnea is gone. Or it could mean that he was a real trooper and did a great job with cooperating. Does sleeping on his back indicate that he is breathing better?

I have to stop it, because we simply won't know anything until we get the official report back. Of course, this means that in 10 days, I will begin calling the pediatrician's office to see if the official report is in - the stress of waiting for test results can be quite annoying at times.

Nolan did get an "award" for cooperating so well - he was quite pleased with the Lightning McQueen stickers. This technician was really wonderful with kids - she gave him a Lightning McQueen blanket and a Lightning McQueen pillow to sleep with. Our former technician never gave us cool stuff to sleep with! She also gave Nolan some crayons and a Batman notebook. Batman is Nolan's second favorite superhero (Spiderman > Batman > Superman). He was quite pleased with his spoils - he has already drawn a "rocket ship that can't go because a little fly is sitting on it" in his notebook.


This nurse was really amazing - she won't remove any of the stickers in the sleep lab, because she doesn't want the kids to associate sleep studies with any "ouchies." She gave me some adhesive removal wipes and we went home with the stickers still attached. Unfortunately, Nolan regards these things as his "powers" and wouldn't let me take them off. I finally convinced him to let me take off the ones on his face - he is still sporting the EKG leads and the leads on his legs.

We did give him a shower to get the goop out of his hair, and then he proceeded to run around the house like the energizer bunny hyped up on speed. It is 9:30am now, and he has crashed on the family room sofa, while watching Signing Time.

Now we just have to sit back and wait for the test results!

Let the Exhaustion Marathon Begin!

Nolan has his third sleep study tonight. Basically, this is a test of how long Mommy can survive without sleep: we will leave our home at 6:00pm to make the two hour drive to Buffalo. We check in to Buffalo Children's at 8:00pm, and the hook-up begins shortly thereafter. The hook-up involves many different electrodes, wires, and belts - usually the process is complete by 9:30 or 10:00pm. Then the technician turns the lights off, and leaves.

Nolan usually falls asleep in less than an hour. I, on the other hand, stare at the CO2 monitor and watch his breathing. Since Nolan is connected by so many wires and tubes, they get tangled frequently - which requires the sleep technician to come in and disentangle the mess of wires. I would guess that I manage to sleep about 3 hours during the sleep study - the lights come on at 5:00am, and I drag a goopy-headed Nolan to the car for a 2 hour drive home. Thankfully, Dennis is going to take Matthew to school in the morning: this means I get to take a shower before doing Nolan's speech therapy and doing the afternoon routine. Matt has a school program tomorrow, so I will be going from the Pre-K pick-up spot to drop Nolan off at the babysitter's house, then to Matthew's "Mommy and Me Tea."

I suspect I might be a zombie by Wednesday evening - just in time for swimming lessons!

All kidding aside, I truly hope Nolan passes this sleep study. On the one hand, it would be really nice to ditch these tests. On the other hand, we really don't want to have to do a revision supraglottoplasty for his laryngomalacia. If he passes the obstructive apnea portion, we should be free and clear. The central apnea was markedly reduced at the sleep study in August, so I have all of my fingers and toes crossed that this sleep study shows a clear airway and no apneas.

Swimming Lessons Are Fun

I wasn't quite sure how swimming lessons were going to proceed with Nolan. He certainly loves the water, and he is convinced he already knows how to swim. Matthew, being the sage, older brother, advised him: "No, Nolan. This is different. You can't touch the bottom in this pool." His words were said with a hint of dread, because Matthew is a wee bit terrified of water.

I was extremely relieved and happy to see the coordinator arrive. She was Matthew's infant swim teacher, and is aware of some of the medical hullabaloo we've gone through with Nolan, including the minor "he can't hear" issue. I talked to her for a second, explaining that he can hear within a distance of about 5 feet in a quiet environment, but that you have to shout if you get outside of that range. I had no idea how he would fare in the poor acoustic environment of the indoor pool.

The coordinator called down a second life guard to get into the pool with Nolan. This turned out to be a very good idea, since it is hard for people to remember that he can't hear. The practical implications of this become very obvious when the teacher tells the class to get their float packs. Nolan didn't hear the instruction, so he just started walking down the side of the pool with another class. The second life guard realized he was wandering off, and told him to stop (which Nolan couldn't hear). Fortunately, the guard realized this fairly quickly, and went to physically stop him and help him back to the rack containing the flotation backpacks.

This happened a few times - Nolan ended up sitting with the wrong class or wandering off in the wrong direction at the beginning of the class. Once he was sitting on the edge of the pool (and facing his teachers at all times) things got a little easier.

I am going to make up a set of simple sign-language flashcards, because it is quite apparent that Nolan can't hear anything in the pool area. He did really well, despite not hearing, because he absolutely loves the water. He was squealing with delight and splashing at the water as he took his turn swimming in the water with his appointed life guard.

I was worried about how he would cope without his hearing during swimming lessons, but the YMCA has bent over backwards to make sure he is (1) safe, and (2) getting an appropriate level of instruction by adding in a second teacher to the class.

You can see how much fun the little guy is having during class - if you can see his face in the video below, he is all smiles!



[There is no audio/speech in the video, just background noise from the pool environment]