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.

Matt's Sleep Study: Post-Supraglottoplasty

I was happy to get a sleep study booked for Friday night - this meant that I would be able to sleep on Saturday during the day, since Dennis would be home to watch the boys. I drove Matthew up to Buffalo Children's and we were ushered into the waiting room just as the sun was starting to set.

The sleep study tech was not quite ready for us, so she sent us to the playroom. Matthew was happy to play with the toys. I, however, was not happy that someone had changed the channel on the waiting room television to play Bones. This television show is definitely not for children, and Matthew was rapt with attention as they showed a half-rotted corpse on the show. I finally found a power button on the side of the TV. Matt went back to playing with his pirates. I was not happy.

We were finally ushered into a room and Matt changed into his pajamas. Matt played on the Kindle Fire with the Bad Piggies game, using it to test out several rocket designs. Sleep studies are much easier at this age, as the kiddos can entertain themselves!

At 9:30, hook-up started. By 10:00, Matt was settled in bed and I was reading a chapter of Harry Potter and the Sorcerer's Stone. He wasn't happy about the nasal cannula, but left it alone. He's actually giggling in the picture below - I was worried it would take him a long time to fall asleep.

Fortunately, he fell asleep almost as soon as I turned the lights out. He stayed asleep until 2:30 am, when he woke briefly and said, "I think it is dusk outside," then immediately fell back to sleep. I managed to get about an hour of sleep, which is much better than getting no sleep at all. At least I would be somewhat coherent on my drive back to Jamestown.

The lights came on at 5:00 am, and we grabbed some Tim Horton's donuts (and a coffee for me). We were home by 7:30 am. I thought I would nap during the day, but I have a hard time sleeping during daytime hours. Matt and I both had early bedtimes that night, though!

I won't get the results from Matthew's sleep study until October 28, which is when we have our follow-up appointment with the ENT. Hopefully his sleep apnea is gone!

Sleep Study Number Six

Nolan had his follow-up sleep study from his revision supraglottoplasty last night. I am absolutely exhausted, so this post may be a complete jumble of incoherent mumbling. Fair warning.

We arrived at Buffalo Children's for the hook-up at 8:00 pm. The children's hospital is much better than the alternate sites because the tech is familiar with children and all of the equipment is properly sized. They also perform CO2 monitoring at the children's hospital and do not perform this test at the alternate sites.

Nolan was a champ with the hook-up. He's so used to the process that he didn't blink an eye.

The only part of the process that he hates is the nasal cannula. He couldn't remember what it was called and kept telling us that he "hated that mustache!" He turned the volume up to maximum on the TV speaker and watched some Sesame Street.

The same episode plays in a loop, so our wonderful respiratory technician brought in a DVD player with Toy Story. Nolan was quite happy with the arrangement, particularly when she rearranged his head wrap so he could put his hearing aids back in.

He fussed a little when the movie was over, and asked us to take his "mustache" off several times. He finally fell asleep, but not for long, He cried out frequently, but the tech came in and told me it was during stage 3 sleep. His legs moved all the time, waking him from sleep. I heard a few apneas, but I am not sure if they were central (some centrals are normal) or if they were obstructive apneas.

With the constant thrashing and crying out, I did not sleep at all. At 5:00 am, the lights came on.

We woke Nolan up. Well, we sort of woke him up. He was in a zombie-state for quite some time.

We left the hospital and drove 2 hours to our house. Nolan fell asleep on the way home, so he got a little more sleep in the car. I am beyond exhausted.

Tomorrow, we get up at 4:00 am to take Matt to the hospital for his supraglottoplasty.

At least we're never bored.

Signs of Childhood Sleep Apnea

Several people have asked what led to my request for a sleep study for Matthew. I thought I would write down some of the signs and symptoms that caused me to realize he had a high probability of having sleep apnea.

Firstly, Matthew has always had epic melt-downs. Particularly in the afternoon and early evening. Since this pattern is common in toddlers, I largely ignored it when he was younger. When the pattern continued at the age of six and seven years old, however, I knew something had to be causing the tears, anger, and inability to function past 3:00 pm.

Matt also has "tunnel vision" and it is impossible to get his attention. He obtains a glazed-eye appearance and you can call his name over and over again with no response. If you tap him on the shoulder, he will "snap out of it" and respond.

These two things would not be enough to definitively say he had sleep apnea, but I have a little secret to share.

When everyone else was shocked that Matt had laryngomalacia, I was expecting the diagnosis.

In May of last year, I heard a sound that caused me to look twice at the boy sleeping on the sofa. Stridor. Not snoring (stertor), but stridor. My heart thumped a little harder, and I took a video. I posted it to a laryngomalacia support group I belong to, and everyone agreed it sounded like stridor. Here is the video from last year:



 After that afternoon, I rarely heard stridor from Matt. It is very soft and difficult to hear, but I know that sound very well. Matt was growing well and coping well in school, so I decided to tuck it into the back of my mind and ignore it until it presented a real problem.

The real problems began this year in school. Matt can't focus in school. Suggestions of ADD had been tossed around. His teacher, for example, stated that everyone would be lined up at the door, and Matthew would still be at his desk. She would tap him on the shoulder and say, "Hi, buddy, all of our friends are lined up at the door. You need to join them."

Matthew would say, "Oh, why didn't you call my name?"

"I did, buddy, three times!"

And the after-school meltdowns became worse. He couldn't play tennis, because he would be a sobbing heap on the tennis court. He cried when it was time to do anything: to eat, shower, read, or practice piano.

It was time to take action. When Nolan had his ENT appointment in the fall, I mentioned his mouth-breathing, crying, and "ADD" ways to the specialist. She said, "You don't need a referral. How old is he? Seven? Get him in. NOW."

Of course, this is when I preemptively booked the sleep study to save time, since I would have bet money on the fact he had sleep apnea. And the sleep study showed that he had obstructive and central apneas. His sleep architecture was seriously disturbed. No wonder we had an unfocused, crying mess of a boy on our hands every evening.

I had every finger and toe crossed, hoping it was "just" big tonsils and adenoids. When everyone noted that his tonsils were not big, I was pretty sure we were dealing with laryngomalacia.

Despite the cause of the apnea, the symptoms are similar for children who are sleep deprived:

• Bed-wetting
• Behavioral problems (tantrums, inability to cope, crying)
• Exhaustion (falling asleep instantly at night, in the car)
• "Zoning out" (may be confused with ADD)
• Snoring may or may not be present
• Growth failure (Matt did not have this problem)
• Restless sleep 

For those parents who have children diagnosed with ADD or ADHD, please, please have your child assessed for other disorders that may mimic or cause ADD/ADHD. The American Academy of Pediatrics states that:

1. 95% of children with obstructive sleep apnea have attentional deficits.
2. For children with full syndromal ADHD, 20-30% have obstructive sleep apnea.

These statistics are from several research studies (found here) that indicate children with ADD/ADHD should be assessed for sleep apnea and treated if apnea is found, as a significant improvement in symptoms is seen after the child's obstructive apnea is alleviated.

ENT Appointment, Part 3: Tonsillectomy and Adenoidectomy Scheduled

After the ENT finished with Nolan, she moved onto Matt. By this time, we were all exhausted (and hungry): the office was running 2 hours behind schedule, so it was now 1:20 pm and our original appointment time was 10:45 am. No lunch = grumpy boys.

I gave the ENT Matt's sleep study results, which we had ordered through the pediatrician. As a helpful hint to other families, if you suspect sleep problems and are booking an ENT appointment, make sure to get a sleep study run first. It will save a lot of time and an appointment (and copay). If we hadn't had this run, this appointment would have been a consult, a polysomnogram would have been ordered, and then we would have had to return in a month or two to get the results. Since we already had the sleep study results in hand, we saved a $25 copay and about 2 months worth of time.

Matt's sleep architecture is abysmal (I wrote an article about sleep architecture - you can find it here). He has no stage 4 sleep and only about half of the REM sleep he should have. This explains a lot of his behavior and his complete inability to memorize facts (REM is required for memory formation). He desaturates to the low 80% level, which causes him to wake frequently. He also has a lot of periodic leg movements (so does Nolan).

She reviewed the results and was not very concerned about the central apneas and the heart rhythm issues. She sees both of these problems in children who have long-standing, untreated apnea. Matt is a mouth breather, so the problem is likely due to tonsils and adenoids. Since both of our boys have/had central apnea, there is also a chance that our kids just have central apnea when they're young - it could be a genetic thing. It is more likely that the central apnea is a direct result of long-standing obstructive apnea.

Matt is going to have a tonsillectomy and adenoidectomy on March 21, just a few days before Easter Break. His ENT is going to do a bronchoscopy and sleep laryngoscopy just before the surgery, because she wants to make sure he has no other airway anomalies. It would be unlikely, since he doesn't have the reflux or other health issues that Nolan has, but it is better to verify that he doesn't have any airway malacias.

Matt's surgery will be at an ambulatory surgery center, and he'll be released about an hour after surgery. The "surgery" part will be the easiest portion of his procedure: the recovery will be the hardest. Tonsillectomies are horrendous. We've been through 2 of them (Nolan had his tonsils and adenoids removed when he was 2 years old, and he had his lingual tonsils shaved at the time of his last supraglottoplasty).

I'm worried about getting Matt to take the Tylenol with Codeine (he's awful about taking medication of any kind). I'm worried about keeping him hydrated. I am going to blatantly bribe my child provide positive incentives to get him to drink: I have a "reward" bag started. Matt will have a follow-up appointment on June 3 to see how he is doing post-surgery. He'll probably need another sleep study to verify his apnea is gone (and so will Nolan, so we'll probably do another 2-for-1 sleep study).

Our family will have two surgeries in a back-to-back manner. Nolan's will be harder for the actual surgery - staying inpatient is always trying. His recovery will be easier to manage because we have the g-tube and can give him meds, food, and hydration via that route while he is recovering. Matt's surgery day will be easier because he will be home that same evening, but his recovery will be much more difficult since we need him to cooperate to drink and take his medication.

One thing is sure: I am looking forward to the end of March, when everyone will be recovered and on the road to better health!

Matt's Full Sleep Study Results

I picked up a copy of Matt's sleep study results from our pediatrician. We see the ENT on February 18 for both boys, and I have a feeling it will be an "involved" appointment, since we have to discuss Nolan's hearing, Nolan's apnea and laryngomalacia (and probably revision supraglottoplasty surgery), and Matt's results.

As a side note, some have asked about Matt and Nolan both having apnea - and whether that might mean that Nolan's apnea issues are not related to the other issues he has. In short, our kids both have apnea, but probably from radically different causes. Nolan has a condition called laryngomalacia, and he has an atypical version of the disorder. It didn't show until he was 2 years old (not diagnosed until 3 years old) and he has the form that is associated with neurological problems. A supraglottoplasty and epiglottopexy (lasering out the prolapsed tissue and tacking up his epiglottis to the base of his tongue) have been unsuccessful and his condition appears to be progressive. In Matt's case, he probably just has big tonsils and adenoids. The effect is the same, but the cause is radically different.

In any case, Matt's study shows the following (with abnormal results in bold):

EEG/EOG: Latency to sleep onset was within normal limits at 21.9 minutes. Latency to REM sleep was slightly prolonged at 188.5 minutes. Overall sleep efficiency was normal at 92.8%. The patient was observed in both the supine and non-supine position during the recording. Frequent arousals were present and were commonly associated with respiratory events. Spontaneous arousals and arousals associated with limb movements were also observed. All stages of sleep were identified.

What this means (in English): Matt was asleep for a normal percentage of the time, but it took longer than normal for him to fall into REM sleep. He woke up a lot, and he often woke up because he couldn't breathe.

Respiratory Parameters: The baseline respiration rate was 16-20 breaths per minute in NREM sleep. No snoring was noted by the technician. The overall respiratory disturbance index was 5.03 with a minimum oxyhemoglobin saturation of 83%. Mean oxyhemoglobin saturation was 96%. The respiratory events were most prominent in REM sleep. Clear hypopneas were noted. Rare central apneas were also observed.

What this means (in English): Matt has mild obstructive sleep apnea - something is blocking the airflow. His oxygen level dropped frequently, and the lowest it ever got was 83% (normal is above 95%). He had occasional central apneas, where his brain forgets to tell him to breathe. While some central apneas are normal for kids, the central apneas Matt is having are outside that range and are associated with desaturations.

EKG: The baseline heart rate was 82 beats per minute in REM sleep and 78 beats per minute in NREM sleep. Rare premature wide QRS-complex beats vs. artefact were observed.

What this means (in English): This will have to be discussed with the ENT. I am not sure what this might mean (or not mean). There is a good chance we'll have a consult with a cardiologist about this. 

EMG: There were 5.5 periodic limb movements per hour of sleep noted. They were occasionally assoicated with arousals.

What this means (in English): Matt's legs moved frequently while he was asleep. This sometimes woke him up. They don't know why he is doing this. He had 33 periodic limb movements during the sleep study. 

Interpretation:

These findings indicate the presence of mild 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: 5.03 and minimum oxyhemoglobin desaturation: 83%). If clinically indicated, evaluation by ENT for possible tonsillectomy and adenoidectomy should be considered. Clincal correlation is suggested. Central apneas were also observed. Although central sleep apnea can occur in the setting of obstructive sleep apnea, a cardiogenic or neurogenic cause could be considered and warrant further investigation. Please also note the presence of periodic limb movements. They are of unclear clinical significance in the setting, but may warrant further attention if the patient remains symptomatic despite adequate control of sleep-related breathing disturbance. If follow-up with a Sleep Center physician is required, please contact the center at xxx-xxxx.

What this means (in English): Matt has mild obstructive sleep apnea, which is causing his oxygen levels to drop and has wreaked havoc on the normal progression of sleep stages. He should see an ENT to determine if he needs his tonsils and adenoids removed. The central apnea needs to be evaluated and Matt should see a neurologist and cardiologist to rule out heart problems or a problem with the respiratory center of his brain. His legs are moving a lot, and we don't know why, but we can ignore it for now and see if it goes away once his breathing troubles are treated. If his leg movements remain once his breathing is better, they may need to further evaluation.

Sleep Architecture: In a normal child of Matt's age, about 7% of sleep time should be spent in stage 1 sleep, 46% of time should be spent in stage 2 sleep, ~5.5% in stage 3 sleep, and ~18.5% of the time in stage 4 sleep. 22% of the total sleep time should be spent in REM sleep (data from this article: "Polysomnographic Characteristics in Normal Preschool and Early School-Aged Children").

Matt spent the following % of time in each sleep stage, with the normal % in parathesis:

Stage 1: 1.2% (normal 7%)

Stage 2: 57.0% (normal 46%)

Stage 3: 32.6% (normal 5.5%)

Stage 4: 0% (normal 18.5%)

REM: 9.2% (normal 22%)

Essentially, the kid is sleep deprived. He isn't getting any slow-wave, deep sleep and most of his time is spent in light sleep. He is only getting about half of the necessary REM sleep a child his age should get. 

He had 88 arousals in 6 hours of sleep, many due to a lack of oxygen and others due to periodic leg movements. 

I'm anxious to talk to our ENT to find out what the plan is. The sleep neurologist wants to either have his tonsils and adenoids removed (with follow-up sleep study to prove his respiration is better at night) or to start C-Pap therapy. I have a feeling we'll be removing Matt's tonsils and adenoids, and hopefully that will improve his nighttime breathing. 

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.

Sleep Studies Tonight

Nolan has a C-Pap titration tonight, which is essentially a souped-up sleep study. We want to make sure his pressures are set appropriately and that his apnea is controlled. Sleep studies are never fun, but since his sleep laryngoscopy shows progressively worse laryngomalacia, we need to verify that his C-Pap is handling the airway obstruction.

Since Matt occasionally snores and has a few other symptoms that may indicate sleep apnea, he's having a sleep study done, too. Hopefully he is in the clear, but as long as we're doing the study for Nolan, we might as well have Matt done at the same time.

We'll head to the sleep center this evening - our arrival time is 8:30 pm. Fortunately, Nolan is now five and can go to the sleep center in Dunkirk - this is much closer than the children's hospital in Buffalo. We're grateful for the closer location. Dennis will be coming with me, as we need one parent per child.

We'll all be exhausted after our little family "camping trip," so Saturday will be a quiet, "resting" day for all of us!

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!

C-Pap Titration

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

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


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



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



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



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

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

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

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

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

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

Sometimes I Love Living in a Small Town

Every boy needs a dog.

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

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

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

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

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

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

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

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

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.

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.

Stop-Gap Measures

Nolan has been off of his Nexium for about four or five days. After two days on the phone and an hour spent at Rite-Aid, I managed to get his former prescription restored. He was originally approved for 10mg, twice daily Nexium through the end of December, and the new prescription shouldn't have voided that approval.

He is still denied the dosage he needs (20mg, twice daily), but the 10mg dosage is better than nothing. I gave him a dose of medication this morning, so he'll be back on the Nexium for four full days prior to the sleep study.

I have heard some stridor at night, and he still wakes frequently (even prior to the insurance company denial of the Nexium), so I am anxious about this sleep study. If he fails it, then he'll be scoped to determine if the laryngomalacia has returned. If it has returned, the next step is a revision supraglottoplasty. Revision supraglottoplasties usually come with a fundoplication to curtail the reflux - obviously not a path we want to go down. I'd really rather the Nexium and Zantac do their job to control the crazy level of reflux than go through (more) major surgery.