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.

Pediatric Rheumatologist: Finding Answers

We had a visit with Nolan's new doctor today - a pediatrician and a pediatric rheumatologist. Nolan's leg pain can be excruciating at times, occurs during the day and night, and can affect either leg. He often complains of knee pain, though sometimes it is the entire leg that hurts. He conveniently had an attack the other night and I recorded it:


And then, of course, there are all of the other issues. The progressive laryngomalacia with obstructive apnea, the lingering central apnea, the gastroparesis and vomiting attacks, the reflux, the posterior urethral valves, the vision, the progressive hearing loss, and the fatigue.

We saw the new doctor and went over his history. The first thing she did when she heard about the vomiting attacks was to ask us if we had a supply of Zofran on hand. For a child with a Nissen Fundoplication and cyclic vomiting issues, Zofran is a magic bullet to stop the vomiting. No prior doctor has offered it to us before - she had written the script before I could say "no, we don't have that at home." We now have a script for Zofran to keep on hand - if he starts vomiting again, we can give him the Zofran and (hopefully) halt the vomiting attack.

She then did a physical exam. She looked into his ears and saw the 2 tubes in his right ear and 1 tube in his left ear. She looked into his eyes and then started moving him around a bit. She showed me a few "fun tricks" Nolan can do.

His fingers, hands, wrists, elbows, hips, and knees bent ways that joints should not really bend.

While we were aware that he had some hypermobility in his hands, we were told a year ago that it was limited to his small joints. Now all of his joints are affected, and his knees and hips are extremely "loose." They are so loose that they can slip out of joint and dislocate. The awful knee pain Nolan gets is likely due to a dislocation. One way to get the joint back into position is to pull on his leg... this is probably the reason Nolan yells, "Pull my leg! Pull my leg OFF!" when he has leg issues. He has had one incident where his leg locked into a bent position and he was in agony - this was due to a dislocation of his knee. The rest of his pain comes from a type of arthritis caused by hypermobility.

Now, of course, we have to ask the question of why Nolan has extremely loose joints, hearing loss, digestive problems, fatigue, progressive laryngomalacia, etc.

Typically, a neuromuscular disorder or connective tissue disorder is the underlying cause.

The first thing the doctor is going to do is to request a copy of Nolan's brain MRI from when he was 2 years old. He fits the profile of a child with Chiari Malformation. While the MRI supposedly had "ruled out" this problem, they only scanned his brain. Apparently, the spine should also be surveyed to absolutely rule out Chiari. We may have to repeat an MRI - but the rheumatologist is going to review the CD's of Nolan's MRIs to see how much of the brain stem and cervical spine they managed to scan.

There are several other possibilities, and lab work has been ordered to rule out autoimmune conditions. A CPK has been ordered to rule out muscular dystrophies, and another test has been ordered for a gene called MTHFR (which can cause a host of various chronic diseases). This round of lab work may not give us an answer, but it will rule out several things so we can focus on other options. Of course, we may not get "The Answer," but many things make more sense now.

Nolan will have difficulty walking long distance and will become fatigued more easily than other children. His body requires more energy just to keep his joints in alignment and functioning than a typical child. The digestive system and laryngomalacia make more sense, too - those systems also rely on muscle tone and/or proper collagen formation to function properly. Nolan's severe astigmatism also fits into this picture - the eyes require proper connective tissue formation to keep their proper shape. The hearing loss doesn't quite fit into the picture yet, though several connective tissue disorders (and neuromuscular disorders) are associated with hearing loss.

As a side note, Matthew probably has a milder form of the same disorder. Children with these disorders often suffer from constipation (as Matt does) because the colon cannot keep its proper shape and doesn't "rebound" like a typical person's colon. He also has the reflux and late-onset laryngomalacia.

In any case, we will have the lab work done for Nolan and will see the specialist again next week. Even if we don't get a name, at least we will have tried and we know the general "category" of disorder that Nolan has.

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 #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.

Early Christmas Magic

Sometimes, Christmas Magic comes in abundance. With a lake effect storm swirling outside the PICU window, my little boy was tucked safely in a warm bed. Breathing, completely on his own.

He didn't feel very good, but Santa Claus made his way through the PICU and gave each child a small gift and their parents a great dose of hope and joy.

A little Christmas Magic

The night fell and my small boy continued to breathe, having only one temporary desaturation (likely a central apnea, unrelated to the procedure). In the morning, the nurse brought a tray full of breakfast. Aspiration is a big risk from this surgery, so we needed to have him eat successfully before he could go home. Nolan took one look at the eggs, and immediately wolfed them down. Then he asked for some cereal. Then a Popsicle. Then some macaroni and cheese.

The ENT called to see how he was doing, and she was amazed and thrilled. This surgery almost NEVER results in such a fast recovery. To everyone who said a prayer for our dear boy, thank you. They worked, and worked well!

By the afternoon, the ENT said he was doing so well he could be discharged a day early. We unhooked the I.V., took off the blood pressure cuff, and prepared Nolan to go home. There was, however, a little glitch. Nolan became rather attached to his EKG leads, and called them his "powers." Whenever anyone would come to his bedside, he would look at them out of the corner of his eyes and would say, "I have powers." We also learned very quickly not to reason with a three year old on drugs. Loratab is a wonderful invention, but makes Nolan a wee bit goofy!

When the nurse wanted to remove his "powers," he threw a fit. She had to give him her cell phone as a diversion, then removed the wires. She left the stickers on to avoid a full-scale revolt. She also gave him extra small leads to use at home.

Nolan, with Powers.

As we left the PICU, we ran into a group of elves handing out teddy bears. Nolan's Very Sad Eyes earned him a free teddy bear, then we walked to the elevator to go home. The elevators were not working for some reason, so we went over to the employee-access one (PICU patients are walked out by a staff member). The elevator was going in the wrong direction, so we went along for the ride. As it turns out, this was a very lucky decision! The Niagara County Sheriff's Department volunteers boarded our elevator with a giant bin full of toys. They saw Nolan's Very Sad Eyes and immediately gave him a V-Tech electronic reader and software.

By the time we left the hospital, we were lugging enough loot to rival Santa's sleigh. Despite feeling rather poorly and being in pain, these gifts brought a big smile to our little guy's face. Well, most of the time, anyway!

Feeling poorly, but healing quickly!

So now we sit, with a little boy who is healing rapidly and asleep in his own bed, breathing under his own power. And what better Christmas gift could possibly exist?

Sleep Study Results: Round 2

The wait for the results of Nolan's most recent sleep study has been excruciating. Since breathing seems to be rather vital for growth and survival, it is an issue that frequently weighs on my mind. The results are a bit of a mixed bag- I'll start with the good news.

Central Apnea:

Nolan's central apnea has improved by quite a bit. In the first study, he had 60 central events with his oxygen plummeting to 79%. The apneas were about 10 seconds in length, which was troubling considering his apneas were so short.

In the most recent study, Nolan only had 10 central apneas. TEN. The lowest oxygen saturation was 90% (for 12 seconds), which is a considerable improvement! The length of the apneas was anywhere from 10-15 seconds.

And now the "bad" news:

Obstructive Apnea:

Nolan's obstructive apnea in the first study consisted of 3 obstructive events and 41 hypopneas.

The current study shows that his obstructive apnea has actually gotten worse, post-tonsillectomy. He had 69 obstructive hypopneas. A normal Respiratory Disturbance Index (RDI) is <1.5 for children. Nolan's RDI is 11.5.

This is considered "moderate" obstructive sleep apnea for the pediatric age range, and is generally treated with C-PAP. Unfortunately, the neurologist noted that he has mild frontal bossing and a decent amount of midface hypoplasia. This means she's not very keen on the idea of a C-PAP mask on a still-developing face, when there is already an underdeveloped midface. The pressure of the mask could cause further problems with his still-growing bones.

Our treatment options are as follows:

Option 1: C-PAP. We could start C-PAP now and just watch Nolan's face carefully for any worsening of his hypoplastic mid-face area. The benefit is that it should solve the obstructive apnea and aid in any central apneas. The negative is the obvious issue with Nolan's facial structure.

Option 2: Medication. We could start Nolan on two different drugs (essentially steroids) to see if they help clear up the obstructive apnea. This is usually reserved for milder cases of obstructive apnea, but it remains an option. I'm not keen on this plan, for two reasons.

One is that all medications have side effects, and I don't know if the benefits outweigh the risks. The medications may not help, and the effects of the medications on a growing child concern me. The other reason I'm not keen on this plan is that we don't know why Nolan has obstructive apnea. He's not overweight (actually slightly underweight) and his tonsils were clearly not the cause of the apnea. Throwing him on more medications in the "hope" that it "might" help seems a tad rash to me.

Option 3: Watch and wait. We'll do nothing now, and follow-up with a sleep study in 6-12 months to see where things stand. Based on the information at that point, we'll make treatment decisions.

This is the option we chose. We'll do another sleep study in May, nine months from now. If the apneas (of either type) get any worse, we'll initiate C-PAP or BiPAP. If they stay the same, the likely solution is C-PAP or BiPAP. If they get much better, then I'm throwing a party and you're all invited!

We'll see what the ENT thinks of the latest round of information. Overall, the sleep study results are much better than the baseline study. We'll remain cautiously optimistic until we get the results from the May 2011 study!

Sleep(less) Study #2

It started raining on our drive up to Buffalo Children's yesterday evening. Sleeping in one's clothes is never a comfortable business, but sleeping in damp clothes is rather unpleasant. Ugh.

We checked in at 8:00pm and were old pros at getting hooked up- Nolan got into his jammies and we started applying the 6 quatrillion leads and wires to his body. Two leads on each leg, a few on his chest, several on his head (including under his eyes and on his chin), two belts around his chest, and a nasal cannula for monitoring CO2 on his face. And the pulse-ox, too. We can't forget about the pulse-ox.

Nolan was generally a good sport, though he did get anxious from time to time and declared, "These stickers hurt me!" They didn't really hurt, of course, but Nolan was worried the would start to hurt, or were harbingers of worse to come. The pulse-ox brought more tears, and we gave him the option of wearing it on his toe or his finger. Through sobs, he announced, "ON MY FINGER!" The nasal cannula was the coup de grâce, causing general shrieks and outrage. He settled down fairly easily, however, and was soon only whimpering about getting the "tubie" off.

I promised him that if he went to sleep, that we would take everything off in the morning. He looked at me and said, "Sun come up, take tubie off. Take stickers off." This seemed to calm him down greatly, though he was still anxious. The pulse-ox on his finger was of considerable concern. He decided he'd rather have it on his toe, or better: he'd rather have it taken off entirely.

A flash of brilliance entered my mommy brain. "It's a light saber. You're a Jedi." Adding in some detailed accounts of how jealous his brother would be to have a light-saber-finger sealed the deal. Nolan started battling "light sabers" with his fingers, and fell asleep in short order.

I stayed awake for a while to watch his CO2 monitor (his respiration rate and CO2 concentration are the only monitors in the sleep study room). I'm pretty sure he had some apneas, but we won't know for sure until we get the report back from the neurology center on August 19th. In any case, he slept better than he did for the last sleep study, giving us a good deal of sleep time to monitor his breathing.

I happened to wake up at 5:00am, which was good because the Sleep Study center comes in to wake everyone at this time. The technician came in and we unhooked Nolan from his myriad tubes and wires. The EEG goo left his hair looking rather disgusting, so I washed some of it out in the sink. I just got his temples, but it was enough to allow him to wear his hearing aids again! It was a relief to get to the parking garage, because we looked like zombies- covered in splotches of goop with a bad case of bed-head.

70 miles later, Nolan fell asleep again. This was rather convenient, because I placed him on the couch and brewed the Biggest Pot of Coffee That Ever Existed.

I'm going to end this post now, because the coffee is calling. Here's to a good report, with no future sleep studies required!

Of Birthdays, Preschool, and Sleep Studies

Yesterday, I received Nolan's preschool registration papers in the mail. On one hand I can't believe my baby is ready to head out with a tiny backpack and join a class of other children-the time from his birth to now seems far too short to produce a three year old child. On the other hand, Nolan is incredibly ready for the extra enrichment that a preschool program provides, and I am thrilled that he will be in a class with many of his friends.

His birthday is at the end of this month, and I want to throw a small birthday party for him. One with only a few children, of course- owing to his age and to his general dislike of noisy situations. I don't have anything put together yet, but it will consist of dinosaurs (Nolan's favorite animal), cake, and possibly a pinata.

Before preschool and birthday parties can commence, however, we have one last medical hurdle to clear. Nolan's sleep study is on Monday night, and I am praying with every fervent bone in my body that the central sleep apnea will disappear. If it disappears, then it means all of the apnea was caused by his acid reflux (reflux inflames the tonsils, which then causes obstructive apnea, and long-standing obstructive apnea can induce central apnea spells). If it doesn't, then he has a separate neurological issue causing the apnea, and we will have to begin bi-pap trials.

Here's to a successful sleep study, completed birthday party planning, and a great year in his three-year-old preschool class!

Getting Ready to Fly Home

I have several John Tracy related updates, but not enough time to blog about them! I will write about our final days at the Clinic when I get home. Here are a few updates:

1. Nolan's ear is still infected, but not draining as badly as it was in the beginning. The Ciprodex drops are actually going into his ear (rather than floating on top of the fluid), so his tube is probably unclogged.

2. We took our final picture, said tearful goodbyes, and drove up to Oxnard for a final weekend with our family. We fly to Buffalo tomorrow morning.

3. Nolan's IEP meeting is on August 5, and the psychological testing will be done sometime on the 3-4th of August.

We are going to be extremely busy when we get home. Besides the typical post vacation clean-up, we have more than a few appointments on our plate. The general schedule is now:

August 3 or 4: Psychological testing for Nolan's IEP.
August 5: IEP Meeting
August 9: Sleep study at Buffalo Children's
August 16: Follow-up appointment with neurologist (for sleep study)
August TBD: ENT appointment to discuss conflicting hearing test reports
August TBD: Audiological testing from a third site to confirm bone conduction results

We are going to be busy, but it will be good to get Nolan's hearing situation sorted out, his amplification increased to the appropriate levels, and determine if his central apnea has disappeared (fingers crossed)!

General Update, Or In Which Nolan's Tonsillectomy Has Amazing Benefits

People have been asking if Nolan is three lately. This wouldn't normally be a striking question, since he will be three in a little over two months. Earlier this winter, however, we had been asked how many months remained until he turned two.

Nolan isn't really very short, but his "toddle" run and low weight made him look a tad younger than his true age. He was also tired much of the time, so we had to carry him a lot- adding to the age confusion.

Three months ago, we couldn't walk the length of the mall without Nolan crying and falling to the ground, too tired to go on. I actually had visions of carrying a five-year-old Nolan on my hip to the bus stop, because he literally couldn't walk more than 100 feet without collapsing in exhaustion.

Then he had his tonsillectomy and adenoidectomy this April. I expected a reduction in snoring and better breathing. We had hoped for better sleeping. We did not expect the sudden burst of energy and general health following the procedure. Nolan's post-tonsillectomy health improvements include:

Weight: While we were at the vet's office, I decided to throw the boy on the scale. It was accurate for our cat, so I'm pretty sure it was accurate for Nolan. The reading? Twenty eight pounds. Twenty eight pounds. Less than a year ago, he was nineteen pounds. This is some major weight gain, folks. If the vet's scale was accurate, he's now in the 20% range for weight. Much of this improvement was made with the Nexium, but the tonsillectomy seems to have added some benefit to his appetite. We have discontinued the DuoCal, and he is eating food without calorie supplementation.

Energy: Nolan has become, frankly, a bit of a terror with his energy level. Running laps around the house, running ahead of us in the mall, racing his brother in the driveway... doing what a typical two year old should do.

Height: Nolan's height was never much of a concern, but it had stalled out and dropped from the 80% to the 10% mark. He is growing again, and is now almost 36" tall. We're happy to see him climbing the charts and outgrowing his size 24 month shirts!

Happiness: While his screaming/crying episodes are not completely gone, they are greatly diminished. It is a rare morning that we have an hour of "inconsolable Nolan." His general happiness is improved.

Sturdiness: Perhaps because he is running more, his legs seem a bit more sturdy. He doesn't fall as often when he runs, and he seems less "loose" in his stride. This is probably a result of better muscle tone, since he is running around a lot more with his increased energy.

We are absolutely thrilled that Nolan is doing so amazingly well. You may have noticed that I didn't include "sleep" in the benefits- "sleep" seems to be a mixed bag. Since the tonsillectomy (2 months ago), Nolan has slept through the night six times. This is good, because it is approximately six times the number of nights he has slept through since birth. It is not, however, the improvement we had hoped for.

He also still snores, and has some noisy daytime breathing. We're not sure what to make of that, but the sleep study in August will give us more information on whether he is having any apnea. Judging by his increased energy levels, his quality of sleep must be improved (even if the quantity is not greatly improved). His nighttime wakings may also be a product of his severe reflux, so we're trying not to read too much into his nighttime capers.

If we had known a simple tonsillectomy/adenoidectomy would have had this much effect on Nolan's well-being, we would have been much less worried about having the surgery done. Here's to more energy and more growth from this point forward!

ENT Visit and Noise Overload

Nolan's tonsillectomy/adenoidectomy/PE tubes post-op appointment was today. These trips to Buffalo are quite wearing, with four hours in the car (round-trip). The ENT is Nolan's least favorite doctor (apart from the urologist, though we rarely see him anymore). I promised him a treat after the appointment, since he was quite upset about seeing the ear doctor.

The post-op appointment went well. We saw the nurse practitioner and she noted Nolan's runny nose (he nearly always has a cold) and checked on his tubes. They're in and functioning, which is no big surprise since our audiologist checked them on Thursday. We also discussed his post-operative state, which is generally good. He still does not sleep through the night most nights, but this could be due to his reflux and not necessarily due to apneas (central or obstructive). The sleep study in August will tell us if the obstructive apnea has been eliminated, and hopefully the central apnea will be greatly reduced, as well.

She was concerned about having to increase the volume on his hearing aids, but they don't have his hearing test report yet. I told her that his hearing levels aren't drastically different, but his aids had been set to levels recorded a year ago. There has definitely been change in a year, so he needed to be reprogrammed. She was concerned about the progression, though we're not sure if it is a permanent conductive or permanent sensorineural change (we didn't run bone conduction). John Tracy will run testing in July, so I'm not worried about it. Either way, it is a permanent change, so the increase in amplification is only a good thing.

We left the ENT's office in Depew, and I thought about heading to East Aurora to the Explore and More Children's Museum. Unfortunately, they're closed on Monday. Not knowing what to do, I headed toward Orchard Park and took them to Chuck E. Cheese.

Chuck E. Cheese must be the worst place on the planet for a deaf or hard-of-hearing child. The "background noise" must have been at 90dB! Still, Nolan was literally dancing for joy, and we were the only people in the place. The employees had just run the tickets through the machines, so they told the kids that they could grab all of the left-over tickets. Matt and Nolan ran around the arcade in a giddy, hyperactive state, clutching tickets and thrusting tokens into games and rides.

This ride had its own camera.

I did use sign language at Chuck E. Cheese, because Nolan couldn't hear anything in the place. I was concerned about losing him in the arcade, but I only handed him one token at a time. Within a few seconds, he would reappear, calling out, "Money! I need more money!"

Pizza, juice, and several cheap plastic "reward" toys later, we headed home. We don't have to see the ENT again until October, which is a great relief! We are now "doctor free" until late June (kidney ultrasound- routine monitoring because of his posterior urethral valves)!

Off to the Library

Dennis is working all day today, thunderstorms are expected, and I don't want to get stuck in the house today. Two small boys have far too much energy to keep cooped up, even if the weather is threatening.

We're heading out to the library, to make use of the children's area (puzzles galore). I also have a book on hold ("The Help" by Kathryn Stockett, if anyone cares).

Nolan is quite whiny again today, so this might be a very l o n g Saturday. Sometimes I wish there was an indoor playground for children in our area, since we have a lot of inclement weather. I hope the library "re-sets" Nolan's attitude (and mine)- we've already had two hours of non-stop crying.

If that doesn't work, I'm investing in earplugs!

A Much Better Day

Nolan, playing at the playground on a rather fine day

Someone must have said a prayer on our behalf yesterday, because Nolan slept through the night last night. This event is so rare, it is blog-worthy. We are all much, much happier after a full night of rest! Nolan has not been crying all day long today: much of his persistent crying behavior is due to lack of sleep.

When I decided to quit my job to stay at home with the kids, I underestimated the "burn-out" factor. Staying at home full time after working in an intellectually stimulating career has been wonderful, for the most part. Unfortunately, there is no break when you are Mom. Add in an insomniac toddler, a winter full of medical problems, and a spate of bad weather... and the weariness factor increases tenfold. There is a high probability that I am losing my ability to communicate with adults.

Truth be told, we need a vacation. I'm not going to get a "real" vacation this year (while Dennis and Matt are camping in the Sequoias with family, Nolan and I will be at John Tracy). Still, some attempt at a vacation must be planned, because I am becoming a rather stressed-out mother. And stressed out mommies are no good to anyone.

So we're going to Pittsburgh. It might not be the Bahamas, but it is close enough to do over a long weekend, and it has plenty to do for small children. I managed to secure a decent three-star hotel in Greentree, PA for $59 per night on hotwire.com. It has an indoor-outdoor pool, is in the outskirts of Pittsburgh, and it has free parking.

We're going to visit the Children's Museum, the Carnegie Science Center, and the Pittsburgh Zoo and PPG Aquarium over Memorial Day weekend. No doctor appointments, no therapy, no rushing to preschool/swimming lessons/ice skating lessons/MOPS/library story hour/speech therapy. Just a nice, long weekend of swimming, playing, and visiting a few new places.

I truly cannot wait for this little trip!

Very Cranky

We have had a week of cranky. I had truly hoped that Nolan would begin having restful nights after the tonsillectomy and adenoidectomy. Unfortunately, our little insomniac has not found his path to slumber, and we have had many wakeful nights. He does not nap, even if we take a long ride in the car. Nighttime usually results in several trips to Mom and Dad's bed, until we eventually are so exhausted we just give up and let him thrash around sleep in our bed.

I'm not sure if the wakings are caused by his apnea or if his reflux is flaring up. He still doesn't have his two-year-old molars (he is a very late teether), so teething is also a possibility.

This mama is worn out by the sleepless nights. Not to mention that he cries all day long. The constant crying pushes me to the edge, and by the end of the day I end up in an exasperated fog. Whatever the cause, I truly hope the boy sleeps soon.

The next sleep study in August seems very, very far away.

Tonsillectomy/Adenoidectomy and PE Tube Surgery

Nolan was surprisingly cooperative and in a good mood on Tuesday morning as we headed up to Buffalo Children's. He allowed the nurse to take his vitals, we discussed the runny nose issue (his lungs were clear, so we were allowed to proceed), and we headed down to the surgical waiting room. Things were on-time, so the little guy only had to wait for 2 hours and 15 minutes once we arrived at the hospital, and Nolan was entertained by Mickey Mouse cartoons on the waiting room television.

Our ENT came in and took our little guy from us, and we waited for about an hour until the pager went off to let us know he was out of surgery. Everything went very well, and Nolan was taken directly to the PICU (Pediatric Intensive Care Unit) for monitoring. He was awake and screaming when we arrived, both in pain and confused from the anesthesia.

Because he has severe central apnea, the anesthesiologist only gave him half the dose of Morphine most children receive. He also received no pain medication after the surgery, to ensure that his breathing was stable. Our ENT prescribed Tylenol with Codeine for pain relief, but the Codeine has been cut in half to reduce any potential complications with respiration.

He was quite upset for the first two hours in the PICU, crying and becoming hysterical. He looked like a Very Sad Boy, with bloody ears (from the PE tube placement- this can be shocking the first time you go through the procedure, but sometimes the ears are a tad "messy") and a swollen neck. Luckily, the Tylenol with Codeine took over and he fell asleep.

He slept for about 2 hours, with no desaturations or apneas. When he woke up, he was a tad fussy, but not hysterical. A few popsicles and watching Cars on the PICU television settled him down nicely. He was fussy off and on throughout the day, and fell asleep for the night at 11:00pm.

He set off the alarms quite frequently during the night, but only once for a desaturation and only about 5-6 times for apneas. The alarm was constantly going off for "extreme brachycardia" (slow heart beat) which caused me some panic. The nurses explained this is a common (and normal) heart rhythm in children.

In the morning, he ate some jello and had a few more popsicles. The attending came in the morning to discharge us, and we headed home. Nolan was doing extremely well on the car ride home, even crying for french fries from McDonald's. We kept him to smooth foods that first day, just to be safe with his throat- so no fries for poor Nolan.

He ate macaroni and cheese for dinner last night and had oatmeal for breakfast this morning. He is refusing to drink anything, but will eat Popsicles. We're using Popsicles for his hydration needs, and hopefully it will be enough to stave off dehydration. Keeping up on the Tylenol with Codeine seems to be the most important thing at the moment, as he gets cranky as he gets toward the end of the 4 hour interval between doses. I have been warned, however, that the real pain of a tonsillectomy occurs later in the week- something that someone else who has been there confirmed. Sometime around day 7-10, the scabs in Nolan's throat will fall off and his throat will be extremely raw- this is also the time he is in danger of bleeding.

For now, we are managing quite well and he is becoming quite the Popsicle addict. Those Mighty Minis ("slow melt") are wonderful. I don't know what we would do without them!

Tonsillectomy Update

Recovering with a "Mini Melt" Popsicle

This is short and sweet because we're all tired. The tonsillectomy/adenoidectomy/PE tube placement went exceptionally well, Nolan had a completely uneventful night in the PICU, and we are home. He's eating popsicles and is doing amazingly well when we keep up on the Tylenol with Codeine. So far, this has been much, much easier than I expected it to be!

A more detailed update to follow.

MRI Results

I called the ENT's office today to see if Nolan's MRI results were in yet. The nurse practitioner called me back within a half hour to say that the results were in. With the caveat that the ENT has not personally reviewed the scans and that he is just going by the report written by the radiologist, he gave me the result:

Normal.

There is no Chiari, as expected. There is also no tumor or mass on the brainstem, lesions, or anything indicative of degeneration. This is one time that I am unbelievably grateful for the term "idiopathic." Sometimes, having an unknown cause is better than all the known ones.

The tonsillectomy/adenoidectomy/PE tubes can continue on without delay, and if the central sleep apnea is still there in August, we will begin evaluating the use of a Bi-Pap machine.

There is nothing wrong with my baby's brain. To say that I am relieved would be the biggest understatement in the history of the world.