Matt's pH Probe Results

In November, we received a call from our ENT's office with Matthew's pH probe results. The probe shows he has severe nighttime reflux - over 109 episodes while he was asleep. This explains his frequent apneas and sleep arousals, which results in very little REM sleep.

Coincidentally, we had noticed a lot of behavior problems starting in October - I didn't make the connection at the time, but we took him off his anti-reflux medication prior to the pH probe. In hindsight, his emotional meltdowns and ADD type behavior was directly caused by the increase in reflux when the medication was removed.

The probe was extremely valuable for a number of reasons, including:

1. We know he has acid reflux affecting his airway, as opposed to other conditions that can mimic reflux.
2. We know he doesn't reflux during the day at all, but has severe reflux when sleeping.

The second point is particularly important. Originally, Matthew was on a low-dose (10 mg) of Prilosec, given once per day in the morning.  The information gained from the probe gave us the ability to medicate his GERD more appropriately: he is now on 40 mg of Nexium, given in the evening.

Once we started the Nexium, the turn-around in his behavior was phenomenal. He is no longer melting down on a regular basis, is able to focus, and is able to reason again.

We return to the ENT's office on February 5 to discuss his progress and determine what the next step should be (likely another sleep study to keep tabs on his apnea).

I have been absent from this blog - I am now working outside the home part-time and life is insanely busy! It is a whirlwind of work/school/tennis/piano/ski club: all positive, wonderful things, but writing has been placed on the back burner!

I will update on Nolan in another post - Happy 2014, everyone!

pH Probe for Matthew

We drove up to Buffalo again this morning to have Matt's pH probe placed. His supraglottoplasty failed and he still has sleep apnea, so we need to work out the reasons why he still has poor sleep and respiratory issues during sleep.

During his last laryngoscopy, his airway was very red and inflamed. Since he has the laryngomalacia, a presumptive diagnosis of reflux was made and he was placed on Prilosec. A very conservative supraglottoplasty was done (just trimming one floppy side instead of both).

Since he still has apnea, we need to accurately determine if he has airway reflux before considering any further steps. Since he has never been formally tested for reflux, we were told to take him off his anti-reflux meds a month ago. This morning, a probe was placed to detect reflux in aerosol form in his upper airway.

Placing the probe was, of course, an awful ordeal. No child wants to have a tube placed up their nose and down their throat. I won't go into detail, but let's just say that four nurses were involved, along with a papoose board and a lot of tears. Fortunately, the probe was placed quickly and in one try. Once it was in, Matt was fine.

We went home and he's been building a big Lego airplane we bought for him. Thank goodness for Legos.

We return in the morning to have it removed. I haven't actually seen any reflux events, so it will be good to determine if he actually has reflux or not. If he doesn't, then he doesn't need to take medication for it. In addition, revision surgery for his airway would be much less risky.

We'll see what the data shows.. we will get the results on December 4.

Hearing Tests

We took the boys up to Buffalo for hearing evaluations on Tuesday. Nolan didn't have a hearing test, but we did do another aided audiogram (new audiologist) and selected new hearing aids. We are still waiting on a price quote, but Nolan will be getting Phonak Bolero Q hearing aids. We're quite excited about this, as we feel it will let him hear better. Technology has improved over the past 6 years, so new hearing aids are a good thing!

Nolan did get new impressions, as he will receive new earmolds with the new hearing aids. He chose a new color, too - he loves changing his earmold colors!

Matthew had testing for central auditory processing disorder, which is called CAPD, CAP, or APD, depending on where you live. He does NOT have APD, which is good to rule out, as it has a similar symptom set as ADD and as sleep apnea. We already know Matt has sleep apnea, so the inattention/moodiness/zoning out are due to the apnea and nothing else.

We did, however, find that Matthew has developed some very minor hearing loss. It is nothing that can or should be aided at this point, but his audiogram looks something like this:

He used to test as 15dB across the board (normal for children). While the main area of the speech banana is not affected, he is now on a yearly monitoring schedule so that we can aid him if and when the loss enters the speech range.

I am waiting to receive his audiogram in the mail, as this might be slightly "off" from the actual one. The right ear is slightly better than the left ear. Basically, he drops to 50dB at 8,000 Hz (ultra high frequencies) and touches mild in the low frequencies. He is "normal" for an adult hearing level, but has a slight hearing loss for the pediatric age range (better than 15dB hearing level). This does explain why he mishears the S vs. SH sounds. He can 'hear,' but might have difficulty in noise and might have some trouble with ultra high frequency sounds.

This does explain some of the "ADD" type behaviors.. calling his name when he's watching TV and in a busy location - it is not the busy nature of the situation that is distracting him.. he is having difficulty hearing with clarity in noisy situations. In any case, this is something to keep a close eye on.

October Update

Wait, that's right... I have a blog! Perhaps I should update it...

I have several very good excuses for not writing:

1) The kids returned to school and our routine activities keep us busy.
2) I have returned to work part-time and now barely have time to breathe.
3) It looks like this outside:

When it is beautiful outside, I relish every last second of it. Snow is due to hit this Wednesday, so we have been outside soaking up the vibrant beauty of fall.

Nolan's ENT has not been able to return to work. I  heard a very devastating rumor, but have not been able to confirm it. In the meantime, we are praying for her and for her family. We have been moved to another ENT in the same practice, who is now managing both boys' care.

Nolan is fairly stable at the moment. He did have another ear infection in September, but a round of Ciprodex cleared it up fairly quickly. He did have to start school with one ear, but managed to compensate well for the lack of hearing. We do want to look into new hearing aids for him and to obtain an aided audiogram/real-ear testing on a regular basis. He has a hearing aid evaluation tomorrow afternoon - we would like to get water resistant aids with updated technology.

Matthew failed his last sleep study, so his appointment was a bit longer. The new ENT was reviewing his records and decided that we should perform a pH probe to determine if his red/burned airway was truly due to reflux - or some other reaction (there are conditions that mimic reflux). Matt will have his pH probe placed on Friday and will have it taken out on Saturday.

In addition, we aren't certain if some of his ADD type behaviors are due to sleep apnea or to other factors. The new ENT would like to run testing for central auditory processing disorder, which is often confused with ADD (inattentive type). The testing for this disorder is very similar to a hearing test, so he will have testing performed at the same time as Nolan. Two birds, one stone.. at least we're efficient with our appointments.

This means I have three trips to Buffalo this week, and an oral surgeon appointment (I broke a tooth and will have it removed/probably get an implant). Did I mention I am working part time? Some weeks it is very part time. Sigh...

I will try to update more frequently - I keep waiting for things to slow down, but that doesn't appear to ever happen!

Pediatric Rheumatologist Appointment

Goodness gracious, it has been hot and humid lately. It was 95 degrees with 98% humidity the other day, and we were miserable in the sultry heat. Living in Western NY, we don't have central air conditioning, because summer typically lasts approximately 5 minutes and winter lasts for the rest of the year. Nolan sweats more than most kids, so he was literally soaked through in this weather.

Three days ago, a dear friend called to tell me that our beloved ENT was canceling all of her appointments. More specifically, her office was canceling the appointments on her behalf. There has been some sort of catastrophe and they do not know if she will be seeing patients again. I still have no idea what has happened, but I am worried for her and for her family. Of course, this leaves us in a bit of a lurch, as Matthew still has significant sleep apnea after his supraglottoplasty. Nolan is fairly complex as an ENT patient as well, so we need to find a replacement that can handle the boys' issues. I have a call into our ENT's nurse practitioner and we will see what she recommends.

Since Nolan had a pediatric rheumatology appointment, I thought I'd ask her opinion, too. His rheumatologist is fantastic, and always takes a good deal of time with our family.

We talked about our latest concerns, which include:

• Bradycardia (slow heart rate) when sleeping - his heart rate dips into the 30's on some occasions.
• Difficulty with sustained walking.
• Hearing loss and management.
• Periodic leg movements and waking at night.

Nolan's weight is hanging in there at 42 pounds, and he is 45.5 inches tall. His nighttime feeds are going well, so that part of his care is fine.

She wants Nolan to see a cardiologist, because his last echocardiogram and EKG were when he was 3 years old, before he had developed the heart murmur he has now. Adding in the bradycardia, it would be good to get a consultation. It is likely that everything will be reported as fine, but kids with connective tissue disorders have a higher risk of having cardiac issues. Better safe than sorry, in any case.

As far as his hearing and other ENT issues, she has a few recommendations. She is going to contact a few other families in the area to see what they are doing with regard to seeing an ENT. 

The periodic leg movements are not due to electrolyte deficiencies or to anemia (he had had blood work during the same time period as his sleep study), so it is likely there is a muscular or nervous system issue at work there. He has some minor central apnea as well as the gastroparesis, so there is something "going on" with his autonomic system. They can treat leg movements with amitriptyline, but this relaxes the palate and airways... which makes the medication a bad idea for a child with congenitally floppy airways. We'll just ignore the problem for now - there isn't much we can do about it. I am driving him to school, which lets him sleep a bit longer in the mornings.

Nolan's lack of endurance and fatigue is becoming a serious issue. Currently, we are carrying him when we are in a city or on vacation. As he grows, carrying him is becoming more difficult. He doesn't fit into a standard umbrella stroller anymore, so the time has come to look into a medical stroller. He won't need to be fitted for a pediatric wheelchair, as he doesn't need full-time use - he just needs a mode of transportation when his legs and body give out. 

We're going to look for one second-hand (Maclaren makes a medical stroller called the "Major," which isn't horrifically expensive - less than $1,000). With Nolan's complexity and mounting medical costs, she wants to investigate acquiring Medicaid as a back-up for covering medical costs. Most medically complex children have Medicaid, because private insurance does not cover DME - in the past, we have paid for Nolan's g-tubes, C-pap, and hearing aids out of pocket. Now we're adding a medical stroller. If he qualifies, we'd use our private insurance for most things, but the Medicaid for the surprising number of things private insurance doesn't cover. His medical costs can be staggering at times, so having a little help for things our insurance company won't cover.

It was a very productive appointment, and she gave us a few good ideas on where to look for a second-hand medical stroller.

We return to Buffalo on Saturday for Nolan's MRI (inner ear). 

Ear Infection (Again)

Nolan had a fantastic time the week of his birthday. He went tubing on the lake, went to a water park, and we took a trip to Toronto.

Unfortunately, all the heat/humidity/water activities  resulted in a raging ear infection in his right ear. I was able to acquire more Ciprodex drops and started treating it quickly, but Nolan had to start first grade with only one ear.

Thankfully, his new teacher of the deaf (TOD) is beyond fantastic. She actually trained at the Clarke School (an auditory-verbal program) and is thrilled to see Nolan is reading at an advanced level. He is having significant problems hearing, so his desk has been moved to the front of the teacher's desk, and he has daily pull-outs to work on the curriculum in a 1:1 environment. He is adjusting to first grade well, and loves his teacher(s).

His ear took about a week to clear up. Hopefully we won't see another ear infection for a while!

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.