Pre-CPSE Meeting

We had considered canceling the pre-CPSE (for those not in the know, CPSE stands for the Committee on Preschool Special Education) meeting because of all the medical appointments we knew we would have this spring. We decided to reinstate the meeting once we had all the dates nailed down for Nolan's future medical procedures.

This meeting is not the official transition meeting, but just a "meet and greet" to let the district know about Nolan and to get a feel for what his needs are. Everything went great until we started discussing what Nolan would need once he started pre-kindergarten. There were two things the special education chairperson paled at: a teacher of the deaf (TOD) for Nolan and an FM system.

Children in the preschool age group are at a distinct disadvantage. They are no longer covered by Early Intervention, but are not yet covered by the services offered in school. Frankly, a 0-5 age group for Early Intervention makes more sense, but the system isn't set up to be practical. Thus, the TOD cannot consult on Nolan's education until he is in kindergarten, which will be in 2012-2013.

When we mentioned an FM system, the chairperson got excited and said, "every room in our elementary school has an FM system!"

"No, we don't want a sound-field for Nolan. We want a personal, ear-level FM system. Not now, but by pre-kindergarten."

Her demeanor changed slightly. Oh, we were those parents. The ones who know what their child needs to succeed in the classroom and won't back down. The ones who aren't afraid to break out the "equal access to his education" phrase. Darn those pesky IDEA laws.

She did state that he cannot have an FM system until kindergarten, even if he attends pre-kindergarten at their school. They might not have enough, you see, and if they don't have an extra one laying around... then he doesn't get one.

The school district isn't responsible for funding technology until kindergarten, and he isn't in Early Intervention for preschool. It will be very interesting to see how this will be covered, because he will have a personal FM system for pre-K. I don't care which agency covers it- he has to have one. It's the law.

The meeting wasn't negative in any way, but I did sense that we might have a fight or two coming up over technology. To be fair, the school district has never dealt with a child who needed assistive technology (AT) at such a young age. This is new for them, and it looks like we're going to pave the path for families who follow after us. Hopefully, by the time we're through the preschool years, the system will be "smoothed out" for any other mainstreamed children who require AT at the preschool level.

Booked

Nolan, being very sneaky.

July seems like it is very, very far away. Especially considering all the events that will transpire between now and then: an MRI, a tonsillectomy/adenoidectomy, (hopefully) Matt's graduation from an IEP, the end of the preschool school year, the arrival and passing of spring, and two weeks of Vacation Bible School.

July seems like it belongs on another planet. Still, time marches steadily on and our trip to the John Tracy Clinic will arrive faster than I expect it to. Time seems to fly when we're busy, and we are very, very busy this year. In the hopes of finding fairly reasonable airfare rates, I booked our travel to Los Angeles. We will be leaving on July 10 and returning on August 1, 2010. We'll be spending nearly a month in sunny, gorgeous Southern California.

I am very, very excited about this trip. This is an amazing educational opportunity for Nolan. He will get intensive language and speech therapy at an amazing oral-deaf school, all for free (minus the cost of the flights and modest boarding fees). We'll get to meet some friends from the blogging world, and spend some time with family while we're on the west coast.

I am also very excited for the most excellent food in the world. Since moving to the Midwest, we regularly mourn the lack of good sushi (oh, Ozeki's... we have a date!) and Mexican food. I may come back to Western NY a good 15 pounds heavier than when I left, but it will be well worth it. Yolanda's, I miss you.

California, here we come (in four months)!

A Proposed Graduation and Update

Matt has been doing really well in speech therapy. When he started speech at the age of 2, he had severe articulation, moderate expressive, and mild receptive language delays. By the age of three, the receptive language score was above average, and the expressive was average (with a severe articulation delay).

We recently had him evaluated with the Arizona Articulation Proficiency Scale, and the results were a mild delay, only one standard deviation away from "normal." He still completely lacks the /r/ sound, but this is a later developing sound in children. He also lacks the /ch/ and /sh/ sounds, pronouncing them as /ts/ and /s/, respectively. The only sounds that placed him in the "delayed" category were vowel sounds attached to /r/, because the lack of the /r/ sound distorts the vowels. For instance, the word "chair" would be pronounced "cha-oh," which distorts the long a sound in the word.

Since Matt has an isolated speech delay, the rapid ascent to the "mild" category means that he will probably graduate speech therapy this spring. Since we currently have five speech therapy sessions per week between both boys, this will free up our schedule considerably. Over the next several months we are going to focus on the final /r/ sound to help clear up those words, and we will work on the /ch/ and /sh/ sounds as well. It looks like Matt is going to leave the world of IEP's altogether this year, which is very good news!

As for Mr. Nolan, he is also doing well. I haven't heard anything about his transition meeting. I need to give our EI coordinator a call, because we have so many medical appointments this spring that I don't want to have his meeting scheduled when we are in the hospital or at an appointment. We hope to keep his current speech therapy schedule, but it is likely that we will be dropped down to 2x per week (closer to the standard speech therapy quantity for the school district).

I did call our audiologist to reschedule Nolan's hearing test. It was originally scheduled for April 12, but having a hearing test the day before his scheduled tonsillectomy/adenoidectomy, and PE tubes seems rather useless. He'll still have a conductive component because of fluid/negative pressure, so I rescheduled for May 20. By May, any swelling from the tonsillectomy should be gone and he should be free of middle ear fluid.

We have been having many restless nights: last night he woke at 1:00am and went back to sleep at 4:00am- itching (he has a persistent rash on his torso that never really goes away), crying, and saying he hurt... but we couldn't get him to tell us where he hurt. I deeply hope he doesn't have another ear infection forming, and I also pray that we'll be able to get more sleep SOON!

A Call From Dr. B

I've never had a doctor care so deeply about Nolan's medical situation as our new neurologist. She called me this morning, to reassure us about Nolan's breathing and to go over the game plan with the tonsillectomy.

We were (obviously) concerned with the apneas and the oxygen desaturations to 79%, and had asked about obtaining some sort of monitor. There are several reasons why a monitor is not feasible right now:

• We have no way to treat the apneas at the moment, so having an alarm without a treatment option is fairly useless*.
• Nolan's arousal mechanism is working perfectly, so he WILL wake up and start breathing again on his own, after each apnea.
• Nolan's apneas are approximately 10-13 seconds long. By the time an alarm went off, he would have started breathing again on his own.
• Nolan has 20 apneas per hour that result in oxygen desaturations of less than 90%. Having an alarm go off every 3 minutes throughout the night would simply leave us exhausted, and would not benefit Nolan in any way.
  
• The apnea monitors they use on infants with central apnea (prematurity or central apnea of infancy) will not work for a very mobile and active toddler.

She discussed our concerns about the Tylenol with Codeine with our ENT. She also contacted pulmonology to discuss the monitoring situation. Our ENT feels very strongly that Nolan should receive the Tylenol with Codeine, because children often refuse to eat or drink after a tonsillectomy. The pain often peaks 5-7 days after the procedure, when the scabs fall off the back of the throat. Nolan is already "low on the charts" to begin with, and the danger of dehydration and repeat hospitalization without the hydrocodone is high. So we will be treating him with the codeine for about two weeks. The main priority now is to keep him safely breathing while on that codeine.

Pulmonology does have some apnea monitors, but they do not work for mobile toddlers of Nolan's age and size. At-home monitoring isn't really an option in our case, so we need a different game plan. That game plan is:

• After the tonsillectomy/adenoidectomy/PE tube placement, Nolan will be placed in the pediatric intensive care unit.
  
• The post-operative/post-anesthesia time period is the most vulnerable for children with central apnea. If we are going to see a problem with oxygen desaturation, we will see it soon after the surgery.
• Tylenol with codeine will be trialed in the PICU, while he is fully monitored. We will trial the Tylenol with codeine during this 24 hour period. If he desaturates, he will be kept in the hospital until he is completely recovered and stable. If he remains stable, then we know the codeine isn't affecting his respiratory center and he'll be safe to take home.

I really appreciated the fact that this doctor took the time to immediately look into our concerns to keep our little guy as comfortable and safe as possible.

*We are not treating the central apnea until after we receive the results of the MRI and second sleep study. We need to confirm the absence of the obstructive component, the severity of the central component, and verify the CSA is not caused by a Chiari malformation. If the CSA is caused by a Chiari, then surgery will be required. If the CSA is "idiopathic," then we will treat the apneas with a V-Pap (a specialized Bi-PAP machine used for central apnea) and will be referred to pulmonology.

An Informative Visit with Dr. B

We had our appointment with neurologist #2 today, and it was a much better visit. She was much more thorough with Nolan's medical history, and had a clear sense of direction for the treatment of Nolan's central apnea. We also learned a few new things today- not that they are really "new" things, but confirmation of things we have always noticed with the little guy.

As far as the central apnea, we are on the right course. The MRI must be done first- to absolutely rule out a Chiari (even though this is unlikely) and to verify there is nothing amiss with Nolan's brain. She also agreed that a tonsillectomy was a good idea, to get rid of the obstructive component.

We did learn that central sleep apnea (CSA) is rare enough that there really isn't a standard treatment protocol for children with CSA following a tonsillectomy. We have two options- to forgo the Tylenol with Codeine in favor of regular Tylenol, or to try and obtain an apnea monitor for those two weeks from Pulmonary and keep the little guy on the codeine. Codeine is a respiratory suppressant, so it isn't really safe to have him on the medication without a monitor. The neurologist is going to talk to our ENT to plot a course of action.

We are going to wait a full three months after the tonsillectomy to have a repeat sleep study. This is to give Nolan time to heal completely, and for all residual swelling to dissipate. We will do the follow-up sleep study in early August, with a follow-up appointment the week after. If the central apnea is still there in measurable quantity, we will pursue artificial ventilation at night via V-Pap (a special version of biPap for central apnea).

As far as the rest of Nolan's symptoms, she does feel that another visit to genetics is warranted. We won't go back to the original geneticist, because he was completely off base.

Remember the worry about frontal bossing? Yeah. The neurologist confirmed that it is there. So we can add that back to the roster of symptoms.

Also, remember the head lag? She did a brief work-up and told us that he has some decreased muscle tone (hypotonia) in his arms and trunk. It is mild, but another piece to the puzzle.

I do have the name of a geneticist at Cleveland Clinic, and I think we'll attempt any further genetic work-ups there. We haven't had much luck at Buffalo Children's (CHOB).

For a brief re-cap, here's the "list of symptoms:"

Congenital moderate hearing loss
Posterior urethral valves
Failure to thrive (resolved)
Severe GERD
Mild Delayed Gastric Emptying (DGE)
Mild frontal bossing
Mild hypotonia
Elevated AST (liver or muscle enzyme marker)
Periodic temperature fluctuations
Periodic rash on trunk
Chronic otitis media
Severe obstructive sleep apnea
Severe central sleep apnea

Dr. Block called this "multi-system involvement," and does believe a syndrome is at play. The question is, which one?

Brief Update

I called the pediatrician's office to get a referral to the neurologist who deals with central apnea. After a few conversations with the office staff and nurse, I was told they would try to get an appointment for Nolan. There is usually a wait of about 2 weeks between the referral and the actual appointment date, because of scheduling issues.

They called back a few minutes later, and said they had an appointment... for this Thursday! Talk about getting in quickly!

This neurologist (who writes the sleep study reports and can treat the central apnea) also has an office in Dunkirk- only 45 minutes from our house.

With an appointment scheduled so quickly, and so close to our home, I am walking on air!

Hopefully this appointment will yield some answers to our questions!

Neurology Appointment: Unanswered Questions

We saw the neurologist today, hoping to get some answers regarding Nolan's central apnea and a general "plan of attack" for monitoring and treating the apnea. He was a great neurologist, and examined Nolan and talked at length about the staring spells and the hearing loss (mostly because he was fascinated at Nolan's verbal ability). Unfortunately, he's the "epilepsy" neurologist and not the "central apnea" neurologist. While he was full of insight on the staring spells, he couldn't help us at all with our main area of concern.

I gave him my list of questions at the beginning of the appointment, and he walked off with them. I never did get them back (so not only did I not get written answers, but I also lost my questions)! Luckily I remembered most of them.

As for the staring spells, he watched the video and does believe they are more "moments of inattention" than true seizures. This, coupled with the normal 30 minute EEG, leads us to believe that a seizure disorder is completely off the map (relief)! The only way to absolutely rule out a seizure disorder is to place Nolan in the hospital for three days and run a video EEG, pressing a button when we see a "staring spell" occur. Of course, as when you take a car to the mechanic and the strange noise stops, Nolan's staring spells have dissipated. In December, he was having 3-4 per day. Now, he is having one spell every other week. We did book the 3 day v-EEG, but may cancel it after talking to the other neurologist. We'll give it a bit of time, however, to make sure the spells are really disappearing for good and are not just on hiatus.

As for the central apnea, he is not well versed in this. He did tell us that Nolan's first MRI was really more focused on the inner ear, so that a Chiari malformation is still a possibility. He told us we would have to see the other neurologist, the one who takes care of kids with central apnea. I will have to call the pediatrician tomorrow and get another referral- this time to the neurologist who wrote up Nolan's sleep study report. Sigh...

Here were his answers to my questions (to the best of my recollection, since he still has my neatly typed out list!):

Should we have a monitor for Nolan at night, since he has central apnea?

That sounds like a good idea.

Will the Tylenol with codeine be safe for him after the tonsillectomy?

I don't know. I don't think it will be a problem.

How can the central apnea be treated?

You'll have to see the other neurologist.

Can the staring spells be related to the central apnea?

Not directly.

Could the hearing loss, posterior urethral valves, severe GERD, and central apnea be caused by one problem?

That's not my area. Maybe you could see genetics or something?

Is there any testing we can do to see if these things all tie together?

Maybe genetics could put the puzzle together. Central apnea and hearing loss is very unusual.

He does think there are some "unusual" things that should be looked at by a geneticist, but Nolan doesn't fit the description of any known syndrome. We don't really know any more after today's visit than we did yesterday, except that we're pretty sure Nolan doesn't have a seizure disorder. And that a kid with hearing aids who walks into a room chattering away is going to amaze doctors that obviously haven't had exposure to hard-of-hearing or deaf children in the past decade.

I hope our pediatrician's office won't give me a hassle about the referral to the "apnea" neurologist, and I hope this neurologist will help us figure out what we should be doing as far as monitoring and treating the central apnea.