John Tracy Clinic: Pre-Planning

I received our informational packet for the John Tracy Clinic Summer Session. We have already purchased plane tickets to Los Angeles, but there are a lot of things to do prior to our trip. John Tracy was wonderful and included a checklist for the forms which need to be submitted before we leave:

• Confirmation of Participation (Check! Completed online).
• Housing Reservation Request (Check! Completed online).
• Children's Immunization record
  
• TB Test/Clearance for children participating in program
• Audiology documentation request
• Adult health statement and TB test/clearance
• Arrival date and time (Check! Completed online)
• Any test results (hearing and developmental) recently received.
  

I will schedule Nolan's TB test after we finish the tonsillectomy/adenoidectomy/PE tubes- probably sometime in late April or early May. Dennis and I will get ours around the same time- Matt doesn't need the test because he is too young to participate in the sibling program and will be staying with grandparents during the trip.

The audiology documentation request is for our audiologist. We will take it to her when we go for Nolan's hearing test in May. She will send in all of Nolan's hearing test results and will fill out a "permission form" for John Tracy to adjust Nolan's hearing aids. Knowing our audiology clinic, I have a feeling they will decline this portion of the form and will check off the "I would prefer changes not be made if John Tracy has suggestions for this child's device programming or audiological care." Our local audiology clinic does not do aided audiograms and believes they are invalid for digital hearing aids. This will be interesting, since Nolan has never had an aided audiogram and I have a feeling there will be a bit of a disagreement between John Tracy and Buffalo with regard to Nolan's hearing aid settings.

Nolan is having language and developmental testing performed in April, so those results will be sent along to John Tracy as soon as I can make a copy of the records. I'll also send along a copy of his hearing test report when it is completed in May.

July is only four months away, and I am getting really excited for this trip! Los Angeles, here we come!

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.

MRI, Episode II: The Wrath of Kid

Nolan, playing with his "prize."

We started our day at 6:00am, waking Matt early to feed him before Nolan awoke. Then we woke Nolan, gave him his Nexium and a small amount of diluted apple juice, and headed up to the hospital. Check-in was uneventful, though Nolan howled at the indignity of the medical bracelet around his ankle.

Buffalo Children's has always been an "easy" hospital for us, because they have always had a "siblings allowed" policy. With no family in the area to watch Matthew, it has always been a policy we loved. Unfortunately, they changed their policy- and failed to notify us. Upon check-in, we were told that Matt and Dennis would have to stay in the lobby area. If we had known about the policy change, Matt and Dennis would have stayed home, because Buffalo Children's is the least "child friendly" hospital in the world, as far as facilities go. The lobby area has nowhere to wait- no chairs, no waiting room... nothing. The waiting room is on the second floor, where siblings are not allowed to go. For the record, they also have no changing tables in the bathrooms: if you need to change your child during a clinic visit, the dirty tile floor or the chairs in the waiting room are the only available space. There is a small cafeteria, and Dennis was prepared to stay there for the entire day with Matt.

Don't get me wrong- I totally understand the policy in the wake of the latest flu season. I just wish we had been made aware of the policy change prior to arriving with the entire family in tow! Next time, I'll handle Nolan on my own and Dennis will stay home with Matt.

I headed up to the 9th floor with Nolan, and he began to scream. He was upset at being separated from Matt, and hysterically yelled, "WHERE MATTHEW?" at the top of his lungs. There were several other families on that floor with siblings in tow, which was a tad frustrating- apparently we were the only ones to get singled out for this new rule. He continued screaming for so long that they couldn't get good vitals on him. His O2 levels wouldn't go above 95% and his blood pressure was high. The nurse got us a small private room (he was frightening the other children) and called Dennis. Then she phoned security and told them to allow Dennis and Matt to the same-day surgery ward. In a few minutes Matt and Dennis arrived, which calmed Nolan considerably.

Our MRI was scheduled at 11:00am, but things were a bit delayed. We were brought down to radiology at about 11:45, and Nolan began to really freak out in that area. This area is the waiting room for his most unpleasant procedures: the VCUG after his painful PUV ablation surgery, and the upper GI which required an NG tube. Luckily, they didn't hold us there for long, and moved us into the MRI waiting area for the duration of our wait.

We talked to the anesthetist and to the MRI technician, and then I brought Nolan to the MRI machine to be sedated. He cried, but fell asleep quickly with the gas. I left the room, and we all went to the surgical waiting area until he was done.

45 minutes later, I was called back to the waiting area. "Mad as a wet hen" is an understatement for Nolan's mood. No one has witnessed such wrath from a two year old before. He was screaming, writhing, and pushing himself away from the nurse trying to soothe him. This was the same nurse we have had for prior procedures, so at least she recognized him.

We had to stay in the recovery area for about 20 minutes. She explained that they had to fully intubate Nolan during the MRI (I never found out if this was because he quit breathing, or if it was preemptive because of the central apnea). With a sore throat and the IV attached, he was an unhappy camper. We got him some apple juice, and he started screaming, "I want my HEARING!"

I had given Dennis his hearing aids prior to going into the MRI room (no electronics are allowed near the MRI machine). The nurse went into the waiting room to hunt down Nolan's "hearing." From Nolan's perspective, being in a scary, painful environment with diminished hearing must have been terrifying. We got his hearing aids in, and he calmed down a little. He still yelled at any nurse that happened to walk by, just to let them know he was still angry. The nurse in the curtained area next to us had to stay behind her curtain, or else Nolan would wail in protest.

We did have to go to the 9th floor for about an hour after the procedure for monitoring. Luckily, the room already had cartoons on the TV and the nurse brought both boys popsicles. It is amazing how a popsicle can erase toddler-anger instantly. 1 hour, a "Coobie-Doobie*" sticker, and two popsicles later, we were allowed to leave the hospital.

Dennis and I were STARVING (Nolan was pretty hungry, too), so we headed to the Olive Garden for a belated lunch/early dinner. Then we went to the mall to ride the escalators (hey, it's cheaper than Disneyland) and to buy Nolan his prize: a kid-sized hockey stick.

I'm not too fond of the idea that we get to repeat this entire scene in three weeks. Luckily, the recovery room nurse is on duty that day and will request Nolan to her area. This will be nice, because he sort of "knows" her and familiarity helps to keep things calm.

It will be about a week before the results are available. I am pretty sure they'll be normal (considering the normal MRI when he was 11 months old), but getting the official "normal" report will be reassuring. Until then, we are playing outside and enjoying the fresh spring air!

*Coobie-Doobie is "Scooby Doo" in Nolanese.

Nolan's New Word

Nolan, playing on the thingy.

Nolan has learned a new word, and it is a very frustrating word. This single word is thwarting our attempt at getting new language out of him. He's rather smug about it, too- proving how very clever he is. The new word: "Thingy."

He uses this word in place of any noun, making verbal processing/auditory memory tasks a cinch. A typical exchange goes like this:

"Nolan, what would you like to play with today?"

"A thingy. Take thingy out of the thingy."

"What thing do you want to play with? The train?"

"No. Not that thingy. Other thingy."

He has realized the substitution of "thingy" is easier than coming up with the name of the object. It is quite clever of him, but we do need to break the habit before it gets ingrained!

Now I need to finish writing this thingy and make a thingy for dinner.

Impromptu Lesson

In my attempt to avoid any and all viruses circulating in our area, I took the boys to the library early (to avoid any other kids) and then we headed over to the local hardware store. I wanted to get some paint to spruce up some old nightstands, and I also wanted to buy some Stargazer lily bulbs.

We headed over to the paint department and we found the paint color we wanted. The boys were rather curious about the process of making paint, and the lady behind the paint counter was awesome. She invited both boys behind the counter to help her add the color, seal the lid, and put the paint can in the shaker. There were so many words and concepts in that little encounter: seal, rocker, shake, colorize, lever, roller, combine, gallon.

We headed home and the boys painted with water on the sidewalk while I painted the nightstands. I was so thankful to that anonymous employee who gave my boys an impromptu "field trip" and made their day. Sometimes you never know when you are going to happen upon a great language lesson!

In Isolation

Nolan, pouting at the park

There are a lot of viruses going around our area right now. In our local preschool and Mothers of Preschoolers (MOPS) group alone, there have been children with RSV, stomach flu, strep, and various other cold viruses.

If Nolan gets sick, we won't be able to do his MRI on Tuesday. It could take months to get another MRI scheduled. If he can't do his MRI on Tuesday, then we'll have to put off the tonsillectomy/adenoidectomy/PE tubes (T&A). If we have to put off the T&A, then we'll have to push back Nolan's repeat sleep study to October or November.

To prevent illness, we are in voluntary isolation. We skipped the library story hour, filled with runny-nosed two year old children. We are not going to MOPS or to Nolan's preschool. I am letting Matt go to preschool, but the children at his preschool do not seem to be as ill (possibly because they are four years old and aren't as liable to slobber on things). We're even going to the park in off-hours, to avoid sick kids.

My attempt at keeping Nolan well seems futile, however. We were outside at our neighbor's house, when another neighbor girl came over. She's in her teens, and was tickling Nolan and playing "chase" with Matthew. Then I noticed that her left eye was crusty and fused shut. She was coughing into her elbow.

"Erm... if you are sick, please stay away from Nolan. He has a medical procedure on Tuesday and he can't get sick," I explained to the girl.

Her response:

"Oh, I'm not really sick. My brother just has a really contagious cold."

This, dear people, proves that teenagers have brains which do not function on all cylinders.

I scooped up Nolan and headed home. So far, so good (no runny nose yet)... I am fervently praying that Nolan avoids the "really contagious cold."

Ugh.

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.