For those who missed it on Twitter: Bertrand watching an Elmo video on an iPhone.
Our first easy meal at a restaurant!
Our first easy meal at a restaurant!
Tomorrow begins my carefully orchestrated week of daily visits to Carmen B. Pingree with each of Bertrand's therapists. Bertrand's therapists will be training the staff at CBP on his quirks, strengths, and how to integrate what they've been working on with him into his lessons at CBP. The staff-to-student ratio at CBP is 2:1, but Bertrand will be getting his very own 1:1 aide. To prevent him from becoming overly dependant on an individual, everyone has to be trained so they can rotate through with him. Bertrand starts school on February 8th.
Bertrand's sleep study results came back this past week. I've been more bummed about them than I really should be. I guess I'd convinced myself that sleep was one area in which he excelled. Turns out that Bertrand has 3.9 apnic episodes per hour. Kids under age 10 are suposed to have less than 1. He barely enters REM sleep, which is an important sleep state for memory and learning. He also has restless leg syndrome. In my opinion, this is all seizure related. I don't see the point in removing his tonsils. Control of the seizures will improve his sleep quality. That is what I am telling myself. We'll do another study in a year.
Iron deficiency can be one of the causes of restless leg syndrome, since iron is essential for dopamine transmission. We'll be testing Bertrand's iron levels, along with vitamin D, selenium, usual metabolic and liver functions, as well as the genetic test: SCN1A. SCN1A is the gene associated with over 80% of the cases of Dravet's Syndrome, also known as Severe Myoclonic Epilepsy of Infancy (SMEI). Let's hope this is another genetic test that comes back negative, but if it comes back positive this would open the possibility of using an orphan drug like stiripentol.
On the keto front, I'll be picking up Bertrand's fancy-schmancy compounded keppra Rx (400mg twice a day) from University Pharmacy tomorrow! It is being made with stevia and the taste of vanilla (mint, chocolate and marshmellow were the other non-carb options). Also, we've gotten the green light to up Bertrand's ratio to 3.5:1!--a 50/50 mix of KetoCal 3:1 and KetoVolve 4:1. We need to wait another week before shifting ratio to ensure we've seen all we are going to from the keppra increase. Then we have to wait more time before adding the branched chain amino acids! Yes, we got the yellow light for the BCAAs! Our dietitian is currently researching what the appropriate therapeutic dose for Bertrand would be.
Hmmm, I wonder if the diet could have had any impact on the sleep study results. Jade went through a couple of months of sleeplessness when we started the diet, and sleeps poorly whenever we make a ratio change. I know I've heard this a number of times on the keto group, too, and a few kids are on melatonin. I'm not saying that keto would cause sleep apnea (!) but I wonder if it could contribute to the restless legs?
ReplyDeleteMmm, that Keppra sounds good enough to eat! ;)
I'm excited to see how this school situation plays out, especially knowing that B will have one-on-one aide service. We are going to start looking into that for Hannah (as long as we can get her to cut the cord a little bit from me!).
ReplyDeleteThe sleep study results don't seem that drastic,but I guess if you put it all together, it definitely would make a difference. I know a sleep study is in Hannah's future, and I'm very fearful of the results, as I know some type 2 nGD kids have dozens of apneic episodes each hour.
BTW, this pic of B is so cute!! I love it :)