Tonsillectomy?

Bertrand as he was discharged last week.

Bertrand wakes up a lot at night.  His prior sleep study was hard to interpret due to his epilepsy.

However, it's believed that he may have some sleep apnea.  He will be evaluated by an ENT since his tonsils are a bit large.

If the doctor believes there is merit, Bertrand may get his tonsils removed.  This may actually be easier and less costly than another sleep study.

Despite having a referral, getting in to see the ENT has been a nightmare.  I'll be going in person tomorrow.

School, sleep apnea and more

For those who missed it on Twitter: Bertrand watching an Elmo video on an iPhone.
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.

And much hair pulling ensues...

If I could sum today up in one word, it would be "Arghhh!"

First off, I apologize for not putting up a Bertrand video yet this week; it'll have to be tomorrow. I took video tonight, but since I waited until too late to take it, Bertrand is just falling asleep in the video. Don't get me wrong, he is adorable, but it doesn't really showcase any of the progress he has made on the diet... Unless falling asleep sitting up can be considered progress. :-P

That is not what has me pulling my hair out though. Bertrand has been sleeping a LOT on the ketogenic diet. He slept a lot before the diet, but he is breaking new records while on it. Almost 20 hours one day! But most days he sleeps closer to 18 hours, which is still a LOT for his age. This is very concerning to me as well as his Keto Team. There could be several causes.

• Hormone or glucose levels
  
• Sleep apnea (because of Bertrand's adorable chunk)
  
• Sleep seizures
• Healing
• Toxicity

Bertrand's fasting lab results from this morning all came back normal, which is good and rules that out as the cause for the sleepiness. However, what now has me frustrated is, that instead of saying, "Hey, let's keep moving forward with the one treatment which has helped him the most" his keto team is saying, "Hold up. He needs a sleep study which will take two months to get, so until then no more fine-tuning the diet, and we're not going to check his med levels at all, but if anything we'll increase them." ARGHH!

I've been really trying to be more patient and work with our Utah team instead of around them, but everything I heard today makes me want to run to Johns Hopkins! At least Bertrand's neurologist gave us the option of staying the current course without upping his medicine yet.

At least five other keto parents and one adult on the diet have indicated that YES keppra can be a cause of sleepiness and toxicity when on the diet, as it was in their cases. Wouldn't it be easier to test that now, by reducing it 50ml than waiting two months for a sleep study and EEG (because we have to be seen in sleep clinic first before they will schedule a study)? If that doesn't work, we could then try moving the dosage up. Or at the very least, if his labs are fine, why should this prevent us from moving forward with tweaking the diet and increasing at least his dinner to a 3.5:1 ratio? Something is being lost in translation or this is just CYA on our keto team's part.

*Sigh.*

To be honest, I was sick for three weeks and have been exhausted for the past week and a half. I can't even imagine what it would be like to be Bertrand: sick for two years?! Following that logic, it is not too far fetched that Bertrand could be catching up on all the sleep and healing that missed out on for a very long time. I hope it is just that. And, I hope that by the time his sleep study rolls around, he won't need one anymore. :)