Haya's Super Tuesday results

Well, we had our own Super Tuesday yesterday right here.

First, a miraculous visit to the neurologist. On Sunday, after countless phone calls to the doctor's secretary and receptionist over six weeks, we finally scored an appointment. She's that very senior and popular neurologist to whom we brought Haya in August 2019. 

Subsequent to that first visit, on that doctor's advice, we tried adding Phenytoin to the Vimpat, Keppra and medicinal cannabis which Haya already receives. After only ten days, it proved disastrous - read: oodles of additional seizures. We promptly stopped it, also on that doctor's advice.

 
Since then, the only change we've made has been independently adding Curcumin supplements. They seem useless. We've observed no changes in her condition whatsoever.

Now here's what today's visit - at which only my husband was present - yielded:

• Doctor recommends trying the ketogenic diet. I'm wary of that. It was such a nightmarish 10 months when we last did that some twenty years ago. But the doctor assures us that nowadays we'll have a qualified professional dietician guiding us and Haya should not end up vomiting incessantly from all the fat - as she did twenty years ago when no neurologist or dietician in this region had a clue about the diet. So we'll see about that option. I recall reading that it isn't terribly effective with SCN2A patients.
• The doctor will be administering a 24 hour Video EEG in the near future. We await a date. One hurdle here is getting the head nurse in the pediatric ward to admit Haya despite her being nearly 25. Since our doctor is a pediatric neurologist, Haya must be in that ward.
• The doctor found my video clips of Haya's seizures and twitching - which Haya obliged me with the morning of our appointment - to be very helpful. You're welcome to view them below.
• The doctor told us that both of the meds we've read about as unorthodox treatments for SCN2A epilepsy - Lidocaine and Mexiletine - are unavailable here in Israel. But she said she would inquire about Mexiletine and is agreeable to trying it with Haya.
• She isn't a proponent of cannabis for epilepsy claiming there are no reliable studies to back that up. She only recommends Epidiolex, the first cannabis-based medication approved by the US Food and Drug Administration. While it's been available by prescription in all 50 states since 2018, it's not available yet in Israel.. 

Super Tuesday also had Haya doing hydrotherapy and flipping herself over several time of her own accord. Usually, I initiate the flipping. But yesterday she proved to be a flipping enthusiast - I had no idea she actually enjoys it so much.

Here she is at the start of one:

And then at night it was on to the assisted walking I am scrupulous about. Here we are plodding away, as we do for 45 minutes:

VNS vacillation

M.E.D.E.K. walking: Chaya and me

Chaya's latest pressure sore is pressuring me these days. It appeared nearly healed last week but then did a 180 and looks terrible today. I texted a photo of it to our incomparably competent, reliable and kind home-visiting nurse. He works for Kupat Holim Meuhedet and, predictably, responded with revised treatment instructions (back to the Flaminal Hydro ointment). (Yes, he is a real person, not a figment of my wild imagination).

On Tuesday, he came by and diagnosed an infection. He says to continue with that ointment but warned to call him if there's fever or increased redness. (Might be a bit dicey with all those neurological fevers Chaya likes to run - they are unrelated to any infection or illness.)

Otherwise Chaya's seizure status has been pretty stable. This could be thanks to her raised cannabis dosage. Nearly a year ago. under the neurologist's instructions, we stopped raising it at 11 mg/kg/day which translated into 11 drops of oil three times a day. But at our visit to her in April, we learned that our dosage was far from the maximum. (Why had the doctor stopped at 11? I have a hunch she only prescribes the stuff and raises its dosage when parents prod her to.)

Anyway, when I suggested raising it this time, she approved. We're now at 14 drops, 3 times a day (i.e. 14 mg/kg). The doctor said that benefits peak for most patients at 15 but that 25 is considered the maximum.

True to form, I'm afflicted with a serious case of optimism again. The doctor didn't give any specifics about the rate of dosage rise so I'm just winging it. Wednesday night, C. seemed very tired and did her M.E.D.E.K. walking with her eyes closed for the first 20 minutes.

(I remember that she was also very sleepy when we first raised her to 11 drops. She gets used to the increases quickly and hasn't been sleepy since.)

At that April visit, the neurologist also pushed the VNS option. So, on Tuesday, we trekked with C. to a neurosurgeon who implants the VNS. He was very friendly and honest. Actually, to a fault (the honesty, that is. I liked the friendliness.) For instance, he told us there's no chance that the VNS would improve C.'s cognitive skills. None whatsoever. And that the assurances we were given to the contrary by the VNS distributer's nurse/salesperson were bunk. She had insisted that even where seizures remain unaffected by the device, cognitive improvement was often detected.

His honesty even included this self assessment: "I haven't got the most VNS experience in this country but I started implanting them before everyone else." Not exactly a confidence-building statement. He went on to assure us that the device was very likely to improve her seizure situation by either reducing their frequency or intensity. ("I don't quote statistics", he added.)

But then he proceeded to the risks. Since the device in C.'s chest which was implanted in December 1999 is very obsolete, the surgeon said he can't replace it by connecting the new one to the old wires. That would have been  a simple procedure done with a local anesthetic. Instead, the entire device and its wires would need to be removed and replaced - surgery that requires a general anesthetic.

Chaya's VNS device was implanted in 1999
just below her collar-bone where it's quite visible
as this snap shows.

In addition, the risks posed by the implant itself include infection in skin and even spreading to the device - which would necessitate a second operation to remove it. Oh and the risk of a stroke. He said it's never happened to any of his patients. But still, I'd say.far from confidence-building.

I then asked him for his opinion about the procedure in C.'s case. Once again he waxed honest: "It's a doable and reasonable move," he said. He added that while seizure control wouldn't improve C.'s level of functioning "since her brain is already fully developed", it would halt her decline.

The thing is, she hasn't been declining for many years. Her condition plateaued a long time ago. Which left me in serious doubt that the benefits outweigh the risks. (I actually raised that with my husband while we were in the office and he ssshhh-ed me emphatically. He doesn't want the surgeon to suspect that we're less than enthusiastic.)

The surgeon was plainly blown away by C.'s gauntness, ("She has zero skin or muscle") and said that were she able-bodied she'd have been committed to the hospital ward for anorexics. We assured him she consumes large quantities of calorie-rich food and we have no clue where it all goes. (She's on a mild version of the Modified Atkins Diet which is said to help control epilepsy.) He was as stumped as we are. He prescribed generous quantities of avocado in advance of the surgery since some fat on her bones would minimize post-op complications.

Well, that's all the VNS news for now. My husband and I have agreed to give the raised dosage of cannabis a chance before deciding about the VNS.

Oh, and when we mentioned in passing that Chaya takes cannabis, the surgeon pronounced: "I don't believe in cannabis."

We're clearly still a long way from acceptance among the medicos.