Haya's update: It's a downer

I'm holding a made-in-Israel Cannabis

THC drops product in this photo

Meds

Today is Haya's first day without any Vimpat. We had her on 25 mg per day for about ten days, down from her maximum of 150 mg twice day. I intended to wait until her seizures stabilized before zapping Vimpat entirely but seizures have been rampant. 

And we need Vimpat out of the picture before we can accurately assess Fycompa.

My hunch is this med isn't Haya's magic bullet. Which means we'll probably move on to the next gun - the Keto Diet.

These days, when all else fails I've been giving THC drops to stem the strings of seizures. Here she is (below) after 22 drops of it looking, I'd say, rather high.

Blood

It took about two weeks to organize but I was finally notified by our health fund that tomorrow, weather permitting, a visiting nurse will come to our home to draw Haya's blood. It will be used to do routine tests along with a Covid-19 serological test.

If we find that she has antibodies, we can stop deliberating over the vaccine - at least for a few months.

Hydro

On Monday, after a five week, Covid-19-caused hiatus, Haya finally returned to the pool. She relaxed and floated independently for the most part but her body often tilted to the left. That was disappointing. 

I showed my son-in-law who is a physiotherapist/hydrotherapist a video clip of her in the water. He recommends re-positioning her arms to the right as they rest on her chest and placing her left hand on her right. The clip shows that they were reversed. 

Can't wait to try those tips next session.

Haya

Labels

I often read posts by parents of children with a range of disabilities on various support group sites. Some are caused by the same genetic mutation as Haya's, some by other mutations and some by none. It's a very disheartening pastime.

I'm usually struck by how much higher everyone's level of functioning is than Haya's. And when they have epilepsy, by how much their seizure-control surpasses hers.

On a site devoted to people with Downs and their families I encountered a word now circulating: other-abled, as an alternative to the "D" word.

The truth is it's entirely inapplicable to Haya. She simply isn't other-abled. She truly is disabled, profoundly and I prefer to call a spade a spade.

Sweet home, Jerusalem

In the hydro pool this week

First, we are at home, thankfully, having avoided the morass of the ER and the hospital ward ["Status report: Status epilecticus"]

Last Friday night, the hard seizures reached 3-minute intervals. My med drawer only had Diazepam which expired June 2018 and the pharmacies had none in stock. (Doctors tend to discourage its use at home so there's not much demand for it). 

But I was determined to keep Haya at home. So I relied on the pediatrician's assurances that those dates are probably posted just to compel us to buy fresh meds. And I gave it.

Folks, you can now rest assured that those expiry dates truly are, at least in some instances, somewhat advisory only. That 2018 Diazepam worked like a charm, at least for us. Haya slept seizure-free for some eight hours.

(Apparently, not all drugs are as resilient as our Diazepam, but undoubtedly, many are. So check before you discard. I'm so thankful - but have no idea why - I didn't include that Diazepam along with all the other expired drugs I recently tossed.)

I've still got six more doses here but for now paracetamol and Advil (ibuprofen), given alternately throughout the day, have been adequate.

That's in addition to her anti-epileptics and cannabis, of course.

While I can't say she's fine, relative to what she just endured, things look positively peachy.

She floated calmly in the hydrotherapy pool where we take her for an hour on Monday, although, "slept" would also be an accurate description.

And has even done her 45 minutes of assisted walking a couple of nights this week..

Today her carer took her outside for her first time since her deterioration began.

Here is Haya, sleep-floating (at the top) and bundled up for her walk (on the right).

My Monday

My Monday was largely devoted this week to my daughter Haya and her disabilities.

First was a morning tele-visit with her newest neurologist. We had consulted him a couple of times some fifteen years ago but haven't had contact since. Now we have returned to him primarily because of his expertise in medicinal cannabis. He joins our list of the 20 or so neurologists already consulted for Haya throughout her life. 

But the upshot of Monday's consult with him was to leave our cannabis dose as-is and weigh other treatment changes. Our quest for the elusive epilepsy control continues.

These are the three options the doctor served up:

• Replace Vimpat with a new drug, Fycompa (generic name: perampanel)
• Give the Ketogenic Diet a second shot. We tried it for about 10 months when Haya was about four years old and failed
• VNS surgery which would involve removal of the old one implanted in 1999 and non-functional for most years since then, and implantation of a new, improved version.

We intend to first try Fycompa which has the least hope of success given the many drug failures that Haya has already chalked up. So we've already submitted the requisite Form 29 Gimel to our health fund to receive authorization of "private importation of a drug that isn't registered in Israel". 

It was approved by the FDA in October 2012 but somehow remains in that category in Israel. 

Simultaneously, we'll pursue the Ketogenic Diet and the VNS surgery, both of which will take a while to arrange. Both offer more hope than medications.

The second event yesterday involving Haya was a Zoom session with a half dozen other parents of children who have the SCN2 mutation. It was organized by Dr. Anne Berg who is researching treatments for SCN2A patients. She wanted to speak to parents in advance of upcoming clinical drug trials. 

At Aleh during Sukkot, extremely vulnerable but maskless children

It was interesting to hear from parents around the globe, and I mean that literally: a father in India, a mother in Dubai (she actually splits her time between Dubai and Spain), and mothers from various States in the US including Pennsylvania.

Here is some background info about our group which has 670 members. The mother speaking on this clip, Leah Schust Myers (in the screenshot at the top of this post), is the one who organized yesterday's Zoom encounter:

And while the devotion and determination of the SCN2A parents I met was impressive, the ongoing institutionalization of Israel's children with disabilities is very UNimpressive. 

Our Ministry of Welfare remains mum about where the victims of Covid-19 with disabilities residing in their institutions lived, although their deadline to answer me under the Freedom of Information Act passed four days ago. They've now had over two months to provide me - and by that I mean "the public" - with that easily attainable information.

And here [Facebook link] is Aleh broadcasting to the public how very high risk its residents are for becoming seriously ill with Covid-19. 

They've posted this on their Facebook page:

Uzi and the oxygen balloons at Aleh

"Here in Aleh:
There's a program for life-saving respiratory therapy. Most Aleh residents suffer from respiratory compllications, breathing difficulties which often cause pneumonia and hospitalization.
Consequently, the respiratory therapy program of Aleh offers respiratory therapy personally adapted to the residents. The therapy includes the use of equipment and sophisticated devices that clear the lungs and intensify breathing. 
Breathing activation several times a day is incorporated into all daily activities. This photo shows Uzi bringing more oxygen balloons for reinforcement at Aleh."

And that's Aleh's Uzi and the oxygen balloons in the photo above.

Nevertheless, Aleh's high risk residents continue to be housed and placed in close contact to one another and without masks. See the photo above from their Facebook page showing extremely vulnerable - but maskless - children seated close together under a large umbrella being waved by the attendants, "celebrating" Sukkot.

Why???

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:

CBD vapors: Our new frontier

Last week, we brought Chaya back to the epileptologist who first examined her in September 2018 ["Making it a round dozen"].

During that first visit, she had spent a solid hour patiently recording Chaya's history and recommending several new steps we should take. But once home, we hit snags that prevented us from proceeding. The primary one was our inability to contact her staff. They never returned calls or emails and we needed to coordinate everything with them. 

 
I was prepared to just forget about this epileptologist notwithstanding her superb credentials and top grade recommendations. But the Hubby preferred to persevere. 

So, last week we expended a second round of time, energy and money to shlep back to her with Chaya. As it turned out, the latter proved entirely unnecessary: the doctor didn't so much as look at Chaya, let alone touch or medically examine her. 

One of her concerns back in September was Chaya's thinness. This time she asked us whether she had gained weight since our last visit. But she didn't trouble to weigh her on either occasion. Chaya might as well have been invisible and our efforts to bring her to the office were clearly wasted. 

I was puzzled and annoyed. What's the consensus out there? Am I being unreasonable?

This time around, her assistant did contact us a few days after the appointment. She is instructing us on how to switch Cannabis CBD suppliers. This is a complicated process involving reams of paperwork required by the Ministry of Health. 

The switch is necessary in order to procure CBD in vapor form. which the epileptologist believes is absorbed more thoroughly than the oil Chaya currently receives. 

To start with, we will divide dose between the two forms.

We are pursuing this option over the one that the epileptologist urged on us most enthusiastically, namely: surgery. She is keen to have Chaya's twenty year old, inactive Vagal Nerve Stimulator (VNS) removed and replaced with an updated, more sophisticated version. 

We had this urged on us three years ago by another neurologist: the one who destroyed Chaya's liver with Valproic Acid and then washed her hands of us. So, I'm sure you'll understand why it isn't an option for which I harbor any warm sentiments.

At that time, we got as far as meeting the surgeon himself. He informed us that the surgery would be "complicated" but doable. He too was eager, though he warned us that a pre-requisite would be Chaya's gaining some weight.

But before Chaya could oblige us with some weight, liver failure struck and the neurologist who had been touting the VNS surgery, as I mentioned, fled the scene. (After alleging that the liver failure was more likely caused by the CBD than by the Valproic Acid.)

Fast forward to last week's visit: I've had a couple of years to mull the surgical option and am far less enamored with it than I was then. Chaya has been through the liver ordeal and several severe urinary tract infections in the interim.

So this time I pressed hard on the brakes. "Thanks, but no thanks." I told the epileptologist. "Chaya will try the CBD vapors before we subject her to any complicated surgery." Or something to that effect.

Of course, the doctor tried to convince me of its "uncomplicatedness". But I wasn't buying it this time. 

So here's hoping those vapors bring Chaya's ravaged brain some respite from the two decades of daily seizures she has endured.

A hospitalization journal

Sunday, October 7

I'd hoped we could keep these hospitalizations annual - but it was not to be. Yesterday, a half hour after feeding herself a big meal with gusto, Chaya vomited it up in its entirety. Afterwards she copiously vomited liquid every couple of hours.

We tried caring for her at home administering Pramin suppositories - acquired from a local gemach  - as her pediatrician advised. He said that without fever or diarrhea the likelihood of dehydration was slim. But the Pramin had no effect. Horrific doesn't come close to describing that night. 

By morning, there were strands of brown blood in the vomit rendering the question of "what to do" a no-brainer. So here we are back in the ER, 11 months after Chaya's last hospitalization. 

So far, she's weathered a chest x-ray and a 12 hour struggle to get urine via a catheter (an incredibly incompetent nurse couldn't get any urine even though Chaya's on a fluid drip. The next shift's nurse collected it with ease. The consensus: it's another UTI. 

This leaves us at a loss as to how to prevent them. We had to stop giving cranberry juice several weeks ago because that seemed to cause vomiting. Should we now resume that, perhaps in a more diluted form?

Tuesday, October 9

Here we are, finally, in a proper ward (where we waited several hours in the corridor for a room):

The doctors' diagnosis has done a 180: UTI is now negated and replaced with "some infection, somewhere", possibly gastroenteritis. Presumably that triggered aspiration of vomit since the blood test results indicate infection. The profuse vomiting may then have caused intestinal bleeding which appeared in her vomit. 

She's still getting IV Controloc (pantoprazole) to prevent a repeat of that bleeding although there's no sign of it anymore, not even in her stools.

Bumped into that neurologist who we concluded had dumped us when she ignored our last email. Apparently she never saw it and is happy to resume her role as Chaya's neurologist. While she's pleased we consulted that senior epileptologist she recommended, she warns that getting regular appointments there won't be easy.

So she promptly ordered a CT and an EEG for Chaya. She also assured us she'll contact that terrific American neurologist whom we encountered during last November's hospitalization. He's the doctor who rescued Chaya from the avalanche of drugs that had reduced her to semi-comatose. 

I may have mentioned here that we can't contact consult him directly because he doesn't have a practice; this neurologist who will be our conduit to him decided to search for info about central fever and told us she learned it's not uncommon among uncontrolled epileptics!

Chaya is now off the IV drip and has begun to eat and drink. She's back on her Vimpat which was only available in pill form. (They had Keppra in IV form, so she's been getting that regularly). But I haven't resumed the cannabis yet.

We've missed hydrotherapy - Chaya's pinnacle of pleasure - which takes place on Tuesdays. We may also miss the long-awaited delivery of Chaya's new wheelchair scheduled for Thursday.

Wednesday, October 10

Back on the drip because, after a batch of heavy seizures, Chaya was unable to eat and could drink only a bit. A CT was done this morning. Not clear why it was ordered but haven't heard any results yet in any case. UTI has been ruled out even more emphatically today. It's not looking as though we'll have C. home in time for the wheelchair delivery tomorrow.

And here are a couple of gems from the medicos...

Nurse about to finish her shift comes around with next shift's nurse to do what is known here as חפיפה (= passing the baton). I hear her mention that she had taken Chaya's temperature which was slightly elevated but that she decided against Tylenol. I told both nurses that she is seizing badly and could actually benefit from Tylenol for her fever. Then the nurse about to leave delivers her pearls of wisdom: "You see, she's seizing badly because her epilepsy isn't controlled. It needs to be brought under control. She needs to see a neurologist." To me: "You should consult a neurologist." 

I regret that I didn't ask her: "Why? Do you think she might have epilepsy?"

Wednesday night, October 10

I witnessed an argument between two doctors at the station which was situated immediately outside our cubicle. I had the curtain open so couldn't avoid seeing and hearing one, a male , tell the other, a female: "You're a hussy". The other responded: "Well tell me, do you know the patient's background?" "You ought to tell me it", snarled the guy back at her. "No, you're supposed to know it," she repeated. "Why don't you just do your job?" The guy shot back adding once again: "You're a smart-ass." 

Help! So many lives are in the hands of these nincompoops.

That same male doctor had earlier declared to my husband as he saw him approaching: "Whatever you're about to say, I'm not interested unless it's urgent. I only deal with urgent matters."

Thursday, October 11

Chaya will be released today. Didn't see that coming. The CT negated anything alarming and the EEG showed no change since the last one.

While she isn't eating or drinking much, I nixed the nurse's suggestion of a feeding tube. On Tuesday, a speech pathologist assessed Chaya's swallowing skills and they passed muster. So I figured it was best to just persevere with feeding by mouth. Minimize intervention is our credo.

Thursday night, October 11

Home! And, the icing on the cake is that the new wheelchair is here too! Here is Chaya, sitting in it for the first time:

Next up: Aleh actually encourages parents to give up their children to its institutions. It does so via its new Hotline. No wonder that hospital staff repeatedly asked us about Chaya: "Where does she live?" And were surprised by our response.

Making it a round dozen

Last week, we tried out a new neurologist, one who specializes in epilepsy, aka an epileptologist. This brings our tally to roughly a dozen since, when she was three months old, we discovered that Chaya was neurologically impaired.

In the hospital waiting area

Here (right) is Chaya at the hospital while we wait to be admitted to the new doctor's office. 

We had to pay out-of-pocket because the earliest appointment with her that we were offered in the public clinic was April 2019! At a time like this, our system feels like "public medicine" only nominally. We are forced into the private domain too often.

This epileptologist gave us an hour and a half during which we covered Chaya's history, symptoms and functioning. 

Afterward the doctor conveyed her thoughts and recommendations. This probably sounds rather routine but, trust me, in our experience, it's a rarity. We really felt that we got our money's worth.

Of course, that's not to say we left her office uplifted. There was much for us to digest and ponder. So here goes.

Med changes

First, she recommended raising one of the two anti-epileptics Chaya's on, Vimpat, by 50 mg/dose. I promptly did that the following night. After two weeks, we raise it in the morning as well. She said the current dose is considered low. Who knew? (And who knew that nabbing those 50mg pills of Vimpat would be so challenging. So, beware, they are in short supply and available only in a handful of Kupat Holim pharmacies).

She also advised doing a blood test to check the level of the second drug Chaya gets, Keppra. Her current dose of that drug - 1,500 mg. twice/day - is also deemed low. Again, news to us. Our previous neurologist either was unaware of that or just kept it a secret. 

In fact, at our last visit she had recommended adding a third anti-epileptic! Frisium! That's one of the benzodiazepams that rendered Chaya semi-comatose during her hospitalization back in November! (As the exclamation points indicate, I haven't yet recovered from that nightmare.)

By the way, that incompetent neurologist never bothered to answer our last email. In it, we mentioned, inter alia, that we had not added the third med, Frisium, as she had instructed us to do. I suppose she wasn't too pleased with that.

Back to the new neurologist...

Video EEG

We've never done one and she'd like us to. So some time in the coming weeks, Chaya will be hospitalized for 24 hours. Here's hoping we glean some helpful revelations. Our Kupah has already given us a hit'hayvut for it.

Chaya's frequent fevers

New neurologist wasn't impressed with the exclusion testing that C.'s pediatrician did. As I've written, he did a thorough clinical exam and blood test before concluding that the fevers Chaya's now getting - they max at 100.6 degrees Fahrenheit rectally - are not caused by an infection. Ergo, they must be central.

New neurologist said that those exams weren't enough to reach that conclusion. She said she's seen patients whose seizures are more numerous than Chaya's but who never get central fevers. She suspects there could be some underlying condition, perhaps related to her life-long thinness, triggering the fevers. She said the pediatrician should have referred us to a specialist - she couldn't specify but tossed out endocrinologist and gastroentereologist as possibilities - who could run further tests. 

"Ouch", I thought when I heard that criticism of our ever-supportive, brilliant and kind pediatrician. But the following day, he read the new neurologist's summary with equanimity, then promptly gave us referrals for a couple more blood tests to tighten the "central fever" theory. He never ceases to amaze us.

VNS

New neurologist strongly urged having a new VNS implanted. The one Chaya sports now dates back to December 1999 and never helped her one iota. She advises trying it again. Of course, this time we'll have somebody better equipped to calibrate it afterwards. Back in the year 2000, there wasn't any such expert in this city. (I know, I know: so why did we implant it then anyway? That was just another of our many blunders.)

But removing this old one isn't easy, we've been told, because the wires are now overgrown with skin - or something along those lines. 

Also, the last neurologist to push a VNS replacement was the one who destroyed Chaya's liver with Valproic Acid and refused to own up to it. Negative associations.

Cannabis

I had braced myself for dismissiveness so I was relieved when new neurologist simply noted that Chaya's CBD dose is very high. Which would explain why the Health Ministry refused to renew our license for 11 bottles (= 100gm) per month. Our new one permits only 10 bottles/month.

She said Chaya's current dose would be more efficacious in the form of vapor which is absorbed far more quickly. I haven't gotten around to inquiring about the availability of this form at our supplier, Tikun Olam. My hunch is we'll need to switch to another supplier (the one that the doctor recommended); not a process I relish.

Hydro

Last week yielded a brief respite from frequent seizures and fevers during an extra long hydrotherapy session where Chaya truly rocked. Here she is:

Sadly, the therapy pool will be closed for the entire coming month. Wishing everybody a G'mar Hatimah Tova and an easy fast on Yom Kippur.

Still in the tunnel, looking for the light

Still grappling with Chaya's liver disease - as yet undiagnosed definitively - and from the persistent pressure sores.

We had a harrowing Saturday night involving an hour and a half of consecutive seizures which had us with phone in hand to call an ambulance. But thank G-d we were able to avoid hospitalization as well as starting that new anti-epileptic, Vimpat. But we do now have a stock of the stuff in our medicine drawer at the ready,

As long as feasible, we'll continue to treat Chaya with CBD oil, three times a day and THC oil whenever the seizures erupt. (Those are the two forms of cannabis oil available.)

Chaya is also getting diuretics to remove the ascites (fluid accumulation) caused by her liver damage, Ridding her of fluid has resulted in an extreme gauntness that shocks us anew every day. Bones and veins that were not meant to be seen by the naked eye are protruding everywhere.

We were also blown away by an email we received a few days ago from the pediatric neurologist who had been treating Chaya for the last five years and who had basically told us to go jump in a lake when we first notified her of Chaya's liver damage 6 weeks ago. This week she wrote a one-line inquiry about Chaya's condition. Neither my husband nor I have yet managed to deal with our disappointment or to figure out what sort of response to write.

And while we struggle through this without any help from "the system", that institutionalization empire, Aleh, churns out its lies in a steady flow of fresh PR releases.

Here's one that caught my eye. I was struck specifically by the repetition of the term "family" to describe Aleh. As if hammering away at that appealing mendacity will make it true.

The Aleh Family - an Unbreakable Bond | ...ALEH is a family, so when a member of the family celebrates an important milestone, it only makes sense that everyone is involved... As the ALEH residents, staff, and volunteers danced with Elisheva and her groom, it was clear that they were a real family.  

We all know what a family is - and no residential institution comes close to fitting the description.

Our new reality. Or: Don't do Depalept!

My daughter Chaya undergoing a battery of tests in hospital last week

Chaya endured several days in the hospital last week and, naturally, so did my husband and I (although he magnanimously volunteered to spend the nights there with zero-to-minimal sleep).

We brought her there after an ultrasound ordered by her pediatrician revealed the condition known as ascites. He'd been concerned by blood tests of the past five months which have consistently included several anomalies including low protein levels.

In hospital, she was drained of 4 litres (that's no typo!) of fluid that had accumulated in her abdomen due to a damaged liver. Tests were done to learn what caused that damage. Theories entertained by our pediatrician along with the new gastro and neurologist, now on the case are:

• The anti-epileptic, valproic acid, known in Israel as Depalept which Chaya has been on for some five years. (And see below for some up-to-minute news about that specific drug.)
• The genetic syndrome, Wilson's Disease (test results for this were all negative unfortunately)

The pediatric neurologist who first prescribed valproic acid and has been monitoring her since, basically dumped Chaya when we sent him the ultrasound results  Here's the email response we received:

"This is beyond the scope of my expertise. You need to talk to your family physician ASAP"

When we also asked that same neurologist whether he had submitted the request to the Ministry of Health for an increase in Chaya's cannabis allotment/month, as he had promised to do a month ago, he wrote us:

"I would stop the cannabis now. There is a problem with her liver and no one knows if this is the cause."

We haven't heard anything further from that MD. I'm still overcome with disillusionment - we thought he cared about Chaya.

And the unanimous view of the hospital staff is noteworthy:

Don't stop the cannabis. It is not the cause of her liver problems, they said  It's the valproic acid that's the likely culprit and she must be weaned off it asap.

Three days later, after receiving IV fluids and albumin, a visit from the hospital's wound specialist and a second one from the gastro specialist, Chaya was released.

She had suffered brand-new blisters/sores on her hands from the plastic tape the staff  used for the IV even though I warned them that she is intolerant of it. Her hand also swelled to three times its size when the IV malfunctioned. (that took two days to recede). And the gastro, though kind and sensitive about it, gave us a bleak prognosis: Chaya is now a chronic liver patient who will need fluid drainage every week or so.

So, what are the morals to be learned?

• First: Beware any abnormal blood test results when your child is on Depalept. Always delve further. Chaya had abnormal blood test results for several years but her neurologist reassured us that everything was fine. (Our pediatrician, on the other hand, was persistently concerned but politely yielded to the  prescribing neurologist's opinion.)
• Second: Try cannabis oil - both CBD and THC - to treat the epilepsy. While Chaya was being drained of the ascites, she began to seize repeatedly, racking up some 15 of them. The ultrasound technician who also inserted the drainage needle pressured me - actually badgered is more accurate - to give Chaya some sort of drug to stop them. Instead, I waited for my husband to bring our THC oil from home. In the past it has halted such attacks and, once again, thank G-d, it worked its magic.

The same neurologist who just dumped us was appalled when we informed him several months ago that we'd easily gotten THC from our government-licensed cannabis supplier and were using it on rare occasions.

"You should have checked first with me. There are no studies results to prove THC to be effective against epilepsy. Only CBD oil." (The reason is that no THC studies have ever been carried out.) 

Our personal experiences with the stuff didn't interest him in the slightest.

And while we grapple with this new reality - a child who is both profoundly disabled and now chronically ill - we learn of yet another new term and concept that  Aleh has concocted to keep government and private donor funding flowing in to its coffers. They've dubbed it the "High Dependency Wing".

It is a puzzling concept. After all, for decades Aleh has been advertising its product as aimed at children with severe disabilities. Naturally, that translates as  "highly dependent". Yet now, suddenly, they announce the planned construction of its first separate wing with that label.

Wasn't the entire enterprise already "High Dependency"?

Here is the way Aleh's PR team touts it:

In Israel today, children and young adults with complex medical conditions requiring advanced nursing care are often hospitalised long term or placed in old-age homes, due to the lack of a better solution within a safe medical environment. These settings offer little or no opportunities for rehabilitation, education, social activity, or the warmth and love of a home. Being confined in this way drastically inhibits the children’s development, and is an agonising and painful option for parents and families.
The solution is to establish high-dependency wards within a rehabilitative environment so that children with complex disabilities and medical conditions can live a more enriching life.

Yikes!  When will our government and society get it: An institution is not a home! A ward is not a home! The services of a paid staff do not constitute "warmth and love".

Now back to the valproic acid update I mentioned above. Minutes before this post "went to press", I was alerted by a MedpageToday email bulletin to a Reuters report [here] dated November 16, 2016 that France has just created a compensation fund for valproic acid victims.

The French parliament voted to create a nationwide compensation fund and amended the 2017 budget bill late on Tuesday to set aside an initial 10 million euros ($10.7 million) for claims relating to Depakine, a brand name for valproate, which is also used to treat bipolar disorder. Parents of those affected say the French state and Sanofi were too slow to warn of the side effects of the drug, which has been used to successfully treat epilepsy since 1967, after the risks to fetuses became clear by the early 1980s... "The 10 million euros are a starting point," Health Minister Marisol Touraine told lawmakers in the lower house of parliament. "In future years, the sums should be much higher." Touraine told parliament later on Wednesday that compensation would ultimately be paid by "those deemed responsible" without giving details. "I regret that Sanofi didn't seek an amicable settlement as a matter of principle. I hope (Sanofi) will change its position," she said... French social affairs inspection agency IGAS estimated earlier this year that between 2006 - when the medicine started carrying a health warning - and 2014 - when prescription rules were further tightened - some 425 to 450 babies suffered congenital birth defects or were still-born due to the drug... ["France sets up fund for Sanofi epilepsy drug victims", Reuters, November 16, 2016]

And that's without factoring in hepatotoxicity (i.e. drug-induced liver injury, acute or chronic ) which is included in the drug's black box warning and which is now making Chaya's life even more of a misery than it already was.

We will undoubtedly be returning to the hospital either tomorrow or Sunday to drain Chaya's fresh accumulation of fluid. Stay tuned for a tally of litres removed this time.

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.

A new law for others and a new oil for Chaya

Israel's parliament, the Knesset [Image Source]

Every so often, Bizchut ("The Israel Human Rights Center for People with Disabilities") pats itself on the back and shares the news with its supporters. Today, the organization considered itself worthy of a fresh pat. The occasion was passage of a new guardianship law:

We are delighted to announce a historic achievement that will impact on 50,000 people in Israel who have a guardian. Supported decision-making, an alternative to guardian promoted by Bizchut during the past two years, has been included in the new guardianship law just approved by the Knesset.  This change was actively supported by a coalition comprising 18 organizations who worked together on the issue and impacted by a Bizchut-led Facebook campaign that reached over 200,000 people. [From a Bizchut e-mailer I received today]

The Bizchut website explains that on March 29, 2016,

the Knesset voted in favour of the Amendment to the Legal Capacity and Guardianship Law. This amendment constitutes a substantial reform to the law and includes a number of dramatic changes: Recognition of supported decision-making; Recognition of lasting power of attorney; Cancellation of the term ‘ward’; Reduction of cases in which a guardian can be appointed to situastions in which this is necessary in order to prevent harm to a person when no less restrictive option is available; Cancellation of the option of appointing a general guardian without detailing the issues under his or her authority; Defining the individual’s wishes as a guiding element in the guardian’s considerations; Defining the rights of persons under guardianship such as the right to receive information from his or her guardian and the right to independence and privacy; Defining the right to legal aid representation in cases of involuntary hospitalization; Limiting the ability of a guardian to force a decision relating to fundamental issues; The new law constitutes a rare opportunity for recognizing the right of every person to legal capacity and to make the decisions that affect their lives...

This is indeed welcome and important news. But it won't affect the thousands of severely and profoundly impaired individuals including my Chaya. For her, full guardianship isn't even at the bottom of her list of worries because she couldn't survive for a moment without it.

While involved in campaigns like the one described above, Bizchut has been neglecting the needs of those like my daughter for whom this law is utterly irrelevant.

Specifically, it has neglected its past, forgotten mission to push for de-institutionalization and a redirection of funding to families caring for their children at home.

As the recent selection of recipients of the 2016 Israel Prize for Lifetime Achievement makes abundantly clear [see my earlier post, "In love with institutions"], this segment of the population with disabilities isn't on anybody's radar in Israel. In the eyes of mainstream NGO's and our government, the solution to their problems is simple: institutions, and the larger the better.

On a happier note, a lengthy CNN segment profiled our largest grower and distributor of medicinal cannabis, Tikun Olam, Another video report ["Video: Israeli boy finds relief through medical marijuana", April 2, 2016 - here] profiled a Haifa couple who - as we do - purchase CBD oil for their son who suffers from intractable epilepsy and severe CP. The couple noted that it supplements the CBD, which their son receives several times/day, with THC oil which it administers only on "bad days.".

Our THC bottle from Tikun Olam

I have been toying with the idea of trying THC but presumed that getting the stuff would involve a daunting string of phone calls and paperwork. But that video piece prompted me to  take the plunge.. I was blown away to learn from Tikun Olam's nurse that all we needed was a doctor's written authorization - and a pediatrician would suffice!.

Now, just three days after watching the video clip online, we are the proud possessors of this bottle of THC.

Today, while Chaya is actually having a "bad day", i.e. having lots of seizures, they are accompanied by her "central fever". This is an inexplicable neurological symptom which occasionally appears on the scene through no fault of any of us. When it does, Advil does the trick. Once the fever is lowered, the seizures taper off.

So I'm saving the THC for a "no-fever-bad-day". And, of course, you, readers, will be the first to hear how Chaya responds to it.