Drugs and hope: An update

Haya in the hydro pool last week

I thought a med update for Haya would be appropriate, so here goes.

She has been entirely off Keppra for nearly one week. She had been receiving 1,500 mg of it twice/day for several years. 

Her neurologist's instructions were to reduce her dosage by 250 mg. every 2 weeks. She admitted the move was a shot in the dark - just "something to try". But since it's known that epileptics can become inured to drugs after taking them for long periods, I embraced it. 

One less med sounded appealing.

I adhered to the weaning regimen, more or less, at times extending the two weeks by a few days. But I weaned her off the last 250 mg much more slowly. 

First I removed the nighttime 250 mg. and then two weeks later, removed the morning 250 mg.

Whew. It was a huge relief to see no repercussions. 

The last med we weaned her off successfully was cannabis But about two years before that we made an attempt at removing Vimpat. It triggered status epilepticus and a hospitalization.

So for now, Haya is only two anti-epileptics: Fycompa and Vimpat. Quite an achievement!

The new med

Here she is (above, right) having her first hydro session minus Keppra:

And while I'm aware of that birthday promise I made to opt for realism over optimism, I can't help hoping that deleting Keppra will bring some minor improvement. Not fussy about that - any shred of heightened functioning would do us.

Alongside the Keppra weaning we've introduced the new drug that the neurologist recommended to treat her kidney stones. It's Urocit-K and she gets one pill 3 times/day.

If those stones were painful, and I understand they tend to be, then I hope Haya is enjoying some relief already.

We can only hope and guess.

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:

Next Up: Phenytoin!

Haya's first dose of the new med last night

Very excited to share that Haya will finally try out a new anti epileptic - one that has been found successful in a significant percentage of EIEE11 cases. It's the first med change we're making since her diagnosis with that syndrome three months ago (see "At last - a pretty solid diagnosis")

I loathe giving her three anti-epileptics at once. But as the neurologist emphasized, the cardinal rule is one med change at a time. Once Haya's settled into the therapeutic dose of Phenytoin (also sold as Dilantin and Phenytek), the doctor promises to remove at least one of the others. 

She even asked me whether I thought either of them is effective. I told her I couldn't judge but did remember which was added last, Vimpat (lacosamide), and didn't think it had made a difference. 

The truth is, her other med, Keppra (levetiracetam), isn't anything to write home about either. 

I got the script for Phenytoin yesterday. And the photo above shows Haya getting her very first Phenytoin pill sold here as Epanutin.

When Haya's pediatrician heard that this was the drug selected by the neurologist, he was surprised. "When I was doing my residency, back in prehistoric days", he recalled, "we had only two anti-epileptics to administer: phenobarbitol and phenytoin. There are so many new ones on the market now." 

But those new ones just don't help Haya. Wikipedia says:

Phenytoin was first made in 1908 by the German chemist Heinrich Biltz and found useful for seizures in 1936. It is on the World Health Organization's List of Essential Medicines, the most effective and safe medicines needed in a health system.

Here's hoping that stellar reputation will prove well deserved!

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.

Mastering the art of gripping and dropping

Occupational therapy sessions entered Chaya's life about two months ago.

Scheduling them isn't easy because both Elvie, her caregiver, and I want to be present and Chaya must be seizure-free. 

We've already had one fiasco - Chaya in the throes of a string of seizures when the therapist arrived. (I had called to notify her but she was already on her way). 

But her other sessions were rather productive ones. 

Over on the right is C. getting her ligaments gently stretched at the session's start to increase her range of movement.

And below is a photo of her gripping an object while our therapist, Neria, shakes her hand. She is improving at closing her hand to grasp an object and then, on demand, opening her hand to release it. 

Modest goals, but they elicit bravos from all three of us - the therapist, C.'s caregiver, Elvie and me. 

You've got to admit, we're a realistic bunch.

We had about two weeks of relative relief from seizures (i.e. about three a day) which I attributed to the rise in the night-time Vimpat dose. But here we are after raising the morning dose too and the seizures are back with a vengeance. 

We still haven't managed to check the Keppra blood levels. So maybe therein lies a solution.

The Kupat Holim (health fund) nurse tells us that test will only be available after Sukkot.

No hydro photos because the therapy pool is also closed until after the chagim (the Jewish festival season).

Finally, we are excitedly awaiting delivery of Chaya's new wheelchair on October 8.

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.

Urinary Tract Infection - Take Two

A fortnight ago, Chaya treated us to some more spikey fevers. So it was off to the pediatrician again. While in the waiting room, I snapped a shot of a painting I did and which I gave the doctor many years ago in gratitude for his unflagging devotion to Chaya. He's hung it right next to a Mary Cassat. His generosity know no bounds.

But we could have avoided that trek to his clinic since he suspected it was a return of C.'s Proteus Mirabilis (a version of UTI - urinary tract infection) as soon as he heard she had high fevers without any apparent symptoms. I wrote about it here: Mea culpa.

But when I called her in advance of bringing Chaya to her, the nurse at our Kupah clinic was extremely reluctant to take a second catheter-urine sample. She had done the one preceding the first round of Proteus M. and it was smooth going. 

We now had an explicit written request from Chaya's doctor that she repeat it. But she expounded on the risk of catheter-introduced infection and how those fevers could be from aspirational pneumonia (even though I assured her she had no related symptoms. She stressed that a physical exam should be done first. 

I wasn't a match for her. But when I related all that to our perenially sweet doctor, he was truly pissed off. "I hate to pull rank, but..." was his response.

Anyway, a long story short, as I said, Chaya was thoroughly examined and no symptoms were found. The following day,  the nurse took up her catheter for us. 

Chaya has now completed her course of Zinnat. The new challenge is preventing another recurrence. A few of the doctor's tips: Don't let her become constipated (who knew that could trigger a UTI?);  keep her well hydrated; give her cranberry juice.

We have a neurologist's appointment lined up for this week which Arnold arranged. We'll probably go without Chaya just to discuss the meds she's on. Since they aren't delivering any more control than we had prior to her hospitalization back in December 2017 (for status epilepticus), I am keen to lower the Keppra and remove the Vimpat. That was her pre-hospitalization regimen.

I am dreading this encounter with the neurologist because she was one of the doctors in the hospital back in December who bombarded Chaya with meds to the point of semi-consciousness and was prepared to release her in that state. The hitch is, though, that we can only access the wise neurologist who saved her from all those meds via this neurologist. The wise one doesn't treat patients outside the hospital ward.

And who would have imagined that the ubiquitous but unnoticed straw would one day become a contentious item? And central to the fight for disability rights too? Well, that day has arrived. The issues are explained here.

Thinking of Canada

Chaya was rushed to hospital by ambulance this week
[Illustrative image from Shaarei Zedek's website]

We have been weathering an overwhelmingly difficult period with our youngest child, Chaya.

Then, just to stir things up a bit, my heart decided to "break" on me: I was hospitalized with Broken Heart Syndrome, a.k.a. Takotsubo Syndrome a.k.a. Stress Cardiomyopathy.

But after I’d returned home, thank G-d on the mend, Chaya decided to spice things up again with uncontrolled seizures rendering her unable to swallow either food or water. My husband rushed with her by ambulance to the ER. Throughout the 24 hours that Chaya spent in the aisles of the ER, my husband sat glued to a chair beside her.

After receiving IV fluids, she was launched on two new meds which we hope will improve her seizure and liver problems – Keppra and Prednisone. Perhaps they’ll also enable her to retrieve those minimal skills she’d acquired over many years – feeding herself and walking with assistance – which have entirely evaporated in the past week.

Our quest for some help at home is dragging on so we are still caring for Chaya ourselves while I try to sandwich in a bit of  recovery from my unexpected coronary event. “The system” (i.e. Ministry of Welfare and National Insturance) washes its hands of parents who opt to keep their children at home despite disability and illness (as I described in "Aleh 101" a year ago). “How dare you reject institutions like Aleh!” is basically the message.

Thus it was with particular disappointment that I encountered yet another PR plug for Aleh's chain of institutions. Dated December 23, 2016, it appeared in the local daily, Yisrael Hayom, a known mouthpiece for Prime Minister Netanyahu. Purportedly penned by a Canadian pediatrician, Dr. Lynn Hierlihy, it spouted the customary Aleh hype. But because it was a medical professional this time advocating the institutionalization of our most needy children, I was especially incensed.

Her blog post [here] first sang the praises of volunteering:

Volunteers are critical partners in the growth and development of society... And while these individuals are no doubt selfless, they will often admit to a single "selfish" motive: Volunteering makes them feel incredible. Numerous scientific studies have proved that volunteering reduces stress and improves physical and mental health.

She elaborated on the topic and I have no gripes about that. True, I have rarely found volunteers very helpful in caring for Chaya and much prefer professional input for people with profound disabilities. But, fair enough, she made an innocent point.

Afterwards, though, she segued to a florid profile of Aleh. I would have expected a pediatrician to appreciate the crucial role that a family and its love play in a child’s healthy development. And I mean all children – including those with disabilities. But apparently this one doesn’t.  To hear one instead extolling the virtues of closed institutions is baffling.

Here is her paean to Aleh:

Needless to say, our visit to ALEH Negev-Nahalat Eran was magical. Never before had I seen a rehabilitation or long-term care facility that so clearly exemplified how "the world is built on loving kindness." I fell in love with the children and their dedicated caretakers, and I embarked on a mission to raise awareness about ALEH upon my return home. I now do everything in my power to connect our community in Canada to the ALEH family. When my son celebrated his bar mitzvah, he requested that guests make donations to ALEH in lieu of gifts. Our synagogue has established an ongoing relationship with the organization, which I coordinate, to ensure that we continually give back to our newfound Israeli family in any way we can. And I take every opportunity to visit ALEH and lend a hand.

Dr. Hierlihy’s blog post is particularly confusing coming as it does from an Ontarian. Canada deinstitutionalized its care for people with disabilities several years ago. In the Spring 2009 (Vol. 28, No. 1) edition of  the Canadian Journal of Community Mental Health [here], we read that:

Ontario has recently closed its last 3 institutions for persons with developmental disabilities. Very little research has been conducted on Canadian deinstitutionalization projects, and the impacts and bona fides of such endeavours have not been well documented in Canada. However, the closing of institutions has occurred in most Western jurisdictions and has been the subject of much research in Australia, the United Kingdom, and the United States. Although community services are of variable quality, this literature review suggests that the Ontario plan to close institutional facilities in favour of community-based residential services will be of general benefit to former institutional residents.

That CJCMH article concluded that

Very simply, the institution cannot replace the community in providing individuals—including those with developmental and serious psychiatric disabilities—with the opportunities for the good life. There are no compelling client-related arguments left for keeping people with cognitive limitations, and possibly people with psychiatric disabilities, away from their families and communities.

Don’t Israelis with disabilities deserve the same compassion, the same dignity, the same loving care within their family and community as do Canadians, Dr. Hierlihy?