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 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.

Update: Epilepsy medication

We are nearing the end of the 6 mg phase of Fycompa (generic name: perampanel) and approaching the therapeutic dose of 8 mg.

We still haven't located anyone else taking this drug which received its first FDA approval in 2012, for patients over 12 years old. And in 2018, was approved by the FDA for anyone over the age of 4. 

Some of the possible side effects are irrelevant to Haya.

For instance slurred speech; we're actually hoping it does give her some slurred speech!

And deterioration in gait: any sort of gait would be great!

March of the ministers

Image Source: JNS

The parade of politicians paying their respects to Aleh and ADI has been nothing short of disgraceful. 

We saw Yoav Galant, Minister of Education; Yuli Edelstein, Minister of Health; President Reuven Rivlin; Knesset members Haim Katz and Etti Attia trotted out by Doron Almog in recent weeks. They all dutifully parroted the PR team's fulsome praise of Adi - Nahalat Eran.

Not to be outdone by Almog, his ex-partner, Rabbi Yehuda Marmorstein, collared Knesset member Bezalel Smotrich who visited his Bnei Brak branch where he extolled Aleh. (True, he's a low-ranking celeb but Aleh appears to be trailing ADI in every realm of the PR competition. Its websites and Facebook pages are all still out-of-action.)

A logical conclusion after that Ministers' March would be that our government still just doesn't get it. It is incapable of grasping what the rest of the world realized years ago: that institutionalization injures and violates the human rights of individuals with disabilities.

But here is a touch of encouraging news. Our Ministry of Justice has just taken a stance against institutionalization. It is urging residents of institutions and their families to share their tales of woe. The goal is to enable the Ministry's intervention on their behalf.

Bizchut, Israel's Center for the Human Rights of People with Disabilities, has circulated the Ministry's new questionnaire which covers abuse, neglect and other human rights violations.

Here is Bizchut's Whatsapp memo to its supporters (translated from Hebrew by me):

"Residents of institutions and hostels and their families: It is time for you to make your voices heard! You have contacted us - at Bizchut - many times with disturbing accounts of the situations in institutional frameworks. Generally you reach us after failing to receive an appropriate response from the Ministry of Welfare. We have flooded the Ministry of Justice with these difficulties and following our pleas, the Ministry has disseminated a questionnaire aimed at examining how to enhance the protection of people with disabilities living in institutions. 
!! It is important that the maximum number of people log in and portray the conditions in institutions! Please spare a few minutes to answer the questionnaire, and also forward it further...
Please note - there are separate questionnaires for residents (which must be returned by 30/1) and for families (which must be returned by 20/1). All questionnaires are at the bottom of the page in the link.

The version for residents is in simple language and is 20 pages long. Here are a few sample questions:

How would you like to answer the questionnaire?
I would like to answer the questionnaire without writing my name:
I would like to write my name (write your name here):
Which sort of disability do you have? -

mental, developmental or cognitive

emotional (mental health)

autism

physical disability (in movement)

hearing disability (deafness or hearing impaired

visual disability (blindness or serious visual impairment)

I don't want to answer

Other disability (write here which):

And here is how the Ministry described its new effort:

"As part of the work we do in the social cluster of the Advice and Legislative Department of the Ministry of Justice, we are examining ways of improving our involvement, and where necessary the protection of people with disabilities who reside in out-of-home frameworks, with an emphasis on the means of presenting complaints and their investigation."

I am so thankful every day that our Haya has been spared the plight of other Israeli citizens with disabilities who are locked up in large institutions - like Aleh and ADI!

Here (over on the right) is Haya receiving her first Fycompa pill of 6 mg. It only took about ten hours on the phone to various offices of our health fund to finally purchase them. And for no comprehensible reason other than bureaucratic whim! 

Nothing works like a haranguing mom.

Status report: Status epilecticus

I don't normally write in midst of a horrific period for Haya. 

But while I type this, she is in nearing 48 hours of what would undoubtedly be deemed by a hospital team as Status Epilepticus. Basically, that's the epileptic's nightmare - non-stop seizing - and I figured I ought to record it. 

Until this struck us, she had been doing extremely well and was down to only 3-4 seizures a day. I was growing ridiculously optimistic about her new med, Fycompa, already dreaming about her possible future milestones. 

Tomorrow night, her Fycompa dose rises from 4 mg a day to 8, as her neurologist instructed us. That's the therapeutic dose we've been aiming for these past six weeks.

But now I don't have the same high hopes I had for it's success as I did two days ago. And in any case, it will be some time before the higher dose kicks in.

Haya right after a seizure today

UPDATE December 25, 2020 - Friday afternoon 2:30 pm: Her seizures have worsened so we have one foot out the door in the direction of the ER. The memories of our last stay there are still so vivid and horrible. And I know the experience will only be worse now in the midst of our huge Covid wave. 

So before resigning ourselves to the ER, I  am making last-ditch efforts to get her under control at home with paracetamol, Advil and raising her Vimpat back to 100 mg. Insider tip: Avoid those paracetamol suppositories even when swallowing is problematic. They can escape from the rectum quite a while after administering. There's no way of knowing how much was lost so you can't re-administer. It just happened here!

As a last resort, I have prepared our Diazepam rectal tubes that have an expiry date of 2018. I've read the instructions carefully and studied the diagrams. We can't find any new stock in any of the pharmacies here. Haya's pediatrician believes they're probably still potent as drug expiry dates tend to be exaggerated. 

I also called her neurologist who had succinct advice: "Get her to the ER." 

In three days we've gone from counting the number of seizures/day to the number of minutes between seizures! 

Will update - hopefully from home. Meanwhile here is Haya, whacked by her ordeal.

Give up? Never

Haya, yesterday

My daughter Haya, now 25 years old, has just mastered a new sign - one which she devised independently. 

It had me and her speech pathologist snapping away elatedly with our phones to record the "miracle". She's been doing it a bit at the sessions for a while, but less emphatically than she did yesterday. 

Previously, the only sign she had made was pointing her index finger for "yes" and occasionally making a fist for "No". We taught her those signs many years ago. 

But, as I noted, the new one is her own creation and means "give me food." She only uses it when seated in her chair, wearing a bib and awaiting my feeding her. 

Normally, we give her a spoon and she feeds herself so there's no need for this new sign. She only uses it when I'm feeding her - which I only do during the speech therapy session. 

So it's an appropriate and clear sign. Here it is on the right in a photo I snapped yesterday.

Another milestone reached this week was the start of the higher dose of her newest med, Fycompa. She went up from 2 mg. pills to 4 mg. pills for the first time.

I have a request. Please remember these milestones when you next read Aleh's or ADI's disingenuous propaganda about "severely disabled" children requiring institutional care to achieve their "fullest potential". 

I named both organizations - Aleh and ADI - because I am still befuddled as to their identities. Are they connected to each other? Are they separate but co-existing? Are they at war?

 

I have messaged the ADI Facebook page with that query but there's been no response till now. 

Hmmm. Predictable.

D-Day

[Image Source]

To mark the International Day of Persons with Disabilities, our national TV station (KAN 11) is treating us to a series of programs dealing with the topic of disabilities. 

Each one that I've watched was a gem and it was difficult to choose the best for sharing here. Nevertheless, I settled on the two below:

First - 

סליחה על השאלה ילדים | ילדים עם עיוורון | שידורי בכורה ביוטיוב

On YouTube here
 
It's a segment of the program "Sorry for the Question" in which blind children answer questions about their lives.

And -

The documentary Master Class which premiered at the Docaviv Film Fetival of 2017 portrays a workshop conducted by actor/director Arnon Zadok for a group of people labeled cognitively disabled or having special needs who attend a day center operated by Chimes Israel. 

Once a week for two hours, the group attends this acting workshop. It is unique in that everything is done by the participants. They act and film themselves. 

The documentary (see it on YouTube) is comprised solely of their material and focuses on several participants who present moments from their lives, unrealized dreams, fears and concerns. The film describes a world parallel to ours but pours a magical light on the truth.

It demonstrates, yet again, why I'm such an avid fan of Israeli documentary films.

Meanwhile, back at the ranch, we are monitoring Haya closely for her reactions to the med changes we are making for her.

Her neurologist responded within a few hours to my query yesterday. He advised us to return her Vimpat to its previous dosage of 150 mg. We had lowered it to 100 mg. with the goal of gradually removing it entirely.

He advised us to wait with lowering it again until we've reached the highest dose of her new drug, Fycompa.

Confession: We've decided to return to 150 mg only in the morning and remain with 100 mg at night. Haya seems to be seizing a bit less but is more fatigued than usual. That's to be expected with Fycompa, according to the drug info. Her dosage is still the lowest possible, 2 mg. 

So there's a chance that we'll enjoy better times with the goal dosage of 8 mg.

For now, our hopes, bets and prayers are on Fycompa - a drug we'd never even heard of a short two months ago.

More med misery

The medicinal changes Haya is enduring began taking their toll on her some five days ago - many tough seizures throughout the day. 

Occasionally, acetaminophen gives her a few hours of relative calm. And, of course, the swimming pool worked its wonders on her yesterday. She performed there beautifully - see video below.

Haya in the pool with me

But otherwise it's an ordeal for all of us. We're aware that any changes in meds can throw these children off kilter. Yet, on the other hand, this may signal the need to return to her previous regimen and abandon the Fycompa.

I wrote to the neurologist for advice a few hours ago and await his response.

Finally: Fycompa

First Fycompa

Nearly two months after our tele-visit with Haya's new neurologist, we have, at long last, begun to administer the new anti-epileptic that he prescribed. The bureaucracy confronting us before acquiring it was daunting and, for the most part our health fund (HMO) gets the credit for that. 

We have given Haya two doses thus far and are holding our breath as we wait the six weeks to reach therapeutic levels. 

But we have already dropped one of her other meds - Vimpat - from 150 mg. to 100 mg. The aim is to eliminate that one. I have never observed any benefit from it; only gave it due to pressure from another neurologist and will be pleased to have it in our rear view mirror. 

And here's another "finally": a response from one dignitary who visited Aleh. 

Predictably, it was disappointing and wishy washy. To wit:

"I agree with you that every child deserves the best care possible. Ideally this would be provided at home by the people closest to the child like you describe. Yet, I have visited ALEH’s facility in Jerusalem and learned that many of the children ALEH cares for have severe and very complex medical conditions, where such quality care in a home-environment is sometimes very difficult or almost impossible to realize. I have met very committed and professional caretakers and loving families."

Stay tuned for my response to him.

To quote Diana Ross: I'm still waiting

The Diana Ross song is here

Waiting, waiting and waiting some more. Aside from the US presidential election results, we're doing a heck of a lot of other waiting.

1. While she suffers from daily, debilitating seizures, we're waiting to get that new med, Fycompa (generic name: perampanel), for Haya. The neurologist mistakenly sent us on the 29 Gimel track for authorization. It turns out he isn't familiar with the workings of Israel's health funds so he's not to blame. I've been speaking to both our pediatrician - who must write us the script - and the secretary at the health fund office in order to get this process on the right track. For now, the ball isn't even rolling yet. And authorization remains a dream. 
2. I am also still awaiting a response from the Ministry of Welfare regarding the residents of institutions under its supervision. Specifically, what I want to know is in which institutions the victims of Covid-19 lived when they fell ill. Under the Freedom of Information Act, the public is entitled to that. It even has a name: segmentation. I was only given general numbers by the Ministry back in July. To date, the Ministry has only responded to extend its time limit. I've learned that it is entitled do that three times to reach a maximum of 120 days At that point I can proceed to the government ombudsman.
3. Waiting is ongoing for a response from the Ambassador of the Netherlands to Israel who is scheduled to speak at an Aleh event next week. Why would an ambassador from a country where family care is the accepted policy for its children with disabilities promote the removal of children from families in Israel!? I will share his response as soon as it arrives here.

But at least we're not waiting for magnificent hydro sessions for Haya. This week, due to some malfunction, the pool we rent for her therapy was heated to 34 degrees Celsius. That meant we were able to dispense with her wetsuit, allowing for greater freedom of movement. 

Here she is, relaxed and floating skillfully even without the added buoyancy of the wetsuit.

Good job, Haya!

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???