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.

Update: Meals and therapies

We are persevering with the Ketogenic Diet. 

True, we can't be sure that it's helping Haya as we simultaneously resumed her full dose of Vimpat from which we had weaned her. 

Of course, two changes at once is a cardinal sin when treating epilepsy. But since Haya was in a grave crisis only two weeks ago, we agreed with her doctor that every gun in the arsenal should be utilized at once.

We still haven't succeeded in getting the ketone blood test strips that match the device we purchased (photo right). They're due to arrive in a few days. Then when we've ascertained whether or not Haya is in ketosis, we'll be better able to assess its efficacy. 

For now, I can only say, it's a time-consuming and exhausting diet to administer.

This diet has also denied Haya the opportunity to feed herself which she had been doing so nicely for years. Every drop of every pre-weighed Keto meal must reach her gullet. When she fed herself, some of the food dripped or fell. 

So for the time being, self-feeding is banned on dietician's orders.

Haya's keto diet in no way resembles the Google images that pop up for that term, such as this one below. 

Here below is a photo of an actual keto meal I prepared for Haya:

We managed to bring Haya to hydrotherapy again this week. Here she is executing one of her skillful flips. 

She shuts her mouth tight beforehand and instantly gives her head a 360 while I flip her body. When you consider that she does almost nothing else independently, you have to agree it's quite a feat. 

We've suspended the weekly speech pathology sessions which weren't feasible while she was seizing uncontrollably. Now we're also waiting for our Health Fund to respond to our request for subsidization of those therapies. Until 2021 we were included in a pilot project operated and funded by the JDC for two years. That project has now excluded children from Jerusalem for some unknown reason.

Which brings us back to the infuriating conundrum of care for children with disabilities who are institutionalized. It's almost entirely funded by the government. In contrast, when children with disabilities live where they deserve to - at home with loving parents - the government largely abandons them. I hasten to clarify that: the Israeli government abandons them. This isn't the case in other Western countries.

And so institutions like Aleh/ADI continue to pocket lavish public funding. Of course, that's along with generous private donations they savvily solicit. These days, according to ADI's Facebook page, American Christians are also being duped into donating. Just bandy about those vital words "inclusion" or "diversity" and you've won the respect of your target audience.

And just to spice up your fundraising message, dig up an old Barbie Doll news item and dub it Breaking Disability News as ADI's PR wizards do here. 

Note: Those "new" Barbie Dolls - in wheelchair and with a prosthesis - were actually introduced by Mattel two years ago!

To hospital, to hospital... Home again, home again (but no jig)

Home-prepared food for Haya 

 

To market, to market, to buy a fat hen. Home again, home again, jiggety-jig...

Wikipedia

Start with this background: "Haya's update: It's a downer" [February 18, 2021]

I wrote the following this past Sunday night:

At this point, even rectal Diazepam is stumped. It used to knock Haya out for hours. Even an old 2018 dose had that effect a few months ago. Today, the seizures have broken through after an hour. I fear we are approaching the last option - the ER. It's a nightmare we make every effort to avoid. But these seizures are destroying her.

And wrote this on Monday:

Well, we finally succumbed and decided to bring her to the ER. At that point, Murphy's Law kicked in and seizures stopped. She hasn't had one since we placed her in the stroller at home for the trip to the hospital.

And then this on Wednesday:

Needless to say, Haya resumed her Status Epilepticus once we were in the hospital and several med strategies were employed:
Vimpat was resumed, starting with an IV surge of it, followed on Tuesday morning by a return to oral.
She was also given a low IV dose of Valium together with the Vimpat.

Today, the IV meds were stopped. We also learned that blood and urine tests and a chest x-ray eliminated the potential diagnosis of infection.

In addition, the neurologist has recommended adding on the modified Ketogenic Diet from tomorrow. The dietician called me to discuss Haya's food sensitivities (tomatoes) and has dispatched me to locate and purchase flax seed bread, She says it's manufactured by only one company in these parts. With our lockdown over since Sunday, local streets have been jam-packed throughout the day and I don't relish driving around in that traffic, from shop to shop hunting for that bread. And that's not all: If/when I do locate it, the dietician has instructed me to crush it in a blender which she advised me to bring to the hospital! Well, I made it clear that wasn't happening. 

(Gently) If I don't sound enthusiastic about the diet it probably stems from the fact that we tried it when Haya was around three years old and didn't achieve seizure control.

For what it's worth Haya was mostly calm tonight, ate with relish, and lifted her head off the pillow repeatedly. Here she is pointing her index finger to sign "Yes. I'd like to eat" and then opening her mouth even before the spoon reaches her." [Video clip to come]

I'm writing this on Friday and Haya returned home two hours ago.

I will (frantically) attempt to prepare her Keto meals for the first time. I just realized that the toothpaste we use may not be Keto-friendly. So I sent off the ingredients to the dietician for her verdict. My daughter, a pediatric dentist, just told me about a patient on the diet who needed several teeth extracted because his parents brushed his teeth without any toothpaste!

And now it's Saturday night:

The dietician nixed the toothpaste we use for Haya and offered an alternative brand which my daughter, in turn nixed because it's not only sugar-free but fluoride-free too, adding "It's as effective as brushing with water."

That concern is relegated to the the back burner. For now we are focusing on:

• Preparing and weighing meals
• Getting them into Haya without any dripping/spitting out.
• Counting the seizures.

So definitely no jiggetty-jigging yet.

PS The hospital water fountains were all covered up with plastic wrapping.

PSS Every so often when walking in the corridors, shouting staff would alert us "Stand back! Wait where you are! A Corona patient is being wheeled by!"\

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.

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.

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:

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.

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.