Phenytoin is up next

Very excited to share that my daughter C. 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. 

Aside from CBD, this addition will bring C.'s anti-epileptic tally to three. And while I loathe giving her so many at once, as the neurologist emphasized, the cardinal rule is one med change at a time. So, we'll wait until C. has settled into the therapeutic dose of phenytoin and then, the doctor promises me, we'll remove at least one of the others. 

She even asked me which I believe is less effective. I told her that's hard to judge but since Vimpat was added last and there hasn't been an improvement since, I'd like to see the back of that one. 

Truth be told, her other med, Keppra, isn't anything to write home about either.

Hoping to get the script for Phenytoin (marketed as Dilantin in some markets) tomorrow.

UPDATE

Yay, got the script and C. has taken her very first Phenytoin pill.

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

But, I reminded him, those new ones just don't help C.

Here's some Wikipedia input:

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.

Hoping that stellar reputation will prove well deserved!

Voting for the vapors

Last month, we brought my daughter C. back to the epileptologist who first examined her in September 2018.

At that first visit, this  doctor patiently recorded C's history as we related it. She then recommended several new tests and treatment options. We left her office feeling optimistic.

But once we tried to implement her advice, we hit snags. The primary one was our inability to contact her staff. They never returned calls or emails and without coordinating everything with them we couldn't proceed.

I was prepared to just forget about this epileptologist notwithstanding her superb credentials 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 C. As it turned out, the latter proved entirely unnecessary: the doctor didn't so much as look at C., let alone touch or medically examine her - even when she had a huge seizure in the office.

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

I was disappointed and annoyed. What do you all think? Am I quibbling?

This time around, her assistant did contact us a few days after the appointment, as the doctor had promised. She is instructing us on how to switch Cannabis CBD suppliers. This is a complicated process involving reams of paperwork required by the relevant government department.

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

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

But this option was not the epileptologist's first choice. What she plugged most enthusiastically was surgery. She is keen to have C.'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, when we notified her of the liver crisis, washed her hands of us. So, I'm sure you'll understand why I don't harbor any positive feelings for that option.

Back then, we even met with the surgeon himself. He informed us that the surgery would be "complicated" but doable. He too was eager, though he warned us that he would only proceed once C. gained some weight.

Before C. could oblige us that way, liver failure struck and the neurologist who had been touting the VNS surgery, as I mentioned, fled the scene. (After making the preposterous assertion that the liver failure had probably been triggered by the CBD and not by the Valproic Acid!)

Fast forward to last week's visit: I've had a couple of years to mull that surgical option and am far less enamored with it than I was then. C. has been through the liver ordeal and several severe urinary tract infections in the interim. What kind of candidate for surgery does all that make her?

So this time, I pressed hard on the brakes. "Thanks, but no thanks." I told the epileptologist. "C. 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.

Here's hoping those vapors deliver C.'s ravaged brain a bit of respite from the decades of daily seizures she has endured.

New neurology nightmares

Last week, we went to a new neurologist who specializes in epilepsy. Here is C. at the hospital while we wait to be admitted to the doctor's office:

We had to pay out-of-pocket because in the public clinic, the earliest appointment with her was April 2019. But C. is doing so badly seizure-wise that we felt this was urgent.

The doctor gave us an hour and a half during which we covered C.'s history, symptoms and functioning. Afterward the doctor gave us 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 C.'s on: the Vimpat by 50 mg/dose. I already did that tonight. In two weeks we raise it in the morning as well. She said the current dose is considered low. Who knew?

She also advised doing a blood test to check the level of the second drug she gets, Keppra. The current dose of that drug - 1,500 mg twice/day - is also deemed low. Likewise, who knew? Our previous neurologist never told us that. In fact, at our last visit she had recommended adding a third anti-epileptic! Frisium! That's one of the benzodiazepams that rendered C. semi-comatose during her hospitalization back in November 2017 

Btw, that incompetent neurologist never bothered to answer our last email. In it, we mentioned, inter alia, that we did not add 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, C. will be hospitalized for 24 hours. Here's hoping we glean some helpful revelations. Our HMO has already authorized the expense.

C.'s frequent fevers

She 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 she's now getting - they max at 100.6 rectally - are not caused by an infection. Ergo, they must be central.

This neurologist said that those exams weren't enough to reach that conclusion. She said she's seen patients who seize more than C. but 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 threw out endocrinologist and gastroenterologist as possibilities - who could run tests to determine whether in fact there is another cause for the fevers. 

Whew, not exactly an inviting task.

VNS

She strongly urged having a new VNS implanted. The one inside C. 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 straightforward we've been told because the wires are coated by now with skin - or something along those lines. Also, the last neurologist who pushed a VNS replacement was the one who destroyed C.'s liver with Valproic Acid but refused to own up to it. Instead she pointed a finger at the cannabis! Which brings me to this new doctor's take on - 

Cannabis

I had braced myself for negativity so I was relieved when she just told us that C.'s CBD dose is very high. Which would explain why the government authority refused to renew our license for 11 bottles (= 100gm) per month. Instead we were given one for only 10 bottles.

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

Hydro

This week yielded a brief respite from frequent seizures and fevers during an extra long hydro session where C. just rocked. The proof is in the photo above.

The hunt for CBDA

I used to believe that this country is a medicinal cannabis paradise. We've got an abundance of doctors willing to apply on your behalf for a license (and that's a pre-requisite). Provided your ailment is one of the many that the government's health officials recognize as treatable with cannabis your license will arrive within a month. We've got nine suppliers to choose from.

But this week disillusionment struck. After a concerted search for CBDA, we have concluded that it is definitely not sold in this country. Every one of the suppliers I contacted told me that either they'd never heard of it or had but didn't sell it.

What is it?

Cannabidiolic acid (CBDA) is a chemical compound found in the resin glands (trichomes) of raw cannabis plants. In this case, raw means unheated and uncured. Basically, raw cannabis is fresh flower and leaves trimmed directly from the plant. CBDA is the acidic precursor to the more widely known molecule cannabidiol (CBD).] Source: https://www.cibdol.com/blog/368-cbd-science-the-difference-between-cbd-and-cbda

It is readily available on line but a rep of our supplier has warned me that importing it is illegal. She noted that our government-issued user license only permits us to receive the specified products sold by our chosen local supplier.

In any case, it appears that none of the online producers ship to this country. We're still awaiting a response from the ones we've approached.

We're very disappointed by this state of affairs. After consulting our cannabis guru, Elizabeth (https://elizabethaquino.blogspot.com/) and reading further about the product on line, it seems that CBDA is now our best option.

CBD seemed to help C. back in 2015 when we started her on it. The number of daily seizures dropped and she even enjoyed isolated seizure-free days. But that's a faded memory. Her "normal" now is a daily minimum of around 4-5 seizures along with long strings of them on average four times a week.

Elizabeth also advised tweaking the dosage of CBD and THC. We reached the maximum dose of 25 drops of CBD as recommended by our neurologist about 18 months ago. We've remained at that level ever since. But last week I began lowering it. We're currently at 23 drops. I also raised the THC to 4 drops. We give those doses 3x/day.

It's a shame that there is nobody here, neither neurologist nor cannabis supplier, who can guide us more effectively. I'm tired of all this groping in the dark.

Despite her constant seizure assaults, C. managed once again to perform nicely in the pool. Here is a video from her last hydro session a few days ago:

That pot of gold

C. gets hydrotherapy

I was thrilled with C.'s hydro session this week where she surpassed last week's performance. Here she is floating unaided (over on the right).

Her walking has also been advancing. Still nothing like what it was pre-liver saga but better than it has been since her liver recovered.

She's actually sturdily on her feet cooperating slightly while I move her legs. We do this for 45 minutes every night. It's so strenuous that Ive apparently injured my left hand in some way. For the last week, I've been waking up after about five hours of sleep with numbness and pain in my left fingers.

I unearthed a hand brace which I bought many years ago. (That's no mean feat in my chaotic household.) The exercises I was doing back then with C. triggered similar symptoms in the same hand.

Me walking C. today

I've now been wearing it during the walking and while I sleep for a couple of days with amazing results.

UPDATE: I wrote all that a few days ago but things aren't looking so rosy now. We get periods of intense and repeated seizures for which I give either Advil or 20 drops of Cannabis THC, or both. We're also treated periodically to those tiny eye seizures that lock her trembling eyeballs into the left side. The latter aren't as visually alarming as the whoppers but they're still very disconcerting. Needless to say, she doesn't function at her peak while in their grip which can be for over an hour.

We're at the same 25 drops of Cannabis CBD, three times a day, 3 drops of Cannabis THC three times a day, 1,500 mg. of Keppra twice a day and 100 mg of Vimpat twice a day. Still haven't learned if and how we can buy that CBDA. So, for now, I am at a loss as to what changes to make.

That pot of gold - seizure control - seems more elusive than ever.

Rising stress

IV Drip

Nothing uplifting to report about C.

When the hospital doctors tried stopping the IV Dormicum (aka Midazolam), she returned to Status Epilepticus with a vengeance within a couple of hours (one minute between seizures).

So she was rehooked-up immediately to the stuff.

Then today the doctor lowered the dose from 3% to 1% and before our very eyes she began seizing again. So he promptly re-raised it and the seizures disappeared. Not much of a solution.

I inquired about the longer term plan.

He told me their plan was to get her off Dormicum and replace it with an oral benzodiazepine, Frisium (aka Clobazam); raise her Keppra (aka Levetiracetam) dose; stop the Phenytoin; and continue with Vimpat (aka Lacosamide) aiming to raise it from 100 mg twice a day to 200 mg twice a day.

Can't imagine there will be much of C. left on that potent a cocktail.

At the same time he said the team is encouraging continuation of Cannabis CBD and THC.

That's all very nice (I'm referring to the cannabis green light) but there's no point in giving THC if she has already been zapped with the other stuff. How will we know whether it's worked?

And we are currently allotted such a small quantity by the government's medical cannabis agency. We'd need a lot more to administer it freely.

A quick bedside sketch

I'm giving CBD whenever she's sufficiently awake to enable me. But that's rarely.

Well, if any of you would not find the above stressful, please let me know. Because the senior neurologist who dealt with yesterday's setback told the hubby: "Your wife seems very stressed. She reminds me of my mother. I see I can't share all information with her."

Who knew senior neurologists go in for crude mansplaining?

PS: C.'s own neurologist came by today after being away all week. She offered the option of an induced coma which would entail intubation accompanied by the risk of C. never being able to breath independently again. I said thanks but no thanks.

She then warned that even C's current treatment protocol could at some point necessitate intubation. Would we agree to that? Something to ponder.

But, of course, not get stressed over. Perish the thought.

Stuck in status - Part 3

C. and friend in her hospital bed

Written Saturday, November 11

We're still in the hospital, third day running, but no longer in the Emergency Room. Now we're in the ward.

The major seizures are nearly under control and feeding C. meals is much easier. She even seems to have a better appetite than usual.

The severe seizures do still erupt from time to time. We've also noticed some small, new sorts involving only the face or only her right arm and hand.

A neurologist came by today and promised he'd order another EEG to ascertain whether those new movements are actually seizures.

The doctors are piling on the drugs like there's no tomorrow. When I expressed concern about that, the neurologist promised that two of them will be removed as soon as she's stabilized but I'll believe that when I see it.

So C.'s drug tally now stands at: Keppra (also known as Levetiracetam and given IV while in hospital); IV Phenytoin (aka Dilantin) (to replace the IV Valium (Diazepam) which was ineffective); oral Vimpat (aka Lacosamide); Cannabis CBD (Cannabidiol); Advil (aka Ibuprofen) for her central fevers which are proliferating; and Cannabis THC (aka Tetrahydrocannabinol) for strings of seizures without fever.

As you see, the positive news is in short supply.

Uplifted by Downs

Isaac Levya, actor

It's been a while since the last post but not much has happened. Still the daily 3-5 intense seizures which wear me down.

So we finally wrote to the neurologist for guidance, enquiring specifically about starting a daily dose of THC to try and tackle them. (We now thankfully have a precious new bottle of the stuff.)

She recommended that we raise the dose of Keppra by 250 mg/day. But our THC idea didn’t appeal to her. She noted that a colleague who is more knowledgeable about cannabis assured her there is no evidence that THC affects seizures in any way, notwithstanding our anecdotal evidence to the contrary.

Nevertheless, she OKed one drop of it/day maximum.

For now, we’re trying the additional Keppra, so far without success. We’ll give it 2-3 weeks and then return to the previous dose of 2,500 mg./day. Afterwards we’ll try that daily drop of THC. Not that I have any hope a micro-dose like that will help. To zap C.’s long strings of seizures, we give 17 drops.

I’ve been encountering many Downs children lately when I pick up one of my grand-daughters from kindergarten. She attends a semi inclusive kindergarten. That translates into a small group of Downs students joining her “typical” class for a couple of hours each morning. The group then returns to its segregated class for the rest of the day.

I know, I know, that’s just token inclusion and the school could do more. But it’s better than none at all.

I mention this because I’ve noted how longingly I watch the Downs kids. I envisage the rosy life we’d have if only C. had been blessed with Downs instead of the disabilities she’s lumbered with.

That’s quite a switch from the days of my pregnancies when my deepest dread was giving birth to a Downs baby. It seemed the direst scenario possible.

Live and learn.

A propos of Downs Syndrome, I just learned of a film starring a Downs actor, "Any Day Now". As usual, I’m playing catch up – the film was released back in 2012. It hasn’t aired yet on TV in these parts so I’ve only seen clips from it on YouYube. It strikes me as a gem and the many awards it’s won attest to that.

Playing alongside its stars, Alan Cumming and Garret Dillahunt, is a young actor with Downs, Isaac Levya. The film is about a gay couple in the 'seventies trying to adopt a child with Downs Syndrome and the legal battles they face along the way. In an interview [here], Dillahunt describes Levya’s professionalism and how, during filming, Levya would occasionally tell his co-actors: “Give me some quiet; I’m trying to get in character”.

He attends acting school, auditioned for the part and endearingly discusses his acting experiences here.

I live in a place where mothers are still known to abandon Downs newborns in the hospital and where some who do bring them home later institutionalize them. Success stories like Isaac Levya's are particularly uplifting in this environment.

Water and oil

I'm pleased to report that C.'s second Mommy-hydro stint far surpassed her first. She hardly crossed her legs at all which enabled her to center her body and float independently most of the session.

Looking forward to this week's session.

We are still pursuing our quest for eleven (instead of ten) bottles of Cannabis CBD along with one bottle of THC per month.

In response to our request,the government bureaucrats who supervise the issue of medical cannabis have determined that a daily log of every CBD dose administered to C. over the last year is what's missing in their lives.

The recommendations of C.'s neurologist, along with all the requisite paperwork already submitted, just didn't cut it. So we've dutifully supplied them with our log.

A hospital-based neurologist to whom we brought C. years ago now apparently occupies a position of influence over the government's medical cannabis office. Our current neurologist has solicited his intervention.

All we remember about the few visits we made to him with C. years ago is that he routinely asked us "So, what do you think?" and "What would you like to try now?" We never received a suggestion from him. For obvious reasons, we didn't stick with him for long.

Let's hope he'll be quick and obliging now.

Piling on the therapies and the Keppra

It's been a rough three days for C. with seizures up the wazoo, some accompanied by fever, others without. Up to eight major ones a day and many small ones involving only her eyes and mouth (too many to count).

THC didn't work its magic. Nor did Advil when there was a bit of fever so I finally raised the Keppra from 750mg twice a day to 200mg twice a day. The neurologist had advised doing that a few months ago but I put it off, hoping that raising her CBD dose would suffice.

The physiotherapist from our HMO came twice last week and is due for another visit today. He does the same sort of limb-stretching that the other one, hired privately, did only minus the white coat and the almond oil massage, and he does it for 15 minutes instead of one hour. Well, these sessions are totally free so some corner-cutting is to be expected.

C.'s spasticity has become rather serious so we'll also persevere with these stretches on our own throughout the day. So far, no results, though.

At this point, we're postponing starting a new drug recommended by the gastroenterologist to reduce spasticity. You know that cardinal rule: never introduce multiple new med variables simultaneously.

And, after a nine month hiatus, C. is having her first hydrotherapy session today.

I'm hoping she'll function as well, or nearly as well as she used to: floating on her back, kicking her legs slightly, scrupulously keeping her face out of the water. But I have a hunch that won't be the case. Stay tuned for her hydro "report card".

The snapshot above, slightly obscured to protect his privacy, is of our HMO physiotherapist at work with C.

My daughter thinks she's a surfboard

Stiff as a surfboard: Trying to get C. into her wheelchair today

Once again, it's a mixed bag of news regarding my daughter C.

We did fresh blood tests a week ago which showed that her liver has improved and her albumin level is within the normal range, though just barely. Also, without taking any diuretics, she is free of fluid retention anywhere in her body for the first time in years.

(Admittedly, that’s the layman’s take of the Hubby and me. The extremities are easy to assess; the abdomen, less so but we’re both pretty confident it’s all clear).

Another perk of that albumin rise is the dramatic progress in the healing of her pressure sores. I’m already allowing myself to dream of life without daily bandaging. (For those as ignorant of albumin’s significance as I was a few months ago, note: low albumin level means failure of sores to heal.)

The doctors, in particular the gastro, had been convinced that C.’s liver was seriously diseased, possibly with a chronic autoimmune illness. You’d expect them to now backtrack and blame the Valproic Acid for the liver mess – which was their original theory. But they haven't. They just concede that C. is a “complex case” and “a puzzle”. (The pediatrician did mention a viral infection as the possible culprit but didn’t elaborate.)

Presumably at our next visit to the gastro, we’ll hear some firmer hypothesis. For now, we’re just reveling in C.’s rehabilitated liver.

But, as I said, this is a mixed bag so there's been no partying to celebrate all the above. Because, despite those positive developments, C. hasn't been functioning very well. She has resumed feeding herself and we're really thrilled about that. But she has also become extremely stiff and won't bend her knees either to walk or to be seated. In the past, she has had a tendency to lock them periodically but she always loosened up eventually. These days, they are constantly rigid to the umpteenth degree. It takes two-three adults to seat her. And when I try to walk her, she just goosesteps.

Seizures have been more or less under control (around two a day) with a raised Cannabis dosage: 22 drops, 3 times a day. She’s also getting Keppra – 750 mg twice a day and Carnitine 500 mg, 3 times a day.

We continue to enjoy our daily help with caring for C. She’s a fantastic young Australian woman who just earned her BA in chemical engineering. She arrived without any experience in this field but is a quick learner, reliable and very compassionate towards C., which has enabled me to sleep more at night, dote on my grandchildren and, so far, keep that Takotsubo at bay.

C. delivers a few surprises

Heeding those cardiologists who treated my Takotsubo, we've finally, for the very first time,
hired somebody to help us care for C. Somehow, inexplicably, I now have less time to blog. So quite a lot has happened since my last update.

C. has amazed us all - including the doctors - with an unexpected turnaround. While on Prednisone to treat her malfunctioning liver, she took to screaming a lot so the gastro instructed us to wean her off it. Since she'd only been on it for ten days, we can't really attribute her improved liver to it. Nevertheless, for some reason, she is no longer suffering from Ascites, nor from fluid retention in her feet, legs, hands or face. It's uncanny.

There's a slim possibility that the L-Carnitine which the gastro prescribed two weeks ago deserves some credit for the change. It's a supplement that sometimes benefits the liver. But after two weeks? I wouldn't bet on it.

Presumably, this is why the gastro/liver specialist now believes that her liver has been repairing itself. Does this mean he has ditched his diagnosis of Chronic Autoimmune Hepatitis? Is he now convinced that the Valproic Acid - which we weaned C. off as soon as we learned that her liver was damaged - was the culprit after all? Perhaps. (If he does, then he and I are on the same page.)

We'll find out at our next appointment. Details like that don't really interest us know. We're just revelling in the new reality.

Along with the liver improvement, we've seen an equally surprising drop in seizure activity. That's happening with Keppra at 750 mg twice/day and Cannabis raised from 19 drops 3 times a day to 20 drops. We've finally been able to raise C.'s Cannabis dosage because the government just issued C. a new license with the increased dosage that her neurologist requested.

On the functioning front, C. has also astounded us. She has gradually resumed placing the spoon in her mouth to feed herself; today she performed that feat at her peak. She's also been standing and walking somewhat better than she has over the last two months - albeit while screaming quite a bit of the time. Protesting an activity she doesn't like is progress for C. So we're not complaining about that.

Of course, we aren't deluding ourselves. This isn't our first time down the road of miraculous improvement and we're well aware it can lead right back to deterioration and despair.

But for now, we're savoring the respite, the calm and the sweet taste of progress.

Our mammoth meds mess

We are deeply submerged in a morass of horrific seizures, new meds that aren't working and may be harming, deterioration in functioning and doctors whom we like and trust but who have no firm convictions, only wild guesses.

The silver lining is that at least they are humble enough to admit that to us.

My daughter C. has been suffering bouts of severe seizures on a daily basis now which we can only halt with some 13 drops of Cannabis THC. That in turn zombies her out, so there's no food or fluid for a while afterwards.

Keppra is the only anti-epileptic she is currently getting. It replaces the Valproic Acid she had been on for about two years. We had to wean her off the VA because doctors suspected it either caused or exacerbated the liver damage we discovered some six months ago. (The jury is still out on that point.)

The liver damage was uncovered when we tested C.'s blood albumin levels and found them to be much lower than normal. We had tested for albumin because the half dozen pressure sores scattered over her body hadn't been healing for several months.

C.'s damaged liver triggered Ascites in her abdomen and extremities, So after drainage in the hospital - and removal of 4 litres of abdominal fluid - she was placed on daily doses of two diuretics.

After a month of that regimen, C. had grown frighteningly gaunt. Her bones and veins protruded, her cheeks were sunken and she looked about ten years older. A parade of dietitians was consulted but none could figure out where all that wholesome and calorie rich food I was feeding C. was vanishing.

Blood tests finally showed high levels of saline, indicating extreme dehydration which was fingered and blamed for her gauntness. Here's how the dietitian explained it to me: Every cell in the body contains fluid. When there's dehydration, they all shrivel up just like a dried out plant.

So we had been shrinking C. with our very own hands. The liver expert ordered the diuretics halted immediately.

We had reluctantly started Vimpat for C.'s seizures since the Cannabis CBD alone wasn't doing the trick. But one day into that, she went into status epilepticus which even THC couldn't tackle. So it was off to the ER again.

There the neurologist ordered Keppra for the seizures (replacing the Vimpat) and the liver diseases guy chose Prednisone for the liver.

That brings us full-circle back to our current morass which we reported to both the gastro/liver doctor and the neurologist.

Their responses were the following changes:

• Wean C. off the Prednisone and replace it with a different steroid
• Increase the Keppra dose from 500mg 2x/day to 750 mg 2x/day and then, perhaps, to 1,000mg.

To view the video

For some reason we can't fathom, the gastro doctor has shelved the liver biopsy idea, leaving us in the dark about the source of C.'s liver issues. (In his email conveying this decision, the doctor factored in my recent Takotsubo event. Huh??)

Since the gastro doctor prescribed L-Carnitine, I've been reading a lot about it and see it's quite trendy. Have any of you seen impressive results from it in your children?

I stumbled across a sobering clip [on this page] after writing the above update and am reconsidering: We may actually be living on easy street.

Takotsubo 101

My body finaĺly managed to stem that downward spiral I was in. Overwhelmed by preparations for my own surgery (to correct severe POPS) along with my daughter C.'s deterioration, I sensed impending implosion - the emotional sort. (My conversations were peppered with "I can't go on", "I'm going to have a breakdown", "I need help".) Certainly nothing that would land me in hospital.

But Friday night, I developed weird symptoms - extreme weakness, nausea, vomiting and low blood pressure - and reluctantly got into an ambulance. When the blood test results arrived and the ER doctor declared "You've had a cardiac event", I was shocked, distressed, blown away and frightened. Something along the lines of virus/dehydration was what I'd expected.

"When could I possibly have had one?" I asked him.

After an angiogram which, thank heavens, revealed clear arteries, the doctors settled on a probable diagnosis of Takotsubo Syndrome.

If you ever feel compelled to develop a heart problem, this is definitely the way to go. But, sorry guys, it's overwhelmingly a post-menopausal women's option.

For those of you caregivers desperate for respite, you can see some more info via Wikipedia about this relatively newly categorized (since 1998 in the West) cardiac syndrome. 

And for you over-stressed guys, we'll see what the Hubby digs up. He's been single-handedly caring for both C. and home as well as visiting me every day. A perfect Takotsuba candidate were it not for his gender.

In the meantime C., treated only with CBD, is still keeping her seizure rate low - but don't forget that's top secret!

The Hubby also succeeded in getting a great sodium and potassium reading in C.'s latest blood test on Sunday so her dehydration is history. And while she's definitely not out of the gaunt range yet, the thin jawbone vein that had been bulging is no longer visible.

I'm looking forward to getting her back on her feet and feeding herself again - the two activities that have been omitted from her routine during my absence. I'm also struggling to get some home help from "the system" but so far only encountering voice messages.

Takotsuba is apparently a recurrent event in many cases - must somehow alter the reality here to prevent that.

This sketch of C. (above) helped me while away the hours in hospital.

Doc disses the new drug (THC)

This week brought three "perfect bad days" so we finally cracked open our bottle of THC.

On the first, I gave one drop, waited a few hours, observed no adverse effects, saw another couple of seizures and gave a second drop. Still no adverse effects and seizures stopped.

The following day, C. had a string of severe seizures (or so they were described over the phone by the school nurse). But we were too far away to race over there (at the eye surgeon to plan cataract surgery). So I just advised giving Advil even though it wasn't obvious she was feverish. Eventually, after missing her hydrotherapy because of them, the seizures abated. (so I was told).

When she seized twice at home that evening, and with the bad morning in mind, I gave her one drop of THC. A few hours later, after another seizure, I gave a second drop.

This seemed to render her tired and floppy. She did manage to do her MEDEK-walking for 40 minutes. But she was incredibly and unusually loose-limbed which I attribute to the THC. Not at all terrible as side effects go.

The following day, after an awful string of seizures (approximately eight), I gave three THC drops at once and after just one more drop, they abated.

C, waiting to see the
neurologist. She was
having a very bad day

Yesterday, again a horrific seizure tsunami (around ten), I gave her four drops and again, after one last seizure - calm! Yes, limp, perhaps lethargic, but seizure-free until the evening when she just had one.

Right after the awful morning, which also resulted in a bitten and bloody tongue - as her clothing attests (see photo) - we saw the neurologist.

I handed her the boxes of CBD and THC oil for her perusal and - wham! She blew a gasket. In her gentle and restrained manner, she rebuked me for ordering and administering the THC oil without first consulting her. I explained that I had expected to do that but when the cannabis nurse said the pediatrician would suffice, I opted for that. With our scheduled visit only a week away, I figured I'd notify her, the neurologist, then.

"You could have emailed me first," she noted        .

I didn't want to quibble but the truth is she often takes ages to respond to emails and I wanted that THC asap. Nonetheless, I apologized and promised to halt it after she emphasized that there's no evidence of its efficacy in treating epilepsy. (What choice did I have? We've been through many awful neurologists and don't want to risk losing this one).

She is particularly opposed to using THC with children but when she remembered that C. turns 21 next week, conceded that it isn't a concern here.*

C's VNS implant

The neurologist quickly recovered from her THC meltdown and was her kind and supportive self for the remainder. At my suggestion, she agreed to apply to the Ministry of Health for a new license citing a higher dose. We are still far from the maximum, she said. She also convinced us to submit C. to a second VNS implantation in the near future. More about that next time.

In the meantime, here's a glimpse of the old one.

---
* Once out of the office, the Hubby and I agreed we'd continue giving THC in future tsunami circumstances. It's clear to us it works.

Cannabis Chapter 2: We're starting with THC oil

Video still from the CNN clip: An Israeli father gives his
son THC oil [Video Source]

After reading about it, thinking about it, talking about it and blogging about it, I watched a CNN video clip about it: that is, THC oil used in conjunction with CBD oil.

The clip [online here] finally spurred me to call our Cannabis supplier's nurse to inquire about the bureaucracy involved in getting THC. Mind-blowing answer: no requirements.

As Murphy's Law would dictate, to get the CBD oil, which is low in THC and can't produce a high, you need a neurologist who has treated your child for an extended period already and who is willing to submit the demanding Ministry of Health paperwork. Then you must wait a month to, hopefully, receive the license which states precisely the amount your child is permitted to receive per month. That license must be produced every time you purchase the CBD oil. If you raise the dosage to a point where the stated amount won't suffice, the neurologist must re-submit all those documents to apply for a brand new license.

But for the THC oil which does induce highs and possible other effects, you need only text a doctor's recommendation to the Cannabis nurse. Any MD at all will suffice. Even your garden variety family doctor, even one who has never met your child. Anybody who'll oblige you! (Provided, of course, that you already have a license for CBD oil.)

And thus, within 48 hours of watching that CNN segment, we had ourselves a bottle of THC along with these precise phone  instructions from the nurse:

• Start with one drop.
• Watch for reactions. 
• Raise the dosage as required. 

I kid you not.

I haven't given C. any yet, but am very eager to. The family profiled on the CNN clip, whose child appeared to be as severely epileptic and CP-disabled as C. is, said they use it only on "bad days". So I'm eagerly awaiting a "bad day" that isn't caused by C.'s periodic neurological fevers which we zap with a couple of Advils. I'll keep you posted.

And how about this? Our Ministry of Education will be granting its most prestigious annual award in 2016 to a man who founded one of our largest closed institutions for children and young adults with severe disabilities.  I feel as though we are locked in a time machine hurtling back to the Middle Ages.

EEG enigma and dosing in the dark

Last week was our quarterly visit to the neurologist, but my cataract and dental issues left no time to blog. I'm glad to have a few minutes to share what she told us.

The visit began with C.'s first EEG in several years. She was so calm that an anesthetic was unnecessary. She even stopped grinding her teeth after I stroked her cheeks. The technician asked me to intervene because the grinding made the EEG go haywire. After that it was smooth sailing. C. even fell asleep.

To my layman's eyes, the lines on the screen looked a lot less zig-zaggy than in the past. But that may just be wishful thinking at work. The neurologist herself explained that the zig-zags definitely indicate epileptic activity but without seeing her previous EEGs - something she had no time to dig up - she couldn't comment on improvement. Hopefully she will eventually compare them (and confirm my rosy assessment).

She then gave the green light to raise the cannabis dosage by another 50% of her current dose. So we can go up from her current 46 drops/day to 65 drops/day and then stop raising it. That final dosage will only be a moderate one. The doctor doesn't recommend reaching the maximum. She says the dearth of reliable studies leaves cannabis dosing in uncharted territory. A higher dose might even be detrimental.

So here are a few numbers to crunch:
- C.'s oil contain 15% CBD and 0.75% THC which equals a 20:1 ratio. 

- Her 45 drops/day equal 7 mg per kg. 

- We aim to raise her to 65 drops/day or 10 mg per kg 

I'll keep you posted as to her progress. 

At this point, in addition to a reduction in the number of seizures on most days, I have noticed an improvement in her walking. (It's the MEDEK assisted sort.) She seems to stand straighter and takes bigger steps with her right leg. The left does not step independently. 

But I still crave a touch of cognitive progress. No headline-grabbers; I'm too realistic for that. Just a few new, minor, non-verbal reactions from C. would do me fine. 

And while on that topic, we still haven't heard from the neurologist who promised to canvass his colleagues about minocycline as a treatment for cognitive impairment. We emailed him a reminder today. I didn't ask the neurologist we saw last week about the drug because she's an epileptologist, so I figured this subject is not her field. 

Other brief updates:

Our grand-daughter's aplasia cutis was officially declared history by the neurosurgeon this week. No more bandaging and no follow up visits. We are one very thankful family this week.

My article about institutionalization of people with disabilities - sadly, it still thrives here - was published last week. After its umpteen revisions, the piece didn't feel like 'my baby' anymore so I wasn't quite thrilled. But at least the message it conveyed is circulating. Today's tally is 75 tweets and 135 recommends

A day-long saga

That's me walking C. for the PT

Last Wednesday was a long, exhausting day revolving entirely around C. So here is a long, exhausting post about it.

The first half of the day was spent at a rehabilitative center for C.’s annual multi-disciplinary assessment by a  physiotherapist, an occupational therapist, a social worker, a neurologist and an orthopedist.

In previous years, our health fund footed the bill but warned us after last year’s visit that they would no longer do so. Nonetheless, we tried our luck this year again. Since C. is now an adult, the decision was no longer in the hands of that same health fund pediatric neurologist who has been the bane of parents’ lives throughout this city for decades. Many children have been denied therapies or specialists’ exams by this health fund thanks to this s.o.b. So many in fact that TV and newspaper exposes about him  have abounded  - but to no avail. 

In any case, our approval for funding this year arrived without a hitch.

Now we don’t usually hear much that’s earth-shattering  from the team at this center but the visit always proves worthwhile in some way.

The neurologist, reminded us that we had told him C. seizes 3-4 times a day. Since we used to count only the ones she had awake and out of bed, that tally reinforces our conviction that C. has improved on cannabis. We’re now giving her 14 drops, 3 times/day of the 20:1 ratio, a.k.a. 15% of CBD and 0.75% of THC. (Why these percentages translate into a 20:1 ratio is something I’ve got to ask the nurse next time we speak.)

I mentioned to the neurologist the research into minocycline as a treatment for cognitive impairment. He was totally unaware of it and promised to inquire about it among his colleagues. I’m eager to hear what information he culls so we can start C. on the drug asap.

The physical therapist was impressed with C.’s MEDEK walking which we demonstrated for her and in particular with her straight torso. (Photo on the right.) But she noticed that C. barely lifts her right foot when stepping. Instead, she drags her toes on the floor. She thought a brace might help (she currently only wears one on the left foot) But in order to determine whether it would actually be beneficial, she recommended first trying the cheaper "Dictus" (video here)

I’m reluctant to try either of these. From my experience, C.’s walking doesn’t take well to even the slightest adjustments. So I fear introducing either device would do more harm than good. But I plan to try the Dictus just to be sure. 

The occupational therapist referred us to an article about restraining the strong limb in order to strengthen a weak one. I have limited the work I do with C. to her right and stronger hand/arm, ignoring her weaker left one entirely. This therapist urged me to focus more on her left since it might improve her brain/cognitive function. The words “cognitive improvement” are like light to a moth for me. So I intend to give C.'s left hand some attention from now on while holding down her right.

The orthopedist scrutinized C.’s overlapping toe for an inordinate amount of time and actually concluded that she’s a candidate for surgery to repair it! Since she walks a maximum of one hour per day and only with constant support, this seemed like overkill and we told him that. So he drew another solution out of his bag of tricks: bringing C. to an orthotics expert who will make a custom “toe separator”.

I might add that the standard silicone insert (photo on the right) I’d tried on this doctor’s advice last year produced sores. I can assure that expedition is nowhere near the top of our “to-do” list.

I had to remind the orthopedist to assess C.’s scoliosis which he promptly did by glancing at her position in the wheelchair. “No need for any intervention” was his verdict.

Needless to say, I wouldn’t write home about this orthopedist  

The social worker, poor woman, was subjected to one of my rants about C.’s school and about the limited options available for children with profound disabilities after “graduating” at 21. She listened patiently, commiserated and if. she found me tiresome – like my immediate family tends to - then she did a great job of hiding it.

Following my tirade she advised having our pediatrician send a referral note to the home-care advisory unit of our health fund. They’ll assess our home for accessibility and might offer to fund some minor improvements.

After this we headed to another city, with C., to pick up her two bonus bottles of cannabis. Our always-supportive neurologist arranged for a new license from the Ministry of Health. This one permits us 30 grams per month which translates into 6 bottles instead of the previous 4.

As we approached that city, the hubby realized that we had left our license at home. The distributor insists on the presentation of that piece of paper before each hand-over even though they’ve got everything on line. We knew they’d give us a hard time but figured they’d ultimately hand the bottles over.

Well, we figured wrong.

The guy who runs it did give me a hard time – cannabis wafting  from his every pore. But, never losing his cool, he would not budge. My kvetches and pleas fell on deaf ears (he was very cannabis-cool). The only way he’d part with those bottles was if I presented an official police declaration that I’d “lost” the license. He kindly wrote down the address of the nearest precinct.

The chance of my getting such a document seemed slim to nil and would undoubtedly be a lengthy process. But the alternative was driving back the following day, along with C. (mandatory, since hers is the sole name appearing on the license) and with the license. All told, about four hours total on the road. By this time the hubby and I were deep into the blame game. We had by then picked up our dental-student daughter from her campus and it was “less than pleasant” in the car.

To make this already very long story shorter: the policeman at the front desk whisked out the desired document before I’d even finished my sob story and filled it in according to my dictation. I was out of there with the signed and stamped piece of paper in three minutes. 

But this joke of an “official” declaration satisfied  the distributors. Within seconds the liquid gold was in my hands.

Like I’ve said: when it comes to cannabis here, we’re practically neck and neck with Colorado.