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.

Fever, stress, vomit. Life.

True, we might have gotten bored with the same old, same old. I mean profound developmental impairment both physical and cognitive, central fevers, cortical blindness and refractory epilepsy. They might not have kept us on our toes. So, it's understandable that we've now had retching and vomitting added to the mix.

It's been nearly two weeks since those new symptoms debuted. Last week's exam by the pediatrician didn't explain them so we did blood tests on Monday. We also consulted by email with the neurologist..

The pediatrician - who has kindly acquiesced to keeping C. on despite her nearly 23 years - thought that perhaps one of C.'s anti-epileptics is the culprit. The one added on last is Vimpat/Lacosamide. I recall that the neurologist who saved her from a near-coma during her last hospitalization said then that her Vimpat dosage was too high. But he ultimately left that unchanged because she recovered just by his removal of several other drugs that the hospital team had been administering her.

But the neurologist treating C. now - a member of the above hospital team - dismisses that theory.

The Hubby and E., the caregiver, are convinced that phlegm is to blame for the vomiting. But I'm not. She just shows no signs of phlegm, doesn't have a runny nose and isn't coughing. Besides, she's never vomited from a cold in the past.

We also emailed the gastro who had prescribed Carnitine to repair her liver when it was damaged by the Valproic Acid medication. She's been taking 250 mg. of it three times a day for over a year. Then this past weekend we went away for 24 hours with the entire family. I forgot to pack in the Carnitine and C. did not seize, vomit or retch the entire time.

So I hoped we'd found the cause. But now, after receiving the gastro's OK to stop carnitine, it's clear that was wishful thinking.

So the mystery of the vomiting remains.

C.'s Central Fevers have also increased in frequency and are stumping everybody as well. Her neurologists don't seem to have experience with them at all. In fact, while C. was last hospitalized one of the doctors posited that we have it all back to front: the seizures are inducing the fever.

Now, that's such a presposterous hypothesis, I can't decide where to begin debunking it. One, C. also has seizures without fever. Two, when she has a fever with seizures, they stop once the fever is lowered. Three, central fevers are a known phenomenon. I didn't invent them. But nobody on the team was listening.

I'm hoping that our neurologist will be able to track down a doctor who is familiar with central fevers. We plan to meet up with her for a face-to-face consult next week, something I'm not eager to do since the last time we spoke, in the hospital, I sensed she wasn't enamored with my ideas. (They tended toward pleading for lowering the meds, asap).

In the meantime, it's just stress, stress and more stress. Here's a self-portrait in the works that illustrates that.

A healthy liver doesn't cut it

Illustration: Doing an ultrasound

My daughter C.  had her second abdominal ultrasound a few days ago. I know, she'd just earned a superlative score from her liver expert three weeks ago. So why the scan?

Well, you could call it maternal over-concern. Or, if you want to be really accurate, hypochondria-by-proxy is probably better. (I thought I'd invented that term but Google insists others beat me to it.)

In any case, after C.'s tussle with liver failure and mine with Takotsubo Syndrome, the vaguest hint of illness sends me into a panic (e.g. I just need to feel weakness and nausea to see myself right back in the cardiac intensive care unit).

So when C.'s feet were slightly swelled one night for a few hours, I began examining her stomach scrupulously every day for a week. I concluded that the Ascites (abdominal fluid) had returned.

After convincing C.'s pediatrician to send a referral for the scan, I dispatched the Hubby with C. to the local scanning center. The findings: mother has hypochondria - no sign of Ascites. The technician's assessment is all we've got for now. But she was very confident and even added that the liver looked healthy too. The doctor's report will arrive any day. 

But while we're in the clear liver-wise, C. seems pretty out of things. Her standing is unsteady and her walking still non-existent. She's not very responsive, sleeps a lot during the day and no longer signs "I want to eat" by placing her hand in her mouth.

In short, the deterioration triggered by her liver illness still hasn't been reversed and I'm losing hope that it ever will.

From liver toxicity to muscle spasticity

Artist: Edward Siebold (1829)

Onwards and upwards. I'm now post surgery and slowly recovering with the emphasis on slowly.

I finally had that gynecological procedure I've been mentioning on and off for one and half years. I'll leave it at that to avoid TMI.

The crux of it is I can't lift for a while, can't even turn C. over while she's in bed, have been awfully weak and have even had a low grade fever for the past few days. Today was the first day of semi normality, thank heavens.

So parents of children like C.: Beware! All that lifting and carrying isn't only dangerous for your back. Your pelvic organs could be victims too. Nobody ever shared that secret with me until surgery was my only option.

Our Australian darling of a caregiver has left us to resume the life of a recent university graduate in her late twenties with a boyfriend from these parts. We wish her much happiness but already miss her terribly.

Her replacement as a carer for C., a kind, experienced woman of 53, is stressing me out despite her good intentions. There seems to be a communication gap since English is her second language and we don't speak a word of her first. She also seems eager to take charge even when I'm around -- but is caring and sensitive towards C. so we'll just need to iron out the kinks.

Enough grumbling. We would never manage without her.

This week she and the Hubby brought C. to the gastro guy who specializes in liver diseases and who gave us some good news. "The first good news we've had about C. in twenty years" as the Hubby put it.

For starters, the gastro's report states that the "probable diagnosis" for her liver is Valproic Acid-Induced Liver Injury, although elsewhere he qualifies that by writing Valproic Acid is, in his view, the "DD (=Differential Diagnosis) with a fair suspicion of Autoimmune or Idiopathic".

So, a warning for any of you with kids on Valproic Acid: Be scrupulous about follow-ups and insist that the neurologist address any anomalies in blood test results. Also insist on tests for Ascites (there's an ultrasound to confirm and quantify that symptom) and any other fluid retention. Our neurologist had been dismissive of blood results that were red flags for our pediatrician. And she (the neurologist) never bothered to ask us about fluid retention, let alone examine C. for signs of it.

I would question one point that the gastro made in his report: "C. commenced a low salt diet and Aldectone 50 and Fusid 50 with excellent results". That's inaccurate in two ways:

• One, C. already was, and always has been, on a very-low-salt diet when her liver problems were first noticed. 
• Second, the results of all that Aldectone and Fusid (both forms of diuretic) were not as he described them. Unless you call rendering C. gaunt, seriously dehydrated and requiring IV fluids in the ER "excellent results".

The gastro guy also recommended a drug to remedy C.'s spasticity (I mentioned that here: "My daughter thinks she's a surfboard"): Baclofen, which he obviously doesn't prescribe since that's outside his field. He suggested we consult with our neurologist about it.

We did that, and she expressed surprise at the gastro's advice because this drug is known to pose liver risk. That, along with the info my daughter in law the clinical pharmacologist gave us, that Baclofen can exacerbate seizures - has left us in a quandary.

If any of you have ever given your children Baclofen,  I'd appreciate hearing what results you saw.

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.

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.

Trial and error - with the emphasis on error

The gauntness and pressure sores are still plaguing C. but - ssh, don't divulge this to a soul - she's seizing less than she has in years and is only getting cannabis. The Hubby and I don't even dare to utter the above so we "nudge, nudge, wink, wink" to report our observations to one another.

The health maintenance org people finally sent us a dietitian to assess C. and her diet. She was stumped, along with everybody else. Where is all that nutritious, high calorie and protein food I so lovingly cook for her going? She said she'd consult with more senior colleagues and get back to us but so far, nothing.

This morning the visiting nurse took more blood from C. for a laundry list of tests ordered including one that C.'s pediatrician believes may shed some light on the source of her liver damage.

And this afternoon a second health fund physiotherapist came to finish the assessment begun by her colleague three months ago so that a new insert/wheelchair and bath seat can be ordered, courtesy of the health ministry.

Victims of Valproic Acid on France's radar

I'm down to giving only 200g of valproic acid to C. as we
wean her entirely off

To quote the Hubby: "Holy cow!"

It was an apt reaction to this Reuter's report I shared with him. They say that last Tuesday, November 11th, the French Parliament amended the 2017 budget bill to include 10 million Euros earmarked for a new compensation fund.

The beneficiaries will be the victims of none other than valproic acid!!

Yes, the very drug that C. has been imbibing for three and a half years and that has apparently wreaked havoc with her liver.

Last night, we started C. on two diuretics - Furosemide and Spironolactone - to replace weekly drainage in the hospital. And, of course, we're still hoping that once we have finished the valproic acid weaning, her liver will recover somewhat. (We're already down from 700 to 200 mg twice a day).

And still no word from that pediatric neurologist who prescribed the friggin' stuff and ignored the signs that her liver was being damaged by it. Right up to the very end of her relationship with us!

The liver plot thickens

The good news about C. is scarce these days.

She has still been signing "I want to eat" impressively (by putting her finger in her mouth) and putting the spoon in her mouth until her bowl is empty. But on every other score, she's not doing well. She does her daily MEDEK assisted walking with me - but not nearly as proficiently as she once did.

But most alarming are the awful blood test results which were unchanged today. And along with those, yesterday's ultrasound findings, the outstanding of which were abnormality of texture of the liver and mild to moderate ascites which the pediatrician referred to in his summary as "liver disease".

I can't recall whether I blogged about this but I did notice that C.'s stomach was enlarged and hard about two months ago. Inexplicably, and unforgivably, I brushed it off as insignificant or perhaps weight gain from the dietary changes I'd made on the dietician's recommendation.

There was so much else to tend to that shlepping C. to the pediatrician to have him examine her stomach didn't seem essential. After all, we were sending him photos of her pressure sores which were topping our list of headaches back then. And he didn't urge us to bring her in.

Besides, when we brought her to the wound clinic, she was examined and her fluid retention was declared "to be expected" and "to be ignored". The doctor doing the declaring was an orthopedist and we'd been scheduled to have the vascular specialist examine her.  But when I expressed doubt, the clinic nurse explained  that he was "unavailable". She assured us that this orthopedist was equally competent since all the doctors in the clinic were "one team".

The icing on this catastrophe cake is that after reading the ultrasound summary which we took the initiative of forwarding to C.'s neurologist, she promptly wrote back: "This is beyond the scope of my expertise. You need to talk to your family physician ASAP".

And then came the cherry on the icing: when we asked her whether she had submitted the request to the relevant government licensing office to raise C.'s cannabis dosage which she'd promised to do, she wrote back: "I would stop the cannabis now. There is a problem with her liver and no one knows if this is the cause."

Of course, it's more likely to be related to the valproic acid (which by the way she prescribed). But she isn't buying that theory.

Now I'm afraid she'll request that our license be rescinded.

Liver worries liven things up

Source: http://www.hepatitiscnewdrugresearch.com/liver-health.html

Here's an update on C.'s bad blood test results and persistent plague of pressure sores.

Our pediatrician - despite C.'s reaching 21 years I implored him to keep her on - has done another 180. But before convicting him of vacillation, bear in mind that he's a Harvard Med School graduate, an ace diagnostician, and a humble, compassionate human being.

So to recap: he initially blamed C.'s low protein and albumin on the valproic acid of which she gets 1400 mg/day. He then switched to blaming her diet and recommended as a remedy more eggs and dairy. Then last week he switched back to the valproic acid theory. He said his research revealed that every one of C.'s blood abnormalities - and she's had several for quite some time* - could be traced to valproic acid.

He couldn't fathom why C.'s neurologist doesn't agree and guesses it might be because this condition is rare. To confirm his suspicion, he has referred us to a hematologist and an ultrasound of her upper abdomen including liver, gall bladder, bile ducts, porta hepatis, portal vein and spleen.

He's even referring to this possible condition with a very medical name: mild hepatic dysfunction.
Here's the Medscape entry on Drug-Induced Hepatotoxicity:

Updated: Oct 09, 2014

Background
Drugs are an important cause of liver injury. More than 900 drugs, toxins, and herbs have been reported to cause liver injury, and drugs account for 20-40% of all instances of fulminant hepatic failure. Approximately 75% of the idiosyncratic drug reactions result in liver transplantation or death. Drug-induced hepatic injury is the most common reason cited for withdrawal of an approved drug. Physicians must be vigilant in identifying drug-related liver injury because early detection can decrease the severity of hepatotoxicity if the drug is discontinued. The manifestations of drug-induced hepatotoxicity are highly variable, ranging from asymptomatic elevation of liver enzymes to fulminant hepatic failure. Knowledge of the commonly implicated agents and a high index of suspicion are essential in diagnosis.

So while I'm still awaiting the ultrasound scheduled for this Wednesday and an appointment with the hematologist, I feel that we're inching toward a final resolution of this mess.

And, who knows, a reversal of this condition may also herald an improvement in C.'s general functioning. (that old optimism rearing its delusional head again).

As for the pressure sores, they are healing nicely now though they're still around and requiring that time-consuming daily bandaging.

Still haven't organized any at home physiotherapy or occupational therapy or hydrotherapy in C.'s former school's pool. We've begun to collate the names of a few senior, respected ones to contact.
___
* Abnormally low total protein and Albumin
Abnormally low hemaglobin, Red Blood Cells and Hematocrit
Abnormally high MCV [Mean Corpuscular Volume] ) and MCH [Mean Corpuscular Hemoglobin] Levels are tested as part of a complete blood count test. The MCV test measures the size of the average red blood cell. The MCH test measures the amount of hemoglobin in the average red blood cell.