Tuesday, December 6, 2016

Walking the gauntlet

We've managed to hold off with starting C. on the Vimpat, though it's sitting at the ready in our med drawer. That's basically the only good news.

Otherwise, C. has been growing gaunter by the day - despite eating well (though not feeding herself, unfortunately). She's also very weak and unable to do assisted walking as well as she used to. And she kvetches with every step. Quite the nightmare.

Her blood test results were all bad (CRP and IgG rose) except for her albumin level which rose. One thyroid reading was bad for the second time although the other two were fine again.

The pediatrician confesses that he's confounded. He'll be speaking to the gastro guy today.

In the meantime, here is a glimpse of C.'s gauntness (I did the sketch). The bandage over her right eye is from the gash she sustained at the hands of her mother. Yes, I dropped her last week while walking with her. It was glued up in the ER 10 days ago. So amidst this mess we can at least rest assured that she won't have a bad scar to mar her beautiful face.

Saturday, December 3, 2016

In real-time, a trying night

Caring for C. has become a consuming and stressful ordeal.

While I type, we are deliberating over whether to call an ambulance as her pediatrician advised, She's just been in status epilepticus for an hour and a half. For the moment, the seizures have petered out and she's asleep so we're holding off because I just hate being in hospital with her.

What precipitated this crisis is that she has been off the Valproic Acid  for one week now. The only treatment she's been receiving is cannabis oil: CBD 3x/day and THC whenever she had a string of seizures which wasn't often.

But we had been instructed by her new neurologist to start her on Vimpat while weaning off the VA and I disobeyed those instructions. Why? I had this pipe dream of having her off any anti-epileptics for the first time in twenty years and perhaps glimpsing a modicum of cognitive recovery.

But I now regret my little "experiment". Our devoted and wise pediatrician sounded puzzled (even annoyed) tonight when I confessed that C. hasn't been on Vimpat all along.

And in the background of this crisis is the new liver diagnosis we received on Sunday when we visited  her new gastro: Autoimmune Hepatitis. He is now 80% certain that it wasn't the Valproic Acid that damaged her liver - as he'd presumed earlier - but rather this disease which almost never afflicts anybody as young as C. Further tests - a fibroscan and a biopsy - will clinch the diagnosis.

To add salt to the wound: we have been giving C. diuretics to drain the fluid which has been accumulating due to her liver damage. The result is a shockingly gaunt child. Clearly, she had been harboring excess fluid everywhere, even her face, for quite some time.

Tuesday, November 22, 2016

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!

Wednesday, November 16, 2016

Now disabled *and* chronically ill (Part 3)

C.'s elbow and the latest sore
[Written Sunday, November 13]

We've been home for four days now, all without the Hubby who is overseas. We were reassured that nothing untoward in the next week was anticipated by any of the doctors. But here we are, already seeing several worrisome manifestations.

The ascites seems to have returned. Her stomach seems swollen and hard, though less so than it did before her hospitalization.

Also, her hands are plagued with blisters that, after bursting, remain open sores for a long time.

Finally, there's a large new sore on her elbow accompanied by swelling and redness that had me deliberating over an ER visit.

Fortunately, my older daughters were around with counsel and support and we all decided against the ER. Fortunately it was the right call - after a night with the iodine-and-bandage treatment, the area surrounding the wound had subsided.

But we're not out of the woods and the visiting nurse was duly impressed with C.'s elbow when he saw it this morning - and he usually downplays C.'s pressure sores.

These days, I vacillate between acceptance of the gastro's dire prognosis and hope that our optimistic pediatrician knows better: "She'll probably be fine after she's weaned off the VA" he assured us.

We are now down from 700mg/day to 400mg/day of Valproic Acid. I haven't managed to get the Vimpat script yet so we're in risky territory. But I may hold off with it even after I've purchased it. I am dreaming of having C. on cannabis alone and enjoying some pleasant surprises.

Of course that would mean resisting the doctors pushing that Vimpat and I'm not sure I'm up to the challenge.

Just to offset some of this doom and gloom, here's a clip that is bound to send your spirits soaring. .

Tuesday, November 15, 2016

Now disabled *and* chronically ill (Part 2)

My daughter's hand, swollen as a result of an unsuccessful
insertion of an IV port
[Written Thursday November 10]

I wrote the previous post after C.'s first day of hospitalization on Sunday. She was subsequently drained of 4 litres of abdominal fluid which itself triggered a string of seizures. The woman who administered the ultrasound and the drainage procedure - presumably an M.D. - was a pain. She addressed me as "Mommy", initially instructed me to leave the room but gave me a reprieve provided I didn't touch C. "if I think it will help her".

When she was poised to puncture her abdomen, she told me "Now Mommy, you mustn't look." Honest, she said that to me. And she's at least half my age.

When C. began seizing, she donned a neurologist's hat and told me that C. needs benzodiazepam asap and that she'll get me some. I assured her that this sort of thing has happened before, will eventually pass and certainly doesn't warrant any benzo. She insisted that my past experiences with strings of seizures were irrelevant and that they must be halted immediately because "they're harmful for C.".

Thanks for the "Epilepsy 101" lecture, I thought.

Ultimately, the Hubby arrived about a half an hour later with our trusty THC oil from home and after dosing her with eight drops the seizures stopped cold.

We had been instructed by the neurologist to delay the valproic acid weaning because of the bout of seizures. But by nighttime, after hours of calm and with no neurologist around at that hour, I just began reducing the dose.

After several blood tests, urine collections, IV albumin and IV fluids and another visit from that gastro doctor, C. was pronounced able to go home. For those wondering, the IV fluids were to replace the fluids that were removed. Only this time, they'll be properly distributed throughout her body. Trouble was, though, that the staff got a bit carried away with the quantity and C. ended up with a swollen face.

By the time we arrived home, her left eye was totally sealed shut.

The kind gastro told us to treat C. as chronically ill from here on in and to anticipate regular, even weekly, draining of her abdomen. At my insistence he gave a prognosis: 2-5 years.

Now disabled *and* chronically ill (Part 1)

The bag on the floor contains what was
being drained from C.'s peritoneal cavity

[Written Monday, November 7]

C. is officially hospitalized, although we brought her home yesterday evening and returned this morning. This arrangement is known as a "vacation" here. And while the staff were reluctant to grant C. one, I told them that we were bringing her home regardless and then lo and behold it was hastily "organized". The reason I was so truculent (internal seething is usually more my style) was that we had been ignored the entire day. No specialist doctors examined C., no tests were done and no treatment administered.

After my meltdown, one gastro specialist did appear on the scene and, as I later told our pediatrician who had unearthed him to treat C., he was worth waiting for an entire day: young, friendly, knowledgeable, savvy.

He had two possible theories as to why C. has developed liver disease and ascitis.
  1. The valproic acid (VA) caused it.
  2. She has the genetic syndrome Wilson's Disease.
He leans more towards theory #1 but will test her for #2. It can be diagnosed rather easily without genetic testing.

And please note everybody: He utterly rejected the neurologist's opinion that cannabis could be the culprit and should be stopped asap. "Why hasn't she blamed the valproic acid?" he wondered.

But he instructed me to wean C. off the VA quickly: reduce from 700mg/dose twice a day immediately and then over a few days remove the remainder.

Two intrepid medical clowns try their hardest
to cheer up C.
Now I've had some experience with anti-epileptics and asked him whether that protocol might not trigger seizures. In an instant, he back-tracked, advised me to leave the dosage untouched and said he'd consult with a neurologist.

Hmmm. Another instance of proof that we can never rest on our laurels and blindly entrust our children to the doctors - regardless of superlative experience, expertise and reputation. We've go to question and double check everything.

To be continued

Friday, November 4, 2016

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.

Monday, October 31, 2016

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
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.

Thursday, October 13, 2016

Where have all the proteins gone, long time passing?

Me walking C in her new shoes
C's blood test results from three days ago were very disappointing. Last month the dietitian I consulted assured me that with the re-introduction of lots of carbohydrates - after years of nearly none under the Modified Atkins Diet - we'd see a turnaround. Instead C.'s low protein, albumin and hemoglobin levels hardly budged. (The hemoglobin even dropped further). Bummer.

I consulted our pediatrician who initially suspected that the valproic acid was to blame since liver malfunction is a known risk of that drug and since the liver  plays the major role in producing proteins, including albumin.

He promised to get back to me with a more definitive answer but seemed to forget. When I called him today, he surprised me with a 180. He's now convinced that C.'s blood results are not low enough to indicate signs of liver failure, that the valproic acid is not involved and that the low protein levels must have a dietary cause. 

But he dissed the dietitian - politely, of course. (Yes, I'm now back to my old disdain for dietitians.) He thinks the heaps of chicken and turkey which I've been giving C. are at fault. They're not the ideal protein sources. The best ones, he said, are milk products and egg whites.

Needless to say, I'll be incorporating those foods into C.'s diet immediately and will repeat the blood tests in a few weeks.

Aside from the plague of pressure sores,  C. seems weakened by this mess as well. Even her assisted walking involves more of a struggle for her. Ah, for the old status quo.

C.'s neurologist, whom we emailed last night, agrees that the valproic acid is not a likely culprit.

So here's hoping the milk and egg solve the protein puzzle..                     .

C.'s post-school existence: We haven't organized any at-home therapies yet. So I'm still caregiver, nurse, physiotherapist and OT. The hubby does the heavy lifting whenever he's here. Those forbidding 24 steps outside our front door haven't budged yet either. So C. only gets fresh air on the balcony these days. I know, it's nothing to write home about. Hoping for better news soon.

C.'s feet, like the rest of her body, don't grow at a normal rate so she doesn't outgrow her shoes. And with only one hour of use a day it takes years for her to wear them out. We finally reached the point where I could justify buying her a new pair - one size up to accommodate her edema.

Friday, September 23, 2016

Charlatans, snake oil peddlers, quacks, alternative practioners - take your pick and beware!

Hyberbaric oxygen therapy chamber
I have just read a piece (here) about children with autism and their parents' quests for cures. It describes the treatments - sometimes dangerous, usually exorbitantly expensive and unproven - that they try. I was transplanted back to the years the Hubby and I chased a cure for C.'s disabilities because the phenomenon described there is endemic to the entire world of children with disabilities.

As I've mentioned here before, in those days of desperation and blind hopes, we tried every approach and supplement we heard or read of. Often we repeated failed ones with a new "healer" just in case he'd be successful where a colleague of his hadn't.

I once tallied the number of "cures" we tried and reached 35. I never calculated the dollars and hours we squandered in those years when irrationality reigned.

Occasionally, the charlatans offered us free introductory sessions - which we invariably accepted. Once they started charging us, we often stopped the therapies. But there were those we pursued and financed for months - such as cranial sacral therapy, osteopathy (with two different osteopaths), acupuncture (both Japanese and Chinese), chiropractic (also with two different practitioners) and more. Some of the cures came in dark brown bottles and were home-brewed concoctions.

There was one compassionate woman, highly regarded in the world of this poppycock, who offered a grab bag of alternative remedies to C. without charge and indefinitely. After a couple of years, the futility of it finally struck us and we couldn't even bring ourselves to waste any more time on them, free though they were.

A healer in another city explained before the third - and last - free "introductory" treatment that he was "reconnecting C.'s brain cells". Despite the grip of desperation,we still retained a modicum of rationality and never trekked back to him.

I was reminded of his boast when I read this line in the above-cited Spectrum News article:
Once she pays off her $11,000 credit card debt from Cancun, Shearer knows what’s next: the Plasticity Brain Centers, which claims to reconnect neural pathways after their proprietary diagnostic technology pinpoints “the exact brain functions that need treatment.” Skye has tried other brain-related treatments, but this one, Shearer says, seems to take a different approach.
Admittedly more sophisticated than the claim we'd gotten. But then our charlatan wasn't charging big bucks. In fact, while we did squander thousands of dollars, there were far more pricey snake oil cures on the market which we just couldn't afford.  Thank goodness for our financial straits - I now know that some of those were actually risky (for instance hyperbaric oxygen therapy).

In any case, I highly recommend this piece to all you parents considering questionable treatments because there seem to be no other options out there.

Right now, we are still battling C.'s pressure sores, seeing significant progress with most but not all of them. Hoping the new diet - we reintroduced carbs after a hiatus of several years - will herald improvement in C.'s total protein/albumin levels and sore healing.

As for the edema she began sporting this past year, here (over on the right) is what her leg looked like one day this week. The doctor at the wound clinic saw it and wasn't concerned, so I've deleted it from my "worry list" (which is ample enough without as is).