Saturday, April 15, 2017

Massage or physical therapy - you be the judge

I finally hired a physical therapist for C. It was the first time she had this therapy since she "graduated" school at the end of August 2016 - and was thrown to the dogs by the powers that be.

Since then her liver crisis and pressure sores consumed all our time and attention. Only now, with those issues resolved, could we focus on her less urgent needs.

I was surprised to discover that few physical therapists will travel to their patients, regardless of the remuneration. And since we aren't up to driving C. anywhere on a regular basis, we were down to one candidate who agreed to come here (she lives a five minute drive away).

The woman was French, nice and gentle with C. But she spent nearly the entire session oiling, massaging and stretching C.'s limbs. Now I'm sure there's no harm in that, and possibly huge benefits of some sort. But it's what I'd expect from a certified masseuse - not a physical therapist.

For the remainder of the hour, she listened to my summary of C.'s medical history and watched me demonstrate MEDEK-standing and describe the MEDEK-walking I used to do with C. in pre-liver-crisis times. (Surprisingly, she'd never heard of that superlative physical therapy method.)

I was impressed by how very calm C. looked and by her repeated "smiles" throughout the session. (Just to clarify: C. hasn't really smiled since her epilepsy struck 21 years ago. But she does stick out the tip of her tongue through the side of her mouth when she is happy and we've designated that her "smile".)

The therapist was confident that we'd see unspecified improvements in the coming week and urged me to call her with a report. But nothing resembling improvement eventuated. In fact for some reason, C.'s standing has worsened somewhat in the last three days. Not that I'm blaming the massage.

Still, desperate times require desperate measures. So with no alternatives around for now, I asked this one to come again. The second session was much like the first. This time, though, I observed more intently and photographed and videoed the routines so that C.'s caregiver and I will be able to do them daily.

But somehow this doesn't pass for physical therapy in my book and so I've resumed the hunt for another therapist who'll work at our place.

Have a look at the photos. What do you think?

Monday, April 3, 2017

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.

Saturday, March 11, 2017

C. is communicating - but it's exhausting us

A screen grab from the great video clip I mention below
The Hubby and I have been stumped by a brand new challenge that C. is posing these days.

Until a week ago, she slept right through the night almost without exception. And by that I mean 8-10 consecutive hours. Now she has taken to waking us up every couple of hours with a medley of screaming, crying and moaning.

When it was an isolated event, we actually welcomed the interruption to our sleep because, after all,  it's a form of communication with us. And, heaven knows, there precious little of that.

But it's worn out its welcome. I mean, two hours after feeding C., giving her drinks, changing her diaper and turning on the clip of "8 Hours of Soothing Lullabies", what on earth could be bothering her?

Hubby has been the hero, getting up to give C. water and gain us another couple of hours of quiet. Last night, I urged him to wait and C. actually stopped crying and fell asleep again for a few hours.

Maybe it's just a bad habit that needs breaking. I'll let you know how tonight turns out.

And here's a priceless video clip [via YouTube] I chanced on and urge you to watch. It has people with disabilities advocating for in-community living - deinstitutionalization - for themselves. No non- disabled activists take part in it. And it's even humorous. I hope you'll be as impressed with it as we were and that you'll agree it deserves to go viral.

Wednesday, March 1, 2017

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.

Thursday, February 9, 2017

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.

Monday, January 23, 2017


I wrote a few days ago that my daughter C. has been astounding us. She has gradually resumed placing the spoon in her mouth to feed herself. Here's a video clip from last week.


Wednesday, January 18, 2017

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.

Thursday, January 5, 2017

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.

Tuesday, December 27, 2016

A brief, eventful ER visit

That's me, briefly visiting C. and hubby in ER and giving
a hand with feeding
So much is happening and so fast.

Following my footsteps, C. took an ambulance yesterday accompanied by the hubby and they have been in the ER since 2:30 yesterday afternoon. I am home trying to keep calm (hah!) and recuperate from my hospital stint.

After the requisite couple of hours of being ignored by harried hospital staff, C.'s treatments are rolling in fast and thick. She has been started on IV Keppra, an anti-epileptic we did many years ago and abandoned. While we don't recall any details, it was probably for lack of results.

But some of these drugs are known to impact differently at different times so re-tries are common. Besides, unlike Vimpat, which we started yesterday and are now dropping, we were advised Keppra is harmless to the liver.

Then, at my suggestion, the gastro specialist gave C. her first dose of steroids for her malfunctioning liver. We'd been waiting for a liver biopsy to ascertain whether or not the gastro's hunch about Autoimmune Hepatitis was correct. But organizing that was shlepping on for so many weeks that I thought it might be better to just jump to steroids without it. I'm still in shock over the gastro's agreement to actually go that route.

Now, with hospitals here overcrowded to bursting - and C. and hubby languishing in an ER aisle for 24 hours - the doctors have decided to release her imminently.

Hoping the new treatments herald better days for us all.

Sunday, December 25, 2016

Bummer: Back to anti-epileptics

It was with a sense of disappointment and defeat that I deposited a 50 mg Vimpat pill down C.'s gullet today. I did it quickly, in the midst of a slew of rough seizures.

A small blessing was that the hubby and I were unequivocally on the same page. C. has had several days of such events and we felt we could no longer rely on the THC oil to rescue her. We've been giving it too often and it zombies her out.

She has lost so much of her always-minimal functioning in recent weeks. I had hoped that a C. off all anti-epileptics would be a more alert and higher functioning.child. Yet the only "positive" result we've seen is her return to crying at appropriate times. Last night, for example, she cried when I removed the bandage from the most horrific of her pressure sores. Not exactly cause for confetti. Particularly in light of her loss of the ability to do her assisted walking and to feed herself.

Now I'm hoping that, with Vimpat, she'll regain those skills and return us to the "good ol' days" - if you can call them that.

Of course I've had to weather all this while reigning in my nerves to avoid a return visit of my new Japanese friend.

Update: The hubby just paid a visit to C.'s gastro/liver expert on his own. I didn't didn't feel up to joining him and C. was in a bad postictal state. They hooked up with me by phone mid-visit so I learned the following.

The doctor said he's still organizing C.'s liver biopsy which will involve a general anesthetic and an overnight hospital stay. When I asked about her functional deterioration, he said that could be caused by a toxic excess of ammonia in the brain due to the liver malfunctioning. This can occur even when blood levels of ammonia are normal (which C.'s have been).

After steroid treatment, which he's considering if the biopsy confirms that she has an autoimmune illness, C. might retrieve those skills.

Dare I hope?