Thursday, May 18, 2017

Yay - we're back to hydrotherapy!


C. had her first, post-school-hydrotherapy session yesterday.

She seemed relaxed and at times floated nearly independently - that is, after I donned my "meddling mom" hat and urged the therapist to reduce her support.

But those seizures persisted during the session. A couple of whoppers and many of the minis.

And while I did meddle, I was mindless of the proper gear for C. For instance, I entirely forgot about bringing along Huggies Little Swimmers. So C. had to contend with a weighty water-logged diaper.

The photo shows her contending.

Meanwhile we're giving THC when the going gets tough, and it has been effective the last couple of times. (Despite the bottle's expiry date of 03/17 - it's all we've got while awaiting our new, amended medical cannabis license that will enable us to purchase a fresh bottle.)

Tuesday, May 16, 2017

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.

Monday, April 24, 2017

For good cheer, move on

If you were seeking a lift from extreme parenting and the demoralization it entails, well, you've come to the wrong address. This post is limited to bad cheer.

For a full week now, C has been running a low grade fever along with other disconcerting symptoms: sleepiness, unresponsiveness, unsteadiness, seizures both major and minor throughout the day.

Neither the blood tests nor her pediatrician's clinical exam revealed any alarming findings and he thinks C. could just have a virus. But he did notice two anomalies for which he's referred us to an abdominal ultrasound and x-ray:
  • "Vertically-oriented abdominal mass to L of midline, partly pulsatile, probably abdominal aorta" (but he assured me it's probably so prominent because C. is so thin)
  • "L-sided abdominal masses palpated, oriented vertically. Suspect stool masses" (and he assured me this is easily remedied with laxatives).
He wants us to do an abdominal ultrasound and x-ray before treatment. So, with Wednesday the earliest appointment date I could get for those tests, until then it's just wait and endure.

On the right is my sketch of what C. has looked like this past week.

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

Eating

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.

video


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.