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?

Wednesday, December 21, 2016

Takotsubo 101

My body fina─║ly 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.

Friday, December 16, 2016

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.

News alert: Kappa, lambda and sodium

The diagnostic lab called C.'s pediatrician yesterday because the blood test results showed alarmingly high sodium levels which translate into serious dehydration.

The diuretics clearly did that. This revelation explains the puzzling gauntness and the dietitian explained how: Every cell contains water so when somebody is dehydrated every cell in his body dries up and shrinks.

Well, those blood tests are the gift that keeps on giving: today some more results rolled in including some that had never been tested for C. According to the liver specialist, they indicate a hematological problem.

"I think you should consult a hematologist also due to the high kappa/lambda ratio."

When I consulted Dr. Google about these results, the illness that was always mentioned in its context was multiple myeloma which took my father from us twenty years ago.

So the list of worries and specialists on C.'s case is growing fast. But the answers still elude them all.

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.