Phenytoin is up next

Very excited to share that my daughter C. will finally try out a new anti epileptic - one that has been found successful in a significant percentage of EIEE11 cases. 

It's the first med change we're making since her diagnosis with that syndrome three months ago. 

Aside from CBD, this addition will bring C.'s anti-epileptic tally to three. And while I loathe giving her so many at once, as the neurologist emphasized, the cardinal rule is one med change at a time. So, we'll wait until C. has settled into the therapeutic dose of phenytoin and then, the doctor promises me, we'll remove at least one of the others. 

She even asked me which I believe is less effective. I told her that's hard to judge but since Vimpat was added last and there hasn't been an improvement since, I'd like to see the back of that one. 

Truth be told, her other med, Keppra, isn't anything to write home about either.

Hoping to get the script for Phenytoin (marketed as Dilantin in some markets) tomorrow.

UPDATE

Yay, got the script and C. has taken her very first Phenytoin pill.

When I told C.'s pediatrician that this was the drug selected by the neurologist, he was surprised: "Back in prehistoric days, when I was doing my residency", he recalled, "we had only two anti-epileptics to administer, Phenobarbitol and Phenytoin. There are so many new ones on the market now."

But, I reminded him, those new ones just don't help C.

Here's some Wikipedia input:

Phenytoin was first made in 1908 by the German chemist Heinrich Biltz and found useful for seizures in 1936. It is on the World Health Organization's List of Essential Medicines, the most effective and safe medicines needed in a health system.

Hoping that stellar reputation will prove well deserved!

Back to hydro - and doctor problems

C. finally scored one hydro session during the month of her therapy pool's closure. I had hesitated to give her one because the water in the available pool - it's the one I swim in - is much colder than C.'s therapy pool. But our past experiences there, about a year ago, usually proved worthwhile nonetheless. 

So with E. and the Hubby we gave it a shot on Sunday. 

The first ten minutes she was tense, limbs bent and unable to float on her own. I was tempted to admit defeat. But eventually she floated independently, kicking slightly - admittedly shivering - but thrilling us all nonetheless for around twenty minutes. 

On the seizure front, we're still seeing somewhat of an improvement with the raised Vimpat dose though not the sort that halts the hunt for better control. 

But for now we're occupied with trying to get a neurologist to submit the paperwork required by our health fund for renewal of our Vimpat authorization. It expires on November 24th and the paperwork involves a neurologist filling in forms along with numerous other signatures, a time consuming process. 

The problem is we're getting the email silent treatment from the two neurologists who have most recently treated C. One is the doctor who recommended adding on Frisium while also recommending that we see an epileptologist since epilepsy isn't her area of expertise (she's a headache specialist!) We notified her that we'd heeded her advice re an epileptologist but not the Frisium tip. We were pleading with her to contact a senior neurologist who had helped us immeasurably in the hospital last year. 

No response from her. Apparently she had her heart set on C. getting Frisium - which would have brought her ant-epileptic tally three! (Excluding cannabis.)

The second neurologist ignoring us is the epileptologist we first saw last month - and paid out-of-pocket for the privilege. She is the one who urged us to raise the Vimpat dosage. And now she won't help is out with the Vimpat authorization paperwork!

Is there something in the water these neurologists drink that makes them so heartless?

New neurology nightmares

Last week, we went to a new neurologist who specializes in epilepsy. Here is C. at the hospital while we wait to be admitted to the doctor's office:

We had to pay out-of-pocket because in the public clinic, the earliest appointment with her was April 2019. But C. is doing so badly seizure-wise that we felt this was urgent.

The doctor gave us an hour and a half during which we covered C.'s history, symptoms and functioning. Afterward the doctor gave us her thoughts and recommendations. This probably sounds rather routine but, trust me, in our experience, it's a rarity. We really felt that we got our money's worth.

Of course, that's not to say we left her office uplifted. There was much for us to digest and ponder. So here goes:

Med changes

First, she recommended raising one of the two anti-epileptics C.'s on: the Vimpat by 50 mg/dose. I already did that tonight. In two weeks we raise it in the morning as well. She said the current dose is considered low. Who knew?

She also advised doing a blood test to check the level of the second drug she gets, Keppra. The current dose of that drug - 1,500 mg twice/day - is also deemed low. Likewise, who knew? Our previous neurologist never told us that. In fact, at our last visit she had recommended adding a third anti-epileptic! Frisium! That's one of the benzodiazepams that rendered C. semi-comatose during her hospitalization back in November 2017 

Btw, that incompetent neurologist never bothered to answer our last email. In it, we mentioned, inter alia, that we did not add the third med, Frisium, as she had instructed us to do. I suppose she wasn't too pleased with that. Back to the new neurologist:

Video EEG

We've never done one and she'd like us to. So some time in the coming weeks, C. will be hospitalized for 24 hours. Here's hoping we glean some helpful revelations. Our HMO has already authorized the expense.

C.'s frequent fevers

She wasn't impressed with the exclusion testing that C.'s pediatrician did. As I've written, he did a thorough clinical exam and blood test before concluding that the fevers she's now getting - they max at 100.6 rectally - are not caused by an infection. Ergo, they must be central.

This neurologist said that those exams weren't enough to reach that conclusion. She said she's seen patients who seize more than C. but never get central fevers. She suspects there could be some underlying condition, perhaps related to her life-long thinness, triggering the fevers. She said the pediatrician should have referred us to a specialist - she couldn't specify but threw out endocrinologist and gastroenterologist as possibilities - who could run tests to determine whether in fact there is another cause for the fevers. 

Whew, not exactly an inviting task.

VNS

She strongly urged having a new VNS implanted. The one inside C. dates back to December 1999 and never helped her one iota. She advises trying it again. Of course, this time we'll have somebody better equipped to calibrate it afterwards. 

Back in the year 2000 there wasn't any such expert in this city. (I know, I know: so why did we implant it then anyway? That was just another of our many blunders.)

But removing this old one isn't straightforward we've been told because the wires are coated by now with skin - or something along those lines. Also, the last neurologist who pushed a VNS replacement was the one who destroyed C.'s liver with Valproic Acid but refused to own up to it. Instead she pointed a finger at the cannabis! Which brings me to this new doctor's take on - 

Cannabis

I had braced myself for negativity so I was relieved when she just told us that C.'s CBD dose is very high. Which would explain why the government authority refused to renew our license for 11 bottles (= 100gm) per month. Instead we were given one for only 10 bottles.

She said C.'s current dose would be more efficacious in the form of vapor. It's absorbed far more quickly than the oil C. now takes. I haven't gotten around to inquiring about the availability of this form at our supplier. My hunch is we'll need to switch to another supplier (the one that the doctor recommended); not a process I relish.

Hydro

This week yielded a brief respite from frequent seizures and fevers during an extra long hydro session where C. just rocked. The proof is in the photo above.

The hunt for CBDA

I used to believe that this country is a medicinal cannabis paradise. We've got an abundance of doctors willing to apply on your behalf for a license (and that's a pre-requisite). Provided your ailment is one of the many that the government's health officials recognize as treatable with cannabis your license will arrive within a month. We've got nine suppliers to choose from.

But this week disillusionment struck. After a concerted search for CBDA, we have concluded that it is definitely not sold in this country. Every one of the suppliers I contacted told me that either they'd never heard of it or had but didn't sell it.

What is it?

Cannabidiolic acid (CBDA) is a chemical compound found in the resin glands (trichomes) of raw cannabis plants. In this case, raw means unheated and uncured. Basically, raw cannabis is fresh flower and leaves trimmed directly from the plant. CBDA is the acidic precursor to the more widely known molecule cannabidiol (CBD).] Source: https://www.cibdol.com/blog/368-cbd-science-the-difference-between-cbd-and-cbda

It is readily available on line but a rep of our supplier has warned me that importing it is illegal. She noted that our government-issued user license only permits us to receive the specified products sold by our chosen local supplier.

In any case, it appears that none of the online producers ship to this country. We're still awaiting a response from the ones we've approached.

We're very disappointed by this state of affairs. After consulting our cannabis guru, Elizabeth (https://elizabethaquino.blogspot.com/) and reading further about the product on line, it seems that CBDA is now our best option.

CBD seemed to help C. back in 2015 when we started her on it. The number of daily seizures dropped and she even enjoyed isolated seizure-free days. But that's a faded memory. Her "normal" now is a daily minimum of around 4-5 seizures along with long strings of them on average four times a week.

Elizabeth also advised tweaking the dosage of CBD and THC. We reached the maximum dose of 25 drops of CBD as recommended by our neurologist about 18 months ago. We've remained at that level ever since. But last week I began lowering it. We're currently at 23 drops. I also raised the THC to 4 drops. We give those doses 3x/day.

It's a shame that there is nobody here, neither neurologist nor cannabis supplier, who can guide us more effectively. I'm tired of all this groping in the dark.

Despite her constant seizure assaults, C. managed once again to perform nicely in the pool. Here is a video from her last hydro session a few days ago:

That pot of gold

C. gets hydrotherapy

I was thrilled with C.'s hydro session this week where she surpassed last week's performance. Here she is floating unaided (over on the right).

Her walking has also been advancing. Still nothing like what it was pre-liver saga but better than it has been since her liver recovered.

She's actually sturdily on her feet cooperating slightly while I move her legs. We do this for 45 minutes every night. It's so strenuous that Ive apparently injured my left hand in some way. For the last week, I've been waking up after about five hours of sleep with numbness and pain in my left fingers.

I unearthed a hand brace which I bought many years ago. (That's no mean feat in my chaotic household.) The exercises I was doing back then with C. triggered similar symptoms in the same hand.

Me walking C. today

I've now been wearing it during the walking and while I sleep for a couple of days with amazing results.

UPDATE: I wrote all that a few days ago but things aren't looking so rosy now. We get periods of intense and repeated seizures for which I give either Advil or 20 drops of Cannabis THC, or both. We're also treated periodically to those tiny eye seizures that lock her trembling eyeballs into the left side. The latter aren't as visually alarming as the whoppers but they're still very disconcerting. Needless to say, she doesn't function at her peak while in their grip which can be for over an hour.

We're at the same 25 drops of Cannabis CBD, three times a day, 3 drops of Cannabis THC three times a day, 1,500 mg. of Keppra twice a day and 100 mg of Vimpat twice a day. Still haven't learned if and how we can buy that CBDA. So, for now, I am at a loss as to what changes to make.

That pot of gold - seizure control - seems more elusive than ever.

A dismal discharge

We are now hopeful that C. will be released from hospital tomorrow. Well, I'm not certain "hopeful" is all that accurate given the trepidation I'm feeling about the prospect. C. will be returning home on four anti-epileptics. She left 18 days ago on one! (excluding cannabis).

She will also be returning in an extremely sleepy state and without any appetite.

What has been achieved is a halt to her Status Epilepticus. I realize I'm supposed to be grateful for that. But given the high price we've paid - the loss of every ounce of functioning she previously had - I'm finding that hard.

None of the staff dares predict whether this is a temporary phenomenon.

They are just as vague about how long they'd like her to stay on her current drug cocktail. They maintain that there's no way to ascertain which one actually did the zapping so it's risky to remove any. They say eventually her neurologist will guide us on weaning C. off most of them.

To quote myself: I'll believe it when I see it.

Rising stress

IV Drip

Nothing uplifting to report about C.

When the hospital doctors tried stopping the IV Dormicum (aka Midazolam), she returned to Status Epilepticus with a vengeance within a couple of hours (one minute between seizures).

So she was rehooked-up immediately to the stuff.

Then today the doctor lowered the dose from 3% to 1% and before our very eyes she began seizing again. So he promptly re-raised it and the seizures disappeared. Not much of a solution.

I inquired about the longer term plan.

He told me their plan was to get her off Dormicum and replace it with an oral benzodiazepine, Frisium (aka Clobazam); raise her Keppra (aka Levetiracetam) dose; stop the Phenytoin; and continue with Vimpat (aka Lacosamide) aiming to raise it from 100 mg twice a day to 200 mg twice a day.

Can't imagine there will be much of C. left on that potent a cocktail.

At the same time he said the team is encouraging continuation of Cannabis CBD and THC.

That's all very nice (I'm referring to the cannabis green light) but there's no point in giving THC if she has already been zapped with the other stuff. How will we know whether it's worked?

And we are currently allotted such a small quantity by the government's medical cannabis agency. We'd need a lot more to administer it freely.

A quick bedside sketch

I'm giving CBD whenever she's sufficiently awake to enable me. But that's rarely.

Well, if any of you would not find the above stressful, please let me know. Because the senior neurologist who dealt with yesterday's setback told the hubby: "Your wife seems very stressed. She reminds me of my mother. I see I can't share all information with her."

Who knew senior neurologists go in for crude mansplaining?

PS: C.'s own neurologist came by today after being away all week. She offered the option of an induced coma which would entail intubation accompanied by the risk of C. never being able to breath independently again. I said thanks but no thanks.

She then warned that even C's current treatment protocol could at some point necessitate intubation. Would we agree to that? Something to ponder.

But, of course, not get stressed over. Perish the thought.

Stuck in status - Part 3

C. and friend in her hospital bed

Written Saturday, November 11

We're still in the hospital, third day running, but no longer in the Emergency Room. Now we're in the ward.

The major seizures are nearly under control and feeding C. meals is much easier. She even seems to have a better appetite than usual.

The severe seizures do still erupt from time to time. We've also noticed some small, new sorts involving only the face or only her right arm and hand.

A neurologist came by today and promised he'd order another EEG to ascertain whether those new movements are actually seizures.

The doctors are piling on the drugs like there's no tomorrow. When I expressed concern about that, the neurologist promised that two of them will be removed as soon as she's stabilized but I'll believe that when I see it.

So C.'s drug tally now stands at: Keppra (also known as Levetiracetam and given IV while in hospital); IV Phenytoin (aka Dilantin) (to replace the IV Valium (Diazepam) which was ineffective); oral Vimpat (aka Lacosamide); Cannabis CBD (Cannabidiol); Advil (aka Ibuprofen) for her central fevers which are proliferating; and Cannabis THC (aka Tetrahydrocannabinol) for strings of seizures without fever.

As you see, the positive news is in short supply.

Stuck in status - Part 1

Written Wednesday night, November 8 (but posted on November 11)

I have just spent three hours getting a meal into C. She takes forever to swallow the smallest mouthful. Throughout, she was having small seizures that affected her face. (That grimace in the photo is relatively new to us.) Now have put her to bed but she has begun to have really hard seizures affecting her entire body, and every five minutes or so. I am at a loss.

She had a fever a few hours ago so I gave her Advil followed by Cannabis THC and the hard seizures subsided. But now they've resumed and without any fever.

I am loathe to give her another round of THC only four hours after the last. Haven't touched the rectal Diazepam - wary of it.

Tomorrow we have an appointment with C.'s neurologist but still must get through this night. I often wonder what I did to deserve this.

Status Epilepticus strikes C.

From an online guide to administering Diazepam rectally

We are enduring rough seizure-laden days. C. has even gotten tangled in status epilepticus a couple of times. It's reached the point where a few hours' stretch without seizures is pure heaven.

This bad run began out of the blue on Friday. As usual, there is no change in her routine or in her diet we can blame.

The Cannabis THC did work this morning, zapping a particularly intense status session. But after my daughter-the-dentist reminded me of it, I asked C.'s pediatrician for a Stesolid script.

So we are now stocked with two boxes of 10 mg Diazapam rectal tubes (the equivalent of Stesolid). We haven't used the stuff in at least a decade but it seems a better option than the ER. We are braced for a fresh round of this curse.

PS: The dreaded status struck again tonight. THC took a little longer to work but eventually reined in the seizures. To inject some variety, C. has also vomited twice in the last 24 hours, raising the suspicion that there's something non-neurological underlying this mess. If there's no improvement tomorrow, we will try to do blood tests.

Uplifted by Downs

Isaac Levya, actor

It's been a while since the last post but not much has happened. Still the daily 3-5 intense seizures which wear me down.

So we finally wrote to the neurologist for guidance, enquiring specifically about starting a daily dose of THC to try and tackle them. (We now thankfully have a precious new bottle of the stuff.)

She recommended that we raise the dose of Keppra by 250 mg/day. But our THC idea didn’t appeal to her. She noted that a colleague who is more knowledgeable about cannabis assured her there is no evidence that THC affects seizures in any way, notwithstanding our anecdotal evidence to the contrary.

Nevertheless, she OKed one drop of it/day maximum.

For now, we’re trying the additional Keppra, so far without success. We’ll give it 2-3 weeks and then return to the previous dose of 2,500 mg./day. Afterwards we’ll try that daily drop of THC. Not that I have any hope a micro-dose like that will help. To zap C.’s long strings of seizures, we give 17 drops.

I’ve been encountering many Downs children lately when I pick up one of my grand-daughters from kindergarten. She attends a semi inclusive kindergarten. That translates into a small group of Downs students joining her “typical” class for a couple of hours each morning. The group then returns to its segregated class for the rest of the day.

I know, I know, that’s just token inclusion and the school could do more. But it’s better than none at all.

I mention this because I’ve noted how longingly I watch the Downs kids. I envisage the rosy life we’d have if only C. had been blessed with Downs instead of the disabilities she’s lumbered with.

That’s quite a switch from the days of my pregnancies when my deepest dread was giving birth to a Downs baby. It seemed the direst scenario possible.

Live and learn.

A propos of Downs Syndrome, I just learned of a film starring a Downs actor, "Any Day Now". As usual, I’m playing catch up – the film was released back in 2012. It hasn’t aired yet on TV in these parts so I’ve only seen clips from it on YouYube. It strikes me as a gem and the many awards it’s won attest to that.

Playing alongside its stars, Alan Cumming and Garret Dillahunt, is a young actor with Downs, Isaac Levya. The film is about a gay couple in the 'seventies trying to adopt a child with Downs Syndrome and the legal battles they face along the way. In an interview [here], Dillahunt describes Levya’s professionalism and how, during filming, Levya would occasionally tell his co-actors: “Give me some quiet; I’m trying to get in character”.

He attends acting school, auditioned for the part and endearingly discusses his acting experiences here.

I live in a place where mothers are still known to abandon Downs newborns in the hospital and where some who do bring them home later institutionalize them. Success stories like Isaac Levya's are particularly uplifting in this environment.

A small cannabis victory and a mysterious laugh

Since Wednesday, I've been revelling in giving C. her full dose of CBD. That's when our cannabis provider notified us that the government's health authority granted us a new license with the increase we'd requested (more like begged for) in our monthly allotment of cannabis.

This license entitles us to take that extra bottle either as CBD or as THC. So we've got one bottle awaiting us for August which we'll take as THC. I intend to begin experimenting with a very low daily dose of THC.

Then, starting with our September batch, we'll opt for an extra bottle of CBD bringing our total to 11 per month.

It's unclear why the government officials acquiesced after initially refusing. But C.'s neurologist earnestly went to bat for us with a written complaint and a nudge to her colleague with some position in the government's cannabis section. Presumably her efforts played a role.

All this has renewed my pride in this country's trailblazing in the field of medicinal cannabis. Close to 30,000 citizens are license holders and we are slated to become a major medical cannabis exporter.

But back to C. She has been seizing several times a day without running a fever. That's why I intend to introduce daily THC, though the Hubby wants me to run it past C.'s neurologist first.

C. enjoyed two hydro sessions this week, one with me as therapist, the second in the heated therapy pool with a professional hydro-therapist.

Upon her return from that session she began to vocalize - a lot - until late that night. It resembled laughter. But the Hubby and I were creeped out by it. I was convinced she was just getting creative with her seizures. Her caregiver, E., insisted it was a positive step.

Here's a brief video of the sound so you can judge for yourselves.

Whatever it was, we've only heard it once more, two days later.

And as the summer draws to an end, here's a suggestion for those still enjoying hot weather: a disability-friendly beach equipped with special chairs that maneuver easily on sand and water. You just fly to Italy and ecco là: real "access to the sea".

A snap from the beach as captured in the NYT article:

Here's a description of the beach.

Water and oil

I'm pleased to report that C.'s second Mommy-hydro stint far surpassed her first. She hardly crossed her legs at all which enabled her to center her body and float independently most of the session.

Looking forward to this week's session.

We are still pursuing our quest for eleven (instead of ten) bottles of Cannabis CBD along with one bottle of THC per month.

In response to our request,the government bureaucrats who supervise the issue of medical cannabis have determined that a daily log of every CBD dose administered to C. over the last year is what's missing in their lives.

The recommendations of C.'s neurologist, along with all the requisite paperwork already submitted, just didn't cut it. So we've dutifully supplied them with our log.

A hospital-based neurologist to whom we brought C. years ago now apparently occupies a position of influence over the government's medical cannabis office. Our current neurologist has solicited his intervention.

All we remember about the few visits we made to him with C. years ago is that he routinely asked us "So, what do you think?" and "What would you like to try now?" We never received a suggestion from him. For obvious reasons, we didn't stick with him for long.

Let's hope he'll be quick and obliging now.

Some inspiration from India

I had planned on personally giving C. this week a hydrotherapy session at the pool I swim in every day. I've watched years of her therapies and figured that by now I could tackle it myself. For years I've dreamed of doing so when the therapy pool at her school was closed for vacation. But good intentions were as far as I got.

One reason was the challenge of getting her there and the other was my reservations about using a barely heated pool vs. the well heated therapy pool.

Now, with her caregiver here to help me. it seemed feasible. The therapy pool will be closed for the next three weeks and I really am curious about how C. would react to the water. If she'd respond well, it would mean a huge savings of money.

So I was suitably psyched up and set aside Thursday for our session. Then one of her nearly-healed pressure-sores re-erupted with redness all around the center. The wound clinic team reopened it, prescribed a fresh course of oral antibiotic and forbade swimming for at least a week.

This is exactly one year after the blasted sore first erupted! That's got to be a world record for persistent pressure sores.

The other "good" news is that the government's department of health has responded to our request for a raised dosage of CBD and one bottle per month of THC.

It was "Nothing doing!"

I'm flabbergasted. This is supposed to be one of the most progressive countries in the world with regard to medicinal cannabis and a leader in research into its benefits. What gives?

We'll see what the neurologist has to say about this verdict. I dread having to cope with C.'s seizures without any THC.

But I won't end on that negative note.

I have a soft spot for success stories starring people with disabilities. Remember that remarkable Argentinian kindergarten teacher?

This story hails from India and is as much about a plucky young woman with Downs as it is about her devoted parents who gave her a restaurant of her own to run. All three of them have a message for other parents of children with disabilities and for the children themselves.

The story is all the more remarkable against the backdrop of the general plight of Indians with disabilities and specifically with Downs Syndrome.

The video is at https://www.facebook.com/thebetterindia/videos/1613392548695052/

From a World Bank report:

India has some 40 to 80 million persons with disability. But low literacy, few jobs and widespread social stigma are making disabled people among the most excluded in India. Children with disabilities are less likely to be in school, disabled adults are more likely to be unemployed, and families with a disabled member are often worse off than average.

And the plight of those specifically with Down Syndrome:

Downs Syndrome affects 23,000-29,000 children born in India every year. Though the numbers are alarming but there is very little open dialogue on this topic in India. Though it is not fatal, not in the developed countries but in India it continues to be fatal. This is mostly because of the low awareness levels and outdated medical facilities.

India has the highest number of people suffering from Down Syndrome in the world. The numbers are alarming but what is more frightening is the fact that this condition is fatal in India due to negligence, lack of awareness, and obsolete medical and technological facilities... Out of the 23,000 to 29,000 kids born with Down Syndrome in India every year, the survival rate is only 44% for those who have congenital heart disease [Source]

Summertime and the livin' is none too easy

C. is lying beside me and I'm afraid to move or touch her lest I trigger another seizure.

She has been having lot of them today. Not enough to qualify as status epilepticus, but nonetheless, not a pretty picture.

Yesterday, they were frequent but mild. Today, they're the full blown sort; the ones that engage every cell in her body, contort her face, cause moans and cries and devastate me.

I write this with the expectation and dread of the next one.

Unfortunately, she has no fever - and I mean not a trace of it. The thermometer read 37.3 degrees Celsius rectally (say 99.1 in Fahrenheit terms) which can't be considered fever by any stretch of the imagination. So I haven't given her any Advil. When she gets her neurological fevers, Advil banishes the seizures.

Then there's the THC oil which usually zaps strings of seizures. When I gave it to her this morning, it had no effect. Since that was ten hours ago, I could try another dose now. But I'm reluctant to because we're down to our last few drops of it.

Our neurologist's request from the medical cannabis authority for a new license which adds a bottle of THC to the ten bottles of CBD we receive has gone unanswered.

Our own numerous calls, emails and faxes to the frigging government office have also been in vain. The only response we have gotten was a couple of weeks ago when we received a new, temporary license. It permits the same number of bottles of CBD we've been receiving and makes no mention of THC! 

________

I wrote that last night. Today she's only marginally better; slightly fewer seizures, but still looking like a rag doll most of the time.

We've emailed her neurologist for her advice and just to "kvetch" a bit. I asked her whether she thought we could raise the Keppra dose although that really isn't a route I relish taking. (C. is now at 1,000 mg of it twice/day.)

Here's a photo of C. (below) taken on Tuesday before this downturn - at her weekly hydrotherapy session.

And here (below) she is last week with her little nephew. The interest they show in her as babies fades fast when they realize she can't interact. By around the age of one, they ignore her.

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.)

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.

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.

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.

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.