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!

On vomit and the vicissitudes of hospitalization

Sunday, October 7

I'd hoped we could keep these hospitalizations down to annual events but didn't manage that. 

C. suddenly began vomiting copiously yesterday. We tried caring for her at home. On her pediatrician's advice, we tried Pramin suppositories. He said that with no fever or diarrhea, she wouldn't dehydrate overnight. 

But the med had no effect. The gushes of liquid vomit kept coming at two-hour intervals. Horrific doesn't come close to decribing that night. By morning, there were strands of brown blood in the vomit so the question of what to do was a no brainer.

So here we are back in the ER, 11 months after C.'s last hospitalization. 

So far, she's weathered a chest X-ray and a 12-hour struggle to get urine via a catheter. (An incredibly incompetent nurse couldn't get any urine even though C.'s on a fluid drip. Nurse on the next shift collected it with ease.) Temporary predictable diagnosis: another UTI.

Now I'm at a loss as to how to prevent them. We had to stop giving cranberry juice several weeks ago because it seemed to cause vomiting. Should we now resume that, perhaps in a more diluted form?

Tuesday, October 9

Here we are, finally, in a proper ward (where we waited several hours in the corridor for a room).

No cubicles available, so we're in the corridor

The doctors have done a diagnosis 180°: UTI is now ruled out, replaced with "some infection, somewhere", possibly gastroenteritis. That may have led to aspiration of vomit and may also have caused the intestinal bleeding we saw in her vomit. 

She's still getting IV Controloc (pantoprazole) to prevent a repeat of that bleeding although there's no sign of it anymore, not even in her stools.

Bumped into the neurologist who we believed had dumped us when she ignored our last email around six weeks ago. Turns out she never saw it and is happy to re-enter C.'s life. She promptly ordered a CT and EEG; assured us she'll contact that terrific American neurologist whom we can't consult directly because he doesn't have a practice.

She also decided to search for info re central fevers and told us she learned it's not uncommon among uncontrolled epileptics!

C. is off the IV drip and has begun to eat and drink. She's back on her Vimpat which was only available in pill form. (They had Keppra in IV form is so she's been getting that regularly). But I haven't resumed the cannabis yet.

We've missed hydrotherapy - the highlight of C.'s existence - which takes place on Tuesdays. We may also miss the long-awaited delivery of C.'s new wheelchair scheduled for Thursday.

The EEG

Wednesday morning, October 10

Back on the drip because after a batch of heavy seizures, C. was unable to eat and could drink only a bit. A CT was done this morning. Not clear why it was ordered but haven't heard any results yet in any case. UTI has been ruled out even more emphatically today. It's not looking as though we'll have C. home in time for the wheelchair delivery tomorrow.

And here are a couple of gems from the medicos:

Nurse about to finish her shift comes around with next shift's nurse. I hear her mention that she had taken C.'s temperature which was slightly elevated but that she decided against Tylenol. I told both nurses that C. is seizing badly and could actually benefit from Tylenol for her fever. Then the nurse about to leave delivers her pearls of wisdom: "You see, she's seizing badly because her epilepsy isn't controlled. It needs to be brought under control. She needs to see a neurologist." To me: "You should consult a neurologist." 

I regret that I didn't ask her: "Why? Do you think she might have epilepsy.?"

Wednesday night, October 10

I witnessed an argument between two doctors at the station which was situated immediately outside our cubicle. I had the curtain open so couldn't avoid hearing one, a male, tell the other, a female: "You're a hussy". The other responded: "Well tell me, do you know the patient's background?" "You ought to tell me it", snarled the guy back at her. "No, you're supposed to know it, " she repeated "Why don't you just do your job?" The guy shot back adding once again, "You're a smartass." 

Help, many lives are in the hands of these nincompoops!

First seating

That same male doctor had earlier declared when the Hubby approached him: "Whatever you're about to say I'm not interested unless it's urgent. I only deal with urgent matters."

Thursday, October 11

C. will be released today. CT negated anything alarming and the EEG showed no change since the last one. 

While she isn't eating or drinking much, I nixed the nurse's suggestion of a feeding tube. On Tuesday, a speech pathologist assessed her swallowing skills and they passed muster. So I figured it was best to just persevere with feeding by mouth. The less intervention, the better.

Thursday night

Home! And, the icing on the cake is the new wheelchair is here too! Here is C. over on the right. Sitting in it for the first time:

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?

Nieces, nephews and one huge hare

C. joined us at her niece and nephew's new home on Tuesday. I wish we could bring her more often to family gatherings but the Hubby's aging back isn't amenable to that.

The highlight of the Tuesday's visit was C.'s tete-a-tete with the latest addition to my daughter's family - "Rabbit" (no name yet). Her hutch is stashed in the utility room and she never ventures out on her own so I had forgotten all about her. Fortunately I remembered her in time for C. to enjoy a lengthy pet therapy session. 

Here she is with what I'm certain is a look of concentration and intrigue:

Her nephew is in the background looking adorable as always. 

There were other nieces and a nephew in the room and I have no doubt their noise, laughter and action benefitted C. immeasurably.

And this news is strictly confidential: C.'s seizures increased immediately after I added the extra 50 mg of Vimpat to the morning dose one week ago. (I had increased the night-time dose 2 weeks ago). But three days later she settled back down to about three a day, with only one bout of fever and seizures. Could it be her body needed to adjust to the Vimpat change?

 

Remember, seizures have been Scientifically Proven Susceptible to superstitions. So improvements are definitely not to be circulated.

I wanna hold your hand

I haven't written about C.'s occupational therapy sessions which have been ongoing for two months. 

A few weeks ago we had one fiasco with C. in the throes of a string of seizures when the therapist arrived. (I had called to notify her but she was already on her way). 

Since then, though, we've had a few productive ones. 

Here is C. (above) getting her ligaments gently stretched at the session's start to maximize her range of movement:

And here below she is working on gripping an object while therapist, N., shakes her hand. 

She is improving at closing her hand to grasp an object and then, on demand, opening her hand to release it.

Modest goals, but enthusiastic responses from all three of us - the therapist, C.'s caregiver, E. and me. You've got to admit, we're a realistic bunch.

We had about two weeks of relative relief from seizures (i.e. only about three a day) which I attributed to the rise in the nighttime Vimpat dose. 

But here we are, after raising the morning dose too and the seizures are back with a vengeance. 

We still haven't managed to check the Keppra blood levels so maybe therein lies a solution. The HMO nurse tells us that test will only be available in about a week. 

No hydro photos because the therapy pool is also closed for a few weeks. 

Excitedly awaiting delivery of the new wheelchair on October 8. 

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.

Time for a new neurologist

We visited C.'s neurologist last week. 

Just a reminder: this doctor was on the team that rendered C. semi-comatose with a barrage of drugs in November 2017 to combat her status epilepticus. Her specialty is the treatment of headaches. We had no clue about that when we began bringing C. to her. She came highly recommended by C.'s pediatrician.

At this latest visit, we related C.'s fever and seizure woes to her and showed her a video of C.'s aquatic skills. We noted that the raised dose of Keppra and the addition of Vimpat, which has been C.'s regimen since hospitalization, has left her in the same state she was in pre-hospitalization. No improvement whatsoever. We'd like to try her on Keppra alone again, we said.

The doctor then told us we ought to consult an epileptologist. For me, that translated into: "This case stumps me; it's not my field of expertise. Try an expert." No news to us. But we reminded her that the neurologist who helped rescue C. from that near-coma in 2017 and is a colleague of hers, advised us to use her as a conduit to him. We can't consult him directly because he doesn't have a practice in this country. He earns his living from home by assessing scans from overseas patients and advising treatments. And then, once a month he spends a week in the neurology ward where C. was hospitalized last year.

After the neurologist assured us she would seek that other neurologist's input, she added, almost as an afterthought: "I have a feeling that the Frisium we gave her in the hospital would help her. Add it on." 

The Hubby noted we don't want any more benzodiazepams in C.'s life and inquired whether Frisium is in that drug category. The doctor conceded that it is - but that "We'll start her on a low dose and raise it very gradually so it shouldn't cause her to be sleepy the way it did in the hospital".

"Sleepy" is a generous term to describe C. in the hospital. As I can't overly stress, what she most closely resembled was semi-comatose.

The doctor claims her goal is to rise to a therapeutic level of Frisium and only then begin to wean her off Vimpat. She left us with instructions on introducing the Frisium. But none for the weaning of Vimpat.

Needless to say, we haven't filled the prescription for Frisium and have booked an appointment with that specialist epileptologist.

It's nearly a week since the visit but no word from this neurologist about her consult with the other neurologist. I didn't really expect her to, though.

Through it all C. has somehow managed to advance her walking to this: here (above) is some evidence from a few nights ago.

Proteus Mirabilis revisits

The picture on the left is my work

Last week, C. treated us to some more spikey fevers so it was off to the pediatrician again for a thorough checkup. 

While in the waiting room, I snapped a shot of a painting I did and which I gave our doctor many years ago in gratitude for his unflagging devotion to C. As you see over on the right, he has it hanging right next to a Mary Cassat. His generosity knows no bounds.

But we could have avoided that trek to his clinic since he suspected it was a rerun of C.'s Proteus Mirabilis (a version of UTI - urinary tract infection) as soon as he heard she had fever without any apparent symptoms. 

But when I called her in advance of bringing C., the nurse at our local health insurance clinic was extremely reluctant to take a second catheter-urine-sample.  

The explicit referral we had from C.'s doctor was to do just that. But the nurse felt it could introduce infection and that C.'s fevers could have been caused by aspirational pneumonia (even though I assured her she had no related symptoms). She urged that a full clinical exam should be done first. 

I wasn't a match for her. But our perenially sweet doctor was truly pissed off when I related all of that: "I hate to pull rank, but..." was his response. Anyway, long story short, as I said, we did the exam the following day. The nurse obliged us with her catheter and now C. is half-way through her course of Zinnat. 

The new challenge is preventing another recurrence. A few of the doctor's tips: Don't let her become constipated (who knew that could trigger a UTI?). Keep her well hydrated. Give her cranberry juice.

We have a neurologist's appointment lined up for this week which the Hubby arranged. We'll probably go without C. just to discuss the meds she's on. 

Since they aren't delivering any more control than we had prior to her hospitalization back in December 2017 for status epilepticus, I am keen to lower the Keppra and remove the Vimpat. That was her pre-hospitalization regimen.

I am dreading this encounter with the neurologist because she was one of the doctors in the hospital back in December who opted to barrage C. with meds rendering her near-comatose. The hitch is, though, that we can only access the wise neurologist who saved her from all those meds via this neurologist. The wise one doesn't treat patients outside the hospital ward.

And who would have imagined that the ubuquitous but unnoticed straw would one day become a contentious item? And central to the fight for disability rights too? Well, that day has arrived. The issues are explained here: "I need plastic straws. Banning them puts a serious burden on people with disabilities".

Fever, stress, vomit. Life.

True, we might have gotten bored with the same old, same old. I mean profound developmental impairment both physical and cognitive, central fevers, cortical blindness and refractory epilepsy. They might not have kept us on our toes. So, it's understandable that we've now had retching and vomitting added to the mix.

It's been nearly two weeks since those new symptoms debuted. Last week's exam by the pediatrician didn't explain them so we did blood tests on Monday. We also consulted by email with the neurologist..

The pediatrician - who has kindly acquiesced to keeping C. on despite her nearly 23 years - thought that perhaps one of C.'s anti-epileptics is the culprit. The one added on last is Vimpat/Lacosamide. I recall that the neurologist who saved her from a near-coma during her last hospitalization said then that her Vimpat dosage was too high. But he ultimately left that unchanged because she recovered just by his removal of several other drugs that the hospital team had been administering her.

But the neurologist treating C. now - a member of the above hospital team - dismisses that theory.

The Hubby and E., the caregiver, are convinced that phlegm is to blame for the vomiting. But I'm not. She just shows no signs of phlegm, doesn't have a runny nose and isn't coughing. Besides, she's never vomited from a cold in the past.

We also emailed the gastro who had prescribed Carnitine to repair her liver when it was damaged by the Valproic Acid medication. She's been taking 250 mg. of it three times a day for over a year. Then this past weekend we went away for 24 hours with the entire family. I forgot to pack in the Carnitine and C. did not seize, vomit or retch the entire time.

So I hoped we'd found the cause. But now, after receiving the gastro's OK to stop carnitine, it's clear that was wishful thinking.

So the mystery of the vomiting remains.

C.'s Central Fevers have also increased in frequency and are stumping everybody as well. Her neurologists don't seem to have experience with them at all. In fact, while C. was last hospitalized one of the doctors posited that we have it all back to front: the seizures are inducing the fever.

Now, that's such a presposterous hypothesis, I can't decide where to begin debunking it. One, C. also has seizures without fever. Two, when she has a fever with seizures, they stop once the fever is lowered. Three, central fevers are a known phenomenon. I didn't invent them. But nobody on the team was listening.

I'm hoping that our neurologist will be able to track down a doctor who is familiar with central fevers. We plan to meet up with her for a face-to-face consult next week, something I'm not eager to do since the last time we spoke, in the hospital, I sensed she wasn't enamored with my ideas. (They tended toward pleading for lowering the meds, asap).

In the meantime, it's just stress, stress and more stress. Here's a self-portrait in the works that illustrates that.

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.

Baby-steps

At last, C. is out of bed and able to be wheeled
around the ward

Well, this discharge is proving elusive.

Saturday was pretty disappointing with C. extremely sleepy, even lethargic. The IV fluids were discontinued in the afternoon to test her capacity for drinking and eating enough by mouth. It didn't look promising, as she clamped her mouth shut in response to the spoon and bottle.

Nightime brought the icing on the cake - copious vomiting immediately after a small meal I'd coaxed into her.

The junior neurologist ordered blood tests and a chest x-ray without delay although C. had no fever or cough. She didn't say why. But our next-door neighbor, a pulmonologist, later explained that it was a routine procedure to rule out aspiration of vomit. It would have been nice for the neurologist to note that.

I went home to sleep, consumed by guilt over her vomiting - which everyone intimated was my fault. (While the hubby stayed in the hospital as he's been doing every night. Yes, I know, he's a treasure.)

The following morning I awoke to a WhatsApp message from him that read like a hallucination:

"The resident for this coming week is Dr. J.K. He was here now with Dr. L. and Dr. M. in tow [two more junior neurologists who have been treating C. until now]. He agrees with everything you say. He's immediately taking her off Phenytoin as the first step in reducing the drug regimen and getting her out of the current apathy/lethargy. Next culprit in his eyes is Vimpat of which he says she's getting too much for her size."

C., now off Frisium and Phenytoin, has been progressing in baby steps. With my maternal magnifying glass, though, they appear as giant leaps.

For instance, she's begun opening her mouth for food. And when we wheeled her around the ward tonight for the second time, she lifted her head more frequently than she did last night.

And, of course, equally significant, the seizures are infrequent and mild, thank heavens. But you know the score: keep that one hush, hush.

This godsend of a neurologist departs the ward at the end of the week for overseas where he works over the next three weeks. He's promised to be a consultant for C.'s care via her regular neurologist - who was his student once upon a time. But it remains to be seen how that will pan out.

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 2

Doing the EEG

Written Friday, November 10 (but posted on November 11)

Well, 20 hours later we are ensconced in the ER and, true to form, the medical team is swamped. In the last eight hours not much has been done for C.

Here's the sum total of intervention thus far:

1. An EEG (see the photo). No surprises. The usual haywire scribbles.
2. Two rounds of Valium IV (intravenous). Result: Longer intervals between seizures and an "out" C.
3. Keppra IV - even though she received her morning dose before we got here. No clue as to reason. Didn't bother asking.
4. First dose of Vimpat. The neurologist intended to start her on that but she later learned that the hospital's wards had none and that the hospital pharmacy was closed. She notified us she'd give something else instead of Vimpat when I remembered having purchased some a year ago. The neurologist had first given us a prescription for it - to replace Valproic Acid - but then nixed it at the last minute. She had discovered it poses a slight risk of liver damage. Not something we needed again. But I had already bought the stuff so I stashed it away in my old-drug closet.

So I brought it from home and C. has had her first dose of Vimpat. Sometimes it pays to be a hoarder.

She's had only one meal today. The rest of the day, due to her seizures or her Valium-induced sleep, eating wasn't feasible.

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.

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.

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?

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.

In real-time, a trying night

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

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

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

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

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

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

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

Now disabled *and* chronically ill (Part 3)

C.'s elbow and the latest sore

[Written Sunday, November 13]

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

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

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

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

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

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

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

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

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

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