Voting for the vapors

Last month, we brought my daughter C. back to the epileptologist who first examined her in September 2018.

At that first visit, this  doctor patiently recorded C's history as we related it. She then recommended several new tests and treatment options. We left her office feeling optimistic.

But once we tried to implement her advice, we hit snags. The primary one was our inability to contact her staff. They never returned calls or emails and without coordinating everything with them we couldn't proceed.

I was prepared to just forget about this epileptologist notwithstanding her superb credentials but the Hubby preferred to persevere.

So last week we expended a second round of time, energy and money to shlep back to her with C. As it turned out, the latter proved entirely unnecessary: the doctor didn't so much as look at C., let alone touch or medically examine her - even when she had a huge seizure in the office.

One of her concerns back in September was C.'s low weight. This time she asked us whether she had gained at all since our last visit. But she didn't trouble to weigh her on either occasion. C. might as well have been invisible and our efforts to bring her to the office were clearly unnecessary.

I was disappointed and annoyed. What do you all think? Am I quibbling?

This time around, her assistant did contact us a few days after the appointment, as the doctor had promised. She is instructing us on how to switch Cannabis CBD suppliers. This is a complicated process involving reams of paperwork required by the relevant government department.

The switch is necessary in order to procure CBD in vapor form which the epileptologist believes is absorbed more thoroughly than the oil C. currently receives.

To start with, we will divide dose between the two forms.

But this option was not the epileptologist's first choice. What she plugged most enthusiastically was surgery. She is keen to have C.'s twenty year old, inactive Vagal Nerve Stimulator (VNS) removed and replaced with an updated, more sophisticated version.

We had this urged on us three years ago by another neurologist: the one who destroyed Chaya's liver with Valproic Acid and then, when we notified her of the liver crisis, washed her hands of us. So, I'm sure you'll understand why I don't harbor any positive feelings for that option.

Back then, we even met with the surgeon himself. He informed us that the surgery would be "complicated" but doable. He too was eager, though he warned us that he would only proceed once C. gained some weight.

Before C. could oblige us that way, liver failure struck and the neurologist who had been touting the VNS surgery, as I mentioned, fled the scene. (After making the preposterous assertion that the liver failure had probably been triggered by the CBD and not by the Valproic Acid!)

Fast forward to last week's visit: I've had a couple of years to mull that surgical option and am far less enamored with it than I was then. C. has been through the liver ordeal and several severe urinary tract infections in the interim. What kind of candidate for surgery does all that make her?

So this time, I pressed hard on the brakes. "Thanks, but no thanks." I told the epileptologist. "C. will try the CBD vapors before we subject her to any complicated surgery." Or something to that effect.

Of course, the doctor tried to convince me of its "uncomplicatedness". But I wasn't buying it this time.

Here's hoping those vapors deliver C.'s ravaged brain a bit of respite from the decades of daily seizures she has endured.

On Status Epilepticus, the status quo and my status mentis

I've written these words several times in the past few minutes to family members: 

"I'm at my wit's end. We've had a full day of seizures with a brief respite of about two hours. Otherwise they are coming every four-five minutes."

That was me losing it on Thursday night. Much of Friday night saw me similarly frantic. 

Saturday's string of seizures was shorter, so I drifted closer to sanity. Then today, after a couple of seizures, C. managed to perform well in the pool for a full 45 minutes. Here she is in aquatic bliss as we approached the chair that lifts her out of the pool.

A few hours later, she also performed passably well for both her OT and her ST who met together for the first time to decide which sort of communication board/switch would suit C. best. They concluded that what would be best for C. is a large switch/button with a rough surface that can activate either music or a story. 

The ST promised to procure such a device for her via one of the local rehabilitation centers. Hoping this won't prove to be as long and frustrating a process as procuring a wheelchair has been.*

And since then, C. has accommodated us with a few isolated seizures now and then, occasional low grade fevers responsive to Paracetamol and otherwise her "normality"'. 

It's amazing what a few days of status epilepticus can do for one's state of mind. I'm now OK with her few daily seizures and very low level of functioning. Even thankful.

But we need to forge ahead with doing something about her status quo. We'll search further for a local CBD supplier who can provide it as an inhalant. The only one we've located so far only has CBD with a fraction of the strength of the oil we now use: 11%, while ours is 30%.

Unfortunately, we aren't receiving any meaningful assistance from C 's neurologist or her staff. Somehow, I imagine she'd be far more pro-active had we chosen the surgery route i.e. switching C.'s old VNS for a new one.

* Update: C. is still stuck in her old chair while we await our May appointment in that same rehabilitation center. We'll have to bring her there seated in her new chair via a special van provided by one of our local NGO's. The hope is that the seating specialist there will be able to finally solve the problems rendering the new chair unusable.

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.

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.

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.

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.

From liver toxicity to muscle spasticity

Artist: Edward Siebold (1829)

Onwards and upwards. I'm now post surgery and slowly recovering with the emphasis on slowly.

I finally had that gynecological procedure I've been mentioning on and off for one and half years. I'll leave it at that to avoid TMI.

The crux of it is I can't lift for a while, can't even turn C. over while she's in bed, have been awfully weak and have even had a low grade fever for the past few days. Today was the first day of semi normality, thank heavens.

So parents of children like C.: Beware! All that lifting and carrying isn't only dangerous for your back. Your pelvic organs could be victims too. Nobody ever shared that secret with me until surgery was my only option.

Our Australian darling of a caregiver has left us to resume the life of a recent university graduate in her late twenties with a boyfriend from these parts. We wish her much happiness but already miss her terribly.

Her replacement as a carer for C., a kind, experienced woman of 53, is stressing me out despite her good intentions. There seems to be a communication gap since English is her second language and we don't speak a word of her first. She also seems eager to take charge even when I'm around -- but is caring and sensitive towards C. so we'll just need to iron out the kinks.

Enough grumbling. We would never manage without her.

This week she and the Hubby brought C. to the gastro guy who specializes in liver diseases and who gave us some good news. "The first good news we've had about C. in twenty years" as the Hubby put it.

For starters, the gastro's report states that the "probable diagnosis" for her liver is Valproic Acid-Induced Liver Injury, although elsewhere he qualifies that by writing Valproic Acid is, in his view, the "DD (=Differential Diagnosis) with a fair suspicion of Autoimmune or Idiopathic".

So, a warning for any of you with kids on Valproic Acid: Be scrupulous about follow-ups and insist that the neurologist address any anomalies in blood test results. Also insist on tests for Ascites (there's an ultrasound to confirm and quantify that symptom) and any other fluid retention. Our neurologist had been dismissive of blood results that were red flags for our pediatrician. And she (the neurologist) never bothered to ask us about fluid retention, let alone examine C. for signs of it.

I would question one point that the gastro made in his report: "C. commenced a low salt diet and Aldectone 50 and Fusid 50 with excellent results". That's inaccurate in two ways:

• One, C. already was, and always has been, on a very-low-salt diet when her liver problems were first noticed. 
• Second, the results of all that Aldectone and Fusid (both forms of diuretic) were not as he described them. Unless you call rendering C. gaunt, seriously dehydrated and requiring IV fluids in the ER "excellent results".

The gastro guy also recommended a drug to remedy C.'s spasticity (I mentioned that here: "My daughter thinks she's a surfboard"): Baclofen, which he obviously doesn't prescribe since that's outside his field. He suggested we consult with our neurologist about it.

We did that, and she expressed surprise at the gastro's advice because this drug is known to pose liver risk. That, along with the info my daughter in law the clinical pharmacologist gave us, that Baclofen can exacerbate seizures - has left us in a quandary.

If any of you have ever given your children Baclofen,  I'd appreciate hearing what results you saw.

The liver plot thickens

The good news about C. is scarce these days.

She has still been signing "I want to eat" impressively (by putting her finger in her mouth) and putting the spoon in her mouth until her bowl is empty. But on every other score, she's not doing well. She does her daily MEDEK assisted walking with me - but not nearly as proficiently as she once did.

But most alarming are the awful blood test results which were unchanged today. And along with those, yesterday's ultrasound findings, the outstanding of which were abnormality of texture of the liver and mild to moderate ascites which the pediatrician referred to in his summary as "liver disease".

I can't recall whether I blogged about this but I did notice that C.'s stomach was enlarged and hard about two months ago. Inexplicably, and unforgivably, I brushed it off as insignificant or perhaps weight gain from the dietary changes I'd made on the dietician's recommendation.

There was so much else to tend to that shlepping C. to the pediatrician to have him examine her stomach didn't seem essential. After all, we were sending him photos of her pressure sores which were topping our list of headaches back then. And he didn't urge us to bring her in.

Besides, when we brought her to the wound clinic, she was examined and her fluid retention was declared "to be expected" and "to be ignored". The doctor doing the declaring was an orthopedist and we'd been scheduled to have the vascular specialist examine her.  But when I expressed doubt, the clinic nurse explained  that he was "unavailable". She assured us that this orthopedist was equally competent since all the doctors in the clinic were "one team".

The icing on this catastrophe cake is that after reading the ultrasound summary which we took the initiative of forwarding to C.'s neurologist, she promptly wrote back: "This is beyond the scope of my expertise. You need to talk to your family physician ASAP".

And then came the cherry on the icing: when we asked her whether she had submitted the request to the relevant government licensing office to raise C.'s cannabis dosage which she'd promised to do, she wrote back: "I would stop the cannabis now. There is a problem with her liver and no one knows if this is the cause."

Of course, it's more likely to be related to the valproic acid (which by the way she prescribed). But she isn't buying that theory.

Now I'm afraid she'll request that our license be rescinded.

Yikes: The VNS rep called already!

From a Howcast video

This VNS business is galloping ahead at break neck speed.

I'm used to medical matters that take ages to be resolved. Like my left-eye's cataract which has been rendering my vision progressively more blurry for three years. I am only now about to have surgery on it. (And that hysterectomy I was told I need which I've put on hold for now.)

In contrast, not even one week after our visit to the neurologist, the rep/nurse called to set up an appointment here at home. I am not psyched up for C. having another surgery just yet and feel that the powers-that-be are disconcertingly eager to implant the thing in her.

But as the Hubby reminded me, we've only committed to a chat; no knives or anesthetics or hospital stays involved at this stage. Still, I have a hunch that we'll be offered a date for all that which will leave us little time to  ponder this move thoroughly.

I suppose my reluctance to race ahead with this is also related to blessed calm C. has been enjoying since those four awful days last week.

Well, in any case, the meeting is set for Tuesday and I'll keep everybody posted.