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.

My misdiagnosis

Proteus mirabilis

I'm still wiping the egg off my face.

For the longest time, I've been carping about C.'s neurological fevers. In recent posts, I've noted their increasing frequency and severity. The Hubby and I even wrote to the neurologist about it last week.

But for certainty's sake we brought her to the pediatrician on Wednesday for a clinical exam.

He found nothing pathological. But our thorough prize-of-a-doctor ordered blood and urine tests nonetheless. If those were clear, he said, it would be safe to presume she's really got rising neurological fevers.

On Thursday, after the visiting home nurse drew blood, C.'s fever spiked to 40.2 Celsius (about 104.4 Fahrenheit)!

So despite the difficulty involved, the Hubby rushed C. to the local HMO clinic for the nurse to take urine via a catheter and then rushed the specimen to another clinic for transfer to the lab. (The visiting nurse, a male, refuses to insert catheters into female patients.)

It was immediately clear that C. had a urinary tract infection.

So within an hour, we'd started her on Cefuroxime. Since then, her fever has been steadily dropping. She's back to her usual slightly elevated temperature with an amazing, fluky 36.8 C thrown in last night! In Fahrenheit, that's 98.2. I can't remember her ever having a reading that low.

Her pediatrician surmises this infection has been simmering (and C. silently suffering, of course) for a while.

Today the urine culture results arrived: Proteus mirabilis

It's actually quite a work of art (above).

As for the moral of this story, I'm sure it's obvious. Never presume anything about our complicated children who can't convey to us what they're feeling.

Febrile fun and games

C. kicking really well during
last week's hydrotherapy session

C. has been quite unwell these days.

I always feel ridiculous describing her that way when the fact is she is always "quite unwell". But what I mean is she's been running fevers of over 39° C and vomited several times yesterday.

Needless to say, to cap it all off, there were seizures. But, oddly, the seizures weren't any more frequent than with milder fevers. In fact, they subsided without the lowering of fever.

So today she'll be checked by her pediatrician who will tell us whether she can go to hydrotherapy. Tuesday is the one day that our local therapeutic pool admits females. We are all eager to see her in the water, the only context where she flowers.

Last week, while back floating, she kicked vigorously with both legs for the entire session!

Still no word from the local Liberty Swing distributor who promised us he'd deal directly with the reluctant municipality.

And still haven't located an Occupational Therapist or a Speech Therapist experienced in cases like C. who gives receipts. Without names we can't proceed with the pilot project we've been accepted to. The idea is to provide C. at home with the services and therapies she would receive were she warehoused in an institution. Currently, in this country, she can't.

UPDATE: After a thorough exam today, the pediatrician has declared C. fit for hydro. While he couldn't find a cause for her fevers, he recommended doing some blood readings, testing her urine to eliminate UTI and having her elbow wound seen at the wound clinic to eliminate infection. He doubted either is causing the fever. If the tests prove negative, we'll be left with a diagnosis of neurological fever that has climbed higher than ever before. Did I really believe there were improvements on the horizon?

Frustration overflow

First a swing update:

The Hubby met with the local distributor of that enticing Liberty Swing I am trying to have installed in our city. Currently there are just two in this entire country, both in a city that's over an hour's drive from us.

The parks department of our municipality won't permit us to donate and install it in one of its playgrounds without first winning the approval of some committee. The first step toward that approval is a written request. But here was their response to the email I sent detailing what we'd like to do:

Equipment of this sort has never been installed in our city. We need to examine the technical details of this item of equipment and determine whether the municipality will be able to maintain this type of equipment. Only after examining the above topic and receipt of authorization from the relevant bodies will it be possible to install such a swing and to maintain it. We will be in touch with you in order to survey possible optimal venues for installation of the swing.

To be clear, Liberty Swing's website lists 215 such swings extant throughout the world - excluding the two in our country. So we're not taking about inventing the wheel here.

But the distributor assured us the ball is now in his court. He'll deal with the municipality directly and hopefully move the project to fruition.

I'll still tackle the crowdsourcing, of course. We'll see whether he delivers on his promises.

C. is consistently unpredictable these days. We never know whether she'll hand us an hour or so of hard seizing or remain calm. If she seizes, we never know whether it will be accompanied by central fever or not.

When I put her brace and shoes on, I can't predict whether she'll stand erect, walk nicely, bending and straightening her legs. Or whether she'll just tilt her torso sideways and keep her legs stationary.

At mealtimes, I never know whether she'll place her spoon into her mouth independently and swallow her food quickly. Or whether she'll drop her spoon, need to be fed and then store each mouthful in her mouth for eternity.

Definitely not an easy period.

I've been writing locally about my pet peeve - warehousing children with disabilities - and growing increasingly frustrated with my failure to have an impact.

This country's largest chain of warehouse institutions is constantly sprouting new, greedy tentacles. It is partnering with a growing list of organizations, several of which claim to champion true equality and inclusion for people with disabilities. They should know better.

One of its many outrageous operations is a "prisoner rehabilitation" program whereby imprisoned criminals are bused several times a week to its institutions to interact one-on-one with people who have profound disabilities.

Some of the prisoners are serving substantial sentences. But the institution insists they have all been vetted for safety and even permits them to change out of their prison garb into civies during their visits. Yet they concede that the men aren't permitted to interact with female residents or with minor residents. Nor are they admitted to the hydrotherapy pool.

Hmmm. Sound safe to you?

My written inquiries have revealed that in all likelihood no other institution in the world "rehabilitates" prisoners in this manner. Two major prison service entities - one international, one local - assured me they have never heard of one.

If you are aware of such a program, I would very much appreciate your sharing details of it. I would also welcome any tips on how to foster de-institutionalization in countries that are resistant to that transition.

Like mine, of course.

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.

For good cheer, move on

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

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

Neither the blood tests nor her pediatrician's clinical exam revealed any alarming findings and he thinks C. could just have a virus. But he did notice two anomalies for which he's referred us to an abdominal ultrasound and x-ray:

• "Vertically-oriented abdominal mass to L of midline, partly pulsatile, probably abdominal aorta" (but he assured me it's probably so prominent because C. is so thin)
• "L-sided abdominal masses palpated, oriented vertically. Suspect stool masses" (and he assured me this is easily remedied with laxatives).

He wants us to do an abdominal ultrasound and x-ray before treatment. So, with Wednesday the earliest appointment date I could get for those tests, until then it's just wait and endure.

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

No silver lining in this cloud

C. wants a drink (explained at the end
of this post)

I haven't posted here for a while because caring for C. has been almost exclusively drudgery, hassles, worry and frustration. Since it's tough to relate the negatives while in the thick of them, I waited for respite to descend but in vain. So now I'll try to blog straight through this cloud.

C.'s pressure sore remains horrific. It's infected, deep, large and persistent. I cringe and shudder each time I re-bandage it. Our dear home visiting nurse instructed me to do that only every two days. But, while I still admire and appreciate him, I've switched to every day.

The makers of the ointment (flaminal hydro) advise to expose and examine the wound every day and to re-dress it when the stuff isn't visible any more. Since C.'s bandage fills with blood and pus within hours, this was a no-brainer for me.

But the sweet nurse seemed annoyed with my disobedience and my panicking. 

Next are the cannabis license hassles. I finally scored a real live voice at the Ministry of Health which told me our renewal license will be at our supplier immediately after the previous one expires.

But there's a major hitch: the dosage on the license - 40mg per kg per day - is way too low. In fact it's lower than the dose I've already risen to. And with the neurologist's approval I had intended to raise it further - perhaps as high as 60mg.

So why had the neurologist requested this dose? She hasn't responded to my email.*

C. is currently staging another of her hunger strikes (here's what I wrote the last time this happened), barely puts the spoon in her mouth and then parks the food there for an eternity. Each meal drags on for ages.

And, the surgeon who will be implanting her VNS (link) warned us that she absolutely must gain weight before the surgery. At this rate, she'll probably lose instead.

Then there are C.'s feet, swollen from fluid retention. She's had this problem before and I know it plagues everyone who spends most of the day sitting or lying down. It usually clears up quickly when I'm careful to keep her legs raised but to be honest, I've neglected to do that of late since they weren't swollen..

The central fevers have been manifesting frequently and C.'s walking hasn't been up to scratch, though I don't believe there's a connection between the two. She often stiffens her right leg and refuses to step with it. I'm forced to struggle to move it forward making the whole effort far more exhausting than usual.

School's about to end - forever

On Sunday, C. officially "graduated" the state's educational system. To mark the end of the academic year, her teacher threw a party. Photo on the right.

In mid-August, the "powers that be" will throw her to the wolves. After that, we have two options. We can either banish her from our home to one of those large, closed institutions that I'm always lambasting. Or we can send her to a "day center" as they're called here where only her most basic needs would be met by some very basic staff members. The staff/student ratio is 2:10 which, I'd say, tells you all you need to know.

Today we met with the school social worker again to discuss those bleak options and the possibility of receiving some therapies at home via our Health Fund.

Now she's a lovely, sweet, kind young woman with what she described as ADD. Her desk is coated with scribbled-on papers that constantly get blown to the floor by the fan. Every time we meet with her, she asks us the same questions, we give her the same answers and she promises to make the same inquiries to the same offices on our behalf. The trouble is she never does and we are always back at square one. I'm reminded of the film "Groundhog Day".

The lobbyist at our local advocacy NGO - with whom I spoke last week (link) - has promised to file a petition to the High Court demanding government funding for therapies and care at home, rather than in an institution.  Toward that end we are hunting for the requisite four other families interested in joining this class action. So far, we've got one. The school's head of therapies has promised to find us three more.

As you see, the good news is currently lying low but at the bottom of the barrel I scraped this up: we're having unseasonably hot weather and C. is always thirsty. I've noticed that whenever she hears me walking over to her, she opens her mouth wide for more water (see photo at the top).

Her mouth stays shut when I'm not in her vicinity. The Hubby and I consider that that a clear sign of some impressive cognitive calculations.

---
* I followed up our unanswered email to the neurologist with a phone call. She said she was taking care of the dosage problem. While she didn't explain why she'd originally requested only 40mg/day, she assured me that she'll now resubmit her request for 60mg/kg/day this time. Now for the bureaucracy budge.

Dehydration update

C. is well hydrated these days but seizing like her life depended on it  Lots of that "central fever" (as the doctors here refer to that neurological, asymptomatic fever) and, we presume, withdrawal seizures due to that sudden drop in benzo dosage which the neurologist ordered. 

Another cannabis update

Our medical cannabis license was just renewed by the Ministry of Health without a hitch.

Until the piece of paper was actually in our hand, I had this niggling fear that the bureaucrats might refuse. I even concocted a reason they might give: that there had been no evidence of a significant improvement in C.'s condition. I wouldn't have been able to argue with that.

But apparently they didn't delve that deep, and our med drawer is stocked with our first batch of the oil in the second year of its use.

Still no drama to report. Plenty of those "neurological fevers" that send C. seizing away until the Advil kicks in. Other days, just one or two seizures.

But the awaited neurological bonanza, the "lifting of the fog", still tarries.