As good as it gets

Happy birthday, Haya

I suppose it's time for an update on Haya's Keto diet and her epilepsy. I've procrastinated because there has been nothing good to report.

But today is Haya's birthday so the timing feels right.

We did finally achieve a ketone level which satisfied our dietician. But it wasn't accompanied by the greater seizure control we've been hoping for. She's still having around four big ones daily. She's also been having around an hour's worth of twitching every day. I've begun giving her Paracetamol to zap that. 

I sent a video clip of the twitches to the neurologist who responded that the Keto diet is ineffective against that symptom. And we may just be stuck with those four or so seizures every day. 

What is more disappointing, though, is that Haya hasn't shown any improvement in her functioning at all, despite the drop in seizure activity.

Worse, her walking has even deteriorated. Her stamina for it has diminished and since we can't have her feed herself Keto meals lest she drop or drip precious grams of the stuff, she's left doing nearly nil. 

Fortunately, she still floats and flips in the pool as well as she used to. But she used to kick occasionally and that's disappeared.

I've even put her Speech Pathology sessions on hold because they would probably be a waste of time and money. After two months of foot dragging, our health fund (the kupat holim of which we are members) finally decided on awarding us twelve sessions at NIS 123 participation per session. That means they are covering roughing half the cost.

For now I'm just persevering with Haya's walking. I even did it twice yesterday.

Eventually, we'll need to reassess the diet and decide whether it's really worth all the effort.

Along with this bleak reality came the following article [online here] via my Google Alert. It hails from "New paradigms for the treatment of pediatric monogenic epilepsies: Progressing toward precision medicine" by Nicola Specchio, Nicola Pietrafusa, Emilio Perucca and J Helen Cross. And published in the latest edition of "Epilepsy and Behavior"

And is it ever blunt and bleak.

It's laden with equally stark statistics about seizure control in children with developmental and epileptic encephalopathies (DEEs) which is what Haya has been "blessed" with.

Here's its opening paragraph:

"The past 30 years have seen the introduction... of over 20 second-generation antiseizure medications (ASMs). Despite this enlarged pharmacological armamentarium, seizures in about one-third of people with epilepsy cannot be completely controlled and outcomes are even poorer for certain syndromes, such as developmental and epileptic encephalopathies (DEEs)"

That's what Haya is "blessed" with.

"One possible reason... is that the large majority of currently available ASMs have been developed with the aim of suppressing symptoms (seizures), and were not designed to address the specific etiologies and mechanisms responsible..."

It goes on

"Treatment responses in SCN2A-related epilepsies appear to be more complex... for children with later-onset epilepsies [like Haya] sodium-channel blockers were rarely effective and at times even worsened seizures... patients with later-onset epilepsies [again, that's Haya] had truncating mutations, which were associated with lack of seizure improvement after administration of sodium-channel blocking ASMs."

And 

"For most of the severe DEEs... the overall prognosis... remains poor in terms of seizure control, intellectual disability, and other comorbidities."

There's even a chart which makes that bad news perfectly clear: For Scn2a Loss of Function Mutations in Nav.1.2 ASD/ID and Childhood-onset seizures, the chart bluntly says 

"We Do Not Currently Have Targeted Precision Treatments."

But hang on.

I may be stretching it but I believe I see a silver lining: It means that Haya's awful predicament probably isn't due to our having missed something. My husband and I aren't to blame.

Given the awful hand she's been dealt this is "As Good As It Gets". I know, I know, that's a small comfort - but a comfort nonetheless.

Happy birthday, Haya!

Words my murdered child left behind

Every year at Yom Hazikaron time (it begins at sunset today), I comb through my Malki's last diary for an excerpt to publish. It isn't an easy task.

First, it breaks my heart to read her words. And second, it is an eyestrain because her script is miniscule. 

She tried her utmost to milk that pocket-sized diary for all it's worth. Details of nearly every day of her last year of life are recorded: youth group activities, quotes of her friends, tallies of hours studied for an exam, help she gave me in caring for her severely-disabled little sister, thoughts, worries. and records of every single terror attack and its victims. 

When the pages didn't suffice, she would glue on strips of paper to enable her to complete the account. It's in the photo on the right. 

Just as she squeezed every bit of writing possible into that diary, so too she squeezed into every day of her short, special life the experiences and deeds that spread joy and pleasure to everyone she encountered, whether through her words, her artwork or her flute and guitar playing.

What a precious girl she was.

And here below is a photo of the new diary she had just purchased and dedicated shortly before a Hamas terrorist, Ahlam Tamimi, cut short her life. Malki was carrying it in her backpack on that sunny, August afternoon. 

The condition of the diary, which is here beside me, demonstrates the brutal force of those 10 kgs of explosives Tamimi used to execute the Sbarro massacre - the terrorist outrage she has publicly called "my operation".

In the diary entry of June 11, 2001 to which I randomly turned today and which I've photographed, she records something I remember her telling me that had touched her. She and her older brother, Shaya, went to buy themselves new glasses - and independently selected the identical frames.

She ended that day's entry: 

"I studied all day. I didn't go to the snif [a Hebrew word that means her youth group's hangout]. I went in the middle on a tour with Mommy. And that's it."

I don't recall that tour. Nor can I imagine where it was. I will never know.

Institutionalized duping

It's both distressing and disappointing that so many well intentioned and intelligent people have jumped on the ADI bandwagon.

To advance their own worthy goals, they partner with an enterprise that discriminates against people with disabilities, separates them from families and communities and denies them their most basic human rights.

When uninformed donors are duped by ADI's incessant mouthing of the words "inclusion" and "respect", we can, to a certain extent, forgive them. After all, duping is what ADI's PR teams excel at. Donors are led to believe that their dollars are actually benefiting the residents locked up in ADI's institutions.

But when researchers and academics fall prey to ADI's misrepresentations, it's harder to excuse.

One such ADI partner is an esteemed researcher from Ben Gurion University. In this clip Simona Bar Haim, Executive Director of ‘The Negev Lab,’ explains the vision of the new Translational Research Center at ADI Negev-Nahalat Eran.

"Through partnerships with Ben-Gurion University, the Shirley Ryan Ability Lab, Johns Hopkins University, the Sheba Medical Center, the Weizmann Institute, and the Irbid National University in Jordan, researchers at The Negev Lab are studying the trauma and recovery of everything from car accidents to COVID-19," she tells us, adding: "In addition to enhancing care at ADI Negev-Nahalat Eran through the immediate implementation of research findings, the research will also be shared with hospitals across the country and around the world to improve global best practices – changing the face of rehabilitative care in Israel and beyond."

The accompanying text refers to ADI's large, closed, isolated institution as "a home and family for more than 150 children and young adults."

The violation of their rights is mentioned nowhere in her presentation.

For those of you who are interested in that aspect of institutionalization, I recommend speaking - as I have - to parents of children who have been abused in closed institutions. 

Recently, one such mother poured her heart out to me while I overheard her cognitively-impaired adult child, whom she had returned home hours earlier, saying repeatedly, "He hit me! He hit me!"

Guilt-free institutionalization

I mention this video below

It must be wonderful to banish any trace of hesitation or guilt about drastic steps you've taken with your children. I would have thought it was impossible, to be honest. But apparently I erred.

There are parents who relinquish their children to strangers. For the rest of their lives. They do this despite those children's inability to ever relay their experiences in those strangers' care. 

It doesn't get much more drastic than that.

But some of those parents go even a step further. They replace the hesitation or guilt that you'd expect, with pride. They convince themselves that doing so was in their child's best interests. They literally boast of evicting their vulnerable children, even babies.  

They are either ignorant of, or dismissive of professional warnings about the evils of institutionalization. 

I once had a friend whose youngest child was about the same age as my daughter Haya and similarly disabled. When her daughter was five years old, she evicted her. I recall her relating to me that the caregivers at her daughter's residence maintained that the child cried whenever her mother's  left after a visit. 

My friend's reaction? "They're wrong. I don't believe she is crying for me."

Another mother I view as delusional has cooperated with ADI in producing a YouTube clip plugging ADI institutions and parroting the lines used in their fundraisers. 

She extols the decision she made to hand her toddler over to the care of ADI employees. It has benefitted her son's body and his heart, she insists. It will give him the love and the confidence to try harder, to conquer the next steps. 

To be fair, Israeli society and professionals brainwash parents like us to believe that abandoning our profoundly disabled children is in their best interests . In a sense, like her son, that mother is a victim too. 

So, I won't provide a link to her YouTube promo of institutionalization. (That's a screen shot from it above.)

But for the curious among you, it was posted on ADI's Facebook page on Thursday, April 1, 2021.