Lots of Updates on Ezri

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This year has really flown by for our entire family. Up until now, Ezri has done fantastically in her first grade class. This is not to say she’s functioning at a ‘normal’ level, but she continues to make progress with her school work and grow socially. School is still hard, but she is reading simple books (she’s actually reading! and before Christmas was tested as knowing all her kindergarten sight words), is able to add single digit numbers and can (mostly) count to 100 (math is always hard). Considering at the start of kindergarten she could barely count to 10 on a good day, we can’t complain. She continues to say all sorts of funny things that remind of just how much is really going on inside that head of hers and how much she is so often unable to communicate to us. She also finishes our sentences all the time and when we forget words, knows them in an instant, and I find it so amusing as SHE’s the one with the speech delay.

As for seizures and emotional disturbances/episodes, she has been pretty stable. There have even been times when I have even wondered if her staring spells had disappeared because there had not been one that was noticeable to me in such a long time (of course I often only see her a few hours of her entire day, and they are subtle enough most people don’t see it). However she had a cold a few weeks back that increased her emotional difficulties and I definitely saw the (brief) staring a few times again. What we deal with for the most part is exaggerated emotions. Ezri can be super lovey and happy, like a gelastics happyness/giddiness, and she can cry at the drop of a pin like her heart is broken because of some idea she got into her head that isn’t even true (and if it was true, it definitely wouldn’t be worth crying over). She gets angry over things that don’t matter as well, things that she shouldn’t be angry about and normal kids wouldn’t be angry over, but she doesn’t quite have ‘rages.’ Instead, she sort of stands there with an angry, crumpled up face, maybe saying “I’m mad so and so……” and gets herself through the feeling. It can take seconds or minutes. She obsesses over the color purple and most people think she’s being cute, but it’s ‘too much’ if you know what I mean. This morning I was sitting on the chair and she came over to hug me and be sweet (it was that exaggerated lovey emotion). She then turned around and saw her sister and her brother sitting on the bed with Daddy and immediately got angry that there was no place for her to sit. It didn’t cross her mind for one second that she could ask them to scoot over so she could sit and absolutely remarkable how in less than a second she went from so giddy-happy to so angry. That’s exactly HH for you.

We have not seen the neurologist since August, which is probably the longest ever in Ezri’s lifetime. We forgot to make an appointment when we left the VEEG in Aug, they haven’t called with a reminder, and from our side, nothing has really changed so it didn’t seem so pertinent (though I do still need to make an appointment for a general neuro check up soon!). Ezri’s meds have been mostly the same. Halfway through the fall, after thinking on it some I decided to very very slowly start tapering her Keppra. We had taken her off cold-turkey for the last VEEG and nothing abnormal (except some slowing) had shown up on top of the fact that her typical HH episodes did not increase dramatically (some, but not dramatically) so I questioned how effective the Keppra still was for Ezri. It dramatically decreased the staring spells when she was 2, and on various other VEEGs, taking her off the Keppra had increased the spells, but this time it didn’t so much. Not only that, the VEEG ~2 years prior and 6months post the last surgery was epileptically abnormal whereas this one wasn’t. Maybe her brain was healing some? Starting about the beginning of November we have reduced Ezri’s Keppra from 10ml (1000mg) bid to 7.5ml (750mg) bid. Reducing a half a ml a month, sometimes going a couple of months at the same level (very, very slow!).

We have also had to make changes with her Lupron medication this year as the 3 month version she needs became completely unaffordable, even with *good* insurance and deductibles paid. She came to need a 30mg/3month version of this medication that retails for about $6,000 (so she wouldn’t have to get a shot every month – I’m not sold on the implant yet, and Ezri has learned to tolerate shots pretty well). Administered at the hospital, with a 90/10 plan left us with a cost of $600 every 3 months. Now, on an 80/20 plan, it’s a cost of $1,200 every 3 months. You would think there would be some forewarning about a medication cost that high, and not only that, the billing cycle for the first shot didn’t bill us until after the second shot had been given. Luckily, with some research, our new insurance, unlike the old one, allows us to get the medication from a specialty pharmacy (mail order) and since it’s an outpatient pharmacy charge rather than a hospital charge and a tier 2 medication, it only costs us $50 and is shipped overnight with free shipping straight to our front door. Can’t beat that–or the time saved of not going to the hospital. With my newly learned skills in nursing school (learning to do injections, etc.) and some helpful one-on-one training by the clinic nurse, I am giving her the shots. It is turning out to be in the least restrictive and most relaxed environment (doing it at home) that we could give her. So out of a really unfortunate situation, something very good has come.

Another appointment we had in the fall (Oct) was with Ezri’s neuro-opthamologist. She was diagnosed with optic atrophy, something that the doctor had been looking for each time we had been previously, but Ezri had been unable to sit still enough for her to see. And it means that her optic nerves are a different color, meaning they have damage, than they should be. People with optic atrophy characteristically have visual field loss and loss of color distinction, however because Ezri’s damage (from her 3 OZ surgeries where they had to dissect around her optic nerves/tract) occurred so early in her life she has basically healed and has no issues that the doctor can find. This is not to say that there aren’t or never will be, but for now her sight is quite good.

There are probably a million more things I could remember about this year, but this is more than enough for now – I hope all of you are doing well!