I (finally) wrote a friend a handwritten card this week and sent it via the United States Postal Service. It was LONG overdue. So much so, that I was almost too embarrassed to send it. My message included citing a personal flaw: sometimes in my desire to do something perfectly, I end up doing nothing at all. Do other people do that? As I texted another friend yesterday, how would she know I was thinking of her if I never said so? I can’t expect all of you to be able to read my mind. (Except my husband. He won a game of Hangman the other day after guessing only two letters of a long sentence.)

First update: Medically-speaking, Evan’s surgery went well other than several failed attempts to get an IV. Beforehand, I’d reminded the anesthesiologists and the surgeons that if malignant hyperthermia commences, their cooling fans wouldn’t be effective unless they wet Evan’s skin first. (The ichthyosis diagnosis had been forgotten once “malignant hyperthermia” entered the picture.) Crisis averted. Post-op, I think everyone breathed a collective sigh of relief.  I even managed not to become a weepy mess! I give credit to the comic relief provided by watching our baby on an LSD trip Versed. Afterwards, he wasn’t so happy; I was glad I came prepared with his blankie and a warm bottle.

Second update: I didn’t mean for it to take so long to write about my appointment with the specialist in Philadelphia; I needed some time to process what happened (and unpack!). Lately my body hasn’t been behaving as I’ve expected, so there’s that (hello, holidays!). And then my website crashed right after I launched it (never ever happens, according to my brother). So… I’m sorry; I’m sorry; and I’m sorry.

I used to think that being prepared meant I could protect myself from the unexpected. While that may be true in the realm of my children’s road trip needs, there are some things you really can’t prepare yourself for. I’d envisioned a back-and-forth dialogue between the doctor and Todd and me. He’d ask questions, I’d answer and vice versa. So it threw me a bit when we sat down in his office and he opened up by saying, “Go ahead…”

I was immediately flustered. What did he want to know? Was I supposed to start from the beginning of my life? Include every detail or just the things I thought might be related to periodic paralysis? Was I saying too much? Too little? Todd interrupted me; I interrupted Todd as we both reminded each other of things we’d forgotten. “In 2003, wait… earlier, when I was 16.” We were all over the place, and I was worried we sounded crazy.

When I checked in with the doctor for guidance, he was reserved, giving little feedback. In another life, he should be a professional poker player, I kept thinking. As someone whose business depended on building immediate report with all types of people, I could not get a read on him. That means he’s smart. It’s like when I want an honest answer from my 7-year old, and I don’t want her to know the truth hurts. After two and a half hours of Todd and me talking, the doctor taking notes, and Todd shuffling through my massive box of medical records, it was the neurologist’s turn.

Have you ever had a conversation start and wonder where the other person was headed only to end up at the expected destination? Well, that’s kind of what happened. After an hour, I had almost all my questions answered without me asking them. He explained why I need which medications and is confident on how to move forward. I got no warm and fuzzies, but that’s ok. That’s not why I’m seeing him. I’m seeing him because he’s an expert on an extremely rare condition that I have and his plan is going to give me the best chance at a better quality of life.

I understand periodic paralysis better because of him, and that’s important. Here’s what I learned:

1. Attacks of weakness and full-paralysis have caused scar tissue to form in the fibers of my muscles. There’s nothing that can be done to remove it, like any other scar in the body.
2. It’ll take time and a lot of patience, but he’s had patients improve dramatically on the right therapies (wheelchair-bound to walking). More on dramatic swings in physical condition later…
3. Service dogs can be of incredible benefit to people living with periodic paralysis. He has seen them in action firsthand do things he would not have believed them to be capable of doing. (He’s a self-proclaimed skeptic.)

The doctor also explained which medical therapies I need and why and how they work. For nerds like me who like to know the nitty gritty, I need some combination of these four medications (of undetermined quantities):

1. Extended release potassium tablets (taken at regular intervals).
2. Liquid potassium (when an attack could be imminent).
3. Carbonic anhydrase inhibitor (to help prevent more muscle scarring).
4. A potassium-sparing diuretic (to help my body hold on to potassium).

That’s an overly-simplified explanation. The doctor will focus on changing one at a time. Since #3 is the most important, we’re starting there. If you’re wondering what “carbonic anhydrase inhibitor” means, I can’t tell you. I’ve tried multiple times to understand it, and I just don’t get it. In pharmaceutical terms it’s a medicine called Keyvesis; it’s only been FDA-approved to treat periodic paralysis within the last year, and it costs $90,000. (Thank you, insurance!) The side effects are AWFUL. He’s starting me on it SLOWLY. I am to crush up a tablet and lick a little off the dip of one finger until I can work up to a full tablet. I’m not kidding. It’s that bad.

I won’t lie. I think the next 3-6 months are gonna be bad. I’ve been playing with the timing of taking this medicine. (i.e. “Do I want to ruin my morning or ruin a good night’s sleep?”) The kids need me. The house needs me. I’ve been wearing the same clothes for two days, and there are zero Christmas decorations up. One day at a time. I think if I can tough it out, six months from now, it’ll all be worth it.