No help today, so I was on my own with the three of them. Of course nothing got done around here, and the only reason I'm even typing now is because S came home early from work to "save" me and the girl from up the block whom I pay $5/hour to keep an eye on Smacky on Friday afternoons while he plays with the neighborhood kids showed up early. For now I'm in bed while the littles are napping next to me. I thought about flipping on the video monitor and heading downstairs to do some dishes, but...nah. I need a break. That and I need to talk something out - something serious.
Lest I bore you to tears, I'll try to summarize the situation with my health as briefly as possible:
My blood counts have been dropping again. For those of you new to my site, I've had this for 12 years now. Until a few years ago, the only cure was a bone marrow transplant, preferably by a sibling donor. Neither of my siblings are matches. At this juncture, BMT science has not perfected a "safe" BMT (or stem cell transplant - for our purposes here the terms shall be used interchangeably) for a related imperfectly-matched donor. In the same breadth, perfectly-matched non-related donor BMTs are quite dangerous, with a success rate as low as 50%. When I say success rate I'm referring to the number of PNH patients who die after their transplants. So for that basic reason and many more emotional reasons, a BMT is out for now. I have Smacky's umbilical cord stem cells frozen for use when medicine creates a more optimistic outcome.
For now, my overall health is actually okay. Despite my blood showing a return to my classic PNH counts which are consistently below normal (hematocrit hovers around 31, WBC 3, hemoglobin 11), my marrow is still riding high from the 10 units of blood I received back in June during the horror we refer to as the second childbirth. I'm feeling pretty all right but the PNH has been creeping back - I know this will not last. And my family needs me to feel good. Really good. Especially now.
There is an option beyond regular blood transfusions (which introduce antibodies, making a future BMT riskier) or iron/folate therapy. A new drug, Soliris, was approved by the FDA early this year. Soliris has worked wonders in clinical trials in most PNHers, rendering them essentially normal, healthy people. Soliris has to be transfused and I believe the schedule averages out to once every three weeks, which ain't a thing considering the huge health benefit it brings. Soliris took PNH from being a scary, terminal illness to a scary, treatable illness. And when the FDA approved the drug in March 2007, we PNHers yelped in glee. We were joyful and grateful for having our lives spared, until we learned of the price of the treatment:
$750,000 per year.
Allow me to repeat that number: $750,000.00.
Yes, those zeroes are correct. And yes, a PNHer has to stay on the treatment for as long as he or she continues to cheat death.
I'm going to keep my soapbox safely tucked away for the moment but can't rule out a rant in the near future about ups and downs of capitalistic medicine. I get that the pharmaceutical company (American, who for now I won't name) needs to please its investors and needs to recoup the millions they put into research and production. Yes, PNH is a rare disease and we survivors number only in the thousands, but given that it extends our lifespan and we'll need it for however long it's as good as they claim it to be, can't they make their money over the longterm? It's hard to not to feel blackmailed, cheated, and held hostage by this company. Our quality of life is determined by their bottom line. And it's hard not to imagine some newly-rich board members and VCs strutting like roosters during their quarterly reports.
Lifetime caps for health insurance coverage are $2M. Let's assume I've eaten up a good, oh, say $400,000 of my benefits to date. That leaves approximately 1.6M left to play with (for as long as we continue to be insured with Big Blue Ubiquitous Health Insurance Company). At the current rate, we'd eat up what's left in 2 years.
After that, I'd be uninsured. The options? S or I find another job somewhere that offers a plan with a different carrier, or, find one on our own and pay the ridiculous premiums out of pocket. Neither option is good or workable at this time...the situation would bring considerable hardship on our family.
Dr. Cutting Edge and I planned on just a short term of Soliris for me to make my delivery safer, but shelved the idea after the hospital he was then working at (and I delivered) refused to order the medicine, citing that insurance would never agree to pay for it and they didn't want to get stuck with that kind of invoice on their hands. Even if it meant my life. And so our plan to start it right after the twins' delivery was scrapped. And instead of being spared an agonizing recovery, my PNH sent a mild preeclampsia spinning into severe territory, rendering me in the ICU with failing kidneys. Soliris would have put a stop to all that. I wouldn't have hemorrhaged, I wouldn't have clotted, and my kidneys wouldn't have been doubly compromised.
Not long after, Dr. Cutting Edge announced his plans to leave that hospital. He'd never tell me, but I often wonder if what happened with me was part of his decision. He now has his own unit at another teaching hospital - at the hospital I first started seeing him at 8 years ago. I saw him the first week he was setting up shop there - we had a quick appointment and talked about how my counts were going back to doing their old thing.
Yesterday I got a long phone message from D, my NP. We've been talking about you, she says. And as it turns out, this new hospital? Has a program. A program that gets patients the medicines they need when their insurance companies balk. A program that works to keep the treatment costs from disrupting the lives of the patients. They're not afraid to order Soliris, and we need to talk.
Holy.
moly.
I've returned the call - had to leave a message.
And I'm waiting to hear back, a nervous wreck. This is huge, people. I could be like, better. Soon.
God.
Waiting. It's that kind of day.
