Hope

It's a funny thing, hope. It's something you don't realize how much you're missing it until it comes back.

Tuesday of this week we took my wife to a specialist in PH at the University of Chicago Hospitals. Working with a doctor who actually knows what she's talking about is a  pleasant experience. It is clear that the cardiolgist in Peoria is essentiall out of his depth with my wife's treatment. For example, unbeknownst to him, a couple years ago the name of my wife's disease was changed from "Primary Pulmonary Hypertension" to "Idiopathic Pulmonary Arterial Hypertension," by the WHO. The name change is significant for two reasons: first, they broke out two new causes of the disease, a genetic tendency "Familial Pulmonary Arterial Hypertension" and PAH caused by the HIV virus (not, to be clear, a complication of AIDS, but a separate condition caused by the same virus). Additionally, by changing from "Pulmonary Hypertension" to "Pulmonary Arterial Hypertension," it separates out people with hypertension in the pulmonary veins, which is a consequence of left-side heart disease.

It is also clear that he is not familiar with the newest treatments of her disease. He didn't want to talk about sildenafil or other new oral medications (which I'll discuss more in a bit). He banned her from exercise, which the specialist reversed (though she's absolutely not allowed to lift anything heavier than ten pounds, or exercise with more than two), encouraging her to swim laps. My wife may also be put onto a trial odrug, and she's in a study to compary high-resolution CT scans to right-heart catheterisation. Just being able to talk to someone who knew what was going on, not just how to treat the disease but also was knowledgeable about the disease itself was very reassuring.

I'd like to point out that this isn't a criticism of the local doctor. Given the rarity of this disease, one or two new patients per million per year, and there's probably between three hundered thousand to a half-million or so people in the area that would come to the local hospitals, and any number of cardiologists in the area, my wife may be the only IPAH (or, if you prefer the older acronym, PPH) patient he's seen. Additionally, there is a significant amount of research going on right now, and several new oral medications that have only been approved in the last two years, including the very recent approval of sildenafil, so it's to be expected that he wouldn't be up on everything. Also in his favor, the doctors in Chicago were very pleased at how thorough his testing was. The only tests my wife ended up needing were an HIV test (he didn't do that because she tested negative a couple years age when we bought life insurance) to rule that out, and a lung-stress treadmill test that isn't available in Peoria. She's also on a low-sodium diet (to minimize water retention), off the calcium-channel blocker (for now), and has had her diuretic cut in half.

In the mean time, my wife goes back to Chicago on Monday for another heart catheterisation test (one of the numbers in the Peoria test was either unclear or inconsistent with the rest, so they need to re-do it to see if she can benefit from the relatively inexpensive calcium channel blockers). At that point, she'll be put on the real medical treatments for this disease, and well try to find a combination of drugs that work.

As far as survival goes, it's an open question at this point. Most of the numbers I've tossed around in my LiveJournal here are out-of-date. As recently as the mid 90's PPH (now IPAH, if you've been paying attention) was a literal death sentence, with a mean life expectancy under three years. In 1995 the drug Flolan was approved for treating PH, and survival times increased dramatically. In the last couple of years a number of new, oral medications (Flolan is continually injected by an automatic pump by a catheter that feeds into a major vein) that can be used before Flolan becomes necessary. At this point, these drugs are too new to know how they will affect long term survival (some people on Flolan have survived since the drug became available; it dramtically changed how we can look at the disease, but it's neither a cure nor that effective in all patients). Most of these drug were approved on an "orphan drug" basis, and several (sildenafil, for example) are re-packagings of older drugs, so while the safety isn't a big issue, long term studies haven't been done yet. These drug are also hard-core expensive, literally more than we make in a year, so it's a damn good thing we're insured. We will definitely change the numbers on the precription drug group rates, though.

The above paragaraph is to say the we have, now, hope for a reasonably long life; my wife may live to retirement and beyond, but she also may not. Everyone responds differently, so while we have a real hope now, we don't even have odds. That's hard, too, though in a different way than despair.

PS - I haven't checked my friends list in about three weeks, since I went off to Champaign for training, so I'll try to get around to that later today.