In most of the articles I've seen and heard today, Harold Ramis dies of a rare disease, auto-immune vasculitis (correct) – and journalists have shown little curiousity to explain it.
I'm not a doctor, but since my wife, Ms T to you, suffers from auto-immune vasculitis, I thought I'd set down a brief explainer; I haven't seen many media outlets bothering.
We never saw it coming. Recently, she pointed out that in the January before the disease took hold, she managed 13 bushwalks in 30 days – all of them in the 5-15 km range, all rated “medium” or “hard”. Within 12 weeks of that glorious time, she was 32 kg, unable to eat or walk any distance, and (as it turned out) toxemic. Her once-d-cups were replaced by ribs.
What turned out to be auto-immune vasculitis had closed her celiac artery; this caused liver failure, which was manifest in stomach ulcers of horrifying severity.
The rest of the toll of the vasculitis, before it was discovered, was: a 95 per cent occlusion of her right carotid artery (with a thankful growth of “collaterals” that have actually kept her alive); the anterior communicating artery (in the head) doesn't show up on scans; one renal artery; one brachial artery in one arm, and a radial artery in the other.
The proximate cause is that the immune system rejects the blood vessels and attacks them. The resulting inflammation closes the vessels; and once closed, they don't come back. A vein taken from her leg keeps Ms T alive by feeding her liver; the others are gone.
The ultimate cause - what sends the immune system on a kill-its-host mission? - is unknown. Here's an observation: since 2000 the National Health and Medical Research Council has spent $86 million on quack medicines, and sod-all on auto-immune vasculitis.
And those who have read our travails over the years also know that in a serious case, the immune system has to be brutally suppressed. If corticosteroids don't work, the “gold standard” therapy is cyclophosphamide.
Now, chemotherapy has been the subject of a huge amount of research over the years, with two aims in mind: make it more directed to the disease it's treating, and reduce the toxicity of the side-effects.
Which has improved both the effectiveness of treatment and the quality of life for cancer sufferers.
Cyclophosphamide isn't one of the new, kinder drugs. It's a nasty, nasty piece of work. Developed in 1954, it's only a couple of generations away from mustard gas.
That's because this is a rare disease. If USA Today got things straight, Harold Ramis' presentation was that the vasculitis attacked the blood vessels feeding nerves, which Ms T believes would be even worse than her own suffering.
Prognosis? We hope that the cyclo dose doesn't kill, because without it, the vasculitis will.