Congressman Matsui (D-CA) Dies of Rare Disease

January 2, 2005 12:13 PM

Congressman dies of rare disease Congressman Bob Matsui, who was recently elected to a 14th term in Congress, has died due to a rare stem cell disease. Matsui, who was one of the leading opponents of President Bush's plan to eliminate Social Security, was the ranking Democrat on the Congressional subcommittee on Social Security.
posted by expriest (26 comments total)

Was he for or against stem cell research?
posted by painquale at 12:28 PM on January 2, 2005

Does anyone know what California's policy on replacing senators is?
posted by clockworkjoe at 12:28 PM on January 2, 2005

clockworkjoe,

California will hold a special election at a date determined by the governor.
posted by expriest at 12:29 PM on January 2, 2005

painquale : I thought precisely the same thing when I read the headline. ;)
posted by Plinko at 12:30 PM on January 2, 2005

Matsui was a member of the House of Representatives, not the Senate.
posted by billsaysthis at 12:34 PM on January 2, 2005

I believe Matsui was in favor of scientific research into Stem Cell based cures, but to be fair, I don't think the stem cell disease that killed him is related to the type that is under debate.

Matsui's disease effected his blood producing stem cells, which are present in human bone marrow and which create such things as red blood cells. So far, the "Christian" right has not found a reason to oppose bone marrow research.

The controversy is over embryonic stem cells, which are special early developmental cells which are capable of developing into pretty much any specialized cell in the human body.
posted by expriest at 12:37 PM on January 2, 2005

"The controversy is over embryonic stem cells, which are special early developmental cells which are capable of developing into pretty much any specialized cell in the human body."

Like replacing the marrow in your bones...
posted by Balisong at 1:08 PM on January 2, 2005

Balisong let's not use that logic. By that accord stem cell research can cure anything, when in reality it is a much narrow berth of diseases capable of realistically being cured by stem cells.

I've met a couple people with rare disorders and their depression lies in that nobody cares. There's not enough money in the research community to find a cure for a disease that only 50 people are affected with. Even if stem cells could possibly cure it, I really doubt they'd start or even ever explore this.
posted by geoff. at 1:48 PM on January 2, 2005

The Boston Globe referred to his disease as Milo Dysplastic disorder.

Someone needs to start a web comic starring Milo Dysplastic, protagonist.
posted by jesourie at 1:57 PM on January 2, 2005

someone dies, and your response is to crack a weak joke about the obscure name of the disease?
posted by foxy_hedgehog at 2:13 PM on January 2, 2005

See? God really is on Bush's side! He's whacking his opponents one by one!
posted by fungible at 2:17 PM on January 2, 2005

Foxy, jesourie wasn't making fun of the name of his disease, but rather the Globe's -- and/or AP's -- lack of editors. (If that's not clear enough, it's myelodysplastic disorder, not Milo Dysplastic disorder.)
posted by delfuego at 2:25 PM on January 2, 2005

someone dies, and your response is to crack a weak joke about the obscure name of the disease?

About 150,000 people die every day.

What is your response?
posted by mcguirk at 2:26 PM on January 2, 2005

About 150,000 people die every day.

What is your response?

McGuirk, I'm not sure I understand the point of your comment. I was responding to what I saw as an off-topic remark that trivialized the thread- and trivialized the disease that the subject of the thread died from.
posted by foxy_hedgehog at 2:32 PM on January 2, 2005

Foxy, jesourie wasn't making fun of the name of his disease, but rather the Globe's -- and/or AP's -- lack of editors. (If that's not clear enough, it's myelodysplastic disorder, not Milo Dysplastic disorder.)
posted by delfuego at 5:25 PM EST on January 2

jesourie, my apologies! I'll direct my bile elsewhere.
posted by foxy_hedgehog at 2:38 PM on January 2, 2005

According to the Merck Manual, myelodysplastic syndrome seems to be a stage before full blown acute Leukemia may be diagnosed.

"Myelodysplastic syndromes represent progressive bone marrow failure but with an insufficient proportion of blast cells [30% for definite diagnosis of aml; 40 to 60% of cases evolve into aml." - Leukemia, Merck Manual

"The prognosis is highly dependent on classification (see Table 138-6) and on any associated disease. Patients with refractory anemia or refractory anemia with sideroblasts are less likely to progress to the more aggressive forms and may die of unrelated causes.... There is no established treatment for MDS.... Response of MDS to AML chemotherapy is similar to that of AML, after age and karyotype are considered." - Myelodysplastic Syndrome, Merck Manual

posted by roboto at 2:45 PM on January 2, 2005

There seem to be a lot of rate diseases going around these days.
posted by fleener at 3:23 PM on January 2, 2005

geoff, not to be to blunt, but that's just fucking absurd. I'm not sure who you've known with a terminal illness, but patients I've treated with ALS (Lou Gehrig's disease), Parkinson's, and a few dozen elderly patients with myelodysplasia are certainly not lacking for people who "care".

Caring is the disingenuous, empty mantra of people too lazy to formulate an informed opinion about the merits of stem cell research. You could fill tanker ships with empathy and power them to the sun and it won't buy a person with a spinal injury or a degenerative neurological condition or retinitis pigmentosa one extra second of ambulation, life, or sight. So take caring and shove it as far up the ass of the nearest evangelical, for all I or they care.

People with terminal or permanent or degenerative diseases will tell you that hope is what keeps them sane. And right now, as we peer over the threshold into another Dark Age, hope is pretty damn scarce.
posted by docpops at 3:37 PM on January 2, 2005

I would not consider ALS and Parkinson's as rare diseases at all. There's a ton of money being poured into both those diseases. Hope exists there because there are probably whole branches dedicated just to those diseases at major universities/hospitals. A quick google reveals that myelodysplastic syndrome isn't exactly rare, in fact I'm now wondering what exactly constitutes as rare in medical definitions, but I would consider anything unprofitable for companies to research as rare.

And yes, it is not fucking absurd, when you and only 50 other people have a disease to be unbelievably depressed. Yes I went to school with a girl who was diagnosed with a very, very rare disorder. She's under the very real reality that even though the doctors of course care, and want to help her, there's nothing they can really do without a team of research scientists and a huge grant. Of course the question really lies in should they do something, should society pour resources and labor into something that benefits a couple dozen people?
posted by geoff. at 4:40 PM on January 2, 2005

No worries, foxy.

I'm not sure how rare MDS is, either; my grandmother died of it, and none of her docs or hospice nurses seemed to think it was a particularly odd way to go. (Believe me, I asked early and often.)
posted by jesourie at 5:04 PM on January 2, 2005

That's a shame, Bob Matsui was a great congressperson and his service to the country which put him in a camp as a kid suggests that he was a great american. Just wanted to put in a word of condolence and to honor the guy. Now back to your regularly scheduled discussions of stem cells, rare diseases and now, most likely internment of Americans of Japanese ancestry.
posted by mikojava at 6:05 PM on January 2, 2005

That's a shame, Bob Matsui was a great congressperson and his service to the country which put him in a camp as a kid suggests that he was a great american. Just wanted to put in a word of condolence and to honor the guy. Now back to your regularly scheduled discussions of stem cells, rare diseases and now, most likely internment of Americans of Japanese ancestry

He was the top democrat on the House Social Security subcommittee, a key role in this time of reform. He served 13 terms in congress.
posted by mikojava at 6:09 PM on January 2, 2005

oops sorry posted twice
posted by mikojava at 6:11 PM on January 2, 2005

My dad died from a cancer that sounds similar to Myelodysplastic Syndrome called Multiple Myeloma. Donate here.

My dad had a stem cell transplant in 1996, but the stem cells were taken from his own bone marrow cells, so I would imagine treatment for Matsui's condition would involve something similar and not fetal stem cells.
posted by Frank Grimes at 6:25 PM on January 2, 2005

No, society should keep pouring money into treating people who turn themselves into medical sinkholes after eating for three and smoking. People who are unfortunate enough to have their nervous systems turn to swiss cheese deserve our "caring", but not the benefit of the only real promising research on the horizon because it offends the sensibilities of Christians. But, see, we care so much, so it's OK.

ALS - last I checked affected around 30,000 people. Haven't googled it, so I could be off. That's pretty rare.

Parkinson's: around 300,000 in US.

Type 2 Diabetes: roughly 16 million cases currently, with 800K to a million new cases a year. 80 per cent of Type 2 diabetics are obese.

The fucking absurdity I referred to was your comment "Even if stem cells could possibly cure it, I really doubt they'd start or even ever explore this.". I guess, uh, well, haw, uh, why even try, said Laura Bush to a crowd of hand-picked cretins in her best Westworld impression. Stem cell opponents need to shut the fuck up until they actually understand what they are saying, how they are contradicting themselves at every turn, and just who they are denying hope to. Medical science is being held hostage by people who think they might be sucked into the sky on their way home from work. Meanwhile the rest of the world marches ahead.

Brilliant.
posted by docpops at 6:56 PM on January 2, 2005

Myelodysplastic syndrome is a lumping together of some presumably different disorders of the bone marrow. As in bone marrow cancers, production of normal blood cells is suppressed. Myelodysplasia differs from frank leukemia, however, in that there is no clonal line of cells multiplying out of control. To further confuse the picture, there are a set of similar disorders known as the myeloproliferative disorders, which are in between myelodysplasia and frank leukemia.

Sadly, it's not particularly rare. I'd be surprised if you could get through med school without seeing 5 or 10 cases. And people are researching treatments frantically - when Gleevec first came on the market, it looked like it might make a treatment for some of these disorders, and people (doctors, stock speculators, patients) all got very excited.

We look for cures for difficult diseases based on a number of different things. If it looks like it'd be easy to cure, or there'd be a scientifically interesting odyssey in the process of discovering a cure, it doesn't matter how rare the disease is, it'll attract attention. If a disease affects a lot of people, we look for cures for it for that reason. If a disease causes profound suffering, that may stimulate a search for cures.

But limited resources dictate that if a disease doesn't affect many people and there doesn't appear to be an easy angle to attack it - well, what do you propose be done in that situation? I can't think of much.

The chairman I trained under began his career under Houston Merritt in the 40's. Merritt told him to pick a disease, to "carve out his niche." He picked ALS, figuring that they knew how to diagnose it, knew exactly which cells in the body (a very restricted subset) were affected by it and how; he said he supposed they'd trot a cure out in 10 or 20 years.

50 years later, he said to me ruefully, and no progress at all.
posted by ikkyu2 at 10:33 PM on January 2, 2005