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Christmas disease

Haemophilia B (also spelled Hemophilia B or Hæmophilia B) is a blood clotting disorder caused by a mutation of the Factor IX gene. It is the second most common form of haemophilia, rarer than haemophilia A. It is sometimes called Christmas disease after Stephen Christmas, the first patient described with this disease. In addition, the first report of its identification was published in the Christmas edition of the British Medical Journal. Treatment (bleeding prophylaxis) is by intravenous infusion of factor IX. more...

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Genetics and pathophysiology

The factor IX gene is located on the X chromosome (Xq27.1-q27.2). It is inherited X-linked recessive, which explains why - as in haemophilia A - only males are generally affected.

Factor IX deficiency leads to an increased propensity for haemorrhage. This is in response to mild trauma or even spontaneously, such as in joints (haemarthrosis) or muscles.

Reference

  • Biggs RA, Douglas AS, MacFarlane RG, Dacie JV, Pittney WR, Merskey C, O'Brien JR. Christmas disease: a condition previously mistaken for haemophilia. Br Med J 1952;2:1378-1382. PMID 12997790.

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Hard right
From Christian Century, 11/1/05 by Bill McKibben

Off Center: The Republican Revolution and the Erosion of American Democracy.

By Jacob S. Hacker and Paul Pierson. Yale University Press, 272 pp., $25.00.

How the Republicans Stole Christmas: The Republican Party's Declared Monopoly on Religion and What Democrats Can Do to Take It Back.

By Bill Press. Doubleday, 288 pp., $23.95.

THE BRIEF LULL between Katrina and Rita was an instructive time for observers of American politics. Because the Bush administration had done such a bad job of preparing for the storm's impact, it found itself scrambling to look humane in its response--it didn't blanch at figures like $200 billion. But it refused to even entertain the idea of revoking any of the massive tax cuts it's delivered to the wealthiest Americans.

Instead, various parts of the GOP began identifying the fat that could be cut from the federal budget. The Republican Study Committee issued a 23-page document called "Operation Offset" with their recommended cuts. Under the heading "Tough Choices for Tough Times" they called for delaying implementation of the Medicare prescription drug bill, increasing copayments for Medicaid, increasing Medicare Part B premiums, and imposing a home health copayment. They also wanted to repeal the most egregious parts of the new highway bill. Oh, and "eliminate subsidized loans to graduate students" because students "make an informed decision to invest in their own futures and should bare the costs of schooling" (sic, but funny). One is left wondering: whose Tough Times?

On its Web site the same Republican Study Committee helpfully reprinted a single article about those who had suffered from the hurricane: "FEMA/Taxpayer-Funded $800 Louis Vuitton Handbags for Katrina Evacuees." Two of them, as it turned out, according to an unidentified employee of a leather-goods store in a Georgia mall.

These kinds of reactions are in the DNA of the modern COP, according to the two books under review. The more impressive, Off Center, is by a pair of well-known political scientists who use a wide array of statistics to prove the point that the Republicans have defied political gravity. Instead of trimming their sails to the moderate breezes of the American middle, they have lurched far to the right. "According to the conventional wisdom about American politics, this shouldn't be possible," they write.

And yet that's clearly what has happened. Using a formula for figuring out the legislature's "median ideological position," they conclude that the average House Republican is 73 percent more conservative than the lawmaker's peer of the early 1970s. In the Senate the shift is even more dramatic--the median Senate Republican is "twice as conservative," just shy of Pennsylvania's Rick Santorum, the guy who compared consensual gay sex to polygamy, incest and "man-on-dog" action.

Democrats, by contrast, have become only slightly more liberal over the same period. The much-bemoaned polarization of American politics, insist Jacob Hacker and Paul Pierson, is mostly a product of the rightward Republican shift. And meanwhile, polling data show that the American people have hardly moved at all. They cite two wide indexes of political views to show that "their aggregate opinions were virtually identical to their aggregate opinions in 1972." Very few, it turns out, really wanted a massive tax cut designed to benefit the richest among us.

So how have the Republicans pulled it off? Hacker and Pierson offer a series of process-oriented explanations. The Bush administration, and especially the congressional leadership, they write, have grown adept at controlling the agenda ("Operation Offset" indeed!). They've figured out how to change laws quietly, without a vote. They've rewritten the traditions that governed institutions like House-Senate conference committees to all but eradicate the power of the opposition party. And they've pushed the consequences of their tax cuts and other policies far enough into the future that they haven't aroused much opposition.

Take, for instance, environmental policy. The administration quietly changed the rules for upgrading coal-fired power plants, achieving what a former Vermont Republican called "the biggest rollback of the Clean Air Act in history." The EPA developed only three new regulatory standards in its first three years under W., compared with 21 in the same period during his father's administration. Enforcement personnel within the EPA were cut by 12 percent, EPA inspections dropped by roughly 3,000 a year and civil environmental penalties fell by 50 percent. At refineries inspections fell by 52 percent. But who noticed? No one, really, except of course the oil company executives who are among the administration's biggest financial supporters.

Hacker and Pierson have written their book with the slightly pedantic tone one expects from academics. (They reprint an editorial cartoon showing Homer Simpson exulting when he receives his measly tax cut while his boss, Mr. Burns, rubs his hands over his massive gain. It is probably not necessary to spend a paragraph explaining the meaning of the cartoon, nor to helpfully declare that it is an attempt to "humorously make the point".) Still, their research is devastating. Without preaching a sermon of any kind or even dropping their deadpan tone, they make it clear how far we have strayed from a society that clothes the naked and feeds the hungry--and how difficult it will be to return. (Though not impossible, the authors insist; they find a glimmer of hope for rebalancing American politics in liberals' ability to raise money over the Internet during the 2004 contests.)

More than anything, they say, conservative Washington represents a triumph of "the base"--of the people who give money and make noise. Since most congressional districts have been gerrymandered to make them safe for one party or the other ("about 90 percent of the districts are now preordained to go to a certain party," they report), the real action is in the primaries. And it is there, where the turnout and media attention are low, that the gun groups, the hard-right tax-cutters, the Limbaugh ditto-heads and the rest of the base usually have their way. Hacker and Pierson tell the story of New Jersey Republican Marge Roukema, elected in 1980 as a moderate. Eighteen years later she retired rather than face a third straight challenge from hard-right Republicans with the support of House power Tom DeLay, and she was succeeded by Republican Scott Garrett, who is so antigovernment that he voted against radon testing in day-care centers as an unwarranted regulation of free enterprise.

Of course, evangelical Christians have been key components of this base ever since the early 1980s and the rise of the Moral Majority. Bill Press is not the first to make the case that this is a literally unholy alliance, but he is among the most vigorous. In How the Republicans Stole Christmas the former cohost of CNN's Crossfire brings a cable sensibility to his analysis of the gospel message. To wit:

"While the rich may have a hard time getting to Heaven, Jesus tells us it's a slam dunk for the poor."

"If Jesus cured people of leprosy, dropsy, and palsy, is there any doubt He would embrace today's efforts to find a cure for Parkinson's or heart disease using embryonic stem cell research? I think not."

But amidst this collection of proof texts and debater's points, Press performs several valuable services. For one, he makes it pungently clear that ours was not founded as a Christian nation--that, indeed, Jefferson (who published his own edition of the Gospels, eliminating all references to Christ's divinity) and Madison (who opposed having chaplains in Congress and accepted them in the military only if they were volunteers and received no government funds) would quickly be blackballed if they even thought of running for office today. Though he accomplishes it a little crudely, Press deserves credit for standing straight up to the religious right where it is most vulnerable--in its hijacking of Jesus and his message for purposes that run counter to almost everything he stood for.

But that doesn't end the argument. One reason religious conservatives have had success is that they have appealed to the latent idea that something in our culture is not working. They have paraded a series of unlikely scapegoats (feminists! gay people! biologists!) as targets for this unease. It's necessary but not sufficient to defend such groups; it's more important to acknowledge that something bad has happened to our society.

That something, more visible than usual in the wake of Katrina, is the breakdown of community. Americans, most data suggest, feel more isolated and less satisfied with their lives than at any time in our history. We have bought into the idea--promulgated most effectively by the business interests that are the ironic other half of the Republican base--that we should think of ourselves mostly as individuals, never as part of some larger kingdom; that we should resent the claims of others, who probably just want to score Louis Vuitton handbags anyhow.

If the hard-right march of the ultraconservatives is to be turned back, it will only be with a revived vision of a more connected community where we look out for each other's needs--for health care, for education, for security, for community. When someone finally offers that vision, it will split off the uneasy-but-goodhearted from the hateful and the greedy, and our politics will start to bend back toward the middle. And maybe even toward the gospel.

Reviewed by Bill McKibben, a CENTURY editor at large and author of Wandering Home: A Long Walk Across America's Most Hopeful Landscape: Vermont's Champlain Valley and New York's Adirondacks (Crown).

COPYRIGHT 2005 The Christian Century Foundation
COPYRIGHT 2005 Gale Group

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