Sickle Cell Anemia Quotes

Old measures of health not only have failed to improve significantly but have stayed the same: some have even worsened. Mainstream newspapers and magazines often report disease in an ethnocentric manner that shrouds its true cost among African Americans. For example, despite the heavy emphasis on genetic ailments among blacks, fewer than 0.5 percent of black deaths—that’s less than one death in two hundred—can be attributed to hereditary disorders such as sickle-cell anemia. A closer look at the troubling numbers reveals that blacks are dying not of exotic, incurable, poorly understood illnesses nor of genetic diseases that target only them, but rather from common ailments that are more often prevented and treated among whites than among blacks.

One of the favorite speakers was a man in red who warned of sickle-cell anemia, 'a deadly organism lurking in all nigger blood.' 'If so much as one drop of nigger blood gets in your baby’s cereal,' he said, 'the baby will surely die in one year.' He did not explain how he thought a negro would come to bleed in anyone’s cereal.

And fewer than 5 percent of people on the planet are born with some genetic condition—like type 1 diabetes, Down syndrome, or sickle-cell anemia. The other 95 percent of us who develop such a condition acquire it through lifestyle and behaviors.

Furthermore, some researchers have suggested a strong association with the presence of the protective gene in populations who have historically farmed yams. To plant yams, farmers clear forests. Cleared forest means more standing water. More standing water means more mosquitoes. More mosquitoes means more malaria—so the idea goes. The emergence of the disease, and as a consequence the resistance gene, may well have been enabled, or at least nurtured, by yam farming. The persistence of sickle cell anemia is the cost of positive selection for resistance against the most destructive disease in our history.

The problem with racial discrimination, though, is not the inference of a person's race from their genetic characteristics. It is quite the opposite: it is the inference of a person's characteristics from their race. The question is not, can you, given an individual's skin color, hair texture, or language, infer something about their ancestry or origin. That is a question of biological systematics -- of lineage, taxonomy, of racial geography, of biological discrimination. Of course you can -- and genomics as vastly refined that inference. You can scan any individual genome and infer rather deep insights about a person's ancestry, or place of origin. But the vastly more controversial question is the converse: Given a racial identity -- African or Asian, say -- can you infer anything about an individual's characteristics: not just skin or hair color, but more complex features, such as intelligence, habits, personality, and aptitude? /I/ Genes can certainly tell us about race, but can race tell us anything about genes? /i/ To answer this question, we need to measure how genetic variation is distributed across various racial categories. Is there more diversity _within_ races or _between_ races? Does knowing that someone is of African versus European descent, say, allow us to refine our understanding of their genetic traits, or their personal, physical, or intellectual attributes in a meaningful manner? Or is there so much variation within Africans and Europeans that _intraracial_ diversity dominates the comparison, thereby making the category "African" or "European" moot? We now know precise and quantitative answers to these questions. A number of studies have tried to quantify the level of genetic diversity of the human genome. The most recent estimates suggest that the vast proportion of genetic diversity (85 to 90 percent) occurs _within_ so-called races (i.e., within Asians or Africans) and only a minor proportion (7 percent) within racial groups (the geneticist Richard Lewontin had estimated a similar distribution as early as 1972). Some genes certainly vary sharply between racial or ethnic groups -- sickle-cell anemia is an Afro-Caribbean and Indian disease, and Tay-Sachs disease has a much higher frequency in Ashkenazi Jews -- but for the most part, the genetic diversity within any racial group dominates the diversity between racial groups -- not marginally, but by an enormous amount. The degree of interracial variability makes "race" a poor surrogate for nearly any feature: in a genetic sense, an African man from Nigria is so "different" from another man from Namibia that it makes little sense to lump them into the same category.

In this study and others like it, guesswork about a peculiar black predisposition toward unhealthy births imports an old notion about sickle cell disease “afflicting the black race.”25 Whenever I give a talk on this topic, there is inevitably someone in the audience who invokes the mantra that sickle cell anemia is a black genetic disease and therefore proves that race is a genetic category. This misconception was first popularized in the early twentieth century by hematology experts who believed the capacity to develop sickled cells was uniquely inherent in “Negro blood.”26 Stereotypes about black resistance to malaria and susceptibility to sickle cell justified sending black workers to malaria-infested regions in the first part of the century and later led to discriminatory government, employer, and insurance-testing programs in the 1970s.27 The error is easily exposed by looking at two world maps, one highlighting the regions around the globe where malaria is prevalent, the other highlighting areas where sickle cell disease is present. The maps mirror each other perfectly. By comparing them, it is plain to see that malaria and sickle cell aren’t restricted to Africa and that much of Africa is unaffected. High frequencies of the trait also occur in parts of Europe, Oceania, India, and the Middle East, all places where there is malaria. In fact, people in the town of Orchomenos in central Greece have double the rate of sickle cell disease reported among African Americans.28 If frequency of the sickle cell gene determined racial boundaries, it certainly would not prove there is a black race. Instead, as Jared Diamond pointed out in the November 1994 issue of Discover , if we grouped together people by the presence or absence of the sickle cell gene, “we’d place Yemenites, Greeks, New Guineans, Thai, and Dinkas in one ‘race,’ Norwegians and several black African peoples in another.”29 It would be more accurate to call the groups with the sickle cell gene the “antimosquito race.” Of course, that would be a silly way of grouping people, except for studying the sickle cell gene. But “black race” is an equally silly way of grouping people for identifying genetic contributions to disease.

DNA will be stored by the clinic or government. Why not screen the baby before it’s born? Advances in genetic technology allow embryos to be screened for hundreds of genetic risk factors, including Down syndrome, breast cancer, and sickle-cell anemia, using nothing but a blood sample from the mother. Parents who desire the ideal baby, and nothing less, will go for that. Already companies are offering services from pre-implantation diagnoses to direct-to-consumer genetic testing. Genetic technology will impact our emotions and values. Some parents see genetic optimization as a great possibility and a basic right; others are morally appalled by the thought of designer babies. One thing we can be sure of is that genetic screening will lead to more abortions. Moreover, if parents decide

Mom’s a hypochondriac, too, so the best part was that every week she would get the disease that the medical shows were dramatizing. I’ll never forget, they did an episode on sickle cell anemia, which as far as I know, is almost exclusively an African-American affliction.

Americans may already have begun adapting genetically to the American environment in the several generations since their ancestors arrived in the United States. The malaria-protecting genetic variants common in Africans, such as the variation that causes sickle-cell anemia, are no longer a necessity of survival in the United States, so the pressure of natural selection to retain these variants would be relaxed.

A fourth possibility: It could be that belief is causally efficacious semantically as well as syntactically with respect to behavior, but maladaptive. As Stich points out, it is quite possible (and quite in accord with current evolutionary theory) that a system or trait that is in fact maladaptive, at any rate less adaptive than available alternatives should nonetheless become fixed and survive. Perhaps the belief systems of these creatures are like the albinism found in many arctic animals, or like sickle cell anemia, maladaptive, but connected with genes coding for behavior or traits conducive to survival. They could be maladaptive in two ways. First, perhaps their beliefs are a sort of energy expensive distraction, causing these creatures to engage in survival enhancing behavior, all right, but in a way less efficient and economic than if the causal connections by passed belief altogether. Second, it could be that beliefs in fact produce maladaptive behavior. Perhaps a mildly maladaptive belief behavior structure is coded for by the same genetic structure that produces some adaptive behavior. Suppose these creatures' beliefs do not for the most part produce adaptive behavior: the mechanisms that produce them might nonetheless survive. Perhaps on balance their behavior is sufficiently adaptive, even if not every segment of it is. Some probability, then, must be reserved for the possibility that these creatures have cognitive faculties that are maladaptive, but nonetheless survive; and on this possibility, once more, the probability that their beliefs would be for the most part true is fairly low.

A fourth possibility: It could be that belief is causally efficacious semantically as well as syntactically with respect to behavior, but maladaptive. As Stich points out, it is quite possible (and quite in accord with current evolutionary theory) that a system or trait that is in fact maladaptive, at any rate less adaptive than available alternatives should nonetheless become fixed and survive. Perhaps the belief systems of these creatures are like the albinism found in many arctic animals, or like sickle cell anemia, maladaptive, but connected with genes coding for behavior or traits conducive to survival. They could be maladaptive in two ways. First, perhaps their beliefs are a sort of energy expensive distraction, causing these creatures to engage in survival enhancing behavior, all right, but in a way less efficient and economic than if the causal connections by passed belief altogether. Second, it could be that beliefs in fact produce maladaptive behavior. Perhaps a mildly maladaptive belief behavior structure is coded for by the same genetic structure that produces some adaptive behavior. Suppose these creatures' beliefs do not for the most part produce adaptive behavior: the mechanisms that produce them might nonetheless survive. Perhaps on balance their behavior is sufficiently adaptive, even if not every segment of it is. Some probability, then, must be reserved for the possibility that these creatures have cognitive faculties that are maladaptive, but nonetheless survive; and on this possibility, once more, the probability that their beliefs would be for the most part true is fairly low.

A fourth possibility: it could be that belief is causally efficacious—'semantically' as well as 'syntactically'—with respect to behavior, but maladaptive. As Stich points out, it is quite possible (and quite in accord with current evolutionary theory) that a system or trait that is in fact maladaptive—at any rate less adaptive than available alternatives—should nonetheless become fixed and survive. Perhaps the belief systems of these creatures are like the albinism found in many arctic animals, or like sickle-cell anemia: maladaptive ,but connected with genes coding for behavior or traits conducive to survival. They could be maladaptive in two ways. First, perhaps their beliefs are a sort of energy-expensive distraction, causing these creatures to engage in survival enhancing behavior, all right, but in a way less efficient and economic than if the causal connections by-passed belief altogether. Second, it could be that beliefs in fact produce maladaptive behavior. Perhaps a mildly maladaptive belief-behavior structure is coded for by the same genetic structure that produces some adaptive behavior. Suppose these creatures' beliefs do not for the most part produce adaptive behavior: the mechanisms that produce them might nonetheless survive. Perhaps on balance their behavior is sufficiently adaptive, even if not every segment of it is. Some probability, then, must be reserved for the possibility that these creatures have cognitive faculties that are maladaptive, but nonetheless survive; and on this possibility, once more, the probability that their beliefs would be for the most part true is fairly low.

It could be that belief is causally efficacious—semantically as well as syntactically—with respect to behavior, but maladaptive. As Stich points out, it is quite possible (and quite in accord with current evolutionary theory) that a system or trait that is in fact maladaptive—at any rate less adaptive than available alternatives—should nonetheless become fixed and survive. Perhaps the belief systems of these creatures are like the albinism found in many arctic animals, or like sickle-cell anemia, maladaptive, but connected with genes coding for behavior or traits conducive to survival. They could be maladaptive in two ways. First, perhaps their beliefs are a sort of energy expensive distraction, causing these creatures to engage in survival enhancing behavior, all right, but in a way less efficient and economic than if the causal connections by-passed belief altogether. Second, it could be that beliefs in fact produce maladaptive behavior. Perhaps a mildly maladaptive belief-behavior structure is coded for by the same genetic structure that produces some adaptive behavior. Suppose these creatures' beliefs do not for the most part produce adaptive behavior: the mechanisms that produce them might nonetheless survive. Perhaps on balance their behavior is sufficiently adaptive, even if not every segment of it is. Some probability, then, must be reserved for the possibility that these creatures have cognitive faculties that are maladaptive, but nonetheless survive; and on this possibility, once more, the probability that their beliefs would be for the most part true is fairly low.

A fourth possibility: It could be that belief is causally efficacious semantically as well as syntactically with respect to behavior, but maladaptive. As Stich points out, it is quite possible (and quite in accord with current evolutionary theory) that a system or trait that is in fact maladaptive, at any rate less adaptive than available alternatives should nonetheless become fixed and survive. Perhaps the belief systems of these creatures are like the albinism found in many arctic animals, or like sickle-cell anemia, maladaptive, but connected with genes coding for behavior or traits conducive to survival. They could be maladaptive in two ways. First, perhaps their beliefs are a sort of energy expensive distraction, causing these creatures to engage in survival enhancing behavior, all right, but in a way less efficient and economic than if the causal connections by-passed belief altogether. Second, it could be that beliefs in fact produce maladaptive behavior. Perhaps a mildly maladaptive belief-behavior structure is coded for by the same genetic structure that produces some adaptive behavior. Suppose these creatures' beliefs do not for the most part produce adaptive behavior: the mechanisms that produce them might nonetheless survive. Perhaps on balance their behavior is sufficiently adaptive, even if not every segment of it is. Some probability, then, must be reserved for the possibility that these creatures have cognitive faculties that are maladaptive, but nonetheless survive; and on this possibility, once more, the probability that their beliefs would be for the most part true is fairly low.

A fourth possibility: it could be that belief is causally efficacious—semantically as well as syntactically—with respect to behavior, but maladaptive. As Stich points out, it is quite possible (and quite in accord with current evolutionary theory) that a system or trait that is in fact maladaptive—at any rate less adaptive than available alternatives—should nonetheless become fixed and survive. Perhaps the belief systems of these creatures are like the albinism found in many arctic animals, or like sickle-cell anemia: maladaptive, but connected with genes coding for behavior or traits conducive to survival. They could be maladaptive in two ways. First, perhaps their beliefs are a sort of energy expensive distraction, causing these creatures to engage in survival enhancing behavior, all right, but in a way less efficient and economic than if the causal connections by-passed belief altogether. Second, it could be that beliefs in fact produce maladaptive behavior. Perhaps a mildly maladaptive belief-behavior structure is coded for by the same genetic structure that produces some adaptive behavior. Suppose these creatures' beliefs do not for the most part produce adaptive behavior: the mechanisms that produce them might nonetheless survive. Perhaps on balance their behavior is sufficiently adaptive, even if not every segment of it is. Some probability, then, must be reserved for the possibility that these creatures have cognitive faculties that are maladaptive, but nonetheless survive; and on this possibility, once more, the probability that their beliefs would be for the most part true is fairly low.

A fourth possibility: It could be that belief is causally efficacious semantically as well as syntactically with respect to behavior, but maladaptive. As Stich points out, it is quite possible (and quite in accord with current evolutionary theory) that a system or trait that is in fact maladaptive, at any rate less adaptive than available alternatives—should nonetheless become fixed and survive. Perhaps the belief systems of these creatures are like the albinism found in many arctic animals, or like sickle-cell anemia, maladaptive, but connected with genes coding for behavior or traits conducive to survival. They could be maladaptive in two ways. First, perhaps their beliefs are a sort of energy expensive distraction, causing these creatures to engage in survival enhancing behavior, all right, but in a way less efficient and economic than if the causal connections by-passed belief altogether. Second, it could be that beliefs in fact produce maladaptive behavior. Perhaps a mildly maladaptive belief-behavior structure is coded for by the same genetic structure that produces some adaptive behavior. Suppose these creatures' beliefs do not for the most part produce adaptive behavior: the mechanisms that produce them might nonetheless survive. Perhaps on balance their behavior is sufficiently adaptive, even if not every segment of it is. Some probability, then, must be reserved for the possibility that these creatures have cognitive faculties that are maladaptive, but nonetheless survive; and on this possibility, once more, the probability that their beliefs would be for the most part true is fairly low.

A fourth possibility: it could be that belief is causally efficacious—semantically as well as syntactically—with respect to behavior, but maladaptive. As Stich points out, it is quite possible (and quite in accord with current evolutionary theory) that a system or trait that is in fact maladaptive—at any rate less adaptive than available alternatives—should nonetheless become fixed and survive. Perhaps the belief systems of these creatures are like the albinism found in many arctic animals, or like sickle-cell anemia: maladaptive ,but connected with genes coding for behavior or traits conducive to survival. They could be maladaptive in two ways. First, perhaps their beliefs are a sort of energy-expensive distraction, causing these creatures to engage in survival enhancing behavior, all right, but in a way less efficient and economic than if the causal connections by-passed belief altogether. Second, it could be that beliefs in fact produce maladaptive behavior. Perhaps a mildly maladaptive belief-behavior structure is coded for by the same genetic structure that produces some adaptive behavior. Suppose these creatures' beliefs do not for the most part produce adaptive behavior: the mechanisms that produce them might nonetheless survive. Perhaps on balance their behavior is sufficiently adaptive, even if not every segment of it is. Some probability, then, must be reserved for the possibility that these creatures have cognitive faculties that are maladaptive, but nonetheless survive; and on this possibility, once more, the probability that their beliefs would be for the most part true is fairly low.

A fourth possibility: it could be that belief is causally efficacious—semantically as well as syntactically—with respect to behavior, but maladaptive. As Stich points out, it is quite possible (and quite in accord with current evolutionary theory) that a system or trait that is in fact maladaptive—at any rate less adaptive than available alternatives—should nonetheless become fixed and survive. Perhaps the belief systems of these creatures are like the albinism found in many arctic animals, or like sickle-cell anemia, maladaptive, but connected with genes coding for behavior or traits conducive to survival. They could be maladaptive in two ways. First, perhaps their beliefs are a sort of energy expensive distraction, causing these creatures to engage in survival enhancing behavior, all right, but in a way less efficient and economic than if the causal connections by-passed belief altogether. Second, it could be that beliefs in fact produce maladaptive behavior. Perhaps a mildly maladaptive belief-behavior structure is coded for by the same genetic structure that produces some adaptive behavior. Suppose these creatures' beliefs do not for the most part produce adaptive behavior: the mechanisms that produce them might nonetheless survive. Perhaps on balance their behavior is sufficiently adaptive, even if not every segment of it is. Some probability, then, must be reserved for the possibility that these creatures have cognitive faculties that are maladaptive, but nonetheless survive; and on this possibility, once more, the probability that their beliefs would be for the most part true is fairly low.

the preamble of the National Sickle Cell Anemia Control Act, enacted in 1972 to foster sickle-cell research, screening, counseling, and education, is untrue: “Two million Americans suffer from sickle cell disease.” Actually, 2 million people were healthy carriers22 and fewer than 100,000 Americans suffered from sickle-cell anemia. The erroneous claim coupled with its constantly reinforced perception of sickle-cell disease as a black disorder left Americans with the mistaken impression that a good portion—one in twelve—of African Americans suffered from sickle-cell anemia.

Weeks earlier, we’d had our blood drawn during a med check, and the doctors discovered I carried the Sickle Cell Trait. I didn’t have the disease, Sickle Cell Anemia, but I had the trait, which was believed at the time to increase the risk of sudden, exercise-related death due to cardiac arrest. The Air Force didn’t want me dropping dead in the middle of an evolution and pulled me out of training on a medical.

An assortment of prenatal tests is available to identify physical defects in the fetus, chromosomal abnormalities such as Down syndrome, and genetic anomalies such as sickle-cell anemia. There are two general types of fetal tests: screening and diagnostic. Screening tests, including ultrasound and maternal blood tests, provide the information needed to determine whether diagnostic testing should be conducted. Diagnostic tests definitively show the absence or presence of a fetal abnormality.

My male friends, even the ex-marines, rocket scientists, and hunters who’ve shot six-hundred-pound boars, are all pretty gentle, sensitive guys who don’t need to get blacked-out drunk to talk about their relationship troubles or admit that they enjoy each other’s company. None of them gives a shit about cars. Some of them do get excited about professional football, but this I regard as a regrettable genetic defect, like the predisposition toward sickle-cell anemia among African-Americans.

In 1922, a fourteen-year-old boy with type 1 diabetes was resuscitated from a coma—born anew, as it were—by the infusion of insulin extracted from the pancreatic cells of a dog. In 2010, when Emily Whitehead received her infusion of CAR (chimeric antigen receptor) T cells, or twelve years later, when the first patients with sickle cell anemia are surviving, disease-free, with gene-modified blood stem cells, we are transitioning from the century of the gene to a contiguous, overlapping century of the cell.