Friday, January 6, 2017

Racism and/or sickle cell disease?

PhysEd article about racism in health care. Yes I know: it just points out the white/black disparity in heath care in the US but it comes down to the talking point memo: racism.

Uh, things are not so simple.

First of all, which black population are you discussing? Inner city blacks who have to go to clinics where they barely know the docs and nurses there? But  among these hard working folks a lot of individuals whose lives are full of social pathology. (not to mention high lead levels if you live in Flint Michigan or Washington DC, but that is another cause for ADHD/impulsivity etc).

And yes there is a lot of prejudice against blacks, especially among the foreign medical graduates from Muslim countries, who look down on them for cultural reasons (Arab and Egyptian disdain of Africans has a long history). But also among white upper class types who lean over backwards not to be prejudiced.

But one of the undiscussed demographic changes in the USA is the decrease in "social pathology" among the black community in recent decades.

One is because of the civil rights movement, that allowed many blacks to move into decent housing (leaving behind, alas, those who are less successful and have more social pathology. This is not new, but see in immigrant groups in the past).

 but also TWO: immigration. West Indians, Africans, and Somalis. They have fewer pathological problems because they stress family values and don't carry the luggage of slavery.

So are these health care disparities seen in other inner city populations: Somalis, Hmong, poor ethnics? West Indian blacks? They also face a similar barrier to local medical care (often using Emergency rooms since the clinic means sitting in a large building for impersonal care).

But there are other factors. Hispanics often have extended families, but on the other hand, so do a lot of inner city Blacks (these families are just invisible to outsiders).

But some are genetic. Is the high infant mortality from teenagers having babies? Or is it from Pre Eclampsia? Or because drug/alcohol use causes sick babies?

Compare and contrast the statistics to Hispanics, or maybe more pertainent, Amerindians, whose substance abuse problems are a major problem.

And then there is diabetes/ hypertension. Again genetic..

AmerIndians have a lot more diabetes, but hypertension is common in Afro Americans, even at a young age, and is a killer. You have to take expensive medicine every day, and often it is a "silent killer" because it is not diagnosed early.

so yes, racism, but there are also genetic factors in this.

And the elephant in the living room is actually none of the above.

It is sickle cell disease.

  • People of African descent, including African-Americans (among whom 1 in 12 carries a sickle cell gene)
  • Hispanic-Americans from Central and South America
  • People of Middle Eastern, Asian, Indian, and Mediterranean descent
Because sickle cell anemia symptoms can begin by four months of age, early diagnosis is critical. All newborns in the United States are now tested for the disease. Sickle cell anemia can be identified before birth by testing a sample of amniotic fluid or tissue from the placenta. People who carry the sickle cell gene can seek genetic counseling before pregnancy to discuss options.

yup Abort the kid and voila, no  problem?

Imagine if 100 thousand white people lived with a devesating, painful disease?
There would be marathons and all sorts of stuff out there to educate patients.

and with aggressive treatment, the deaths can be limited:

hydroxyurea helps, as does giving pneumonia vaccine and daily penicillin prophylaxis (one common cause of death is pneumococcal pneumonia/sepsis, mainly because the sickle cells destroy the spleen that produces immunity to fight off this germ).

 In the last 50 years, survival has improved dramatically for people with SCD in the United States. Their average life expectancy in the 1970s was <20 years of age. By the early 1990s, the Cooperative Study of Sickle Cell Disease estimated a median life expectancy of those with sickle cell anemia, the most severe form of the disease, of 42 years of age for males and 48 years of age for females.
The red blood cells sickle/become a crescent shape when oxygen is low, and this clogs tiny blood vessels, causing pain etc.

One way to stop them from sickling is blood transfusions of normal blood cells, but alas HIV, the shortage and price of blood, and long term iron overload make that a difficult option.

and presumably a complete bone marrow transplant would work also: But again, very expensive and possibly fatal, so not used for a disease that might not kill ou for 30 years.

The reason that babies don't get symptoms, is that babies have Fetal Hemoglobin (wth a slightly different oxygen dissociation level) but this turns off after birth.

So could stopping this help? If you had maybe 20 to 30 percent of your cells as fetal hemoglobin, could you stop sickling crisis?

or why not just give them a gene that produces normal hemoglobin?

SicentificAmerican article on genetic treatments (2016)

read the whole thing.

This disease is mainly seen in West Africans, so yes, you see it in London and Europe.

If you have sickle cell trait, usually you don't have many problems. This is where the good gene makes most of the cells but you have some sickling genes: rarely a problem unless you are a paratrooper exposed to high altitude.

But the theory is that the trait helps protect you against malaria (malaria parasites grow inside red blood cells). A similar theory explains why you seen the various thalessemias in Greeks and other Mediterranean immigrants.

So i f you live in West Africa, the baby with sickle cell disease will die, but the ones with just the trait are more likely to live. Voila. Population drift to more people with sickle cell disease.
mediInteresting factoid: We didn't see sickle cell anemia in the Mashona of Zimbabwe.

They descended from the high veldt tribes where there was no malaria. Indeed, I saw exactly one case in the three years I worked there: And that mother was a local prostitute who worked at a local mine. She (and most of the miners) were from Mozambique or Malawi.

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