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Time for another heme #tweetorial
Let’s talk about Racism in medicine.
Sadly, sickle cell disease (SCD) highlights this issue all too well.
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Let’s talk about Racism in medicine.
Sadly, sickle cell disease (SCD) highlights this issue all too well.
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SCD encompasses a group of diseases characterized by a single gene mutation in the beta globin chain.
This decreases the solubility of hemoglobin leading to pathological polymerization and vaso-occlusive crises.
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This decreases the solubility of hemoglobin leading to pathological polymerization and vaso-occlusive crises.
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SCD has been thought to be a disease that affects Black people.
More accurately, prevalence is highly correlated w/ geography.
Because the mutation is not all bad!
Sickle cell trait confers protection against severe malarial infxn
propagated in malaria endemic areas
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More accurately, prevalence is highly correlated w/ geography.
Because the mutation is not all bad!
Sickle cell trait confers protection against severe malarial infxn
propagated in malaria endemic areas3/n
The credit for first case report goes to Herrick who described a wealthy dental student from Grenada in 1910.
But SCD was recognized much earlier as shamans knew it was inherited and marked patients with limb girdle tattoos to ensure prompt treatment of pain.
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But SCD was recognized much earlier as shamans knew it was inherited and marked patients with limb girdle tattoos to ensure prompt treatment of pain.
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Up until the 1930s, malaria was a big problem in the American south.
As far back as the 17th century it was noted that some Black people were spared.
This became the basis for so called medical “differences” btw Blacks and whites and led to pro-slavery arguments.
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As far back as the 17th century it was noted that some Black people were spared.
This became the basis for so called medical “differences” btw Blacks and whites and led to pro-slavery arguments.
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There are no specific mentions in pre-civil war records but SCD was clearly present given chronic leg ulcers, joint pains in kids, high childhood mortality.
A report from 1834 describes a slave with frequent infections who on autopsy was found to not have a spleen.
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A report from 1834 describes a slave with frequent infections who on autopsy was found to not have a spleen.
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Another case appeared in the literature in 1910, followed by one in 1915 then 1922.
The medical community began to learn more.
It was not until 1949 that Linus Pauling found that the sickling of RBCs was due to abnormal hemoglobin.
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The medical community began to learn more.
It was not until 1949 that Linus Pauling found that the sickling of RBCs was due to abnormal hemoglobin.
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SCD began to gain more publicity in the 1950s.
Again it was used to further Racism as opponents of civil rights used its existence to argue genetic superiority and the need to send Black people back to Africa where the defect could protect against malaria.
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Again it was used to further Racism as opponents of civil rights used its existence to argue genetic superiority and the need to send Black people back to Africa where the defect could protect against malaria.
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A lot of the attention that SCD received was brought by the Black Panthers.
Bobby Seale (one of the founders) referred to SCD as “Black genocide.”
The group started the Sickle Cell Anemia Foundayion and advocated for dedicated treatment!
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Bobby Seale (one of the founders) referred to SCD as “Black genocide.”
The group started the Sickle Cell Anemia Foundayion and advocated for dedicated treatment!
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This advocacy resulted in legislation signed by Nixon in 1972 to fund sickle cell research.
An NIH grant funded 10 comprehensive sickle cell clinics across the US.
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An NIH grant funded 10 comprehensive sickle cell clinics across the US.
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But by this time Pauling was advocating for eugenics including mandatory screening programs.
12 states and DC passed these types of laws.
And they absolutely targeted Black people.
In NY, premarital testing was mandatory but kids were only tested in urban areas.
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12 states and DC passed these types of laws.
And they absolutely targeted Black people.
In NY, premarital testing was mandatory but kids were only tested in urban areas.
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Although Nixon increased funding by 5x he also incorrectly stated that SCD “strikes Blacks and no one else”
Targeting people with SCD became a way to target Black people without explicitly making it about race.
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Targeting people with SCD became a way to target Black people without explicitly making it about race.
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And things haven’t improved.
Studies repeatedly show patients with SCD experience racial bias, and experience greater race based discrimination than other Black patients.
This discrimination also affects their disease course as it is associated with
levels of pain.
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Studies repeatedly show patients with SCD experience racial bias, and experience greater race based discrimination than other Black patients.
This discrimination also affects their disease course as it is associated with
levels of pain. 13/n
These experiences lead to 
adherence w/ tx and trust.
Patients w/SCD wait longer in the ER despite higher pain and triage scores.
A 1997 survey of hematologists and ER physicians reported that pt report was not reliable indicator
of pain and that pts fabricate.
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adherence w/ tx and trust. Patients w/SCD wait longer in the ER despite higher pain and triage scores.
A 1997 survey of hematologists and ER physicians reported that pt report was not reliable indicator
of pain and that pts fabricate. 14/n
Some providers cite concern about drug abuse yet patients with SCD have lower rates of opioid addiction (0.5-8%) than other chronic pain conditions (3-16%) and comparable to the general population (4.8%)
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SCD is INADEQUATELY funded.
A comparison to CF (which affects mostly white people) in 2020 found federal funding per person of $812 compared to $2807, foundation funding of $102 compared to $7690, 926 research articles to 1594 and 1 drug approval compared to 4.
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A comparison to CF (which affects mostly white people) in 2020 found federal funding per person of $812 compared to $2807, foundation funding of $102 compared to $7690, 926 research articles to 1594 and 1 drug approval compared to 4.
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To look at the bright side, there have been exciting recent advances in the treatment of SCD.
Which is fantastic.
Maybe the great @drzsicklecell can do a tweetorial on the cool new drugs available!
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Which is fantastic.
Maybe the great @drzsicklecell can do a tweetorial on the cool new drugs available!
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The @NHLBI_BLOODDir and the @gatesfoundation have each committed $100 million to speed the development of gene therapies for SCD and HIV.
These are serious resources and the science is exciting.
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These are serious resources and the science is exciting.
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But the reality is that patients with SCD to this day continue to receive care, funding, and support that is less than.
It is UNACCEPTABLE.
And if we don’t talk about it, how are we ever going to fix it?
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It is UNACCEPTABLE.
And if we don’t talk about it, how are we ever going to fix it?
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