Imagine for a minute that your blood cells are shaped like sickles -- sorta like that
thing that the image of death carries?
Ouch right?
But what exactly causes our friendly little red blood cells to turn against us?
Sickle Cell Anemia is a blood disorder, where instead of supple soft-frisbee red blood cells
that live for 120 days, your body forms stiff pointy sickle-shaped cells that only live
for 10 to 20 days.
RBCs need to be soft and pliable to squeeze through your blood vessels safely and efficiently,
so when they’re pointy they can get stuck in vessels.
When this happens in the chest, abdomen or joints it can cause pain, if they get stuck
in the hands or feet you can get swelling, and of course if they get stuck in organs
they can cause infections or (if they get caught in the eye) vision problems.
And if that wasn’t bad enough, the brittle cells can break apart, delaying oxygen delivery
and causing fatigue!
This all sounds terrible.
It was first described in 1910, in a dental student named Walter Clement Noel who went
to a doctor in Chicago complaining of pain, but this student wasn’t from Chicago -- he
was from Grenada.
Sickle cell anemia predominantly affects approximately 1 in 400 African American births.
It was a disease known in Africa for five thousand years, but it hadn’t been described
in Western Medicine before 100 years ago.
The doctor in Chicago, who’s name was James B. Herrick was the first to describe it in
a paper and over the next century many doctors have attempted to uncover how this debilitating
disorder works, and why it mainly affects those of African descent.
In the 1930s, a D.C.-based doctor named William Cardozo was one of the first black doctors
to conduct research into this disease… and he did so with a grant from Alpha Phi Alpha
the first black Fraternal organization.
Cardozo felt that research into sickle cell anemia had “reached an impasse” and that
no one had yet found the CAUSE of this horrible disease.
IF someone could provide a “new stimulus” to get the research going, he wrote, maybe
we could discover more.
His research, published in 1937, helped medicine realize sickle cell anemia was inherited.
Thanks to his research we now knew sickle cell came from us -- and he helped explain
why it mainly affected people of African descent.
Which is huge.
While he was working on that other doctors started to realize this disease was based
on oxygen content.
In fact, Québécois researchers in 1930 Montreal took a seven year old black girl and restricted
blood flow to her finger with a rubber band -- no, this probably wouldn’t pass an ethics
board today -- In doing so, they found sickle cells formed in the low oxygen environment
of her fingertip.
Thus, they learned that with a bit of hypoxia, or lack of oxygen, the cells would collapse
into all manner of weird pointy shapes, nearly instantly returning to normal when exposed
to air.
Their methods were questionable, to say the least, but the results did shape future research.
In the mid-20th century doctors discovered that a single amino acid was responsible for
the hemoglobin on the red blood cells that cause the sickling.
But now that we understand the disorder enough to try and solve it… but…
Unfortunately, we’re at another moment when we may need more “new stimulus” for SCD
research.
According to a Clinical Investigation from 2007, there are many possible treatments,
including a bone marrow transplant, but there’s still no cure.
One option is medicine that helps fetuses develop more hemoglobin, but it CAN make SCD
worse…
Maybe in the future an option could be genetic manipulation of the system that helps our
bodies create blood, using genome engineering basically, gene therapy.
But for now, sickle cell anemia is just out there, a life-threatening thorn in many people’s
sides.
Cardozo was one of many doctors of many races in the hunt for the causes of sickle cell
anemia.
He’s notable because in the 1930s doctors that looked like him were often not welcome
in the annals of medicine.
That HE was able to make a discovery for a disorder that affected so many people that
also looked like him, is a pretty big deal, so we’re happy to recognize the part he
played in this story for Black History Month.
For more on red blood, watch Jules’ video about what bone marrow actually does!
(Hint: it’s important).
And please subscribe for more seeker.
Whenever I think of red blood cells, I think of those soft frisbees that I used to throw
at my brothers.
Maybe that’s why I think of RBCs as so darn friendly!
Thanks for watching.