June 19 is World Sickle Cell Awareness Day
For those who are newer followers to my blog, sickle cell is a topic near and dear to my heart, and it’s a condition that far too few people are aware of. I’ll touch on the basics here, then include a more in-depth section lower for the science nerds like me (I realize not everyone gets that excited about genetics.)
The TLDR; on sickle cell
According to the World Health Organization (WHO), sickle cell disease affects 7.74 million people worldwide, with about 80% of those cases in sub-Saharan Africa. More people have sickle cell trait (a much less severe condition, but one that still should be monitored.) Sickle cell is a genetic disorder of the hemoglobin gene, and affects red blood cells.
Sickle cell disease is serious, and can be fatal. Patients require periodic blood transfusions and often suffer from anemia. While some progress has been made in the last few years with CRISPR technology, and a cure might be on the horizon, it’s still undergoing testing, and that testing can be incredibly expensive.
Sickle cell trait is the less serious form, but under certain conditions, can also be problematic (and in a crisis, it can be fatal too.)
Why I’m mentioning it on my blog
I have sickle cell trait, which I inherited from my dad. I’ve written a little about the condition in a couple of my books, hoping to shine a spotlight on it, but it’s still something too many people are completely unaware of.
And since tomorrow is World Sickle Cell Awareness Day, I wanted to mention it again.
How you can help (if you’re inclined)
The first way is to consider becoming a blood donor if you aren’t already. As I mentioned above, patients with sickle cell disease often need blood transfusions (and those of us with the even one copy of the gene can’t donate.)
There are also several organizations actively pursuing research into curing sickle cell disease that will accept monetary donations, if needles aren’t your thing:
Extra info for the science-types
This is where I’m going to get into the science and genetics of sickle cell. I find this stuff interesting, but if it’s not for you, thanks for reading this far. 😊
Sickle cell is caused by a single base-pair mutation in the hemoglobin gene, typically designated the hemoglobin-S gene. Using Mendelian genetics, it’s a codominant gene (meaning both the hemoglobin and hemoglobin-S genes are expressed equally. The -S gene’s affects are present even when the person only has one copy of the gene. There’s no dominant/recessive dynamic here.)
Sickle cell trait is a condition where the person has one “normal” hemoglobin gene, and one hemoglobin-S gene. It’s less severe because the normal gene mitigates some of the effects of the -S gene.
Sickle cell disease is when the person has two copies of the hemoglobin-S gene.
Hemoglobin-S will cause the blood cell to deform and make a sickle shape. The cells don’t carry oxygen through the bloodstream as well in that state, and because of their jagged corners, they also have a tendency to form blood clots and damage blood vessels or organs.
There’s a theory that hemoglobin-S coevolved with the malaria parasite, since the sickling cells provide some immunity to malaria, and both are most common in sub-Saharan Africa.
And if you want to know where I’ve mentioned sickle cell in my books…
The main character of Wraith and the Revolution has sickle cell trait, and it’s the catalyst driving his decision to join Zylar Inc. They have his cure, but in exchange, they turn him into a cyborg soldier.
The related short story/prequel, Survivor, focuses more heavily on his condition.
Anyway, thanks for hearing me out, and please consider donating if you can.