“I did a lot of suffering with my sickle cell anemia, a lot of frequent hospitalizations and a lot of frequent pain crisis and episodes,” said sickle cell patient Lydia Langford, 39.
Sickle cell anemia disease affects about 100,000 Americans, but African-Americans are disproportionately impacted. The Centers for Disease Control and Prevention says 1 in 13 African-American babies are born with the sickle cell trait.
People with sickle cell anemia lack enough healthy blood cells to carry oxygen throughout the body. Their blood cells are shaped like sickles or crescent moons rather than round blood cells. When part of the body is deprived of oxygen needed, the chronic pain occurs.
Langford describes the pain associated with sickle cell disease as unimaginable. She once asked an emergency room doctor to amputate her feet because that’s where the pain was most severe during that sickle cell episode.
Dr. Yvette Miller is the executive medical officer for the American Red Cross. She explains why people of African descent are most impacted, attributing the link to genetics and the malaria disease. Malaria is most prevalent in sub-Saharan Africa.
“Having sickle cell trait in those parts of the world has a survival advantage,” said Dr. Miller. She goes on to say that when the malaria organism “enters into that odd-shaped cell, instead of a normal round-shaped cell, the environment is not conducive to growth, so people can get infected but then the organism won’t grow.” Without a healthy environment for malaria infection to flourish, people infected won’t suffer as severely.
Dr. Miller says blood transfusions are the most common form of treatment for sickle cell anemia by replenishing tissues with oxygen rich blood cells. She says modern medicine has been slow to advance treatment of sickle cell anemia, with only a handful of drugs available.
Dr. Miller also attributes racial health disparities and sickle cell being considered an orphan disease meaning it impacts smaller groups of people.
Bone marrow and stem cell transplants are the only known cures says Dr. Miller, but very few patients can find a match for the procedure. She encourages more Black people to donate blood because people of African descent have slightly different antigens in their blood compared to non-Black people making them more compatible with other Black sickle cell patients.
“African Americans and people of African descent, we have something unique, called antigens on our red blood cells, and for people like Lydia who require frequent transfusions, those units of blood need to be more closely matched, than the standard A, B, O and Rh type,” said Dr. Miller.
People living with sickle cell anemia disease tend to have shorter lifespans which is why Langford is not taking a single day for granted.