As a Black woman who serves as MDwise’s Equity Officer, Anye Carson is aware of sickle cell disease (SCD).
She knows that it predominantly affects Black people, can cause debilitating pain and shortens life spans by as much as two decades. She knows that SCD is passed from parent to child, and even if someone does not have the disease, they might carry a gene. If a carrier is matched with another carrier of that gene, it could result in a baby being born with SCD.
Still, Carson can’t recall her doctor asking her about sickle cell disease when she was pregnant, or whether she discussed the results of the routine sickle cell screening with anyone after her little girl was born. She also doesn’t recall ever having conversations with other Black women about whether they might carry the gene.
MDwise Project Coordinator Michelle Jones, on the other hand, has had plenty of conversations about sickle cell disease. She has seen members of her family who have SCD going in and out of the hospital, and she has watched the disease cause serious health complications. She also knows a couple who, not realizing they each carry a sickle cell gene, gave birth to three daughters who all have the disease.
We need more women to have a higher level of SCD knowledge, especially now, when improved screening and treatment increase the opportunity to limit the disease’s impact. Both Carson and Jones wanted to share their stories to help increase SCD awareness.
Aptly named, sickle cell disease causes red blood cells to take on a crescent shape, like a sickle. At the same time, the cells become hard and sticky, making it possible for them to block blood vessels and keep oxygen and nutrients from getting where they need to go. The results can include extreme pain, organ damage, heart problems, severe infections and even early death.
The Martin Center Sickle Cell Initiative in Indianapolis reports that nearly 1,700 Hoosiers live with SCD. Roughly 87% of them are Black, most live in either Marion or Lake counties, and they have a median age of 21.
Traditionally, treatment of sickle cell disease has focused on managing pain and responding to other health effects. However, treatment has evolved in recent years, with new medications becoming available and success being found with blood transfusions and bone marrow transplants. Stem cell transplants also have proven possible if a patient has a perfectly matched sibling, and gene editing has occasionally been employed, but that approach carries significant risks and costs.
While these advancements in treatment are important, our biggest weapon against sickle cell disease and its painful impact is information.
The problem is not a lack of screening. Every newborn (and every child adopted from another country) is tested for SCD, and parents are informed if their child has the disease. However, the parents of a child who is born with the sickle cell trait (but not the disease) often aren’t informed or, if they are, that fact might be quickly forgotten because it doesn’t seem immediately consequential.
Doctors need to increase the attention paid to SCD and make sure they educate parents of children with the sickle cell trait. Parents need to ask their children’s providers about SCD and the sickle cell trait if the providers don’t bring up the subject. Young people need to know whether they carry the sickle cell trait, and they need to discuss SCD with partners before having children. Parents need to pass SCD trait information along to their child later in life, so their child knows the risk of passing SCD to another generation. Otherwise, this important information is forgotten over time.
September is Sickle Cell Awareness Month. Let’s use this time to close SCD knowledge gaps and to make sure we’re having essential conversations. We might not be able to eliminate SCD by talking about it, but we can move closer to the day when no one has to live the painful, difficult and shortened life that comes with sickle cell disease.
