In recognition of National Sickle Cell Awareness Month, the UBI is spotlighting the importance of treatments and research for sickle cell disease. But what is it?
About Sickle Cell Disease
Sickle cell disease, or SCD, is a genetically inherited blood disorder that causes an abnormality in red blood cells. SCD causes a malformation to occur in red blood cells, making them sticky and crescent-shaped, rather than healthy, round, and flexible. This clogs blood flow and depletes the transportation of oxygen to the entire body. SCD can also cause anemia, or a red blood cell shortage, since the cells tend to die early (you may have heard SCD referred to as “sickle cell anemia” for this reason). Complications of SCD include strokes, pain, kidney disease, acute chest syndrome (chest pain, coughing, difficulty breathing, and fever), and pulmonary embolism (blood clotting in the lungs). SCD can be fatal, causing the median life expectancy for individuals in the United States with SCD to be just 42-48 years, and this number is notably lower in countries where medical care is less accessible.
Fortunately, there are a number of lifesaving treatments available to patients with SCD. Blood transfusions can counteract anemic symptoms by increasing red blood cell count, and medications can help target the pain SCD patients face. In young children with SCD, a miracle cure can be found in the form of stem cell (bone marrow) transplants. The new bone marrow from these transplants allows the body to produce healthy red blood cells and cease the production of sickle-shaped blood cells.
Scientists believe that SCD may have been an evolutionary adaptation that formed in individuals located in malaria-ridden areas to protect against malaria. Historically, this primarily meant African regions, but over time malaria has spread to the Mediterranean, Caribbean, Asia, and Central and South America. Because of ancestral genetics, the African American community contains a significant majority of the country’s 100,000 SCD cases. About 1 in every 365 African American babies is born with SCD, and 1 in 13 African American babies is born a carrier of the sickle cell trait, causing them to have a chance of passing on the disease.
For such a dangerous disease, we don’t give SCD nearly enough recognition, which can be primarily attributed to structural racism. For context, let’s compare SCD initiatives to cystic fibrosis initiatives. Cystic fibrosis is an inherited disease that decreases quality of life and life span, and in these ways is similar to SCD. Unlike SCD, however, cystic fibrosis occurs primarily in white Americans. Cystic fibrosis research receives 7 to 11 times more funding per patient than SCD research, causing a disparity in the number of medications available between the two diseases. 15 cystic fibrosis medications have been approved by the FDA, whereas only 4 have been approved for SCD. Considering that around 30,000 Americans currently have cystic fibrosis, in comparison to 100,000 with SCD, the comparative lack of funding and available medications makes clear the incredibly harmful impact of structural racism.
Though structural racism is a massive issue facing SCD treatment in America, it’s not the only one. The United States is currently facing a severe blood shortage, which means that there is less healthy blood to use in transfusions for SCD patients. These patients may require transfusions of blood containing specific antigens (antibody-producing spikes on the outsides of red blood cells).
If presented with an antigen mismatch, a recipient risks an extreme, sometimes fatal immune response. While people of all backgrounds have the same basic blood types, ancestral genetics means that certain antigens--those required to treat SCD patients included-- are most commonly found among members of a specific ethnicity. Since the majority of individuals with SCD in America are of African descent, more African American blood donors are needed to ensure that SCD patients can safely receive transfusions.
Statistically, however, African American donors give blood at 25-50% the rate of white Americans. The American medical system is at fault for this, as it has historically marginalized African Americans in vile ways, fueling valid distrust among many members of the African American community.
Our Commitment to an Equitable Blood Supply
Here at the UBI, we are committed to sustainability and equitability. We believe that no individual should have to face the effects of discrimination in the medical field. We aim to foster a safe environment where individuals of all backgrounds, races, and ethnicities can feel comfortable to discuss their past experiences, as well as work towards righting the wrongs of our medical predecessors. We aim to raise awareness about the realities of SCD that are so often overlooked. With this educational approach, we strive to increase donations from groups that are consistently underrepresented in the donor pool. It’s critical for our blood supply to reflect the diversity of our communities in order to ensure that all SCD patients obtain needed care. Through this initiative, we hope to play a small role in a larger movement to change the fate of SCD patients who face challenges in accessing treatment and amplify the voices of those that have been silenced.
Written by Julien Rentsch
Edited by Claire Schultz & Eliana Spiess