Olivia (above) suffered from Sickle Cell Disease until she received a bone marrow transplant from her matching donor, Brandon. The two met for the first time in 2022, a year after the tranplant. Click here to read their story.
Sickle Cell Disease (SCD) is a group of inherited red blood cell disorders. Red blood cells contain hemoglobin, a protein that carries oxygen. Healthy red blood cells are round which allows them to move through blood vessels carrying oxygen to all parts of the body. However, in someone with SCD, the hemoglobin is not normal, causing the red blood cells to become hard, sticky, and look like a C-shaped sickle. These C-shaped cells can get stuck in the narrow passageways in the bloodstream causing blockages which can be extremely painful and can prevent oxygen from reaching all parts of the body.
Sickle cells die early, only living about 10 – 20 days (compared to the normal 70 – 140 days), which can cause a shortage of blood cells resulting in anemia. When these sickle cells travel through small blood vessels, they can get stuck and clog the flow of blood. This can cause pain and other serious health problems like infection, acute chest syndrome (a severe complication in the lungs), strokes, organ damage, and a shorter life span.
SCD is a genetic condition that is present at birth and occurs in one of every 365 Black or African-American births. It is inherited when a child receives two genes – one from each parent – that code for irregular hemoglobin. A person who inherits only one gene is healthy and unaffected, but is a “carrier” of the disease. Carriers have an increased chance of having a child with sickle cell disease if they have a child with another carrier.
SCD is diagnosed with a blood test. For children in the United States, diagnosis is most often at birth during routine newborn screening tests at the hospital. Additionally, diagnostic testing prior to birth, such as chorionic villus sampling and amniocentesis, can check for chromosomal or genetic abnormalities like SCD.
Because children with SCD are at an increased risk of infection and other health complications, early diagnosis and treatment are important.
While there are treatments for the symptoms of SCD and for the complications the disease causes, the only known cure for the disease is a blood stem cell or bone marrow transplant. Prior to the transplant, the cells that produce the abnormal hemoglobin are cleared from the system by chemotherapy. Then, healthy cells, donated by a family member or a donor with the same Human Leukocyte Antigen type, are transplanted to replace these cells. These healthy blood stem cells then begin to make normally-shaped blood cells.
However, for the transplant to work, the donor and the patient need to have matching immune system factors, called Human Leukocyte Antigens (HLA). People get their HLA types from their parents, one half from each parent. Only about 30% of patients can find a match with another family member; the other 70% of patients must search the registry for an unrelated donor. People with the same ethnicity have the best chance of being HLA matches. Looking at where your ancestors were living generations ago can give doctors a good idea of who might be the perfect match.
Becky (l) donated bone marrow to save Nelson's life. Nelson received his transplant when he was a 16-year-old high school student. When he met Becky a year after his transplant, he told her, “I never thought this day would come. Through all of the struggles, I kind of just wanted to give up, but something told me, ‘keep on going, push forward, something great will happen to you in the future.’ If it wasn’t for you, I could be dead – but now anything is possible from this point on!” Read their story.
Because the only known cure for SCD is a bone marrow or stem cell transplant, the best way to help is joining the registry so more donors are available. Anyone 18 to 35 years old and in general good health can join the registry by completing a cheek swab kit at an in-person drive or by ordering a kit to have sent to your home. Being on the registry is incredibly important, especially in regards to Sickle Cell Disease and related diseases like Beta Thalassemia. These diseases disproportionately affect Black Americans, people of Mediterranean ancestry, and South Asian ancestry, and only 25% of Black and Multiracial people are able to find a donor match. We need everyone to join the registry, but especially peoples of color to combat genetic diseases like SCD.
You can also organize and host your own donor recruitment events. Gift of Life has over 30 years of experience organizing events, and we stand ready to help you coordinate yours.
You can also help out by spreading the word. Following Gift of Life on social media and sharing this article and others like it will help share information about what our organization does and why our mission is so important. This can help others join the registry!
Anyone 18 to 35 and in general good health can join the stem cell/marrow registry. Click here to start the registration process and receive a cheek swab kit delivered to your home.
To learn more about the urgent need to increase the diversity of donors in the registry, so that more lives can be saved, click here.
Gift of Life expresses its gratitude to the William G. Pomeroy Foundation for their generous support of the organization’s initiatives to increase diversity in the registry.