Today (June 19th) is World Sickle Cell Day! Every year, the international health community recognizes Sickle Cell Disease (SCD) as a debilitating genetic disease that impacts families across the globe. Keeping individuals and communities informed about the struggles that come with daily management of SCD, can help raise awareness as well as debunk stereotypes and stigmas associated with persons who have SCD.
SCD affects millions of people worldwide and is particularly common among people originating from sub-Saharan Africa, Saudi Arabia, India, South America and Central America, and Mediterranean countries, such as Turkey, Greece and Italy.
SCD affects approximately 100,000 Americans and occurs in about 1 out of every 365 African-American births. Individuals living with SCD suffer from both acute and chronic complications that require frequent contact with the medical system. These complications include acute sickle cell pain, fever, and acute chest syndrome (ACS), which is the term used for a number of different findings that includes chest pain, cough, fever, hypoxia and new lung infiltrates.
Here are some quick facts about SCD:
- Sickle Cell Disease is a group of inherited red blood cell disorders. The red blood cells become hard and sticky and look like a C-shaped farm tool called a “sickle” and the cells die early, which causes a constant storage of red blood cells. Healthy red blood cells are round and move through small blood vessels to carry oxygen to all parts of the body.
- To get SCD, the trait must be inherited from both parents who already have the SCD trait. People with the trait usually do not have any of the signs of the disease and live a normal life, but they can pass the trait to their children.
- SCD is diagnosed with a simple blood test. It is most often found at birth during routine newborn screening tests. Early diagnosis and treatment are important.
- There are several treatment options available for individuals living with different complications of SCD, but the only cure for SCD is a bone marrow or stem cell transplant. Transplants are very risky, and can have serious side effects, including death. For the transplant to work, the bone marrow must be a close match (usually a brother or sister).
SCD and COVID-19
Amid the COVID-19 pandemic, there is significant concern that the overlap of lung disease from COVID-19 with ACS may result in increased complications and amplification of healthcare utilization among individuals with SCD. Patients with SCD often have underlying cardiopulmonary co-morbidities that may predispose them to poor outcomes if they become infected with COVID-19. Source: hematology.org.
Below are recommendations from the Sickle Cell Disease Association of America (SCDAA) for patients with SCD regarding COVID-19:
- Patients and parents should be educated about COVID-19 signs and symptoms and the importance of physical distancing to limit chances of exposure and infection.
- Patients and parents should receive counseling to continue to monitor for fever or other signs of infection. Call hospital, doctor, or nurse first for advice on where to go for an evaluation.
- Be sure that patients have an ample supply of all prescribed medication at home (including analgesics) to manage both acute and chronic pain.
- Patients should adhere closely to the correct use of medications.
DHEC is committed to educating the community and public about the availability of resources and services for individuals and families living with sickle cell disease. DHEC’s Division of Children and Youth with Special Health Care Needs (CYSHCN), provides assistance to persons with sickle cell disease by covering services, such as, medical expenses, physician visits, durable medical equipment, medical supplies, and prescription drugs. Assistance is offered to both children and adults who meet eligibility requirements.
Additionally, CYSHCN engages with the four Sickle Community Based Organizations in the state — the James R. Clark Memorial Sickle Cell Foundation, – Louvenia D. Barksdale Sickle Cell Anemia Foundation, – Orangeburg Area Sickle Cell Foundation and – COBRA Human Services Agency Sickle Cell Program – to ensure persons living with SCD have access to community resources and support services.