Sickle cell anemia is an inherited blood disorder that causes blood cells to become hard, sticky, and shaped like a sickle or "C." As a result, while traveling through the blood vessels, cells may become stuck and block the flow of blood throughout the body. This may cause a variety of symptoms and medical problems, including bacterial infections, pain, tissue damage, stroke, and anemia. Blocked blood flow also can cause damage to the spleen, kidneys, and liver.
Normal red blood cells are smooth, donut-shaped and contain hemoglobin A, which helps red blood cells transport oxygen through the blood vessels to the body. People with sickle cell anemia have red blood cells that contain hemoglobin S, an abnormal type of hemoglobin that causes the sickle shape change.
Every September, people living with sickle cell disease, their caregivers, advocates, healthcare providers, and others come together to bring awareness to the disease and dispel the myths and stigmas surrounding it. The month also brings attention to the ongoing need for research, better care practices, new treatments, and an eventual cure.
Facts about sickle cell disease:
- Most common inherited blood disorder in U.S.
- Affects approximately 100,000 Americans
- 1 out of every 365 Black babies are born with sickle cell disease
- 1 out of every 16,300 Hispanic-American babies are born with sickle cell disease
- About 1 in 13 Black babies are born with sickle cell trait
Current therapies mainly focus on managing the symptoms of sickle cell and require lifelong use. Recent progress in sickle cell has led to more treatment options that have the potential to help manage acute and chronic symptoms of the disease. Treatment options may include:
- SICKLE CELL TREATMENTS can help reduce the frequency of pain crises and hospitalizations or reduce the number of blood transfusions a person with sickle cell needs. Other supportive
therapies include, but are not limited to antibiotics, vitamins, vaccinations, and IV and oral hydration
- MEDICATIONS TO MANAGE PAIN can help people with sickle cell manage acute and chronic pain
- BLOOD TRANSFUSIONS can help relieve symptoms of sickle cell and potentially prevent
stroke in high-risk patients but can also lead to iron overload and other serious complications
- HEMATOPOIETIC STEM CELL TRANSPLANT can cure sickle cell using the stem cells of a person who doesn't have the sickle HBB gene mutation, but is only available to people who are under
the age of 18 and have a matched related donor available
General health maintenance and preventative care:
- Drink plenty of water. Avoid getting too hot or too cold. Avoid exposure to high altitudes like flying or mountain climbing. Avoid situations where you may be exposed to low oxygen levels (e.g., strenuous exercise)
- Stay current on vaccinations
- Wash your hands frequently to prevent infections
- Attend regular checkups with your healthcare team
Significant strides have been made in the treatment of sickle cell, but it is not enough. There is still a need for treatments that can eliminate lifelong symptom management and its side effects. Learning about the current treatment options can help you and your loved ones create a comprehensive care plan with your doctor to manage your sickle cell.
Sources: National Institutes of Health, UCSF Benioff Children’s Hospitals, Food and Drug Administration, Be the Spark
