Sickle cell disease
What is Sickle cell disease?
Sickle cell disease is a genetic blood disorder that affects how your red blood cells work. Normal red blood cells are round and flexible, moving easily through blood vessels. In sickle cell disease, red blood cells become crescent or sickle shaped and rigid. These abnormal cells can block blood flow and break apart more quickly than healthy cells.
The condition affects hemoglobin, the protein in red blood cells that carries oxygen throughout your body. When hemoglobin is abnormal, it causes cells to become sickle shaped. This leads to chronic anemia, painful episodes called crises, and potential organ damage over time. The disease is inherited, meaning you get it from your parents through genes.
Sickle cell disease is lifelong and requires ongoing medical care. Early diagnosis through blood testing helps manage symptoms and prevent complications. Understanding your red blood cell count, hemoglobin levels, and inflammation markers helps guide treatment decisions and track disease progression.
Symptoms
- Painful episodes or crises, especially in hands, feet, chest, and abdomen
- Chronic fatigue and weakness from anemia
- Swelling in hands and feet, especially in young children
- Frequent infections due to spleen damage
- Delayed growth and development in children
- Vision problems from blood vessel damage in the eyes
- Yellowing of skin and eyes, known as jaundice
- Pale skin and nail beds from low red blood cell count
- Shortness of breath during physical activity
Symptoms vary widely from person to person. Some people have mild symptoms while others experience severe complications. Symptoms often begin in infancy, though newborn screening now catches most cases early.
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Causes and risk factors
Sickle cell disease is caused by inheriting abnormal hemoglobin genes from both parents. The condition follows an autosomal recessive pattern, meaning you need two copies of the sickle cell gene to have the disease. If you inherit only one copy, you have sickle cell trait, which usually causes no symptoms but can be passed to children. The genetic mutation affects the beta-globin gene that tells your body how to make hemoglobin.
You cannot develop sickle cell disease from lifestyle choices or environmental factors. It is present from birth and cannot be prevented if you inherit the genes. Sickle cell disease is most common in people with African, Mediterranean, Middle Eastern, and South Asian ancestry. The gene may have persisted in these populations because carrying one copy provides some protection against malaria.
How it's diagnosed
Sickle cell disease is diagnosed through blood tests that examine red blood cells and hemoglobin structure. Most cases are detected through newborn screening programs that test all babies shortly after birth. If diagnosed later in life, doctors use a hemoglobin electrophoresis test to identify abnormal hemoglobin types. A complete blood count measures red blood cell levels and helps assess anemia severity.
Rite Aid offers blood testing through Quest Diagnostics that measures hemoglobin, red blood cell count, and inflammation markers like sedimentation rate. These tests help monitor disease activity and detect complications. Regular blood testing is essential for people with sickle cell disease to track anemia, watch for infections, and adjust treatment as needed. Testing helps your care team catch problems early and prevent serious complications.
Treatment options
- Hydroxyurea medication to reduce painful crises and anemia
- Pain management with over-the-counter or prescription medications during crises
- Drinking 8 to 10 glasses of water daily to prevent cell sickling
- Avoiding extreme temperatures, high altitudes, and dehydration triggers
- Folic acid supplements to support red blood cell production
- Vaccinations and antibiotics to prevent infections
- Blood transfusions for severe anemia or complications
- Newer medications like voxelotor and crizanlizumab for specific cases
- Bone marrow or stem cell transplant, the only potential cure
- Regular medical checkups every 3 to 12 months to monitor health
Concerned about Sickle cell disease? Get tested at Rite Aid.
- Simple blood draw at your nearest lab
- Results in days, not weeks
- Share results with your doctor
Frequently asked questions
Hemoglobin electrophoresis is the primary test to diagnose sickle cell disease. It identifies abnormal hemoglobin types in your blood. A complete blood count measures red blood cell levels and hemoglobin concentration. Sedimentation rate tests can show inflammation and help monitor disease activity.
Bone marrow or stem cell transplant is currently the only potential cure for sickle cell disease. This procedure replaces abnormal blood-forming cells with healthy donor cells. However, it carries significant risks and is usually reserved for severe cases. Most people manage the condition with medications and lifestyle changes rather than pursuing transplant.
Most people with sickle cell disease need blood tests every 3 to 6 months to monitor hemoglobin and red blood cell levels. Your doctor may order tests more frequently if you have complications or start new treatments. Regular testing helps catch problems early and adjust medications as needed.
Painful crises happen when sickle-shaped cells block blood flow in small vessels. This prevents oxygen from reaching tissues, causing pain and potential damage. Triggers include dehydration, temperature extremes, stress, illness, and high altitudes. Staying hydrated and avoiding triggers helps reduce crisis frequency.
No, sickle cell trait means you inherited one sickle cell gene and one normal gene. People with trait usually have no symptoms and live normal lives. Sickle cell disease requires two sickle cell genes and causes ongoing health problems. Trait carriers can pass the gene to their children.
Hydroxyurea is the most common medication and reduces painful crises by increasing healthy red blood cells. Newer drugs like voxelotor help prevent cell sickling, while crizanlizumab reduces blood vessel inflammation. Pain medications manage acute crises. Your doctor will recommend treatments based on your specific symptoms and disease severity.
Yes, staying well hydrated is one of the most important lifestyle measures. Drinking plenty of water helps prevent cells from sickling. Avoiding temperature extremes, getting adequate rest, and managing stress also reduce crisis risk. Eating a balanced diet with folic acid supports red blood cell health.
Sickle-shaped red blood cells break apart more quickly than normal cells, living only 10 to 20 days instead of 120 days. Your body cannot make new cells fast enough to replace them. This causes chronic anemia with low red blood cell counts and hemoglobin levels. The ongoing cell destruction also releases substances that can damage organs.
Yes, pregnancy poses higher risks for people with sickle cell disease. Complications can include more frequent painful crises, anemia, infections, and high blood pressure. Babies may be born early or smaller than average. Close monitoring with a specialized care team throughout pregnancy helps manage risks and ensure the best outcomes.
Sickle cell disease can damage many organs over time due to blocked blood flow and repeated oxygen deprivation. Common complications include stroke, acute chest syndrome, organ damage to kidneys and liver, vision loss, and chronic pain. Infections are more common because the spleen becomes damaged. Regular medical care and blood testing help detect and prevent these complications.