What Is Sickle Cell Disease?
Sickle cell disease is a group of genetic red blood cell disorders. It is caused by abnormal hemoglobin, a protein in the red blood cells responsible for transporting oxygen throughout the body.
Hemoglobin becomes stiff within the blood cells, changing their shape into a crescent or “sickle” shape. Because of their shape, these blood cells can burst easily, stick to vessel walls, cause blockages in blood flow and prevent oxygen from circulating throughout the body.
The most common sickle cell disease is sickle cell anemia. Other common forms include hemoglobin Sβ+ thalassemia and hemoglobin SC disease.
Sickle cell diseases are inherited, which means they are passed from parents to children.
Most people with sickle cell disease in the United States have African ancestry or identify as black. Approximately one in 365 African American children is born with sickle cell disease.
Symptoms
People are born with sickle cell disease, but it often doesn’t cause symptoms until a baby is 5 or 6 months old. Every state in the U.S. is required to screen babies for sickle cell disease, which allows the parents to find out before the baby has symptoms.
Early signs of the disease include:
- Anemia – Low red blood cell count
- Dactylitis – Painful swelling in the feet and hands
- Icteris – A yellowing of the whites of the eyes
- Jaundice – A yellowish color of the skin
In adults, sickle cell disease can cause sudden onset of acute pain, most often in the abdomen, arms, chest, legs or lower back. This onset of pain can be caused by dehydration, high altitudes, illness, stress and temperature fluctuations.
It can also cause chronic pain.
Because the condition damages the spleen, you may be more susceptible to life-threatening bacterial infection.
When sickling occurs in the blood vessels of the lungs, you can become deprived of oxygen, known as acute chest syndrome. Symptoms include chest pain, cough, fever, rapid breathing and shortness of breath.
IMPORTANT: This is a very serious condition, and you should seek emergency medical care immediately.
It can also increase the risk of:
- Delayed puberty
- Eye problems
- Gall stones
- Heart disease
- Joint problems
- Kidney problems
- Leg ulcers
- Liver problems
- Mental health issues like depression from living with a chronic disease
- Pregnancy complications
- Pulmonary hypertension
- Priapism (prolonged, unwanted, painful erection in men)
- Stroke
Treatment Options
Treatment options include:
- Antibiotics – Medications to fight bacterial infections
- Blood transfusions – When healthy donor blood is given intravenously to replace your blood
- Childhood immunizations – Vaccines that reduce the risk of infections
- Hydroxyurea – Medication that reduces the need for blood transfusions
- Pain relievers – Medications to relief discomfort
Ready for an Appointment?
If you're experiencing signs or symptoms of sickle cell disease, schedule an appointment or call 800-TEMPLE-MED (800-836-7536) today.
Learn more about our doctors and care team who diagnose and treat sickle cell disease.