Sickle Cell Anemia – Causes, Symptoms, Diagnosis, And Treatment
What is sickle cell anemia?
Sickle cell anemia or sickle cell disease (SCD) is a group of inherited red blood cells disorders. The red blood cells that are normally shaped like a disc break down and turn into a sickle or crescent shape. Red blood cells deliver oxygen throughout the body.
What causes sickle cell anemia?
Sickle cell anemia is caused by an abnormal type of hemoglobin called hemoglobin S. Hemoglobin is a protein inside red blood cells that carries oxygen. Hemoglobin S causes changes in the red blood cells. The red blood cells become fragile and shaped like crescents or sickles. The abnormal red blood cells carry and deliver less oxygen to the body’s tissues. The abnormal red blood cells can also easily get stuck in small blood vessels and break into pieces; this can interrupt smooth blood flow and further cut down the amount of oxygen flowing to body tissues.
Sickle cell anemia is inherited from both parents. If you get the sickle cell gene from only one parent, you will have sickle cell trait. People with sickle cell trait do not have the symptoms of sickle cell anemia.
What are the symptoms of sickle cell anemia?
Sickle cell anemia symptoms usually do not occur until after the age of 4 months. Younger children with sickle cell anemia have attacks of abdominal pain.
Almost all people with sickle cell anemia have painful episodes called crises. These can last from hours to days. Crises can cause pain in the lower back, leg, joints, and chest.
Some people have 1 episode every few years. Others have many episodes each year. The crises sometimes can become severe enough to require a hospital stay.
When the sickle cell anemia becomes more severe, the following symptoms may occur:
- Rapid heart rate
- Shortness of breath
- Yellowing of the eyes and skin (jaundice)
The following symptoms may occur when small blood vessels are blocked by the abnormal cells:
- Priapism (painful and prolonged erection)
- Poor eyesight or blindness
- Problems with thinking
- Confusion caused by small strokes
- Ulcers on the lower legs (in adolescents and adults)
Over a period of time, the spleen may stop working. As a result, people with sickle cell anemia may have following symptoms of infections:
- Osteomyelitis (bone infection)
- Cholecystitis (gallbladder infection)
- Pneumonia (lung infection)
- Urinary tract infection
The following are the other symptoms of sickle cell anemia:
- Delayed growth and puberty
- Painful joints caused by arthritis
- Heart or liver failure due to too much iron (from blood transfusions)
How is sickle cell anemia diagnosed?
Your doctor will review your symptoms. If he suspects sickle cell anemia, the following tests may be ordered for diagnosis:
- Blood oxygen saturation
- Complete blood count (CBC)
- Hemoglobin electrophoresis
- Serum creatinine
- Serum potassium
- Sickle cell test
How is sickle cell anemia treated?
The main goal of treatment would be to manage and control symptoms and to limit the number of crises. People with sickle cell disease need to be treated constantly even when not having a crisis. People with sickle cell anemia should take folic acid supplements to help make new red blood cells.
Your doctor may recommend the following treatment for a sickle cell crisis:
- Blood transfusions (may also be given regularly to prevent stroke)
- Pain medicines
- Plenty of fluids
Your doctor may also recommend the following other treatments for sickle cell anemia:
- Hydroxyurea (Hydrea): This medication is used to help reduce the number of pain episodes (including chest pain and breathing problems) in some people.
- Antibiotics: Antibiotics are used to help prevent bacterial infections that are common in children with sickle cell disease.
- Medicines to reduce the amount of iron in the body.
Your doctor may recommend the following treatments to manage complications of sickle cell anemia:
- Dialysis or kidney transplant for kidney disease
- Counseling for psychological complications
- Gallbladder removal in people with gallstone disease
- Hip replacement for avascular necrosis of the hip
- Surgery for eye problems
- Treatment for overuse or abuse of narcotic pain medicines
- Wound care for leg ulcers
Your doctor may recommend taking the following vaccinations to lower the risk of infections:
- Haemophilusinfluenzae vaccine (Hib)
- Pneumococcal conjugate vaccine (PCV)
- Pneumococcal polysaccharide vaccine (PPV)
This feature is for informational purposes only and is not intended to substitute the expert guidance of a doctor. We advise seeing a doctor if you have any health concerns.