Sickle Cell Anaemia
Or Sickle Cell Disease, is a hereditary blood disorder.
What causes this disorder?
How is it inherited?
Symptoms usually do not occur until after the age of 4 months.
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 one episode every few years. Others have many episodes each year. The crises can be severe enough to require a hospital stay.
When the anemia becomes more severe, symptoms may include:
Younger children with sickle cell anemia have attacks of abdominal pain.
The following symptoms may occur because small blood vessels become blocked by the abnormal cells:
- Painful and prolonged erection (priapism)
- Poor eyesight or blindness
- Problems with thinking or confusion caused by small strokes
- Ulcers on the lower legs (in adolescents and adults)
Over time, the spleen stops working. As a result, people with sickle cell anemia may have symptoms of infections such as:
- Bone infection (osteomyelitis)
- Gallbladder infection (cholecystitis)
- Lung infection (pneumonia)
- Urinary tract infection
Other symptoms include:
- Delayed growth and puberty
- Painful joints caused by arthritis
The goal of treatment is to manage and control symptoms, and to limit the number of crises. People with sickle cell disease need ongoing treatment, even when not having a crisis. It is best to get care from health care that take care of many patients with sickle cell anemia.
People with this condition should take folic acid supplements. Folic acid helps make new red blood cells.
Treatment for a sickle cell crisis includes:
- Blood transfusions (may also be given regularly to prevent stroke)
- Pain medicines
- Plenty of fluids
Other treatments for sickle cell anemia may include:
- Hydroxyurea (Hydrea). Helps reduce the number of pain episodes (including chest pain and breathing problems) in some people
- Antibiotics. Help prevent bacterial infections, which are common in children with sickle cell disease
Treatments that may be needed to manage complications of sickle cell anemia include:
- 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
Bone marrow or stem cell transplants can cure sickle cell anemia, but this treatment is not an option for most people. Sickle cell anemia patients often cannot find well-matched stem cell donors.
People with sickle cell disease should have the following vaccinations to lower the risk of infection:
- Haemophilus influenzae vaccine (Hib)
- Pneumococcal conjugate vaccine (PCV)
- Pneumococcal polysaccharide vaccine (PPV)