

What is it?





Signs and symptoms of sickle cell anemia usually show up after an infant is 4 months old and may include:

  • Anaemia. Sickle cells are fragile. They break apart easily and die, leaving you chronically short on red blood cells. Red blood cells usually live for about 120 days before they die and need to be replaced. However, sickle cells die after only 10 to 20 days. The result is a chronic shortage of red blood cells, known as anemia. Without enough red blood cells in circulation, your body can't get the oxygen it needs to feel energized. That's why anemia causes fatigue.
  • Jaundice. Jaundice is a yellowing of the skin and eyes that occurs because of liver damage or dysfunction. Occasionally, people who have sickle cell anemia have some degree of jaundice because the liver, which filters harmful substances from the blood, is overwhelmed by the rapid breakdown of red blood cells. In people with dark skin, jaundice is visible mostly as yellowing of the whites of the eyes.
  • Frequent infections. Sickle cells can damage your spleen, an organ that fights infection. This may make you more vulnerable to infections. Doctors commonly give infants and children with sickle cell anemia antibiotics to prevent potentially life-threatening infections, such as pneumonia.


Sickle cell anemia is caused by a mutation in the gene that tells your body to make hemoglobin — the red, iron-rich compound that gives blood its red color. Haemoglobin is a component of every red blood cell. It allows red blood cells to carry oxygen from your lungs to all parts of your body, and to carry carbon dioxide waste from throughout your body to your lungs so that it can be exhaled.

Under normal circumstances, your body makes healthy hemoglobin known as haemoglobin A. People with sickle cell anemia make hemoglobin S — the S stands for sickle.


People with sickle cell trait have one normal haemoglobin gene and one defective form of the gene. So their bodies make both normal haemoglobin and sickle cell haemoglobin. Their blood may contain some sickle cells, but they generally don't experience symptoms. However, they are carriers of the disease, which means they can pass the defective gene on to their children.

With each pregnancy, two people with sickle cell traits have:

  • A 25 percent chance of having an unaffected child with normal haemoglobin

Risk factors

The risk of inheriting sickle cell anemia comes down to genetics. For a baby to be born with sickle cell anaemia, both parents must carry a sickle cell gene.

The gene is particularly common among people with African, Spanish, Mediterranean, Middle Eastern and Indian ancestry. 



  • Stroke. A stroke can occur if sickle cells block blood flow to an area of your brain. Stroke is one of the most serious complications of sickle cell anemia. Signs of stroke include seizures, weakness or numbness of your arms and legs, sudden speech difficulties, and loss of consciousness. If your baby or child has any of these signs and symptoms, seek medical treatment immediately. A stroke can be fatal.
  • Acute chest syndrome. This life-threatening complication of sickle cell anemia causes chest pain, fever and difficulty breathing. Acute chest syndrome can be caused by a lung infection or by sickle cells blocking blood vessels in your lungs. It requires emergency medical treatment with antibiotics, blood transfusions and drugs that open up airways in your lungs. Recurrent attacks can damage your lungs.
  • Pulmonary hypertension. About one-third of people with sickle cell anemia will eventually develop high blood pressure in their lungs (pulmonary hypertension). Shortness of breath and difficulty breathing are common symptoms of this condition, which can ultimately lead to heart failure.
  • Blindness. Tiny blood vessels that supply your eyes can get blocked by sickle cells. Over time, this can damage the retina — the portion of the eye that processes visual images — and lead to blindness.
  • Gallstones. The breakdown of red blood cells produces a substance called bilirubin. Bilirubin is responsible for yellowing of the skin and eyes (jaundice) in people with sickle cell anemia. A high level of bilirubin in your body can also lead to gallstones.
  • Priapism. Men with sickle cell anemia may experience painful erections, a condition called priapism. As occurs in other parts of the body, sickle cells can block the blood vessels in the penis. This can damage the penis and eventually lead to impotence.





Additional tests




Once diagnosed with sickle cell anemia, your child will need to see his or her doctor more often than most children do. For children under 2, ask your child's doctor how frequently checkups need to be scheduled. For youngsters over age 2, at least two visits a year are usually scheduled.