Sickle Cell Anemia

Sickle cell disease is a general term for hemoglobin abnormalities in which the sickle gene is inherited from at least one parent. Sickle cell anemia or hemoglobin SS disease is the condition in which the sickle gene is inherited from both parents. A smaller number have hemoglobin SS disease. Sickle cell hemoglobin causes the red blood cells to become deformed and break up. There are a number of medical problems that happen in people with SS disease. These include:

  • Pain crisis. This is caused by small blood vessels being blocked. This is due to infection or dehydration. There is usually severe pain in the muscles, joints, back, or stomach. Treatment may include IV fluids, transfusion, and pain medicine. Hospital care is often needed.

  • Severe anemia. This often follows an infection. Symptoms include severe shortness of breath, weakness, and shock. Transfusions are needed to correct the problem.

  • Complications. People with sickle cell disease are more likely to get infections, strokes, eye problems, and heart failure.

Athletes with sickle cell trait need to be very careful to avoid dehydration because of the increased risk of pain crisis. Always drink before, during, and after exercising in the heat. Avoid extreme exertion at altitudes above 2,500 feet (762 meters) or if you feel sick.

Many patients in pain crisis or with severe anemia need hospital care to treat the problem.


  • You or your child develops dizziness or fainting, numbness in or difficulty with movement of arms and legs, difficulty with speech, or is acting abnormally.

  • You have a fever.

  • Your baby is older than 3 months with a rectal temperature of 102° F (38.9° C) or higher.

  • Your baby is 3 months old or younger with a rectal temperature of 100.4° F (38° C) or higher.

  • With fevers, do not give medicine to lower the fever right away. This could cover up a problem that is developing.

  • You or your child has other signs of infection (chills, lethargy, irritability, poor eating, or vomiting).

  • You or your child develops pain which is not helped with medications.

  • You or your child develops shortness of breath or coughs up pus-like or bloody sputum.

  • You or your child develops any problems that are new and are causing you to worry.

  • You or your child develops a persistent, often uncomfortable and painful penile erection. This is called priapism. Always check young boys for this. It is often embarrassing for them and they may not bring it to your attention. This is a medical emergenc y and needs immediate treatment. If this is not treated it will lead to impotence.

  • You or your child develops a new onset of abdominal pain, especially on the left side near the stomach area.

  • You have any questions about your child or problems that you feel are not getting better. Return immediately if you feel you or your child is getting worse, even if seen only a short while ago.