Sickle Cell Disease

Sickle cell disease is also known as sickle cell anemia. This condition affects red blood cells (RBC's). Sickle cell disease is a genetically inherited disorder. RBC's are important in the body, because they contain hemoglobin. Hemoglobin carries oxygen to all of the tissues in the body. People who suffer from sickle cell disease have abnormally shaped hemoglobin molecules that look like sickles, and they cannot carry oxygen as well as normal hemoglobin molecules. The sickle cells also have a chemical on their surface that causes them to stick to vessel walls, so they have a more difficult time squeezing through small vessels. Sickle cells also have a shorter lifespan compared to normal RBC's. This means that the body's bone marrow, where RBC's are produced, must work harder to try and make RBC's faster than the die. However, for many individuals suffering from sickle cells disease, there bone marrow is not efficient enough. The resulting condition is known as anemia (a low number of RBC's). The severity of sickle cell disease depends on many factors, including, oxygen deprivation, concentration of hemoglobin, and the amount of a protective molecule called hemoglobin F (F for fetal). The people with greater amounts of hemoglobin F are better protected.

RISK FACTORS

  • Family members with sickle cell disease (both parents have to have the abnormal gene).

  • Illness.

  • Exertion.

  • Dehydration.

  • Family origins in areas with high incidence of malaria (Africa, India, the Mediterranean, South and Central America, the Caribbean, and the Middle East).

  • Physical stress.

  • High altitude.

SYMPTOMS

  • Fever.

  • Swelling of the hands and feet.

  • Enlargement of the belly (heart, liver, and spleen).

  • Frequent lung infections.

  • Fatigue.

  • Irritability.

  • Yellowing of the skin (jaundice).

  • Severe bone and joint pain.

  • Delayed puberty.

  • Shortness of breath.

  • Pain in the belly, especially in the upper right side of the abdomen.

  • Nausea.

  • Prolonged, sometimes painful erections (priapism).

  • Rapid or labored breathing.

  • Frequent infections.

PREVENTION

  • There are no proven methods for preventing sickle cell crises.

  • Regularly follow up with your doctor.

  • Avoid dehydration.

  • Avoid high-altitude travel, especially rapid increases in altitude.

  • Avoid stress.

  • Get plenty of rest.

  • Stay warm.

  • Female patients may drink cranberry juice to help prevent urinary tract infections.

  • Maintain good nutrition by eating green, red, and yellow vegetables; fruits; and juices that are rich in antioxidants and other important nutrients.

  • Eat fish and soy products that are high in omega-three fatty acids.

  • Make sure you consume enough folic acid, zinc, vitamin E, vitamin C, and L -glutamine.

  • Blood transfusions.

  • Immunizations, particularly against the flu and some bacteria, may reduce the risk of infection and thus sickle crisis.

TREATMENT

If sickle cell disease causes painful symptoms, then over-the-counter pain medications (ie. acetaminophen or ibuprofen) may be used, but consult your caregiver before use. Sickle cell disease is treated by managing pain and maintaining hydration. For patients with shortness of breath or difficulty breathing, oxygen may be given. Children who are at high-risk for the disease may be give blood transfusions. Other medications exist to help the body produce protective hemoglobin, it is important to discuss these with your caregiver.