Sickle Cell Disease

A sickle cell compared to two normal cells

Sickle cell disease is an inherited blood disorder affecting red blood cells. Normal red blood cells contain hemoglobin A. People with sickle cell disease have red blood cells containing mostly hemoglobin S, an abnormal type of hemoglobin. These red blood cells become sickle-shaped (crescent-shaped), and have difficulty passing through small blood vessels. There are several different types of sickle cell disease, the most common types are homogzygous sickle cell disease (SS disease), sickle cell-hemoglobin C disease (SC disease) and sickle-cell beta thalassemia (Sßor Sßdisease).

How do people get sickle cell disease?

  • Sickle cell disease is not spread like a cold and cannot be caught from another person.
  • It is an inherited condition.
  • Sickle cell trait is a carrier condition for sickle cell disease .
  • Sickle cell trait originated many years ago in areas of the world where malaria was present.
  • People with sickle cell trait inherit one gene for normal hemoglobin A and one gene for defective hemoglobin S.
  • People with sickle cell disease inherit a hemoglobin S gene from one parent and another abnormal hemoglobin from the other parent (i.e., hemoglobin S, hemoglobin C or beta thalassemia).
  • If both parents have hemoglobin S trait there is a one-in-four chance with each pregnancy the child will have SS disease.
  • If one parent has hemoglobin S trait and one parent has hemoglobin C trait, there is a one-in-four chance with each pregnancy the child will have SC disease.
  • If one parent has hemoglobin S trait and one parent has beta thalassemia trait, there is a one in four chance with each pregnancy the child will have Sß + or Sß 0 disease.

Important facts about sickle cell disease

  • Diagnosis of sickle cell disease can only be determined by a special blood test.
  • Comprehensive care includes early diagnosis, preventive measures, treatment of complications, and ongoing patient education.
  • Many people with sickle cell disease live long and productive lives.
  • Individuals with sickle cell disease can pursue a variety of vocations and professions.
  • Many adolescents with sickle cell disease experience delayed puberty (the average delay is about two years).
  • Yellowing of the eyes is common and should not be confused with hepatitis.
  • The use of alcohol, "street" drugs and tobacco can greatly increase the risk of developing serious complications.