Sickle Cell Anemia

Sickle cell disease is a genetic disorder of red blood cells in which the normal flow of blood in the body is hampered because of the change in shape of red blood cells due to abnormal hemoglobin S (also called sickle hemoglobin) which can form hard fibers inside the red cells, causing them to reshape into sickle (banana) shape. Abnormally shaped red blood cells cannot move through blood vessels easily and at times are blocked from delivering oxygen to some body tissues.

Symptoms

• Painful swelling of hands and feet requiring hospitalization
• High fevers because of bacterial infection secondary to autosplenectomy
• Stroke
• Paleness, fatigue and rapid pulse because of anaemia from fewer red blood cells
• Chest pain
• Yellowing of eyes and skin (jaundice)
• Delayed growth or onset of puberty
• Abdominal pain due to sickle crisis

Treatment

• Hydroxyurea
  Transfusion Dependent Thalassemia (TDTs) requires regular blood transfusion for a patient to survive. Without adequate transfusion support, they can suffer several complications and have a short life. This includes beta thalassemia major.
• Transfusions of red blood cells
  Non-transfusion-dependent thalassemia (NTDT) patients do not require lifelong regular transfusions for survival, but they may require occasional or even frequent transfusions in certain clinical settings.
• >Haematopoeitic Stem cell transplant
  Third group includes persons with Thalassemia minor/Trait who have mild anaemia only. They do not need any treatment.

Prevention

Genetic counselling plays an important role in prevention of this disease.