Sickle Cell Disorders cause the red blood cells to change, under certain conditions, to a shape resembling that of a farmer's sickle or half-moon. These cells break down quickly and block the small blood vessels causing problems like anaemia, tissue damage, mild to excruciating pain and infections. This is called “Sickling “and can cause bone and joint problems, problems in the kidneys, spleen, lungs and strokes in early childhood. Symptoms generally start after the age of three months.
Treatment includes a daily dose of penicillin in childhood to reduce the risk of getting an infection, plenty of fluids and painkillers when needed. Admission to hospital is needed in a “Sickling Crisis” when the pain becomes so severe, that very strong pain relief (for example morphine or pethidine) has to be given. With raised awareness of these conditions and medical advances many people are surviving into late adulthood. However there are still occasional deaths in childhood mainly due to infections. Recent developments in treatment include using cytotoxic drugs such as Hydroxyurea and bone marrow transplantation.
