sickle-cell disease

Sickle cell disease (SCD), also simply called sickle cell, or sickle cell anemia is a group of inherited hemoglobin-related blood disorders. The most common type is known as sickle cell anaemia. Sickle cell anaemia results in an abnormality in the oxygen-carrying protein haemoglobin found in red blood cells. This leads to the red blood cells adopting an abnormal sickle-like shape under certain circumstances. With this shape, they cannot deform as they pass through capillaries, causing blockages.

Problems in sickle cell disease typically begin around 5 to 6 months of age. Several health problems may develop, such as attacks of pain (known as a sickle cell crisis) in joints, anaemia, swelling in the hands and feet, bacterial infections, dizziness and stroke. The probability of severe symptoms, including long-term pain, increases with age. Without treatment, people with sickle cell disease rarely reach adulthood, but with good healthcare, median life expectancy is between 58 and 66 years. All of the major organs are affected by sickle cell disease. The liver, heart, kidneys, lungs, gallbladder, eyes, bones, and joints can be damaged by abnormal sickle cells and their inability to flow through the small blood vessels.