26 September 2017 - The medical definition of sickle cell disease – a group of inherited red blood cell disorders caused by abnormal haemoglobin, called haemoglobin S or sickle haemoglobin, in red blood cells – does not come close to describing the condition from the patient’s perspective. 

Sickle cell disorders have devastating effects on patients and their families. Patients often experience recurrent episodes of excruciating pain, or sickle cell crisis, debilitating fatigue, infections, cognitive disorders, strokes, a life-threatening condition called acute chest syndrome, and damage to their vital organs, tissues, and bones. In some patients, the disease may trigger frequent and very painful sickle cell crises that require hospitalisation. 

In others, it may cause less frequent and milder attacks. Although mortality during childhood has improved progressively, the life expectancy among individuals with sickle cell disease in the United States is on average 30 years less than the general population.

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