Although newborns can be screened for sickle cell disease (SCD) from birth, signs and symptoms typically don’t appear until 5 or 6 months of age. The type and intensity of these signs and symptoms vary among individuals and may change over time, depending on which organs are affected.[1]
SCD is characterized by the accelerated breakdown of sickled red blood cells (hemolysis), which reduces the number of circulating red blood cells, causing anemia. This decreases the oxygen supply to organs and tissues. Common symptoms of anemia include fatigue, tiredness, irritability, and in more severe cases, shortness of breath. Accelerated hemolysis can also cause jaundice (yellowing of the skin), or icterus (yellowing of the white part of the eye, also called the sclera). These are among the earliest signs of SCD.[2]
Sickled red blood cells are rigid and adhesive, which can obstruct small blood vessels. This can cause episodes of pain, known as pain crises, of varying intensity and frequency. Pain crises can affect any part of the body, but most commonly the lower back and lower extremities.[3] Reduced blood flow can also result in swollen hands and feet.[4]
Children’s growth rate and development during puberty may be impacted due to inadequate delivery of oxygen and essential nutrients to the body. Children affected by SCD are also more susceptible to frequent infections.[2]