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Signs and symptoms

The following is a list of symptoms and complications associated with sickle cell disease. However, each individual may experience symptoms differently. Symptoms and complications may include, but are not limited to, the following:

  • Anemia – the most common symptom of all the sickle cell diseases. In sickle cell disease, red blood cells are produced but then become deformed into the sickle shape, which causes them to lose their oxygen-carrying capacity. The body subsequently becomes dehydrated or feverish. This sickle shape makes the cells stiff and sticky causing them to become stuck in the vessels, to be destroyed by the spleen or simply to die because of their abnormal function. The decrease in red blood cells causes anemia. Severe anemia can make a person pale and tired, and makes the person’s ability to carry oxygen to the tissues more difficult. Healing and normal growth and development may be delayed because of chronic anemia.
  • Pain crisis or sickle crisis – when the flow of blood is blocked to an area because the sickled cells have become stuck in the blood vessel. These are also called “vasoocclusive crises.” The pain can occur anywhere, but most often occurs in the chest, arms and legs. Painful swelling of the fingers and toes, called dactylitis, can occur in infants and children under 3 years of age. Priapism is a painful sickling that occurs in the penis. Any interruption in blood flow to the body can result in pain, swelling and possible death of the surrounding tissue not receiving adequate blood and oxygen.
  • Acute chest syndrome – when sickling is in the chest. This can be a life-threatening complication of sickle cell disease. It often occurs suddenly, when the body is under stress from infection, fever or dehydration. The sickled cells stick together and block the flow of oxygen in the tiny vessels in the lungs. It resembles pneumonia and can include fever, pain and a violent cough. Multiple episodes of acute chest syndrome can cause permanent lung damage.
  • Splenic sequestration (pooling) – crises are a result of sickle cells pooling in the spleen. This can cause a sudden drop in hemoglobin and can be life threatening if not treated promptly. The spleen also can become enlarged and painful from the increase in blood volume. After repeated episodes of splenic sequestration, the spleen becomes scarred and permanently damaged. Most children affected by sickle cell do not have a functioning spleen by the age of 8 — either from surgical removal or repeated episodes of splenic sequestration. The risk of infection is a major concern in children without functioning spleens. Infection is the major cause of death in children under the age of 5 years in this population.
  • Stroke – another sudden and severe complication of persons with sickle cell disease. The misshapen cells can block the major blood vessels that supply the brain with oxygen. Any interruption in the flow of blood and oxygen to the brain can result in devastating neurological impairment. Having had one stroke, a person is 60 percent more likely to have a second and third stroke.
  • Jaundice or yellowing of the skin, eyes and mouth – a common sign and symptom of sickle disease. Sickle cells do not live as long as normal red blood cells and, therefore, they die more rapidly than the liver can filter them out. Bilirubin (which causes the yellow color) from these broken-down cells builds up in the system causing jaundice.

Any and all major organs are affected by sickle cell disease. The liver, heart, kidneys, gall bladder, eyes, bones and joints can suffer damage from the abnormal function of the sickle cells and their inability to flow through the small blood vessels correctly. Problems may include the following:

  • Increased infections.
  • Leg ulcers. 
  • Bone damage. 
  • Early gallstones. 
  • Kidney damage and loss of body water in the urine.
  • Eye damage.

The symptoms of sickle cell disease may resemble other blood disorders or medical problems. Always consult your physician for a diagnosis.