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Symptoms of Sickle Cell Anemia

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

  • Anemia. This is the most common symptom of all the sickle cell anemia. In sickle cell disease, red blood cells are produced but then become deformed into the sickle shape, which causes red blood cells to lose their ability to carry oxygen. This sickle shape makes the cells stiff and sticky causing them to become stuck in the vessels, destroyed by the spleen, or simply die because of their abnormal function. The decrease in red blood cells causes anemia. Severe anemia can make the person's ability to carry oxygen to the tissues more difficult, possibly causing them to be pale, dizzy, short of breath, and tired. Healing and normal growth and development may be delayed because of chronic anemia.
  • Pain crisis or sickle crisis. This occurs 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 vaso-occlusive 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 younger than age 3. 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. This occurs when sickling is in the lungs. This can be a life-threatening complication of sickle cell anemia. 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. Damage to the blood vessels in the lungs can lead to high blood pressures in the lungs (pulmonary hypertension), a condition that can cause death in adults with sickle cell anemia.
  • Splenic sequestration (pooling). This complication is 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 can also become enlarged and painful from the increase in blood volume. After repeated episodes of splenic sequestration, the spleen becomes scarred, and permanently damaged. By age 8, most children do not have a functioning spleen either from damage related to the sickled cells, surgical removal, or from repeated episodes of splenic sequestration. The risk of infection is a major concern of children without a functioning spleen. Infection is the major cause of death in children younger than age 5 in this population.
  • Stroke. This is another sudden and severe complication of people with sickle cell anemia. 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 from sickle cell anemia, a person is more likely to have a second and third stroke.
  • Jaundice, or yellowing of the skin, eyes, and mouth. Jaundice is a common sign and symptom of sickle cell disease. Sickle cells do not live as long as normal red blood cells and, therefore, they are dying 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.
  • Priapism. A painful obstruction of the penis by sickle cells. If not promptly treated, it can result in impotence (an inability to have an erection) and possibly infertility.

Any and all major organs are affected by sickle cell anemia. The liver, heart, kidneys, gallbladder, eyes, bones, and joints can suffer damage from the abnormal shape and 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
  • Multiple organ failure

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