Pain episodes, also known as crises, are hallmark clinical manifestations of the disease. These episodes can begin in childhood and are experienced periodically throughout the individual’s lifetime. They may be felt in various parts of the body including the arms, legs, chest, and abdomen. Pain episodes often impair one’s normal function, can affect the quality of life, and can be disabling resulting in frequent visits to emergency departments or hospitalizations.
An individual with sickle cell disease who has pain or symptoms of the disorder should not automatically assume that the cause is sickle cell. Not all pain or illness is sickle cell related! Individuals should have their health care providers diagnose any major illness. However, if an illness was caused by sickle cell disease, most likely it will be a direct result of the sickled red blood cells blocking circulation to tissues and organs.
When sickle cells block blood flow, many organs can be damaged. There are numerous tiny blood vessels in the lungs, spleen, bone, brain, eyes and penis. Damaged lung tissue can cause acute chest syndrome. Damage to the spleen may increase the possibility of being overwhelmed by certain infections (e.g., bacterial infections in young children). Damage to brain tissue can result in strokes. Blocked veins in the penis can cause priapism (painful prolonged erection).
Normal red blood cells (hemoglobin A) have a life span of about three to four months. Sickle red blood cells (hemoglobin S) have a life span of about four to six weeks. New red blood cells produced by the bone marrow cannot keep up with the rate of the destroyed sickle cells. Individuals with sickle cell disease who overexert themselves may experience sudden exhaustion because of the anemia. Overexertion will cause shortness of breath and will decrease oxygen supply for the body. Major symptoms of sickle cell disease include fatigue, anemia, pain crises, and bone infarctions (areas of the organ are nonfunctional due to destruction and the death of organ tissues due to the lack of blood supply). Other complications that may occur in an individual’s early years of life include fever, abdominal pain, bacterial infections, painful swelling of the hands and feet (dactylitis), and splenic sequestration (sudden pooling of blood in the spleen). Complications of adolescents to older individuals include leg ulcers, aseptic necrosis (localized bone tissue death), eye damage, and gall stones.