Pain in sickle cell disease: rates and risk factors

Article Abstract:

Sickle cell disease is a genetic disorder that causes anemia and is characterized by the formation of abnormal, sickle- or crescent-shaped red blood cells. These sickle cells have an abnormal form of hemoglobin (the oxygen-carrying pigment of the blood) which is referred to as hemoglobin S. Anemia results when the hemoglobin does not provide adequate oxygen. Sickle cell disease is prevalent among Mediterranean and African populations and American blacks. Patients with sickle cell disease may suffer periodic attacks of pain in skeletal muscles, which are referred to as ''crises''. Sickle cell crises are largely responsible for disability in patients with this disease. The number of episodes of pain, the risk factors associated with crises, and the relation between crises and early death were assessed in 3,578 patients with various forms of sickle cell disease. The ages of the patients ranged from newborn to 66 years old. The rate of pain, in number of episodes per patient-years, was 0.8 in patients with sickle cell anemia, 1.0 in patients with beta-0-thalassemia, and 0.4 in patients with hemoglobin sickle cell disease and beta(+)-thalassemia. Rates of pain varied widely within groups, with 39 percent of patients with sickle cell anemia experiencing no pain and one percent suffering more than six attacks each year. Results showed that 32.9 percent of all episodes of pain occurred in 5.2 percent of patients who had 3 to 10 crises each year. Factors associated with early death in patients aged 20 years were a high number of pain attacks, a high hematocrit (a measure of the red blood cell volume), and low levels of hemoglobin in the fetus. There was no relation between pain and alpha-thalassemia, which was associated with increased hematocrit. These findings show that an increased number of pain episodes in patients with sickle cell disease is associated with early death. Factors or methods that improve fetal hemoglobin levels, such as hydroxyurea, may help to reduce pain attacks and improve survival. (Consumer Summary produced by Reliance Medical Information, Inc.)

Evaluation, Symptomatology, Signs and symptoms

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Prediction of adverse outcomes in children with sickle cell disease

Article Abstract:

Three complications of sickle cell anemia may predict serious complications later in life if they occur in the first two years of life. Researchers followed 392 babies diagnosed with sickle cell anemia until they were 10 years old. Children who subsequently died or had a stroke, frequent pain of acute chest syndrome were more likely to have had pain and tenderness in the hands or feet, a hemoglobin level less than 7, and elevated white blood cell counts in the absence of infection. These signs and symptoms all occurred in the first two years of life.

Sickle cell anemia in children, Pediatric diseases

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The acute chest syndrome of sickle cell disease

Article Abstract:

Researchers continue to find better ways to treat acute chest syndrome in patients with sickle cell disease. This syndrome occurs when the sickled red blood cells damage the lungs. A study published in 2000 found that infection was involved in many of these cases. For this reason, antibiotics may be useful in the treatment of acute chest syndrome. Bronchodilator drugs and blood transfusions were also useful. The drug hydroxyurea is also effective in preventing acute chest syndrome and other complications of sickle cell disease.

Care and treatment, Editorial, Lung diseases

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