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Overview
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Priapism, a prolonged erection of the penis that may lead to impotence, is a specific type of painful crisis that commonly occurs in patients with sickle cell disease. The current treatment of priapism can be improved by comparing therapeutic strategies in prospective clinical trials, but first an assessment of the natural history of priapism is needed. This project will conduct an interview of 1,650 males with sickle cell disease in order to determine in which patients, how often, and under what circumstances priapism occurs.
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Focus Areas
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1. Enumerate the cross-sectional prevalence, demographics, and common clinical characteristics of priapism in males with sickle cell disease among the CSCC patient population.
2. Identify patients eligible for randomized clinical trials of promising secondary prevention strategies.
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Outcomes
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1. Enumeration of the prevalence of priapism in males with sickle cell anemia and sickle beta zero thalassemia.
2. Characterization of priapism in males with sickle cell anemia with reference to time of onset, duration of events, frequency of episodes, precipitating or associated activities, treatment modalities used, and outcome of treatments.
3. Descriptive comparison of the prevalence of priapism in males with sickle cell anemia to that described in older patients with other sickle hemoglobinopathies.
4. Assessment of general patient and parent understanding of priapism as a complication of sickle cell disease gained from completion of protocol.
5. Enumeration of patients available for clinical trials of secondary prevention.
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