Priapism in Boys and Men With Sickle Cell Disease - Demographics, Characteristics and Prevalence

Study Overview
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Brief Summary
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Priapism, a prolonged erection of the penis, is a medical issue that often affects men with sickle cell disease. The purpose of this study is to collect demographic and clinical information on priapism by interviewing men with sickle cell disease.

Detailed Description
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Priapism is a prolonged, painful erection of the penis that lasts for more than four hours and occurs without sexual stimulation. It occurs when blood in the penis becomes trapped and is unable to drain properly. If it is not treated immediately, it can lead to scarring and permanent erectile dysfunction. Many cases of priapism are the result of sickle cell disease; approximately 42% of all adults with sickle cell disease will eventually develop priapism. Current treatments include medication, ice packs, or surgery. More research is needed to better understand the demographic and clinical characteristics of priapism. The purpose of this study is to collect information and further characterize priapism by conducting interviews with men with sickle cell disease. In turn, these findings may guide future priapism clinical trials. This study will consist of two standardized questionnaires that will be administered to 1,650 men with sickle cell disease. Participants will complete an initial five-item questionnaire about priapism. If a participant indicates past experience with priapism on this initial questionnaire, he will be asked to complete a second questionnaire. This questionnaire will ask in-depth questions to further characterize the participant's episodes of priapism. If health issues such as drug use, harmful sexual behaviors, or impotence are identified upon reviewing the questionnaire, clinic staff will suggest care options and provide appropriate referrals to the participants. All participants will receive an educational brochure about priapism and compensation for completing the questionnaires.

Official Title
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The Epidemiology of Priapism (Sickle Cell Disease)

Conditions
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Priapism, Anemia, Sickle Cell

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Diagnosis of sickle cell anemia or sickle beta zero thalassemia, if 5 years of age or over OR Diagnosis of sickle beta plus thalassemia or sickle hemoglobin C disease, if 15 years of age or over Participant and/or parent or guardian must be able to communicate adequately with the interviewer May participate if currently taking hydroxyurea, undergoing chronic transfusion, or participating in other research studies, including those involving treatments such as arginine, if all other inclusion criteria are met Exclusion Criteria Subjects who meet any of the following criteria are disqualified from enrollment in the study: Patient or parent/guardian declines participation. Female. Subject or parent/guardian unable to communicate adequately with the interviewer.

Ages Eligible for Study
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Minimum Age: 5 Years

Sexes Eligible for Study
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Male

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| 1<br>200 subjects ages 5 to 9.9 years with a diagnosis of HbSS/HbSβ0 | |
| 2<br>400 subjects ages 10 to 14.9 years with a diagnosis of HbSS/HbSβ0 | |
| 3<br>400 subjects ages 15 to 24.9 years with a diagnosis of HbSS/HbSβ0 | |
| 4<br>400 subjects over the age of 25 with a diagnosis of HbSS/HbSβ0 | |
| 5<br>250 subjects age 15 and older with a diagnosis of HbSC or HbSβ+ | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Enumeration of the Prevalence of Priapism in Males With Sickle Cell Anemia and Sickle Beta Zero Thalassemia. | Subject responded YES to survey Question Have you ever had priapism?. By diagnosis and age group. Enumeration of the prevalence of priapism in males with sickle cell anemia and sickle beta zero thalassemia. | At time of interview | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| 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 | 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. | Cross-sectional single survey visit | 
| Descriptive Comparison of the Prevalence of Priapism in Males With Sickle Cell Anemia to That Described in Older Patients With Other Sickle Hemoglobinopathies | Descriptive comparison of the prevalence of priapism in males with sickle cell anemia to that described in older patients with other sickle hemoglobinopathies. | Cross-sectional single survey visit | 
| Assessment of General Patient and Parent Understanding of Priapism as a Complication of Sickle Cell Disease Gained From Completion of Protocol | Assessment of general patient and parent understanding of priapism as a complication of sickle cell disease gained from completion of protocol. | Cross-sectional single survey visit | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Sickle Cell Anemia