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

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.

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:

1. Patient or parent/guardian declines participation.

2. Female.

3. Subject or parent/guardian unable to communicate adequately with the interviewer.