Anemia, Sickle Cell, Priapism
Conditions
Keywords
Sickle Cell Anemia
Brief summary
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
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.
Interventions
None listed
Sponsors
National Heart, Lung, and Blood Institute (NHLBI)
University of Texas Southwestern Medical Center
Study design
Observational model
COHORT
Time perspective
RETROSPECTIVE
Eligibility
Sex/Gender
MALE
Age
5 Years to No maximum
Healthy volunteers
No
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.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Enumeration of the Prevalence of Priapism in Males With Sickle Cell Anemia and Sickle Beta Zero Thalassemia. | At time of interview | 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. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| 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 | 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. |
| 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 | Descriptive comparison of the prevalence of priapism in males with sickle cell anemia to that described in older patients with other sickle hemoglobinopathies. |
| 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 | Assessment of general patient and parent understanding of priapism as a complication of sickle cell disease gained from completion of protocol. |
Countries
United States
Participant flow
Participants by arm
| Arm | Count |
|---|---|
| Age 5-9.9 HbSS/HbSβ0 Subjects 5 to 9.9 yrs w/SCD diagnosis HbSS/HbSβ0 | 208 |
| Age 10-14.9 HbSS/HbSβ0 Subjects 10 to 14.9 ys w/SCD diagnosis HbSS/HbSβ0 | 240 |
| Age 15-24.9 HbSS/HbSβ0 Subjects 15 to 24.9 yrs w/SCD diagnosis HbSS/HbSβ0 | 398 |
| Age >25 HbSS/HbSβ0 Subjects \> 25 yrs w/SCD diagnosis HbSS/HbSβ0 | 362 |
| Age >15 HbSC/HbSβ+ Subjects \> 15 yrs w/SCD diagnosis HbSC/HbSβ+ | 251 |
| Total | 1,459 |
Baseline characteristics
| Characteristic | Age >25 HbSS/HbSβ0 | Age >15 HbSC/HbSβ+ | Total | Age 10-14.9 HbSS/HbSβ0 | Age 5-9.9 HbSS/HbSβ0 | Age 15-24.9 HbSS/HbSβ0 |
|---|---|---|---|---|---|---|
| Age, Categorical <=18 years | 0 Participants | 84 Participants | 735 Participants | 240 Participants | 208 Participants | 203 Participants |
| Age, Categorical >=65 years | 1 Participants | 2 Participants | 3 Participants | 0 Participants | 0 Participants | 0 Participants |
| Age, Categorical Between 18 and 65 years | 361 Participants | 165 Participants | 721 Participants | 0 Participants | 0 Participants | 195 Participants |
| Age, Continuous | 36.0 years STANDARD_DEVIATION 8.93 | 29.8 years STANDARD_DEVIATION 13.69 | 22.5 years STANDARD_DEVIATION 12.65 | 12.6 years STANDARD_DEVIATION 1.46 | 7.4 years STANDARD_DEVIATION 1.44 | 19.3 years STANDARD_DEVIATION 2.72 |
| Region of Enrollment United States | 362 participants | 251 participants | 1459.0 participants | 240 participants | 208 participants | 398 participants |
| Sex: Female, Male Female | 0 Participants | 0 Participants | 0 Participants | 0 Participants | 0 Participants | 0 Participants |
| Sex: Female, Male Male | 362 Participants | 251 Participants | 1459 Participants | 240 Participants | 208 Participants | 398 Participants |
Adverse events
| Event type | EG000 affected / at risk | EG001 affected / at risk | EG002 affected / at risk | EG003 affected / at risk | EG004 affected / at risk |
|---|---|---|---|---|---|
| deaths Total, all-cause mortality | — / — | — / — | — / — | — / — | — / — |
| other Total, other adverse events | 0 / 208 | 0 / 240 | 0 / 398 | 0 / 362 | 0 / 251 |
| serious Total, serious adverse events | 0 / 208 | 0 / 240 | 0 / 398 | 0 / 362 | 0 / 251 |
Outcome results
Primary
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.
Time frame: At time of interview
Population: All particpants who completed survey were analyzed.
| Arm | Measure | Value (NUMBER) |
|---|---|---|
| Age 5-9.9 HbSS/HbSβ0 | Enumeration of the Prevalence of Priapism in Males With Sickle Cell Anemia and Sickle Beta Zero Thalassemia. | 18 participants |
| Age 10-14.9 HbSS/HbSβ0 | Enumeration of the Prevalence of Priapism in Males With Sickle Cell Anemia and Sickle Beta Zero Thalassemia. | 58 participants |
| Age 15-24.9 HbSS/HbSβ0 | Enumeration of the Prevalence of Priapism in Males With Sickle Cell Anemia and Sickle Beta Zero Thalassemia. | 160 participants |
| Age >25 HbSS/HbSβ0 | Enumeration of the Prevalence of Priapism in Males With Sickle Cell Anemia and Sickle Beta Zero Thalassemia. | 189 participants |
| Age >15 HbSC/HbSβ+ | Enumeration of the Prevalence of Priapism in Males With Sickle Cell Anemia and Sickle Beta Zero Thalassemia. | 60 participants |
Comparison: This was a survey designed to estimate prevalence, no formal comparisons between age or genotype groups were performed.95% CI: [32.5, 37.9]
Secondary
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.
Time frame: Cross-sectional single survey visit
Secondary
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.
Time frame: Cross-sectional single survey visit
Secondary
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.
Time frame: Cross-sectional single survey visit