Psychometric Impact of Priapism on Lives of Adolescents and Adults With Sickle Cell Anemia: A Sequential Independent Mixed-Methods Design.

Despite priapism being one of the most frequent complications of sickle cell anemia (SCA) in male individuals, little has been reported about the impact of priapism in this population. The authors used a sequential independent mixed-methods design, which used both international multicenter focus group discussions (n=35) and a quantitative patient-reported outcome measure (n=131) to determine the impact of priapism on men with SCA in Nigeria and the United States. The authors analyzed data from focus groups using an iterative inductive-deductive approach. Comparison of the Priapism Impact Profile data was done using the Kruskal-Wallis H test. Our result showed that priapism, across cultures, is associated with shame and embarrassment. These emotions interfere with timely clinical and family communication about priapism symptoms and complications. Participants were dissatisfied with the quality of care at emergency facilities. The quality of life and physical wellness of men with SCA-related priapism were significantly different for the 3 groups: (1) priapism condition getting better, (2) priapism condition getting worse, and (3) priapism condition remain the same (P=0.002 and P=0.019, respectively). Psychological, sexual, and physical wellbeing are all adversely affected by priapism. Evidence-based methods are necessary for adequate medical, educational, and psychological treatment for recurrent priapism.

External validation of the priapism impact profile in a Jamaican cohort of patients with sickle cell disease.

BACKGROUND: Priapism impairs quality of life and has a predilection for males with sickle cell disease (SCD). The Priapism Impact Profile (PIP) is a novel 12-item instrument designed to measure general health-related impact of priapism. The aim of the study was to evaluate the validity and reliability of the PIP in a Jamaican cohort of SCD patients experiencing priapism. METHODS: One hundred SCD patients with a history of priapism were recruited from a sickle cell clinic in Kingston, Jamaica and administered the PIP questionnaire. Patients rated each item of the PIP for clarity and importance. Statistical testing was employed to evaluate the psychometric performance of the PIP. Content validation was assessed based on patient descriptive rating of the items based on clarity, and importance and criterion-oriented validity were assessed by evaluating the PIP's ability to distinguish between patient subgroups. Test-retest repeatability was assessed in 20 of the 100 patients. RESULTS: Patients were stratified into active (54) and remission (46) priapism groups based on their experience of priapism within the past year. Patients in the active priapism group were younger (p = 0.011), had a shorter duration of disease (p = 0.023), and had more frequent priapism episodes (p = 0.036) than the remission group. PIP questionnaire scores differed significantly with respect to priapism activity (p < 0.001) and prevalence of erectile dysfunction (p < 0.05) but not by priapism severity (p = 0.62). The PIP questionnaire had good content validity, with questions rated as having medium or high clarity and importance by an average of 82.8% and 69.2% of patients, respectively. CONCLUSION: The PIP questionnaire was successfully validated in a Jamaican cohort of SCD patients and adequately discriminated patients with active priapism from those in remission. The instrument may be utilized in routine clinical management of patients with SCD-associated priapism. Further clinical investigations are warranted in other populations.

Rare disorders of penile erection.

This literature review presents two unusual and mystifying disorders of penile erection: painful nocturnal erections, alternatively termed sleep-related painful erections, and idiopathic stuttering priapism, a variant of recurrent ischemic priapism in which no cause is discernible. The disorders are closely related although they are distinct clinically and pathologically. The main subject areas of discussion are recognition, clinical evaluation and management although current concepts surrounding their causes and mechanisms are also addressed. It is acknowledged that despite the perceived rarities of these disorders they are impactful in terms of their disease profiles and consequences. Future advances in their management will require continued development of evidence-based treatments.

Knowledge, Experiences and Coping Mechanisms for Priapism among Persons with Sickle Cell Disease in Ibadan, Nigeria.

BACKGROUND: Priapism is a prolonged, painful penile erection common among males with Sickle Cell Disease (MWSCD) predisposing to erectile dysfunction (ED) when treatment is delayed. Unlike in women with sickle cell disease (SCD), there has been little attention to male reproductive health complications of SCD. OBJECTIVE: To investigate knowledge, experiences and coping mechanisms for priapism among MWSCD in Ibadan, Nigeria. METHODS: This descriptive cross-sectional study employed purposive sampling technique to select 95 consenting MWSCD attending haematology clinics in Ibadan for interview. A semi-structured, interviewer-administered questionnaire was used to collect information on knowledge, coping mechanisms, and experiences of priapism. Knowledge of priapism was measured and categorised as poor and good respectively. Psychosocial Experiences (PEs) were measured and categorised as mild, moderate and severe, while the Sexual Experiences (SEs) were recorded. Coping mechanisms for priapism were grouped into Medical, Psychosocial and Harmful coping mechanisms respectively. Data were analysed using descriptive statistics and Fishers' Exact test at p<0.05. RESULTS: Respondents' mean age was 23.6±8.8 years. Over half (55.8%) had good knowledge of priapism. Thirty-nine respondents (41.1%) had experienced priapism. Sexual Experiences reported include: total ED 10.3% and apathy for sexual intercourse 23.1%. Majority 30(76.9%) developed mild PEs especially fear of reoccurrence of priapism (56.4%) and sleeplessness (43.6%). The most used Medical Coping Mechanism (MCM) was cold shower (46.2%). There was no significant association between age and knowledge of priapism. CONCLUSION: Knowledge of priapism among respondents was good. Psychosocial therapy through appropriate health education, counseling and social support may help improve knowledge among people with SCD.

Self-care of men with priapism and sickle cell disease.

OBJECTIVE: To identify self-care demands of men with sickle cell disease and priapism and describe self-care measures in light of Orem's Self-Care Theory. METHOD: This is a descriptive exploratory study with qualitative approach conducted with nine men with a history of sickle cell disease and priapism. Data were analyzed using Orem's Self-Care Theory. RESULTS: Some demands were identified: from universal self-care - difficulty in social interaction and solitude, changes in self-image, self-esteem and sexual activity; from development - the experience with priapism and little knowledge about the pathophysiology of the disease; regarding health deviations - pain crises. CONCLUSION: Orem's theory allowed to identify self-care demands, which are essential for the nursing care provided for men with priapism. Nursing has an essential role in the measures for the different demands presented.

Self-care of men with priapism and sickle cell disease / Autocuidado de hombres con priapismo y enfermedad falciforme / Autocuidado de homens com priapismo e doença falciforme

ABSTRACT Objective: To identify self-care demands of men with sickle cell disease and priapism and describe self-care measures in light of Orem's Self-Care Theory. Method: This is a descriptive exploratory study with qualitative approach conducted with nine men with a history of sickle cell disease and priapism. Data were analyzed using Orem's Self-Care Theory. Results: Some demands were identified: from universal self-care - difficulty in social interaction and solitude, changes in self-image, self-esteem and sexual activity; from development - the experience with priapism and little knowledge about the pathophysiology of the disease; regarding health deviations - pain crises. Conclusion: Orem's theory allowed to identify self-care demands, which are essential for the nursing care provided for men with priapism. Nursing has an essential role in the measures for the different demands presented.

RESUMEN Objetivo: Identificar las demandas sobre autocuidado de hombres con enfermedad falciforme y priapismo y describir las medidas del autocuidado a la luz de la Teoría de Orem. Método: Se trata de una investigación descriptiva, exploratoria, de abordaje cualitativo, realizada entre nueve hombres con historia clínica de enfermedad falciforme y priapismo. El análisis de los datos se llevó a cabo según la Teoría del Autocuidado de Orem. Resultados: se identificaron algunas demandas: del autocuidado universal: dificultad de interacción social y soledad, alteraciones de la autoimagen y autoestima y actividad sexual; del desarrollo: la experiencia con el priapismo y el conocimiento insuficiente sobre la fisiopatología de la enfermedad; sobre desviaciones de la salud: crisis de dolor. Conclusión: La teoría de Orem permitió identificar las demandas del autocuidado, esenciales para la atención de enfermería en hombres con priapismo y resaltó la importancia de la enfermería acerca de las medidas a ser tomadas en las diferentes demandas.

RESUMO Objetivo: Identificar as demandas de autocuidado de homens com doença falciforme e priapismo e descrever as medidas de autocuidado à luz da Teoria do Autocuidado de Orem. Método: Trata-se de uma pesquisa descritiva, exploratória, de abordagem qualitativa, com nove homens com história clínica de doença falciforme e priapismo. A análise dos dados foi realizada por meio da Teoria do Autocuidado de Orem. Resultados: Foram identificadas algumas demandas: de autocuidado universal - dificuldade de interação social e solidão, alterações na autoimagem e autoestima e atividade sexual; de desenvolvimento - a experiência com o priapismo o e pouco conhecimento sobre a fisiopatologia da doença; em relação a desvios de saúde - crises de dor. Conclusão: A teoria de Orem possibilitou identificar as demandas de autocuidado, que são essenciais para cuidado de enfermagem a homens com priapismo, e a importância da enfermagem frente às medidas para diferentes demandas apresentadas.

Steroids, drugs and stuttering priapism; the rock-and-roll lifestyle of a 24-year-old man.

The authors present a case of a 24-year-old, poorly controlled insulin-dependent type 1 diabetic Caucasian man who presented to the emergency department, with a painful erection of 36 h duration that had failed to resolve with conservative management. This was the patient's seventh priapism, with his most recent attendance 1 week previously for which he underwent a distal cavernosal shunt. He admitted to taking several recreational drugs, including marijuana and cocaine, during the preceding few days, in addition to the long-term use of the oral anabolic steroid oxandrolone. He had no family history of sickle cell disease or trait. On examination, a tensely erect penis was noted. A diagnosis of stuttering priapism was made and 750 mL of blood subsequently drained via a distal corporoglandular shunt resulting in successful detumescence.

Erectile dysfunction after sickle cell disease-associated recurrent ischemic priapism: profile and risk factors.

INTRODUCTION: Risk factors associated with erectile dysfunction (ED) that results from recurrent ischemic priapism (RIP) in sickle cell disease (SCD) are incompletely defined. AIM: This study aims to determine and compare ED risk factors associated with SCD and non-SCD-related "minor" RIP, defined as having ≥2 episodes of ischemic priapism within the past 6 months, with the majority (>75%) of episodes lasting <5 hours. METHODS: We performed a retrospective study of RIP in SCD and non-SCD patients presenting from June 2004 to March 2014 using the International Index of Erectile Function (IIEF), IIEF-5, and priapism-specific questionnaires. MAIN OUTCOME MEASURES: Prevalence rates and risk factor correlations for ED associated with RIP. RESULTS: The study was comprised of 59 patients (40 SCD [mean age 28.2 ± 8.9 years] and 19 non-SCD [15 idiopathic and four drug-related etiologies] [mean age 32.6 ± 11.7 years]). Nineteen of 40 (47.5%) SCD patients vs. four of 19 (21.1%) non-SCD patients (39% overall) had ED (IIEF <26 or IIEF-5 <22) (P = 0.052). SCD patients had a longer mean time-length with RIP than non-SCD patients (P = 0.004). Thirty of 40 (75%) SCD patients vs. 10 of 19 (52.6%) non-SCD patients (P = 0.14) had "very minor" RIP (episodes regularly lasting ≤2 hours). Twenty-eight of 40 (70%) SCD patients vs. 14 of 19 (73.7%) non-SCD patients had weekly or more frequent episodes (P = 1). Of all patients with very minor RIP, ED was found among 14 of 30 (46.7%) SCD patients vs. none of 10 (0%) non-SCD patients (P = 0.008). Using logistic regression analysis, the odds ratio for developing ED was 4.7 for SCD patients, when controlling for RIP variables (95% confidence interval: 1.1-21.0). CONCLUSIONS: ED is associated with RIP, occurring in nearly 40% of affected individuals overall. SCD patients are more likely to experience ED in the setting of "very minor" RIP episodes and are five times more likely to develop ED compared with non-SCD patients.

Anxiety disorders in patients with idiopathic priapism: risk factor and pathophysiologic link?

INTRODUCTION: A scientific explanation remains elusive for many presentations of recurrent ischemic priapism. AIM: The aim of this article was to evaluate the possible clinical association between idiopathic priapism and anxiety disorders. METHODS: Twenty-one patients without identifiable, presumed etiologies for their priapism disorders presented consecutively to a single practitioner's clinical practice and underwent clinical history evaluation and management over a 2(1/2)-year interval. MAIN OUTCOME MEASURES: The main outcome is the documentation of patient demographics and clinical profiles relating to medical history and priapism presentations. RESULTS: Anxiety disorders including generalized anxiety, attention-deficit hyperactivity disorder, and obsessive-compulsive disorder were self-reported in 10 patients. CONCLUSIONS: The possible association between idiopathic priapism and anxiety disorders lends support for a central neurobiologic pathophysiology for the erectile disorder. Further neuropsychiatric studies of this clinical population is required to confirm the hypothesis.

[Priapism in sickle cell anaemia in Senegal: prevalence, attitudes and knowledge]. / Priapisme chez le drépanocytaire sénégalais: prévalence, attitudes et connaissances.

We interviewed 108 sickle cell anaemia patients aged 5 years older on priapism, and 113 healthy subjects in a control group. They were recruited in the out-patient consultation of the two sickle cell anemia care units of the Fann teaching hospital in Dakar. Ten cases of priapism were identified, all in the group of sickle cell patients (the difference is significant, p = 0.004). Prevalence of priapism was 9.3%. Actuarial probability of having priapism was 8.3% by 10 years of age, and 38.9% +/- 5.7 by 20 years of age. Before the interview, only 10.2% of the sickle cell patients and 8% in the control group knew about priapism, and most of them were unaware of its association with sickle cell disease (75% of sickle cell anaemia patients and 80% of control subjects). This lack of information should be improved by an educational program.

Counseling patients about sexual issues.

In recent years, the frequency of antidepressant drug-induced sexual dysfunction has increased, along with the use of new drugs for the treatment of erectile dysfunction and premature ejaculation. It has thus become common for pharmacists to counsel patients about sexual issues. Pharmacists must not only become knowledgeable about these drugs and their indications, but they must also become skilled and comfortable with counseling patients and answering questions from both patients and other health care providers. In addition to therapeutic information, pharmacists' discussions with patients should take into account factors that contribute to treatment nonadherence and treatment failure. Patient education is essential to ensure optimum outcomes for pharmacologic treatments for both erectile dysfunction and premature ejaculation. Improper use of phosphodiesterase-5 inhibitor drugs for erectile dysfunction accounts for most nonresponsiveness and discontinuation of treatment. Drug-induced sexual dysfunction is common with some psychotropic drugs. Up to 50% of men will experience delayed ejaculation, and at least 30% of men and women will experience anorgasmia from antidepressant drugs with serotonin agonist activity. Trazodone is the drug most commonly associated with the rare but very serious adverse effect of priapism. The pharmacist who is both competent and comfortable discussing sexual function and dysfunction with patients can make positive contributions to their therapeutic outcomes as well as their quality of life.

Idiopathic stuttering priapism treated successfully with low-dose ethinyl estradiol: a single case report.

INTRODUCTION: Chronic priapism represents a challenging therapeutic dilemma. Inadequate or deferred treatment can result in impaired quality of life and permanent erectile dysfunction. The etiology of stuttering priapism is speculated to be initially an ischemic injury that damages the neurologic and/or endothelial-mediated mechanisms that normally regulate detumescence and maintain penile flaccidity. AIM: We report a case of stuttering priapism successfully treated with low-dose ethinyl estradiol. METHODS: A 51-year-old man presented with idiopathic stuttering priapism. Treatment was given in the form of low-dose ethinyl estradiol for six consecutive weeks. RESULTS: Moderate lowering of testosterone level and effective prevention of priapism for at least 6 months with retaining of erectile function. CONCLUSION: The use of low-dose estrogen shows a potential to be an effective and relatively rapid treatment option for some cases of idiopathic stuttering priapism.

A case of unrelenting pursuit of castration.

OBJECTIVE: Explore the limitations of the classification of body dysmorphic disorder. CLINICAL PICTURE: A chinese male relentlessly pursued castration to relieve painful erections which were never substantiated. The testes and corporaspongiosum had been removed. No psychotic symptom was prominent. TREATMENT: Cognitive therapy and psychotropics were tried. OUTCOME: The remaining corpora cavernosa was removed subsequently. CONCLUSION: The distorted somatic perception and secondary cognitive amplification (painful erections) could represent a variant of body dysmorphic disorder involving internal body image; diagnostic classifications should accommodate such a category.