Priapism in Homozygous Sickle Cell Disease: A 40 year Study of the Natural History

Abstract

INTRODUCTIONPriapism, an involuntary and usually painful penile erection, is a major problem in patients with homozygous sickle cell (SS) disease, and is most common in adolescence and early adult life. There are two principal patterns, stuttering episodes and major attacks. Stuttering episodes are predominantly nocturnal, last three to four hours, are relieved by simple physical procedures such as cold showers or exercise, and patients maintain normal erectile function. Major attacks last more than six hours and sometimes for several days, are often excruciatingly painful, and tend to be followed by permanent damage to the vascular erectile system and impotence. Stuttering episodes are inconvenient since they interfere with sleep and lead to daytime sleepiness but, more seriously, may be a prodrome for major attacks. These events are under-reported partly because of embarrassment but also because patients do not realize that they are caused by sickle cell disease. It is therefore good practice in the management of sickle cell disease to question patients directly about these events, and routine questionnaires found these events in 40% of post-pubertal males attending sickle cell clinics (1, 2). A large bibliography has accumulated on priapism in sickle cell disease but most are case reports or small series focussing on risk factors and treatment of priapism. Almost all of this experience has been derived from symptomatically acquired patients with many inherent biases. In a representative sample of patients followed from birth, there is little information on the prevalence of stuttering or major attacks, the relationships between these events and their final outcome in terms of erectile function. This information has now been provided in the Jamaican Cohort Study where, following newborn diagnosis, patients have been systematically reviewed for the last 33−40 years.

Prevalence of priapism in individuals with sickle cell disease and implications on male sexual function

“…(6) These episodes are usually underestimated, not only because patients do not seek care out of embarrassment, but also as many of them do not see it is as a result of SCD. (16) In this study, the prevalence of priapism in men with SCD was similar to those found in the literature. (10,16,17) The genetic type HbSS was related to episodes of priapism, which is also in accordance with other series.…”

“…(16) In this study, the prevalence of priapism in men with SCD was similar to those found in the literature. (10,16,17) The genetic type HbSS was related to episodes of priapism, which is also in accordance with other series. (10,18) Priapism initially manifests itself as minor episodes or PE in childhood and adolescence.…”

“…Severe episodes have less favorable and less predictable outcome. (16) Episodes of priapism often lead to erectile tissue necrosis and subsequent fibrosis by fibroblast proliferation. Erectile dysfunction is the most common negative consequence in these populations, since irreversible damage to erectile tissue occurs, with documented rates of up to 90% for priapism lasting longer than 24 hours.…”

Evolving locally appropriate models of care for indian sickle cell disease

“…Painful involuntary erection of the penis is a common problem among African patients especially in adolescence and early adult life. The prevalence is often underestimated because of embarrassment and the lack of realization that the complication is due to sickle cell disease but direct questioning in adults indicates frequencies of 38-42 per cent 61 62 , and in the Jamaican Cohort where direct questioning was routine, the cumulative incidence rose to nearly 60 per cent in those surviving to the age of 40 yr 63 (Serjeant & Hambleton - unpublished data). In Indian patients, this complication may occur 64 but appears to be rare.…”

Access of men with sickle cell disease and priapism in emergency services

“…SCD interferes in man's life, delays sexual maturation, compromises physical development and causes limitations in several levels due to the clinical variability of this disease 4 . Priapism is among the SCD complications and retrospective data points out that it affects approximately 30% of men with SCD 5 . SCD causes ischemic priapism, with time-dependent hypoxia, hypercapnia, and acidosis.…”