6123 A case series of stuttering priapism in boys with sickle cell disease; challenges with (under)reporting and observations of treatment responses

Objectives Βy the age of 20, up to 89% of males with SCD will have experienced one or more episodes of priapism.1 Etilephrine is the medication of choice for treatment and prophylaxis of stuttering priapism2; our regional guidelines suggest a treatment duration of 4 weeks and then stop/wean. We aimed to investigate the incidence, clinical presentation and treatment of priapism in our paediatric sickle cell population. Methods Retrospective observational study, utilizing data from our pediatric sickle cell database and electronic patient records Results Out of 74 boys with sickle cell disease in our service, 6 cases reported episodes of priapism within the past 5 years, which equates to an incidence rate of 8%. Clinical and treatment characteristics of all cases are summarised in table 1. All reported cases were classified as stuttering priapism, with no instances of fulminant priapism recorded. None of the episodes required attendance at the Accident and Emergency department or administration of opiate analgesia. Diagnosis was made during consultations with the specialist sickle cell team, either by a consultant or a clinical nurse specialist, in the outpatient or daycare setting. The mean age of onset for priapism was 10.5 years (range 6–16 years), with an average duration of 16.6 minutes per episode. The frequency of episodes varied significantly, ranging from daily occurrences to one-time events. The majority of cases were receiving disease-modifying interventions such as hydroxycarbamide or a blood transfusion program. This higher prevalence of priapism among patients on disease modifying treatments suggests that more regular communication and closer relationships between families and specialist sickle cell teams facilitate the discussion and reporting of this issue. Half of the episodes required pharmacological treatment with etilephrine, administered twice daily, and the mean length of treatment was 4 months. Discontinuation of etilephrine resulted in a relapse of priapism episodes in 67% of cases. No side effects were reported from the use of etilephrine. Conclusion In conclusion, priapism remains an underreported complication in boys with sickle cell disease. Further interventions and quality improvement initiatives should be targeted at increasing awareness both for patients as well as healthcare professionals. Etilephrine is well tolerated and effective in the management stuttering priapism symptoms; however, achieving long-term control may require lengthy or recurrent courses of treatment. References Mantadakis, et al. Prevalence of Priapism in Children and Adolescents With Sickle Cell Anemia. Journal of Pediatric Hematology/Oncology21(6):518–522. Gbadoé AD, et al. Management of sickle cell priapism with etilefrine. Arch Dis Child. 2001 Jul;85(1):52–3. doi: 10.1136/adc.85.1.52. PMID: 11420201.