Year: 2024
Type: article
Study design: Case Report
Abstract: Introduction Patients with sickle cell anemia are prone to developing stuttering priapism. Contributing factors include red blood cell deformation, vascular occlusion, nitric oxide dysregulation, endothelial dysfunction, and hypoxia. Some studies have indicated that oxyhemoglobin desaturation occurs at a higher rate during sleep and may lead to priapism episodes in patients with an underlying risk factor. Objective To assess the role of continuous positive airway pressure (CPAP) and improvement in obstructive sleep apnea symptoms in reducing night-time priapism episodes in a patient with sickle cell anemia. Methods A 43-year-old man who was a known case of sickle cell anemia presented to us with a 3-year history of recurrent episodes of stuttering priapism. Interestingly, these episodes occurred exclusively at night during his sleep which caused major disruption to the normal sleep pattern. The patient was initially commenced on hydroxyurea and pseudoephedrine which did not result in definitive improvement. Additional therapy such as adding terbutaline or increasing the dosage of hydroxyurea dosage by his hematology team was also inconclusive in reducing the burden of priapism. Other treatment options such as daily tadalafil was also trialed in this patient but did not show any improvement. At this stage, we decided to start the patient on bicalutamide which resulted in partial resolution of symptoms. A thorough review of the patient’s history revealed that he was a loud snorer and had not sought any treatment for this condition. We referred the patient for sleep evaluation and determined that he was suffering from concurrent obstructive sleep apnea. The patient was started on CPAP therapy and short-term follow-up at 3 months showed a resolution of priapism episodes following the addition of this treatment modality. Results Despite positive findings in our case, it cannot be fully determined whether or not the CPAP therapy was a definitive solution to relieving the priapism episodes. This improvement however warrants a closer look at the concurrent occurrence of obstructive sleep apnea and stuttering priapism in the setting of sickle cell anemia. Healthcare providers may inform sickle cell anemia patients regarding established obstructive sleep apnea screening protocols such as the STOP-BANG questionnaire. Conclusions The role of obstructive sleep apnea in the setting of sickle cell anemia-induced priapism is rather understudied. Further long-term trials are required to determine the definitive role of CPAP therapy in the setting of sickle cell anemia-induced stuttering priapism and obstructive sleep apnea. Disclosure No.
Source: The Journal of Sexual Medicine
Institution: University of Cincinnati
Language: English
Subfield: Physiology
Field: Medicine
Domain: Health Sciences
Open Access status: bronze
OpenAlex ID: W4405221880