Treatment dilemmas: strategies for priapism, chronic leg ulcer disease, and pulmonary hypertension in sickle cell disease

Abstract

Sickle cell disease is a disorder characterized by chronic hemolytic anemia and multiorgan disease complications. Although vaso-occlusive episodes, acute chest syndrome, and neurovascular disease frequently result in complication and have well-documented guidelines for management, the management of chronic hemolytic and vascular-related complications, such as priapism, leg ulcers, and pulmonary hypertension, is not as well recognized despite their increasing reported prevalence and association with morbidity and mortality. This chapter therefore reviews the current updates on diagnosis and management of priapism, leg ulcers, and pulmonary hypertension.

Trial registration: ClinicalTrials.gov NCT02633397.

Conflict of interest statement

Roberta C.G. Azbell: no conflicts to disclose.

Payal Chandarana Desai: consultant for GBT for grant review; advisory board for Forma; funding from the National Institutes of Health, University of Tennessee, and University of Pittsburgh; and speaker for Novartis.

Copyright © 2021 by The American Society of Hematology.

Figures

Graphical abstract

Figure 1.

How I treat priapism. For episodes lasting greater than 4 hours, urgent urologic consultation for prompt aspiration +/- sympathomimetic injection is recommended. For stuttering episodes, it is reasonable to attempt increased hydration and pain management. Long-term oral sympathomimetics may be beneficial in the prevention of recurrent episodes, as well as therapy with hydroxyurea (HU) or chronic transfusions. Hu, hydroxyurea; IV, intravenous.

Figure 2.

Multimodal approach to SCLUs. The treatment of leg ulcers requires comprehensive wound care, adequate analgesics, and preventative measures. NSAID, nonsteroidal anti-inflammatory drug.

Figure 3.

How I approach PH screening. Every patient with SCD should be screened for “red-flag” signs and symptoms of PH at routine visits (when there is no acute illness present). Any red-flag sign/symptom, or the presence of certain comorbidities or disease-specific complications, should be further evaluated with a diagnostic echocardiogram. A moderately elevated TRV, or a mild elevation with other prognostic factors, should warrant further referral to a specialist. IV, intravenous; 6MWD, 6-minute walk distance.