Editing Priapism (section)

===== Sickle Cell Disease and other Hematologic Disorders =====
* '''<span style="color:#ff0000">The best intervention is to relieve episodes with prompt intracavernosal phenylephrine and corporal aspiration, with or without irrigation, as in other acute ischemic priapism patients, before proceeding to systemic therapies specific to the underlying disorder</span>[https://pubmed.ncbi.nlm.nih.gov/35536142/ ★]'''
*'''Standard sickle cell assessment and interventions should be considered concurrent with initiation of urologic intervention. Specifically, disease specific systemic care should address:'''
** '''Hydration with IV fluid only if made NPO (maintenance rate) or dehydrated (replace deficit plus maintenance rate)'''
*** '''Hyperhydration is not indicated and may predispose to acute chest syndrome.'''
** '''Supplemental oxygenation only if hypoxic.'''
** '''Pain management with oral or parenteral opioids as per usual painful events (remembering that some patients with SCD may be tolerant to analgesia because of those prior experiences).'''
** '''Hematologic status comparison of CBC and reticulocyte count to baseline values'''
*** '''Best done in consultation with the patient’s hematologist.''' 
*** '''Transfusion is not indicated if hemoglobin is near usual value, and over-transfusion may be associated with neurologic events.'''
*** '''Acute exchange transfusion is not indicated.'''
*** If operative shunting procedures are required, consideration should be given to a simple transfusion of packed red blood cells to raise the hemoglobin to 9-10 g/dl prior to general anesthesia
*** Rarely are blood products required before an aspiration and irrigation procedure, the one exception may be with a very low platelet count (<20,000/uL).
** '''Presence of other acute sickle cell events: neurologic disorders including acute stroke, acute chest syndrome, biliary colic, renal insufficiency which while not associated with a higher frequency of priapism may present at the same time.'''
* '''Ice packs and other cold compresses should never be used in SCD patients as they may worsen painful events by precipitating intravascular sickling.'''
*Most patients with SCD experience recurrent short ischemic priapism events, (lasting <4 hours and commonly referred to as stuttering priapism) but acute episodes and particularly recurrent acute episodes occur commonly enough (both before and after shorter, stuttering events) that education about when to seek urologic attention is a critical part of the patient education in SCD disorders.
*Drugs that have been tried to prevent subsequent priapism episodes
**Etilefrine
**Ephedrine
**Pseudoephedrine
**Terbutaline
**PDE5is (e.g., sildenafil, tadalafil)
**5 alpha reductase inhibitors (dutasteride or finaseride)
**Anti-androgens (cyproterone, bicalutamide, leuprolide, stilboesterol)
**Ketoconazole/prednisone
**Hydroxyurea
*Ongoing chronic (monthly) exchange transfusions do appear to be associated with a reduction in acute and stuttering priapism episodes.
**Exchange transfusion should not be as the primary treatment in patients with acute ischemic priapism associated with sickle cell disease.
**For prolonged acute priapism events that cannot be relieved with intracavernosal phenylephrine and corporal aspiration, exchange transfusion can be considered
*