What should a male patient with a history of sickle cell disease do if he experiences an erection lasting more than four hours?

Immediate Emergency Management for Priapism in Sickle Cell Disease

A male patient with sickle cell disease experiencing an erection lasting more than 4 hours must seek immediate emergency medical attention, as this represents ischemic priapism—a urological emergency requiring urgent intervention within 4-6 hours to prevent permanent erectile dysfunction. 1, 2

Why This Is a Medical Emergency

• Ischemic priapism lasting >4 hours causes progressive, irreversible tissue damage with smooth muscle edema and atrophy beginning as early as 6 hours after onset 1
• The risk of permanent erectile dysfunction increases dramatically with duration: reasonable preservation chance if treated <24 hours, significantly increased risk at 24-36 hours, and permanent dysfunction highly likely after 36 hours 1, 2
• Untreated ischemic priapism results in corporal fibrosis, penile shortening, and complete loss of erectile function 1

Immediate Actions Required

Patient Should:

• Go directly to the emergency department—do not wait to see if the erection resolves on its own 1
• Do not attempt home remedies or delay care due to embarrassment, as every hour increases permanent damage risk 1
• Inform providers immediately about sickle cell disease history, as this is a known risk factor but does not change the urgent urologic management approach 2, 3

Emergency Department Will:

• Perform corporal blood gas analysis to confirm ischemic priapism (PO₂ <30 mmHg, PCO₂ >60 mmHg, pH <7.25) 2
• Physical examination will reveal completely rigid, tender corpora cavernosa with severe pain, while the glans and corpus spongiosum remain soft 1, 2
• Immediately initiate intracavernosal phenylephrine injection (100-500 mcg/mL, maximum 1000 mcg in first hour) combined with corporal aspiration as first-line treatment, with success rates of 43-81% 2, 4

Critical Management Principles for Sickle Cell Patients

• Standard urologic intervention takes absolute priority over hematologic interventions 2
• The same emergency priapism protocol applies regardless of sickle cell disease—phenylephrine injection and aspiration remain first-line treatment 2, 3
• Exchange transfusion should NOT be used as primary treatment for acute ischemic priapism in sickle cell patients 2
• Standard sickle cell supportive care (hydration, analgesia) should occur concurrent with—not instead of—urologic intervention 1, 2

Common Pitfalls to Avoid

• Never delay seeking care beyond 4 hours hoping for spontaneous resolution—the natural history without treatment is permanent erectile dysfunction 1
• Do not prioritize hematologic management over immediate urologic decompression in sickle cell patients 2
• Delaying corporal blood gas analysis leads to delayed diagnosis and inappropriate treatment 1, 5

Prognosis and Counseling

• Prompt treatment within the first 24 hours offers the best chance of preserving future erectile function 1
• Patients should understand that likelihood of erectile dysfunction recovery is directly related to duration before treatment 1, 2
• After 36 hours, minimal chance of recovery exists even with aggressive surgical intervention 1, 2

Prevention of Future Episodes

• For patients with recurrent stuttering priapism (common in sickle cell disease), preventative strategies include PDE5 inhibitors (tadalafil or sildenafil), hydroxyurea, or home self-injection of phenylephrine 2, 3
• All sickle cell patients should be educated about the 4-hour threshold and instructed to seek immediate care for any erection exceeding this duration 1, 3