Clinical Presentation and Diagnosis of Priapism in SCD General

• Mean age of initial episode is 12 years of age

• Most males with SCD will have one episode by age 20

• Repeated episodes can lead to fibrosis and impotence Symptoms

• Patients may complain of painful and unwanted erection lasting anywhere from less than 2 hours (stuttering type) to more than 2 hours (prolonged type)

Signs

• Urinary obstruction Laboratory Tests

• CBC with reticulocyte Other

• Monitor for duration of episode

• Prolonged episodes should be considered medical emergencies

Pseudoephedrine dosed at 30 to 60 mg/day taken at bedtime, has been used to prevent or decrease the number of episodes of priapism.5 Terbutaline 5 mg has been used orally to prevent priapism with mixed results.4 ,42 Leuprolide, a gonadotropin-releas-ing hormone, also has been used for this indication. Hydroxyurea may be helpful in some patients. The use of antiandrogens is under investigation.5,9

Treatment of Acute Complications

Aplastic Crisis

Most patients in aplastic crisis will recover spontaneously and therefore treatment is supportive. If anemia is severe or symptomatic, transfusion may be indicated. Infection with human parvovirus B19 is the most common cause of aplastic crisis. Isolate infected patients, because parvovirus is highly contagious. Pregnant individuals should avoid contact with infected patients because midtrimester infection with parvovirus may cause hydrops fetalis and still birth.5,9

Sequestration Crisis

RBC sequestration in the spleen in young children may lead to a rapid drop in hematocrit, resulting in hypovolemia, shock, and death. Treatment is RBC transfusion to correct the hypovolemia, as well as broad-spectrum antibiotics because infections may precipitate the crisis.5,9

Recurrent episodes are common and can be managed with chronic transfusion and splenectomy. Observation is used commonly in adults because their episodes are milder. Splenectomy is usually delayed until after 2 years of age to lessen the risk of postsplenectomy septicemia. Patients with chronic hypersplenism should be considered for splenectomy.5,43

Blood Pressure Health

Blood Pressure Health

Your heart pumps blood throughout your body using a network of tubing called arteries and capillaries which return the blood back to your heart via your veins. Blood pressure is the force of the blood pushing against the walls of your arteries as your heart beats.Learn more...

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