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Evidenced Based Management: A Journey for Physicians

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  1. Historical Perspective, Epidemiology, and Methodology
  2. Overview of the SCD guidelines and chapters
  3. Process and methodology
  4. Consensus Statements
  5. Clinical Practice Guidelines and the institute of Medicine
  6. Prevention of invasive infection
  7. Screening for Renal Disease
  8. Electrocardiogram Screening
  9. Screening for hypertension
  10. Screening for Retinopathy
  11. Screening for risk of stroke using neuroimaging
  12. Screening for Pulmonary disease
  13. Reproductive counseling
  14. Contraception
  15. Clinical Preventive services
  16. Immunizations
  17. Vaso-Occlusive Crisis
  18. Fever
  19. Acute Renal Failure
  20. Priapism
  21. Hepatobiliary Complications
  22. Acute Anemia
  23. Splenic Sequestration
  24. Acute Chest Syndrome
  25. Acute Stroke
  26. Multisystem Organ Failure
  27. Acute Ocular Conditions
  28. Chronic pain
  29. Avascular Necrosis
  30. Leg Ulcers
  31. Pulmonary Hypertension
  32. Renal Complications
  33. Stuttering/Recurrent Priapism
  34. Ophthalmologic Complications
  35. Summary of the Evidence
  36. Hydroxurea Treatment Recommendations
  37. Consensus Treatment Protocol and Technical remarks for the implementation of Hydroxyurea Therapy
  38. Indications for transfusions
  39. Recommendations for Acute and Chronic Transfusion Therapy
  40. Appropriate Management/ Monitoring
  41. Consensus Protocol for Monitoring Individuals on Chronic Transfusion Therapy
  42. Complications of Transfusions
  43. Recommendations for the Management and Prevention of Transfusion Complications
  44. New Research is Needed
  45. Data Systems That Meet the Highest Standards of Scientific Rigor Can Be Invaluable
  46. Improved Phenotyping is needed
  47. Broad collaborations for Research and Care
  48. Beyond Efficacy
  49. Look, Listen, Empathize and Ask
Lesson 20 of 49
In Progress


SCFA_Coach September 25, 2023

Priapism is a sustained, unwanted painful erection lasting 4 or more hours. Stuttering priapism is the occurrence of multiple self-limited episodes of shorter duration (<4 hours) and can be a harbinger of sustained events. Priapism is a common complication of SCD, affecting 35 percent of boys and men. It is usually of the low­ flow ischemic type and characterized by pain and a soft glans. Blood aspirated from the corpora cavernosa of the penis is dark, with a low pO2, pH, and glucose concentration. Prompt recognition of priapism and initiation of conservative medical management may lead to detumescence and limit the need for more aggressive and invasive intervention. Delayed diagnosis and therapy can result in impotence. Summary of the Evidence Seven observational studies and 39 case reports described priapism in the setting of SCD.

Overall, the quality of the evidence in this area was low due to the observational and uncontrolled design of the available studies. The observational studies included more than 220 people and studied approaches such as shunts, aspiration, exchange transfusion, hydroxyurea, hormonal therapy (e.g., stilbestrol, finasteride, and leuprolide), bicalutamide, hydralazine, sildenafil, oxygen, and hyperhydration to treat priapism in men and boys with SCD. Results were limited, reporting variable success. Several of the studies highlighted the importance of prompt recognition and initial conservative medical management with analgesics, intravenous fluids, oxygen, and sedation if needed. Red blood cell transfusion therapy was inconsistently associated with improvement in acute priapism. In addition, case reports of acute neurological events following exchange transfusion for priapism further limit enthusiasm for routine adoption of this therapy in the absence of proven benefit. Both observational studies and case reports found that a variety of subsequent interventions used to treat symptoms that persist after initial conservative medical management appear to result in detumescence and retained potency. These include penile aspiration, corporal irrigation using a-adrenergic agents (e.g., pseudoephedrine, epinephrine, etilefrine), and the use of oral agents (e.g., PDE-5 inhibitors, pseudo-ephedrine). Surgical intervention, including shunting, has been utilized most often after more conservative measures fail, with inconsistent benefit. In developing recommendations for the care of males with SCD presenting with acute priapism, the expert panel placed great value on preventing pain and future long-term sequelae.

Recommendations For an episode of priapism lasting 4 hours or longer, initiate interventions to include vigorous oral or intravenous hydration and oral or intravenous analgesia (Strong Recommendation, Low-Quality Evidence); and consultation with an urologist who can perform further evaluation and intervention for symptoms which do not remit with initial conservative medical management. (Consensus-Panel Expertise) Do not use transfusion therapy for immediate treatment of priapism associated with SCD. (Moderate Recommendation, Low-Quality Evidence) Consult with a hematologist for possible preoperative transfusion if surgical intervention is required. (Consensus-Panel Expertise)

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