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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 36 of 49
In Progress

Hydroxurea Treatment Recommendations

SCFA_Coach September 25, 2023

Educate all patients with SCA and their family members about hydroxyurea therapy. (See consensus treatment protocol on page 145). (Consensus-Panel Expertise)

In adults with SCA who have three or more sickle cell-associated moderate to severe pain crises in a 12-month period, treat with hydroxyurea. (Strong Recommendation, High-Quality Evidence)

In adults with SCA who have sickle cell-associated pain that interferes with daily activities and quality of life, treat with hydroxyurea. (Strong Recommendation, Moderate-Quality Evidence)

In adults with SCA who have a history of severe and/or recurrent ACS, treat with hydroxyurea.*
(Strong Recommendation, Moderate-Quality Evidence)

In adults with SCA who have severe symptomatic chronic anemia that interferes with daily activities or quality of life, treat with hydroxyurea. (Strong Recommendation, Moderate-Quality Evidence)

In infants 9 months of age and older, children,and adolescents with SCA, offer treatment with hydroxyurea regardless of clinical severity to reduce SCD-related complications (e.g., pain, dactylitis, ACS,anemia).(Strong Recommendation, High-Quality Evidence for ages 9–42 months;

Moderate Recommendation,Moderate-QualityEvidence for children >42 months and adolescents).
Note: The panel intentionally used the term “offer” realizing that patients’ values and preferences may differ particularly considering treatment burden (e.g., laboratory monitoring,office visits),availability of drug in a liquid form, and cost. Therefore, the panel strongly encourages shared decision making and discussion of hydroxyurea therapy with all patients.

In adults and children with SCD who have chronic kidney disease and are taking erythropoietin, hydroxyurea therapy can be added to improve anemia. (Weak Recommendation, Low-Quality Evidence)

In females who are pregnant or breastfeeding,discontinue hydroxyurea therapy.
(Moderate Recommendation, Very Low-Quality Evidence)

To ensure proper use of hydroxyurea and maximize benefits and safety, use an established prescribing and monitoring protocol.(Strong Recommendation, High-Quality Evidence)

In people with HbSW-thalassemia or HbSC who have recurrent sickle cell-associated pain that interferes with daily activities or quality of life,consult a sickle cell expert for consideration of hydroxyurea therapy.
(Moderate Recommendation, Low-Quality Evidence)

In people not demonstrating a clinical response to appropriate doses and duration of hydroxyurea therapy, consult a sickle cell expert. (Moderate Recommendation, Very Low-Quality Evidence)