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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 2 of 49
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Overview of the SCD guidelines and chapters

SCFA_Coach September 25, 2023

This report begins with a chapter on comprehensive health maintenance. Many children and adults with chronic diseases such as SCD do not receive the recommended preventive care provided to other children and adults.

Therefore, the guidelines summarize recommendations for health maintenance screening, testing, and immunizations as they apply to infants, children, adolescents, and adults with SCD. Generally speaking, recommendations for screening to facilitate primary and secondary prevention (e.g., asking a teen about smoking behavior or an adult woman about mammography, respectively) are often confused with recommendations for evaluating early symptoms of a disease or condition. For this and most other documents such as the recommendations of the U.S. Preventive Services Task Force (USPSTF), screening is considered testing or evaluation for a relatively common condition for which there is effective therapy prior to symptom recognition or during an asymptomatic phase. Generalized or universal screening is not recommended when existing therapies have not been shown to improve patient outcomes when implemented in this early presymptomatic phase. Information on screening and preventive care is important for all clinicians who work with individuals with SCD, including specialists who may serve as the continuity health care source for them.

Acute complications are common at all ages in individuals with SCD and are addressed in the chapter, Managing Acute Complications of Sickle Cell Disease. Recurrent acute pain crises (also known as vaso- occlusive crises) are the most common manifestation of SCD. These crises occur, usually without warning, when obstructed blood flow results in ischemic tissue injury and pain. The vascular occlusion, generally at the level of capillaries and post-capillary venules, results not only from an accumulation of adherent and sickled erythrocytes, but also from alterations involving the vascular endothelium and adhesive proteins in the plasma and on white blood cells and platelets. The management of acute pain is central to the care of individuals with SCD, yet pain is often poorly or inadequately addressed in all types of health care settings.

These guidelines include recommendations for rapid and effective pain management in people with SCD who present with such pain crises. Other significant acute complications addressed in this chapter include acute chest syndrome (ACS), stroke, splenic sequestration, acute renal failure, and cholecystitis. Neuropsychological, educational, and vocational impairment as well as common mental health issues such as depression and anxiety, which often accompany chronic illness, were considered beyond the scope of this guideline work.

Chronic complications of SCD may occur as a result of acute episodes or as chronic or recurrent events. Several of the most common of the chronic complications—including chronic pain, cholelithiasis, renal dysfunction, pulmonary hypertension, and retinal problems—are addressed in the fourth chapter, Managing Chronic Complications of Sickle Cell Disease.”

Each of the two major therapies used in individuals with SC hydroxyurea and chronic blood transfusions— are described in separate chapters (see “Hydroxyurea Therapy in the Management of Sickle Cell Disease, and Blood Transfusion in the Management of Sickle C’ell Disease). These are the only currently proven disease- modifying treatments for people with SCD. Both therapies are used in primary and secondary stroke prevention. Although neither has been shown to prevent all SCD-related organ damage, these treatment modalities can improve the quality of life for individuals with SCD. Treatment with hydroxyurea is underutilized for many people with SCA who could benefit from it. Blood transfusion therapy has at times been underutilized, overutilized, or prescribed inappropriately for both acute and chronic complications. These two chapters provide guidance regarding the appropriate use of these therapies for SCD.