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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 1 of 49
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Historical Perspective, Epidemiology, and Methodology

SCFA_Coach September 25, 2023

SCD was first reported in the literature in November 1910 by James B. Herrick, who referred to “peculiar elongated and sickle-shaped red blood corpuscles in a case of severe anemia.”’ We have gained substantial knowledge about SCD since that first description. Today there is hope for a cure using hematopoietic stem cell transplantation (HSCT). However, at present, the procedure is infrequently performed and very expensive. Additional research regarding patient and donor selection and the specific transplantation procedure is required before this potentially curative therapy will become more widely available. Two effective disease-modifying therapies for SC hydroxyurea and chronic transfusion—are potentially widely available but remain underutilized .

The sickle cell mutation results in substitution of the amino acid valine for glutamic acid at the sixth position of the beta globin chain, causing formation of hemoglobin S. More than 2 million U.S. residents are estimated to be either heterozygous or homozygous for the genetic substitution. Most of those affected are of African ancestry or self-identify as Black; a minority are of Hispanic or southern European, Middle Eastern, or Asian Indian descent.” It is estimated that between 70,000 and 100,000 Americans have SCD. Although SCD is associated with major morbidity, currently more than 90 percent of children with SCD in the United States and the United Kingdom survive into adulthood. However, their lifespan remains shortened by two or three decades compared to the general population.

The most prevalent SCD genotypes (exhibit I) include homozygous hemoglobin SS (HbSS) and the compound heterozygous conditions hemoglobin S§0-thalassemia (HbSb0-thalassemia), hemoglobin S§ -thalassemia (HbSb’-thalassemia), and hemoglobin SC disease (HbSC). HbSS and HbS§O-thalassemia are clinically very similar and therefore are commonly referred to as sickle cell anemia (SCA); these genotypes are associated with the most severe clinical manifestations. These guidelines are not applicable to individuals with sickle cell trait (HbAS), the carrier state.

Currently in the United States, there are no comprehensive, systematically reviewed, evidence-based guidelines to assist health care professionals in the management of individuals with SCD. Providing care to these individuals can be challenging. As a result of the condition’s relative rarity, there are few health care professionals who are prepared to deliver continuity care or expert consultation for patients with serious acute or chronic SCD complications. These guidelines are therefore being made available to help provide the latest evidence-based recommendations to manage this condition and to help engage health care professionals in supporting their implementation at the practice level.


Exhibit 1a: Typical Laboratory Findings in Sickle Cell Disease

Exhibit 1b: Typical Laboratory Findings in Sickle Cell Trait (Provided for Comparison)