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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 16 of 49
In Progress


SCFA_Coach September 25, 2023

Immunizations are one of the most useful preventive measures available to infants, children, and adults. This benefit should be extended to all individuals regardless of other chronic conditions, unless there is a specific disease-related or personal (e.g., allergy) contraindication. For people with SCD, there are no disease-related contraindications.

Summary of the Evidence
The Advisory Committee on Immunization Practices (ACIP) reviews the evidence for each immunization it recommends. The expert panel determined that the methodology used for those reviews was compatible with its own methodology. Therefore, evidence reviews for this topic were not performed by the methodology team.
The expert panel based its recommendations on those made by the ACIP (see exhibit 6).22

Evidence reviews on this topic were not performed by the methodology team. Therefore, the expert panel based its
recommendations on those developed by the ACIP (see exhibit 6).
1. All individuals with SCD should receive immunizations according to the ACIP harmonized immunization schedule unless they have a personal contraindication as noted in the ACIP schedule.
2. Because of their increased susceptibility to invasive pneumococcal disease, all infants with SCD should receive the complete series of the 13-vaIent conjugate pneumococcal vaccine series beginning shortly after birth and the
23-vaIent pneumococcal polysaccharide vaccine at age 2 years, with a second dose at age 5 years.‘
(Consensus—Panel Expertise}

All individuals should be immunized as recommended by the ACIP. The most up-to-date schedule should be followed, as changes can be made up to four times per year. Consult the immunization schedule at: http://www.cdc.qov/vaccines/schedules. The following immunizations are of special importance or unique to people with SCD as recommended by the ACIP. These recommendations may also change periodically, and the above ACIP recommendations should be consulted for confirmation.

• Pneumococcal (PCV13) vaccine—Children
Children aged 6 to 18 years with functional or anatomic asplenia should receive one dose of PCV13.
• Pneumococcal vaccine•naive Adults
Adults aged >’19 years with functional or anatomic asplenia who have not previously received PCV13 or PPSV23 should receive
• One dose of PCV13 first, followed by a dose of PPSV23 at least8 weeks later.
• Subsequent doses of PPSV23 should follow current PPSV23 recommendations for adults at high risk.
A second PPSV23 dose is recommended 5 years after the first PPSV23 dose for persons aged 19-64 years with functional or anatomic asplenia.
Additionally, those who received PPSV23 before age 65 years for any indication should receive another dose of the vaccine at age 65 years, or later if at least 5 years have elapsed since their previous PPSV23 dose.
• Previous vaccination with PPSV2 adults
Adults aged >’19 years with functional or anatomic asplenia who previously have received >’1 dose of PPSV23 should
• Be given a PCV13 dose >’1 year after the last PPSV23 dose was received.
— For those who require additional doses of PPSV23, the first such dose should be given no sooner than 8 weeks ager PCV13 and at least 5 years ager the most recent dose of PPSV23.
• Hib
One dose of Hib vaccine for people aged >5 years who have SCD if they have not previously received Hib vaccine
• Meningococcal vaccine
Vaccinate infants at high risk (including those with SCD) at 2, 4, and 6 months of age, and again at 12 through 15 months with this vaccine, which is generically known as HibMenCY.
Persons aged 9 months through 55 years at increased risk for meningococcal disease (e.g., adults with anatomic or functional asplenia or persistent complement component deficiencies) should receive MenACWY.
Children aged 2 months to 6 years should receive an additional dose of MenACWY 3 years after primary immunization; boosters should be repeated every 5 years thereafter.
Children >’7 years of age should receive an additional dose of MenACWY 5 years after primary immunization; boosters should be repeated every 5 years thereafter.


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