TABLE 4

Chronic Indicators According to Topic

IndicatorTopicIndicator DefinitionFunctionaModalitybStrength of Evidencec,26Importance Rating
33AsthmaChildren with SCD and asthma should have a written asthma planFRI777
34Cardiac careChildren with SCD with blood pressure values above the 95th percentile for age should be evaluated and treated with antihypertensivesTrIII786
35Hematopoietic stem cell transplantChildren with sickle cell anemia younger than 16 without significant end organ dysfunction should be counseled about hematopoietic stem cell transplantation as a curative interventionERIII6
36Hematopoietic stem cell transplantFamilies of children with sickle cell anemia younger than 16 with a history of stroke, recurrent acute chest syndrome, or recurrent painful crises, should be offered the option of a hematopoietic stem cell transplantTrIII79817.5
37HydroxyureaeHydroxyurea should be recommended for children 5 or older with recurrent painful episodes or ACS, and the recommendation should be documentedTrII828
38StrokeChildren with SCD with a confirmed clinical stroke should be started on a chronic transfusion program with the goal of maintaining HbS levels <30% to prevent recurrent strokeTrIII838
39dTransfusionChildren with SCD should be transfused with a minimum of ABO, C, D, E, and Kell-matched red cellsTrIIII63,648.5
40TransfusionChildren with SCD who are 2 or older who are receiving a chronic transfusion program should have a comprehensive annual screening assessment for iron overloadSTIII108
41TransfusionChildren with SCD receiving a chronic transfusion program should receive chelation therapy to maintain ferritin <1500 or liver iron <7 mg/g dry weight by biopsy or MRITrIII84868
  • a Function: S indicates screening; E, education; D, diagnosis; Tr, treatment; F, follow-up.

  • b Modality: H indicates history; Ph, physical examination; T, tests; I, intervention/medication; R, return/referrals.

  • c Strength of evidence: I indicates randomized controlled trial; II, nonrandomized controlled trial, cohort or case control study, or multiple time series; III, descriptive study or expert opinion.

  • d Indicator rated higher than 8 for importance.

  • e Since the panel meeting, findings from the BABY HUG study on the use of hydroxyurea in children younger than 5 have been released.87 This indicator should be re-evaluated as more evidence becomes available.