Routine comprehensive care for children with sickle cell disease
- Zora R Rogers, MD
Zora R Rogers, MD
- Professor of Pediatrics
- The University of Texas Southwestern Medical Center
- Section Editor
- Donald H Mahoney, Jr, MD
Donald H Mahoney, Jr, MD
- Section Editor — Pediatric Hematology
- Professor of Pediatrics
- Baylor College of Medicine
The remarkable progress in the care of children with sickle cell disease (SCD) over the past several decades is directly attributable to the evolution of a standard of anticipatory guidance offered to families of affected children by most pediatric hematologists . Although there is a paucity of formal evidence-based standards of care, the National Institutes of Health (NIH) has released new evidence-based guidelines for the management of SCD [2,3]. While this document was developed with rigid evidence-based guideline methodology, many of the recommendations are moderate strength with low quality of evidence, usually based on expert consensus. This topic review represents a synthesis of the standards promoted by various professional societies [4-6], state and regional guideline efforts, as well as by more recent formal attempts to define quality care.
Each location at which clinical care is provided to children with SCD will have a different approach of how to implement the policies and patient education goals outlined below. Many providers use a roadmap or outline of care that is signed off at each visit or when a milestone is reached. In addition, formal assessment of these elements of care may serve as the basis for a quality improvement project [7-9]. However it is executed, it is beneficial to develop an outline of care that can be used to verify that planned educational and screening maneuvers have occurred. This is particularly true with respect to readiness for transition to adulthood education for medically complex patients.
The key elements of routine care and anticipatory guidance for a child with SCD are outlined here. Overviews of other aspects of the evaluation and management of SCD are discussed in separate topic reviews:
●Diagnosis – (See "Diagnosis of sickle cell disorders".)
●Clinical manifestations – (See "Overview of the clinical manifestations of sickle cell disease".)
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- NEWBORN SCREENING FOLLOW-UP
- First visit with an experienced SCD clinician
- - Timing
- - Laboratory testing
- - Medication
- Prophylactic penicillin
- Folic acid
- - Education and anticipatory guidance
- How to contact the sickle cell specialist
- ADDITIONAL EDUCATION
- Painful crises or events
- Acute chest syndrome
- Hydroxyurea, chronic transfusion, and stem cell transplantation
- FOLLOW-UP VISITS WITH A SCD EXPERT CLINICIAN
- Routine monitoring at all visits
- Additional age-specific recommendations
- - Zero to two years
- - Two to five years
- - Five years to adolescence
- - Older adolescents
- Other components of a comprehensive care program
- - Care plans
- - Other specialists for consultation
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS