Essential strategies and tactics for managing sickle cell disease
Key to patients’ well-being is the family physician’s watchfulness—through periodic lab testing and health checks and diligent application of preventive measures.
PRACTICE RECOMMENDATIONS
› Offer rapid access to narcotic analgesia for patients with sickle cell disease (SCD) who have recurrent vaso-occlusive crises, to prevent unnecessary hospitalization. A
› Provide oral penicillin prophylaxis against pneumococcal disease in patients < 5 years of age who have sickle cell anemia (SCA), but not in children whose SCD is less severe. A
› Screen all patients with SCA annually, beginning at age 2 years until age 16 years, for their risk of stroke, using a transcranial Doppler study. A
› Administer the COVID-19 mRNA vaccine series to all patients with SCD, unless contraindicated. A
Strength of recommendation (SOR)
A Good-quality patient-oriented evidence
B Inconsistent or limited-quality patient-oriented evidence
C Consensus, usual practice, opinion, disease-oriented evidence, case series
The need for coordinated, comprehensive care
Patients with SCD report how challenging their disease is. All patients with SCD are more likely than age-matched counterparts to experience loss, including workdays for disability, educational potential, workdays for caregivers of affected children, and time spent in the hospital or the emergency department.4,5 These losses, with the concomitant stress associated with chronic illness and the struggle to manage recurrent pain crises and chronic complications, are often overwhelming.
Comprehensive care can, as we have illustrated in this discussion, mitigate these losses. Such care should include extensive education, genetic counseling, infection prevention, pain management, and implementation of evidence-based management guidelines.3,4,6 Patients with SCD report that their illness outlook would be better with
- greater provider knowledge of SCD,
- destigmatization of narcotics for SCD vaso-occlusive pain management,
- optimal coordination among members of the health care team, and
- improved transportation for appointments.
Patients also report that barriers associated with the unique US health care financing system are often insurmountable. As patients with SCD live longer, improved care management should focus on reducing these barriers and enhancing their quality of life.2,18,19
CORRESPONDENCE
Robert Allen Perkins, MD, MPH, Department of Family Medicine, University of South Alabama College of Medicine, 1601 Center Street, 2N, Mobile, AL 36604; perkins@health. southalabama.edu
