Assessment of Evidence-Based Pharmacologic Therapy for Pediatric Patients With HbSS Disease at a Single Pediatric Institution.

OBJECTIVE

In the United States, approximately 100,000 people currently live with sickle cell disease. Despite the prominence of disease, there remains room for enhancing patient care. The use of hydroxyurea has improved patient outcomes, showing a decrease in vaso-occlusive crises, acute chest syndrome, the need for blood transfusions, and mortality. Other strides in management include antibiotic prophylaxis for younger patients, optimization of immunizations, and institution of novel agents.

METHODS

The purpose of this study was to evaluate pediatric patients with sickle cell disease at a single institution by assessing adherence to the prescribing practices of evidence-based pharmacologic therapy. The primary outcome was evaluation of prescribing practices of hydroxyurea or erythrocytapheresis, antibiotic prophylaxis, and immunizations. Secondary outcomes include the prescribing of novel therapies, the evaluation of hydroxyurea dosing, and the comparison of vaccination rates with a previous study completed at the same institution. Data collected include active therapy with hydroxyurea or erythrocytapheresis, supportive care with antibiotic prophylaxis for patients younger than 5 years, and vaccination adherence per current guideline recommendations.

RESULTS

A total of 104 patients were included. Of those reviewed, 79% were prescribed hydroxyurea, and 17% were receiving erythrocytapheresis. Supportive care results showed 100% of patients younger than 5 years were receiving appropriate antibiotic prophylaxis. On comparison with a previous institutional -analysis, those up-to-date on vaccinations were 66% vs 9.4% (p < 0.05).

CONCLUSIONS

Results demonstrated high prescribing rates of therapy with either hydroxyurea or -erythrocytapheresis and antibiotic prophylaxis but highlighted the continued opportunity to improve -vaccination rates.