The use of bilevel positive airway pressure for the treatment of acute chest syndrome of sickle cell disease.

Objective: To assess the outcome of bilevel positive airway pressure (BPAP) use for pediatric patients with sickle cell disease and acute chest syndrome.

Design: Retrospective chart review of 25 occurrences of acute chest syndrome in nine children from 1994 to 2000.

Setting: A tertiary care children's hospital.

Patients: Seven boys and two girls (average age, 11.8 years; range, four to 20 years). Prior to admission, 80% had chest pain, 48% had back pain, 48% had extremity pain, 24% had fever, and 20% had cough.

Interventions: Bilevel positive airway pressure therapy.

Measurements And Main Results: Patients' clinical values before and after BPAP were as follows: oxygen support (L), 4.1 +/- 3.2 and 1.4 +/- 1.7 (p < 0.001); oxygen saturation (%), 96.3 +/- 2.8 and 97.9 +/- 1.6 (p < 0.05); respiratory rate (per minute), 28.5 +/- 8.6 and 25.1 +/- 6.6 (p < 0.05); heart rate (per minute), 109 +/- 18 and 92 +/- 13 (p < 0.001). Patients' average highest intermittent positive airway pressure was 12 cm H2O, and the average highest expiratory positive airway pressure was 6 cm H2O. Patients spent an average of 3.1 days receiving BPAP. Of the patients, 4% suffered skin irritation over their nasal bridge, and 56% were admitted to the intensive care unit. The BPAP therapy was ineffective for only one patient. Most patients (96%) received BPAP in response to respiratory distress; 4% received it in response to increasing oxygen requirements after administration of narcotics and inability to perform incentive spirometry.

Conclusion: Data suggest that BPAP therapy can be used to improve oxygenation and decrease work of breathing among patients with acute chest syndrome. We believe that BPAP may prevent progression to acute hypoxic respiratory failure requiring intubation and ventilation. It may reduce costs, especially if intensive care unit admission can be avoided by beginning therapy early. This therapy may become a standard of care for children with acute chest syndrome. The study design (a retrospective chart review) was subject to limitations and bias. A multicenter, prospective, randomized trial is recommended.