A comprehensive Sickle Cell Pulmonary Clinic is held monthly at Children’s Healthcare of Atlanta’s Scottish Rite Campus co-attended by a Pediatric Pulmonologist and Pediatric Hematologist .
Sickle Cell Disease (SCD) patients with a history of ACS or suspected lung disease are referred to our clinic. Routine outpatient monitory and management of their SCD is continued under the direction of the attending hematologist. Cardiopulmonary evaluation includes pulse oximetry, spirometry and plethysmography, overnight polysomnography, and echocardiography. Pulmonary management includes treatment of obstructive and restrictive lung disease, management of obstructive sleep apnea, and nocturnal oxygen supplementation when indicated. Patients with elevated estimated pulmonary artery pressure are referred to a cardiologist for further evaluation and management. Cardiopulmonary dysfunction is considered as an indication for intensified management of SCD including transfusion therapy and hydroxyurea.
Over the last 25 months in 29 clinic sessions, 109 patients have been followed in a total of 253 clinic visits. 57 patients (52.2%) were male. The mean age at the initial visit was 9.1 years (median=8.3, range 1.9 to 18.2). Complete pulmonary functions were obtained in 63 patients at their initial visit with a mean TLC of 82.5 % of predicted (37% < 80%) and mean RV/TLC of 0.36 (93% > 0.25). Patients are typically seen at 6 month intervals. The annualized rate of Acute Chest Syndrome (ACS) in 70 patients seen for two consecutive visits (average interval-221 days) was reduced from an initial rate of 0.79 events/year to 0.33 events/year. (p = .0113) The annualized rate of ACS in 31 patients seen for 3 consecutive visits (average interval between visits 1 and 3-393 days) was reduced from 1.14 events/year to 0.30 events/year. (p<0.001) .
Therapeutic interventions based on cardiopulmonary assessment in pediatric SCD patients followed in a comprehensive Sickle Cell Pulmonary Clinic is associated with a reduced incidence of ACS.
Cardiopulmonary evaluation and the collaboration of pediatric hematologists and pulmonolgists are important but often overlooked components in the management of pediatric patients with SCD.
L.M. Graham, None.