Pulmonary dysfunction among adolescents and adults with sickle cell disease in Nigeria: Implications for monitoring.

Background: Pulmonary complications of sickle cell disease (SCD) contribute to excess morbidity and mortality. The burden of pulmonary dysfunction among Nigerians with SCD has not been well elucidated.

Objectives: The objectives of this study are to describe the frequency and pattern of spirometry abnormalities in SCD and to explore the association between pulmonary dysfunction and selected parameters.

Methods: A cross-sectional study among adolescents and adults with SCD attending a University Teaching Hospital and healthy age- and gender-matched controls. Respiratory symptoms, oxygen saturation, spirometry, complete blood counts, and fetal hemoglobin (Hb) were measured.

Results: A total of 245 participants with SCD and 216 controls were included in the study. Frequency of respiratory symptoms was similar between the two groups. The median forced expiratory volume 1 (FEV1), forced vital capacity (FVC), and the FEV1/FVC were significantly lower in SCD as compared to controls ( = 0.000 in all instances). The frequency of abnormal pulmonary patterns was higher in SCD as compared to controls with abnormal spirometry pattern in 174 (71%) and 68 (31.5%) of participants with SCD and controls, respectively ( = 0.000). The suggestive of restrictive pattern was predominant (48% vs. 23%), but obstructive (11.8% vs. 7.4%) and mixed patterns (11% vs. 0.9%) were also found among SCD versus controls. Hb concentration was positively associated with FEV1 and FVC, whereas white cell count and age were negatively associated with FVC and FEV1, respectively.

Conclusion: There is a high burden of pulmonary dysfunction in SCD among Nigerians which may be related to the severity of disease. There is a need for further research to explore the effectiveness of potential interventions so as to harness the benefits from monitoring and early detection.