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. Author manuscript; available in PMC: 2023 Jul 6.
Published in final edited form as: Hematol Oncol Clin North Am. 2022 Dec;36(6):1217–1237. doi: 10.1016/j.hoc.2022.07.014

Table 2.

Summary of studies on lung function in sickle cell disease

Studies (year) Study Designs Populations Findings
Ivankovich et al39, 2019 Cross sectional case-control 22 infants with SCD, 37 healthy control infants aged 6–18 months Normal lung function in 77% of infants with SCA but those w SCA had lower FVC, FEF0.5, FEF25–75 compared with controls
Koumbourlis et al40, 1997 Cross sectional 20 infants with SCD aged 3–30 months (12 with HbSS) Elevated FRC, decreased maximum expiratory flow at FRC, and increased time to maximum expired flow/total expiratory time, suggesting hyperinflation and lower airway obstruction
Cohen et al41 2016 Cross sectional and longitudinal cohort 149 children aged 6–19 years with HbSS with spirometry and plethysmography; 139 had morbidity data from birth, 136 with prospective morbidity data (Sleep and Asthma Cohort Study) 70% with normal lung function, 16%, 7%, and 6% with obstructive, restrictive, nonspecific, and mixed patterns, respectively. Lung function pattern not associated with prior or future pain or ACS episodes
Arteta et al42, 2014 Cross sectional 146 children with HbSS or HbSβ0 (PUSH study) 19% had obstruction (44% of whom had asthma), 9% had restriction, 11% had nonspecific abnormal lung function, and 19% had reduced DLCO
MacLean et al46, 2008 Single center longitudinal cohort study 312 children with SCD aged 8–18 years Average decline in FEV1: 2.93% per year for males, 2.95% per year for females; average decline in TLC: 2.15% per year for males, 2.43% per year for females
Lunt et al47, 2016 Longitudinal case-control study in 2 cohorts Cohort 1: 47 children with SCD, 26 controls; mean age 8.8 (range 3–14) years, followed up for 2 years
Cohort 2: 45 children with SCD, 24 controls; mean age 10.2 (range 4–17) years, followed up for 10 years
Cohort 1: At baseline: 34% with obstruction, 2% with restriction. FEV1 decline 1.45% per year
Cohort 2 at baseline: 24% with obstruction, 11% with restriction. FEV1 decline 0.9% per year.
Klings et al53, 2006 Multicenter longitudinal cohort study 310 adults with HbSS (Cooperative Study of SCD) 74% had restriction, mean TLC 70% of predicted; 13% had isolated reduced DLCO. DLCO inversely associated with age.
Field etal52, 2008 Single center longitudinal cohort study 92 adults with SCD, lung function testing obtained for clinical reasons 35.7% with restriction, 18% with obstruction. FEV1 decreased by 49 cc per year
Hodges et al51, 2022 Single center longitudinal cohort study 193 adults with SCD
309 adults with cystic fibrosis
Age 18–65 years
FEV1 declined 23 and 26 mL/year in those with SCD and cystic fibrosis, respectively, no difference between these two cohorts

Abbreviations: ACS, acute chest syndrome; DLCO, diffusion capacity for carbon monoxide; FEF0.5, forced expiratory flow in 0; 5 seconds; FEF25–75, forced expiratory flow from the 25th to 75%ile of the maneuver; FEV1, forced expiratory volume in 1 second; FRC, functional residual capacity; FVC, forced vital capacity; SCA, sickle cell anemia; SCD, sickle cell disease; TLC, total lung capacity.