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Longitudinal decline in lung volume in a population of children with sickle cell disease

Joanna E MacLean1, Eshetu Atenafu, Melanie Kirby-Allen

  • 1Department of Pediatrics, Division of Respiratory Medicine, Research Institute, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. joannam4@med.usyd.edu.au

American Journal of Respiratory and Critical Care Medicine
|September 9, 2008
PubMed

Insights

Lung function in children with sickle cell disease (SCD) declines significantly with age, showing a restrictive pattern. This decline starts in childhood and is influenced by disease severity, not consistently by sex.

Area of Science:

  • Pediatric Pulmonology
  • Hematology
  • Genetics

Background:

  • Sickle cell disease (SCD) is linked to severe pulmonary complications, morbidity, and mortality.
  • The progression of lung function deficits throughout childhood in SCD patients remains poorly understood.

Purpose of the Study:

  • To compare lung function trajectories in children with SCD against race-matched predicted values.
  • To characterize the pattern of lung function changes during childhood in SCD.
  • To investigate the impact of clinical factors like age, sex, hemoglobin levels, and beta-globin genotype on these changes.

Main Methods:

  • Retrospective analysis of lung function data from children (8-18 years) with SCD.
  • Utilized mixed-model analysis to compare patient data with race-matched reference equations.
  • Examined the influence of age, sex, Hb level, and beta-globin genotype on longitudinal lung function trends.

Main Results:

  • Children with SCD exhibit a significant decline in spirometric lung volumes during childhood.
  • Average annual decline rates for FEV1 and total lung capacity were approximately 2.9-3.0% predicted for males and 2.4-2.9% predicted for females.
  • More severe beta-globin genotypes correlated with a faster decline in lung function; sex had an inconsistent effect.

Conclusions:

  • Lung volumes in children with SCD progressively decrease with age.
  • This decline initiates in childhood, indicating a predominantly restrictive lung defect.
  • Findings highlight the importance of monitoring pulmonary function in pediatric SCD patients.
Abstract

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