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Age is a predictor of a small decrease in lung function in children with sickle cell anemia.

Shaina M Willen1, Robyn Cohen2, Mark Rodeghier3

  • 1Department of Pediatrics, Division of Hematology/Oncology, Vanderbilt-Meharry Center for Excellence in Sickle Cell Disease, Vanderbilt University Medical Center, Nashville, Tennessee.

American Journal of Hematology
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Summary
This summary is machine-generated.

Lung function in children with sickle cell anemia (SCA) shows a minimal decline over time. Disease characteristics and therapies did not predict this FEV1% predicted decrease in young patients.

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Area of Science:

  • Pediatric Pulmonology
  • Hematology
  • Sickle Cell Disease Research

Background:

  • Lung function decline, indicated by FEV1% predicted, is a mortality risk in adults with sickle cell anemia (SCA).
  • Predictors for FEV1% predicted decline in pediatric SCA populations are not well understood.

Purpose of the Study:

  • To determine if FEV1% predicted declines longitudinally in children with SCA.
  • To identify SCA-specific characteristics and therapies that predict this decline.

Main Methods:

  • A prospective, longitudinal, multi-center cohort study (Sleep and Asthma Cohort - SAC) of children with SCA (HbSS or HbSβ0 thalassemia).
  • 197 participants underwent spirometry and lung volume assessments over an average of 4.4 years.
  • Multivariable analysis was used to assess the association between age, disease features, therapy, and FEV1% predicted decline.

Main Results:

  • FEV1% predicted showed a minimal decline of 0.3% per year of age.
  • No significant associations were found between FEV1% predicted decline and sex, asthma history, hemoglobin levels, reticulocyte count, white blood cell count, pain episodes, acute chest syndrome, or hydroxyurea therapy.

Conclusions:

  • FEV1% predicted demonstrates a minimal longitudinal decline in children with SCA over approximately 4 years.
  • Current disease features and therapies examined do not predict this decline in pediatric SCA patients.