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Lower airway obstruction is associated with increased morbidity in children with sickle cell disease
Jessica H Boyd1, Michael R DeBaun, Wayne J Morgan
1Division of Allergy and Pulmonary Medicine, Department of Pediatrics, Washington University School of Medicine, St. Louis, Missouri 63110, USA. boyd_j@kids.wustl.edu
Insights
Children with sickle cell disease (SCD) and lower airway obstruction experience twice the rate of pain or acute chest syndrome (ACS) hospitalizations. Increased surveillance is recommended for these children to monitor for future health issues.
Area of Science:
- Pediatric Pulmonology
- Hematology
- Clinical Medicine
Background:
- Pulmonary function abnormalities are not well-studied in relation to morbidity in pediatric sickle cell disease (SCD).
- Morbidity in SCD is often assessed by hospitalizations for pain or acute chest syndrome (ACS).
Purpose of the Study:
- To investigate the association between pulmonary function test results and the incidence of pain or ACS hospitalizations in children with SCD.
- To determine if specific pulmonary function abnormalities predict future morbidity events in this population.
Main Methods:
- Pulmonary function tests (PFTs) were performed on children aged 6-18 years with SCD.
- PFT results were categorized into lower airway obstruction, restriction, or normal lung function.
- Hospitalization rates for pain or ACS were compared between groups with abnormal PFTs and those with normal PFTs.
Main Results:
- Children with lower airway obstruction had a significantly higher rate of hospitalizations for pain or ACS (2.5 vs. 1.2 per patient-year, RR 2.0, P=0.003).
- No significant difference in morbidity rates was observed for children with restrictive lung function compared to those with normal lung function (1.4 vs. 1.2 per patient-year, RR 1.1, P=0.68).
Conclusions:
- Lower airway obstruction in children with SCD is associated with increased future morbidity, specifically pain or ACS hospitalizations.
- Children with SCD and lower airway obstruction warrant closer monitoring and increased surveillance for adverse health events.
Rationale:
The association between pulmonary function and morbidity in children with sickle cell disease (SCD) has not been previously evaluated. Our objective was to study the relationship between abnormalities in pulmonary function and morbidity as represented by the rate of hospitalizations for pain or acute chest syndrome (ACS) in children with SCD.
Methods:
Results of pulmonary function tests obtained for clinical indications in children ages 6-18 years were classified as lower airway obstruction (forced expiratory volume in 1 sec/forced volume capacity <95% confidence interval adjusted for age, gender, race, and height), restriction (total lung capacity <80% predicted adjusted for gender, age, race, and height), and normal lung function. Incidence rates of pain or ACS were compared between children with lower airway obstruction or restriction and children with normal lung function.
Results:
A total of 102 children, mean age at evaluation 12.0 years with follow-up of 3.8 years, were included. Children with lower airway obstruction had twice the rate of morbidity compared to children with normal lung function (2.5 vs. 1.2 hospitalizations for pain or ACS per patient-year, P = 0.003) (Risk ratio: 2.0; 95% CI: 1.3-3.3). Children with restriction did not have different rates of future morbidity compared to children with normal lung function (1.4 vs. 1.2 hospitalizations for pain or ACS per patient-year, P = 0.68) (Rate ratio: 1.1; 95% CI: 0.6-2.1).
Conclusions:
We conclude that children with SCD who have lower airway obstruction should have increased surveillance for future morbidity.
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