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Pediatrics |

Pulmonary Function in Children With Sickle Cell Disease Following Treatment With Hydroxyurea

Jonathan Popler*, MD; Elizabeth Record, PhD; Tamara New, MD; LaTresa Lang, MD; LeRoy Graham, MD; Burton Lesnick, MD; Patricia Waters, BSN; Renee Mumford, RRT; Rhonda Copeland, RRT
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Georgia Pediatric Pulmonology Associates, Atlanta, GA


Chest. 2012;142(4_MeetingAbstracts):769A. doi:10.1378/chest.1387290
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Abstract

SESSION TYPE: Pediatric Chest Disease

PRESENTED ON: Sunday, October 21, 2012 at 10:30 AM - 11:45 AM

PURPOSE: In patients with sickle cell disease, chronic hemolysis and persistent inflammation may lead to progressive pulmonary vasculopathy, including acute chest syndrome (ACS), chronic lung disease, and abnormal pulmonary function. The purpose of this study was to assess the changes in pulmonary function of children with sickle cell disease treated with hydroxyurea (HU) therapy.

METHODS: A retrospective review was performed to examine changes observed in pulmonary function tests (PFTs) of two groups of children with sickle cell disease and previously abnormal PFTs. Children were selected from patients followed in a multi-disciplinary clinic with care provided by hematology, pulmonology, and respiratory care services. Group 1 consisted of 40 children treated with HU therapy. Group 2 consisted of 25 children who did not receive HU therapy. Paired t-test was used to evaluate PFT and pulse oximetry changes.

RESULTS: In Group 1, the mean age of patients was 10.9 years. There were 18 female and 22 male patients. The mean duration of HU therapy was 19 months. In Group 2, the mean age of patients was 12.3 years. There were 13 males and 11 females. Mean duration between PFTs was 18.9 months. The group treated with HU therapy had statistically significant improvements in Forced Vital Capacity (FVC), Forced Expiratory Volume in 1 second (FEV1), and pulse oximetry. Group 2 did not show improvement in any PFT parameter or pulse oximetry.

CONCLUSIONS: In Group 1, serial PFTs showed improvement in FVC, FEV1 and pulse oximetry following initiation of HU therapy. Group 2 did not show improvement in any PFT parameter. These findings suggest the consideration of routine use of HU therapy in patients with sickle cell disease and abnormal PFTs.

CLINICAL IMPLICATIONS: In children with sickle cell disease and abnormal pulmonary function, initiation of HU therapy may improve FEV1 and FVC, slowing the progression of lung disease. Routine assessment of PFTs in children with sickle cell disease may serve as an objective measure of pulmonary complications, especially in children without a history of ACS.

DISCLOSURE: The following authors have nothing to disclose: Jonathan Popler, Elizabeth Record, Tamara New, LaTresa Lang, LeRoy Graham, Burton Lesnick, Patricia Waters, Renee Mumford, Rhonda Copeland

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Georgia Pediatric Pulmonology Associates, Atlanta, GA

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