Quantifying the Presence of Lung Disease and Pulmonary Hypertension in Children With Sickle Cell Disease
Status:
Completed
Trial end date:
2014-04-01
Target enrollment:
Participant gender:
Summary
The proposed research study is a cross-sectional study enrolling young children with sickle
cell disease between 5 and 12 years of age. They will be screened as outpatients for consent
to perform pulmonary function testing (PFT) and echocardiography. In addition, the degree of
bronchodilator response will be assessed at each session. To estimate presence of pulmonary
hypertension, echocardiography will be performed at the time of PFT measures.
Study Design:
1. Enroll children aged 5 to 12 years of age with sickle cell disease (HbSS, HbSC, HbS beta
plus thalassemia, HbS beta zero thalassemia, and HbS OArab) who are established patients
within the Duke Pediatric Sickle Cell Clinic.
2. Perform a chart review of all enrolled subjects to obtain specific details regarding
birth history, nutritional status (weight, height), family history, sickle cell
genotype, parental smoking history, recent laboratory parameters, parental smoking
history, any concurrent conditions (atopy, asthma, airway anomaly), history of sickle
cell complications and prescribed medications.
3. Perform spirometry and plethysmography with the administration of albuterol.
4. Before or after completion the PFT session, the patient will have echocardiography in
the PFT lab area
5. Using medical record information, determine number of hospitalizations for any pulmonary
symptoms indicative of acute chest syndrome (ACS) (dyspnea, fever, wheezing, hypoxia,
cough, chest pain). In addition, we will track any respiratory or cardiac symptoms or
therapies for each subject 6 years after enrollment up to age 18 years using the
registry.
6. As standard of care, refer any child identified as having lung disease or pulmonary
hypertension to a pediatric pulmonologist and/or cardiologist for monitoring, treatment
and ongoing care.