Cystic fibrosis is an autosomal recessive disease leading to a mutated cystic fibrosis transmembrane conductance regulator protein complex (CFTR), leading to decreased chloride secretion. Systems potentially involved in CF include pulmonary, hepatobiliary, and gastrointestinal. Characteristic pulmonary manifestations include airway obstruction and infection. Hepatobiliary include both pancreatic exocrine and endocrine insufficiency. Gastrointestinal include effects of pancreatic exocrine insufficiency including steatorrhea andmalnutrition, as well as diarrhea and/or constipation. The objective of this study is to characterize the effect of a single bowel purge on pulmonary function tests (PFTs) in patients with cystic fibrosis (CF). To our knowledge, this has not been evaluated in a systematic fashion. Patients will be identified at the time of their clinic appointment at Lurie Children’s by a PI, and will be enrolled by a study coordinator. Baseline PFTs will be performed as per routine. Patients will answer a short survey on bowel habits (PAC-SYM). Patients will be given a prescription for polyethylene glycol 3350 and electrolytes oral solution (GoLytely). Within two weeks of a routine follow up appointment (which will not be more than six months after the initial clinic visit), patients will follow a modified bowel prep (a full day of clear liquids and drinking the entire GoLytely solution). At the subsequent clinic visit, PFTs will be rechecked, and the patients will answer the same short survey (PAC-SYM). Statistical analysis will be performed on change in PFTs from baseline, change from prior patient PFTs (if available), and change in PAC-SYM score. Primary outcome is change in PFTs from baseline. Secondary outcomes are change in PAC-SYM score, and change in other parameters of PFTs from baseline. Pending results of this pilot study, we may expand this study to include more patients and potentially other sites. Funding will be directed at larger grants, including from the Cystic Fibrosis Foundation. This interdisciplinary pilot study is aimed at using digestive health, in the form of improvement in constipation by bowel purge, to improve the pulmonary function in people with cystic fibrosis.
|Effective start/end date||9/1/17 → 8/31/19|
- Northwestern Memorial Hospital (Agmt #12 Sgnd 10/9/17 // Agmt #12 Sgnd 10/9/17)
- Digestive Health Foundation (Agmt #12 Sgnd 10/9/17 // Agmt #12 Sgnd 10/9/17)
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