pilot observational study to determine feasibility of a standardized treatment of pulmonary exacerb. in patients with cf

Conditions: Cystic Fibrosis; Lung Infection; Cystic Fibrosis Pulmonary Exacerbation

Summary: the goal of this research study is to better understand current treatment practices for pulmonary exacerbations (lung infections) and whether the cystic fibrosis national patient registry (cffnpr)can be used for this type of study.

cystic fibrosis (cf), a life-shortening genetic disease, is marked by acute episodes during which symptoms of lung infection increase and lung function decreases. these pulmonary exacerbations (pes) are treated with varying antibiotics for varying time periods based on needs determined by individual patients, their families, and the health care providers. cystic fibrosis pulmonary guidelines for the treatment of pe published by the cystic fibrosis foundation (cff) in 2009 provided recommendations for treatment and also identified key questions for which additional studies were needed. standard treatment for pe involves many facets including selection of antibiotics, duration of use, and outcomes that define treatment success. understanding current treatment practices and measures of treatment success are needed before a study can be designed to define optimal treatment strategies. this is a multi-center, prospective, observational study designed to prospectively follow patients with cf that are initially admitted to the hospital for treatment of a pulmonary exacerbation.

Eligibility criteria:
inclusion criteria: * male or female ≥12 years of age at visit 1 * enrolled in the cffnpr (patients may enroll in the registry at visit 1 if not previously enrolled.) * current hospitalization for treatment of a pulmonary exacerbation * planned hospital admission of at least 5 days with intravenous (iv) antibiotics at visit 1 * able to perform spirometry at admission and willing to perform spirometry on subsequent treatment and visit days * willing and able to complete symptom score daily * willing to return for a follow up visit at end of treatment (if necessary) and 28 days after start of iv antibiotic therapy * written informed consent (and assent when applicable) obtained from the participant or participant's legal representative exclusion criteria: * previous enrollment in this study * treatment with iv antibiotics in the 6 weeks prior to visit 1 * admission to the intensive care unit for current pulmonary exacerbation * pneumothorax on admission * current hospitalization for scheduled pulmonary clean out * current hospitalization for sinusitis as the primary diagnosis * massive hemoptysis defined as \> 250 cc in a 24 hour period, or 100 cc/day over 4 consecutive days occurring within one week of visit 1 * current pulmonary exacerbation thought to be due to allergic bronchopulmonary aspergillosis (abpa) * ongoing treatment with prednisone equivalent \>10 mg/day for greater than 2 weeks initiated prior to visit 1 * history of solid organ transplantation currently receiving antimicrobial therapy to treat non-tuberculous mycobacterium (e.g., m. abscessus, m. avium complex)

Keywords: cystic fibrosis pulmonary exacerbation lung infection cystic fibrosis foundation cystic fibrosis foundation national patient registry