Cystic Fibrosis Foundation pulmonary guideline. pharmacologic approaches to prevention and eradication of initial Pseudomonas aeruginosa infection.

DESCRIPTION: The Cystic Fibrosis (CF) Foundation developed clinical care guidelines for the prevention of Pseudomonas aeruginosa infection, the treatment of initial P. aeruginosa infection, and the use of bronchoscopy to obtain routine airway cultures in individuals with CF. METHODS: A multidisciplinary committee developed questions about the prevention and treatment of initial P. aeruginosa infection and the use of bronchoscopy to obtain routine airway cultures. The outcome measure of interest was cultures without P. aeruginosa growth. Systematic reviews of PubMed, EMBASE, and the Cochrane Central Register of Controlled Trials were conducted in May 2012 and August 2013. Searches combined controlled vocabulary terms and text words for CF and terms relevant to each question. The entire committee reviewed the evidence, and final recommendation statements were graded using the U.S. Preventive Services Task Force system. Recommendation 1: The CF Foundation strongly recommends inhaled antibiotic therapy for the treatment of initial or new growth of P. aeruginosa from an airway culture (certainty of net benefit, high; estimate of net benefit, substantial; grade of recommendation, A). The favored antibiotic regimen is inhaled tobramycin (300 mg twice daily) for 28 days. Recommendation 2: The CF Foundation recommends against the use of prophylactic antipseudomonal antibiotics to prevent the acquisition P. aeruginosa (certainty of net benefit, moderate; estimate of net benefit, zero; grade of recommendation, D). Recommendation 3: The CF Foundation recommends routine oropharyngeal cultures rather than bronchoalveolar lavage cultures obtained by bronchoscopy in individuals with CF who cannot expectorate sputum to determine if they are infected with P. aeruginosa (certainty of net benefit, moderate; estimate of net benefit, moderate; grade of recommendation, B).

Improving chronic care delivery and outcomes: the impact of the cystic fibrosis Care Center Network.

Cystic fibrosis (CF) is a multisystem, life-shortening genetic disease that requires complex care. To facilitate this expert, multidisciplinary care, the CF Foundation established a Care Center Network and accredited the first care centres in 1961. This model of care brings together physicians and specialists from other disciplines to provide care, facilitate basic and clinical research, and educate the next generation of providers. Although the Care Center Network has been invaluable in achieving substantial gains in survival and quality of life, additional opportunities for improvements in CF care exist. In 1999, analysis of data from the CF Foundation's Patient Registry detected variation in care practices and outcomes across centres, identifying opportunities for improvement. In 2002, the CF Foundation launched a comprehensive quality improvement (QI) initiative to enhance care by assembling national experts to develop a strategic plan to disseminate QI training and processes throughout the Care Center Network. The QI strategies included developing leadership (nationally and within each care centre), identifying best CF care practices, and incorporating people with CF and their families into improvement efforts. The goal was to improve the care for every person with CF in the USA. Multiple tactics were undertaken to implement the strategic plan and disseminate QI training and tools throughout the Care Center Network. In addition, strategies to foster collaboration between care centre staff and individuals with CF and their families became a cornerstone of QI efforts. Today it is clear that the application of QI principles within the CF Care Center Network has improved adherence to clinical guidelines and achievement of important health outcomes.

Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health.

RATIONALE: Cystic fibrosis (CF) is an autosomal recessive disease characterized by abnormal airways secretions, chronic endobronchial infection, and progressive airway obstruction. The use of medications to slow the progression of lung disease has led to significant improvement in survival. An evidence review of chronic medications for CF lung disease was performed in 2007 to provide guidance to clinicians in evaluating and selecting appropriate treatment for individuals with this disease. We have undertaken a new review of the literature to update the recommendations, including consideration of new medications and additional evidence on previously reviewed therapies. A multidisciplinary committee of experts in CF pulmonary care was established to review the evidence for use of chronic medications for CF lung disease and make treatment recommendations. Published evidence for chronic lung therapies was systematically reviewed and resulting treatment recommendations were graded based on the United States Preventive Services Task Force scheme. These guidelines provide up-to-date evidence of safety and efficacy of chronic treatments of CF lung disease, including the use of novel therapies that have not previously been included in CF pulmonary guidelines.

Cystic fibrosis foundation: achieving the mission.

The Cystic Fibrosis Foundation is a voluntary, nonprofit, health organization whose mission is "to assure the development of the means to cure and control cystic fibrosis and to improve the quality of life for those with the disease." While substantial progress has been made, as evidenced by a marked increase in the median predicted age of survival, much work remains to be done. Ongoing medical programs and activities of the Cystic Fibrosis Foundation, which span basic science, drug discovery, drug development, clinical care, patient education, and advocacy, will be described in this article. The key role of respiratory therapists in the cystic fibrosis community will be highlighted.

Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health.

RATIONALE: Cystic fibrosis is a recessive genetic disease characterized by dehydration of the airway surface liquid and impaired mucociliary clearance. As a result, individuals with the disease have difficulty clearing pathogens from the lung and experience chronic pulmonary infections and inflammation. Death is usually a result of respiratory failure. Newly introduced therapies and aggressive management of the lung disease have resulted in great improvements in length and quality of life, with the result that the median expected survival age has reached 36 years. However, as the number of treatments expands, the medical regimen becomes increasingly burdensome in time, money, and health resources. Hence, it is important that treatments should be recommended on the basis of available evidence of efficacy and safety. OBJECTIVES: The Cystic Fibrosis Foundation therefore established a committee to examine the clinical evidence for each therapy and to provide guidance for the prescription of these therapies. METHODS: The committee members developed and refined a series of questions related to drug therapies used in the maintenance of pulmonary function. We addressed the questions in one of three ways, based on available evidence: (1) commissioned systematic review, (2) modified systematic review, or (3) summary of existing Cochrane reviews. CONCLUSIONS: It is hoped that the guidelines provided in this article will facilitate the appropriate application of these treatments to improve and extend the lives of all individuals with cystic fibrosis.