Respiratory Care and Cystic Fibrosis

Respiratory Care, 2009

The role of the respiratory therapist in the care of patients with cystic fibrosis has expanded throughout the years. As key members of the multidisciplinary team, respiratory therapists actively participate in the medical management of patients with cystic fibrosis along the continuum of care, from acute in-patient stays to the out-patient clinic and/or home setting. Through their involvement in diagnostic testing, administering therapy, or direct bedside care, patient and caregiver education, and disease management, respiratory therapists strive to preserve lung function, maintain overall health, and improve the patient's quality of life.

Respiratory care, 2018

It is always an exciting challenge to write a Year in Review artcicle identifying the best publications in the preceding year; in this case from October 2016 until the AARC meeting in October 2017. This is particularly true for cystic fibrosis (CF), where there has been an explosion of new data, new medications, and new understanding of the pathophysiology of the disease. PubMed lists more than 2,500 papers published during those 12 months, many of them outstanding. I am indebted to many colleagues and friends who are leaders in the CF community, active readers of the pediatric pulmonary listserv, and scientists and clinicians engaged in the care of CF, for offering their suggestions regarding which articles should be included in this review. I believe that you will enjoy reading this curated selection of manuscripts that I have tried to organize by theme.

Expert Review of Respiratory Medicine, 2015

BMC Medicine, 2012

Since the discovery of the gene associated with cystic fibrosis (CF), there has been tremendous progress in the care of patients with this disease. New therapies have entered the market and are part of the standard treatment of patients with CF, and have been associated with marked improvement in survival. Now there are even more promising therapies directed at different components of the pathophysiology of this disease. In this review, our current knowledge of the pathophysiology of lung disease in patients with CF is described, along with the current treatment of CF lung disease, and the therapies in development that offer great promise to our patients.

European Respiratory Journal, 2020

Cystic fibrosis (CF) is a common multi-system genetically inherited condition, predominately found in individuals of Caucasian decent. Since the identification of the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) gene in 1989, and the subsequent improvement in understanding of CF pathophysiology, significant increases in life-expectancy have followed. Initially this was related to improvements in the management and systems of care for treating the various affected organ systems. These cornerstone treatments are still essential for CF patients born today. However, over the last decade, the major advance has been in therapies that target the resultant genetic defect: the dysfunctional CFTR protein. Small molecule agents that target this dysfunctional protein via a variety of mechanisms have led to lung function improvements, reductions in pulmonary exacerbation rates and increases in weight and quality-of-life indices. As more patients receive these agents earlier an...

2015

Dennis Watt obtained his degree in Medicine (MB BCh) in Cardiff, UK, in 1998 and was awarded Doctor of Medicine (MD) for his research on the impact of respiratory viruses in Cystic Fibrosis from the University of Cardiff. He trained in pulmonary medicine in Cambridge. During this period, he had the opportunity to work in the Cystic Fibrosis Unit at Papworth, UK. In 2009, he was appointed as Consultant Respiratory Physician in the Cambridge Centre for Lung Infection at Papworth Hospital, dedicated to looking after patients with Cystic Fibrosis and non-CF bronchiectasis. In 2012, Dennis moved to Liverpool for family reasons and joined the Knowsley Community COPD Service. Under his stewardship, this service has since won the Health Service Journal 'Respiratory Integrated Care Award' in 2013. Apart from his vast experience practising CT and COPD, he also has experience in asthma, non-CF bronchiectasis, sleep medicine and oxygen therapy for the management of chronic lung diseases. Dennis has published widely in respiratory medicine, including peer-reviewed journals, abstracts and book chapters. He has also been a reviewer for a number of high-impact peer-reviewed journals.

Journal of Cystic Fibrosis, 2012

This is the third article related to a review of the literature based on data from national cystic fibrosis (CF) patient registries up to June 2008 and covering a total of 115 published studies. It focuses on several topics: CF incidence, genotype/phenotype correlation, microbiology, pregnancy/ paternity, clinical complications, lung transplantation, and others.

Journal of Cystic Fibrosis, 2020

Background: Advanced cystic fibrosis lung disease (ACFLD) is common, is associated with reduced quality of life, and remains the most frequent cause of death in individuals with cystic fibrosis (CF). These consensus guidelines provide recommendations to the CF community on management of both common and unique issues that arise when individuals reach a state of ACFLD. Methods: The CF Foundation assembled a multidisciplinary expert panel consisting of three workgroups: Pulmonary management; Management of comorbid conditions; Symptom management and psychosocial issues. Topics were excluded if the management considerations did not differ in ACFLD from in the overall CF population or if already addressed in other published guidelines. Recommendations were based on a systematic literature review combined with expert opinion when appropriate. Results: The committee formulated twenty-three recommendation statements specific to ACFLD that address the definition of ACFLD, pulmonary and intensive care unit management, management of selected comorbidities, symptom control, and psychosocial issues.