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Patient/provider panels will discuss the partnering process among CF patients, families, and providers for sustaining daily CF care to achieve realistic and mutually acceptable nutrition goals through collaboration and education.
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Increasing numbers of people with CF now benefit from disease-altering small molecule therapies that target the basic defect in CFTR. Use of these therapies has been extended recently using a combination of in vitro laboratory experiments to identify responsive mutations, and clinical trials to determine effect on clinical outcomes. This plenary will summarize how recent advances may allow in the near future over 90% of individuals with CF to benefit from therapies that target the basic defect in CFTR, including even those with the rarest disease causing mutations.
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Plenary 2 will review the progression of CF lung disease and the option of lung transplant to treat advanced disease. The presentation will highlight the challenges associated with each phase of the transplant journey, including the referral to a transplant center, waiting for a transplant, undergoing the transplant procedure, and the care requirements and life after transplant.
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This plenary session will focus on ways people with CF and their families make a difference by partnering with the larger community as advocates, by sharing their expertise on living life with CF with their care center teams, and by supporting with each other through virtual events and peer mentoring.
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The recently solved structures of full-length CFTR provide a template on which to evaluate electrophysiological and biochemical studies of this channel forming membrane protein. This symposium will discuss the cryo-electron microscopy structures of CFTR and their use for the interpretation of channel function studies and disease-causing mutations. Additionally, the symposium will explore the application of these structures for the development of new therapeutics for common and rare CF-causing alleles.
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This session will review guidelines for CF therapy, the implementation of guidelines into clinical practice, assessment of CF lung disease, and challenges in the treatment of advanced CF lung disease.
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This session will focus on the utilization of CF animal models for investigation into CF disease manifestations of diabetes, airway clearance, altered intestinal stem cell proliferation and gastrointestinal cancer risk. Studies of CF ferrets, pigs, mice and ex vivo applications (cell or organoid culture) investigate the specific roles of CFTR in beta cell function, mucociliary clearance of the airways, Wnt signaling of stem cells and intestinal tumor suppression. These topics are discussed within the context of the utility and limitations of these animal models for understanding CF disease and its treatment.
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This session will review currently available new antibiotics as well as drugs and compounds currently under development for the treatment of pulmonary infections in individuals with cystic fibrosis.
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An interactive session designed to review the role of various services important in contributing to the nutritional management of individuals with cystic fibrosis and how these services are integrated to provide optimal care.
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This symposium is designed to promote discussion of airway clearance techniques for people with cystic fibrosis based on airway physiology and methods of choice. The first talk begins with an overview of lung physiology and mucociliary clearance, followed by a discussion of airway clearance techniques that do not require a device, such as percussion and vibration, active cycle of breathing techniques, autogenic drainage, and exercise. We will then discuss airway clearance techniques that require a device, such as PEP, Oscillating PEP, HFCWO/HFCC, and IPV. The final talk will discuss personalizing airway clearance techniques for people with cystic fibrosis based on their lung pathology.
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