Biomaterial Database

Cystic Fibrosis Foundation consensus guidelines for the care of individuals with advanced cystic fibrosis lung disease. 2020

Siddhartha G Kapnadak, and Emily Dimango, and Denis Hadjiliadis, and Sarah E Hempstead, and Erin Tallarico, and Joseph M Pilewski, and Albert Faro, and James Albright, and Christian Benden, and Shaina Blair, and Elisabeth P Dellon, and Daniel Gochenour, and Peter Michelson, and Baharak Moshiree, and Isabel Neuringer, and Carl Riedy, and Teresa Schindler, and Lianne G Singer, and Dave Young, and Lauren Vignola, and Joan Zukosky, and Richard H Simon

Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine, University of Washington, Seattle, WA, USA. Electronic address: skap@uw.edu.

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. 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. 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. These recommendations are intended to be paired with previously published management guidelines for the overall CF population, with the objective of reducing practice variability and improving overall care, quality of life, and survival in those with ACFLD.

UI	MeSH Term	Description	Entries
D010166	Palliative Care	Care alleviating symptoms without curing the underlying disease. (Stedman, 25th ed)	Palliative Treatment,Palliative Supportive Care,Palliative Surgery,Palliative Therapy,Surgery, Palliative,Therapy, Palliative,Care, Palliative,Palliative Treatments,Supportive Care, Palliative,Treatment, Palliative,Treatments, Palliative
D010346	Patient Care Management	Generating, planning, organizing, and administering medical and nursing care and services for patients.	Care Management, Patient,Management, Patient Care
D011788	Quality of Life	A generic concept reflecting concern with the modification and enhancement of life attributes, e.g., physical, political, moral, social environment as well as health and disease.	HRQOL,Health-Related Quality Of Life,Life Quality,Health Related Quality Of Life
D003422	Critical Care	Health care provided to a critically ill patient during a medical emergency or crisis.	Intensive Care,Intensive Care, Surgical,Surgical Intensive Care,Care, Critical,Care, Intensive,Care, Surgical Intensive
D003550	Cystic Fibrosis	An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION.	Mucoviscidosis,Cystic Fibrosis of Pancreas,Fibrocystic Disease of Pancreas,Pancreatic Cystic Fibrosis,Pulmonary Cystic Fibrosis,Cystic Fibrosis, Pancreatic,Cystic Fibrosis, Pulmonary,Fibrosis, Cystic,Pancreas Fibrocystic Disease,Pancreas Fibrocystic Diseases
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000083626	Psychosocial Intervention	Psychological and social interventions by providers including psychologists, psychiatrists, social workers, counselors/therapists, primary care and other nonpsychiatric physicians, nurses, physical and occupational therapists, religious leaders, lay and peer providers, paraprofessionals and caregivers, and automated providers (e.g. internet/audio/video-delivered interventions). Combinations of provider options are sometimes used.	Psychological Intervention,Intervention, Psychological,Intervention, Psychosocial,Interventions, Psychological,Interventions, Psychosocial,Psychological Interventions,Psychosocial Interventions
D012720	Severity of Illness Index	Levels within a diagnostic group which are established by various measurement criteria applied to the seriousness of a patient's disorder.	Illness Index Severities,Illness Index Severity
D015897	Comorbidity	The presence of co-existing or additional diseases with reference to an initial diagnosis or with reference to the index condition that is the subject of study. Comorbidity may affect the ability of affected individuals to function and also their survival; it may be used as a prognostic indicator for length of hospital stay, cost factors, and outcome or survival.
D016040	Lung Transplantation	The transference of either one or both of the lungs from one human or animal to another.	Grafting, Lung,Transplantation, Lung,Graftings, Lung,Lung Grafting,Lung Graftings,Lung Transplantations,Transplantations, Lung