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

Abstract

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.

Alpha-1 antitrypsin for cystic fibrosis complicated by severe cytokinemic COVID-19

“…In discussions regarding the allocation of resources and ICU support during the COVID-19 pandemic, patients with significant CF lung disease are at risk of being overlooked. Current CF Foundation guidelines state that PWCF with advanced disease should be considered for ICU [22] . However, as concerns regarding ventilator shortages mount, PWCF risk being included in the same triage categories as older and less functional patients suffering from more common chronic pulmonary diseases [23] .…”

Lumacaftor/ivacaftor-associated health stabilisation in adults with severe cystic fibrosis

“…Given that malnutrition is associated with poor outcomes both pre-and post-lung transplantation, interventions to maintain and improve nutritional status are key components of the management of patients with severe lung disease (reference to be added CFF consensus guidelines on management of CF patients with end-stage lung disease. 30 Our findings suggest that FFM change was associated with reduced requirement for hospitalisation and parenteral antibiotics, although not necessarily associated with improvements in rates of pulmonary function decline. Although our real-world evaluation study was not designed to examine the mechanisms underpinning our observations and therefore cannot delineate the potential contribution of improvements in the anorectic/catabolic state to improvements in nutritional parameters, it does nevertheless suggest that these and other factors are likely to be important.…”

Non-invasive Ventilation for Children With Chronic Lung Disease

“…There are no validated criteria for timing and patient selection for the initiation of NIV, particularly in the pediatric population, despite the physiological benefits of NIV in CF patients. According to the Cystic Fibrosis Foundation recommendations, the presence of the symptoms related to hypercarbia, and a PCO 2 level ≥55 mmHg or; PaCO 2 50-54 mmHg and nocturnal desaturation, or, PaCO 2 50-54 mmHg and ≥2 hospitalizations in the preceding year for hypercarbic respiratory failure are the main indications for initiation of NIV (68). The European CF Society recommends NIV according to the patient's wishes for relief of dyspnea (69).…”

“…According to the CF Foundation recommendations, oxygen supplementation is recommended for patients with nocturnal hypoxemia, while NIV should be considered in cases with hypercarbia (68). Nocturnal implemented NIV can prevent the fall in minute ventilation from NREM to REM sleep in patients with CF (62).…”