Care of Individuals with Advanced Cystic Fibrosis Lung Disease

Publication Date: May 1, 2020
Last Updated: March 14, 2022

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When individuals with CF meet criteria for advanced CF lung disease, the CF Foundation recommends routine advance care planning conversations with them and their caregiver(s), including communication about prognosis and goals of care, documentation of advance directives, and decision-making surrounding lung transplantation.
The CF Foundation recommends that individuals with ACFLD undergo screening for hypoxemia on exertion and sleep, hypercarbia, and pulmonary hypertension.
The CF Foundation recommends supplemental oxygen for individuals with advanced CF lung disease and exercise induced or nocturnal hypoxemia.
The CF Foundation recommends consideration of nocturnal noninvasive ventilation for individuals with advanced CF lung disease and chronic hypercarbia.
The CF Foundation found insufficient evidence to make a recommendation regarding the use of pulmonary vasodilator therapy in individuals with advanced CF lung disease and pulmonary hypertension.
The CF Foundation recommends lung transplantation as a treatment option for individuals with advanced CF lung disease if congruent with goals of care.
The CF Foundation recommends that individuals with advanced CF lung disease and acute respiratory failure be considered eligible for intensive care unit management regardless of transplant status if congruent with goals of care.
The CF Foundation recommends that individuals with advanced CF lung disease and acute respiratory failure be considered for a trial of high flow nasal cannula oxygen and/or noninvasive ventilation.
For individuals with advanced CF lung disease and acute respiratory failure requiring invasive mechanical ventilation, the CF Foundation recommends consideration of early tracheostomy when anticipated need for mechanical ventilation is more than 5–7 days and support remains congruent with goals of care.
The CF Foundation recommends that individuals with advanced CF lung disease who develop refractory respiratory failure requiring invasive mechanical ventilation be considered for early transition to extracorporeal life support if congruent with goals of care.
For individuals with advanced CF lung disease, the CF Foundation recommends a trial of continuous alternating inhaled antibiotics as dictated by bacterial pathogens identified in respiratory culture.
The CF Foundation recommends that individuals with progressive advanced CF lung disease undergo screening for fungal pathogens in addition to standard microbiological screening.
The CF Foundation recommends that individuals with advanced CF lung disease participate in a pulmonary rehabilitation program.
The CF Foundation found insufficient evidence to make a recommendation regarding the use of systemic corticosteroids in individuals with advanced CF lung disease.
The CF Foundation found insufficient evidence to make a recommendation regarding routine screening for gastroesophageal reflux in individuals with advanced CF lung disease.
The CF Foundation recommends the use of enteral tube feeds for individuals with advanced CF lung disease and malnutrition after consideration of procedural risks versus benefits.
For individuals with advanced CF lung disease with frequent prior and continuing exposure to nephrotoxic and ototoxic agents, the CF Foundation recommends increased monitoring for accumulating toxicity.
The CF Foundation recommends that women with advanced CF lung disease contemplating pregnancy carefully consider the risks in consultation with high-risk obstetrics and CF providers.
For individuals with advanced CF lung disease with indications for opioids, the CF Foundation recommends treatment in accordance with established Center for Disease Control guidelines; this should include monitoring for adverse effects, and consultation with pain and/or palliative care specialists as appropriate.
For individuals with advanced CF lung disease and anxiety, the CF Foundation recommends management in accordance with the International Committee on Mental Health in Cystic Fibrosis: Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus statements for screening and treating depression and anxiety, reserving benzodiazepines for refractory symptoms or end of life symptom palliation.
When individuals with CF meet criteria for advanced CF lung disease, and with subsequent changes in clinical or social status, the CF Foundation recommends a formal care conference involving caregiver(s) and selected team members to develop a plan for ongoing psychosocial support.
In individuals with advanced CF lung disease, the CF Foundation recommends assessing the adequacy of financial resources at least biannually, and with changes in clinical or social status.
For pediatric patients with advanced CF lung disease nearing the age of transition to an adult CF care program, the CF Foundation recommends formally outlining a transition plan that provides flexibility in timing and coordination of transfer.

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Care of Individuals with Advanced Cystic Fibrosis Lung Disease

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