Opportunities to Improve Utilization of Palliative Care among Adults with Cystic Fibrosis: A Systematic Review.

Individuals with Cystic Fibrosis (CF) frequently survive into adulthood and many have multi-faceted symptoms that impair quality of life.We conducted a systematic review to investigate opportunities to improve utilization of palliative care among adults with CF.We searched PubMed, Embase, Scopus, Web of Science and CINAHL databases from inception until September 27, 2018, and reviewed references manually. Eligible articles were published in English, involved adults age 18 years and older with CF and contained original data regarding patient outcomes related to presence of advanced care planning (ACP), symptom experience, and preferred and/or received end-of-life care.We screened 652 article abstracts and 32 full text articles; 12 studies met inclusion criteria. All studies were published between 2000 and 2018. Pertinent findings include that while 43% to 65% of adults with CF had contemplated completing ACP, the majority only completed ACP during their terminal hospital admission. Patients also reported high prevalence of untreated symptoms, with adequate symptom control reported in 45% among those with dyspnea, 22% among those with pain and 51% among those with anxiety and/or depression. Prevalence of in-hospital death ranged from 62% to 100%, with a third dying in the intensive care unit (ICU). The majority received antibiotics and preventative treatments during their terminal hospitalization. Finally, treatment from a palliative care specialist was associated with a higher prevalence of patient completion of advanced directives, decreased likelihood of in-ICU death and decreased use of mechanical ventilation at end-of-life.Adults with CF often have untreated symptoms and many opportunities exist for palliative care specialists to improve ACP completion and quality of end-of-life care.