Evaluation of Pain, Dyspnea, and Goals of Care Among Adults With Cystic Fibrosis: A Comprehensive Palliative Care Survey.
BACKGROUND
Palliative care is increasingly important in the care of adults with cystic fibrosis (CF). Symptoms such as pain and dyspnea are prevalent, yet severity may be underestimated. Little information is available to describe patient preferences for end-of-life care (EOLC). The objective of this study was to describe patient perceptions about pain, dyspnea, and advance care planning.


METHODS
We developed a survey to assess pain, dyspnea, and EOLC in adults with CF. Questions were compiled and adapted from existing tools. The survey was administered to all patients in a single adult CF care center. Descriptive data were compiled as counts (proportions) and median (25th and 75th percentile). Mann Whitney U test was used to determine differences between individuals who experienced pain and dyspnea. A P value of .05 was utilized to determine significance.


RESULTS
Thirty-seven of 43 surveys were returned. Twenty-four percent reported chronic pain. Patients who reported pain with airway clearance had lower lung function (predicted forced expiratory volume in 1 [FEV1] 42% vs 65%, P < .05) and body mass index (19.6 vs 22.3, P < .05) than patients without pain. Those reporting dyspnea at rest had lower median FEV1 (28% vs 61%, P < .05). Patients with lower lung function are more likely to have considered end-of-life decisions (73% vs 31%, P < .05).


CONCLUSION
Pain and dyspnea are common among adults with CF. Few had an advance directive in place, but most are open to discussing EOLC issues. Results of this single-center study may not represent the entire population, thus a multicenter investigation should be pursued.
['Chen|Elaine|E|', 'Killeen|Kathryn M|KM|', 'Peterson|Sarah J|SJ|', 'Saulitis|Anna K|AK|', 'Balk|Robert A|RA|']
D000328:Adult D032722:Advance Care Planning D059350:Chronic Pain D003550:Cystic Fibrosis D004417:Dyspnea D005260:Female D006801:Humans D008297:Male D010146:Pain D010166:Palliative Care D010347:Patient Care Planning D011788:Quality of Life D012129:Respiratory Function Tests D012720:Severity of Illness Index D013727:Terminal Care
pain dyspnea goals of care adults cystic fibrosis palliative care quality of life