title: Evaluation of Pain, Dyspnea, and Goals of Care Among Adults With Cystic Fibrosis: A Comprehensive Palliative Care Survey.
abstract: 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).
  
  
  CONCLUSIONS
  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.
authors: 1 Adult Cystic Fibrosis Center, Pulmonary and Critical Care Medicine, Rush University Medical Center, Chicago, IL, USA.
authors: Elaine
authors: E
authors: Chen
authors: 1 Adult Cystic Fibrosis Center, Pulmonary and Critical Care Medicine, Rush University Medical Center, Chicago, IL, USA.
authors: Kathryn M
authors: KM
authors: Killeen
authors: 1 Adult Cystic Fibrosis Center, Pulmonary and Critical Care Medicine, Rush University Medical Center, Chicago, IL, USA.
authors: Sarah J
authors: SJ
authors: Peterson
authors: 1 Adult Cystic Fibrosis Center, Pulmonary and Critical Care Medicine, Rush University Medical Center, Chicago, IL, USA.
authors: Anna K
authors: AK
authors: Saulitis
authors: 1 Adult Cystic Fibrosis Center, Pulmonary and Critical Care Medicine, Rush University Medical Center, Chicago, IL, USA.
authors: Robert A
authors: RA
authors: Balk
mesh_terms: D000328:Adult; D032722:Advance Care Planning; D059350:Chronic Pain / Q000209:etiology / Q000523:psychology; D003550:Cystic Fibrosis / Q000150:complications / Q000523:psychology*; D004417:Dyspnea / Q000175:diagnosis / Q000209:etiology / Q000523:psychology*; D005260:Female; D006801:Humans; D008297:Male; D010146:Pain / Q000175:diagnosis / Q000209:etiology / Q000523:psychology*; D010166:Palliative Care / Q000458:organization & administration / Q000523:psychology*; D010347:Patient Care Planning / Q000458:organization & administration; D011788:Quality of Life; D012129:Respiratory Function Tests; D012720:Severity of Illness Index; D013727:Terminal Care / Q000458:organization & administration / Q000523:psychology
keywords: adults; cystic fibrosis; dyspnea; goals of care; pain; palliative care; quality of life