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).

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.

Keywords

cystic fibrosis palliative care adults quality of life pain dyspnea goals of care

Get full access to this article

View all access options for this article.

References

Robinson

. Palliative care in cystic fibrosis. J Pall Med. 2000;3(2):187–192.

Mitchell

Nakielna

Tullis

Adair

. Cystic fibrosis: end-stage care in Canada. Chest. 2000;118(1):80–84.

Robinson

. Palliative and end-of-life care in cystic fibrosis: what we know and what we need to know. Curr Opin Pulm Med. 2009;15(6):621–625. doi:10.1097/MCP.0b013e3283304c29.

Fitzsimmons

. The changing epidemiology of cystic fibrosis. J Pediatr. 1993;122(1):1–9.

Cystic Fibrosis Foundation Patient Registry. 2013 Annual Data Report to the Center Directors. Bethesda, MD: CF Foundation; 2014.

http://www.who.int/cancer/palliative/definition/en/.

Stenekes

Hughes

Grégoire

Frager

Robinson

McGrath

. Frequency and self-management of pain, dyspnea, and cough in cystic fibrosis. J Pain Symptom Manage. 2009;38(6):837–848. doi:10.1016/j.jpainsymman.2009.04.029.

Selecky

Eliasson

Hall

Schneider

Varkey

McCaffree

; American College of Chest Physicians. Palliative and end-of-life care for patients with cardiopulmonary diseases: American College of Chest Physicians position statement. Chest. 2005;128(5):3599–3610. doi:10.1378/chest.128.5.3599.

Sawicki

Dill

Asher

Sellers

Robinson

. Advance care planning in adults with cystic fibrosis. J Pall Med. 2008;11(8):1135–1141. doi:10.1089/jpm.2008.0051.

10.

Sawicki

Sellers

Robinson

. Self-reported physical and psychological symptom burden in adults with cystic fibrosis. J Pain Symptom Manag. 2008;35(4):372–380. doi:10.1016/j.jpainsymman.2007.06.005.

11.

Goss

Edwards

Ramsey

Aitken

Patrick

. Patient-reported respiratory symptoms in cystic fibrosis. J Cyst Fibros. 2009;8(4):245–252. doi:10.1016/j.jcf.2009.04.003.

12.

Festini

Ballarin

Codamo

Doro

Loganes

. Prevalence of pain in adults with cystic fibrosis. J Cyst Fibros. 2004;3(1):51–57. doi:10.1016/j.jcf.2003.12.001.

13.

Havermans

Colpaert

De Boeck

Dupont

Abbott

. Pain in CF: review of the literature. J Cyst Fibros. 2013;12(5):423–430. doi:10.1016/j.jcf.2013.04.001.

14.

Hayes

Yaster

Haythornthwaite

. Pain is a common problem affecting clinical outcomes in adults with cystic fibrosis. Chest. 2011;140(6):1598–1603. doi:10.1378/chest.11-0132.

15.

Dellon

Shores

Nelson

Wolfe

Noah

Hanson

. Family caregiver perspectives on symptoms and treatments for patients dying from complications of cystic fibrosis. J Pain Symptom Manage. 2010;40(6):829–837. doi:10.1016/j.jpainsymman.2010.03.024.

16.

Braithwaite

Philip

Tranberg

. End of life care in CF: Patients, families and staff experiences and unmet needs. J Cyst Fibros. 2011;10(4):253–257. doi:10.1016/j.jcf.2011.03.002.

17.

Sands

Repetto

Dupont

Korzeniewska-Eksterowicz

Catastini

Madge

. End of life care for patients with cystic fibrosis. J Cyst Fibros. 2011;10(suppl 2):s37–s44. doi:10.1016/S1569-1993(11)60007-6.

18.

Tan

Jensen

Thornby

Shanti

. Validation of the brief pain inventory for chronic nonmalignant pain. J Pain. 2004;2(2):133–137. doi:10.1016/j.jpain.2003.12.005.

19.

Kendrick

Baxi

Smith

. Usefulness of the modified 0-10 Borg scale in assessing the degree of dyspnea in patients with COPD and asthma. J Emerg Nurs. 2000;26(3):216–222. doi:10.1067/men.2000.107012.

20.

Dellon

Chen

Goggin

. Advance care planning in cystic fibrosis: current practices, challenges, and opportunities. J Cyst Fibros. 2016;15:96–101. doi:10.1016/j.jcf.2015.08.004.

21.

Yankaskas

Marshall

Sufian

Simon

Rodman

. Cystic Fibrosis adult care: consensus conference report. Chest. 2004;125(1 suppl):1s–39s. doi:10.1378/chest.125.1_suppl.1 S.

22.

Orens

Garrity

. General overview of lung transplantation and review of organ allocation. Proc Am Thorac Soc. 2009;6(1):13–19. doi:10.1513/pats.200807-072GO.

23.

Colman

Curtis

Nelson

. Barriers to optimal palliative care of lung transplant candidates. Chest. 2013;143(3):736–743. doi:10.1378/chest.12-0830.

24.

Linnemann

O’Malley

Friedman

. Development and evaluation of a palliative care curriculum for cystic fibrosis healthcare providers. J Cyst Fibros. 2016;15:90–95. doi:10.1016/j.jcf.2015.03.005.