Palliative Care of Respiratory Disease in Primary Care

  • Patrick WhiteEmail author
Reference work entry


The palliative care of advanced progressive respiratory disease in the setting of a primary care team is concerned mainly with chronic obstructive pulmonary disease (COPD), idiopathic pulmonary fibrosis (IPF), and cystic fibrosis (CF). COPD is the commonest of these but it is perhaps the most difficult to identify in its final stages. IPF has a trajectory and prognosis more akin to malignant disease. Though relatively rare in general practice, IPF is the disease among these three that has the most easily definable terminal stage, and so primary care teams should be alert to the palliative care needs of these patients.

CF is a remarkable disease because life expectancy at birth with CF has changed from childhood or teenage years to 30 years now, and for those aged 30 years, life expectancy is now in mid-50s. Like COPD, prognosis in CF is difficult to define. Most people with advanced disease in both groups are living with the disease. Most of these people want to continue living in the face of considerable challenges. The task in both of these diseases is to develop an approach to the amelioration of symptoms and to the support of patients and carers, that is, in keeping with the personal objectives of the patients. Information about treatment, future exacerbations, and the risk of dying is all important. Symptom control is difficult, and for intractable breathlessness, oral morphine has a role in many patients.


  1. Almagro PL, Calbo E, Ochoa de Echagüen A, Barreiro B, Quintana S, Heredia JL, Garau J. Mortality after hospitalization for COPD. Chest. 2002;121(5):1441–8.CrossRefGoogle Scholar
  2. Almagro P, Yun S, Sangil A, Rodríguez-Carballeira M, Marine M, Landete P, Soler-Cataluña JJ, Soriano JB, Miravitlles M. Palliative care and prognosis in COPD: a systematic review with a validation cohort. Int J Chron Obstruct Pulmon Dis. 2017;12:1721–9. eCollection 2017.CrossRefPubMedPubMedCentralGoogle Scholar
  3. British Lung Foundation. Chronic obstructive lung disease statistics [online]. 2018. Available at Accessed 2 Jan 2018.
  4. Connors AF Jr, Dawson NV, Thomas C, Harrell FE Jr, Desbiens N, Fulkerson WJ, et al. Outcomes following acute exacerbation of severe chronic obstructive lung disease. The SUPPORT investigators (Study to Understand Prognoses and Preferences for Outcomes and Risks of Treatments). Am J Respir Crit Care Med. 1996;154(4 Pt 1):959–67.CrossRefGoogle Scholar
  5. Currow DC, Abernethy AP, Allcroft P, Banzett RB, Bausewein C, Booth S, et al. The need to research refractory breathlessness. Eur Respir J. 2016;47(1):342–3.CrossRefGoogle Scholar
  6. Curtis JR, Engelberg EL, Nielsen DH, Patrick DL. Patient-physician communication about en-of-life for patients with severe COPD. Eur Respir J. 2004;24:200–205. Scholar
  7. Farquhar M. Assessing carer needs in chronic obstructive pulmonary disease. Chron Respir Dis. 2017. Scholar
  8. Farquhar MC, Prevost AT, McCrone P, Brafman-Price B, Bentley A, Higginson IJ, Todd CJ, Booth S. The clinical and cost effectiveness of a Breathlessness Intervention Service for patients with advanced non-malignant disease and their informal carers: mixed findings of a mixed method randomised controlled trial. Trials. 2016;17:185. Scholar
  9. Gore JM, Brophy CJ, Greenstone MA. How well do we care for patients with end stage chronic obstructive pulmonary disease (COPD)? A comparison of palliative care and quality of life in COPD and lung cancer. Thorax. 2000;55(12):1000–6.CrossRefGoogle Scholar
  10. Herigstad M, Faull OK, Hayen A, Evans E, Hardinge FM, Wiech K, et al. Treating breathlessness via the brain: changes in brain activity over a course of pulmonary rehabilitation. Eur Respir J. 2017;50(3):1701029. Scholar
  11. Higginson IJ, Bausewein C, Reilly CC, Gao W, Gysels M, Dzingina M, et al. An integrated palliative and respiratory care service for patients with advanced disease and refractory breathlessness: a randomised controlled trial. Lancet Respir Med. 2014;2(12):979–87.CrossRefGoogle Scholar
  12. Hutchinson J, Fogarty A, Hubbard R, et al. Global incidence and mortality of idiopathic pulmonary fibrosis: a systematic review. Eur Respir J. 2015;46:795–806.CrossRefGoogle Scholar
  13. Keogh RH, Szczesniak R, Taylor-Robinson D, Bilton D. Up-to-date and projected estimates of survival for people with cystic fibrosis using baseline characteristics: a longitudinal study using UK patient registry data. J Cyst Fibros. 2018. pii: S1569-1993(17)30976-1. [Epub ahead of print].CrossRefGoogle Scholar
  14. Maddocks M, Nolan CM, Man WD, Polkey MI, Hart N, Gao W, et al. Neuromuscular electrical stimulation to improve exercise capacity in patients with severe COPD: a randomised double-blind, placebo-controlled trial. Lancet Respir Med. 2016;4(1):27–36.CrossRefGoogle Scholar
  15. Maddocks M, Lovell N, Booth S, Man WD, Higginson IJ. Palliative care and management of troublesome symptoms for people with chronic obstructive pulmonary disease. Lancet. 2017;390(10098):988–1002.CrossRefGoogle Scholar
  16. McCarthy B, Casey D, Devane D, Murphy K, Murphy E, Lacasse Y. Pulmonary rehabilitation for chronic obstructive pulmonary disease. Cochrane Database Syst Rev. 2015;(2):CD003793. Review.
  17. Nathan SD, Albera C, Bradford WZ, et al. Effect of pirfenidone on mortality: pooled analyses and meta-analyses of clinical trials in idiopathic pulmonary fibrosis. Lancet Respir Med. 2017;5:33–41.CrossRefGoogle Scholar
  18. Navaratnam V, Fleming KM, West J, et al. The rising incidence of idiopathic pulmonary fibrosis in the UK. Thorax. 2011;66:462–7.CrossRefGoogle Scholar
  19. NICE (National Institute for Health and Care Excellence). Nintedanib for treating idiopathic pulmonary fibrosis (TA379). London: NICE; 2016.Google Scholar
  20. Pinnock H, Kendall M, Murray SA, Worth A, Levack P, Porter M, et al. Living and dying with severe chronic obstructive pulmonary disease: multi-perspective longitudinal qualitative study. BMJ. 2011;342:d142. Scholar
  21. Shaw J, Marshall T, Morris H, Hayton C, Chaudhuri N. Idiopathic pulmonary fibrosis: a holistic approach to disease management in the antifibrotic age. J Thorac Dis. 2017;9(11):4700–7. Scholar
  22. Smallwood N, Le B, Currow D, Irving L, Philip J. Management of refractory breathlessness with morphine in patients with chronic obstructive pulmonary disease. Intern Med J. 2015;45(9):898–904. Scholar
  23. White P, White S, Edmonds P, Gysels M, Moxham J, Seed P, Shipman C. Palliative care or end of life care in advanced COPD? A prospective community survey. Br J Gen Pract. 2011. Scholar
  24. White P, Thornton H, Pinnock H, Georgopoulou S, Booth H. Overtreatment of COPD with inhaled corticosteroids – implications for safety and costs: cross-observational study. PLoS One. 2013;8(10):e75221. Scholar

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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Primary Care Respiratory Medicine, School of Population Health and Environmental ScienceKing’s College LondonLondonUK

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