Skip to main content

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic disease of unknown etiology characterized by a deterioration of the structure of the lung parenchyma resulting in a severe and progressive decline of respiratory function and early mortality. The median overall survival of patients with IPF varies from 2 to 7 years in several studies, which is comparable to many malignant diseases. The combination of poor prognosis, the uncertainty of the course of the disease, and the severe burden of symptoms heavily affect the quality of life of patients and their families. The progress of the disease is unpredictable, some patients have periods prolonged by slow and progressive decline, and others instead have sometimes fatal exacerbations. By virtue of this varying trend, physicians, patients, and caregivers often face high levels of stress and anxiety, and this makes patients with IPF a group to be entrusted early to palliative care, this becomes even more important in patients who are not candidates for lung transplantation. Most patients with IPF died in hospital, with ongoing life-prolonging procedures until death. Frequent use of opioids is an indicator of intention to relieve symptoms, but end-of-life decisions are still made very late. Early integrated palliative care with advanced care plan could improve end-of-life care (EoL) in patients with end-stage IPF.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 69.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 89.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 119.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788–824.

    Article  PubMed  PubMed Central  Google Scholar 

  2. Richeldi L, du Bois RM, Raghu G, Azuma A, Brown KK, Costabel U, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2071–82.

    Article  PubMed  CAS  Google Scholar 

  3. Lindell KO, Liang Z, Hoffman LA, Rosenzweig MQ, et al. Palliative care and location of death in decedents with idiopathic pulmonary fibrosis. Chest. 2015;147(2):423–9.

    Article  PubMed  Google Scholar 

  4. Danoff SK, Schonhoft EH. Role of support measures and palliative care. Curr Opin Pulm Med. 2013;19(5):480–4.

    Article  PubMed  Google Scholar 

  5. Lee JS, McLaughlin S, Collard HR. Comprehensive care of the patient with idiopathic pulmonary fibrosis. Curr Opin Pulm Med. 2011;17(5):348–54.

    Article  PubMed  Google Scholar 

  6. Prendergast TJ, Puntillo KA. Withdrawal of life support. Intensive caring at the end of life. JAMA. 2002;288:2732–40.

    Article  PubMed  Google Scholar 

  7. WHO. WHO definition of palliative care. http://www.who.int/cancer/palliative/definition/en/. Accessed 1 June 2017.

  8. Bonella F, Wijsenbeek M, Molina-Molina M, et al. European IPF patient charter: unmet needs and a call to action for healthcare policymakers. Eur Respir J. 2016;47:597–606.

    Article  PubMed  Google Scholar 

  9. Temel JS, Greer JA, Muzikansky A, Gallenger ER, et al. Early palliative care for patients with metastatic non–small-cell lung cancer. N Engl J Med. 2010;363:733–42.

    Article  CAS  PubMed  Google Scholar 

  10. Veigh CM, Reid J, Larkin P, Porter S, Hudson P. The provision of generalist and specialist palliative care for patients with nonmalignant respiratory disease in the North and Republic of Ireland: a qualitative study. BMC Palliat Care. 2018;17:69.

    Article  Google Scholar 

  11. Bajwah S, Higginson IJ, Ross JR, et al. The palliative care needs for fibrotic interstitial lung disease: a qualitative study of patients, informal caregivers and health professionals. Palliat Med. 2013;27:869–76.

    Article  PubMed  Google Scholar 

  12. Sampson C, Gill BH, Harrison NK, Nelson A, Byrne A. The care needs of patients with idiopathic pulmonary fibrosis and their carers (CaNoPy): results of a qualitative study. BMC Pulm Med. 2015;15:155.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Rinnenburger DE, Alma MG, Bigioni D, Brunetti G, Liberati C, Magliacani V, Monaco G, Reggiani L, Taronna G, Cecchini L. End-of- life decision making in respiratory failure. The therapeutic choices in chronic respiratory failure in a 7-item questionnaire. Ann Ist Super Sanità. 2012;48(3):328–33.

    Article  PubMed  Google Scholar 

  14. Quality standards for end of life care of adults. National Institute for Health and Clinical Excellence (NICE) 12/2011.

    Google Scholar 

  15. Overgaard D, Kaldan G, Marsaa K, Nielsen TL, Shaker SB, Egerod I. The lived experience with idiopathic pulmonary fibrosis: a qualitative study. Eur Respir J. 2016;47:1324–6.

    Article  Google Scholar 

  16. Miccinesi G, Crocetti E, Morino P, Fallai M, Piazza M, Cavallini V, et al. Palliative home care reduces time spent in hospital wards: a population based study in the Tuscany Region, Italy. Cancer Causes Control. 2003;14(10):971–7.

    Article  PubMed  Google Scholar 

  17. Navaratnam V, Fogarty A, Glendening R, McKeever T, Hubbard RB. The increasing secondary care burden of idiopathic pulmonary fibrosis: hospital admission trends in England from 1998 to 2010. Chest. 2013;143:1078–84.

    Article  PubMed  Google Scholar 

  18. Collard HR, Ward AJ, Lanes S, Cortney Hayflinger D, Rosenberg DM, Hunsche E. Burden of illness in idiopathic pulmonary fibrosis. J Med Econ. 2012;15:829–35.

    Article  PubMed  Google Scholar 

  19. Stern JB, Mal H, Groussard O, et al. Prognosis of patients with advanced idiopathic pulmonary fibrosis requiring mechanical ventilation for acute respiratory failure. Chest. 2001;120:213–9.

    Article  CAS  PubMed  Google Scholar 

  20. Mollica C, Paone G, Conti V, et al. Mechanical ventilation in patients with end-stage idiopathic pulmonary fibrosis. Respiration. 2010;79:209–15.

    Article  PubMed  Google Scholar 

  21. van Manen MJ, Geelhoed JJ, Tak NC, Wijsenbeek MS. Optimizing quality of life in patients with idiopathic pulmonary fibrosis. Ther Adv Respir Dis. 2017;11(3):157–69.

    Article  PubMed  PubMed Central  Google Scholar 

  22. Kreuter M, Bendsrup E, Russel AM, et al. Palliative care in interstitial lung disease: living in well. Lancet Respir Med. 2017;5(12):968–80.

    Article  PubMed  Google Scholar 

  23. Emanuel EJ, Onwuteaka-Philipsen BD, Urwin JW, et al. Attitudes and practices of euthanasia and physician-assisted suicide in the United States, Canada and Europe. JAMA. 2016;316:79–90.

    Article  CAS  PubMed  Google Scholar 

  24. Supplement to: Kreuter M, Bendstrup E, Russell M. Palliative care in interstitial lung disease: living well. Lancet Respir Med. 2017;5(12):968–80. https://doi.org/10.1016/S2213-2600(17)30383-1.

    Article  PubMed  Google Scholar 

  25. Engelhardt HT Jr, Iltis AS. End-of-life: the traditional Christian view. Lancet. 2005;366(9490):1045–9.

    Article  PubMed  Google Scholar 

  26. Rajala K, Lehto JT, Saarinen M, Sutinen E, Saarto T, Myllärniemi M. End-of-life care of patients with idiopathic pulmonary fibrosis. BMC Palliat Care. 2016;15:85.

    Article  PubMed  PubMed Central  Google Scholar 

  27. Lynn J. Serving patients who may die soon and their families. JAMA. 2001;285:925–32.

    Article  CAS  PubMed  Google Scholar 

  28. Gruppo di Studio Bioetica SIAARTI Grandi insufficienze d’organo “end stage”: cure intensive o cure palliative? “documento condiviso” per una pianificazione delle scelte di cura Documento approvato dal Consiglio Direttivo di Società Italiana Anestesia Analgesia Rianimazione Terapia Intensiva (SIAARTI) 22 aprile 2013.

    Google Scholar 

  29. SIAARTI—Italian Society of Anaesthesia Analgesia Resuscitation and Intensive Care Bioethical Board. End-of-life care and the intensivist: Italian Society of Anaesthesia Analgesia and Intensive Care Medicine (SIAARTI) recommendations on the management of the dying patient. Minerva Anestesiol. 2006;72:927–63.

    Google Scholar 

  30. Curtis JR, White DB. Practical guidance for evidence-based ICU family conferences. Chest. 2008;134:835–43.

    Article  PubMed  Google Scholar 

  31. Gruppo di Studio Bioetica SIAARTI. End-of-life care and the intensivist: Italian Society of Anaestesia analgesia and Intensive Care Medicine (SIAARTI). Recommendations on the management of the dying patient. Minerva Anestesiol. 2006;72:927–63.

    Google Scholar 

  32. Dichiarazioni Anticipate di Trattamento (DAT). http://www.governo.it/bioetica/testi/Dichiarazioni_anticipate_trattamento.pdf. Accessed Feb 2013.

  33. Silveira MJ, Kim SYH, Langa KM. Advance directives and outcomes of surrogate decision making before death. N Engl J Med. 2010;362:1211–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  34. Gabler NB, Cooney E, Small DS, et al. Default options in advance directives: study protocol for a randomised clinical trial. BMJ Open. 2016;6:e010628.

    Article  PubMed  PubMed Central  Google Scholar 

  35. Pirinea H, Simunich T, Wehner D, Ashurst J. Patient and health-care provider interpretation of do not resuscitate and do not intubate. Indian J Palliat Care. 2016;22(4):432–6.

    Article  PubMed  PubMed Central  Google Scholar 

  36. Breu AC, Herzig SJ. Differentiating DNI from DNR: combating code status conflation. J Hosp Med. 2014;9(10):669–70. https://doi.org/10.1002/jhm.2234.

    Article  PubMed  Google Scholar 

  37. Nava S, Sturani C, Hartl S, et al. End-of-life decision-making in respiratory intermediate care units: a European survey. Eur Respir J. 2007;30:1442–50.

    Article  Google Scholar 

  38. Chakrabarti B, Sulaiman CB, Davies L, et al. A study of patient attitudes in the United Kingdom toward ventilator support in chronic obstructive pulmonary disease. J Palliat Med. 2009;12:1029–35.

    Article  PubMed  Google Scholar 

  39. Simonds AK. Ethics and decision making in end stage lung disease. Thorax. 2003;58:272–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  40. Creagh-Brown BC, Shee CD. Noninvasive ventilation as a ceiling of therapy in endstage chronic obstructive pulmonary disease. Chron Respir Dis. 2008;5:143–8.

    Article  PubMed  Google Scholar 

  41. Lee AS, et al. The burden of idiopathic pulmonary fibrosis: an unmet public health need. Respir Med. 2014;108:955–67.

    Article  PubMed  Google Scholar 

  42. Ryerson CJ, Donesky D, Pantilat SZ, Collard HR. Dyspnea in idiopathic pulmonary fibrosis: a systematic review. J Pain Symptom Manag. 2012;43(4):771–82.

    Article  Google Scholar 

  43. Bajwah S. Specialist palliative care is more than drugs: a retrospective study of ILD patients. Lung. 2012;190(2):215–20.

    Article  PubMed  Google Scholar 

  44. Romayne Gallagher MD. The use of opioids for dyspnea in advanced disease. CMAJ. 2011;183(10):1170.

    Article  PubMed  PubMed Central  Google Scholar 

  45. Clemens KE, Quednau I, Klaschik E. Is there a higher risk of respiratory depression in opioid naive palliative care patients during symptomatic therapy of dyspnea with strong opioids? J Palliat Med. 2008;11:204–16.

    Article  PubMed  Google Scholar 

  46. Lewis D, Scullion J. Palliative and endof- life care for patients with idiopathic pulmonary fibrosis: challenges and dilemmas. Int J Palliat Nurs. 2012;18(7):331–7.

    Article  PubMed  Google Scholar 

  47. Lindell KO, Olshansky E, Song MK, et al. Impact of a disease-management program on symptom burden and health related quality of life in patients with idiopathic pulmonary fibrosis and their care partners. Heart Lung. 2010;39(4):304–13.

    Article  PubMed  Google Scholar 

  48. Ahmadi Z, Wysham NG, Lundström S, Janson C, Currow DC, Ekström M. End-of-life care in oxygen-dependent ILD compared with lung cancer: a national population-based study. Thorax. 2016;71(6):510–6. https://doi.org/10.1136/-thoraxjnl-2015-207439.

    Article  PubMed  Google Scholar 

  49. Scelfo C, Caminati A, Harari S. Recent advances in managing idiopathic pulmonary fibrosis [version 1; referees: 4 approved]. F1000Res. 2017;6(F1000 Faculty Rev):2052. https://doi.org/10.12688/f1000research.10720.1.

    Article  PubMed  PubMed Central  Google Scholar 

  50. Lightowler JV, Fau WJ, Fau EM, Ram FS. Non-invasive positive pressure ventilation to treat respiratory failure resulting from exacerbations of chronic obstructive pulmonary disease: Cochrane systematic review and meta-analysis. BMJ. 2003;326(7382):185.

    Article  PubMed  PubMed Central  Google Scholar 

  51. Nava S, Ferrer MF, Esquinas AF, Scala RF, Groff PF, Cosentini RF, et al. Palliative use of non-invasive ventilation in end-of-life patients with solid tumours: a randomised feasibility trial. Lancet Oncol. 2013;14:219–27.

    Article  PubMed  Google Scholar 

  52. Gaudry S, Vincent F, Rabbat A, et al. Invasive mechanical ventilation in patients with fibrosing interstitial pneumonia. J Thorac Cardiovasc Surg. 2014;147(1):47–53.

    Article  PubMed  Google Scholar 

  53. Rush B, Wiskar K, Berger L, Griesdale D. The use of mechanical ventilation in patients with idiopathic pulmonary fibrosis in the United States: a nationwide retrospective cohort analysis. Respir Med. 2016;111:72–6.

    Article  PubMed  Google Scholar 

  54. Shah N, Mehta Z, Mehta Y. High-flow nasal cannula oxygen therapy in palliative care. J Palliat Med. 2017;20:679–80.

    Article  PubMed  Google Scholar 

  55. Frat JP, Thille AW, Mercat A, et al. High-flow oxygen through nasal cannula in acute hypoxemic respiratory failure. N Engl J Med. 2015;372:2185–96.

    Article  CAS  PubMed  Google Scholar 

  56. Steve GP, Steven RH, Gay PC. High-flow nasal cannula therapy in do-not-intubate patients with hypoxemic respiratory distress. Respir Care. 2013;58(4):597–600.

    Google Scholar 

  57. Holland AE, Dowman L, Fiore J, Brazzale D, Hill CJ, McDonald CF. Cardiorespiratory responses to 6-minute walk test in interstitial lung disease: not always a submaximal test. BMC Pulm Med. 2014;14:136.

    Article  PubMed  PubMed Central  Google Scholar 

  58. Kenn K, Gloecki R, Behr J. Pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis—a review. Respiration. 2013;86:89–99.

    Article  CAS  PubMed  Google Scholar 

  59. Ryerson CJ, Cayou C, Topp F, et al. Pulmonary rehabilitation improves long-term outcomes in interstitial lung disease: a prospective cohort study. Respir Med. 2014;108:203–10.

    Article  PubMed  Google Scholar 

  60. Egan JJ. Follow-up and nonpharmacological management of the idiopathic pulmonary fibrosis patient. Eur Respir Rev. 2011;20:114–7.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2020 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Micco, A., Di Sorbo, A., Del Donno, M. (2020). End-of-Life Care of Patients with Idiopathic Pulmonary Fibrosis. In: Esquinas, A., Vargas, N. (eds) Ventilatory Support and Oxygen Therapy in Elder, Palliative and End-of-Life Care Patients . Springer, Cham. https://doi.org/10.1007/978-3-030-26664-6_33

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-26664-6_33

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-26663-9

  • Online ISBN: 978-3-030-26664-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics