Live and let die: existential decision processes in a fatal disease

Abstract

Decisions and determinants of decisions to prolong or shorten life in the course of fatal diseases like ALS are poorly understood. Decisions and desire for hastened death of N = 93 ALS patients were investigated in a prospective longitudinal approach three times in the course of 1 year. Determinants of decisions were evaluated: quality of life (QoL), depression, feeling of being a burden, physical function, social support and cognitive status. More than half of patients had a positive attitude towards life-sustaining treatments and they had a low desire for hastened death. Of those with undecided or negative attitude, 10 % changed attitudes towards life-sustaining treatments in the course of 1 year. Patients’ desire to hasten death was low and decreased significantly within 1 year despite physical function decline. Those with a high desire for hastened death decided against invasive therapeutic treatments. QoL, depression and social support were not predictors for vital decisions and remained stable. Feeling of being a burden was a predictor for decisions against life-supporting treatments. Throughout physical function loss, decisions to prolong life are flexibly adapted while desire to shorten life declines. QoL was stable and not a predictor for vital decisions, even though anticipated low QoL has been reported to be the reason to request euthanasia. In contrast, feeling of being a burden in decision making needs more attention in clinical counselling. Considering a patient’s possible adaptation processes in the course of a fatal disease is necessary.

This is a preview of subscription content, access via your institution.

Fig. 1
Fig. 2
Fig. 3

References

  1. 1.

    Maessen M, Veldink JH, Onwuteaka-Philipsen BD, de Vries JM, Wokke JH, van der Wal G, van den Berg LH (2009) Trends and determinants of end-of-life practices in ALS in the Netherlands. Neurology 73(12):954–961

    CAS  Article  PubMed  Google Scholar 

  2. 2.

    Oliver DJ, Turner MR (2010) Some difficult decisions in ALS/MaND. Amyotroph Later Scler 11(4):339–343

    Article  Google Scholar 

  3. 3.

    Veldink JH, Wokke JH, van der Wal G, Vianney de Jong JM, van den Berg LH (2002) Euthanasia and physician-assisted suicide among patients with amyotrophic lateral sclerosis in The Netherlands. N Engl J Med 346:1638–1644

    Article  PubMed  Google Scholar 

  4. 4.

    Ganzini L, Block S (2002) Physician-assisted death—a last resort? N Engl J Med 346:1663–1665

    Article  PubMed  Google Scholar 

  5. 5.

    Smets T, Bilsen J, Cohen J, Rurup ML, Mortier F, Deliens L (2010) Reporting of euthanasia in medical practice in Flanders, Belgium: cross sectional analysis of reported and unreported cases. BMJ 341:c5174

    PubMed Central  Article  PubMed  Google Scholar 

  6. 6.

    Ganzini L, Johnston WS, Mcfarland BH, Tolle SW, Lee MA (1998) Attitudes of patients with amyotrophic lateral sclerosis and their care givers toward assisted suicide. N Engl J Med 339:967–973

    CAS  Article  PubMed  Google Scholar 

  7. 7.

    Ganzini L, Silveira MJ, Johnston WS (2002) Predictors and correlates of interest in assisted suicide in the final month of life among ALS patients in Oregon and Washington. J Pain Symptom Manage 24(3):312–317

    Article  PubMed  Google Scholar 

  8. 8.

    Ganzini L, Goy ER, Dobscha SK (2009) Oregonians’ reasons for requesting physician aid in dying. Arch Intern Med 169(5):489–492

    Article  PubMed  Google Scholar 

  9. 9.

    Kühnlein P, Kübler A, Raubold S, Worell M, Kurt A, Gdynia HJ, Sperfeld AD, Ludolph AC (2008) Palliative care and circumstances of dying in German ALS patients using non-invasive ventilation. Amyotroph Later Scler 9:91–98

    Article  Google Scholar 

  10. 10.

    Stutzki R, Schneider U, Reiter-Theil S, Weber M (2012) Attitudes toward assisted suicide and life-prolonging measures in Swiss ALS patients and their caregivers. Front Psychol 3:443

    PubMed Central  Article  PubMed  Google Scholar 

  11. 11.

    Hogden A, Greenfield D, Nugus P, Kiernan MC (2012) What influences patient decision-making in amyotrophic lateral sclerosis multidisciplinary care? A study of patient perspectives. Patient Prefer Adherence 6:819–838

    Google Scholar 

  12. 12.

    Burchardi N, Rauprich O, Hecht M, Beck M, Vollmann J (2005) Discussing living wills. A qualitative study of a German sample of neurologists and ALS patients. J Neurol Sci 237(1–2):67–74

    Article  PubMed  Google Scholar 

  13. 13.

    Robinson EM, Phipps M, Purtilo RB, Tsoumas A, Hamel-Nardozzi M (2006) Complexities in decision making for persons with disabilities nearing end of life. Top Stroke Rehabil 13(4):54–67

    Article  PubMed  Google Scholar 

  14. 14.

    Bach JR (2003) Threats to ”informed” advance directives for the severely physically challenged? Arch Phys Med Rehabil 84(4:2):23–28

    Article  Google Scholar 

  15. 15.

    Matuz T, Birbaumer N, Hautzinger M, Kübler A (2010) Coping with amyotrophic lateral sclerosis: an integrative view. J Neurol Neurosurg Psychiatry 81(8):893–898

    Article  PubMed  Google Scholar 

  16. 16.

    Kübler A, Winter S, Ludolph AC, Hautzinger M, Birbaumer N (2005) Severity of depressive symptoms and quality of life in patients with amyotrophic lateral sclerosis. Neurorehabil Neural Repair 19:182–193

    Article  PubMed  Google Scholar 

  17. 17.

    Lulé D, Häcker S, Ludolph A, Birbaumer N, Kübler A (2008) Depression and quality of life in patients with amyotrophic lateral sclerosis. Dtsch Arztebl Int 105(23):397–403

    PubMed Central  PubMed  Google Scholar 

  18. 18.

    Lulé D, Ehlich B, Lang D, Sorg S, Heimrath J, Kübler A, Birbaumer N, Ludolph AC (2013) Quality of life in fatal disease: the flawed judgement of the social environment. J Neurol 260(11):2836–2843

    Article  PubMed  Google Scholar 

  19. 19.

    Ganzini L, Goy ER, Dobscha SK (2008) Prevalence of depression and anxiety in patients requesting physicians’ aid in dying: cross sectional survey. BMJ 337:a1682

    PubMed Central  Article  PubMed  Google Scholar 

  20. 20.

    Singer PA, Martin DK, Kelner M (1999) Quality end-of-life care: patients’ perspectives. JAMA 281:163–168

    CAS  Article  PubMed  Google Scholar 

  21. 21.

    Phukan J, Elamin M, Bede P, Jordan N, Gallagher L, Byrne S, Lynch C, Pender N, Hardiman O (2012) The syndrome of cognitive impairment in amyotrophic lateral sclerosis: a population-based study. J Neurol Neurosurg Psychiatry 83(1):102–108

    Article  PubMed  Google Scholar 

  22. 22.

    Gordon PH, Salachas F, Lacomblez L, Le Forestier N, Pradat PF, Bruneteau G, Elbaz A, Meininger V (2012) Predicting survival of patients with amyotrophic lateral sclerosis at presentation: a 15-year experience. Neurodegener Dis 12(2):81–90

    Article  PubMed  Google Scholar 

  23. 23.

    Rowland LP, Shneider NA (2001) Amyotrophic lateral sclerosis. N Engl J Med 344:1688–1700

    CAS  Article  PubMed  Google Scholar 

  24. 24.

    Sullivan AD, Hedberg K, Fleming DW (2000) Legalized physician-assisted suicide in Oregon—the second year. N Engl J Med 342:598–604

    CAS  Article  PubMed  Google Scholar 

  25. 25.

    Cedarbaum JM, Stambler N, Malta E, Fuller C, Hilt D, Thurmond B, Nakanishi A (1999) The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III). J Neurol Sci 169(1–2):13–21

    CAS  Article  PubMed  Google Scholar 

  26. 26.

    Folstein MF, Folstein SE, McHugh PR (1975) Mini-mental state (a practical method for grading the state of patients for the clinician). J Psychiatr Res 12:189–198

    CAS  Article  PubMed  Google Scholar 

  27. 27.

    Rosenfeld B, Breitbart W, Stein K, Funesti-Esch J, Kaim M, Krivo S, Galietta M (1999) Measuring desire for death among the medically ill: the schedule of attitudes toward hastened death. Am J Psychiatry 156:94–100

    CAS  Article  PubMed  Google Scholar 

  28. 28.

    Bernheim JL (1999) How to get serious answers to the serious question: ‘How have you been?’: subjective quality of life (QOL) as an individual experiential emergent construct. Bioethics 13:272–287

    Article  PubMed  Google Scholar 

  29. 29.

    Hickey AM, Bury G, O’Boyle CA, Bradley F, O’Kelly FD, Shannon W (1996) A new short form individual quality of life measure (SEIQoL-DW): application in a cohort of individuals with HIV/AIDS. BMJ 313:29–33

    PubMed Central  CAS  Article  PubMed  Google Scholar 

  30. 30.

    Hautzinger M, Bailer M, Hofmeister D, Keller F (2012) ADS—Allgemeine Depressionsskala., Tests infoHogrefe, Göttingen

    Google Scholar 

  31. 31.

    Fydrich T, Sommer G, Tydecks T, Brähler E (2009) Fragebogen zur sozialen Unterstützung (F-SozU): Normierung der Kurzform (K-14)Social Support Questionnaire (F-SozU): Standardization of short form (K-14). Z Med Psychol 18:1

    Google Scholar 

  32. 32.

    Aschenbrenner A, Tucha O, Lange K (1999) RWT Regensburger Wortflüssigkeits-test., HandanweisungHogrefe, Göttingen

    Google Scholar 

  33. 33.

    Brickenkamp R (1962) Aufmerskamkeits-Belastungs-Test (d2). Hogrefe, Göttingen

    Google Scholar 

  34. 34.

    Lulé D, Heizmann C, Sorg S, Ludolph AC (2010) With the blink of an eye: d2 attention test in the severely paralysed. Amyotroph Lateral Scler 11(1):143–149

    Google Scholar 

  35. 35.

    Robbins R, Simmons Z, Bremer B, Walsh S, Fischer S (2001) Quality of life in ALS is maintained as physical function declines. Neurology 56:442–444

    CAS  Article  PubMed  Google Scholar 

  36. 36.

    Roach AR, Averill AJ, Segerstrom SC, Kasarskis EJ (2009) The dynamics of quality of life in ALS patients and caregivers. Ann Behav Med 37(2):197–206

    Article  PubMed  Google Scholar 

  37. 37.

    Czell D, Bauer M, Binek J, Schocjh OD, Weber M (2013) Outcome of percutaneous endoscopic gastrostomy insertion in respiratory impaired amyotrophic lateral sclerosis patients under non-invasive ventilation. Respir Care 58(5):838–844

    PubMed  Google Scholar 

  38. 38.

    Johnson JO, Sulmasy DP, Nolan MT (2007) Patients’ experiences of being a burden on family in terminal illness. J Hosp Palliat Nurs 9(5):264–269

    PubMed Central  Article  PubMed  Google Scholar 

  39. 39.

    McDonald ER, Hillel A, Wiedenfeld S (1996) Evaluation of the psychological status of ventilatory-supported patients with ALS/MND. Palliat Med 10:35–41

    CAS  Article  PubMed  Google Scholar 

  40. 40.

    Munroe CA, Sirdofsky MD, Kuru T, Anderson ED (2007) End-of-life decision making in 42 patients with amyotrophic lateral sclerosis. Respir Care 52(8):996–999

    PubMed  Google Scholar 

Download references

Acknowledgments

This is an EU Joint Programme—Neurodegenerative Disease Research (JPND) project. The project is supported through the following organisations under the aegis of JPND—http://www.jpnd.eu e.g. Germany, Bundesministerium für Bildung und Forschung (BMBF, FKZ), Sweden, Vetenskaprådet Sverige, and Poland, Narodowe Centrum Badán i Rozwoju (NCBR). This work was supported by the Deutsche Forschungsgemeinschaft (DFG 336/13-2 und BI 195/54-2) and the (BMBF #01GQ0831 and BMBF #01GM1103A).

Conflicts of interest

The authors declare that they have no conflict of interest.

Author information

Affiliations

Authors

Corresponding author

Correspondence to Dorothée Lulé.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Lulé, D., Nonnenmacher, S., Sorg, S. et al. Live and let die: existential decision processes in a fatal disease. J Neurol 261, 518–525 (2014). https://doi.org/10.1007/s00415-013-7229-z

Download citation

Keywords

  • Palliative care
  • Quality of life
  • Depression
  • Decision
  • Amyotrophic lateral sclerosis