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Therapeutic decisions in ALS patients: cross-cultural differences and clinical implications

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Abstract

Objective

Quantitative analysis of decision-making on therapeutic options in different sociocultural context in amyotrophic lateral sclerosis (ALS).

Methods

ALS patients (n = 244) were consecutively recruited in Germany (n = 83), Poland (n = 83), and Sweden (n = 78) in a prospective cross-cultural study (www.NEEDSinALS.com). They were interviewed on preferences for therapeutic techniques including invasive (IV) and non-invasive ventilation (NIV), as well as percutaneous endoscopic gastrostomy (PEG) and on hypothetical termination of these using quantitative questions. Using standardized questionnaires, religiousness, personal values, quality of life, and depressiveness were assessed.

Results

NIV was most frequently used in Germany and PEG in Sweden. Swedish patients were most liberal on initiation and termination of PEG, NIV and IV. Polish patients were mostly undecided and were least likely to consider discontinuing supportive management. Current use was partly associated with age, gender and state of physical function; also, financial support explained some variance. Future preferences on therapeutic options from the patient’s perspective were also closely associated with cultural factors. The more oriented towards traditional and conservative values, the less likely patients were to decide for invasive therapeutic devices (IV, PEG), the least likely to have ideations to discontinue any device and the more likely to have an undecided attitude.

Conclusions

Current use of therapeutic options is determined by medical condition in analogy to clinical guidelines. For future considerations, other factors such as cultural background are crucial, yielding hurdles to be regarded in the implementation of advanced directives in a multicultural environment.

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Acknowledgements

This is an EU Joint Programme—Neurodegenerative Disease Research (JPND; 01ED1405) project. The project is supported through the following organizations under the aegis of JPND—www.jpnd.eu, e.g., Germany, Bundesministerium für Bildung und Forschung (BMBF, FKZ), Sweden, Vetenskapr å det Sverige, and Poland, Narodowe Centrum Badan i Rozwoju (NCBR). This work was additionally supported by the Deutsche Forschungsgemeinschaft (LU 336/13-2 BI 195/54-2) and the BMBF (#01GM1103A). The authors thank all patients for participating in this study.

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Author notes

  1. Peter M. Andersen and Magdalena Kuzma-Kozakiewicz have contributed equally.

Authors and Affiliations

  1. Institute of Pharmacology and Clinical Neuroscience, Umeå University, Umeå, Sweden

    Peter M. Andersen, Erica Stenberg & Ann-Cristin Häggström

  2. Department of Neurology, Medical University of Warsaw, Warsaw, Poland

    Magdalena Kuzma-Kozakiewicz, Katarzyna Ciecwierska & Natalia Szejko

  3. Neurodegenerative Diseases Research Group, Medical University of Warsaw, Warsaw, Poland

    Magdalena Kuzma-Kozakiewicz

  4. Department of Neurology, University of Ulm, Ulm, Germany

    Jürgen Keller, Helena E. A. Aho-Oezhan, Cynthia Vázquez, Sarah Böhm, Johannes Dorst, Ingo Uttner, Albert C. Ludolph & Dorothée Lulé

  5. Department of History and Ethics of Medicine, University of Ulm, Ulm, Germany

    Gisela Badura-Lotter

  6. Department of Neurology, Charité CVK, Berlin, Germany

    Thomas Meyer

  7. Department of Neurology, Hannover Medical School, Hannover, Germany

    Susanne Petri

  8. Department of Neurology, Technische Universität Dresden, Germany and German Center for Neurodegenerative Diseases (DZNE), Dresden, Germany

    Katharina Linse & Andreas Hermann

  9. Department of Clinical Science, Umeå University, Umeå, Sweden

    Olof Semb & Simona Nackberg

Authors
  1. Peter M. Andersen
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  2. Magdalena Kuzma-Kozakiewicz
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  3. Jürgen Keller
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  4. Helena E. A. Aho-Oezhan
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  5. Katarzyna Ciecwierska
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  7. Cynthia Vázquez
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  8. Sarah Böhm
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  9. Gisela Badura-Lotter
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  10. Thomas Meyer
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  11. Susanne Petri
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  12. Katharina Linse
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  13. Andreas Hermann
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  14. Olof Semb
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  15. Erica Stenberg
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  16. Simona Nackberg
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  17. Johannes Dorst
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  18. Ingo Uttner
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  19. Ann-Cristin Häggström
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  20. Albert C. Ludolph
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  21. Dorothée Lulé
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Corresponding author

Correspondence to Albert C. Ludolph.

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Conflicts of interest

None.

Ethical standards

The study was approved by the medical ethical review boards of the participating medical clinics in Ulm, Warsaw and Umea in accordance with the ethical standards of the 1964 Declaration of Helsinki and its later amendments.

Informed consent

All subjects gave their written informed consent.

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Andersen, P.M., Kuzma-Kozakiewicz, M., Keller, J. et al. Therapeutic decisions in ALS patients: cross-cultural differences and clinical implications. J Neurol 265, 1600–1606 (2018). https://doi.org/10.1007/s00415-018-8861-4

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  • DOI: https://doi.org/10.1007/s00415-018-8861-4

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