Advertisement

Palliative Care and Neurodegenerative Diseases

  • David Oliver
  • Simone Veronese
Reference work entry

Abstract

Neurological disease is a major cause of disability and death across the world. There is increasing evidence that palliative care is effective in managing symptoms, maintaining and improving quality of life, and helping patients and families cope with the deterioration. As the disease progression varies, both between diseases and for individuals, palliative care may be involved for varying periods of time, and may need to be involved episodically throughout the disease progression. Careful assessment of all the issues – physical, psychosocial, and spiritual – will allow appropriate management and support for patients and families. Carer support is very important as families face all the issues of coping with a progressive disease. Recognition of the later stages of life is helpful in enabling patients, families, and professionals to be able to prepare for the dying phase and manage all the issues appropriately.

References

  1. Aboussouan LS. Respiratory disorders in neurologic diseases. Cleve Clin J Med. 2005;72(6):511–20.PubMedGoogle Scholar
  2. Albert SM, et al. Wish to die in end-stage ALS. Neurology. 2005;65(1):68–74.PubMedPubMedCentralGoogle Scholar
  3. Andersen PM, Abrahams S, Borasio GD, de Carvalho M, Chio A, Van Damme P, Hardiman O, Kollewe K, Morrison KE, Petri S, Pradat PF, Silani V, Tomik B, Wasner M, Weber M, The EFNS Task Force on Diagnosis and Management of Amyotrophic Lateral Sclerosis. EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS) – revised report of an EFNS task force. Eur J Neurol. 2012;19:360–75.PubMedGoogle Scholar
  4. Aoun SM, Breen LJ, Howting D, Edis R, Oliver D, Henderson R, O’Connor M, Harris R, Birks C. Receiving the news of a diagnosis of motor neuron disease: what does it take to make it better? Amyotroph Lat Scler FTD. 2015;17:168–78.Google Scholar
  5. Aoun SM, Breen LJ, Edis R, Henderson RD, Oliver D, Harris R, Howting D, O’Connor M, Birks C. Breaking the news of a diagnosis of motor neurone disease: a national survey of neurologists’ perspectives. J Neurol Sci. 2016;367:368–74.PubMedGoogle Scholar
  6. Aoun SM, Breen LJ, Oliver D et al. Family carers’ experiences of receiving the news of a diagnosis of Motor Neurone Disease: A national survey. J Neurol Sci 2017; 372: 144–151.PubMedGoogle Scholar
  7. Baile WF, Buckman R, Lenzi R, et al. SPIKES – a six step protocol for delivering bad news: application to the patient with cancer. Oncologist. 2000;5:302–11.PubMedGoogle Scholar
  8. Bakitas M, Lyons KD, et al. Effects of a palliative care intervention on clinical outcomes in patients with advanced cancer: the project ENABLE II randomized controlled trial. JAMA. 2009;302(7):741–9.PubMedPubMedCentralGoogle Scholar
  9. Bede P, Hardiman O, O’Brannagain D. An integrated framework of early intervention palliative care in motor neurone disease as a model to progressive neurodegenerative disease. Poster at European ALS congress, Turin; 2009.Google Scholar
  10. Bermejo PE, Oreja-Guevara C, Diez-Tejedor E. Pain in multiple sclerosis: prevalence, mechanisms, types and treatment. Rev Neurol. 2010;50:101–8.PubMedGoogle Scholar
  11. Bernheim JL, Raus K. Euthanasia embedded in palliative care. Responses to essentialistic criticisms of the Belgian model of integral end-of-life care. J Med Ethics. 2017;43:489–494.PubMedGoogle Scholar
  12. Boersma I, Miyasaki J, Kutner J, Kluger B. Palliative care and neurology: time for a paradigm shift. Neurology. 2014;83(6):561–7.PubMedPubMedCentralGoogle Scholar
  13. Borreani C, et al. Unmet needs of people with severe multiple sclerosis and their carers: qualitative findings for a home-based intervention. PLoS One. 2014;9:e109679.PubMedPubMedCentralGoogle Scholar
  14. Borsook D. Neurological diseases and pain. Brain. 2012;135:320–44.PubMedGoogle Scholar
  15. Breitbart W, Rosenfeld B, et al. Depression, hopelessness, and desire for hastened death in terminally ill patients with cancer. JAMA. 2000;284:2907–11.Google Scholar
  16. Buecken R, Galushko M, Golla H, Strupp J, Hahn M, Ernstmann N, Pfaff H, Voltz R. Patients feeling severely affected by multiple sclerosis: how do patients want to communicate about end-of-life issues? Patient Educ Couns. 2012;88:318–24.PubMedGoogle Scholar
  17. Catania G, Beccaro M, et al. Effectiveness of complex interventions focused on quality-of life assessment to improve palliative care patients’ outcomes: a systematic review. Palliat Med. 2015;29(1):5–21.PubMedGoogle Scholar
  18. Chapman S. Advance care planning. In: Oliver D, editor. End of life care in neurological disease. London: Springer; 2012.Google Scholar
  19. Creutzfelt CJ, Robinson MT, Holloway RG. Neurologists as primary palliative care providers. Communication and practice approaches. Neurol Clin Pract. 2016;6:1–9.Google Scholar
  20. Edmonds P, Hart S, Gao W, Vivat B, Burman R, Silber E, Higginson I. Palliative care for people severely affected by multiple sclerosis: evaluation of a novel palliative care service. Mult Scler. 2010;16:627–36.Google Scholar
  21. Funk L, et al. Part 2: home-based family caregiving at the end of life: a comprehensive review of published qualitative research (1998-2008). Palliat Med. 2010;24:594–607.PubMedGoogle Scholar
  22. Gaertner J, Wolf J, Hallek M, Glossmann J-P, Voltz R. Standardising integration of palliative care into comprehensive cancer therapy – a disease specific approach. Support Care Cancer. 2011;19:1037–43.PubMedGoogle Scholar
  23. Ganzini L, Johnston WS, McFarland BH, Tolle SW, Lee MA. Attitudes of patients with amyotrophic lateral sclerosis and their care givers toward assisted suicide. N Engl J Med. 1998;339(14):967–73.PubMedGoogle Scholar
  24. General Medical Council. End of life care: glossary of terms. Available from: http://www.gmc-uk.org/guidance/ethical_guidance/end_of_life_glossary_of_terms.asp. Accessed 8 Feb 2017.
  25. Goldstein LH. Control of symptoms: cognitive dysfunction. In: Oliver D, Borasio GD, Johnston W, editors. Palliative care in amyotrophic lateral sclerosis- from diagnosis to bereavement. 3rd ed. Oxford: Oxford University Press; 2014. p. 107–25.Google Scholar
  26. Grande GE, Ewing G. National Forum for hospice at home. Informal carer bereavement outcome: relation to quality of end of life support and achievement of preferred place of death. Palliat Med. 2009;23:248–56.PubMedGoogle Scholar
  27. GSF, G.-S.-F. Proactive identification guidance. 2016. Available from: http://www.goldstandardsframework.org.uk/library-tools-amp-resources. Accessed 8 Feb 2017.
  28. Higginson IJ, Finlay IG, et al. Is there evidence that palliative care teams alter end-of-life experiences of patients and their caregivers? J Pain Symptom Manag. 2003;25(2):150–68.Google Scholar
  29. Higginson IJ, Hart S, et al. Symptom prevalence and severity in people severely affected by multiple sclerosis. J Palliat Care. 2006;22:158–65.Google Scholar
  30. Higginson IJ, Hart S, et al. Randomised controlled trial of a new palliative care service: compliance, recruitment and completeness of follow-up. BMC Palliat Care. 2008;7(1):7.PubMedPubMedCentralGoogle Scholar
  31. Higginson IJ, McCrone P, et al. Is short-term palliative care cost-effective in multiple sclerosis? A randomized phase II trial. J Pain Symptom Manag. 2009;38(6):816–26.Google Scholar
  32. Hussain J, Adams D, Allgar V, Campbell C. Triggers in advanced neurological conditions: prediction and management of the terminal phase. BMJ Support Palliat Care. 2014;4:30–7.PubMedGoogle Scholar
  33. Jennings AL, et al. Opioids for the palliation of breathlessness in terminal illness. Cochrane Database Syst Rev. 2001;(4).  https://doi.org/10.1002/14651858.CD00266
  34. Jox RJ, Krebs M, Bickhardt J, et al. How strictly should advance decisions be followed? The patients’ opinion. Palliat Med. 2008;22:675–6.PubMedGoogle Scholar
  35. Kaub-Wittemer D, et al. Quality of life and psychosocial issues in ventilated patients with amyotrophic lateral sclerosis and their caregivers. J Pain Symptom Manag. 2003;26(4):890–6.Google Scholar
  36. Klit H, Finnerup NB, Jensen TS. Central post-stroke pain: clinical characteristics, pathophysiology, and management. Lancet Neurol. 2009;8:857–68.PubMedGoogle Scholar
  37. Kumar B, et al. Central poststroke pain: a review of pathophysiology and treatment. Anesth Analg. 2009;108:1645–57.PubMedGoogle Scholar
  38. Lambert R. Spiritual care. In: Oliver D, Borasio GD, Johnston W, editors. Palliative care in amyotrophic lateral sclerosis – from diagnosis to bereavement. 3rd ed. Oxford: Oxford University Press; 2014.Google Scholar
  39. Lee MA, et al. Measuring symptom load in idiopathic Parkinson’s disease. Parkinsonism Relat Disord. 2007;13(5):284–9.PubMedGoogle Scholar
  40. Lienard A, Merckaert I, Libert Y, et al. Is it possible to improve residents breaking bad news skills? A randomised study assessing the efficacy of a communication skills training programme. Br J Cancer. 2010;103:171–7.PubMedPubMedCentralGoogle Scholar
  41. Mangera Z, Panesar G, Makker H. Practical approach to management of respiratory complications in neurological disorders. Int J Gen Med. 2012;5:255–63.PubMedPubMedCentralGoogle Scholar
  42. McIlfatrick S. Assessing palliative care needs: views of patients, informal carers and healthcare professionals. J Adv Nurs. 2007;57:77–86.PubMedGoogle Scholar
  43. Miller RG, et al. Practice parameter: the care of the patient with amyotrophic lateral sclerosis (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology: ALS practice parameters task force. Neurology. 1999;52:1311–23.PubMedGoogle Scholar
  44. Miller RG, Jackson CE, Karsarkis EJ, England JD, Forshew D, Johnston W, et al. Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: multidisciplinary care, symptom management, and cognitive/behavioural impairment (an evidence-based review). Neurology. 2009;73:1227–33.PubMedPubMedCentralGoogle Scholar
  45. Modirian E, et al. Chronic pain after spinal cord injury: results of a long-term study. Pain Med. 2010;11:1037–43.PubMedGoogle Scholar
  46. Moens K, et al. Are there differences in the prevalence of palliative care-related problems in people living with advanced cancer and eight non-cancer conditions? A systematic review. J Pain Symptom Manag. 2014;48:660–77.Google Scholar
  47. Moulin DE, et al. Pain in Guillain-Barre syndrome. Neurology. 1997;48:328–31.PubMedGoogle Scholar
  48. Moulton EA, Schmahmann JD, et al. The cerebellum and pain: passive integrator or active participator? Brain Res Rev. 2010;65:14–27.PubMedPubMedCentralGoogle Scholar
  49. National End of Life Care Programme. End of life care in long term neurological conditions: a framework for implementation: National End of Life Care Programme; 2010. www.mssociety.org.uk/sites/default/files/Documents/Professionals/End%20life%20care%20long%20term%20neuro%20conditions.pdf
  50. Neudert C, Oliver D, Wasner M, Borasio GD. The course of the terminal phase in patients with amyotrophic lateral sclerosis. J Neurol. 2001;248:612–6.PubMedGoogle Scholar
  51. NICE. Parkinson’s disease in over 20s: diagnosis and management. 2006. Available from: https://www.nice.org.uk/guidance/cg35.
  52. O’Brien MR. Information-seeking behaviour among people with motor neurone disease. Br J Nurs. 2004;13:964–8.PubMedGoogle Scholar
  53. O’Brien T, Kelly M, Saunders C. Motor neurone disease: a hospice perspective. Br Med J. 1992;304:471–3.Google Scholar
  54. O’Connor AB, et al. Pain associated with multiple sclerosis: systematic review and proposed classification. Pain. 2008;137:96–111.PubMedGoogle Scholar
  55. Oliver D. The quality of care and symptom control – the effects on the terminal phase of ALS/MND. J Neurol Sci. 1996;139(Suppl):134–6.PubMedGoogle Scholar
  56. Oliver D. Palliative care. In: Oliver D, Borasio G, Johnston W, editors. Palliative care in amyotrophic lateral sclerosis- from diagnosis to bereavement. 3rd ed. Oxford: Oxford University Press; 2014.Google Scholar
  57. Oliver DJ, Borasio GD, Caraceni A, de Visser M, Grisold W, Lorenzl S, Veronese S, Voltz R. A consensus review on the development of palliative care for patients with chronic and progressive neurological disease. Eur J Neurol. 2016;23:30–8.  https://doi.org/10.1111/ene.12889.PubMedGoogle Scholar
  58. Otero-Romero S, Sastre-Garriga J, et al. Pharmacological management of spasticity in multiple sclerosis: systematic review and consensus paper. Mult Scler. 2016;22:1386–96.PubMedGoogle Scholar
  59. Pautex S, et al. Pain in severe dementia: self-assessment or observational scales? J Am Geriatr Soc. 2006;54:1040–5.PubMedGoogle Scholar
  60. Radbruch L, Leget C, Müller-Busch C, Ellershaw F, de Conno F, Vanden BP. Euthanasia and physician-assisted suicide: a white paper from the European Association for Palliative Care. Palliat Med. 2015;30:104–16.PubMedGoogle Scholar
  61. Radunovic A, et al. Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database Syst Rev. 2013;28(3):CD004427.Google Scholar
  62. Sampson EL, Candy B, Jones L. Enteral tube feeding for older people with advanced dementia. Cochrane Database Syst Rev. 2009;15(2):CD007209.Google Scholar
  63. Scott A, McPhee M. Multidisciplinary care: speech and language therapy. In: Oliver D, Borasio GD, Johnston W, editors. Palliative care in amyotrophic lateral sclerosis – from diagnosis to bereavement. 3rd ed. Oxford: Oxford University Press; 2014. p. 215–32.Google Scholar
  64. Seeber AA, Pols AJ, Hijdra A, Grupstra HF, Willems DL, de Visser M. Experiences and reflections of patients with motor neuro disease on breaking the news in a two-tiered appointment : a qualitative study. BMJ Support Palliat Care. 2016;(1-9).  https://doi.org/10.1136/bmjspcare-2015-000977.Google Scholar
  65. Shaw C, Quinn A, Daniel E. Amyotrophic lateral sclerosis / motor neurone disease. In: Oliver D, Borasio G, Johnston W, editors. Palliative care in amyotrophic lateral sclerosis- from diagnosis to bereavement. 3rd ed. Oxford: Oxford University Press; 2014.Google Scholar
  66. Simuni T, Sethi K. Nonmotor manifestations of Parkinson’s disease. Ann Neurol. 2008;64(S2):S65–80.PubMedGoogle Scholar
  67. Smith S, Wasner M. Psychosocial care. In: Oliver D, Borasio GD, Johnston W, editors. Palliative care in amyotrophic lateral sclerosis- from diagnosis to bereavement. 3rd ed. Oxford: Oxford University Press; 2014.Google Scholar
  68. Solari A, Giordano A, et al. Home-based palliative approach for people with severe multiple sclerosis and their carers: study protocol for a randomized controlled trial. Trials. 2015;16(1):184.PubMedPubMedCentralGoogle Scholar
  69. Stutzki R, Weber M, Reiter-Theil S, Simmen U, Borasio GD, Jox RJ. Attitudes towards hastened death in ALS: a prospective study of patients and family caregivers. Amyotroph Lateral Scler Frontotemporal Degener. 2014;15(1–2):68–76.PubMedGoogle Scholar
  70. Temel JS, Greer JA, Muzikansky A, et al. Early palliative care for patients with metastatic non-small-cell lung cancer. N Engl J Med. 2010;19:733–42.Google Scholar
  71. Thomas, K. and J. Armstrong Wilson. The Gold Standards Framework Proactive Identification Guidance (PIG). 2016. Available from: www.goldstandardsframework.nhs.uk. Accessed 8 Feb 2017.
  72. Veldink JH, Wokke JH, et al. Euthanasia and physician-assisted suicide among patients with amyotrophic lateral sclerosis in the Netherlands. N Engl J Med. 2002;346(21):1638–44.PubMedGoogle Scholar
  73. Veronese S, Gallo G, et al. Specialist palliative care improves the quality of life in advanced neurodegenerative disorders: NE-PAL, a pilot randomised controlled study. BMJ Support Palliat Care. 2015.  https://doi.org/10.1136/bmjspcare-2014-000788.Google Scholar
  74. Veronese S, Gallo G, et al. The palliative care needs of people severely affected by neurodegenerative disorders: a qualitative study. Prog Palliat Care. 2017;25:11–6.  https://doi.org/10.1080/09699260.2016.1193968.Google Scholar
  75. van Vliet LM, Gao W, et al. How integrated are neurology and palliative care services? Results of a multicentre mapping exercise. BMC Neurol. 2016;16:63.PubMedPubMedCentralGoogle Scholar
  76. Voltz R, Akabayashi A, Reese C, Ohi G, Sass HM. End-of-life decisions and advance directives in palliative care: a cross cultural survey of patients and health-care professionals. J Pain Symptom Manag. 1998;16:153–62.Google Scholar
  77. Walshe M. Oropharyngeal dysphagia in neurodegenerative disease. J Gastroenterol Hepatol Res. 2014;3(10):1265–71.Google Scholar
  78. Whetten-Goldstein K, et al. Financial burden of chronic neurological disorders to patients and their families: what providers need to know. J Neurol Phys Ther. 2000;24:140–4.Google Scholar
  79. World Health Organisation. Global burden of neurological disorders: estimates and projections. In: Neurological disorders- public health challenges. Geneva: World Health Organisation; 2006.Google Scholar
  80. World Health Organization. Palliative care. 2002. www.who.int/cancer/palliative/definition/en/. Accessed 26 July 2015.
  81. Worth PF. When the going gets tough: how to select patients with Parkinson’s disease for advanced therapies. Pract Neurol. 2013;13:140–52.PubMedGoogle Scholar
  82. Zimmermann C, Riechelmann R, et al. Effectiveness of specialized palliative care: a systematic review. JAMA. 2008;299(14):1698–709.Google Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • David Oliver
    • 1
  • Simone Veronese
    • 2
  1. 1.Tizard CentreUniversity of KentCanterburyUK
  2. 2.Fondazione FAROpalliative careTorinoItaly

Personalised recommendations