Advertisement

Palliative Care in Neurological Diseases

  • Shoba Nair
Chapter

Abstract

Many of the most frequent neurological diseases are chronic, incurable, and autonomy-impairing illnesses. Neurological conditions like amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND), Parkinson’s disease (PD), the associated progressive supranuclear palsy and multiple systems atrophy, and multiple sclerosis (MS) exhibit their own unique characteristics and have their own clinical course with complex and variable symptoms, disability, and prognosis. Palliative care focuses on the patient’s and their family member’s quality of life in the entire course of a disabling and life-limiting disease. As healthcare professionals are increasingly recognizing the fact that patients benefit from good symptom management and a holistic approach, the utility of palliative care service comes into focus. It is important to recognize, assess, and manage pain, gastrointestinal symptoms, dyspnea, and neuropsychiatric symptoms in these patients. Caregivers find it extremely difficult to cope with the high amounts of physical and mental needs that patients with neurological diseases have, and this should be addressed. Patients and their family members have a right to know about their disease prognosis, and they can be encouraged to have any advance directives documented, when they have the capacity to make a decision. This would ensure that they have a pragmatic end-of-life care plan in place, which ensures excellent end-of-life care. Palliative care ensures that patients not only have quality in their lives but also quality toward their end of life and dignity in dying and death. As healthcare professionals, it is our responsibility to achieve this goal in patient care. In order to ensure this, it is imperative that neurology and palliative care teams work together.

Keywords

Neurological diseases Palliative care Symptom management Advance directives End-of-life care 

References

  1. 1.
    Oliver D, Borasio GD, Caraceni A, et al. Palliative care in chronic and progressive neurological disease: summary of a consensus review. Eur J Palliat Care. 2016;23(5):232–5.Google Scholar
  2. 2.
    Mohamed IN, Elseed MA, Hamed AA. Clinical profile of pediatric neurological disorders. Child Neurol Open. 2016;15:270–8.Google Scholar
  3. 3.
    Feigin VL, et al. Global, regional, and national burden of neurological disorders during 1990–2015: a systematic analysis for the Global Burden of Disease Study 2015. Lancet Neurol. 2017;16(11):877–97.CrossRefGoogle Scholar
  4. 4.
    GBD 2015 DALYs and HALE Collaborators. Global, regional, and national disability-adjusted life-years (DALYs) for 315 diseases and injuries and healthy life expectancy (HALE), 1990–2015: a systematic analysis for the Global Burden of Disease Study 2015. Lancet. 2016;388:1603–58.CrossRefGoogle Scholar
  5. 5.
    Center to Advance Palliative Care. About palliative care. https://www.capc.org/about/palliative-care/. Accessed 5 Apr 2018.
  6. 6.
    National Hospice and Palliative Care Organization. Hospice care. https://www.nhpco.org/about/hospice-care. Accessed 5 Apr 2018.
  7. 7.
    Lorenzl S, Nübling G, Perrar KM, et al. Palliative treatment of chronic neurologic disorders. Handb Clin Neurol. 2013;118:133–9.PubMedCrossRefGoogle Scholar
  8. 8.
    Saleem TZ, Higginson IJ, Chaudhuri KR, et al. Symptom prevalence, severity and palliative care needs assessment using the Palliative Outcome Scale: a cross-sectional study of patients with Parkinson’s disease and related neurological conditions. Palliat Med. 2013;27(8):722–31.PubMedCrossRefGoogle Scholar
  9. 9.
    Raheja D, Stephens HE, Lehman E, et al. Patient-reported problematic symptoms in an ALS treatment trial. Amyotroph Lateral Scler Frontotemporal Degener. 2016;17(3–4):198–205.PubMedPubMedCentralCrossRefGoogle Scholar
  10. 10.
    Radicati FG, Martinez Martin P, Fossati C, et al. Non motor symptoms in progressive supranuclear palsy: prevalence and severity. CNPJ Parkinsons Dis. 2017;3:35.CrossRefGoogle Scholar
  11. 11.
    Solla P, Cannas A, Mulas CS, et al. Association between fatigue and other motor and non-motor symptoms in Parkinson’s disease patients. J Neurol. 2014;261(2):382–91.PubMedCrossRefGoogle Scholar
  12. 12.
    Young J, Amatya B, Galea MP, et al. Chronic pain in multiple sclerosis: a 10-year longitudinal study. Scand J Pain. 2017;16:198–203.PubMedCrossRefGoogle Scholar
  13. 13.
    Cortez MM, Nagi Reddy SK, Goodman B. Autonomic symptom burden is associated with MS-related fatigue and quality of life. Mult Scler Relat Disord. 2015;4(3):258–63.PubMedCrossRefGoogle Scholar
  14. 14.
    Levinthal DJ, Rahman A, Nusrat S, et al. Adding to the burden: gastrointestinal symptoms and syndromes in multiple sclerosis. Mult Scler Int. 2013;2013:319201.PubMedPubMedCentralGoogle Scholar
  15. 15.
    McKay KA, Tremlett H, Fisk JD, et al. Psychiatric comorbidity is associated with disability progression in multiple sclerosis. Neurology. 2018;90(15):e1316–23.PubMedPubMedCentralCrossRefGoogle Scholar
  16. 16.
    Sampson EL. Palliative care for people with dementia. Br Med Bull. 2010;96(1):159–74.PubMedCrossRefGoogle Scholar
  17. 17.
    Ryan T, Ingleton C, Gardiner C, et al. Symptom burden, palliative care need and predictors of physical and psychological discomfort in two UK hospitals. BMC Palliat Care. 2013;12:11.PubMedPubMedCentralCrossRefGoogle Scholar
  18. 18.
    World Health Organization. Palliative care. https://www.who.int/ncds/management/palliative-care/en/. Accessed 5 Apr 2018.
  19. 19.
    Royal College of Physicians, National Council for Palliative Care, British Society of Rehabilitation Medicine. Long-term neurological conditions: management at the interface between neurology, rehabilitation and palliative care. Concise guidance to good practice series, No 10. London: RCP; 2008. https://www.bsrm.org.uk/downloads/long-term-neurological-conditions-concise.pdf. Accessed 5 Apr 2018.
  20. 20.
    Khan F, Turner-Stokes L, Ng L, Kilpatrick T. Multidisciplinary rehabilitation for adults with multiple sclerosis (Full review). Cochrane Database Syst Rev. 2007;18(2):CD006036.Google Scholar
  21. 21.
    Turner-Stokes L, Nair A, Disler P, Wade D. Cochrane review: multi-disciplinary rehabilitation for acquired brain injury in adults of working age. Cochrane Database Syst Rev. 2015;3:CD004170.Google Scholar
  22. 22.
    Turner-Stokes L. The effectiveness of rehabilitation: a critical review of the evidence. Clin Rehab. 1999;13(Suppl 1):83.Google Scholar
  23. 23.
    Mitchell JD, Borasio GD. Amyotrophic lateral sclerosis. Lancet. 2007;369(9578):2031–41.PubMedCrossRefGoogle Scholar
  24. 24.
    Khan F, McPhail T, Brand C, et al. Multiple sclerosis: disability profile and quality of life in an Australian community cohort. Int J Rehabil Res. 2006;29(2):87–96.PubMedCrossRefGoogle Scholar
  25. 25.
    Borsook D. Neurological diseases and pain. Brain. 2012;135(2):320–44.PubMedCrossRefGoogle Scholar
  26. 26.
    Simuni T, Sethi K. Nonmotor manifestations of Parkinson’s disease. Ann Neurol. 2008;64(Suppl 2):S65–80.PubMedGoogle Scholar
  27. 27.
    Ford B. Pain in Parkinson’s disease. Mov Disord. 2010;25(Suppl 1):S98–103.PubMedCrossRefGoogle Scholar
  28. 28.
    Stacy MA, Murck H, Kroenke K. Responsiveness of motor and nonmotor symptoms of Parkinson disease to dopaminergic therapy. Prog Neuro-Psychopharmacol Biol Psychiatry. 2010;34:57–61.CrossRefGoogle Scholar
  29. 29.
    Franca MC Jr, D’Abreu A, Friedman JH, Nucci A, Lopes-Cendes I. Chronic pain in Machado-Joseph disease: a frequent and disabling symptom. Arch Neurol. 2007;64:1767–70.PubMedCrossRefGoogle Scholar
  30. 30.
    Drake ME Jr. Chronic pain syndrome in amyotrophic lateral sclerosis. Arch Neurol. 1983;40:453–4.PubMedCrossRefGoogle Scholar
  31. 31.
    Gerber RK, Nie H, Arendt-Nielsen L, Curatolo M, Graven-Nielsen T. Local pain and spreading hyperalgesia induced by intramuscular injection of nerve growth factor are not reduced by local anesthesia of the muscle. Clin J Pain. 2011;27:240–7.PubMedCrossRefGoogle Scholar
  32. 32.
    Solaro C, Messmer UM. Pharmacological management of pain in patients with multiple sclerosis. Drugs. 2010;70:1245–54.PubMedGoogle Scholar
  33. 33.
    O’Connor AB, Schwid SR, Herrmann DN, Markman JD, Dworkin RH. Pain associated with multiple sclerosis: systematic review and proposed classification. Pain. 2008;137:96–111.PubMedCrossRefGoogle Scholar
  34. 34.
    Hong JH, Bai DS, Jeong JY, Choi BY, Chang CH, Kim SH, et al. Injury of the spino-thalamo-cortical pathway is necessary for central post-stroke pain. Eur Neurol. 2010;64:163–8.PubMedCrossRefGoogle Scholar
  35. 35.
    Adey-Wakeling Z, Arima H, Crotty M, et al. Incidence and associations of hemiplegic shoulder pain poststroke: prospective population-based study. Arch Phys Med Rehabil. 2015;96(2):241–7.PubMedCrossRefGoogle Scholar
  36. 36.
    Coskun Benlidayi I, Basaran S. Hemiplegic shoulder pain: a common clinical consequence of stroke. Pract Neurol. 2013;12:88–91.Google Scholar
  37. 37.
    Pasero C, McCaffery M. Pain assessment and pharmacologic management. St. Louis: Mosby; 2011.Google Scholar
  38. 38.
    International Network for Cancer Treatment and Research. INCTR palliative care handbook. https://www.inctr-palliative-care-handbook.wikidot.com. Accessed 5 Apr 2018.
  39. 39.
    Herr K, Spratt KF, Garand L, Li L. Evaluation of the Iowa pain thermometer and other selected pain intensity scales in younger and older adult cohorts using controlled clinical pain: a preliminary study. Pain Med. 2007;8:585–600.PubMedPubMedCentralCrossRefGoogle Scholar
  40. 40.
    Gélinas C, Johnston C. Pain assessment in the critically ill ventilated adult: validation of the critical-care pain observation tool and physiologic indicators. Clin J Pain. 2007;23:497–505.PubMedCrossRefGoogle Scholar
  41. 41.
    Payen J, Bru O, Bosson J, et al. Assessing pain in critically ill sedated patients by using a behavioral pain scale. Crit Care Med. 2001;29:2258–63.PubMedCrossRefGoogle Scholar
  42. 42.
    Hutchison RW, Tucker WF Jr, Kim S, Gilder R. Evaluation of a behavioral assessment tool for the individual unable to self- report pain. Am J Hosp Palliat Care. 2006;23:328–31.PubMedCrossRefGoogle Scholar
  43. 43.
    Martin J, Torre F, Aguirre U, et al. Evaluation of the interdisciplinary PSYMEPHY treatment on patients with fibromyalgia: a randomized control trial. Pain Med. 2014;27:682–91.CrossRefGoogle Scholar
  44. 44.
    Nijs J, Van Oosterwijck J, De Hertogh W. Rehabilitation of chronic whiplash: treatment of cervical dysfunctions or chronic pain syndrome? Clin Rheumatol. 2009;28(3):243–51.PubMedCrossRefGoogle Scholar
  45. 45.
    Tang NK, Salkovskis PM, Hodges A, et al. Chronic pain syndrome associated with health anxiety: a qualitative thematic comparison between pain patients with high and low health anxiety. Br J Clin Psychol. 2009;48:1–20.PubMedCrossRefGoogle Scholar
  46. 46.
    World Health Organization. WHO’s cancer pain ladder for adults. https://www.who.int/cancer/palliative/painladder/en/. Accessed 5 Apr 2018.
  47. 47.
    Gupta S, Atcheson R. Opioid and chronic non-cancer pain. J Anaesthesiol Clin Pharmacol. 2013;29(1):6–12.PubMedPubMedCentralCrossRefGoogle Scholar
  48. 48.
    Cherny N, Fallon M, Stein K, et al. Dyspnoea and other respiratory symptoms in palliative care. In:Oxford textbook of palliative medicine. 5th ed. Oxford: Oxford University Press.Google Scholar
  49. 49.
    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
  50. 50.
    Hutchinson D, Whyte K. Neuromuscular disease and respiratory failure. Pract Neurol. 2008;8(4):229–37.PubMedCrossRefGoogle Scholar
  51. 51.
    Kim WH, Kim JH, Kim EK, et al. Myasthenia gravis presenting as isolated respiratory failure: a case report. Korean J Intern Med. 2010;25:101–4.PubMedPubMedCentralCrossRefGoogle Scholar
  52. 52.
    Mahler DA, Wells CK. Evaluation of clinical methods for rating dyspnea. Chest. 1988;93:580–6.PubMedCrossRefGoogle Scholar
  53. 53.
    Guyatt GH, Berman LB, Townsend M, Pugsley SO, Chambers LW. A measure of quality of life for clinical trials in chronic lung disease. Thorax. 1987;42:773–8.PubMedPubMedCentralCrossRefGoogle Scholar
  54. 54.
    Bourke SC, Tomlinson M, Williams TL, Bullock RE, Shaw PJ, Gibson GJ. Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurol. 2006;5(2):140–7.PubMedCrossRefGoogle Scholar
  55. 55.
    Andersen PM, Abrahams S, Borasio GD, et al. EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS)—revised report of an EFNS task force. Eur J Neurol. 2012;19(3):360–75.PubMedCrossRefGoogle Scholar
  56. 56.
    Specialist Pharmacy Service. Hypersalivation—what are the treatment alternatives to glycopyrronium and hyoscine? https://www.sps.nhs.uk/articles/hypersalivation-d-what-are-the-treatment-alternatives-to-glycopyrronium-and-hyoscine/ukmi_qa_hypersalivationalternatives_update-may-2017/. Accessed 5 Apr 2018.
  57. 57.
    National Collaborating Centre for Chronic Conditions. Parkinson’s disease: national clinical guideline for diagnosis and management in primary and secondary care. London: Royal College of Physicians; 2006.https://www.nice.org.uk/guidance/cg35/evidence. Accessed 12 Apr 2018.
  58. 58.
    Hobson EV, McGeachan A, Al-Chalabi A, Chandran S, et al. Management of sialorrhoea in motor neuron disease: a survey of current UK practice. Amyotroph Lateral Scler Frontotemporal Degener. 2013;14(7–8):521–7.PubMedCrossRefGoogle Scholar
  59. 59.
    Voltz R, Lorenzl S, Nübling G. Neurological disorders other than dementia. In:Oxford textbook of palliative medicine. 5th ed. Oxford: Oxford University Press.Google Scholar
  60. 60.
    Carranza-del Rio J, Clegg NJ, Moore A, et al. Use of trihexyphenidyl in children with cerebral palsy. Pediatr Neurol. 2011;44:202–6.PubMedCrossRefGoogle Scholar
  61. 61.
    Parkes JD. A neurologist’s view of nausea and vomiting. In: Davis CJ, Lake-Bakaar GV, Grahame-Smith DG, editors. Nausea and vomiting: mechanisms and treatment. advances in applied neurological sciences, vol. 3. Berlin: Springer; 1986.Google Scholar
  62. 62.
    Edwards LL, Pfeiffer RF, Quigley EMM, Hofman R, Balluff M. Gastrointestinal symptoms in Parkinson’s disease. Mov Disord. 1991;6(2):151–6.PubMedCrossRefGoogle Scholar
  63. 63.
    Antiparkinson Drugs. Gale encyclopedia of neurological disorders. The Gale Group. 2005. https://www.encyclopedia.com/medicine/divisions-diagnostics-and-procedures/medicine/antiparkinson-agents. Accessed 16 Apr 2018.
  64. 64.
    Royal College of Physicians, National Council for Palliative Care, British Society of Rehabilitation Medicine. Long-term neurological conditions: management at the interface between neurology, rehabilitation and palliative care. Concise guidance to good practice series, No 10. London: RCP; 2008.Google Scholar
  65. 65.
    Poirier A-A, Aubé B, Côté M, Morin N, Di Paolo T, Soulet D. Gastrointestinal dysfunctions in Parkinson’s disease: symptoms and treatments. Parkinsons Dis. 2016;2016:23.Google Scholar
  66. 66.
    Toepfer M, Folwaczny C, Klauser A. Gastrointestinal dysfunction in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord. 1999;1(1):15–9.PubMedCrossRefGoogle Scholar
  67. 67.
    Alegre P. Depression and anxiety in individuals with amyotrophic lateral sclerosis: a systematic review. Trends Psychiatry Psychother. 2016;38(1):1–5.CrossRefGoogle Scholar
  68. 68.
    Pham T, Jetté N, Bulloch AGM, et al. The prevalence of anxietyand associated factors in persons with multiple sclerosis. Mult Scler Relat Disord. 2018;19:35–9.PubMedCrossRefGoogle Scholar
  69. 69.
    Djamshidian A, Friedman JH. Anxiety and depression in Parkinson’s disease. Curr Treat Options Neurol. 2014;16(4):285.PubMedCrossRefGoogle Scholar
  70. 70.
    City of Palo Alto Project Safety Net. Diagnostic criteria for major depressive disorder and depressive episodes. http://www.psnpaloalto.com/wp/wp-content/uploads/2010/12/Depression-Diagnostic-Criteria-and-Severity-Rating.pdf. Accessed 12 Apr 2018.
  71. 71.
    Cambridgeshire Palliative Care Guidelines Group Review Date: February 2016 Page 2 of 3 Factsheet 15. https://www.arhc.org.uk/downloadsMCL/download.asp?doc=FACTSHEET-15.pdf. Accessed 5 Apr 2018.
  72. 72.
    Sullivan AB, Deborah Miller D. Who is taking care of the caregiver? J Patient Exp. 2015;2(1):7–12.PubMedPubMedCentralCrossRefGoogle Scholar
  73. 73.
    Akila P, Agnes Monica V. Caregiver’s burden on patients with neurological disorders. Int J Sci Res. 2014;5.611. https://www.ijsr.net/archive/v5i2/NOV161246.pdf. Accessed 5 Apr 2018.
  74. 74.
    Tsai P, Yip P, Tai JJ, Lou M. Needs of family caregivers of stroke patients: a longitudinal study of caregivers’ perspectives. Patient Prefer Adherence. 2015;9:449–57.PubMedPubMedCentralGoogle Scholar
  75. 75.
    Logeais T. Assessing and addressing family caregiver burden: palliative care social work perspective. Master of social work clinical research papers. 764. 2017. https://www.sophia.stkate.edu/msw_papers/764. Accessed 5 Apr 2018.
  76. 76.
    American Psychological Association. About the public interest directorate. http://www.apa.org/pi/about/index.aspx. Accessed 12 Apr 2018.
  77. 77.
    Sullivan AB. Caregiving in multiple sclerosis. In: Rae-Grant A, Fox R, Bethoux F, editors. Multiple sclerosis and related disorders: clinical guide to diagnosis, medical management, and rehabilitation. New York: Demos Publishing; 2013.Google Scholar
  78. 78.
    Cimino JE. A clinician’s understanding of ethics in palliative care: an American perspective. Crit Rev Oncol Hematol. 2003;46:17–24.PubMedCrossRefGoogle Scholar
  79. 79.
    Beauchamp TL, Childress JF. Principles of biomedical ethics. 4th ed. Oxford: Oxford University Press; 1994. p. 206–11.Google Scholar
  80. 80.
    Cassell EJ. The nature of suffering and the goals of medicine. N Engl J Med. 1982;306:639–45.CrossRefGoogle Scholar
  81. 81.
    Chaturvedi SK, Loiselle CG, Chandra PS. Communication with relatives and collusion in palliative care: a cross-cultural perspective. Indian J Palliat Care. 2003;15:2–9.CrossRefGoogle Scholar
  82. 82.
    Emanuel LL, Barry MJ, Stoeckle JD, Ettelson LM, Emanuel EJ. Advance directives for medical care – a case for greater use. N Engl J Med. 1991;324(13):889–95.PubMedCrossRefGoogle Scholar
  83. 83.
    American Medical Association. 150th Anniversary Edition*/Code of Medical Ethics 1997; Section 2.035: 9.Google Scholar
  84. 84.
    New York State Department of Health. Do not resuscitate orders. 2nd ed. 1992. p. 15.Google Scholar
  85. 85.
    Finlay IG, Wheatley VJ. Ethical issues in palliative care. Medicine. 2007;36(2):111–3. https://www.medicinejournal.co.uk/article/S1357-3039(07)00400-8/pdf. Accessed 6 Apr 2018.
  86. 86.
    General Medical Council. End of life care: guidance. https://www.gmc-uk.org/guidance/ethical_guidance/end_of_life_guidance.asp. Accessed 6 Apr 2018.
  87. 87.
    Ellershaw J, Lakhani M. Best care for the dying patient why do so many people die badly when we know how to care for them well? BMJ. 2013;347:f4428.PubMedCrossRefGoogle Scholar
  88. 88.
    MS Society. End of life care in long-term neurological conditions—a framework for implementation. https://www.mssociety.org.uk/ms-resources/end-life-care-long-term-neurological-conditions-%E2%80%93-framework-implementation. Accessed 12 Apr 2018.
  89. 89.
    Joad AK. End of life care. Text book for essentials course in palliative care. 5th ed. Indian Association of Palliative Care; 2017.Google Scholar
  90. 90.
    Lacey J. Management of the actively dying patient. In: Cherny N, Fallon M, Kaasa S, Portenoy RK, Currow DC, editors. Oxford textbook of palliative medicine. 5th ed. Oxford: Oxford University Press; 2015.Google Scholar
  91. 91.
    Cherny N, Radbruch L, Board of the European Association for Palliative Care. European Association for Palliative Care (EAPC) recommended framework for the use of sedation in palliative care. Palliat Med. 2009;23(7):581–93.PubMedCrossRefGoogle Scholar
  92. 92.
    Krakauer EL. Sedation at the end of life. In: Cherny N, Fallon M, Kaasa S, Portenoy RK, Currow DC, editors. Oxford textbook of palliative medicine. 5th ed. Oxford: Oxford University Press; 2015.Google Scholar
  93. 93.
    European Association for Palliative Care. Bereavement care provision in palliative care—a European descriptive and consensus-building exercise. https://www.eapcnet.eu/Themes/ProjectsTaskforces/EAPCTaskforces/Bereavement.aspx. Accessed 6 Apr 2018.

Copyright information

© Springer Nature Singapore Pte Ltd. 2019

Authors and Affiliations

  • Shoba Nair
    • 1
  1. 1.Department of Palliative MedicineSheffield Teaching HospitalsSheffieldUK

Personalised recommendations