Handbook of Clinical Neurology, Vol. 118 (3rd series) Ethical and Legal Issues in Neurology J.L. Bernat and R. Beresford, Editors © 2013 Elsevier B.V. All rights reserved
Chapter 11
Care of the dying neurologic patient ¨ BLING3, AND STEFAN LORENZL3 RAYMOND VOLTZ1,2*, GEORG NU Department of Palliative Medicine, University Hospital, Cologne, Germany
1
2
Center for Integrated Oncology, Cologne and Bonn, Germany
3
Interdisciplinary Center of Palliative Medicine, Ludwig Maximilians University, Munich, Germany
INTRODUCTION Professionals caring for dying neurologic patients may not be knowledgeable about the principles and practice of palliative care (see Chapter 10). Introducing a teaching conference for residents called “death rounds” is one possibility to share the emotions around a death in a group, learn from the experience together, and promote palliative care principles in a team of a neurology service (Khot et al., 2011). The pivotal point of death may be used as a lever to improve palliative care skills in existing services by including palliative care expertise. These rounds may be useful not only in the care of the dying patient but also earlier during the disease course.
CAUSES OF DEATH How do patients with neurologic disorders die and from what specific causes? The answers to these questions mostly may not be known to the specialist neurology services because many neurologists stop caring for patients when they can no longer attend outpatient clinics. These patients are then cared for at home or in nursing homes, with little or no supervision by palliative care or neurology. In addition to neurologic disorders classically considered a “palliative care diagnosis,” evidence is emerging that a palliative care approach is helpful in many more neurologic diseases. One common example is multiple sclerosis (MS) which is generally considered a disease that you “do not die from” (Voltz, 2010). However, MS patients are three times more likely to die prematurely than the age-matched general population (Hirst et al., 2008).
Another frequent neurologic diagnosis that is classically not considered for a palliative approach is ischemic or hemorrhagic stroke. About 30% of these patients die within the “acute stroke” phase, defined as the first 30 days after the event (Roberts and Goldacre, 2003), and stroke leaves another 30% permanently disabled with chronic deficits. Therefore, the stroke patient population has major palliative care needs. The early experience of palliative care services for stroke patients shows that these services help with symptom control, psychosocial issues, and end-of-life decisions, such as withdrawal of mechanic ventilation, artificial nutrition, tracheostomy, and neurosurgic procedures (Burton et al., 2010; Holloway et al., 2010). Even in rarer diagnoses of progressive neurologic disease, such as in sporadic inclusion body myositis, evidence is emerging that there are palliative care issues. Two recent long-term observational studies demonstrated a high symptom burden of patients in end-stage disease. In this series, 13% of patients died by euthanasia or terminal sedation, demonstrating the magnitude and frequency of suffering experienced by these patients at the end of their lives (Hohlfeld, 2011). The causes of death in neurologic disorders most often are those usually expected as complications of a chronic illness such as pneumonia (Hirst et al., 2008; Pennington et al., 2010). However, physicians also are involved in accelerating the deaths of some patients by euthanasia or palliative sedation, as has been reported in countries such as the Netherlands, where active euthanasia and assisted suicide are legal. There, these practices are performed more frequently in neurologic patients than in those with cancer. Whereas about 1% of the cancer population dies by euthanasia, this action accounts for death in about 20% in patients with
*Correspondence to: Raymond Voltz, Prof., Dr. med., Department of Palliative Medicine, University Hospital Cologne, Kerpener Straße 62, 50937 Cologne, Germany. Tel: 0049-221-478-3361, E-mail: [email protected]
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amyotrophic lateral sclerosis (ALS). This number has been stable in recent years. An additional 15% die after palliative sedation and that percentage is increasing (Maessen et al., 2009). In contrast to common lay and professional beliefs, patients with ALS do not suffocate, and usually die a peaceful natural death when under hospice care and also under the care of a neurology service (Neudert et al., 2001). Neurologic patients outside the Netherlands frequently express a wish to hasten death or suicide. In MS, suicide is a frequent cause of death, and a long protracted course with severe neurologic deficit in many patients leads to a wish for hastening death. In these patients, however, a palliative care approach may identify the reasons motivating this wish. With the assistance and support of a palliative care team, many patients regain the will for life (Voltz et al., 2011).
END-OF-LIFE DECISIONS End-of-life decision-making is a complicated and poorly understood process which places a significant burden on all parties involved. Clinicians frequently rely on surrogate decision-makers in end-of life treatment, particular in patients with neurodegenerative diseases. Often physicians do not address the patient’s preferences related to family involvement in decision-making. Physicians should discuss medical treatment goals with patients and their family members at the right time in order to participate in treatment and nontreatment decisions. Family members make up the vast majority of decision-makers and families do not use pure substituted judgments when making decisions on behalf of their loved ones. Some patients desire to have a more active role, even in advanced stages of their disease, when communication becomes difficult. Furthermore, several studies suggest that surrogates may be unable to predict patients’ preferences accurately (Shalowitz et al., 2006). Even the presence of advance directives does not improve the accuracy of surrogates’ predictions of patients’ preferences (Coppola et al., 2001; Ditto et al., 2001). Patients with ALS and MS often retain their capacity to make medical decisions for themselves even into the late stages of their illness. However, in patients with Parkinson’s disease, atypical parkinsonian disorders, and dementia, clinicians often must rely on surrogates because the patients are incapacitated. Frank communication about end-of-life issues not only seems to support patients’ and families’ acceptance of the dying process (Coetzee et al., 2003), but also helps physicians frame treatment goals more explicitly. In ALS patients, the relatively rapid and predictable disease progression and the limited treatment options at the end of life may force ALS specialists to discuss supportive care options in a more timely and frank manner.
Therefore, in all neurodegenerative diseases, neurologists, with their detailed knowledge of disease progression and complications, should be responsible for the timely initiation of discussions about potential treatment restrictions based on the patient’s goals for treatment. The sooner discussions are initiated, the stronger the patient’s acceptance of the medical prospects and the more stable the eventual treatment choices seem to be (Albert et al., 2001; Hirano and Yamazaki, 2010). Early and frank discussion about treatment options and end-of-life issues not only enable the patient and family to participate in difficult treatment decisions during serious complications at the end of life but also enable adequate control of symptoms throughout the course of the disease.
OPTIMIZING CARE OF THE DYING Beginning with the pioneering work of Cicely Saunders in the United Kingdom, clinicians trained in palliative care have clarified the characteristics of excellent care at the end of life (Saunders, 2000). This care should include meticulous attention to the physical, psychologic, social, and spiritual aspects of the patient’s life. Optimal end-of-life care is holistic and multidimensional, hence it should include the patient, the patient’s family, and the care team. Neurology teams inexperienced with optimal care of the dying patient have much to learn from palliative care experts. With the goal of providing a systematic documentation framework, several pathways for end-of-life care have been developed, such as the Liverpool Care Pathway (LCP: Ellershaw et al., 1997; Ellershaw and Ward, 2003). Although skeptics may fear that standardizing care for individual end-of-life situations may create problems, the experience of the LCP has been so positive in England that the LCP was a recommended standard tool within the U.K. End of Life Care Strategy (http://www.mcpcil.org. uk/liverpool-care-pathway/). Prompted by bad clinical care, very recently, a government review concluded that care and communication was more important than the use of an instrument (www.gov.uk/government/ publications/review-of-liverpool-care-pathway-for-dyingpatients), and that the LCP should be phased out. However, this care should be structured, for which the contents of the LCP is a good model.
Structure of care Since its beginning, the LCP has been updated and adapted to clinical experience. In its latest (12th) version, it provides a flowchart indicating how to diagnose the onset of the dying phase. The multidisciplinary care team first should address whether there may be a potentially reversible cause for the patient’s clinical decline, whether further diagnostic testing might be helpful, or whether the team would benefit from other
CARE OF THE DYING NEUROLOGIC PATIENT professionals’ advice before making the decision that the dying patient is in the final phase of life. This decision must be well documented and reassessed at least every 3 days. Clinical courses vary among patients, and some patients who clinically initially appear to be dying may recover to a more stable course. The care during the dying phase is then described through main goals. There may be good clinical reasons for not achieving certain goals, but the team should be aware of the reasons for not reaching a goal.
Goals and monitoring of care Goals for good care of the dying include the following: ● ● ● ● ● ● ● ● ● ● ● ●
The patient and relative are able to take a full and active part in communication. The patient and relative are aware of the approaching death. The team has up-to-date information about the patient’s primary relatives. The relatives have received information about help for them. The patient is given the opportunity to discuss what is important to him/her at this time. The patient has received as-necessary (prn) prescriptions for the main symptoms. A continuous subcutaneous infusion is available if needed. An implantable cardioverter-defibrillator has been deactivated if present. If ongoing, the need for clinically assisted nutrition and hydration has been reviewed. The patient’s skin integrity has been assessed. A full explanation of the current plan of care was given to the patient and relatives. The patient’s primary healthcare team/primary care practitioner is informed that the patient is dying.
The clinical status should be reviewed atleast every 4 hours. The time and situation of death are recorded, and additional goals are set for the care after death, such as: ● ● ●
Final care procedures are conducted according to policy and procedure. The relatives know what they will do next and are given relevant written information. The primary healthcare team/primary care practitioner is informed about the patient’s death.
Evidence The evidence for the usefulness of the LCP approach comes from clinical experience, mainly in the United Kingdom (Kinley et al., 2013). First, the LCP needs a clearly structured implementation process. However,
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this effort will teach teams some basic principles in palliative care but is more useful by increasing knowledge of symptom management and awareness of communication difficulties between nurses and physicians. In the United Kingdom, within the National Health Service, a recent national audit showed that, where LCP has been introduced, 80% of patients dying of cancer and 50% dying of noncancer illnesses died using the LCP (Pugh et al., 2010). The recent UK experience also tells us that good communication around dying is more important than just using a clinical tool. Currently, the LCP is in use in 20 countries, including New Zealand, where a national LCP office has been institutionalized to promote its use (Clark et al., 2012). The first data about its effects came from the Netherlands, where in a pre-post design they demonstrated that implementing the LCP led to a decrease in symptom burden, and an increase in the documentation of important issues in the dying phase (Veerbeek et al., 2008). Results from other controlled trials will be available soon, such as from the ongoing Swedish study in nursing homes, and from an Italian study using cluster randomization (Costantini et al., 2011). In the future, further evidence for improving care of the dying will result from the International OPCARE Research Collaboration (IORC) resulting from the EU-funded OPCARE9 group. OPCARE9 is a European collaboration that seeks to optimize research and clinical care for cancer patients in their last days of life. Further, the International LCP Reference Group plans to bridge the gap between new research evidence and the clinical use by regularly updating the clinical tool with new research evidence.
MEDICATION AT THE END OF LIFE Although there is no cure for patients suffering from neurodegenerative diseases, in many disorders like Parkinson’s disease or MS effective drug treatment can enhance the patient’s functional status by providing better symptom control, and may even prolong life. However, most of these drugs have been developed to influence the diseases at an early stage. In later stages, pharmaceutical options may be further limited by swallowing deficits, malabsorption, polypharmacy, or comorbidity. Especially in neurodegenerative diseases with deficits of swallowing, late-stage medication thus has to be applied via alternative routes, e.g., subcutaneous, transdermal, or per rectum. In Table 11.1, we summarize pharmaceutical symptom control in advanced neurodegenerative diseases, including alternative routes of administration. We do not differentiate between different disorders, since comparable symptoms often occur in late-stage neurodegeneration irrespective of the underlying disease.
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Table 11.1 Symptomatic treatment options in patients with advanced neurodegenerative diseases Symptom control in neurodegenerative diseases Constipation Ensure sufficient hydration Lactulose, prucalopride Dysphagia Compensatory swallowing maneuvers Feeding tube placement prior to respiratory compromise, based on individual decision Dietetics: high-protein, high-calorie diet, oral nutritional supplementation, carer education Dyspnea Opioids, either beginning with 2 mg morphine (e.g., 2 % solution) every 4 hours or Fentanyl patch (starting with 12.5 mg) Dyspnea-related anxiety Fast-acting anxiolytics as prn medication (e.g., sublingual lorazepam 1.0 mg) and/or Continuous benzodiazepines: lorazepam (1–2 mg) or midazolam (10–30 mg/day) p.o./s.c. “Death rattle” Butylscopolamine 20–40 mg/day Fatigue SSRIs (e.g. citalopram 20 mg/day) or tricyclic antidepressants Muscle cramps Quinidine sulfate 200 mg OD Pain Tilidine p.o., p.a. (600 mg/day) Metamizole p.o., p.a., s.c. (5000 mg/day) Amitriptyline p.o., p.a., s.c. (25–100 mg/day) Morphine, levomethadone Pseudobulbar affect First choice: dextromethorphan/quinidine 20 mg/10 mg OD, after 7 days BID SSRI Pseudohypersalivation Sage tea Amitriptyline, 25 mg OD Hyoscine hydrobromide transdermal patch, initially one-quarter patch daily Botulinum toxin A, e.g., 50 IU Dysport injected in the salivary glands Salivary gland radiotherapy Create a calm and stable environment; rule out infection, constipation, pain, urinary retention, Psychosis, agitation, pressure ulcers irritability, dysphoria, Quetiapine (25–50 mg/day), clozapine delirium Midazolam (start with low dose of 5–10 mg/day) Propofol (start with 0.5–1 mg/kg/hour) Clonazepam (0.25–1.5 mg), melatonin (3–12 mg) Rapid eye movement sleep behavior disorder Respiratory failure Noninvasive positive pressure ventilation Tracheostomy Symptomatic treatment with opioids, e.g., morphine (see dyspnea) Amantadine i.v., p.a. (600 mg/day) Rigidity, bradykinesia/ Rotigotine patch (2–8 mg/day) akinesia Apomorphine s.c. (6 mg/hour) Note: Severe akinesia is Midazolam i.v., s.c. (60 mg/day) rare Sleeplessness Zopiclone, zolpidem Amitriptyline (25 mg p.o., especially if hypersalivation is present), mirtazapine Quetiapine (25 mg p.o.) Spasticity Physiotherapy Baclofen (e.g., 10 mg TID) Tizanidine (e.g., 2 mg TID) Botulinum toxin A Thick secretions Ensure sufficient hydration Mucolytics: ambroxol, N-acetylcysteine Beta-antagonists Supportive measures: nebulized saline, manually assisted coughing techniques, modified postural drainage, humidification, mechanic insufflation–exsufflation devices, high-frequency chest wall oscillation Choice of therapy should be made with consideration of the optimal route of application and concomitant symptoms. SSRI, selective serotonin reuptake inhibitor.
CARE OF THE DYING NEUROLOGIC PATIENT
REFERENCES Albert SM, Murphy PL, Del Bene M et al. (2001). Incidence and predictors of PEG placement in ALS/MND. J Neurol Sci 191: 115–119. Burton CR, Payne S, Addington-Hall J et al. (2010). The palliative care needs of acute stroke patients: a prospective study of hospital admissions. Age Ageing 39: 554–559. Clark JB, Sheward K, Marshall B et al. (2012). Staff perceptions of end-of-life care following implementation of the Liverpool care pathway for the dying patient in the acute care setting: a New Zealand perspective. J Palliat Med 15: 468–473. Coetzee RH, Leask SJ, Jones RG (2003). The attitudes of carers and old age psychiatrists towards the treatment of potentially fatal events in end-stage dementia. Int J Geriatr Psychiatry 18: 169–173. Coppola KM, Ditto PH, Danks JH et al. (2001). Accuracy of primary care and hospital-based physicians’ predictions of elderly outpatients’ treatment preferences with and without advance directives. Arch Intern Med 161: 431–440. Costantini M, Ottonelli S, Canavacci L et al. (2011). The effectiveness of the Liverpool care pathway in improving end of life care for dying cancer patients in hospital. A cluster randomised trial. BMC Health Serv Res 11: 13. Ditto PH, Danks JH, Smucker WD et al. (2001). Advance directives as acts of communication: a randomized controlled trial. Arch Intern Med 161: 421–430. Ellershaw J, Ward C (2003). Care of the dying patient: the last hours or days of life. Br Med J 326: 30–34. Ellershaw J, Foster A, Murphy D (1997). Developing an integrated care pathway for the dying patient. Eur J Palliat Care 4: 203–207. Hirano Y, Yamazaki Y (2010). Ethical issues in invasive mechanical ventilation for amyotrophic lateral sclerosis. Nurs Ethics 17: 51–63. Hirst C, Swingler R, Compston DA et al. (2008). Survival and cause of death in multiple sclerosis: a prospective population-based study. J Neurol Neurosurg Psychiatry 79: 1016–1021.
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Hohlfeld R (2011). Update on sporadic inclusion body myositis. Brain 134: 3141–3145. Holloway RG, Ladwig S, Robb J et al. (2010). Palliative care consultations in hospitalized stroke patients. J Palliat Med 13: 407–412. Khot S, Billings M, Owens D et al. (2011). Coping with death and dying on a neurology inpatient service: death rounds as an educational initiative for residents. Arch Neurol 68: 1395–1397. Kinley J, Froggatt K, Bennett MI (2013). The effect of policy on end-of-life care practice within nursing care homes: A systematic review. Palliat Med 27: 209–220. Maessen M, Veldink JH, Onwuteaka-Philipsen BD et al. (2009). Trends and determinants of end-of-life practices in ALS in the Netherlands. Neurology 73: 954–961. Neudert C, Oliver D, Wasner M et al. (2001). The course of the terminal phase in patients with amyotrophic lateral sclerosis. J Neurol 248: 612–616. Pennington S, Snell K, Lee M et al. (2010). The cause of death in idiopathic Parkinson’s disease. Parkinsonism Relat Disord 16: 434–437. Pugh EJ, McEvoy M, Blenkinsopp J (2010). Use of the proportion of patients dying on an end of life pathway as a quality marker: considerations for interpretation. Palliat Med 24: 544–547. Roberts SE, Goldacre MJ (2003). Case fatality rates after admission to hospital with stroke: linked database study. Br Med J 326: 193–194. Saunders C (2000). The evolution of palliative care. Patient Educ Couns 41: 7–13. Shalowitz DI, Garrett-Mayer E, Wendler D (2006). The accuracy of surrogate decision makers: a systematic review. Arch Intern Med 166: 493–497. Veerbeek L, van Zuylen L, Swart SJ et al. (2008). The effect of the Liverpool Care Pathway for the dying: a multi-centre study. Palliat Med 22: 145–151. Voltz R (2010). Palliative care for multiple sclerosis: a counter-intuitive approach? Mult Scler 16: 515–517. Voltz R, Galushko M, Walisko J et al. (2011). Issues of “life” and “death” for patients receiving palliative care – comments when confronted with a research tool. Support Care Cancer 19: 771–777.
Chapter 11
Care of the dying neurologic patient ¨ BLING3, AND STEFAN LORENZL3 RAYMOND VOLTZ1,2*, GEORG NU Department of Palliative Medicine, University Hospital, Cologne, Germany
1
2
Center for Integrated Oncology, Cologne and Bonn, Germany
3
Interdisciplinary Center of Palliative Medicine, Ludwig Maximilians University, Munich, Germany
INTRODUCTION Professionals caring for dying neurologic patients may not be knowledgeable about the principles and practice of palliative care (see Chapter 10). Introducing a teaching conference for residents called “death rounds” is one possibility to share the emotions around a death in a group, learn from the experience together, and promote palliative care principles in a team of a neurology service (Khot et al., 2011). The pivotal point of death may be used as a lever to improve palliative care skills in existing services by including palliative care expertise. These rounds may be useful not only in the care of the dying patient but also earlier during the disease course.
CAUSES OF DEATH How do patients with neurologic disorders die and from what specific causes? The answers to these questions mostly may not be known to the specialist neurology services because many neurologists stop caring for patients when they can no longer attend outpatient clinics. These patients are then cared for at home or in nursing homes, with little or no supervision by palliative care or neurology. In addition to neurologic disorders classically considered a “palliative care diagnosis,” evidence is emerging that a palliative care approach is helpful in many more neurologic diseases. One common example is multiple sclerosis (MS) which is generally considered a disease that you “do not die from” (Voltz, 2010). However, MS patients are three times more likely to die prematurely than the age-matched general population (Hirst et al., 2008).
Another frequent neurologic diagnosis that is classically not considered for a palliative approach is ischemic or hemorrhagic stroke. About 30% of these patients die within the “acute stroke” phase, defined as the first 30 days after the event (Roberts and Goldacre, 2003), and stroke leaves another 30% permanently disabled with chronic deficits. Therefore, the stroke patient population has major palliative care needs. The early experience of palliative care services for stroke patients shows that these services help with symptom control, psychosocial issues, and end-of-life decisions, such as withdrawal of mechanic ventilation, artificial nutrition, tracheostomy, and neurosurgic procedures (Burton et al., 2010; Holloway et al., 2010). Even in rarer diagnoses of progressive neurologic disease, such as in sporadic inclusion body myositis, evidence is emerging that there are palliative care issues. Two recent long-term observational studies demonstrated a high symptom burden of patients in end-stage disease. In this series, 13% of patients died by euthanasia or terminal sedation, demonstrating the magnitude and frequency of suffering experienced by these patients at the end of their lives (Hohlfeld, 2011). The causes of death in neurologic disorders most often are those usually expected as complications of a chronic illness such as pneumonia (Hirst et al., 2008; Pennington et al., 2010). However, physicians also are involved in accelerating the deaths of some patients by euthanasia or palliative sedation, as has been reported in countries such as the Netherlands, where active euthanasia and assisted suicide are legal. There, these practices are performed more frequently in neurologic patients than in those with cancer. Whereas about 1% of the cancer population dies by euthanasia, this action accounts for death in about 20% in patients with
*Correspondence to: Raymond Voltz, Prof., Dr. med., Department of Palliative Medicine, University Hospital Cologne, Kerpener Straße 62, 50937 Cologne, Germany. Tel: 0049-221-478-3361, E-mail: [email protected]
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amyotrophic lateral sclerosis (ALS). This number has been stable in recent years. An additional 15% die after palliative sedation and that percentage is increasing (Maessen et al., 2009). In contrast to common lay and professional beliefs, patients with ALS do not suffocate, and usually die a peaceful natural death when under hospice care and also under the care of a neurology service (Neudert et al., 2001). Neurologic patients outside the Netherlands frequently express a wish to hasten death or suicide. In MS, suicide is a frequent cause of death, and a long protracted course with severe neurologic deficit in many patients leads to a wish for hastening death. In these patients, however, a palliative care approach may identify the reasons motivating this wish. With the assistance and support of a palliative care team, many patients regain the will for life (Voltz et al., 2011).
END-OF-LIFE DECISIONS End-of-life decision-making is a complicated and poorly understood process which places a significant burden on all parties involved. Clinicians frequently rely on surrogate decision-makers in end-of life treatment, particular in patients with neurodegenerative diseases. Often physicians do not address the patient’s preferences related to family involvement in decision-making. Physicians should discuss medical treatment goals with patients and their family members at the right time in order to participate in treatment and nontreatment decisions. Family members make up the vast majority of decision-makers and families do not use pure substituted judgments when making decisions on behalf of their loved ones. Some patients desire to have a more active role, even in advanced stages of their disease, when communication becomes difficult. Furthermore, several studies suggest that surrogates may be unable to predict patients’ preferences accurately (Shalowitz et al., 2006). Even the presence of advance directives does not improve the accuracy of surrogates’ predictions of patients’ preferences (Coppola et al., 2001; Ditto et al., 2001). Patients with ALS and MS often retain their capacity to make medical decisions for themselves even into the late stages of their illness. However, in patients with Parkinson’s disease, atypical parkinsonian disorders, and dementia, clinicians often must rely on surrogates because the patients are incapacitated. Frank communication about end-of-life issues not only seems to support patients’ and families’ acceptance of the dying process (Coetzee et al., 2003), but also helps physicians frame treatment goals more explicitly. In ALS patients, the relatively rapid and predictable disease progression and the limited treatment options at the end of life may force ALS specialists to discuss supportive care options in a more timely and frank manner.
Therefore, in all neurodegenerative diseases, neurologists, with their detailed knowledge of disease progression and complications, should be responsible for the timely initiation of discussions about potential treatment restrictions based on the patient’s goals for treatment. The sooner discussions are initiated, the stronger the patient’s acceptance of the medical prospects and the more stable the eventual treatment choices seem to be (Albert et al., 2001; Hirano and Yamazaki, 2010). Early and frank discussion about treatment options and end-of-life issues not only enable the patient and family to participate in difficult treatment decisions during serious complications at the end of life but also enable adequate control of symptoms throughout the course of the disease.
OPTIMIZING CARE OF THE DYING Beginning with the pioneering work of Cicely Saunders in the United Kingdom, clinicians trained in palliative care have clarified the characteristics of excellent care at the end of life (Saunders, 2000). This care should include meticulous attention to the physical, psychologic, social, and spiritual aspects of the patient’s life. Optimal end-of-life care is holistic and multidimensional, hence it should include the patient, the patient’s family, and the care team. Neurology teams inexperienced with optimal care of the dying patient have much to learn from palliative care experts. With the goal of providing a systematic documentation framework, several pathways for end-of-life care have been developed, such as the Liverpool Care Pathway (LCP: Ellershaw et al., 1997; Ellershaw and Ward, 2003). Although skeptics may fear that standardizing care for individual end-of-life situations may create problems, the experience of the LCP has been so positive in England that the LCP was a recommended standard tool within the U.K. End of Life Care Strategy (http://www.mcpcil.org. uk/liverpool-care-pathway/). Prompted by bad clinical care, very recently, a government review concluded that care and communication was more important than the use of an instrument (www.gov.uk/government/ publications/review-of-liverpool-care-pathway-for-dyingpatients), and that the LCP should be phased out. However, this care should be structured, for which the contents of the LCP is a good model.
Structure of care Since its beginning, the LCP has been updated and adapted to clinical experience. In its latest (12th) version, it provides a flowchart indicating how to diagnose the onset of the dying phase. The multidisciplinary care team first should address whether there may be a potentially reversible cause for the patient’s clinical decline, whether further diagnostic testing might be helpful, or whether the team would benefit from other
CARE OF THE DYING NEUROLOGIC PATIENT professionals’ advice before making the decision that the dying patient is in the final phase of life. This decision must be well documented and reassessed at least every 3 days. Clinical courses vary among patients, and some patients who clinically initially appear to be dying may recover to a more stable course. The care during the dying phase is then described through main goals. There may be good clinical reasons for not achieving certain goals, but the team should be aware of the reasons for not reaching a goal.
Goals and monitoring of care Goals for good care of the dying include the following: ● ● ● ● ● ● ● ● ● ● ● ●
The patient and relative are able to take a full and active part in communication. The patient and relative are aware of the approaching death. The team has up-to-date information about the patient’s primary relatives. The relatives have received information about help for them. The patient is given the opportunity to discuss what is important to him/her at this time. The patient has received as-necessary (prn) prescriptions for the main symptoms. A continuous subcutaneous infusion is available if needed. An implantable cardioverter-defibrillator has been deactivated if present. If ongoing, the need for clinically assisted nutrition and hydration has been reviewed. The patient’s skin integrity has been assessed. A full explanation of the current plan of care was given to the patient and relatives. The patient’s primary healthcare team/primary care practitioner is informed that the patient is dying.
The clinical status should be reviewed atleast every 4 hours. The time and situation of death are recorded, and additional goals are set for the care after death, such as: ● ● ●
Final care procedures are conducted according to policy and procedure. The relatives know what they will do next and are given relevant written information. The primary healthcare team/primary care practitioner is informed about the patient’s death.
Evidence The evidence for the usefulness of the LCP approach comes from clinical experience, mainly in the United Kingdom (Kinley et al., 2013). First, the LCP needs a clearly structured implementation process. However,
143
this effort will teach teams some basic principles in palliative care but is more useful by increasing knowledge of symptom management and awareness of communication difficulties between nurses and physicians. In the United Kingdom, within the National Health Service, a recent national audit showed that, where LCP has been introduced, 80% of patients dying of cancer and 50% dying of noncancer illnesses died using the LCP (Pugh et al., 2010). The recent UK experience also tells us that good communication around dying is more important than just using a clinical tool. Currently, the LCP is in use in 20 countries, including New Zealand, where a national LCP office has been institutionalized to promote its use (Clark et al., 2012). The first data about its effects came from the Netherlands, where in a pre-post design they demonstrated that implementing the LCP led to a decrease in symptom burden, and an increase in the documentation of important issues in the dying phase (Veerbeek et al., 2008). Results from other controlled trials will be available soon, such as from the ongoing Swedish study in nursing homes, and from an Italian study using cluster randomization (Costantini et al., 2011). In the future, further evidence for improving care of the dying will result from the International OPCARE Research Collaboration (IORC) resulting from the EU-funded OPCARE9 group. OPCARE9 is a European collaboration that seeks to optimize research and clinical care for cancer patients in their last days of life. Further, the International LCP Reference Group plans to bridge the gap between new research evidence and the clinical use by regularly updating the clinical tool with new research evidence.
MEDICATION AT THE END OF LIFE Although there is no cure for patients suffering from neurodegenerative diseases, in many disorders like Parkinson’s disease or MS effective drug treatment can enhance the patient’s functional status by providing better symptom control, and may even prolong life. However, most of these drugs have been developed to influence the diseases at an early stage. In later stages, pharmaceutical options may be further limited by swallowing deficits, malabsorption, polypharmacy, or comorbidity. Especially in neurodegenerative diseases with deficits of swallowing, late-stage medication thus has to be applied via alternative routes, e.g., subcutaneous, transdermal, or per rectum. In Table 11.1, we summarize pharmaceutical symptom control in advanced neurodegenerative diseases, including alternative routes of administration. We do not differentiate between different disorders, since comparable symptoms often occur in late-stage neurodegeneration irrespective of the underlying disease.
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Table 11.1 Symptomatic treatment options in patients with advanced neurodegenerative diseases Symptom control in neurodegenerative diseases Constipation Ensure sufficient hydration Lactulose, prucalopride Dysphagia Compensatory swallowing maneuvers Feeding tube placement prior to respiratory compromise, based on individual decision Dietetics: high-protein, high-calorie diet, oral nutritional supplementation, carer education Dyspnea Opioids, either beginning with 2 mg morphine (e.g., 2 % solution) every 4 hours or Fentanyl patch (starting with 12.5 mg) Dyspnea-related anxiety Fast-acting anxiolytics as prn medication (e.g., sublingual lorazepam 1.0 mg) and/or Continuous benzodiazepines: lorazepam (1–2 mg) or midazolam (10–30 mg/day) p.o./s.c. “Death rattle” Butylscopolamine 20–40 mg/day Fatigue SSRIs (e.g. citalopram 20 mg/day) or tricyclic antidepressants Muscle cramps Quinidine sulfate 200 mg OD Pain Tilidine p.o., p.a. (600 mg/day) Metamizole p.o., p.a., s.c. (5000 mg/day) Amitriptyline p.o., p.a., s.c. (25–100 mg/day) Morphine, levomethadone Pseudobulbar affect First choice: dextromethorphan/quinidine 20 mg/10 mg OD, after 7 days BID SSRI Pseudohypersalivation Sage tea Amitriptyline, 25 mg OD Hyoscine hydrobromide transdermal patch, initially one-quarter patch daily Botulinum toxin A, e.g., 50 IU Dysport injected in the salivary glands Salivary gland radiotherapy Create a calm and stable environment; rule out infection, constipation, pain, urinary retention, Psychosis, agitation, pressure ulcers irritability, dysphoria, Quetiapine (25–50 mg/day), clozapine delirium Midazolam (start with low dose of 5–10 mg/day) Propofol (start with 0.5–1 mg/kg/hour) Clonazepam (0.25–1.5 mg), melatonin (3–12 mg) Rapid eye movement sleep behavior disorder Respiratory failure Noninvasive positive pressure ventilation Tracheostomy Symptomatic treatment with opioids, e.g., morphine (see dyspnea) Amantadine i.v., p.a. (600 mg/day) Rigidity, bradykinesia/ Rotigotine patch (2–8 mg/day) akinesia Apomorphine s.c. (6 mg/hour) Note: Severe akinesia is Midazolam i.v., s.c. (60 mg/day) rare Sleeplessness Zopiclone, zolpidem Amitriptyline (25 mg p.o., especially if hypersalivation is present), mirtazapine Quetiapine (25 mg p.o.) Spasticity Physiotherapy Baclofen (e.g., 10 mg TID) Tizanidine (e.g., 2 mg TID) Botulinum toxin A Thick secretions Ensure sufficient hydration Mucolytics: ambroxol, N-acetylcysteine Beta-antagonists Supportive measures: nebulized saline, manually assisted coughing techniques, modified postural drainage, humidification, mechanic insufflation–exsufflation devices, high-frequency chest wall oscillation Choice of therapy should be made with consideration of the optimal route of application and concomitant symptoms. SSRI, selective serotonin reuptake inhibitor.
CARE OF THE DYING NEUROLOGIC PATIENT
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