- Email: [email protected]
Breaking the news in amyotrophic lateral sclerosis
Journal of the Neurological Sciences 160 (Suppl. 1) (1998) S127–S133
Breaking the news in amyotrophic lateral sclerosis a, b c Gian Domenico Borasio *, Richard Sloan , Dieter E. Pongratz a
¨ , Germany Department of Neurology, Klinikum Grosshadern, Ludwig-Maximilians-University, D-81366 Munchen b Joseph Weld Hospice, Dorchester, Dorset DT1 2 SL, UK c ¨ , Germany Friedrich-Baur-Institute, Ludwig-Maximilians-University, D-80336 Munchen
Abstract Telling the diagnosis to patients with amyotrophic lateral sclerosis (ALS) is a daunting task for any neurologist. Obviously, breaking the news in ALS is not a standardizable procedure. However, proven techniques exist to reduce the trauma to the patient and ease the burden on the doctor, thus reducing the risk of burnout and the tendency to ‘pull away’ from the patient. Such communication skills are of fundamental importance to clinical practice and should be more prominent in medical teaching. The way the patient is told the diagnosis is now recognized to be the first and one of the most delicate steps in palliative care. Information is best offered in a stepwise fashion at the patient’s pace with an emphasis on positive aspects, and in the presence of the patient’s family. Reviewing available therapeutic options and current research efforts may foster hope for the future, while pointing out that almost all symptoms of ALS can be alleviated by palliative therapy may help to reduce fears. Encouraging patients to ask questions and disclose anxieties is important for their psychological wellbeing. Available options for mechanical ventilation should be reviewed early enough to allow for unhurried decision-making. We believe that the terminal phase of the disease should be discussed at the latest when dyspneic symptoms appear, in order to prevent unwarranted fears of ‘choking to death’. 1998 Elsevier Science B.V. All rights reserved. Keywords: Bad news; Communication skills; Motor neurone disease; Palliative care; Telling the diagnosis
1. Introduction Amyotrophic lateral sclerosis (ALS), also known as motor neurone disease, is a degenerative disorder involving central and peripheral motor neurons which leads to death within 3–5 years from onset [1]. The unenviable task of telling a patient that he / she has got ALS generally falls to the neurologist diagnosing it. Whilst the messenger can get blamed for bad news, there is anecdotal and researchbased evidence that it is not always performed optimally [2,3]. Because of the poor prognosis there has been reluctance in the past to tell the patient the diagnosis. Often, the patient was told ‘reassuring’ statements, while the relatives were informed more fully, with the addendum ‘‘there is nothing that can be done for him / her’’. This practice still exists, although most clinicians now respect the ethical principle of autonomy in their patients. The majority of patients do want to have information on their condition, even if it is life-threatening [4,5]. Without this, the stress they suffer through uncertainty is often *Corresponding author. Tel.: 149-89-70953671; 70953677; e-mail: [email protected]
fax:
149-89-
worse than knowing they will die. One in five patients with cancer develop full-blown psychiatric disorders [6]. The main predictive factor for this is the way in which bad news is broken [7]. The feeling of helplessness present on both sides when the diagnosis of ALS is disclosed should not lead to withholding information that might be essential for the patient’s life planning [8]. Breaking the news is an ongoing process throughout the course of the disease: it is not limited to the communication of the diagnosis (which in itself is a multi-step procedure), but encompasses all the aspects of the information flow from the physician to the patient regarding the disease process, thus including good as well as bad news. This paper offers some suggestions on how to break the news in ALS and how training in communication skills could help both neurologists and the patients they serve.
2. Background Johnston and co-workers [3] interviewed 50 patients about their experiences of being told they had ALS. Most
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Table 1 Communicating the diagnosis of ALS (after Ref. [3]) ? Most people see positive aspects on being told the diagnosis, especially as it provides a label for the condition ? People prefer the diagnosis to be communicated in a direct, empathetic style ? Being able to ask questions is important ? Doctors should guard against giving too pessimistic a message all at once ? Information on where to get further help (e.g. from a patients’ association) is important at the time of diagnosis ? People generally prefer to be told with someone else present
patients saw the providing of a label for their condition as a positive thing – ‘‘at least I now know what I have to contend with’’. Negative aspects included: being told too late – ‘‘I could have used the past year better if I had known it might have been my last’’; being told the diagnosis without the option of having a relative present; lack of privacy (e.g. in the open wards); being told in vague or confusing terms; too much detail all at once. Although there was no evidence that poor communication at the time of diagnosis was responsible for prolonged mood disturbance, patients may well have undergone unnecessary psychological trauma at the time they were told. A summary of the findings is shown in Table 1. Lack of adequate communication skills also leads to stress in doctors [9]. Table 2 outlines the reasons why breaking bad news is difficult. Burnout is more likely amongst consultants who feel insufficiently trained in communication skills [10]. As the UK Health Service Commissioner [11] points out, improvement in communications between health care workers, patients and relatives would result in a marked reduction in the number of complaints reported to him. Table 2 outlines the reasons why breaking bad news is difficult.
3. What to tell
3.1. Minimum information After the diagnosis is firmly established, the patient should be informed that he / she has a progressive disease of the motor nerves, for which no curative therapy is available. The name of the disease must be told and explained, to avoid confusion, e.g. with multiple sclerosis. If the family history is negative, it is reassuring for patient and family to know that their children are unlikely to be at risk. Positive aspects (e.g. no pain, no disturbances in Table 2 Why breaking bad news is difficult for doctors (after Ref. [30]) ? Fear of the messenger getting blamed for bad news ? Perceived lack of time ? Lack of training ? Fear of causing distress ? Fear of being asked difficult questions ? Fear of not having all the answers ? Invoking fears of one’s own mortality
sensation, cognition, memory, personality, continence, etc.) should be stressed, as well as the availability of efficient palliative measures for practically all symptoms of ALS [12]. Current research efforts and, where available, the possibility of taking part in clinical studies of new drugs should be pointed out as a means to foster hope.
3.2. Beginning of the disease Patients often suspect a connection between the outbreak of the disease and a specific event (e.g. accident, operation, private or professional crises, etc.). This question should be addressed, and patients should be told that the disease starts many years if not decades before the first symptoms show up. Since at least 50% of the spinal motor neurons have to degenerate prior to clinical manifestation, it is impossible to establish when the disease began in any single patient, even with a large degree of approximation.
3.3. Prognosis Many ALS patients ask directly about their remaining life span, since, at the time of diagnosis, they have usually witnessed the progression of the disease for several months if not years. The answer to this question should include the information that there are no sudden worsenings to be expected, that periods of relative stabilization may occur and that rare remissions have been observed. The patients should be told that the course of ALS may vary between months and decades (e.g. Stephen Hawking), making a firm statement on prognosis all but impossible for any single patient. In our experience, many patients appreciate a rough approximation, for example ‘‘I think you have probably got several years rather than several months left’’. This allows them to plan the rest of their lives and is more helpful than an outright refusal to speculate. However, if a patient explicitly asks for available statistics, he / she has the right to know. Careful questioning about suicidal thoughts may be advisable. We believe that such acknowledgement may reduce rather than increase suicidal attempts.
3.4. Available therapies Since 1996, the first substance with a modest lifeprolonging effect in ALS has been on the market (Riluzole [13]). Subject to availability of reimbursement for this
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drug, an open discussion on its pros and cons should take place. Patients must know that they will usually not notice any subjective change in the progression of their disease. The possible side effects (nausea, asthenia, dizziness) should be mentioned. This discussion avoids raising false hopes, which might otherwise eventually lead to disillusion and frustration.
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ALS, will want a second opinion. Again, this is more than understandable and should be addressed directly by the physician who is breaking the news. Possible tertiary referral centers should be discussed with the patient. This may strengthen the doctor–patient relationship and help to avoid the patient self-referring from one doctor to another.
3.8. If the diagnosis is only suspected 3.5. ‘ Unconventional’ treatments It is all too understandable that patients with ALS start looking for help outside the boundaries of ‘classical’ medicine. This topic should therefore be addressed the first time the diagnosis is told. Patients should be reassured that their physician does not object to their trying homeopathic medication, acupuncture, etc. if they so wish. However, patients and families must be warned that some alternative methods (e.g. ‘fresh cell therapy’, snake toxins) may be dangerous to their health, while others may entail serious financial consequences for the benefit of charlatans and common criminals (including, sadly, some physicians).
3.6. Patients’ associations Patients may obtain invaluable help (indeed, more than their doctors can usually offer) from the ALS patients’ associations, which are present in almost every major country in the world and are grouped together in the Alliance of ALS / MND associations (Table 3). It is therefore, to our mind, absolutely mandatory to inform patients and families from the beginning about the existence of these associations, provide them with the relevant addresses and telephone numbers, and explicitly encourage them to get in touch with the association.
3.7. Second opinion Many patients, when confronted with a diagnosis like
What has been said so far presupposes an unequivocal clinical diagnosis of ALS, i.e. ‘probable’ or ‘definite’ ALS according to the El Escorial criteria of the World Federation of Neurology [14]. If these criteria are not met, the physician will obviously take a more careful approach, depending on the degree of clinical certainty. Possible differential diagnoses and the diagnostic plan must first be discussed with the patient. However, if the El Escorial criteria for ‘possible’ ALS are fulfilled, information on appropriate palliative measures, available drug options and therapeutic studies should take place, since these patients rarely turn out to have something else.
3.9. Discussing mechanical ventilation and the terminal phase For many ALS patients, the onset of dyspneic symptoms marks a turning point during the course of their disease. Often, the first dyspneic bouts happen after choking on food, on exertion or during sleep. At the beginning of clinically manifest involvement of the respiratory muscles, anxiety is usually the most prominent symptom during dyspneic attacks (which therefore respond very well to short-acting benzodiazepines, e.g. lorazepam 0.5–1 mg sublingually). If the subject has not been discussed previously, we believe that patients should receive information on the terminal phase of the disease at the latest when the first dyspneic symptoms appear. The reason for this recommendation is that almost all patients, when
Table 3 Patients’ associations United Kingdom
Motor Neurone Disease Association (MNDA) and International Alliance of ALS / MND associations
PO Box 246, Northampton NN1 2PR
Tel.: 144 1604 250505 Fax: 144 1604 24726 Website: www.alsmndalliance.org
Germany
¨ Muskelkranke (DGM) Deutsche Gesellschaft fur
Im Moos 4, D-79112 Freiburg
Tel.: 149 7665 9447-0 Fax: 149 7665 9447-20 E-mail: dgm [email protected] ] Website: www.dgm.org
USA
Muscular Dystrophy Association (MDA)
3300 East Sunrise Dr., Tucson, AZ 85718
Tel.: 11 520 529 2000 Fax: 11 520 529 5300 E-mail: [email protected] Website: www.mdausa.org
USA
ALS Association (ALSA)
21021 Ventura Blvd., Suite [321, Woodland Hills, CA 91364
Tel.: 11 818 340 7500 Fax: 11 818 340 2060 E-mail: [email protected] Website: www.alsa.org
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questioned at this stage, report fears of ‘‘choking to death’’. In our experience, describing the physiological mechanism leading to terminal hypercapnic coma and the resulting peaceful death in sleep will relieve these fears in most cases. Patients and relatives must also be informed that the array of medication available in the terminal phase, if correctly applied, does prevent suffering [15]. This information needs to be reiterated in subsequent visits. At this stage, patients should be asked whether they would wish to be intubated and ventilated in the event of a terminal respiratory insufficiency. Patients who have been informed about the possible subsequent clinical course, which may end up in a ‘locked-in’ syndrome on an intensive care unit [16], will usually refuse such a procedure. This refusal must be documented by the physician and is often best incorporated into a living will. The consequences of such a decision must be discussed with patient, family and the family physician (e.g. concerning the use of opioids in the terminal phase [17]). Symptoms of chronic nocturnal hypoventilation (Table 4) may precede the onset of clinically overt respiratory insufficiency by months or even years, and can be efficiently palliated by non-invasive home mechanical ventilation (HMV) [18,19]. These symptoms must be carefully searched for by the physician at every visit, since some patients will deny any respiratory trouble even in advanced stages of chronic hypoventilation. As soon as these symptoms are recognized (which may be before or after the first dyspneic bouts), non-invasive HMV should be discussed, emphasizing that the primary aim of this measure is palliation rather than prolongation of life. The patient should also be informed that eventually a decision on whether or not to perform tracheostomy will become necessary (see above). The slope of decline in forced vital capacity (FVC) is a better predictor of respiratory failure than the absolute value. A significant drop in FVC when measured supine vs. sitting or standing is also a reliable sign of present or impending hypoventilation, as is an FVC value of ,50% of predicted (except for patients with slow progression, who may be asymptomatic at very low FVCs, due to
Table 4 Symptoms of chronic hypoventilation (from Ref. [12]) ? Daytime fatigue and sleepiness, concentration problems ? Difficulty falling asleep, disturbed sleep, nightmares ? Morning headache ? Nervousness, tremor, increased sweating, tachycardia ? Depression, anxiety ? Tachypnea, dyspnea, phonation difficulties ? Visible efforts of auxiliary respiratory muscles ? Reduced appetite, weight loss, recurrent gastritis ? Recurrent or chronic upper respiratory tract infections ? Cyanosis, oedema ? Vision disturbances, dizziness, syncope ? Diffuse pain in head, neck and extremities
adaptation). These data may serve as additional cues as to when to address the respiratory issue in ALS patients. The overall aim is to discuss the available options early enough, so that patient and family may have sufficient time to make their decision.
4. How to tell Maguire and co-workers [20] have outlined the factors to be considered when breaking bad news (Table 5), which will be discussed in detail below. They define three underlying objectives in the process:
• do not withhold information if the patient wants it; • do not impose information if the patient does not want it; • gauge and respond to the patient’s reaction to the news.
4.1. Setting In some countries (e.g. USA), diagnostic procedures for ALS are usually performed in an outpatient setting, while in others (e.g. Germany and UK), patients are usually referred to a Neurology Department for inpatient workup once ALS is suspected. The latter procedure gives the physician the possibility of a stepwise offering of information over several days, thereby allowing the patient to reflect overnight and ask additional questions. If the diagnosis is told in an outpatient setting, a short-term follow-up visit should be scheduled. The discussion should take place in a private, quiet room. The middle of an open ward is far from ideal. If at all possible, interruptions (phone, beeper) should be avoided. Provided the patient agrees, the diagnosis is best told in the presence of the family to avoid misunderstandings. Roger Carus, a patient who wrote about his experience of being diagnosed with ALS in the mid-seventies, found out inadvertently whilst in hospital for investigations. He was not sure whether or not his wife had been told the diagnosis by his neurologist and recalls ‘‘I spent two of the most agonising weeks of my life trying to find out whether she knew or not’’ [2]. Table 5 The stages in breaking bad news (after Ref. [20]) ? Setting ? Finding out what the patient already knows or suspects ? Finding out how much more they want to know ? Firing the warning shot ? Hierarchy of euphemisms ? Observing and responding to the patient’s reactions at each step ? Contract for the future
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4.2. Finding out what the patient already knows or suspects For example: ‘‘What do you make of your illness / investigations?’’; ‘‘Did you ever think that something serious might be going on?’’ This second question will not perturb patients who are using denial as a coping mechanism – they will be able to shrug it off as not being applicable to them.
4.3. Finding out how much more the patient wants to know ‘‘If you had something seriously wrong with you, are you the sort of person who likes to know exactly what is going on or just the treatment plan?’’ Again, those not wanting to face the reality will opt for the latter without being caused undue distress.
4.4. Firing the warning shot ‘‘I am afraid the results of the tests were not as good as I was hoping for’’. This breaks the unsuspecting patient in gently.
4.5. Hierarchy of euphemisms Small pieces of information of increasing seriousness are told to the patient, with pauses in between for the patient to digest them. This avoids extreme distress and the patient ‘switching off’ to any further explanation through shock. For example: ‘‘The tests we have performed show you have a problem with the nerves which transmit instructions to the muscles’’; ‘‘The nerves are gradually being destroyed so that your muscles can’t work properly’’; ‘‘The condition you have got is called amyotrophic lateral sclerosis, or motor neurone disease’’; ‘‘Unfortunately, there is no known cure at the moment, although there is a drug (Riluzole) which modestly slows the deterioration down’’; ‘‘Yes, the disease is eventually fatal’’.
4.6. Observing and responding to patient’ s reactions at each step In order not to impose information that the patient is not yet ready for, the doctor pauses and waits for a reaction at each step. If the patient asks a question such as ‘‘What do you mean?’’ this is a clear invitation for further information. Conversely, if the patient expresses distress either verbally or non-verbally, it is important not to proceed but to sensitively explore with the patient the reason for their distress. This may appear obvious, but often patients may make false inferences about which the doctor can be truthfully reassuring, e.g. that they are going to die next week. When a patient breaks down, it is important to acknowl-
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edge the distress – touch may be the most empathetic way of doing this. If the physician is not sure whether to continue, one possible question could be ‘‘Have you had enough for now or are there further questions you would like to ask?’’
4.7. Contract for the future Phrases like ‘‘I’m afraid there’s nothing more we can do’’ must be avoided – patients feel devastated, presuming falsely that they are condemned to intractable suffering. We have found it helpful to demonstrate concern for the patient by informing them of sources of help and support, as outlined above, and arranging a short-term follow-up meeting. Often, patients tend to forget information, even if they have fully understood it when it was first told. It is therefore important, on each clinic visit, to find out time and again where the patient stands and to move on jointly from there.
4.8. Reinforcement of information Evidence from the cancer literature suggests that patients might profit from being handed out an audiotape of the consultation or receiving a letter outlining the consultation in lay terms, with a preference for the former [21]. This approach has not yet been tested in ALS patients. Our own data in a small sample of 39 ALS patients and 19 relatives (Table 6) show that (i) about 50% of patients and relatives felt that they received no or insufficient information from their physician at the time of diagnosis; (ii) a brochure containing general information on ALS in lay terms was regarded to be informative by almost all patients and relatives; (iii) however, five of 39 patients and five of 19 relatives did not think that receiving the brochure had helped them in any way, and one couple said that they wished they had never read it. Therefore, handing out written information material after the consultation may be helpful for many patients, but it might be advisable to ask patient and relatives whether they feel that they are ready for it or whether they would prefer to receive the material at a later stage. Table 6 Patient survey: information on the diagnosis and satisfaction with ALS brochure [39 ALS patients (age 35–80, 29 m, 10 f), 19 relatives (age 28–76, 5 m, 14 f)] ALS patients
Relatives
Information received on the diagnosis prior to receiving the brochure None 7 / 39 (18%) 3 / 19 (16%) Insufficient 12 / 39 (31%) 7 / 19 (37%) Sufficient 20 / 39 (51%) 9 / 19 (47%) Satisfaction with the brochure Informative 38 / 39 (97%) Helpful 34 / 39 (87%)
18 / 19 (95%) 14 / 19 (74%)
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5. Learning communication skills In 1981, only 30% of medical schools in the UK gave any teaching in communication skills, but now all but one teach the subject formally. The Royal College of Physicians now recommends the approach adopted in some schools where communication skills are taught throughout the clinical course rather than in one or two modules only. In common with the Royal Colleges of Psychiatrists and General Practitioners, it also suggests that postgraduate examinations should include formal mechanisms such as video tape recordings for assessing communication skills [22]. Walker [23] found that, of 27 consultants questioned, only two had been on formal communication skills courses since qualifying. Several expressed interest if such a course was to be organised locally, but the majority implied that communications skills could not be taught and could only be picked up ‘on the job’. With bad habits as well as good being picked up from observing others at work, perhaps it is not surprising that poor technique is perpetuated in successive generations of doctors. Studies have shown significant improvement in communication abilities in those who have been taught the relevant skills [24], although these skills survive better if they are periodically reinforced [20]. To be properly acquired, the skills should be practised in a safe environment – safe for those learning them and safe for patients. Video recordings of role-played situations using actors or the students themselves can be particularly useful provided they are handled sensitively. Teachers of communication skills must be trained in the ‘rules of feedback’. Instead of focusing on students’ weaknesses, the strengths they already possess are reinforced and alternative methods explored where difficulties are encountered. This way, role play becomes a positive learning experience and not the negative one so many students seem to recall.
6. Conclusion Whilst breaking the news in ALS will never be easy, proven techniques exist to reduce the trauma to the patient and ease the burden on the doctor. Such communication skills are of fundamental importance to clinical practice and should be more prominent in both undergraduate and postgraduate teaching. The way the patient is told the diagnosis is now recognized to be the first and one of the most sensitive steps in palliative care [25]. However, empirical evidence on the consequences of how the diagnosis is told is scanty throughout medicine [26,27]. Although we cite research evidence equating good communication techniques to reduced psychological morbidity in patients, more studies are required to substantiate best practice and teaching techniques. To quote from Caplan [28], ‘‘The increased number of diagnostic and treatment options makes even more crucial the physician’s skill in
managing illness and the art of communicating with patients and their loved ones. There but for the grace of God go we all, for all of us and our families are, or will eventually become, patients.’’
Acknowledgements This paper is based, in part, on a consensus document of the German Neuromuscular Centers of the Deutsche ¨ Muskelkranke (Freiburg, Germany) [29]. Gesellschaft fur We thank Prof. Th. Brandt, Prof. K. Kunze, Prof. B. ¨ Neundorfer and Prof. K.V. Toyka for their contributions, Dr D. Newman for helpful discussions and M. Wasner for editorial assistance. Special thanks are due to Dr R.G. Miller for critical review of the manuscript.
References [1] Mitsumoto H, Chad DA, Pioro EP. Amyotrophic lateral sclerosis. Philadelphia: F.A. Davis Company, 1998. [2] Carus R. Motor neurone disease: a demeaning illness. Br Med J 1980;280:455–6. [3] Johnston M, Earll L, Mitchell E, Morrison V, Wright S. Communicating the diagnosis of motor neurone disease. Palliative Med 1996;10:23–34. [4] Ley P. Communication with patients: improving communication, satisfaction and compliance. London: Croon Helm, 1998. [5] Silverstein MD, Stocking CB, Antel JP, Beckwith J, Roos RP, Siegler M. Amyotrophic lateral sclerosis and life-sustaining therapy: patients’ desire for information, participation in decision making, and life-sustaining therapy. Mayo Clin Proc 1991;66:906–13. [6] Parle M, Jones B, Maguire GP. Maladaptive coping and affective disorders in cancer patients. Psychol Med 1996;26:735–44. [7] Fallowfield LJ, Hall A, Maguire GP, Baum M. Psychological outcomes of different treatment policies in women with early breast cancer outside a clinical trial. Br Med J 1990;301:575–80. [8] Meininger V. Breaking bad news in amyotrophic lateral sclerosis. Palliative Med 1993;7:37–40. [9] Davis H, Fallowfield L. Counselling and communication in health care. Chichester: Wiley, 1991. [10] Ramirez AJ, Graham J, Richards MA, Cull A, Gregory WM. Mental health of hospital consultants: the effects of stress and satisfaction at work. Lancet 1996;347:724–8. [11] Health Service Commissioner. Third report for session 1990–1991. London: HMSO, 1991. [12] Borasio GD, Voltz R. Palliative care in amyotrophic lateral sclerosis. J Neurol 1997;244:S11–7. [13] Lacomblez L, Bensimon G, Leigh PN, Guillet P, Meininger V. Dose-ranging study of riluzole in amyotrophic lateral sclerosis. Lancet 1996;347:1425–31. [14] Brooks BR. El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. J Neurol Sci 1994;124:96–107. [15] O’Brien T, Kelly M, Saunders C. Motor neuron disease: a hospice perspective. Br Med J 1992;304:471–3. [16] Hayashi H, Shuuichi K, Kawada A. Amyotrophic lateral sclerosis patients living beyond respiratory failure. J Neurol Sci 1991;105:73–8. [17] Voltz R, Borasio GD. Palliative therapy in the terminal stage of neurologic disease. J Neurol 1997;244:S2–S10. [18] Cazzolli PA, Oppenheimer EA. Home mechanical ventilation for
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amyotrophic lateral sclerosis: nasal compared to tracheostomy-intermittent positive pressure ventilation. J Neurol Sci 1996;139:123–8. Aboussouan LS, Khan SU, Meeker DP, Stelmach K, Mitsumoto H. Effect of noninvasive positive-pressure ventilation on survival in amyotrophic lateral sclerosis. Ann Intern Med 1997;127:450–3. Maguire P, Fairburn S, Fletcher C. Consultation skills of young doctors. Br Med J 1986;292:1573–8. Tattersall MH, Butow PN, Griffin AM, Dunn SM. The take-home message: patients prefer consultation audiotapes to summary letters. J Clin Oncol 1994;12:1305–11. Working Party of the Royal College of Physicians. Improving communication between doctors and patients – a report of a working party. London: Royal College of Physicians, 1997. Walker B. Breaking bad news: an audit for giving the diagnosis of cancer. London: Kings Fund Publishing, 1996.
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[24] Sanson-Fisher RW, Redman S, Walsh R, Mitchell K, Reid AL, Perkins JJ. Training medical practitioners in information transfer skills: the new challenge. Med Educ 1991;25:322–33. [25] Doyle D, O’Connell S. Breaking bad news: starting palliative care. J R Soc Med 1996;89:590–1. [26] Girgis A, Sanson-Fisher RW. Breaking bad news: consensus guidelines for medical practitioners. J Clin Oncol 1995;3:2449–56. [27] Ptacek JT, Eberhardt TL. Breaking bad news. A review of the literature. J Am Med Assoc 1996;276:496–502. [28] Caplan LR. The effective clinical neurologist. Oxford: Blackwell Scientific, 1990. ¨ [29] Borasio GD, Pongratz DE. Gedanken zur Aufklarung bei amyotropher Lateralsklerose [Considerations on breaking the news in amyotrophic lateral sclerosis]. Nervenarzt 1997;68:1004–7. [30] Buckman R. How to break bad news. London: Papermac, 1996.
Breaking the news in amyotrophic lateral sclerosis a, b c Gian Domenico Borasio *, Richard Sloan , Dieter E. Pongratz a
¨ , Germany Department of Neurology, Klinikum Grosshadern, Ludwig-Maximilians-University, D-81366 Munchen b Joseph Weld Hospice, Dorchester, Dorset DT1 2 SL, UK c ¨ , Germany Friedrich-Baur-Institute, Ludwig-Maximilians-University, D-80336 Munchen
Abstract Telling the diagnosis to patients with amyotrophic lateral sclerosis (ALS) is a daunting task for any neurologist. Obviously, breaking the news in ALS is not a standardizable procedure. However, proven techniques exist to reduce the trauma to the patient and ease the burden on the doctor, thus reducing the risk of burnout and the tendency to ‘pull away’ from the patient. Such communication skills are of fundamental importance to clinical practice and should be more prominent in medical teaching. The way the patient is told the diagnosis is now recognized to be the first and one of the most delicate steps in palliative care. Information is best offered in a stepwise fashion at the patient’s pace with an emphasis on positive aspects, and in the presence of the patient’s family. Reviewing available therapeutic options and current research efforts may foster hope for the future, while pointing out that almost all symptoms of ALS can be alleviated by palliative therapy may help to reduce fears. Encouraging patients to ask questions and disclose anxieties is important for their psychological wellbeing. Available options for mechanical ventilation should be reviewed early enough to allow for unhurried decision-making. We believe that the terminal phase of the disease should be discussed at the latest when dyspneic symptoms appear, in order to prevent unwarranted fears of ‘choking to death’. 1998 Elsevier Science B.V. All rights reserved. Keywords: Bad news; Communication skills; Motor neurone disease; Palliative care; Telling the diagnosis
1. Introduction Amyotrophic lateral sclerosis (ALS), also known as motor neurone disease, is a degenerative disorder involving central and peripheral motor neurons which leads to death within 3–5 years from onset [1]. The unenviable task of telling a patient that he / she has got ALS generally falls to the neurologist diagnosing it. Whilst the messenger can get blamed for bad news, there is anecdotal and researchbased evidence that it is not always performed optimally [2,3]. Because of the poor prognosis there has been reluctance in the past to tell the patient the diagnosis. Often, the patient was told ‘reassuring’ statements, while the relatives were informed more fully, with the addendum ‘‘there is nothing that can be done for him / her’’. This practice still exists, although most clinicians now respect the ethical principle of autonomy in their patients. The majority of patients do want to have information on their condition, even if it is life-threatening [4,5]. Without this, the stress they suffer through uncertainty is often *Corresponding author. Tel.: 149-89-70953671; 70953677; e-mail: [email protected]
fax:
149-89-
worse than knowing they will die. One in five patients with cancer develop full-blown psychiatric disorders [6]. The main predictive factor for this is the way in which bad news is broken [7]. The feeling of helplessness present on both sides when the diagnosis of ALS is disclosed should not lead to withholding information that might be essential for the patient’s life planning [8]. Breaking the news is an ongoing process throughout the course of the disease: it is not limited to the communication of the diagnosis (which in itself is a multi-step procedure), but encompasses all the aspects of the information flow from the physician to the patient regarding the disease process, thus including good as well as bad news. This paper offers some suggestions on how to break the news in ALS and how training in communication skills could help both neurologists and the patients they serve.
2. Background Johnston and co-workers [3] interviewed 50 patients about their experiences of being told they had ALS. Most
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Table 1 Communicating the diagnosis of ALS (after Ref. [3]) ? Most people see positive aspects on being told the diagnosis, especially as it provides a label for the condition ? People prefer the diagnosis to be communicated in a direct, empathetic style ? Being able to ask questions is important ? Doctors should guard against giving too pessimistic a message all at once ? Information on where to get further help (e.g. from a patients’ association) is important at the time of diagnosis ? People generally prefer to be told with someone else present
patients saw the providing of a label for their condition as a positive thing – ‘‘at least I now know what I have to contend with’’. Negative aspects included: being told too late – ‘‘I could have used the past year better if I had known it might have been my last’’; being told the diagnosis without the option of having a relative present; lack of privacy (e.g. in the open wards); being told in vague or confusing terms; too much detail all at once. Although there was no evidence that poor communication at the time of diagnosis was responsible for prolonged mood disturbance, patients may well have undergone unnecessary psychological trauma at the time they were told. A summary of the findings is shown in Table 1. Lack of adequate communication skills also leads to stress in doctors [9]. Table 2 outlines the reasons why breaking bad news is difficult. Burnout is more likely amongst consultants who feel insufficiently trained in communication skills [10]. As the UK Health Service Commissioner [11] points out, improvement in communications between health care workers, patients and relatives would result in a marked reduction in the number of complaints reported to him. Table 2 outlines the reasons why breaking bad news is difficult.
3. What to tell
3.1. Minimum information After the diagnosis is firmly established, the patient should be informed that he / she has a progressive disease of the motor nerves, for which no curative therapy is available. The name of the disease must be told and explained, to avoid confusion, e.g. with multiple sclerosis. If the family history is negative, it is reassuring for patient and family to know that their children are unlikely to be at risk. Positive aspects (e.g. no pain, no disturbances in Table 2 Why breaking bad news is difficult for doctors (after Ref. [30]) ? Fear of the messenger getting blamed for bad news ? Perceived lack of time ? Lack of training ? Fear of causing distress ? Fear of being asked difficult questions ? Fear of not having all the answers ? Invoking fears of one’s own mortality
sensation, cognition, memory, personality, continence, etc.) should be stressed, as well as the availability of efficient palliative measures for practically all symptoms of ALS [12]. Current research efforts and, where available, the possibility of taking part in clinical studies of new drugs should be pointed out as a means to foster hope.
3.2. Beginning of the disease Patients often suspect a connection between the outbreak of the disease and a specific event (e.g. accident, operation, private or professional crises, etc.). This question should be addressed, and patients should be told that the disease starts many years if not decades before the first symptoms show up. Since at least 50% of the spinal motor neurons have to degenerate prior to clinical manifestation, it is impossible to establish when the disease began in any single patient, even with a large degree of approximation.
3.3. Prognosis Many ALS patients ask directly about their remaining life span, since, at the time of diagnosis, they have usually witnessed the progression of the disease for several months if not years. The answer to this question should include the information that there are no sudden worsenings to be expected, that periods of relative stabilization may occur and that rare remissions have been observed. The patients should be told that the course of ALS may vary between months and decades (e.g. Stephen Hawking), making a firm statement on prognosis all but impossible for any single patient. In our experience, many patients appreciate a rough approximation, for example ‘‘I think you have probably got several years rather than several months left’’. This allows them to plan the rest of their lives and is more helpful than an outright refusal to speculate. However, if a patient explicitly asks for available statistics, he / she has the right to know. Careful questioning about suicidal thoughts may be advisable. We believe that such acknowledgement may reduce rather than increase suicidal attempts.
3.4. Available therapies Since 1996, the first substance with a modest lifeprolonging effect in ALS has been on the market (Riluzole [13]). Subject to availability of reimbursement for this
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drug, an open discussion on its pros and cons should take place. Patients must know that they will usually not notice any subjective change in the progression of their disease. The possible side effects (nausea, asthenia, dizziness) should be mentioned. This discussion avoids raising false hopes, which might otherwise eventually lead to disillusion and frustration.
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ALS, will want a second opinion. Again, this is more than understandable and should be addressed directly by the physician who is breaking the news. Possible tertiary referral centers should be discussed with the patient. This may strengthen the doctor–patient relationship and help to avoid the patient self-referring from one doctor to another.
3.8. If the diagnosis is only suspected 3.5. ‘ Unconventional’ treatments It is all too understandable that patients with ALS start looking for help outside the boundaries of ‘classical’ medicine. This topic should therefore be addressed the first time the diagnosis is told. Patients should be reassured that their physician does not object to their trying homeopathic medication, acupuncture, etc. if they so wish. However, patients and families must be warned that some alternative methods (e.g. ‘fresh cell therapy’, snake toxins) may be dangerous to their health, while others may entail serious financial consequences for the benefit of charlatans and common criminals (including, sadly, some physicians).
3.6. Patients’ associations Patients may obtain invaluable help (indeed, more than their doctors can usually offer) from the ALS patients’ associations, which are present in almost every major country in the world and are grouped together in the Alliance of ALS / MND associations (Table 3). It is therefore, to our mind, absolutely mandatory to inform patients and families from the beginning about the existence of these associations, provide them with the relevant addresses and telephone numbers, and explicitly encourage them to get in touch with the association.
3.7. Second opinion Many patients, when confronted with a diagnosis like
What has been said so far presupposes an unequivocal clinical diagnosis of ALS, i.e. ‘probable’ or ‘definite’ ALS according to the El Escorial criteria of the World Federation of Neurology [14]. If these criteria are not met, the physician will obviously take a more careful approach, depending on the degree of clinical certainty. Possible differential diagnoses and the diagnostic plan must first be discussed with the patient. However, if the El Escorial criteria for ‘possible’ ALS are fulfilled, information on appropriate palliative measures, available drug options and therapeutic studies should take place, since these patients rarely turn out to have something else.
3.9. Discussing mechanical ventilation and the terminal phase For many ALS patients, the onset of dyspneic symptoms marks a turning point during the course of their disease. Often, the first dyspneic bouts happen after choking on food, on exertion or during sleep. At the beginning of clinically manifest involvement of the respiratory muscles, anxiety is usually the most prominent symptom during dyspneic attacks (which therefore respond very well to short-acting benzodiazepines, e.g. lorazepam 0.5–1 mg sublingually). If the subject has not been discussed previously, we believe that patients should receive information on the terminal phase of the disease at the latest when the first dyspneic symptoms appear. The reason for this recommendation is that almost all patients, when
Table 3 Patients’ associations United Kingdom
Motor Neurone Disease Association (MNDA) and International Alliance of ALS / MND associations
PO Box 246, Northampton NN1 2PR
Tel.: 144 1604 250505 Fax: 144 1604 24726 Website: www.alsmndalliance.org
Germany
¨ Muskelkranke (DGM) Deutsche Gesellschaft fur
Im Moos 4, D-79112 Freiburg
Tel.: 149 7665 9447-0 Fax: 149 7665 9447-20 E-mail: dgm [email protected] ] Website: www.dgm.org
USA
Muscular Dystrophy Association (MDA)
3300 East Sunrise Dr., Tucson, AZ 85718
Tel.: 11 520 529 2000 Fax: 11 520 529 5300 E-mail: [email protected] Website: www.mdausa.org
USA
ALS Association (ALSA)
21021 Ventura Blvd., Suite [321, Woodland Hills, CA 91364
Tel.: 11 818 340 7500 Fax: 11 818 340 2060 E-mail: [email protected] Website: www.alsa.org
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questioned at this stage, report fears of ‘‘choking to death’’. In our experience, describing the physiological mechanism leading to terminal hypercapnic coma and the resulting peaceful death in sleep will relieve these fears in most cases. Patients and relatives must also be informed that the array of medication available in the terminal phase, if correctly applied, does prevent suffering [15]. This information needs to be reiterated in subsequent visits. At this stage, patients should be asked whether they would wish to be intubated and ventilated in the event of a terminal respiratory insufficiency. Patients who have been informed about the possible subsequent clinical course, which may end up in a ‘locked-in’ syndrome on an intensive care unit [16], will usually refuse such a procedure. This refusal must be documented by the physician and is often best incorporated into a living will. The consequences of such a decision must be discussed with patient, family and the family physician (e.g. concerning the use of opioids in the terminal phase [17]). Symptoms of chronic nocturnal hypoventilation (Table 4) may precede the onset of clinically overt respiratory insufficiency by months or even years, and can be efficiently palliated by non-invasive home mechanical ventilation (HMV) [18,19]. These symptoms must be carefully searched for by the physician at every visit, since some patients will deny any respiratory trouble even in advanced stages of chronic hypoventilation. As soon as these symptoms are recognized (which may be before or after the first dyspneic bouts), non-invasive HMV should be discussed, emphasizing that the primary aim of this measure is palliation rather than prolongation of life. The patient should also be informed that eventually a decision on whether or not to perform tracheostomy will become necessary (see above). The slope of decline in forced vital capacity (FVC) is a better predictor of respiratory failure than the absolute value. A significant drop in FVC when measured supine vs. sitting or standing is also a reliable sign of present or impending hypoventilation, as is an FVC value of ,50% of predicted (except for patients with slow progression, who may be asymptomatic at very low FVCs, due to
Table 4 Symptoms of chronic hypoventilation (from Ref. [12]) ? Daytime fatigue and sleepiness, concentration problems ? Difficulty falling asleep, disturbed sleep, nightmares ? Morning headache ? Nervousness, tremor, increased sweating, tachycardia ? Depression, anxiety ? Tachypnea, dyspnea, phonation difficulties ? Visible efforts of auxiliary respiratory muscles ? Reduced appetite, weight loss, recurrent gastritis ? Recurrent or chronic upper respiratory tract infections ? Cyanosis, oedema ? Vision disturbances, dizziness, syncope ? Diffuse pain in head, neck and extremities
adaptation). These data may serve as additional cues as to when to address the respiratory issue in ALS patients. The overall aim is to discuss the available options early enough, so that patient and family may have sufficient time to make their decision.
4. How to tell Maguire and co-workers [20] have outlined the factors to be considered when breaking bad news (Table 5), which will be discussed in detail below. They define three underlying objectives in the process:
• do not withhold information if the patient wants it; • do not impose information if the patient does not want it; • gauge and respond to the patient’s reaction to the news.
4.1. Setting In some countries (e.g. USA), diagnostic procedures for ALS are usually performed in an outpatient setting, while in others (e.g. Germany and UK), patients are usually referred to a Neurology Department for inpatient workup once ALS is suspected. The latter procedure gives the physician the possibility of a stepwise offering of information over several days, thereby allowing the patient to reflect overnight and ask additional questions. If the diagnosis is told in an outpatient setting, a short-term follow-up visit should be scheduled. The discussion should take place in a private, quiet room. The middle of an open ward is far from ideal. If at all possible, interruptions (phone, beeper) should be avoided. Provided the patient agrees, the diagnosis is best told in the presence of the family to avoid misunderstandings. Roger Carus, a patient who wrote about his experience of being diagnosed with ALS in the mid-seventies, found out inadvertently whilst in hospital for investigations. He was not sure whether or not his wife had been told the diagnosis by his neurologist and recalls ‘‘I spent two of the most agonising weeks of my life trying to find out whether she knew or not’’ [2]. Table 5 The stages in breaking bad news (after Ref. [20]) ? Setting ? Finding out what the patient already knows or suspects ? Finding out how much more they want to know ? Firing the warning shot ? Hierarchy of euphemisms ? Observing and responding to the patient’s reactions at each step ? Contract for the future
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4.2. Finding out what the patient already knows or suspects For example: ‘‘What do you make of your illness / investigations?’’; ‘‘Did you ever think that something serious might be going on?’’ This second question will not perturb patients who are using denial as a coping mechanism – they will be able to shrug it off as not being applicable to them.
4.3. Finding out how much more the patient wants to know ‘‘If you had something seriously wrong with you, are you the sort of person who likes to know exactly what is going on or just the treatment plan?’’ Again, those not wanting to face the reality will opt for the latter without being caused undue distress.
4.4. Firing the warning shot ‘‘I am afraid the results of the tests were not as good as I was hoping for’’. This breaks the unsuspecting patient in gently.
4.5. Hierarchy of euphemisms Small pieces of information of increasing seriousness are told to the patient, with pauses in between for the patient to digest them. This avoids extreme distress and the patient ‘switching off’ to any further explanation through shock. For example: ‘‘The tests we have performed show you have a problem with the nerves which transmit instructions to the muscles’’; ‘‘The nerves are gradually being destroyed so that your muscles can’t work properly’’; ‘‘The condition you have got is called amyotrophic lateral sclerosis, or motor neurone disease’’; ‘‘Unfortunately, there is no known cure at the moment, although there is a drug (Riluzole) which modestly slows the deterioration down’’; ‘‘Yes, the disease is eventually fatal’’.
4.6. Observing and responding to patient’ s reactions at each step In order not to impose information that the patient is not yet ready for, the doctor pauses and waits for a reaction at each step. If the patient asks a question such as ‘‘What do you mean?’’ this is a clear invitation for further information. Conversely, if the patient expresses distress either verbally or non-verbally, it is important not to proceed but to sensitively explore with the patient the reason for their distress. This may appear obvious, but often patients may make false inferences about which the doctor can be truthfully reassuring, e.g. that they are going to die next week. When a patient breaks down, it is important to acknowl-
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edge the distress – touch may be the most empathetic way of doing this. If the physician is not sure whether to continue, one possible question could be ‘‘Have you had enough for now or are there further questions you would like to ask?’’
4.7. Contract for the future Phrases like ‘‘I’m afraid there’s nothing more we can do’’ must be avoided – patients feel devastated, presuming falsely that they are condemned to intractable suffering. We have found it helpful to demonstrate concern for the patient by informing them of sources of help and support, as outlined above, and arranging a short-term follow-up meeting. Often, patients tend to forget information, even if they have fully understood it when it was first told. It is therefore important, on each clinic visit, to find out time and again where the patient stands and to move on jointly from there.
4.8. Reinforcement of information Evidence from the cancer literature suggests that patients might profit from being handed out an audiotape of the consultation or receiving a letter outlining the consultation in lay terms, with a preference for the former [21]. This approach has not yet been tested in ALS patients. Our own data in a small sample of 39 ALS patients and 19 relatives (Table 6) show that (i) about 50% of patients and relatives felt that they received no or insufficient information from their physician at the time of diagnosis; (ii) a brochure containing general information on ALS in lay terms was regarded to be informative by almost all patients and relatives; (iii) however, five of 39 patients and five of 19 relatives did not think that receiving the brochure had helped them in any way, and one couple said that they wished they had never read it. Therefore, handing out written information material after the consultation may be helpful for many patients, but it might be advisable to ask patient and relatives whether they feel that they are ready for it or whether they would prefer to receive the material at a later stage. Table 6 Patient survey: information on the diagnosis and satisfaction with ALS brochure [39 ALS patients (age 35–80, 29 m, 10 f), 19 relatives (age 28–76, 5 m, 14 f)] ALS patients
Relatives
Information received on the diagnosis prior to receiving the brochure None 7 / 39 (18%) 3 / 19 (16%) Insufficient 12 / 39 (31%) 7 / 19 (37%) Sufficient 20 / 39 (51%) 9 / 19 (47%) Satisfaction with the brochure Informative 38 / 39 (97%) Helpful 34 / 39 (87%)
18 / 19 (95%) 14 / 19 (74%)
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5. Learning communication skills In 1981, only 30% of medical schools in the UK gave any teaching in communication skills, but now all but one teach the subject formally. The Royal College of Physicians now recommends the approach adopted in some schools where communication skills are taught throughout the clinical course rather than in one or two modules only. In common with the Royal Colleges of Psychiatrists and General Practitioners, it also suggests that postgraduate examinations should include formal mechanisms such as video tape recordings for assessing communication skills [22]. Walker [23] found that, of 27 consultants questioned, only two had been on formal communication skills courses since qualifying. Several expressed interest if such a course was to be organised locally, but the majority implied that communications skills could not be taught and could only be picked up ‘on the job’. With bad habits as well as good being picked up from observing others at work, perhaps it is not surprising that poor technique is perpetuated in successive generations of doctors. Studies have shown significant improvement in communication abilities in those who have been taught the relevant skills [24], although these skills survive better if they are periodically reinforced [20]. To be properly acquired, the skills should be practised in a safe environment – safe for those learning them and safe for patients. Video recordings of role-played situations using actors or the students themselves can be particularly useful provided they are handled sensitively. Teachers of communication skills must be trained in the ‘rules of feedback’. Instead of focusing on students’ weaknesses, the strengths they already possess are reinforced and alternative methods explored where difficulties are encountered. This way, role play becomes a positive learning experience and not the negative one so many students seem to recall.
6. Conclusion Whilst breaking the news in ALS will never be easy, proven techniques exist to reduce the trauma to the patient and ease the burden on the doctor. Such communication skills are of fundamental importance to clinical practice and should be more prominent in both undergraduate and postgraduate teaching. The way the patient is told the diagnosis is now recognized to be the first and one of the most sensitive steps in palliative care [25]. However, empirical evidence on the consequences of how the diagnosis is told is scanty throughout medicine [26,27]. Although we cite research evidence equating good communication techniques to reduced psychological morbidity in patients, more studies are required to substantiate best practice and teaching techniques. To quote from Caplan [28], ‘‘The increased number of diagnostic and treatment options makes even more crucial the physician’s skill in
managing illness and the art of communicating with patients and their loved ones. There but for the grace of God go we all, for all of us and our families are, or will eventually become, patients.’’
Acknowledgements This paper is based, in part, on a consensus document of the German Neuromuscular Centers of the Deutsche ¨ Muskelkranke (Freiburg, Germany) [29]. Gesellschaft fur We thank Prof. Th. Brandt, Prof. K. Kunze, Prof. B. ¨ Neundorfer and Prof. K.V. Toyka for their contributions, Dr D. Newman for helpful discussions and M. Wasner for editorial assistance. Special thanks are due to Dr R.G. Miller for critical review of the manuscript.
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