Should patients or their families be told of the risk of sudden unexplained death in epilepsy (SUDEP)? There appear to be two considerations. On the one hand, the probability of death is so unlikely that many patients and families will worry unnecessarily if they are told. On the other hand, since there appears to be an association of SUDEP with seizure activity, those who experience frequent seizures might benefit from general preventive measures, even in the absence of definitive evidence that these may prevent SUDEP. The question of discussing the risks of SUDEP presents us with medical, ethical, and possibly legal, implications.
Let’s illustrate these different perspectives. Elson L. So, MD, Mayo Clinic, reported in Epilepsia 47 (Suppl. 1) 87-92, 2006 that “SUDEP seldom occurs, and the generally low and diminishing rate of autopsy in the United States hinders the study of SUDEP.” He also noted that “in the past 5 years, only two retrospective series of pediatric SUDEP cases were published.” Although the cause remains illusive, Dr. So reported studies in which the risk of SUDEP was nearly 14 times higher when there was a history of generalized tonic-clonic seizures in the previous 3-month period.
More recently, a Scottish team of physicians at Glasgow’s Epilepsy Unit, Western Infirmary, also noted that “more people succumbing to SUDEP had had a seizure within the previous year” (Hitiris et al, Epilepsy & Behavior, 2007;10(1):138-141). They added that they “counsel patients regarding SUDEP at diagnosis and when optimal seizure control proves elusive,” yet pointed out that within the United Kingdom, “the risk of SUDEP is not routinely discussed with patients by a majority of neurologists….”
In the United States, one-third of patients with epilepsy have seizures that are uncontrolled by medication. Should this group and/or all of those with epilepsy and their families be informed of the possibility of SUDEP?
The article by Hitiris and colleagues concludes “there is an urgent need for a large-scale, prospective, international, community-based study of SUDEP to explore closely the risk factors to plan preventive strategies.”
In the interim, how and when should physicians communicate the risk of SUDEP to patients and families? We asked two experts, Martin J. Brodie, MD, from Glasgow, Scotland, and Elinor Ben-Menachem, MD, from Götenberg, Sweden, to share their views about the following questions:
Elinor Ben-Menachem, MD, PhD, is Professor of Neurology and Epilepsy at the Institute for Clinical Neurosciences and Physiology, Göteborg University, Göteborg, Sweden. She received her medical degree and PhD degree in neurology at Göteborg University in Sweden. Between 1982-1983 she was an Epilepsy Fellow at the VA-UCLA in Los Angeles, California.
Dr. Ben-Menachem has been the chairman of the ILAE Antiepileptic Drug Guideline Subcommittee, which recently published their guidelines in Epilepsia. She is currently Vice Chair (and Chair in 2007) of the Annual Course Committee at the American Epilepsy Society and has served as Chief Editor of Acta Neurologica Scandinavica since 2001.
Professor Martin J. Brodie, MD, FRCP, is Professor of Medicine and Clinical Pharmacology at the University of Glasgow in Scotland. He directs the Epilepsy Unit at the Western Infirmary, which provides a range of services for people with seizure disorders. His research interests include antiepileptic drug neuropharmacology, the management of epilepsy, and factors affecting response to treatment. Professor Brodie is treasurer of the International League against Epilepsy. He chairs the Management Group and Scientific Advisory Board of European Concerted Action and Research in Epilepsy (EUCARE). Professor Brodie has been appointed "Ambassador for Epilepsy" on behalf of the International League against Epilepsy and the International Bureau for Epilepsy.
His recent publications regarding SUDEP include:
Should physicians tell patients and families about the risk of SUDEP?
Martin Brodie: Patients and families have the right to know that there is a risk of sudden unexpected death when the diagnosis is made. We give them all an information pack containing a number of booklets including one entitled “Epilepsy: be safe,” which has a paragraph about SUDEP. The Joint Executive Committee of UK Epilepsy Charities recommends this approach.
If we have a patient who is having many seizures, then we emphatically say, “If you do not take the medication as prescribed, you could die during a seizure.” We do this when we feel that there is a high risk of SUDEP or when a person decides not to take treatment despite having a number of seizures. After we see the patient in the clinic, we document any conversation about SUDEP in the letter to the general practitioner, as suggested in the Scottish Intercollegiate Guidelines Network (SIGN) and the UK’s National Institute for Health and Clinical Excellence (NICE) epilepsy guidelines.
I will tell you a story illustrating why I feel so strongly about this. This involved a 20-year-old lady who was found dead in bed by her mother. She was interested in homeopathic remedies. I suggested when the diagnosis was made that she take “my” antiepileptic drug in addition to anything else that she felt might be helpful. When I discussed the situation with her mother after the patient’s death, she said, “I didn’t know that there was such a risk.” I had discussed SUDEP in detail with her daughter, but apparently she didn’t mention this conversation to her mother. It was the mother who had persuaded my patient to switch over to a homeopathic preparation. That mother will not sleep well for a long time.
If we had not discussed the risk of death before that young lady transferred to a different treatment, we would not be fulfilling our responsibility. That person would not know about the potential dangers of ignoring the treatment plan.
Elinor Ben-Menachem: I think it depends upon the situation and the person in front of you. This is where the physician’s expertise comes in – understanding the anxiety level of a patient. If a patient has nuances of epilepsy and you are giving a drug for the first time and you anticipate that the patient has a 60 to 70 percent chance of being seizure-free, with so little risk, why would you scare them unnecessarily?
There are many risks we face in life -- going to the store, crossing the street, taking the underground in London. Being born is a lethal procedure. So I do not think that the risk – when it is small – should be mentioned.
I have personally had major conditions with my heart, with stents, with surgeries, and at no time did anyone talk to me about the risk of dying. Regarding the question of telling a person about the risk of SUDEP – if it is minimal risk, I say “no.” Let them get on with their lives.
Now for patients with refractory seizures, we get into another situation. Those with uncontrolled seizures are a different group, because we know about the association between multiple seizures and SUDEP. For this group, “yes” – I offer information about the risk of death. Additionally if I have a person who is non-compliant with regard to their medications, there again, I offer all of the information I can about SUDEP. I have had two people who have died because they were non-compliant, and this was something I could not control. But when speaking with this group of people, I emphasize, “If you do not take your medication, you might just die during the night.”
How do you counsel patients and families?
Elinor Ben-Menachem: If I am treating a child whose seizures are refractory, I definitely tell the parents that there is a risk. However, the problem is this. What can they do? There appears to be a direct correlation between SUDEP and those who sleep alone in one’s own room. In fact, such a child should not sleep in his or her own room, even though this is not ideal in terms of the privacy that everyone hopes to have. Since we know that the risk is greater at night, then if the child (or even a teenager) sleeps alone, he or she is at risk. There are no alarm systems that tell us when the child stops breathing; therefore, we can only advise that they sleep in the parents’ room.
Perhaps if a dog can be trained to perceive when the child stops breathing, then that could be a solution. A dog might push the child, and stimulate in some way so that this will start the child breathing again. At the moment there are no ideal devices that can do this.
For an older child or adult, sleeping alone is a problem. Although here again, a dog might help.
There is no right answer. Every patient is an individual. Neurologists must be psychologists. We treat the patient for years, and we come to know them well. We develop opportunities that help us know how to talk with them.
Martin Brodie: We give all patients a range of guidelines about epilepsy and its treatment, which presents an opportunity for the patient or family to ask questions. If a patient is still having seizures, particularly many seizures, then we need to inform them about the risk of SUDEP.
Now, here is an important issue. What we do not have right now is good evidence as to what may protect the patient from succumbing to SUDEP. We tell them not to take baths, because drowning in the tub is possible. Even showers can be a risk for a person living alone. Then we have the worry about nocturnal seizures. Some people with nocturnal seizures sleep alone and it is not possible to expect all of them to sleep with someone else in their bed or room.
I am always particularly anxious when a pregnant woman comes off medication because of the perceived risk of damage to her unborn baby. This is not an uncommon occurrence.
Can preventive measures with regard to prominent risk factors reduce the likelihood of death?
Martin Brodie: As I said before – we caution about baths, about sleeping alone, etc.
We don’t have specific preventive measures that are backed by good clinical evidence. We need a major international, prospective study of a range of potentially beneficial interventions because no one has enough clinical experience to know what will make a difference for the individual patient with refractory epilepsy.
Elinor Ben-Menachem: As I have said, sleeping alone is a problem. For children, maybe sleeping in their parents’ room is the answer. Maybe a dog will help. For adults, it is difficult because not everyone has someone who will be sleeping with them in the same room. The best we can do is to encourage situations that are protective.
Do physicians have an ethical responsibility to disclose the risk of SUDEP?
Elinor Ben-Menachem: I do not think so. If you have a very high risk patient, then maybe, but we have no solution to the problem. I follow my patients for 30 years – through a lifetime. This gives me a unique perspective. I know the lives of my patients. When advising my patients I try to assess what information will be helpful. Many doctors do not have this opportunity.
Martin Brodie: I feel that they do. When you take this to its logical conclusion, I am not aware of anyone getting into trouble for raising the issue of SUDEP. But take the case I described of the woman who persuaded her daughter to take a homeopathic preparation instead of her prescribed antiepileptic drug. If I had not told the daughter of the risk of SUDEP, and had not documented this, and the family went to a lawyer, would they not have had a case?
Do you worry about the legal ramifications of non-disclosure?
Elinor Ben-Menachem: No, I do not worry about legal ramifications. I feel that there can be no legal ramification for non-disclosure because there is no cure. If there was something constructive that I could tell patients, then I should and would tell all of my patients. But as long as we do not have a solution -- and there are no studies that show that SUDEP can be prevented – then I do not think there is very much we can do. You know I had open heart surgery in the US and no one disclosed to me that I could die – despite the high risk.
Everyone knows that epilepsy is a serious disease. We know people can die from kidney failure, diabetes, a heart attack – but we do not ruin their lives telling them all the negatives. As long as you cannot do anything about SUDEP, then we say what is best, and do our best.
Martin Brodie: I think it is in everyone’s interest to discuss every aspect of this condition. Our job is to do the best we can for our patients. Gone are the days when we could take a paternalistic view of what they need and don’t need to know. What I know, my patients should also know!
Interviews by Rita Watson, MPH
Last Reviewed: 02/12/07
Edited by Steven C. Schachter, MD
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