Living with SUDEP
from an address given by Ann Farrell to the Women & Epilepsy Conference at the University of Toronto, 22 March 1997.
Eighteen months ago, around supper time, I found my daughter Judy lying dead on the chesterfield in her Hamilton apartment. When I first looked at her, it seemed she was just sleeping. However, I knew she had come home from her night shift with the Hamilton police some nine hours earlier, so it was unlikely she was still resting. It only took me a few moments, then, to realize the awful truth. At the time, she was 32 years old. She had been diagnosed with epilepsy at the age of 12.
One of the first people we called shortly after Judy's death was Dr. Mac Burnham, a long-time friend. Mac was shocked, of course. Then he told me he thought it was likely Judy had died from SUDEP (Sudden Unexplained Death in Epilepsy Patients). "SUDEP," I asked him, "What's that?" I had never heard of it.
For those of you who may also not know about SUDEP, a generally accepted explanation is that deaths of this nature typically occur in young adults, more often in males, and are more likely to occur when seizures are not well controlled. The individual simply stops breathing while asleep and the cause is uncertain.
For instance, it is recognized that epileptic seizures can commonly cause apnea (temporary stoppage of breath) and, although less often, cardiac arrhythmias (irregularity in heartbeat). Many coroners do not look for signs that epilepsy may have been a contributory cause of death. The coroner's report on Judy's death, for example, said she died of natural causes. This about a devotee of Tai-Kwon-Do who was to all intents and purposes a healthy young woman, leading a full and active life.
SUDEP has never been easy to pinpoint or define as a quote in the November 1996 issue of the British medical journal The Lancet indicates:
"The development of a working hypothesis on the nature of these deaths has been hampered by their usually being unwitnessed, and by the absence in (post mortems) of an adequate structural cause" [such as apnea].
So learning of SUDEP came as a profound shock to me. I had always been told that epilepsy is seldom life-threatening, except in the case of accidents such as drowning or a road accident — things that can largely be prevented. Even when a seizure led to someone going into
status epilepticus, emergency measures taken in time were generally successful.
However, once I had recovered from the initial shock of Judy's death, my background as a journalist kicked in and I set about seeing what I could find out. It hasn't been easy.
First I visited Judy's family physician and her neurologist in Hamilton. "Yes," they said, both of them knew about SUDEP but it was information they preferred not to pass on. Apparently, doctors not only keep quiet about SUDEP with their patients who have epilepsy, but also with their patients' families. Moreover, if these patients then die in circumstances that suggest SUDEP as the likely cause, the doctors continue to maintain silence about it. That is unless family members, like me, come asking questions!
According to these physicians, the decision is based on a desire not to cause undue alarm. The rationale is that, as little is known about SUDEP's cause and not much more about how to prevent it, they feel it is kinder and less alarming to all concerned to keep the information to themselves. My personal reaction to this attitude on the part of doctors is that it is patronizing, at the very least. Patients and their families are entitled to learn everything they can about epilepsy which, in many ways, continues to be a puzzling condition. It is then up to them to make their own decisions. It's like withholding information about the terminal nature of an illness, lest the patient can't handle it.
I don't say this just because I am determined to stand on my rights with doctors but because there are good reasons why an individual with epilepsy needs this information. He or she needs to know about the crucial importance of doing everything possible to minimize the possibility of seizures and to prevent, as far as possible, anything worse from happening — especially as it is recognized that SUDEP is more likely to occur when seizures are poorly controlled.
By way of illustrating how increased seizure patterns can have a more far-reaching effect than simple injury, in Clinics & Cases (a supplement of Medicine North America), editor-in-chief Dr. A. Guberman writes:
"The deleterious effects of uncontrolled seizures include psychosocial consequences, possible cognitive and memory deterioration, lowered seizure thresholds and also directly or indirectly, seizure-related injury or death."
Dr. Guberman's opinion was born out in Judy's case in the months before her death. During this period she complained of memory loss; in fact, her colleagues said the quality of her work did deteriorate and, because of their affection for her, two of them used to 'tidy up' her mistakes behind her back.
A decreasing level of work performance is one thing, but when it is followed by death, the situation cries out for preventative action.
The question then arises as to just how many people are believed to be dying as a result of SUDEP. In Canada, no firm statistics exist. In the United States, according to an abstract from a 1985 article in the American Journal of Forensic Medicine and Pathology: "sudden unexpected death in epileptic persons may represent 1 to 1.5% of all natural deaths certified by the medical examiner or coroner." And in Clinics & Cases it is claimed that "epilepsy-associated sudden death accounts for approximately 12 to 15% of all deaths in the epileptic population."
In England, Dr. Stephen Brown, a consultant neuropsychiatrist who works in affiliation with a number of internationally-based epilepsy groups, reports that in 1995 a shocking total of 771 deaths in England among the country's epilepsy population of 420,000 were caused by SUDEP. The figure was twice the annual average of between 200-300 SUDEP deaths. By comparison, in the same year England's crib deaths (which resemble SUDEP somewhat) numbered only 332. Incidentally, the amount of money spent on research into the causes of crib death in England far outstrips that spent on SUDEP research.
What is to be done about all this? The first thing that comes to mind, obviously, is education. SUDEP has to come out of the closet, public awareness needs to be significantly increased, and those with seizures need to know the danger implicit in failing to take more personal responsibility in the control of their seizures, over and above following the advice of their doctors regarding medication and so on.
In the 20 years Judy lived with seizures, family doctors and neurologists did caution her from time to time that an appropriate lifestyle was advisable if her seizures were to be minimized in number and severity. This entailed not skipping medication, avoiding alcohol, maintaining regular and adequate sleep patterns, and taking proper nutrition. However, the crucial nature of this advice was never stressed: no one indicated it was likely essential, rather than merely advisable, to observe this regimen. No one warned her that ignoring the rules might even prove fatal.
Now please do not think I fail to recognize doctors' difficulties in this respect. They spend their working lives advising patients how to maintain wellness, their good advice so often falling on deaf ears. Certainly that was the case with Judy who pursued every day as if it were her last. Despite the fact she was an adult at the time of her death, naturally I feel a certain amount of guilt. Her colleagues also wish they had known more about the implicit danger of her happy-go-lucky ways. But Judy was an adult.
Although I, her friends and her siblings all remonstrated with her from time to time, she was not the sort of person who responded to good advice — and certainly not to nagging.
So she went her merry way and in the final years of her life, her seizures did increase in number and severity. Her employers listed her as a disabled person and persuaded her to take out disability insurance shortly before her death.
In hindsight, it appears that people with epilepsy — like Judy — whose medication allows them to lead more or less normal lives may tend to push their epilepsy onto the back burner. Some of the old stigma surrounding epilepsy still remains and, because of this, people whose seizures are fairly well controlled may be tempted to keep their condition to themselves or, possibly, even from themselves. After all, denial is a common problem among those with ongoing medical conditions. The bottom line, though, is that epilepsy calls for regular medical monitoring, proper medication, and a lifestyle that is beneficial for control of seizures.
A generation ago, not much was written about the importance of a factor such as lifestyle in relation to avoidance of killers such as heart disease, stroke and lung cancer. Now many people are aware of the dangers inherent in disobeying lifestyle rules, where these diseases are concerned.
Surely it is time for some serious education on the subject. Falls and drowning, for example, are widely recognized as situations which can potentially result in death among people with epilepsy and advice is given about observing safety precautions. Some of these tragedies, in fact, might actually be averted (or at least lessened in frequency) if individuals would only exercise personal responsibility, thereby maximizing control over their seizures and avoiding potential dangers whenever possible.
Up to this point, we have been talking about what those who have seizures can do for themselves. But what about the rest of us? What can we do? Not long ago a friend in England sent me a newspaper clipping from the Sunday Telegraph. It contained a story about SUDEP and how British families have banded together to fight it from a public forum.
In the beginning, several parents of young adults who had died of SUDEP got together, initially to form a bereavement support group. They call themselves Epilepsy Bereaved. What propelled them into action was the devastation they felt because the deaths of their children were so sudden — so unexpected — when, until the very moment of death , their sons and daughters had apparently been leading healthy lives.
The 130 members of Epilepsy Bereaved say they were outraged that doctors had not shared with them details about SUDEP. They took issue with coroners who often failed to look for or identify epilepsy as a possible cause of death. They set about educating themselves, getting some doctors on side, and lobbying for changes in the whole area surrounding SUDEP. They also approached government, and sought research money. Who knows: perhaps if coroners were more aware of SUDEP when performing autopsies, useful clues for researchers might emerge.
In England, for every £250 spent on muscular dystrophy research, only 20 pence spent on epilepsy research. That's a ratio of 1,250:1 in favour of muscular dystrophy! This is not to deny the research needs of muscular dystrophy but rather to point out the catch-up situation concerning epilepsy research.
In October 1996, an international workshop of neurologists, cardiologists and pathologists was held at the Royal College of Physicians in London under the auspices of Epilepsy Bereaved. Glaxo-Wellcome sponsored the workshop, with 50 delegates from all over the world presenting papers.
Epilepsy Bereaved holds two national support group meetings annually with specialist speakers. Information is exchanged, and there is also a newsletter twice annually, in addition to a data base for families.
What is known in parliamentary terms as an "early day" motion was made to the British House of Commons, where Epilepsy Bereaved was congratulated for its work. The motion was supported by 227 MPs and was instrumental in securing a meeting with the Health Minister whose department later investigated the issue of inaccurate death certification.
Of course, Canada's much smaller population and the fact that health is administered here provincially makes the national scope of Epilepsy Bereaved hard to copy. But perhaps we should make an attempt. I shall be in touch regularly with Epilepsy Bereaved and maybe sometime in the future we in Canada, or at least in Ontario, could think about setting up our own sudden death organization.
Epilepsy gets little enough attention relative to other chronic conditions.
The experience of Epilepsy Bereaved suggests there is a need for an inquiry into education and training on epilepsy, particularly regarding sudden death and epilepsy. So far as the professional handling of bereavement is concerned, there are several such organizations available, such as that provided by Bereaved Families of Ontario.
As it happens, I have two other adult children — a son and a daughter — who experience seizures. Although they are both well controlled, the fear remains at the back of one's mind. Work has indeed begun on learning more about SUDEP and how better to avoid it, if not actually prevent it. An education program is under way, but much more needs doing before public awareness can be adequately established and professional involvement fully engaged.
At the end of the day it is probable that we parents are the ones more likely to start the ball rolling. How about it?
