March 13, 2008


I have been epileptic 52 years now. All along the way, I have suffered from disability bigotry from a wide variety of folks.

is often used as a pejorative term against a person who is obstinately devoted to prejudices even when these views are challenged or proven to be false or not universally applicable or acceptable.

I suppose the most difficult bigotry to accept is physician bigotry. To my way of thinking, epilepsy seems to make doctors with prejudice appear from nowhere!

My own GP believes E. to be a fairly innocucous malady. She has said to me, more than once, that at "least it isn't something that can kill you", so why worry about it?

Sudden Unexpected Death in Epilepsy, or SUDEP is a term used when a person with epilepsy suddenly dies and the reason for the death is not known. The cause of SUDEP is unknown. Post mortem examination usually reveals no abnormalities in victims.

Of those who die from SUDEP, it is most common in people who have generalised tonic-clonic seizures, especially in young adults. The most important 'risk factors' seems to be poor seizure control, and seizures occurring during sleep.

SUDEP is relatively uncommon. Roughly 1 in 200 sufferers of severe epilepsy die of SUDEP each year. For sufferers of mild idiopathic epilepsy (epilepsy of unknown cause), the figure drops to 1 in 1,000 per year. The incidence of SUDEP among people who are in remission from epilepsy is negligible.

Cary Groner is a freelance writer in northern California, writing for Applied Neurology on SUDEP, says that research suggests it may be the cause of death in 7% to 17% of all epileptic patients and in up to half of patients with refractory epilepsy. Almost all victims of SUDEP die at home, usually in bed.

Research is helping clinicians profile which patients are likely to be at highest risk. Important contributing factors include a history of uncontrolled seizures as well as seizure type (tonic-clonic seizures present the gravest danger and are associated with at least 90% of SUDEP cases; complex partial seizures also increase risk, but absence seizures do not). Evidence suggests that epilepsy duration heightens risk (most persons who die of SUDEP have had epilepsy for 15 to 20 years).

Elson So, MD, professor in the Department of Neurology at the Mayo Clinic College of Medicine in Rochester, Minnesota, adapts his decision to the individual patient but favors providing the information about SUDEP. "Patients with a history of uncontrolled, generalized tonic-clonic seizures are at highest risk," So said. "Those are the ones I pay the closest attention to and counsel about SUDEP. I think they need to know."

Okay, great. But what about discussing SUDEP risks with clinicians? Apparently, physicians treating patients with E. are subject to 'myths' about E. How these "myths: came into being is a direct function of sociological dynamics.

Despite early work on SUDEP, during the remainder of the Twentieth Century, the subject of epilepsy deaths was neglected and any research on the subject was ignored. In medical texts, and thus in the minds of medical practitioners, a ‘myth’ was established that epilepsy itself was not fatal. ’As far as longevity is concerned, the patient should definitely understand that epilepsy per se rarely causes death and that there is no reason why an epileptic should not live as long as he would if he did not have epilepsy’. (Dr. S. Livingston, Living with epileptic seizures, 1963) However, Rodin’s textbook, the Prognosis of Patients with Epilepsy, 1968, was a notable exception to the current thinking: ‘It appears to be quite obvious that the life expectancy of the epileptic individual does not reach that of the average person. It is also quite impressive that the figures have not shown a dramatic improvement during the past 5 decades. Although death from a seizure is relatively rare, it does occur on occasion and is not preventable under all the circumstances at the present time.’

An explanation as to why SUDEP was forgotten comes from Dr. Lina Nashef in 1995. Following two World Wars, the subject was addressed again but the setting had altered and new writers did not pick up where others had left off. Effective modern anti-epileptic drugs meant that Physicians felt both optimistic and omnipotent. Patients with epilepsy had moved from asylums into the community and there was much less opportunity for observation. Risks from epilepsy were minimized, then denied; that epilepsy could not be fatal became ‘common knowledge’ despite evidence to the contrary.

From the 1970’s to the 1990’s, scientific interest in epilepsy deaths and SUDEP was increasing steadily but most medical textbooks still chose to either ignore the subject altogether or to go in the face of research and make assumptions about the lack of risk in epilepsy. One exception is the following by G.Jay and J.E.Leestma in 1981: ‘There should be an increased awareness that SUDEP in epileptic patients is probably not an extremely uncommon complication and that as more is known about its substrates and mechanisms, that education of the patients and their physicians regarding preventive measures, including careful attention to medication, may decrease or eliminate this catastrophic complication of epilepsy’. (Acta Neurologica Scandinavica Suppl.82, Vol 63).

During the 1990’s there was collaboration on the subject of SUDEP between researchers in the United States, the U.K. and elsewhere, and in the U.K. a self-help group for relatives, called Epilepsy Bereaved, began raising awareness of SUDEP through other epilepsy organisations, the media and by conferences. The risk of death from epilepsy became a subject for open debate and serious concerns about SUDEP were tackled in an increasing number of medical books.

In 1998 there was front page coverage of the story of Prince John which resurfaced when photographs belonging to the Duke and Duchess of Windsor were published for the first time. ‘HRH Prince John who has since infancy suffered from epileptic fits which have lately become more frequent and severe, passed away in his sleep following an attack this afternoon at Sandringham.’

Prince John, the sixth child of George V and Queen Mary died in 1919 aged 13. (In 2003 the BBC made a drama series about the short life of Prince John).

Epilepsy is often assumed to be a benign condition with a low mortality. There is, however, increased mortality in patients with epilepsy, which is relatively high among younger patients and those with severe epilepsy. (Hauser et al, 1980, Hauser & Hersdorf fer, Nashef et al 1995a).

The risk of SUDEP in the general population of people with epilepsy is of the order of 1:1000 per year, typically a young person, 20-40 years old, with poorly controlled tonic clonic seizures.

Most SUDEP deaths are un-witnessed, but there is evidence that SUDEP may often be preceded by a seizure. For people with severe epilepsy the risk increases to 1:200-300 per year.

In one American study a SUDEP rate of 1 in every 370 people with epilepsy has been suggested. (Leetsma et al 1989). It is however a fact that, through ignorance and misunderstanding - few doctors and even fewer coroners are aware of SUDEP - epilepsy related deaths have often not been accurately recorded and the exact number of deaths falling within the category of SUDEP is not known. By declaring a death resulting from a bath-time seizure as ‘drowning’, or a fatal nocturnal seizure as ‘suffocation’, and by not mentioning the epilepsy connection, valuable research data is being lost. SUDEP is death in an otherwise healthy individual with epilepsy where there is no clear explanation of what caused the death.

Evidence suggests that most sudden deaths are related temporally to un-witnessed seizures and may occur during sleep.

The exact mechanism of SUDEP is unclear although essentially it may be respiratory or cardiac. Indeed there may not be a single explanation for such cases and research is ongoing. One theory that may explain the respiratory factor is that epilepsy itself and/or the medications taken (AEDS) may weaken some major organs, causing patients to have difficulty in breathing, especially during a tonic clonic seizure.

Respiratory problems may be due to an airway obstruction or fluid in the lungs (pulmonary odoema) or the seizure discharges may spread to the respiratory centre and cause a terminal apnoea (cessation of breath). It is known that many people who experience seizures stop breathing for a significant time. While it may be common to recover from a seizure and return to a steady normal breathing pattern, a problem arises when the natural recovery does not happen. In essence the patient could suffocate.

The other possible cause of death is cardiac related problems when seizure discharges spread to areas that control heartbeat and cause a fatal cardiac event. During and / or in between seizures, the part of the brain that controls heartbeat can be affected to the extent that an abnormal heart rhythm develops. It can become so unstable that the heart may suddenly stop beating entirely.

Doctors and neurologists also play a vital role in preventing SUDEP deaths as they are the people who can provide truthful, up-to-date information on epilepsy, and establish a management plan ensuring regular reviews, accuracy of diagnosis, medication, side-effects and impact on lifestyle in order to enhance seizure control. Unfortunately, the syndrome of Sudden Unexpected Death is largely unknown in the medical world, which is potentially very dangerous as it affects the way a patient with epilepsy is treated by his/her doctor, how aggressively the condition might be investigated, and, ultimately, how the patient might approach his/her own epilepsy.

In the U.K. a Government funded report into epilepsy deaths shows that 39% of adult deaths and 59% of deaths in children were potentially avoidable and that short-comings in care may have been a contributory factor. Professor David Fish, Consultant Neurologist at the National Hospital, London, one of the lead authors of the report states: “The report found failures in the provision of care all through the system. This included problems of timely access to expert specialists and a lack of structured and effective review at primary and secondary care. It concluded that poor epilepsy management resulted in a substantial number of potentially avoidable deaths”. The report also found little evidence that the risks of epilepsy had been discussed with patients who subsequently died, or that specialist or doctors made contact with bereaved families to discuss the deaths.

Much of the information here points to doctors and researchers in Britain. It so happens that the UK has a very forward and more complete point of view on E. than does the US. It is a pity that a majority of docs in the US still labor with misunderstandings of E. Telling a patient or that patient's family that there is a significant risk involved with E. might be important for them to know, up front.

Still, perching on one's arrogance, feelings of omnipotence, as a physician, might be the worst thing one can do for an epileptic patient, such as myself. Certainly, telling me I cannot die from E., or that my concerns of E. are all in my head, could be a consequence of either bigotry or ignorance.

The consequence of such ignorance about E., should not be borne by the most vulnerable of us! Nor should it be left for our bereft families to struggle with after we pass on...


Anonymous said...

Excellent words!!

Would you mind posting this to the ecommunities boards? Or do you mind if I create a link there to your blog?

Not a surprise about the UK--my favorite E. author is there and my son's epi always refers to the research and so on coming from there.


athenivanidx said...

I'm not epileptic, but I do know that anyone who thinks epilepsy does not have the potential to be fatal, is a fool.

I lost a friend to SUDEP (well, suspected SUDEP. No one knows exactly how he died but he did have E. and sudep death makes the most sense to me as to what happened.

To not believe that E can be fatal......pure idiocy, or innocence depending on who is thinking that way.....usually the former though

I came to your blog through the disability blog your hardest part submission......

I wrote one as well.

take care

come and visit my blog if you like.

Ivan of athenivanidx

The Bicycle Pilgrim said...

My sister Zein died of suspected SUDEP two months ago, aged just 30 years old. We were never warned by the doctors that a seizure could be fatal. Now I'm wracked with guilt because I know I could have done more to prevent Zein's death. Throughout Zein's life she suffered prejudice and discrimination at every turn. She was fired from many jobs when they found out she had epilepsy. She didn't get enough co-ordinated medical and social support. I feel terrible that she's gone, she was a beautfiul human being.

Mooncalf said...

I am a 30 year old woman with a diagnosis of ideopathic grand mal epilepsy onset at puberty (11). I also have some temporal lobe activity and nocturnal (but no longer diurnal) seizures. A few years back I was diagnosed with sleep apnea, but this article of yours, Paula, makes me wonder if I have sleep apnea or if this is a misdiagnosis.

Jenny said...

I lost my 7 yr old son to SUDEP a little over a year ago. I am trying to raise awareness and offer a support site for other families. Would you mind if I linked your blog to mine?


Anonymous said...

i suffered the seizures, constant prejudice from family and financial destruction for 50 years.
seizures were ended may 22,1981 by surgins knife. the only thing he could not remove is the prejudice and hate people in this country have from the need to be more powerfull than less fortunate.
i am 72 now, have 4 kids and 5 grandchildren who i have never seen or known. meds destroyed me financialy. poor wages like mexicans and blacks were the norm.