Important facts about SUDEP, a brief history of SUDEP, some info on current research, news of what is happening in the 21st century to raise awareness of SUDEP. SUDEP - Information 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....


SUDEP - History

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  Throughout history, epilepsy has, quite wrongly, been associated with the supernatural and with madness. People with the condition were thought to be affected by the moon’s phases, hence the notion of ‘moonstruck’ or ‘lunatic’ and were viewed with fear, suspicion and misunderstanding - there was enormous social stigma.

• In developed countries, there have been many advances in medical treatment, and psychological and social issues, but progress is slow and services are still poor. In developing countries there have been very few advances in the field of epilepsy, and of the 50 million people in the world with the condition, some 35% have no access to appropriate treatment, either because of non-existent services, or because epilepsy is not viewed as a medical problem or a treatable brain disorder. For many of these people supernatural views, social stigma and discrimination still prevail and even in developed countries the disorder is still shrouded in secrecy.

• Early in the 20th century, two Physicians, Spratling and Munson, both working in an epilepsy colony, observed from detailed studies that many epilepsy deaths were potentially preventable. 'A disease which destroys life suddenly and without warning through a single brief attack, unaided by an accident to the patient at the moment and does so in 3 to 4 per cent of all who suffer from it”. (Spratling 1904)"A definite and fairly large group (582 deaths among 2732 patients) where neither accident of any kind nor suffocation can be assigned as the cause of death. (which is intrinsic rather than extrinsic)…death is imminent at a time of seizures unless help is at hand…Each patient must be seen every few minutes, for as has been noted, these deaths occur very rapidly at times… The duration of life after the onset of the disease may be several years but as the onset is very common in the early years of life, the net result is the premature death of the epileptic compared with normal people”. (Munson 1910)

• Despite this 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 minimised, then denied, that epilepsy could not be fatal became ‘common knowledge’ despite evidence to the contrary.

• (Sudden Unexpected Death in Epilepsy, Thesis 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).
  

• Today, 2003, there is an increased awareness among health professionals of the need to consider the implications of epilepsy related death for clinical management and information to parents but sadly, there are still a large number of deaths from SUDEP, many of which may be preventable. There are also too many doctors who fear speaking about this difficult subject or are themselves unaware of the risks. In his annual report, 2002, the Chief UK Medical Officer, Sir Liam Donaldson, admitted awareness of epilepsy among doctors is very patchy.

• In May 2002 a U.K Government funded report into epilepsy deaths shows that 39% of adult deaths and 59% of deaths in children were potentially avoidable and that shortcomings in care may have been a contributing factor. The Report launched at the start of National Epilepsy Week was managed and co-ordinated by Epilepsy Bereaved with the participation of five medical royal colleges. Professor David Fish, Consultant Neurologist at the National Hospital, London, one of the lead authors of the report, outlined the findings of the report. He 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 Government acknowledges the fault lines and there will be new guidelines from the National Institute for Clinical Excellence in two years time and more emphasis on neurological conditions like epilepsy. Doctors however believe that without extra funding - funding for epilepsy is very low compared to that for other life-threatening conditions such as asthma and diabetes - nothing will change. To quote Jane Hanna, director of Epilepsy Bereaved, ‘we need real results not rhetoric to make the real improvements that are needed to help prevent the loss of unnecessary lives’.

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  SUDEP is probably the most common category of epilepsy-related deaths and is likely to be seizure-related in the great majority of cases” (Nashef and Shorvon, 1997).
  

• We have known about sudden deaths in epilepsy for a very long time, the main risk factor is the number of seizures a patient has. We also know that two thirds of patients should get complete control of seizures with drugs and that if there are no seizures the risk of death is negligible.” (Professor David Fish, Consultant Neurologist, National Hospital in London).

• 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).

• 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.

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  Research to date indicates that there are certain factors that may make some people with epilepsy more prone to SUDEP but it is important to bear in mind that just because someone experiences one or several of these factors it does not mean that they will die from SUDEP.

• The risk factors of SUDEP include: young adults (particularly males), generalised tonic clonic seizures, poor seizure control, nocturnal seizures, un-witnessed seizures (living alone), abrupt and frequent changes in medication, non-compliance with medication, alcohol, drugs, tiredness and stress.

• Sensible precautions can diminish the risk of seizures and thus the risk of SUDEP and a person with epilepsy has an obligation to himself/herself to maintain a sensible lifestyle because, although drugs control seizures, they do not remove the cause and, more importantly, can only work when taken properly.

• 'All the current literature emphasises the imperative to strive for a balanced lifestyle, which allows for normal activity to be enhanced by a sensible seizure management programme. Optimum seizure control offers the best chance to cheat SUDEP of its disturbing capability.’ (Epilepsy Foundation of Victoria, Australia)

• 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.

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Published on: 2007-05-14 (3554 reads)