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This page provides a summary of the
history of SUDEP and explains why the subject became a taboo
and how finally these deaths came out of the shadows.
(Extract from a paper, `History of SUDEP’ presented
at the International League against Epilepsy, British Branch
meeting, Birmingham 1999 by Jane Hanna, Director, Epilepsy Bereaved)
Looking back as we enter the new Millennium,
we might ask why it has taken until the end of the twentieth
century for attention to begin to focus on SUDEP. Although SUDEP
was recorded in the 19th century, for most of the twentieth
century the subject has been ignored and even denied in publications
during the 1950’s and 1960’s. Between the 1970’s
and 1980’s there have been occasional publications, but
significant general interest in the issue is relatively recent.
Hence the oddity of front page coverage of the Story of Prince
John in 1998:
“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 ” The Royal Doctor Alan
Reeve Manby recording the death of Prince John, the sixth child
of George V and Queen Mary who died in 1919 aged 13.
This story of Prince John was hidden from
the public and only resurfaced in February 1998 when photographs
belonging to the Duke and Duchess of Windsor were published
for the first time.
Failure to recognise SUDEP may be explained
perhaps by a fairly glib response that there simply hasn’t
been the research evidence necessary to properly inform about
this subject. The obvious response is to wonder why researchers
in the past were not interested in discovering the facts about
this tragic phenomenon. A less obvious point is summarised in
a recent speech in Parliament and is expounded in the historical
piece that follows :
‘On the crucial question
of research, as recently as the 1980’s, few medical textbooks
dealt with mortality or sudden death associated with epilepsy…More
research is needed, but existing research also needs to be taken
seriously – it has often been neglected or ignored, and
many of the lessons that we are starting to learn now could
have been learnt years, if not decades ago, if that research
had been taken seriously’ (Stephen Twigg
MP, Hansard 17th June 1998)
It is difficult to believe that there was
a deliberate and successful attempt to silence discussion of
controversial research. Nevertheless, it is apparent that over
the last hundred years factors were at play, which resulted
in a powerful myth taking hold, that epilepsy itself could not
be fatal.
19th Century
During the 19th century there was significant
interest in epilepsy deaths, perhaps because this was a time
when people with epilepsy were institutionalized in asylums.
In 1867 3,354 patients with incurable epilepsy were in public
asylums in England and Wales. The medical profession observed
at first hand when a person with epilepsy died. The condition
was viewed very seriously as modern anti-epileptic drugs had
not yet been developed and medical treatments were of limited
efficacy.
An important article appeared in the Lancet
in 1868 written by Bacon based on his experience as Medical
Superintendent of the Cambridge County Asylum. Bacon categorizes
deaths due to epilepsy itself as arising from `sudden deaths
in a fit’, `deaths after a rapid succession of fits’
and `deaths from accidents’. He writes with considerable
concern about the lack of any proper classification of deaths
from epilepsy and suggests that to help research in the future
his classification of epilepsy deaths is adopted :
“ If practitioners would adopt
some such system ...we would not have to lament such a meaningless
blank as the word now represents in lists of mortality”
(Bacon, Lancet 1867)
The criticisms of physicians like Bacon in
1868 that lack of proper classification hindered research into
mortality are mirrored in writings today. Although no consensus
existed at the time as to definitions and terminology in describing
SUDEP, epilepsy specialists at the end of the 19th century were
well aware of the dangers of seizures recognizing that seizure
deaths could be accidental or due to single or serial seizures.
Early 20th Century
Two detailed studies by physicians working
in the Craig Colony at he beginning of the twentieth century
were in no doubt that epilepsy itself constituted a risk to
life.
The studies observe a diminution in status deaths, but no signs
of a decrease in sudden deaths. The view of these writers was
that many of these deaths were potentially preventable.
In 1904 Spratling wrote of epilepsy as :
“ 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.“
Munson wrote a report in 1910 on 582 deaths
among 2732 patients and concluded :
‘A definite and fairly large
group 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’
Post World Wars
Surprisingly perhaps despite this early work
on SUDEP, the subject of epilepsy deaths was neglected for most
of the rest of the century. Research that was done on the subject
was ignored and instead a `myth’ became firmly established
in medical texts that epilepsy itself was not fatal.
An example of this appears in a key text
of the period by Dr S. Livingston, Living with Epileptic Seizures,
1963 where having reviewed the literature he summarises the
position as follows :
‘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’
Some emphasis is placed on a study of 77
death certificates by Schwade and Otto and reported in 1954
which concluded :
‘The study and analysis made
here support the thesis that the epileptic, under
adequate medical control with patient and critical guidance
and understanding of the problem, is substantially a
mortality risk no greater than the average normal person’
(our highlighting)
A crucial part of the passage is underlined
because the conclusion is of course consistent with the view
that if deaths are seizure related then appropriate management
of seizures could prevent deaths.
A notable exception of the period was Rodin’s
textbook, The Prognosis of Patients with Epilepsy 1968.
‘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 five 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. However, it should
be emphasized that in view of the variability of life expectancy,
general statements covering all epileptics are likely to be
an oversimplification’
Dr Lina Nashef offers an explanation as to
why SUDEP was forgotten
‘..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 seizures could not be fatal became ‘common knowledge’
despite evidence to the contrary’
(L. Nashef, Sudden Unexpected Death in Epilepsy,
MD, Thesis (1995).
Between 1970 and the present there has been
a steadily increasing, but relatively isolated scientific interest
within the research community in the subject of epilepsy related
deaths and most especially SUDEP.
‘There should be an increased
awareness that SUD 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.’
(G.Jay and J.E.Leestma, Acta Neurologica
Scandinavica Suppl.82, vol.63 (1981)
Despite this research base, most medical
textbooks over this period gain tended to ignore the subject
or have made assumptions concerning the lack of risk without
reference to the full scientific literature.
Out of the shadows into the 21st
century
During the 1990’s the context changed.
The collaboration of committed researchers from the US, UK and
elsewhere and bereaved relatives together with interest from
the pharmaceutical industry created fertile ground for new initiatives.
The formation of Epilepsy Bereaved, a self-help group for relatives,
provided the impetus for awareness- raising through conferences,
epilepsy organizations and the media to ensure that the subject
of epilepsy deaths was considered part of the agenda.
Epilepsy Bereaved convened an international
workshop on Sudden Death and Epilepsy in 1996 which proposed
a definition for SUDEP; established the current state
of research on mechanisms for SUDEP and risk factors for
SUDEP as well as calling for an urgent national confidential
enquiry into Epilepsy Deaths.
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The international workshop acted as a catalyst
for research and led directly to the National Audit of Epilepsy
Deaths 2002.
CLICK
HERE for the history of the Epilepsy Bereaved
campaign
CLICK
HERE for the National Audit of Epilepsy Deaths
2002
and actions as a result of this report.
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