Tuesday, October 4, 2011

Drs. Speight and Spence tell their history

(website has photos)


The Irish ME Trust will be holding a meeting in the Ashling Hotel
www.ashlinghotel.ie, Parkgate Street, Dublin 8, on Saturday 5th
November at 2.00pm. The speakers at this meeting will be Dr Vance
Spence and Dr Nigel Speight. The Ashling is in a central location
adjacent to Heuston Station, Dublin Zoo, Kilmainham Gaol and on the
Luas red line.  Location map at the following link -

Dr Vance Spence was instrumental in the founding and launching of ME
Research UK. A graduate of the Universities ofLondon and Dundee, he
was a Principal Clinical Scientist responsible for vascular services
and research and, in 1997, he rejoined the University of Dundee
Medical School as Honorary Senior Research Fellow in the Department of
Medicine, with the objective of stimulating research into the causes
of ME.

Dr. Nigel Speight was, before his retirement, consultant paediatrician
at The University Hospital of North Durham, County Durham, and is one
of the most renowned authorities on ME and children.

The following articles comprise an interview given by Dr Nigel Speight
and a synopsis of a talk in 2010 by Dr Vance Spence.

Children and Young people with ME-
A Personal Overview of the Last 20 Years By Dr. Nigel Speight

As documented by Invest in ME at the 2007 London Conference

Having just retired after 25 years as a Consultant Paediatrician with
a special interest in ME, I have been asked to give this personal take
on the last 20 years regarding young people with ME and the way the
medical profession has treated them.

Overall the profession has not (in my opinion) exactly covered itself
in glory in many instances.
It is possible I received an
over-pessimistic picture in that the cases coming to me from other
areas tended to be self-selected hard-luck stories. Nevertheless there
were some definite cases which in my view amounted to "Child Abuse by
professionals", and of course these were mainly due to ignorance about
or disbelief in the reality of ME on the part of otherwise well-
meaning professionals.

Fortunately there is currently a brighter picture and better
understanding and acceptance of ME in the profession. However, I was
still called in on two cases of Care Proceedings in young people with
ME in the South of England in the last 6 months of my career.

My personal story regarding ME

I was never taught anything about ME during my student training or
subsequent training in paediatrics, and became a consultant in a state
of almost total ignorance on the subject, like most of my peers. I had
a slight advantage in that two of my nephews developed the condition,
and as they had both been keen sportsmen and were desperately unhappy
at being unable to continue sport I had an instinctive reaction of
belief in ME as a genuine organic/physical illness, and a natural
scepticism for the widespread view that it was "all in the mind".

About 23 years ago I saw my first case, a 13 year old young lady who
announced her diagnosis to me. Her symptoms "rang true" to such an
extent that this experience cemented my belief system along the lines
of an organic causation. The late Alan Franklin had an almost
identical introduction to the condition at about the same time.

Subsequently I took an increased interest in the condition and cases
just seemed to gravitate to me, both locally, regionally and from all
over the UK. By the time of my retirement I had seen personally c 200
cases in North Durham, 150 in the Northern Region and another 150 from
further afield, including Northern Ireland, the Isle of Man and
Scotland. Many of the cases who came from further afield did so
because of failure to obtain an official diagnosis of ME which had led
the family to feel threatened in a number of different ways, the worst
being threats of Care Proceedings, fines for non-school attendance,
and threatened withdrawal of benefits (or failure to be granted
benefits in the first place).

The controversy as to the nature of ME

Seeing young people develop ME out of the blue in the absence of any
psychological trigger made me question the widely held belief that ME
is a "psychosomatic" disease.
I felt as if I was the little boy who remarked that the Emperor's new
clothes were non-existent.
Accordingly I sent a questionnaire to all
consultant Paediatricians in the Northern Region, sometime in the mid
I was heartened by the response, in that a clear majority (19 versus
7, with about 10 don't knows) shared my belief that ME was primarily a
physical illness which can affect people who are at least initially
psychologically normal. Most of these doctors were general
paediatricians. When I repeated the exercise with Child Psychiatrists,
they almost universally refused to tick any of the boxes on offer but
instead deplored the question and gave me lectures on the mind-body

Basically, this was a reflection on how Psychiatry has been allowed to
dominate the field of ME for the 30 years since 1970, when
psychiatrists McEvedy and Beard first alleged that Royal Free Disease
had all probably been a manifestation of mass hysteria in nurses.
(They did not actually see any of the cases but just constructed their
hypothesis from a review of the
notes) The discipline of Adult Medicine seemed only too happy to
abdicate the field to psychiatry, possibly because with increasing
specialisation there wasn't an "ology" that would own ME. (eg
Neurology, Immunology, Rheumatology, Microbiology etc,
although in
each of these specialties there were individuals who took an interest)

I continued to attempt to fly the organic flag. For instance I
demanded the right of reply at the annual paediatric conference in
Cambridge after a prominent Child Psychiatrist had been invited the
year before. Addressing an audience of c 80 paediatricians I won a
majority vote on a show of hands at the end of my lecture. Agreeing to
see cases from outside my own area was a further very effective way of
highlighting the continuing controversy.

My general approach to young people with ME

The first person to influence me was Dr Betty Dowsett who was invited
by one of our local GPs who believed in ME to give a lecture in our
hospital. She gave such a clear exposition of the clinical features
that she made the condition both "real" and respectable for me, and I
felt empowered to make the diagnosis myself in future. Subsequently I
heard both Dr Alan Franklin and Dr David Bell talk on the same
occasion in Newcastle and this increased my confidence in
understanding the condition. I remember that Dr Franklin said we are
training younger doctors to be too dependent on performing tests on
patients and losing the clinical skill of history taking as a result.
I rapidly realised that ME sufferers want above all for their
condition to be accepted by their doctor and their symptoms validated.
They are enormously grateful for this and very forgiving of our
failure to cure them.
They then wish their doctor to remain engaged
with them and their condition, and not to be discouraged by the
failure of the patient to recover. Too often doctors reject patients
with ME on the grounds that there is "nothing they can do for them".
Even this is preferable to the "one way ticket to the psychiatrist
approach" which is again understandably perceived as a form of
rejection by the patient. This need for validation was brought home to
me by my seeing a young teenage girl in a wheelchair sobbing her eyes
out at a meeting for young people. I asked her mother what was the
matter and who had upset her, only for her to reply "Its all right,
those are tears of joy - she has just heard a lecture by Dr David Bell
after which she said "thank goodness there is one doctor in the
universe who understands what I have been suffering from these last
three years" "!

Another telling anecdote is that of a highly intelligent 6yr old girl with ME whose paediatrician allegedly told her "There can't be anything wrong with you because all your tests are normal" (How many times have the ME community heard something to this effect?) The girl replied with perfect logic and even better grammar "Maybe I've got a condition for which you have not yet invented the right test"!

The challenge of the Very Severe Case

My first very severe case took me by surprise and I made big mistakes
in her management. I had already diagnosed her while she was still in
the moderate range of severity only for her to deteriorate suddenly
following a further viral infection. In retrospect I realise that I
was more concerned for my own position than her welfare, in case I had
missed some other more treatable diagnosis. (This is an almost
universal fear in doctors confronted with ME) I accordingly referred
her to tertiary specialists for second and third opinions, and she was
subjected to numerous upsetting tests and examinations over a three
day period in hospital. This so traumatised her that she had
difficulty forgiving both me and her parents over the next three
years, and this may well have delayed her recovery. Subsequently every
fresh professional whom I introduced to her care managed to upset her
further, giving me grounds for being very sceptical of the orthodox
teaching of the virtues of a multidisciplinary approach!

Things were further set back when the GP insisted on calling a meeting
where the Health Visitor wondered out loud if perhaps the father was
sexually abusing his daughter; minutes were sent to the family in a
spirit of openness! (Not surprisingly the family changed their GP
practice after this
episode) Fortunately she eventually made a good recovery despite having been
bed- ridden and tube-fed for 3 years. My next case was almost exactly
similar but I handled her according to my new convictions. I strictly
limited her from too much contact with other professionals,
simply sharing her care with her GP, our home nursing service and our
dietician (to supervise her tube-feeding) This second case did much
better emotionally, and made a total recovery within 2 years.

I did not involve Child Psychiatry, Physiotherapy, Occupational
Therapy or any other disciplines and she did not appear to suffer from
their absence, making a total recovery in 2 years after 9 months of
tube-feeding. In my experience of cases in the rest of the country,
this scenario of the paediatrician being panicked by meeting a very
severe case is really quite common and has contributed to some of the
cases referred to Social Services.

Child Protection Cases
Every one of these was a nightmare for the young person and the family
and in my view added insult to injury for young people deserving
sympathy and support but getting the opposite. I was involved in over
20 of these cases, all of which reached the stage of a Child Protection
Case Conference. There was usually a combination of one or several of
the following factors operating to lead to a Child Protection

Single mother.
A disbelieving and usually absent father.
Other frustrating medical problems eg allergies.
A record of the family having put pressure on doctors in the past eg
for second opinions.
A lack of an official diagnosis of ME.
Another family member suffering from ME, often "unofficially".
Severity of the ME, deterioration or failure to respond to some form
of medical regime.
A reluctance on the part of the family to be referred to Child
Psychiatry, especially if it involved admission to a unit and
restriction of parental access.
A tendency for the case to be driven by doctors who had never actually
been clinically responsible for the young person, who had not
therefore taken a history and were thereby prone to disbelief (usually
Community Paediatricians, often concerned about poor school
A failure of doctors and/or Social Workers to actually talk to the young person.
A belief on the part of doctors in the efficacy of their "treatments",
leading to the mother or young person being blamed for the failure to
A frequent tendency to invoke the spectre of Munchausen Syndrome by
Proxy (MSBP, aka Factitious and Induced Illness) whereby the mother is
alleged to be inventing/exaggerating her child's symptoms for some
perverse motive of her own.
A distressing sense of self-righteousness on the part of the
professionals involved and a reluctance to open their minds to the
possibility they were perpetrating a grave injustice. The term "group
folly" sprang to mind as each professional sheltered in the security
of the group decision, scared to break ranks.

In this last respect Chris Clark (Former CEO of AfME) said to me after
hearing some of these stories "It actually smacks of sadism". The good
news is that in every case bar one I was able to reverse the Child
Protection juggernaut by my report for court. In addition to making an
official diagnosis of ME, I spoke to the young person on his/her own,
was often able to assert that the young person was "Gillick competent"
and did not consent to be taken into care.

I would love to say that we have seen the last of this sort of case
but fear we have not.

Dr. Nigel Speight was, before his retirement, consultant paediatrician
at The University Hospital of North Durham, County Durham, and is one
of the most renowned authorities on ME and children.

Dr Vance Spence was instrumental in the founding and launching of ME
Research UK. A graduate of the Universities of London and Dundee, he
was a Principal Clinical Scientist responsible for vascular services
and research and, in 1997, he rejoined the University of Dundee
Medical School as Honorary Senior Research Fellow in the Department of
Medicine, with the objective of stimulating research into the causes
of ME.

Sheffield ME Group Talk by Dr Vance Spence October 2010

Biomedical research in ME/CFS: Today and Tomorrow

Vance explained that he had been ill for almost 30 years with an
illness which people call "ME" or "CFS", but without appropriate tests
who could tell? He realised there was a burning need for biomedical
research, hence the charity ME Research UK (website
www.meresearch.org.uk) of which he is Chairman, and which celebrates
its 10th anniversary this year. Dr Spence reminded the audience that
he was not a medical practitioner but a clinical scientist; before
contracting ME, he was a Principal Clinical Scientist in Vascular
Disease and Director of Vascular Research at the University of Dundee.
He explained how the charity operates and how it fulfils its main role
- of commissioning and funding scientific (biomedical) investigation
into the causes, consequences and treatment of Myalgic
Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS). In the past
decade, the charity has grown in size and respect, punching above its
weight in a variety of spheres. For instance, it has funded 29
specific investigations, most in the past six years in Britain and
abroad - that's more specific research projects on ME/CFS than any
other single organisation in the world outside the American continent. 


Vance spent a long time discussing ME, "CFS", fatigue and the
complexity of diagnosis nowadays. He described how patients today are
diagnosed with ME/CFS - a diagnostic mess in a variety of ways.
Originally, the label Myalgic Encephalomyelitis (ME) was used for an
illness that had been found to occur in epidemic and sporadic forms,
believed to be caused by a continuing or persisting viral infection.
Its characteristics were a profound, generalised post-exertional loss
of muscle power (fatigability); muscle pain that could include
tenderness and swelling; neurological signs; and a proneness to
relapses, which could take the form of recurrences of the original
systemic illness. But, as there was no specific diagnostic test for ME,
and since post-exercise "fatigue" was one of its prominent symptoms,
people with ME began in the 1990s to be diagnosed with "Chronic
Fatigue Syndrome" (CFS), or the compromise diagnosis ME/CFS, which is
based on a collection of vague non-specific symptoms (fatigue, sleep
disturbance, sore throat etc) shared with other illnesses.

The "F" word

So what do the people today diagnosed with ME/CFS actually have wrong
with them? A good question, and at least 2 studies have tried to tease
this out.
First, some people with a diagnosis of ME/CFS when examined at the
University of Dundee were found to have other organic illnesses (e.g.
muscle, connective tissue,endocrine disorders 21%), while 12% had a
potentially treatable psychiatric disorder that could account for their
symptoms, and 7% had fibromyalgia. In the second, 44% of ME/CFS
patients in Newcastle were found to have other diagnoses, e.g., sleep
apnoea or depression and anxiety, to account for their symptoms.

So, it seems that around 40% of patients given a diagnosis of ME/CFS
might, in fact, have other things wrong with them
. So, the key is for
people to get a thorough medical examination initially to exclude
other more treatable conditions, and only then to be given a diagnosis
of ME/CFS.

Funding crisis

ME/CFS Biomedical Research: Quo Vadis?

Vance mentioned that while these diagnostic problems caused problems
for clinicians like GPs, they also complicated research studies
because volunteers had to be screened and categorised by medical
examination before they could be enrolled into studies - raising the
costs of medical research
substantially.     And  he explained that the funding available for ME/CFS
research was small; for instance, Cancer Research UK's income 2008/9 was
£498,221,000, compared with ME Research UK's income of £264,862 for
the same period.

Biomedical research that patients want to see is not happening. As
most research funding for many, if not all, illnesses comes from
charitable sources, i.e. directly or indirectly from public donations,
we have to beef up our efforts, increasing funding by a factor of 10
to 100, and attracting new blood and fresh ideas into the field. Only
then can we being to see a large number of research groups undertaking
the large number of studies we all want to see, attracting new blood
and fresh ideas into the field of ME/CFS research, as the slide

Research projects

He then went on to describe some ongoing research that ME Research UK is

a) Studies at the University of Dundee

(i) Inflammation and apoptosis - children and adults

With funding from ME Research UK, researchers at the Vascular and
Inflammatory Diseases Research Unit, University of Dundee, have
uncovered a range of potentially important cardiovascular findings in
ME/CFS patients, including increased oxidative stress (these toxic
molecules can, amongst other things, damage blood vessels), abnormal
metabolism of acetylcholine (an important neurotransmitter and dilator
of blood vessels), and increased early death of white blood cells
(which may indicate active inflammation).
All this has provided accumulating evidence of a compromised
cardiovascular system in patients with ME/CFS, and of the potential
importance of inflammation in this disease process. And the most
recent finding - of similar abnormalities in children with ME/CFS -
also points in this direction.

In 2010, Dr Gwen Kennedy and her colleagues in University of Dundee have
published results on children with ME/CFS,     to see whether the
abnormalities found in adult patients are also present in children
with ME/CFS. But another aim was to investigate objectively the quality
of life of children with ME/CFS. Her main finding of the study (funded
by ME Research UK, the Tymes Trust, and Search ME) was that children
with  ME/CFS scored significantly lower than the healthy children in
10 out of 14 areas covered by the Child Health Questionnaire. They had
particularly low scores for global health (21.4 compared with 84.1 in
the healthy children) and for social limitations due to physical
health (24.9 compared with 100).
Self-esteem, mental health, body pain and discomfort, and the effect
of the child's health on family activities were  also significantly
worse for children with ME/CFS. However, there were no differences
between children with ME/CFS and healthy children in how well the
family got along, or in the children's perception of their own
behaviour. Importantly, the illness had started with an infection in
88% of the children, which confirms the known association between
initial infection and subsequent development of illness.
Also, a significant proportion of children in the study had
interrupted schooling, and only 1 of 25 children was able to attend
school full-time, a finding which accords with other studies on the
interruption of education in children with ME/CFS. Fortunately, just
over half of the children who participated felt that their symptoms
were improving, and the prognosis for children with ME/CFS is
generally thought to be better than for adults, although no long-term
studies have been conducted. Overall, Dr Kennedy's findings confirm
that ME/CFS does have a serious impact on children's quality of life,
and she comments: "This experience of illness occurs at a particularly
vulnerable time of life when disruption to education and family has the
severest consequences... it is important that the condition be
recognised and diagnosed so that the consequences on quality of life
can be attenuated."

"We believe that the data presented herein are consistent with the
finding that many patients with CFS/ME have an underlying detectable
abnormality in the behavior of their immune cells consistent with an
activated inflammatory process...."

Coming to the biochemical measurements, compared with healthy control
children, the young people with ME/CFS had:

1.Higher levels of oxidative stress, manifested as elevated levels of

2.Reduced levels of vitamins C and E

3.A greater percentage of white blood cells undergoing apoptosis.

The   increased  apoptosis  (or programmed cell death) may be caused by a
number of factors, including a persistent viral infection or toxic
agent, or an abnormal immunological response. This finding is
particularly intriguing given that many patients, including most
children in this study, report that their disease started following a
viral infection of some kind. At present, however, there is
insufficient evidence to make a causal link between infection and
increased apoptosis, though the finding is tantalising.

(ii) Arterial stiffness

Coming to adults with ME/CFS, Dr Faisel Khan in Dundee found that
patients with ME/CFS had significantly stiffer arteries than healthy,
age-matched control subjects, and they also had higher levels of C-
reactive protein, indicating significant inflammation and oxidative
stress. Furthermore, there was evidence of a relationship between
arterial stiffness, and inflammation and oxidation. The cause of
increased arterial stiffness in ME/CFS is still unknown. While
lifestyle characteristics such as smoking, obesity and physical
fitness also play a role in its development, the patients in this
study were no different from the control subjects in this regard. Dr
Khan is, however, careful to emphasise that this is an association
only and that the current results do not prove cause and effect.

Do these results mean that people with ME/CFS are at an increased risk
of developing cardiovascular problems such as heart disease? At the
moment, no-one knows
but the work does raise the possibility that
suppressing inflammation in carefully selected patients may lead to an
improvement in arterial stiffness and a reduction in long-term
cardiovascular problems, something already achieved in patients with
rheumatoid arthritis using the anti-TNF╬▒ drug, etanercept. Clinical
trials are unfortunately very expensive so conducting a similar trial
on ME/CFS patients would need the sort of investment that only
pharmaceutical companies can deliver. However, further research is
needed before this can be answered definitively.

b) Studies at the University of Newcastle

(i) Autonomic nervous system (ANS), including heart

The autonomic nervous system (ANS) controls cardiovascular, digestive
and respiratory functions, and has a range of other important roles.
When it goes wrong, the consequences can be severe. Since one of the
key difficulties that ME/CFS patients face is standing, most
especially standing still, without experiencing symptoms such as
dizziness, altered vision, nausea, fatigue etc, the possibility exists
that there could be a problem with the autonomic nervous system.
Professor Julia Newton of the School of Clinical Medical Sciences,
University of Newcastle, has been looking at the ANS in ME/CFS
patients since 2006 with funding from ME Research UK. Vance mentioned
that Julia Newton gets her patients from the NHS CFS clinic in
Newcastle, and that it might be possible for some people to obtain a
referral there, though of course all referrals must come via a GP,
since they are the front line access points to secondary care.

In a series of fascinating scientific papers, Prof Newton and colleagues
have  shown that     autonomic dysfunction is present in three-quarters of
the patients - a most unexpected finding. Furthermore, she has also
shown that a simple-to-measure assessment of the heart rate response
to standing was abnormal in nearly 90% of patients
. Because of this,
MERUK has given its largest ever research award of £130,000 to
Professor Newton for a two-year study of autonomic nervous system
(ANS) dysfunction at the University of Newcastle, funding provided in
conjunction with the John Richardson Research Group and the Irish ME
Trust. Vance described how this award was intended to extend and
explore some of the mechanisms behind these autonomic problems in
ME/CFS patients.

In separate studies, Prof Newton is also looking at ways patients can
deal with the symptoms of ANS dysfunction, and there are some drugs
available, as well as non-drug methods, such as tilt-training! After
initial training, patients at home (with another person there for
safety) lean against a wall with their feet 15 cm away, increasing the
length of time to 30 minutes over days to weeks (evidence suggests
that such once-daily tilt training can be effective in preventing the
recurrence of fainting).

Autonomic dysfunction in ME/CFS

Newton JL et al. Symptoms of autonomic dysfunction in CFS.

The autonomic nervous system also plays a part in regulating events in the
exercising muscle, however, and the Prof     Newton    and   colleagues
hypothesised that might be involved in the exercise-induced symptoms
so characteristic of ME/CFS. To examine this, they enlisted the help
of phosphorus magnetic resonance spectroscopy (MRS), a marvellous tool
which allows assessment of acid (pH) handling inside the muscle where
the problems might lie. The results published in Journal of Internal
Medicine (2010) show significant impairment of proton excretion in
recovery phase following exercise - in simple terms, ME/CFS patients
recovered substantially more slowly compared with controls
. Could
simple deconditioning be the cause?
Probably not since both maximum voluntary contraction measurements and
muscle volume were similar in patients and in the sedentary controls.
Rather, the researchers think it more likely that impaired acid
handing could be one of the mechanisms through which autonomic
abnormalities act to produce post-exercise symptoms and fatigue, given
the role played by the autonomic nervous system in regulation of
acid transporter pathways and vascular flow in muscle.

Despite the key role of post-exercise symptoms in the illness, there
has actually been very little scientific investigation into muscle
physiology during exercise in ME/CFS - a fact that makes these novel
findings so important.
Based on these results, ME Research UK has now
actioned funding for the next step, and examination of the function of
an energy-generating enzyme which might be under- performing in people
with ME/CFS.

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