Dublin Lectures by Dr Nigel Speight & Dr Vance Spence
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 - www.ashlinghotel.ie/location
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
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. Neverthelessthere 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. Hersymptoms “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 1980s. 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 continuum!
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 hadbeen 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 thegrounds 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 moderaterange 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 changedtheir GP practice after this episode) Fortunately she eventually made a goodrecovery 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, simplysharing her care with her GP, our home nursing service and our dietician (tosupervise 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 avery 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 ofthese 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 approach:
- 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 attendance).
- 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 respond.
- 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 worldoutside 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 couldinclude tenderness and swelling; neurological signs; and a proneness to relapses, which could take the form of recurrences of the original systemicillness. 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 Dundeewere found to have other organic illnesses (e.g. muscle, connective tissue,endocrine disorders 21%), while 12% had a potentially treatable psychiatricdisorder 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.
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 suggests.
He then went on to describe some ongoing research that ME Research UK is funding:
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, includingincreased 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 withME/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 globalhealth (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 whendisruption 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.Higherlevels of oxidative stress, manifested as elevated levels of isoprostanes
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 theseresults mean that people with ME/CFS are at an increased risk of developingcardiovascular 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-TNFα 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 asnon-drug methods, such as tilt-training! After initial training, patients athome (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 mightlie. 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 bothmaximum voluntary contraction measurements and muscle volume were similar inpatients 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 acidtransporter 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.