The Young ME Sufferers Trust www.tymestrust.org November 2006 ====== Submission to National Institute for Clinical Excellence (NICE) on its draft CFS/ME Guideline ====== NICE requested stakeholder comments on its draft CFS/ME Guideline. This is the full submission made by the Trust. We quote from a new publication - The Nightingale Definition of Myalgic Encephaloymyelitis (ME) - with which our Executive Director was asked to assist. Our submission aims to complement those of many other ME organisations and add something new to the debate, rather than re-iterate all the points so excellently made already. We particularly concentrate on a) the need to separate out ME from CFS and b) the need to provide proper advice on children. All comments are on the short version of the Guideline, the version that would be the most read. Where comments refer to specific page numbers we have inserted them in this transcription. OVERALL VIEW OF THE NICE GUIDELINE The Trust believes that the present draft of the NICE guideline on CFS/ME is unacceptable, and not fit for purpose for patients suffering from ME. ANALYSIS OF THE PROBLEM The problem NICE faces is that it has attempted to put together guidance on a medical condition that has been artificially constructed. CFS is not a discrete disease, it is an arbitrary grouping of symptoms, now with the profile even further widened by NICE. By the very nature of the process by which 'CFS' was created, different pathologies must be trapped within its remit; descriptions of CFS always refer to it as a 'heterogeneous condition' eg the Report of the Chief Medical Officer's Working Group on CFS/ME published by the DOH in 2002. Those who coined the term CFS were divided as to the symptom profile they would research, rather than researching a specific and recognisable disease. Government, physicians and patients are all having to deal with the fall-out of this process. In the Trust's opinion, this guideline as it stands would lead to an unprecedented degree of iatrogenic injury to people with genuine ME, particularly children, those in the early stages of ME, and the severely ill. Those who are not yet severely ill risk being made so, both by the treatments recommended, and by the fact of relapses being trivialised by the term 'setbacks' and patients being urged to continue with programmes despite these setbacks. This is demonstrated in the many accounts we have been given over the years, together with numerous patient surveys such as that by the 25% Group. If a key symptom of a disease is post-exertional malaise, it is illogical and inappropriate to prescribe exercise as a treatment and the damage done by such an approach is evident in patient histories. Before CFS was born (originally for research purposes only) 'ME' was the name for a well-defined, virally triggered, potentially severe and chronic neurological disease. Incorporating it into a collection of symptoms in which 'chronic fatigue' is the main symptom masks its true nature. The fact that the CFS construct has been taken into clinical use compounds the problem. This has put NICE in the position of issuing guidance on an unscientific basis, for a hopelessly mixed group of patients. Consequently, if this guideline were published, physicians face the stark choice of ignoring NICE when dealing with patients who have ME rather than CFS, or risking actively causing harm to this group of patients. They would also have no guidance on how to distinguish this group. Having seen and experienced what comes of trying to put together guidance for 'CFS/ME', the Trust now believes that ME and CFS should be the subject of separate guidelines. Despite the step forward that the (recently updated) Canadian Criteria for CFS/ME represented -criteria which the Trust was the first to recommend in the UK - we believe that ME should now be removed from the CFS bracket and steps taken to issue guidance to doctors as to its true nature, using information from appropriate ME specialists, who will not be those at present advising the government on CFS. They should be drawn from those who have the necessary knowledge, expertise and experience of examining and investigating ME patients and who can point to the infectious origin of ME, its known epidemiology, history of epidemics, known biomedical research profile, testable pathological changes, post mortem findings and other robust scientific evidence. We respectfully submit as evidence selected quotes from the Nightingale Definition of ME by The Nightingale Research Foundation, Ottawa, Canada, with which our Executive Director Jane Colby was invited to assist. The Nightingale Definition will shortly be available in full. The expertise and knowledge that NICE needs on ME is available. The Trust is dismayed that NICE has allowed such a narrow perspective to inform such vital work and requests that it reconsider the whole guideline in the light of our submission, our new evidence, and that of other patient organisations. QUOTES FROM THE NIGHTINGALE DEFINITION OF ME: ME is a clearly defined disease process. CFS by definition has always been a syndrome. At one of the meetings held to determine the 1994 CDC definition of CFS [.] Dr. K Fukuda stated that numerous ME epidemics - he cited the Los Angeles County Hospital epidemic of 1934, the Akureyri outbreak of 1947-48 and the 1955-58 Royal Free Hospitals epidemics - were definitely not CFS epidemics. Dr. Fukuda was correct. [...] Primary ME is an acute onset biphasic infectious disease process, where there is always a measurable and persistent diffuse vascular injury of the CNS in both the acute and chronic phases. Primary ME is associated with immune and other pathologies. [...] Primary ME is a chronic disabling, acute onset biphasic infectious disease process affecting both children and adults. There are both central and peripheral aspects to this illness. [...] Primary Infection Phase: The first phase is an epidemic or endemic infectious disease generally with an incubation period of 3 to 7 days; in most, but not all cases, an infection or infectious process is evident. (See Clinical and Scientific Basis of M.E./CFS, Hyde B, pps.124-126) Secondary Chronic Phase: The second and chronic phase follows closely on the first phase, usually within two to seven days; it is characterized by a measurable diffuse change in the function of the Central Nervous System. This second phase is the persisting disease that most characterizes ME [...] Extent of Injury Type 1: One side of the cortex is involved. Those patients labeled as 1A have the best chance of recovery. Type 2: Both sides of the cortex are involved. These patients have the least chance of spontaneous recovery. Type 3: Both sides of the cortex, and either one or all of the following: posterior chamber organs (the pons and cerebellum), limbic system, the sub-cortical and brainstem structures are involved. Type 3B are the most severely affected patients and the most likely to be progressive or demonstrate little or no improvement with time. Degree of injury Type A: Anatomical integrity is largely maintained in the Brain SPECT scan. Type B: Anatomical integrity is not visible in the CNS SPECT scan. Type 3B are some of the most severely and chronically injured patients. [...] What is new and different about the Nightingale ME Definition is the following: A Testable Definition: The definition is set out in both a clinical diagnostic and scientifically testable fashion. This will allow the physician both an early diagnostic bedside or office understanding of the illness and a scientific and technological method to investigate and confirm the diagnosis. [...] END QUOTES The Nightingale Definition lists the following: Testable Neuropsychological Changes Testable Major Sleep Dysfunction Testable Muscle Dysfunction Testable Vascular Dysfunction. POTS; Cardiac Irregularity; Raynaud's Disease; Circulating Blood Volume Decrease; Bowel Dysfunction; Ehlers-Danlos Syndromes Group; Persantine Effect in ME Patients; ME Associated Clotting Defects Testable Endocrine Dysfunction: This feature is common and tends to be a late appearance. It is most obvious in: Pituitary-Thyroid Axis; Pituitary-Adrenal Axis Changes; Pituitary-Ovarian Axis Changes; Bladder Dysfunction Changes In the Nightingale Definition of ME, more than 30 physicians are listed who have to varying degrees also noted the historical and the more recent investigational findings. We recommend this definition to NICE. FURTHER COMMENT The Trust has been working co-operatively with the ME Association regarding children with ME at their invitation. We endorse the critique of the NICE guideline by the ME Association in its submission. The Trust agrees with the stance taken by the 25% Group on this draft. The Trust agrees with the view of the Edinburgh MESH group and others that patient evidence has not been accorded sufficient weight or respect. This is entirely at variance with the government's own Expert Patient scheme and its aim to involve the Patient Voice. The Trust is in sympathy with virtually all comments that we have read from ME Support Groups and group consortia around the UK. Some responses have included a plethora of detail with research references. We would emphasise that when virtually every patient group and support organisation in the country explains in a respectful and well-defined way that these guidelines are not fit for purpose, NICE would be well advised to take full cognisance of these views. In the Trust's opinion there is a lack of information about children's needs in the guideline and in some ways they are very badly served by it; see our points below, which should be taken to refer to children and young people with ME rather than the broader chronic fatigue. P4 NICE appears to suggest that young people aged 16-19 may choose to remain under the care of a paediatrician rather than transfer to adult services. It is unclear if NICE is suggesting an increase in paediatricians' caseloads and a change in the usual system of transfer at 16. NICE has used the RCPCH guideline to inform this guideline and so has perpetuated some of its mistakes rather than re-considering the issues afresh with new advisers. P34 1.4 1.3 On the severely affected, it is suggested that Graded Exercise Therapy may be appropriate 'to help develop their physical capacity and functioning'. This perception of exercise as being able to 'do the recovery' to the person, is at variance with patient experience, and the clinical experience of other physicians not asked to advise NICE, who maintain that supporting the body's natural recovery process, so that it is able to do more when healing occurs (the same principle as applying a plaster to broken bone) is safer and more effective than trying to force the pace of healing. Capacity extends naturally as healing takes place. In the Trust's opinion, GET should only be considered as an option when a person is sufficiently well into the recovery phase and is much stronger and able to start increasing activity without making themselves worse. Severely affected children are commonly pressurised to increase activity inappropriately and we have seen terrible relapses as a result, with memory loss, paralysis, return to the stage of tube feeding due to inability to swallow. Such relapses can be very long term. One young person of 26 reported still being unable to walk 11 years after receiving such treatment in his teens. Referring to those advocating GET, he wrote to us: 'They must be stopped'. In more than one instance physicians have openly admitted that GET has caused harm and apologised to the family. We do not wish to see further examples of such treatment being meted out to severely affected children. We can see no specific warning such as that set out in Chapter 5 of the CMO's Working Group Report against mistakenly attributing cases of ME to Munchausen's Syndrome by Proxy or FII (Fabricated Induced Illness). This is necessary due to the still persistent mislabelling of families as either neglecting or abusing their children. It seems particularly prevalent in our home county of Essex and our 2005 report 'Our Needs Our Lives' showed an increase rather than a decrease in such problems. We have submitted evidence at the invitation of the parliamentary group that is calling for the withdrawal of the guidelines on MSBP and FII issued in 2002 by the Department of Health under the title 'Safeguarding Children in Whom Illness is Fabricated or Induced'. These same 2002 guidelines are incorporated without amendment at Section 6.6 of the 2006 DfES initiative 'Working Together - A Guide to Interagency Working'. In Chapter 5 of the CMO's Working Group Report, it states: a) In cases of CFS/ME, evidence clearly suggestive of harm should be obtained before convening child protection conferences or initiating care proceedings in a family court. b) Neither the fact of a child or young person having unexplained symptoms nor the exercising of selective choice about treatment or education constitutes evidence of abuse. It is frequently Local Education Authorities and Social Services who report the family because the child cannot get to school in the normal way. Rather than providing them with their legal entitlement to suitable education and abiding by Disability Discrimination legislation, they institute inappropriate proceedings which could be stopped and resolved by well informed physicians. NICE should assist them in this. For further such evidence we refer you to NMEC (National ME Centre, Harold Wood, Essex). We would refer NICE to the short summary document 'Children and Young People: The Key Points', located at www.tymestrust.org/pdfs/keypoints.pdf which lists statements from the CMO's Working Group Report that we consider particularly relevant for professionals working with children. Points such as these need to be clearly incorporated into the NICE guideline. HELPFUL STATEMENTS P10 1.1.1.3 There are some helpful statements in the NICE guideline which we wish to recognise. One is that healthcare professionals for both adults and children should have the appropriate skills and expertise - but who is training them? If the training simply perpetuates the same inappropriate psychologically based attitudes to ME, then this worthy aim will produce harm rather than good. P11 1.1.3.4 The statement that paediatricians should follow advice from the Department for Education and Skills on education for sick children is helpful. However, the publication 'Access to Education for Children and Young People with Medical Needs' by the DfES contains ambiguities that have been the subject of personal discussion between Parliamentary Under-Secretary of State for Schools, Lord Adonis and the Young ME Sufferers Trust. Lord Adonis has, as a result, issued a clarification to state that a GP's support and advice eg for tuition in the home, is valid. A sick child must legally be offered education by other means after 15 days of being unable to attend school through illness. Almost no family will see a paediatrician within this timescale. This should be reflected by NICE by referring to the usefulness of supportive advice from GPs as well as consultants in its guidance. Many GPs do not feel able to give such support at the moment or are ignored when they do. P11 1.1.3.5/6 Sharing supportive information with schools and making recommendations for adaptations to education are both worthy aims but this is only helpful when such information takes full account of the young person's needs, includes full recognition of the disabilities inherent in ME, and recognises that in the 21st century new forms of education are extremely useful to children with ME, such as interactive online education. The Trust works with Nisai Education to provide one such system, which is producing far better educational grades than conventional methods of education for children with ME. School is not a social club and it is perfectly possible to provide for social contact separately. The child has educational rights which are effectively contravened by physicians insisting on school attendance regardless of academic results. There is an increased tendency for medical studies to use attendance at school as a measure of recovery. School attendance is not a suitable measure of recovery in itself; studies shoul d take account of whether or not the young person's academic achievement is on a par with that of their healthy peers as a result of going back into school. P11 1.1.3.6 The recommendation to liaise with Disability advisers is welcome. P11 1.3.1.8 The recommendation to consider the provision of equipment and aids such as wheelchairs, blue badges and stair lifts is particularly welcome as it is not only helpful in itself, it contradicts the view prevalent amongst many physicians that such provision will not facilitate recovery but will instead perpetuate disability. OVERALL COMMENT The guideline uses compassionate language in many places and we do feel that overall the intention is to help patients rather than perpetuate and propagate dogma based on opinion rather than sound evidence. However, the selective use of 'evidence' does just that; it does perpetuate ill-informed opinion and dogma and takes far too much account of studies whose subjects plainly did not have ME at all. Compassionate language in itself is not enough. It is the message that is the most important thing, and the message of this guideline is that many people with ME do not prioritise getting better. This is not only untrue, it is a grave insult to those who are already doing their utmost to cope with one of the most disabling illnesses in existence. Our bottom line is that this guidance should have emphasised helping patients towards autonomy and supported self-management. It does not fulfil this purpose and its sidelining of the specific needs of the young do them a great disservice. We recommend NICE to read Chapter 5 of the CMO's Working Group Report in full. In the Trust's opinion, this guideline needs a complete rewrite, involving a more balanced group of advisers including full patient representation. If it were published as it stands, the political fallout and the personal fallout for patients with ME would be huge. Jane Colby Executive Director The Young ME Sufferers Trust ====== Copyright (c) 2006 The Young ME Sufferers Trust