By David Tuller, DrPH
William Weir is an infectious disease physician in London who has treated many ME/CFS patients. He has been involved in some of the ongoing cases in England in which severely ill teenage girls and young women, and their families, have been in conflict with their local hospitals about the best approach to treatment. In general, hospital consultants and other health care providers have bought into the standard propaganda that ME/CFS patients need either cognitive behavior therapy or graded exercise therapy, most recently expressed in a weekend opinion piece from a prominent member of the CBT/GET ideological brigades. (I responded to that opinion piece here.)
Dr Weir testified last week at the ongoing inquest into the death of Maeve Boothby O’Neill, being held in Exeter, England. He read into evidence two letters that he wrote to express his concerns about the case. The first, dated May 25th, 2021, was addressed to Julia Prague, a consultant endocrinologist at the Royal Devon and Exeter Hospital, where Maeve was hospitalized three times in the months before she died in October, 2021. The second, dated September 9th, 2021, was addressed to Suzanne Tracey, the former CEO of the Royal Devon University Healthcare NHS Foundation Trust.
Because they were read into evidence, both letters can be posted publicly–as I have done below.
**********
Letter to Julia Prague
Dr Julia Prague
Consultant Endocrinologist
Royal Devon and Exeter Hospital
Dear Dr Prague,
Re: Maeve Boothby O’Neill
We spoke about this young lady on the phone last Friday. I want to amplify some of the points I made so that they are on the record.
Firstly, it is very likely that her desire to remain horizontal is related to a low circulating blood volume. A normal blood pressure when supine does not necessarily exclude this. One major contributory cause is likely to be low circulating cortisols, and attempts to sit her up, or even stand her up will always be doomed to failure because of this. There is much in the academic literature to support this, and I have had some success with my own patients with fludrocortisone and regular top-ups with glucose/electrolyte mixture.
Secondly, continuing to lie flat is always going to put Maeve at risk from aspiration, whether or not she has a NG tube in place. It is therefore imperative to try to increase her blood volume, in order to restore her ability to sit up/stand without the risk of postural symptoms, and aspiration. Measuring her blood volume first, as well as her baseline cortisols, will provide the procedural justification for this. Once this has been shown to be the case, it would not be unreasonable to top her up with IV normal saline. I have one patient here in London who does precisely this through a central line, every morning before he starts his day, and colleagues in the United States who have similar patients doing the same thing.
It is also important to recognise that her profound muscular weakness has an organic basis. Essentially, any muscular activity she is capable of is likely to hit anaerobic threshold very early, usually after a very short period of apparently normal muscular activity. One indication of this which is frequently present is a raised serum lactate level, occurring with very low levels of muscular activity. It will be worth checking this in Maeve. In my experience, there is a sequence which many patients like Maeve follow. First, progressive weakness with chewing and swallowing, eventually leading to a very low “exhaustion threshold” for this activity, represented physiologically by the anaerobic threshold being breached. Any test of this activity therefore has to follow her ability to chew and swallow throughout the course of a full meal. This is why a feeding tube often becomes necessary, and it is currently my understanding that Maeve is unable to do this. She may well be having difficulties with the simple act of swallowing a liquid feed on multiple occasions.
Finally I have attached a copy of a synopsis of ME/CFS which I update regularly, and I trust that it will be of help to you in your management of Maeve.
Kind Regards William Weir
**********
Letter to Suzanne Tracey
Ms Suzanne Tracey
Chief Executive
Northern [sic] Devon Healthcare NHS Trust
Dear Ms Tracey
Maeve Boothby-O’Neill dob 26/8/94 xxxxxxxxxxxxxx [Address redacted]
I wish to draw your attention to the problems currently being faced by this young lady. She has recently been an inpatient at the Royal Devon and Exeter hospital (RD&E). I saw her for the first time earlier this year on 21st March when I saw her on a home visit at her parents’ request. She has a long history of ME/CFS, a diagnosis which was first made in 2012 at the Bristol Royal Infirmary ME/CFS clinic. Since January this year she has been mostly bedbound owing to a deterioration in her condition, manifest largely with a sensation of profound exhaustion, giving rise to difficulty in chewing and swallowing, with a tendency also to systemic symptoms with food intake of any kind. This is likely to be due to inappropriate allergic responses to any food that she tries because the mast cells lining her gut are abnormally reactive, a well recognised complication of ME/CFS. As a consequence of all this she has had to resort to liquid feeds, which nonetheless also cause systemic symptoms when ingested. During this last admission to the RD&E I understand that attempts were made to improve her nutritional status, but without much success, probably because of mast cell related damage to the lining of her gut – which can be very difficult to treat effectively. I understand now from speaking to her father, that her nutritional status continues to be seriously compromised, a situation which is potentially life threatening. I have experience of similar cases leading to death and Maeve’s current clinical status shows all the initial hallmarks of this. I understand that Dr David Strain is currently on the case, and wishes to admit Maeve in order to address the possibility of total parenteral nutrition (TPN). This is an entirely logical way of bypassing her gut problems and improving Maeve’s nutritional status. I am fully supportive of this approach, and I am not exaggerating the issue when I say that this may well save Maeve’s life. Furthermore, it is quite likely that, after a period of the nutritional benefits of TPN her gut will have benefited from a period of “rest” and will function normally again.
I now come to the main point of this letter. You may be aware of a major contention within the medical profession concerning the causes of ME/CFS. The belief that ME/CFS has purely psychological causes has been the majority view within the UK medical profession. Consequently patients with this condition have frequently been regarded as perversely inactive, without any regard for the possibility that their inactivity is not due to deliberately perverse behaviour. Incorrect diagnoses of “eating disorder” and “pervasive refusal syndrome” are thus very wide of the mark. They can lead to completely inappropriate management of someone genuinely and severely affected by a condition with demonstrable organic pathology. The latter completely refutes any argument that ME/CFS has psychological causes, and I have set out what is now recognised as the true pathological basis of ME/CFS in the attached synopsis.
A further point I wish to make is that it would appear that a considerable portion of the staff at the RD&E, including some consultants, still hold to the now outdated understanding that ME/CFS has psychological causes. It is also apparent from the way Maeve was treated during her last admission that her illness was regarded by some of the RD&E staff as her fault and that her immobility was self inflicted. Not unreasonably she is reluctant to be readmitted to the RD&E despite her precarious physical state. Dr Strain is therefore facing an uphill task in arranging her readmission, not least because of the apathy of some of his colleagues to what will almost certainly be life saving treatment. I therefore urge you to fully support Dr Strain in what he is attempting to do. At the same time you will need to tackle, head on, the dogma concerning the cause of ME/CFS that some of his colleagues are still perversely adherent to.
William Weir FRCP FRCP (Edin).
I know the details are in the public domain but I think you should remove the family address from your post.
Many thanks for all the work you do for the ME community.
This just sickens me to read. After x2 plus decades myself and thousands of others are treat in this perverse many so called caring health professionals !?
We can not give blood, must be the all in the mind condition we have. There are papers locked away regarding this debilitating illness, must be in our minds.
I do housework and end up unwell for days, it’s in my mind right? Yeah!
From some accounts one might be led to believe that there was nothing that could have helped Maeve, or prevent her untimely death.
From Dr Weir’s letters, it is evident that there were things that could have been done, which sadly included correcting the misperceptions of medical staff about Maeve herself and the disease that was wrecking her life.
IMO, Maeve was “consructively dismissed” from the NHS on which her survival depended. Her ‘treatment’ served only to make her suffering and the disease itself, worse.
Reading this is absolutely devastating. It’s hard to imagine more damning pieces of evidence. David, thank you for continuing to bring this into the light. Is it possible for you to also post the synopsis Dr. Weir attached to his letters?
Day 7 of Maeve’s inquest. More of the same. Everyone’s patient, no-one’s responsibility. That no-one knew about Myalgic Encephalomyelitis is one thing, that they were so biased and unwilling to learn is unforgivable. Her Dad’s (rhetorical) question was repeated again ‘Did you put suspicion before compassion?’ The NHS was not there for Maeve and it’s not been there for my daughter either. People with Severe Myalgic Encephalomyelitis need an NHS that is #ThereforME
Rachel, here is Dr Weir’s synopsis:
Synopsis of ME/CFS
This condition has, in the past, been the subject of considerable controversy. Differing points of view as to its nature and causes are strongly held. There are those who mistakenly believe that it has a psychological basis or even that “it does not exist” and those who recognise it as a genuine physical illness. Suffice to say that, on the 16th July 1998, at a scientific briefing to the press in London at the Royal College of Physicians, the then Chief Medical Officer (CMO) of England and Wales, Sir Kenneth Calman said: “I recognise (ME/)Chronic Fatigue Syndrome is a real entity. It is distressing, debilitating and affects a very large number of people. It poses a significant challenge to the medical profession”. (Ref: Report of the ME/CFS Working Group, January 2002, Page 1, Section 1.1).
Two years after this Professor Anthony Komaroff of Harvard University Medical School stated: “There is now considerable evidence of an underlying biological process which is inconsistent with the hypothesis that (ME/CFS) involves symptoms that are only imagined or amplified because of underlying psychiatric distress. It is time to put that hypothesis to rest” (Ref: The Biology of the Chronic Fatigue Syndrome. Anthony Komaroff. Am J Med 2000:108:99-105).
Both these opinions are further supported by the 300-page landmark report by the American National Academy of Sciences (formerly Institute of Medicine) which reviewed approximately 9,000 peer reviewed studies into ME/CFS in 2015. The report states very clearly that: “ME/CFS is a serious, chronic, and systemic disease that frequently and dramatically limits the activities of affected patients”. (Ref: Report of the Institute of Medicine, Beyond Myalgic Encephalomyelitis/ Chronic Fatigue Syndrome: Redefining an Illness, Clinicians Guide, 10 February 2015, p 4).
The United States Centers for Disease Control and Prevention (CDC) endorses this view. Dr Elizabeth Unger, chief of the CDC’s Chronic Viral Diseases Branch, which runs their ME/CFS programme has stated the following: “ME/CFS is a biological illness, not a psychological disorder. Patients with ME/CFS are neither malingering nor seeking secondary gain. These patients have a variety of abnormalities that affect multiple systems…”
The defining features are an overwhelming sense of fatigue and chronic influenza-like malaise, typically exacerbated after physical or mental exertion. The fatigue is accompanied by a profound lack of energy, it is described as “like no other in type or severity, and is evidently very different from everyday tiredness.” (Ref: Report of the ME/CFS Working Group, January 2002, Page 37, Section 4.2.1.2). The fatigue can be unpredictably variable from day to day and frequently follows a relapsing and remitting pattern. It is unrelieved by sleep, the pattern of which sometimes becomes reversed, with daytime somnolence and night time wakefulness. The incapacity due to this problem is now fully recognised by both the DWP and by NICE. In addition to the physical symptom of fatigue, a number of other symptoms are frequently present.
Common amongst these is cognitive impairment, which can manifest in a number of ways. It has been described thus: “In addition to general cognitive fatigue, other difficulties include reduced attention span, reported impairment of short-term memory, word-finding difficulties, inability to plan or organise thoughts, spatial disorientation, and loss of powers of concentration” (Ref: Report of the ME/CFS Working Group, January 2002, Page 37, Section 4.2.1.2). Other frequently observed symptoms are an extreme sensitivity to any sensory input (Ref: Report of the ME/CFS Working Group, January 2002, Page 37, Section 4.2.1.2), with severely affected patients finding relative comfort only in a darkened bedroom, isolated from even normal levels of environmental noise and light.
The precise cause or causes are unknown, but scientific research has demonstrated immune, endocrine, musculoskeletal and neurological abnormalities (Ref: Report of the ME/CFS Working Group, January 2002, Page 21 Section 3.3). Infections of various kinds seem to act as a trigger in some cases, particularly glandular fever, viral meningitis and viral hepatitis. Other infectious agents such as Herpes viruses, Enteroviruses and Salmonellas can also act as triggers, but to date no known infectious agent has been proven to be the cause of the ongoing illness.
Recent studies have provided further insight into the characteristic intolerance of exercise. It is this feature which is frequently attributed to psychological causes. It has now been shown that calibrated exercise on a bicycle ergometer on two consecutive days indicates clear differences in muscle metabolism between ME/CFS patients and healthy but sedentary, ie deconditioned, controls. In the ME/CFS patients, the “anaerobic threshold” lowers on the second exercise day, whereas it increases in the controls (Ref: Snell et al). In lay terms, the anaerobic threshold is the point at which healthy muscles exercising at maximum level switch to a metabolic pathway which does not use oxygen. This allows for a final burst of energy, followed within a few seconds by a sensation of exhaustion. High anaerobic thresholds are characteristic of athletes, particularly those doing endurance events. In non-athletic but healthy people, repeated daily exercise causes the anaerobic threshold to rise, the result being increasing physical fitness. This does not happen in ME/CFS patients, and as previously stated, the anaerobic threshold goes the opposite way, often with the bare minimum of muscular effort. Misguided attempts to force exercise on ME/CFS patients progressively lowers the anaerobic threshold such that they may become exhausted and bedbound for prolonged periods. The resulting incapacity is sometimes inappropriately attributed to psychological causes, for which there is no scientific evidence base whatsoever.
Exercise thus lowers the anaerobic threshold exclusively in ME/CFS patients with post exertional malaise being one of the clinical expressions of this. This observation has been repeated in more than one academic centre (Ref: Keller et al) It is also supported by a study of biopsied muscle from ME/CFS patients and healthy controls. Repeated electrical stimulation of isolated muscle cells from ME/CFS patients in vitro reveals impairments of metabolism which are not seen in the healthy controls (Ref: Brown et al). Biopsied muscle fibres are self-evidently separate from the owner’s psyche, safely excluding any influence from this source. In badly affected patients the effect of an extremely low anaerobic threshold is severe exercise intolerance which manifests as profound exhaustion, even with the minimal effort of getting out of bed, or such activities as eating and swallowing, or talking for short periods. Tube feeding is thereby a necessity in such patients until recovery begins, often slowly.
Two other conditions which are often associated with ME/CFS must also be mentioned. These are Postural Orthostatic Tachycardia Syndrome (POTS), and Mast Cell Activation Syndrome (MCAS). POTS is characteristically manifest with tachycardia (fast heart rate) and faintness on changes of posture, particularly on sitting or standing up from the horizontal. It is probably due to a combination of a reduced blood volume and dysfunction of the vascular reflexes which normally control blood pressure with changes of posture (Ref: Stewart et al). Consequently, most patients find relative comfort only by lying down for much of the time. MCAS has been discovered relatively recently, with a range of different symptoms. (Ref: Frieri et al) It is due to a tendency for mast cells to discharge inappropriately when “triggered” by normally harmless proteins, or allergens. Triggering results in the release of various substances, particularly histamine. Mast cells are part of the immune system and typically are found in the skin, lungs and the lining of the intestine. Depending on where the triggering occurs, MCAS can manifest as itching, asthma, gastrointestinal disturbances, or in severe cases, anaphylactic shock.
A manifestation of MCAS being increasingly recognised comprises food intolerance, due to mast cells in the intestinal lining being triggered by normally harmless substances in food. Sometimes this is serious enough to cause severe abdominal pain, nausea and vomiting, when food containing these substances contacts the intestinal lining. As a consequence, this can then lead to life-threatening malnutrition because food intake is drastically reduced. Malnutrition is one of the causes of death in ME/CFS if the problem is not treated correctly, and mast cell stabilisers such as sodium cromoglycate (Nalcrom), also antihistamines, can be life saving in this situation. It tends to be an illness of long duration, with some patients remaining ill and incapacitated for several decades (Ref: Report of the CFS/ME Working Group, January 2002, Page 7, Section 1.4.3). Diagnosis is arrived at by undertaking a careful history and physical examination. Other conditions which may cause similar symptoms clearly have to be excluded and laboratory investigation is often required to do this.
Treatment remains both unsatisfactory and a bone of contention. What is not in doubt is that there are no “magic bullets” currently available which will restore complete wellbeing overnight. Management primarily consists of advice to avoid both inactivity and overactivity, to keep active within the individual patient’s (often meagre) limits and to maintain a non-stressful daily agenda – “pacing”. What activity is possible should be kept below the patient’s anaerobic threshold at all times. Cognitive Behavioural Therapy (CBT) has been advocated by some authorities as being of help, but it is by no means curative. Graded Exercise is potentially disastrous because of the potential for exceeding a perilously low anaerobic threshold, and it cannot be more strongly emphasised that exertion, both physical or mental, beyond an individual sufferer’s severely reduced limits causes an exacerbation of symptoms – post exertional malaise. This is characteristically delayed by hours, and sometimes for a day or more, and may last for weeks or months. Emotional stress has the same effect; if overexertion and/or emotional stress persist for a prolonged period of time, this will have a negative impact on prognosis. It should also be re-emphasised that an individual with ME/CFS is particularly vulnerable to the stress arising from light or noise above their threshold of tolerance, which, as stated above, can be abnormally low.
Finally, ME/CFS sufferers do not necessarily look unwell, but symptoms such as fatigue, dizziness, and cognitive dysfunction are often invisible to the uninformed witness. The delay between activity and post-exertional malaise usually means that sufferers may appear normal on a relatively “better” day when they may push themselves beyond their narrow limit, but over the following days they suffer a characteristic crash with further disabling symptoms lasting for days or weeks.
© Dr William Weir
Fellow of the Royal College of London and the Royal College of Edinburgh.
Consultant of Infectious Diseases
Consultant Physician.
WRC Weir
GMC No: 1328909
PS My shortened CV:
I am a Consultant Physician, currently in private practice at 10 Harley Street, London W1G 9PF. My substantive NHS post was as Consultant Physician at the Royal Free Hospital, London NW3, having held this position since September 1987. My main specialty was infectious and tropical disease. I was also an examiner for the Royal College of Physicians for the Diploma of Tropical Medicine and Hygiene during the years 1987 to 2000. During this time I established a major interest in ME/CFS. My interest in this condition included the running of a clinic at the Royal Free dedicated to the care and management of patients as well as providing the basis for a number of academic studies which subsequently appeared in the peer-reviewed medical literature. I was also a member of the Chief Medical Officer’s (of England and Wales) Working Group whose report on ME/CFS was published in January 2002. Since retirement I remain actively involved, both clinically and academically in ME/CFS and a substantial proportion of my present practice comprises patients with this condition. I am also a current member of the NICE Guideline Development Group for ME/CFS. I was elected a Fellow of the Royal College of Physicians of London in 1993 and of Edinburgh in 1998.
WRCW
Thank you, Jenny! I’m loading it into my web reader now.
It is heartbreaking that a young woman was left to die without correct medical intervention, even though those treatments that could have helped her were available and being recommended by doctors who understood her situation. Shameful that doctors who simply “didn’t believe” her illness was real were allowed to remain in charge of her care decisions.
I really wish either Sarah Boothby or Sean O’Neill had asked of each doctor at the Inquest 2 questions.
1) Were they aware then and now that ME/CFS has had an objective marker since 2015 and that 4 diagnostic blood tests are in development?
2) Were they aware then and now that ME/CFS is blood banned in 4 countries including the UK and have they at any time reflected on that?
I think they are questions that the doctors, the media, and the Coroner especially need to hear asked and answered.
Hi Andrea, what is the objective marker? Do you mean the spirogonometry on 2 consecutive days w/ lactate measurement?
Thanks.
I can’t possibly know what happened in this case, but I think there’s a chance that doctors and other healthcare staff may have been trying to do their best by sticking to what they’d been taught. I appreciate that some or many ME/CFS sufferers don’t like to think of their illness as belonging to the MUS set – I’d agree that there are plenty of reasons why it shouldn’t reside there – but I’m afraid that that is how many doctors and other healthcare staff have been taught to view it and may perhaps have been viewing it in this case. In the UK, they’ve been warned that they can cause patients with medically unexplained symptoms iatrogenic harm by focusing their attention on the physical aspect/s of the patient’s condition. They’ve also been taught that the risk of missing organic illness in such patients and misdiagnosing them with ‘MUS’ is quite low (e.g. see presentation available here -https://www.cntw.nhs.uk/style-guide/14422263881427211489dr-sarah-brown-practical-measures-with-your-patients-with-medically-unexplained-symptoms/). Unfortunately, I suspect that most of them have no clue that studies behind (lowish) misdiagnosis rate claims have significant limitations and may not relate to MUS in general or to the particular condition that the patient is suffering from. They may have no idea that the study so often cited for high prevalence rates of MUS in secondary care clinics also revealed unacceptably high rates of MUS misdiagnosis, and that their medical teaching could be leading them badly astray. They may well believe that the illness is ‘real’ if they’ve been taught that ‘real’ means that both physical and mental health components are at play but that in MUS cases the latter predominates – so not “all in the head” but mostly or most often in the head. So when encountering a severe ‘real’ (MIND-body) textbook case, perhaps for the first time, their training could cause them to lean heavily towards psychological care to prevent the patient from suffering that iatrogenic harm they’ve been warned about. If that’s how they think/have been thinking, would they still think that way if they knew that the misdiagnosis rates of MUS in that prevalence study – i.e. the risks of missing and neglecting organic illness, of causing a different (and potentially much worse) sort of iatrogenic harm – were unacceptably high?
If I could, I would try to get inside their heads, to try to understand things from the standpoint of what they’ve been taught – are they incompetent in neglecting a deteriorating patient (e.g. through prejudice, laziness, heartlessness or stupidity) or are they maybe trying to avoid causing their patient what they believe to be significant iatrogenic harm, not realizing that in so doing they may well be causing them terrible iatrogenic harm of a different variety? With the UK’s MUS project, I’d say that physical and mental health have been pitched against each other in the most dualistic way, even to the point of competing against each other as to which approach has the higher misdiagnosis rates. (See the discussion section here – https://academic.oup.com/qjmed/article/93/1/21/1588375 to discover which one won out.) Of course, one shouldn’t generalize about such things – every case is different – for example, for one MUS patient a sizeable dose of radiation via an unnecessary CT scan or X-ray might indeed be harmful while another desperately needs that scan to reveal what is going on; a different MUS patient might gain from a bit of exercise therapy or psychotherapy while another is severely harmed by it. With so many different presentations lumped into the same MUS pot, generalizing is bound to be risky. But an awful lot of generalizing seems to have been done and doctors have been advised that they are far more likely to miss ‘MUS’ in their clinics (read for ‘MUS’ mental/psychological illness) than they are to miss ‘organic’/’physical’ illness. Balance, nuance and flexibility of thought don’t seem too prevalent in MUS teaching – it looks to me like the MUS project and its accompanying teaching have been heavily skewed towards psychological/psychiatric etiology and care and doctors’ heads may indeed have been filled with thoughts of psychological factors rather than physical health problems (see second quote in italics in this paper -https://bmjopen.bmj.com/content/7/4/e014720 ). In this case, when they were weighing up risks, what steered the doctors and healthcare workers towards the decisions that they made? Were they concentrating on Maeve’s individual case and her needs, or were they perhaps lumping her in the MUS/functional category and, with little or no experience of severe cases such as hers, focusing in on what they’d been taught and switching off to warning bells that were sounding around them?
[This is, if you like, a mere personal hypothesis about what might potentially have gone wrong in this case. If ever tested, like all hypotheses (including the ‘functional’ hypothesis), it could of course be proved to be entirely incorrect.]