Pediatric Primer Not For Prime Time

Guest post by Denise Lopez-Majano

Young people with ME face huge difficulty in finding knowledgeable healthcare providers because there is only one full-time pediatric ME specialist in the United States, and he is unable to take on any more patients.

So I was very hopeful that the new pediatric primer – Myalgic Encephalomyelitis/Chronic Fatigue Syndrome Diagnosis and Management in Young People: A Primer – would better equip pediatricians to diagnose and help young patients with ME. After all, the primer’s authors include most of the world’s pediatric ME specialists, each of whom have decades of experience and insight regarding ME.

Unfortunately, for a number of reasons, I do not think the pediatric primer is ready for prime time.

There are some things the primer does very well:

  • The Etiology and Pathophysiology section is very thorough and clearly states this is a multisystem physical disease.
  • The primer addresses the differences between ME and factitious disorders, pervasive refusal syndrome, school refusal, and somatoform disorder. This is critically important given the number of young patients who have been referred to social services or even removed from the home.
  • The “Pediatric ME/CFS Diagnostic Worksheet” ensures that frequency and severity of symptoms is recorded.
  • Table 5, “Symptom comparison between depression/anxiety disorders and ME/CFS in children and adolescents,” clearly shows the differences between ME and depression/anxiety disorders.
  • The section on the very severely ill has good suggestions to help make hospitalization easier.

However, there are serious flaws and deficits in the primer that must be corrected before it receives broad distribution.

First of all, the primer uses problematic language to describe people with ME:

  • “Severely ill young patients are often difficult to manage….” (p.24) This statement is completely inappropriate. It is NOT the patient who is difficult to manage. It is the disease that is difficult to manage.
  • The section on “Very Severely Affected Patients”states “These patients are fortunately, relatively rare.” (p.24) The truth is we do not know how prevalent they are. Severely ill patients may be less likely to go to pediatricians or participate in the very few pediatric studies conducted to date. There have been no prevalence studies in pediatric patients. Until we have good data, no one can know the prevalence of severely or very severely affected patients.
  • “A child or adolescent affected with ME/CFS presents challenges for the entire family.” (p.26) Again, the patient is not a challenge, and should not be made to feel this way. The disease is a challenge, not the child afflicted with it.
  • “A patient’s needs early in the illness might differ from her/his needs in later years, as health improvement is being achieved.” (p.17) This implies that all patients improve, and ignores those who do not. Claiming that all patients improve could have a negative effect on those who don’t, especially in the context of working with pediatricians not expert in ME.

Second, the primer presents confusing and mixed messaging on the diagnostic criteria for ME:

  • The suggested diagnostic criteria do not match any existing case definition. Instead, the authors base their criteria “on their collective experience and insight.” (p. 6) While the authors’ collective experience is incredibly valuable, creating yet another set of diagnostic criteria only adds to the existing confusion we already face.
  • Furthermore, the core symptoms listed on page 6 differ from those in the fact sheet about ME (p.35). These inconsistencies will make it virtually impossible for non-expert pediatricians to accurately diagnose ME.
  • Too many terms are used for what happens when patients exceed their activity levels and they are worse off than usual: post-exertional worsening of symptoms, post-exertional malaise, post-exertional collapse, post-exertional relapse, post-exertional flare-up, post-exertional symptom exacerbation, post-exertional exacerbation, symptom flare-up, crash, etc. In fact, the section on “Fatigue, Post-exertional Symptoms, Exercise Intolerance” (p.15) does not even use a name for this cardinal feature of ME. The authors need to decide on and use one name consistently to emphasize the significance of this cardinal symptom of ME.
  • The primer discusses a number of the case definitions currently in use, but then dedicates an appendix to the Fukuda definition. Detailing Fukuda in its own appendix perpetuates its inappropriate use. This is harmful because Fukuda does not require post-exertional worsening of symptoms, even though this worsening is part of the core criteria in the primer. The authors even acknowledge that Fukuda “can exclude some young patients with ME/CFS and include others, who are later found to have another illness.” (p.6) Why these distinguished authors would highlight Fukuda over other case definitions, despite the flaws they point out, is a mystery to me.

Third, improvements are needed in the discussions of educational and social issues:

  • The section on severely affected patients (pp. 24-25) should discuss the importance of trying to develop/maintain social connections. It should also emphasize that while pediatricians should feel there is reason to hope for improvement, they should never lay the blame on patients who don’t improve.
  • There should be greater emphasis on cognitive exertion as a possible cause of the worsening of physical and cognitive symptoms. It needs to be made very clear that this disease imposes cognitive limits that vary greatly from day to day, and from patient to patient, and cognitive exertion can impact a patient just as much as any physical exertion.
  • It should be emphasized that, for those able to continue schooling, educational courses must be at the student’s intellectual level. It should also be stressed that even though providing these accommodations may be more challenging for schools than putting the student in a class they could more easily pass, doing so would be intellectually frustrating for the young patient.
  • The primer states that “education is a key issue that improves their ability to function regardless of whether or not they recover.” (p.26) I agree that education is critically important, but there are scarce data actually showing that education improves functional outcomes.
  • “IQ scores might be lower than the scores of healthy peers.” (p.27) Properly administered tests will show that scores are artificially low because of impaired processing speed and impaired working memory, etc. Pediatricians (and others) should not be led to believe that the young patients have lower IQs or are less intelligent that their peers. The intelligence is still there; the ability to perform tasks at the same speed or in the same way as others is the problem.

We all want this disease to be taken seriously by pediatricians, and we desperately need more pediatricians who can help young people with ME. As much as I want to share the primer far and wide, I am reluctant to do so in its current form. The primer does not convey consistent information clearly enough to help pediatricians understand this disease and its impact. Don’t get me wrong. I truly appreciate the work of the primer authors. But I think the primer wasn’t ready for publication. I hope there is a revised version soon, so we can truly equip pediatricians to diagnose and manage ME in young patients.

Note: Comments are closed as of August 13, 2017 due to a large number of spam comments.

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41 Responses to Pediatric Primer Not For Prime Time

  1. rob wijbenga says:

    chapeau Jenny, This is very thorough and very appropriate commentary, and should be taken to heart by all reseacrhers and clinicians involved

  2. Cindy Downey says:

    Jennie, thank you all your work on this, and kudos to the authors of this primer for applying their vast experience to this document.

    Jennie thank you for pointing out the thought traps we can all fall into; e.g. the disease is actually the challenge, not the patient. It is not the patient’s fault they have the disease. To blame others for their circumstances is so ingrained in our thinking that we are not aware we are doing it. And, with ME, that’s been a huge problem all the way along. People with ME blamed for their disease.

    And, thank you for discussing the IQ issue. I have read that IQ points are lost, but it makes sense that thinking speed, and short term memory problems are the issues. Of course, then there is the underlying neuroinflammation itself that seems to be causing the above problems.

    Also, pointing out the trap around assumptions everyone with ME improves (recovers?). Since this disease is trivialized by those not really familiar with it, it is assumed by those unfamiliar with ME that everyone recovers, “comes to their senses”, etc. If a person with ME doesn’t improve/recover, they are often blamed for that lack.

    And, the list goes on! A big thank you, not just for this, but for all your years of informing and pushing ME forward!

  3. Chris Heppner says:

    Denise, this is a terrific blog–I read the primer, and felt that though the primer contains much useful information and suggestions, it would not work very well as a primer for pediatricians not already very familiar with ME/CFS. You have focused and nailed down just what is not satisfactory about this potentially very useful text. Thank you! Might I add that it is maybe a bit too long?

    • Jennie Spotila says:

      The primer is too long or Denise’s post? Just kidding. Denise worked hard to make her comments concise.

    • Denise says:

      Thank you Chris.
      The primer is 44 pages including references and appendices. I think that is probably long for many healthcare professionals but others disagree with us. That said, I am not sure what could be cut. (If you have thoughts on what to cut etc, let me know.)

      • Janelle says:

        Maybe they could make some short document that start with the basics, for an uninformed doctor who needs to start with, yes, this is an actual disease and it’s serious.

  4. Cindy Downey says:

    It’s the ME! Sorry, Denise, I didn’t note your name as guest blogger. Super work! Thank you very much!

  5. Many of the complaints about the primer are still true of the way we adults are treated by medical folk!

    I don’t even bother going to any of the doctors I might be able to get to – I live my tiny life and do all the self-help methods. The trial-and-error methods I have tried in the past were exhausting – and didn’t help. The doctors were willing to try things, but I was the one who had to suffer through them. The only one I had to persist with was pain management, a battle I still have to fight.

    I can’t imagine the life of kids with that kind of pain.

  6. Mary says:

    This is so clearly expressed. The points are very well made. I found it helpful to read this as a quite old with ME woman. Thank you for the energy you expended on this on our behalf.

  7. Melee Barkis says:

    The single factor that there are citations related to ANYTHING Wessely School of Cruelty & Abuse incites immediate acute anguish and distress.

    This Primer will – even if in the slightest bit – offer a lifeline to the flailing efforts surrounding the sinking PACE ship, and to be sure, they will grab a hold and use this Primer as evidence of PACE’s valuable contribution to those bastards who don’t want to get well in the ME community.

    Sir Weasel himself, along with the Succubus Crawley, Not-So-Sharpe, and other PACE ideology indoctrination advocates have their names all over this cluttered up jumble of words.

    This Primer’s only use should be as the liner in an experimental lab rat’s cage. It’s a fine mess.

    • Denise says:

      It is likely I did not catch everything so I hope you will point out specific concerns Melee.

      fwiw – I thought the authors did a pretty good job of debunking CBT/GET and PACE.

      • Melee Barkis says:

        Debunking out of one side of your mouth is only as good as the double-talk coming out of the other side of your mouth.

        For example: slippery double-talking (lying) Republicans got elected by promising a clean repeal of Obamacare: “every. single. word.” Now, they’re not only NOT going to send a clean repeal bill to President Dumpster Fire, but they’re using Obama’s Marxist foundation to propel the Republic further into a state of tyranny.

        What the authors of this Primer did is more aptly described as “doing a pretty good job of appeasement” – and we all saw how that turned out for Chamberlain.

  8. Carollynn says:

    What can we do to help these changes to be made?

    • Denise says:

      Good question. I am not entirely sure.
      Perhaps those who are able can list additional concerns here, which can then be compiled and provided to the authors of the primer.

      • Carollynn says:

        I don’t think I’m qualified to make specific suggestions (though I read with curiosity, wondering about myself as a child 50 years ago and my nieces in recent years with different health concerns that make me wonder for them).

        In instances like this, would it be more helpful to suggest edits of specific sections? “Showing” rather than “telling”?

        You did a great job on this blog, Denise. I’m really glad that people with real experience with the Pediatric end of this terrible disease are jumping in. Again, I don’t feel qualified to do the edits, I would be glad to sign in support of them. It may help to have dozens, hundreds of signatures in support.

  9. Laurie P says:

    Great job, Denise! I’m so glad that you’re doing this. It’s unfortunate that it seems to be a big task.

    I was just briefly skimming through parts to see what was in it, stopping to read here and there and I noticed this on page 24:

    “Treatment planning of elective dentistry in more extensive cases should allow time between appointments to recover from fatigue.”

    It should say PEM not fatigue and return to baseline; or something to that effect, is much better than recover. If, as it’s worded, we could recover from fatigue, we wouldn’t have this disease!

    • Denise says:

      Good point. PEM is not fatigue and fatigue is not PEM.
      Something I think the primer would benefit from is a glossary that clearly defines terms such as recover, recovery, relapse, baseline, normal (and so on).

      • Laurie P says:

        Can a group of people with ME and caregivers write a patient glossary and guide type primer to try to standardize how we are all using language? We could then distribute that to doctors. Maybe one of our experts could read it over, add their name and we could try to get it published and added to the CDC site???

    • Melee Barkis says:

      Again this Primer has produced a hot mess, as indicated in just Laurie P’s one-sentence quote. Not only should the word “fatigue” be stricken, but dentistry and ME is a compelling topic, and more expansive information on how dentists must avoid injuring patients should be included. I will use my own experience as an example of how I came to a deeper knowledge of my specific subset of the disease through dentistry.

      After I became severely ill, I could not endure dental work without IV sedation. One dentist after the other threw me out of their office because “CFS is not a real thing and you need to grow up and stop being so sensitive.”

      I had to get smart about dentistry fast, because this was not elective dentistry, and my mouth was in trouble. So my horrific experiences with dentists opened a window in regards to treatment for me, as I searched for sedation dentistry with an empathic dentist.

      I found the kindest, gentlest, most caring dentist anywhere – he is a true gem, and I feel so lucky; plus he’s super young, so he’s not going to retire or die before me, so lucky me!

      Unexpectedly – and shockingly – I experienced short periods of semi-remission after each IV sedation dentistry session: the longer the dental session, the better I felt and the longer I experienced a semblance of remission (about one to two weeks each time). When the drugs finally wore off, I felt like a new person. Of course I always lapsed back to Severe ME again, but the cause and effect gave me personal insight into how to expand my own research efforts, and this is how I came to research Dr. Jay Goldstein’s Ketamine Treatment Protocol.

      My gem-of-a-dentist told me that he uses some of the elements/drugs in Dr. Goldstein’s Ketamine Protocol. Now, because of IV sedation dentistry, I look forward to undergoing treatment at a Ketamine Clinic in the future. It will be rigorous (it’s a 3 hour drive round-trip, for 3 days in a row, for appx. 6 hours a day, and then back again for another round 2 weeks later), but I feel like if I can do it, it just might be worth it. The only question is if I actually CAN do it (I can’t do anything 3 days in a row, let alone get more than 20 feet away from the bed and out of the house, so we’ll see).

  10. Janelle says:

    Very good comments, thanks for writing this. And thanks, Jennie, for giving it space.

    The authors suggest that thinking foods make one worse might be a “food fad.” While I agree there is little value in fad diets just to have a food plan which purports to increase health, there are actually several reasons why eating or eating specific types of foods might cause symptoms. These should be investigated for potentially treatable conditions, and food sensitivity claims should be believed.

    • Denise says:

      Very well said Janelle. Thank you.

      • Denise says:

        I should clarify – your comments about food sensitivities, investigating them and believing the patient were very well said.

        • Janelle says:

          Thanks. I was trying to post again but my device ran out of batteries and I had to come back. In a later section they do mention both sensitivities and some things one might investigate, so I am not sure the point of the first section.

          In the later section, it could be more comprehensive. They don’t mention, for instance, MCAS, nor some specific food classes like FODMAPs, histamine foods, nightshades, fiber (while one thinks of insoluble fiber as being the problem for some subgroups of people with GI problems, it’s actually not unheard of to have problems with both insoluble and soluble fiber), and so on, and additives like excitotoxins (there is actually a paper on this in IBS and fibro). Some have multiple issues from such a list. This should really be a research priority (liver issues? microbiome? GI permeability?).

          Back to the first section, they suggest “picky eating” and “food fad” as a cause for getting underweight. While allergies and intolerances can’t help, I am also concerned with metabolism switching away from glucose-based to protein-based strategies. Possibly an amino acid (protein powder) supplement could help (although powdered foods are excitotoxins regardless of ingredients, some patients can find one they tolerate even with a moderate excitotoxin sensitivity)? Happily this angle is being looked at in research.

          • Janelle says:

            Well they have MCAS in the next section, but apparently unaware it can cause GI problems?

  11. Laurie P says:

    I’m allergic to dairy and some other foods (allergy testing). I’m better when I don’t eat gluten (self-observation and observation that other people then don’t have to continually plunge my toilet). I have reactive hypoglycemia (glucose tolerance test) and I can’t eat anything sweet or fruit and I can only have limited carbs with some nut butters on them but not carbs by themselves. Histamine and tyramine foods are huge migraine triggers for me and it took me years and years and years to figure that out. It never occurred to me that tea and tomatoes and fish and avocados, etc. would all have something in common. That I learned online and in a book called “Heal Your Headache”, where I also learned that behind our backs at conferences, migraineurs are referred to as “pain weasels” by some doctors who don’t fancy having us as patients.

    What I just wrote seems to me mentioned in various ways medically in what they wrote in the primer, but then when it comes to putting it together, they want you to avoid fad diets and eat well balanced meals. Many people are going to need help with their complex diets and help from nutritionists that understand this disease.

    Also, is there really any medical evidence for sleep hygiene? Fighting with myself to try to maintain a normal sleep schedule was one of the worst things I ever did. I’m bedridden now but before that, getting up to go to bed, brush my teeth, and change into possibly cold clothes and having to warm up a possibly cold bed, always woke me up. Whereas, being ready to sleep and in bed, and then doing something quietly until I got drowsy, was always better. People all over the word fall asleep in bed reading or watching TV or listening to a book or soothing music….

    Toni Bernhard J.D., wrote a great article, “Sleep Hygiene for the Rest of Us
    Typical sleep advice does not always help those with chronic pain and illness” Her advice about fake sleeping really helped me. I know there is another article about the dishes and dog on her bed too that’s really good!

    • Laurie P says:

      And if people want to use their electronics before bed, it may work for some people to just turn the brightness down and there are various kinds of tinted glasses available too. (I like these Amazon also sells them. I knew immediately after putting them on that they were better for me than my dark sunglasses or other pink tinted sunglasses. They not only help me with photophobia but they calm my brain down too and they’re not very dark. I also always put them on upon awakening as my eyes are adjusting to being open.) The point is that people may be able to find solutions to using their electronics and their electronics may help them to relax. I’m falling asleep now typing, so I’ll end my comments. I’m not sure where all my spoons came from today!

      • Laurie P says:

        Apparently, I had enough spoons to make sentences yesterday but not enough for accurate memory. I thought I had looked up sleep hygiene in the Pediatric Primer earlier this month. What I realize now, is that what I had read was the CDC website and IACFS Primer. I had wanted to comment on what they said about sleep and also about what sleep hygiene is in general on a PR thread, but I wasn’t able to, and between then and now I confused the sources of my reading!

  12. Steve Hawkins says:

    These are good observations, Denise, and I hope that they might inform an eventual revision of the primer.

    I do think that it should be remembered that it is just meant to be a primer, and that some of the specifics on subtypes and diets, nutrition, and the like, that are being mentioned ought really to be left to ‘further reading’ suggestions if we do not want to lose the attention of the average GP with little time for reading.

    I did read the whole thing when it came out, and did not notice any glaring problems that would detract from its utility for GPs as it is now. I do very rarely attempt to burden my GP with unsolicited information, but I did actually pass on this primer, as it seemed to be about as good as I have read, and it would be better than leaving children to the mercy of the ‘NICE’ guidance, and psychological nonsense. That said, I only suggested that the GP might find it useful: not that I was prescribing it as any sort of bible on treatment of M.E. I’m sure there will be no problem with recommending a revised edition, when one is ready.

    Here’s my email text of introduction to give an idea of what you can say without seeming too pushy:

    “Dear Dr. Xxxxxx

    As you might imagine, I try to keep up with developments in fields which might throw light on my own, still unsatisfactorily diagnosed, illness‎. Much is of academic interest only, and most research work is carried out on the least severely disabled patients–who can easily attend research establishments–so progress is depressingly slow.

    However: occasionally, a report or paper does come up that is worthy of wider dissemination, and, now and then, one appears which I think ought to be brought to the attention of GPs and other health workers in general.

    The above review is one such report, which I think that you and your colleagues could usefully be made aware of.

    It is the work of an international group of specialists and writers with expertise in the diagnosis and management (and the pitfalls around such) of childhood and adolescent ME/CFS‎.

    As you might imagine, this is a difficult group of people to research, and the quality of many of the studies that have been done is questionable at best. However, Dr Speight and his fellow writers seem, to my eyes, to have made a good job of preparing a fairly comprehensive review of the current ‘state of play’‎, that you and your colleagues might find does indeed give you a useful ‘primer’, to bear in mind when meeting with younger patients who might turn out to be suffering from the illness, currently, best described as ME/CFS (The ‘CFS’ being retained purely from US tradition, as it is acknowledged to be an unfortunate term that invites conflation with other fatiguing illnesses lacking the all important symptom of post exertional malaise.).

    Hope you may find this useful.”

    I think this does not lock anyone into thinking that this is advice set in stone, and it could serve to open minds to the wealth of experience that can be referred to when they appreciate that official guidance they may have is of poor quality and little practical help. It leaves me open to pass on revised editions as and when they become available.

  13. Peter C. Rowe, MD says:

    The Perfect is the Enemy of the Good

    I was disappointed by the review of the Primer by my friend Denise Lopez-Majano. She makes some legitimate points about the wording our group used in describing the seriously ill patients, but I find it difficult to understand how any point she mentions rises to the level of a fatal flaw that should disqualify the Primer for more widespread distribution.

    Her first criticism is that the authors of the Primer are blaming the patients by saying that “Severely ill young patients are often difficult to manage…” Point taken. It is the disease that is difficult, not the patient. Which is exactly what any reader should conclude from the rest of our text. In reading the entire section, no one could seriously claim that we’re blaming the severely ill patients for being sick. Here’s what we wrote:

    Some young patients with ME/CFS are severely ill and are
    wheelchair dependent, housebound, or bedridden, sometimes
    for months or years. Many are too debilitated to be brought to
    a doctor’s office. Severe post-exertional symptoms can result if
    a visit to a hospital emergency room should become necessary.
    Published data on the characteristics of this group are lacking.
    Our clinicians estimate that about 5–10% of patients might be
    severely affected and 2–5% might be very severely affected and
    bedridden at some time during the course of the illness, but no
    studies have confirmed these numbers. The course of the severe
    form of the illness is unpredictable. Many severely affected young
    patients do show varying degrees of improvement with time and
    some manage to return to full activities. A few remain severely

    Severely ill young patients are often difficult to manage and
    frequently have received little help from medical practitioners.
    They can be socially isolated and frequently have to confront disbelief
    in the reality of their illness from family members, school
    personnel, social workers and physicians. They are in need of a
    great deal of practical help, emotional support and comfort. In
    addition to medical supervision they might require support from
    home health services and aides perhaps overseen by a nurse manager.
    The patient’s caregivers might also require support. They are
    under great stress and can sometimes benefit from counseling.

    A vital part of management is to gain the trust of the young
    patient and caregivers by reassuring them that the illness is
    recognized as a physical illness, it is taken very seriously, that
    the autonomy of the young patient will be respected, and that
    all management/treatment options that are offered will be fully
    discussed with the patient and caregivers and informed consent
    will be requested prior to implementation of any therapy. The
    patient can be reassured that improvement is common, even if it
    takes months or years, and that recovery is possible, but cannot
    be guaranteed.

    Why isolate one sentence and make this the foundational criticism of the Primer, when in fact the three paragraphs that surround that sentence offer suggestions for compassionate care, taking the severely ill patients very seriously, and not offering false promises about improvement?

    Another criticism has to do with the sentence: A patient’s needs early in the illness might differ from her/his needs in later years, as health improvement is being achieved. (p.17) Denise writes: “This implies that all patients improve, and ignores those who do not.”

    Nowhere, absolutely nowhere, do we suggest that all patients improve (see the last sentence regarding the severely ill patients, above). To claim that we ignore those who do not improve is just not a proper reflection of the Primer’s content. We have included sections on the management of severely and very severely ill patients, so we are hardly ignoring that group.

    Denise challenges the diagnostic criteria we propose as confusing. I am not sure what makes other criteria sacrosanct, as they are certainly imperfect as well. As a group, the authors of the Primer clearly felt that the existing definitions required some refinement, especially for busy clinicians. But, let’s look at the language of the critique:

    “Furthermore, the core symptoms listed on page 6 differ from those in the fact sheet about ME (p.35). These inconsistencies will make it virtually impossible for non-expert pediatricians to accurately diagnose ME.”

    Virtually impossible? As John McEnroe might say: “You can’t be serious!” There is room for debate about diagnostic criteria, but we all think our definition and the scoring sheet should make it much easier to make the diagnosis.

    Denise argues that the many ways we describe PEM are confusing. I find the use of the synonyms helpful rather than a problem, as I think PEM is one of the most poorly understood concepts to others not immersed in the language of the ME/CFS world. I would submit that fleshing out what PEM means, as the Primer does, will be helpful to physicians. Again, no reader of the entire primer could seriously claim that we have detracted attention from the importance of PEM for the diagnosis of ME/CFS. Page 1 of the Primer, in the Abstract, says this: “Cardinal features are malaise and a worsening of symptoms following minimal physical or mental exertion.” Our case definition, furthermore, lists these two as the first two criteria:

    1. Impaired function: there is loss of mental and/or physical stamina and a substantial
    reduction in ability to take part in personal, educational, and/or social activities

    2. Post-exertional symptoms: normal activity or mild/moderate exertion is followed by worsening of malaise, fatigue, and other symptoms. Recovery takes more than 24 h

    The critique is one of style (preferring a uniformity in the terms used) rather than one of substance (the acknowledged importance of PEM in the diagnostic process). How does a minor stylistic preference justify opposition to the distribution of the Primer?

    Denise suggests that any mention of the Fukuda criteria for CFS amounts to an endorsement of those criteria. This is a more baffling criticism. After all, we propose a completely different case definition, which is a strange way of “endorsing” Fukuda. We can’t hide from definitions used in the past, and to fail to describe the Fukuda definition somewhere in the primer would have been silly, given that it has been used for the vast majority of pediatric ME/CFS research in the past. Again, this seems to me to be a hollow criticism, and hardly a reason to oppose the wider distribution of the Primer.

    The critiques of the education section and the cognitive issues also are made in isolation, and out of context. We mention at several junctures the ability for cognitive stress to provoke increased symptoms, and we include this in the case definition. The comments about lower IQ scores in the Primer are followed by a reference to the article that reports tose findings, mainly to alert readers to that reference. Again, by itself, this is hardly a cause for the sensational rejection of the Primer.

    Nothing in the published critique constitutes a fatal flaw, so why did the blog include such incendiary language and the “not for Prime time” headline? It certainly has generated some fiery language in the comments section, likening us to Nazi appeasers who favor CBT and endorse the findings of the PACE trial. I would encourage those commentators to read what the Primer actually contains about CBT:

    The benefit of CBT as a psychotherapeutic intervention for ME/
    CFS is currently ambiguous. CBT has been shown to be helpful
    for some pediatric ME/CFS patients (112–115). However,
    in adults, the effect size of CBT is rather modest and the effects
    are not always sustained (116). CBT can produce adverse effects
    (101). The assertion that CBT can “reverse” or cure the illness
    is not supported by follow-up studies (117) and immunological
    changes found in the illness have been shown not to be reversed
    by CBT (118).

    The hypothesis underlying the rationale for the use of CBT
    in ME/CFS patients proposes that the patient’s poor health is
    perpetuated by avoidance of activity and by maladaptive fears,
    such as anxiety about symptoms that emerge after activity (119).
    CBT aims to improve coping with the illness by changing these
    “maladaptive cognitive responses” and encouraging graded
    exercise as well as incremental increases in other activities. The
    notion that the illness is perpetuated solely by dysfunctional
    attitudes and beliefs is speculative, lacks empirical support and
    is not consistent with our current understanding of the pathophysiology
    of ME/CFS (see Etiology and Pathophysiology). This
    hypothesis has led to patients being blamed for failure to recover,
    because, in the view of the therapist, they have not changed their
    so called “maladaptive cognitive responses.” Furthermore, CBT
    that includes rigidly enforced graded exercise frequently leads to
    severe relapse in patients with ME/CFS.

    Nevertheless, some of the elements of CBT are part of a common
    sense management of many medical problems, provided
    they are introduced in a pragmatic and flexible manner to help
    individuals cope with chronic illness (113–115, 120, 121). These
    elements include recommendations already given in this section.
    It is most important for a patient with ME/CFS that any attempt to
    increase her/his activity level or to exercise be flexible, rather than
    rigid, permitting the patient to avoid exceeding her/his energy
    limit. The patient should also be advised that CBT does not cure
    the illness, but it can help in learning to live within its limitations.
    CBT is not always available and it can be costly. It is likely to be
    more successful if the therapist is knowledgeable about ME/CFS.

    As is the case with any published work, debate is fair and welcomed, but misrepresentation of the text is not. Jennie and Denise are respected patient advocates. I would ask that they consider the overall impact of these negative comments. In the 25 years I have been dealing with ME/CFS, there has been no work like the Primer in circulation for helping with the management of pediatric patients, and there has been a clear need for one. The Primer was written by individuals who have spent most of the last 20-30 years of their lives working on ME/CFS. To receive such grudging mention of the contributions of the Primer while exaggerating perceived flaws seems quite harmful to the overall effort to provide expert care to children and adolescents with ME/CFS. I would urge people to read the Primer and judge for themselves.

    Peter C. Rowe, MD
    Professor of Pediatrics
    Sunshine Natural Wellbeing Foundation Professor of Chronic Fatigue and Related Disorders
    Director, Chronic Fatigue Clinic, Johns Hopkins Children’s Center
    Baltimore, MD

    • Denise says:

      I need to gather my thoughts but I feel it is important to say that even though Jennie provided a location for the post, responsibility for it is mine.

    • Denise says:

      I want to help disseminate the primer. I want to endorse it. I very much appreciate everyone’s work on it. I respect the extensive knowledge base and decades of experience.
      And I want the best for patients.
      That doesn’t mean I want perfection. That is unreasonable to expect.
      What it does mean is I believe we must use consistent clear messaging that in no way (even unintentionally) puts the patient at risk of being labeled for instance as challenging,
      not getting well because they aren’t trying to,
      having diminished IQ (and the pediatrician doesn’t “fight” hard enough for the patient to be in appropriate classes) when in fact what is really needed are appropriate accommodations to be able to continue their education.

      Very very few pediatricians understand this disease. Most of the primer’s intended audience for the primer have misconceptions and biases about it and about the patients. We don’t know if pediatricians will read the primer cover to cover or if they will skim it or …
      (Some of my concern stems from personal experience. When the IOM report came out, I gave the Clinician’s Guide to several people and asked for their feedback. I had a connection to these people so it was more likely that they would read it and give me feedback than my giving it to an unknown person. Most said it was too long and they couldn’t spare the time to read it. And it was about half the length of the pediatric primer.)

      Disseminating the primer as is runs the risk of pediatricians taking things out of context by lighting on things that align with their current confirmation bias and not adopting the good material in the primer. And if pediatricians don’t change their current views on this disease, patients don’t make gains.

      Also I don’t know if the readers will understand all of the context of the recommendations. If there are terms they don’t understand, phrases or references (Fukuda for example) that because of the pediatrician’s bias outweigh the other material, we may miss the opportunity to change their beliefs and their management of young patients.

      Once the material is out there, we have no control over the messaging. We won’t be with each reader to explain terms, correct inconsistencies. So I believe the material that is put out about this disease, should be as clear and consistent as possible.

  14. Steve Hawkins says:

    People do tend to get protective of the wording of documents they have spent a long time working on, with numerous edits and reformattings before they are ready to call it done. I’ve even spent weeks at planning inquiries where every word and comma in a draft plan was fought over–even typos!

    I think we have to remember this before getting irritated with one another. Come back to it as many times as we will, there will always be some little change we might feel like making–and, as likely as not, feel like changing it back next time we read.

    That’s practically all academic as the main problem is that ‘you can take a horse to water, but you can’t make him drink’: in reality few doctors are going to read this at first sight. However: the important thing is to let them know it exists, and make it look professional. Then, if and when they are faced with a sick child and are stumped for ideas, they will remember there is a primer somewhere, and remember where they put it (Probably pays to make hard copies a nice bright colour.).

  15. Laurie P says:

    p.6 “In patients who are improving, fatigue is often the last symptom to resolve, since young patients often prefer to increase their activities and tolerate the resulting fatigue.”

    – ??? I don’t even understand a statement like this.

    Nor do I understand all of the places that talk about improvement and that talk about the disease in terms of years but not decades, or a lifetime, or fatalities. I find this even more disturbing after reading “Birthing the Young Persons ME/CFS Primer” by Kenneth J. Friedman, Ph.D. . It’s always as though there are two different worlds that are going on; the one that some of us know about and the one that is allowed to be told.

    Moreover, the primer perpetuates the idea that I believed when I was diagnosed, that people get better over time. I thought that I was seeing the worst of this disease when I was in my late teens and 20s, but in actuality, I had no idea what worse could even be. I made plans and invested in a future that never came. Each time I adjusted to setbacks and tried to build a new life, I’d lose that too. Now, I’m 51, bedridden for almost a decade, and if I’d understood this disease much earlier, I would have done most things differently. I also would have had a lot more fun so I could at least have the comfort of those memories now. When someone gets an ME/CFS diagnosis, they need to FULLY understand this disease and the family needs to put their house in order based on that knowledge! The primer needs to convey this.

    p.9 “Some young patients despair of medical care and plead to decline hospital admission, due to previously experiencing the hostile and disbelieving attitudes of some health care providers and/or the exacerbation of their illness by increased hospital related activity and the noisy environment. These emotional responses to the illness do not ordinarily rise to the level of a psychiatric disorder, but occasionally, psychiatric symptoms are more severe, and a clinically diagnosable, psychiatric disorder can co-exist with ME/CFS.”

    – “These emotional responses to the illness…”??? Some of these are perfectly rational responses and are to be expected in circumstances of abuse, neglect, harm and powerlessness! How about suggesting consequences against health care providers who harm people with ME and encouraging practitioners to call out their abusive colleagues? How about mentioning that it’s important that patients not be made to see those people again? What about patients with PTSD do to harm? What about the extra care that they need? The only thing that I saw in here about PTSD was on p.34, APPENDIX B Common conditions in the differential diagnosis of pediatric ME/CFS.

    Additionally, in the way that this primer is written in some places, it seems to have an underlying assumption that care is actually available, even though it also states otherwise in other places.

    p.11 “Suicidal thoughts are only seen in ME/CFS when MDD is also present.”

    – Is this true of all illnesses, diseases and all people for that matter or is this unique to ME/CFS?

    p13 “We caution against reliance on internet-based information because much of it is anecdotal, uncorroborated, and may be designed to sell unproven products or services.”

    – The internet, like anything, including doctors’ offices, is full of all kinds of information. Would you tell someone not to go into a bookstore, or library, or not to watch TV, or talk to other people? The internet is full of all kinds of helpful information and it is also an important place for support and advocacy and activism. This knee jerk response about “the internet” is ridiculous and fruitless since you know patients will use it anyway. People with ME and their families are desperate for help, information and support. The internet is also an important consumer tool too, to evaluate doctors! It would be better if patients, families and doctors all learned to discern the best information and keep an open dialog between them about it, which includes what they learn on the internet.

    p.17 “She/he might need to deal not only with physical and cognitive limitations, but also with misunderstanding of the illness, fear, grief, anger, guilt and isolation. Sometimes having ME/CFS can result in abnormal illness behavior, such as denial of the reality of the illness.”

    – Yeah, by doctors, psychiatrists, disability, families, friends, society, the media…. Plus, every time new research comes out, it’s with a story saying something like, now there is evidence that ME/CFS is a real illness and it’s not all in your head or a figment of your imagination, etcetera. Year after year, after year, after year, this happens. When patients haven’t been believed for decades, is it really “abnormal illness behavior” to have a hard time understanding and believing that you are ill when most of the world and medical professionals don’t? Is this really the best kind of wording for this document?

    p. 17
    We recommend:

    • Developing a daily routine, especially for patients who are housebound.
    • Attempting to achieve a normal day/night cycle, to help the patient fit in with the family’s daily schedule and make school possible.

    – These first 2 recommendations are in the primer repeatedly and I didn’t see any cautions or reasons why this may be a really bad idea or impossible for some people and how to accommodate them.

    • Incorporating rewards for achievements.

    – While the heirs of Pavlov and Skinner may appreciate this, this sort of thing never worked for me and it’s something that I personally resent. If this type of thing were to stay in here, I think it should be an idea to consider for children who like a lot of structure and appreciate rewards and not as a recommendation. Furthermore, those giving the rewards need to be evaluated to make sure they are doing so appropriately/lovingly and not coercively. It should also be balanced with ideas for what other types of children would find helpful. One child may like rewards, another a puppy for company, a third may like both and a fourth child may prefer neither. I hope you see my point. Personally, I think doctors are trying to wear way too many hats these days and should be focusing a lot more on medicine.

    • The use (when appropriate) of stress reduction techniques such as music, visualization, self-hypnosis and/or mindfulness-based cognitive therapy (111).

    Footnote 111:
    111. Rimes KA, Wingrove J. Mindfulness-based cognitive therapy for people with
    chronic fatigue syndrome still experiencing excessive fatigue after cognitive
    behaviour therapy: a pilot randomized stud. J Clin Psychol Psychotherapy
    (2013) 20(2):107–17. doi:10.1002/cpp.793

    Cognitive behaviour therapy (CBT) is an effective treatment for chronic fatigue syndrome (CFS; sometimes known as myalgic encephalomyelitis). However, only a minority of patients fully recover after CBT; thus, methods for improving treatment outcomes are required. This pilot study concerned a mindfulness-based cognitive therapy (MBCT) intervention adapted for people with CFS who were still experiencing excessive fatigue after CBT….

    – Is this really the messaging we want?!

    Kenneth J. Friedman, Ph.D. wrote on a Health Rising blog on April 24, 2016:
    “How many years of timidity will it take for us to learn that timidity receives little attention. Timidity did not solve AIDS, and it will not solve ME/CFS.” .

    Respectfully, right back at you, Dr. Friedman! We need a more streamlined, less repetitive, more consistent, more accurate and less timid Pediatric Primer. I think it desperately needs a glossary and extra care that terms are properly used (which I may not have been able to do myself in writing this but you should!). There also seems to be an over emphasis on fatigue, when in actuality a person with ME may have to stop an activity due to any of their other symptoms. It really also needs to be maintained that people are ill all of the time (baseline) and worse some of the time (PEM, crash, relapse, deterioration). I think this gets confusing in places. I also suggest using the words “health crisis”, which even the CDC now uses Please let Denise and other people with ME and advocates help make the Pediatric Primer even better. I hope to put my toes in the sand again someday too. Please fix the messaging about this disease and help us all!

    Thank you.

    I’m going to sleep now and will be sleeping during the day as that is what will work best for me today.

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