Severe myalgic encephalomyelitis/chronic fatigue syndrome in children and young people: a British Paediatric Surveillance Unit study 2022 Crawley et a

Abstract
Objectives Primary objective: to determine the point prevalence and incidence rate of severe myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) in children aged 5–16 years over 13 months. Secondary objectives: to describe the demographic features, symptoms, impact on activities of daily living, school attendance and time to diagnosis.

Design Prospective surveillance study conducted by the British Paediatric Surveillance Unit. Paediatricians was asked if they had assessed a child with severe ME/CFS (screening definition for prevalence and incidence: children (5–16 years) diagnosed with ME/CFS so severe that they are unable to attend school for more than 1 hour a week during the last 6 weeks of the school term).

Participants Patients 5–16 years of age, seen by paediatricians and two large ME/CFS specialist services across the UK and Ireland.

Outcome measures Paediatrician-completed questionnaires describing demographics, symptoms, function and treatment, (applying National Institute for Health and Care Excellence (NICE)-recommended criteria to assess severity of ME/CFS). Diagnosis of severe, probable severe or possible severe ME/CFS was made only with evidence of NICE-recommended screening blood tests.

Results 285 cases were reported, of which of which 33 were severe, 4 probable severe and 55 possible severe. Estimated prevalence was 3.2 per million children (95% CI 2.2 to 4.5). Including possible/probable severe ME/CFS gave 8.9 per million children (95% CI 7.2 to 11). The incidence rate was 0.90 per million children-years (95% CI 0.43 to 1.65) (1.97 per million children-years (95% CI 1.24 to 2.99)). Median age was 13 years and 58% of cases were female. Median time to diagnosis was 0.47 years.

Conclusions Although the incidence of children presenting with severe ME/CFS is low, all were very disabled. In addition, the majority receive little or no education. Paediatricians need to consider how to provide rehabilitation and education for these disabled young people.

https://adc.bmj.com/content/early/2022/11/30/archdischild-2022-324319?rss=1

 

Amazing that these people see severe M.E in the clinic and still don't get it.

 

"Case definition

CYP with severe ME/CFS were defined using a three-stage procedure. First, paediatricians used a surveillance case definition to identify CYP (box 1). The study team then applied the exclusion criteria. Cases were then reviewed by the expert ME/CFS panel, which consisted of three paediatricians with expertise in ME/CFS and two similarly experienced psychiatrists. The group applied the analytical case definition to verify the diagnosis of ME/CFS and to categorise the cases as to whether the CYP had severe ME/CFS or whether further information was required. School attendance featured in the surveillance definition but was not the sole discriminant in the analytical case definition (box 1), which also applied functional impact on activities of daily living (ADLs). Attending school is one of several possible measures because not only is it important and readily available but also it is the usual activity for CYP of this age who do not have ME/CFS."

[Contents of box 1]

Criteria for case identification and definition
Surveillance case definition

• Child aged 5–16 years.
  
  
• Clinical diagnosis of ME/CFS.
  
  
• Fatigue that is so severe that they are unable to attend school for more than 1 hour a week during the last 6 weeks of the school term.

Study team exclusion criteria

• CYP over 16 years of age at the time of reporting.
  
  
• Cases reported outside of the surveillance period.
  
  
• CYP with alternative diagnoses that could explain the fatigue.
  
  
• CYP without routine blood tests and investigations suggestive of another cause for the fatigue.
  
  
• CYP that attended school more than 1 hour a week.

Analytical case definitions

• Severe ME/CFS: fulfils the criteria in item 1 and one symptom from item 2.
  
  
• Probable severe ME/CFS: item 1 criteria met, with the exception of a dependence on assistance with activity of daily living.
  
  
• Possible severe ME/CFS: fulfils most of item 1 and one symptom from item 2, but it is not known if the NICE-recommended screening blood is normal or information on function is missing.
  
  
• Does not have severe ME/CFS.

Item 1

• New onset (not lifelong) of severe fatigue occurring for 3 consecutive months or greater duration, which is unrelated to exertion, is not substantially relieved by rest and is not a result of other medical conditions.
  
  
• The fatigue causes a significant reduction in ability to carry out activities of daily living such that they are unable or can only carry out minimal daily tasks such as face washing and teeth cleaning.
  
  
• Unable to leave the house without a severe and prolonged after-effect. Therefore, either completely housebound or bedbound or only leaves the house occasionally (eg, for medical appointments).

Item 2 (symptoms at presentation required to fulfil diagnostic criteria for ME/CFS)

• Impaired memory or concentration.
  
  
• Postexertion malaise, where physical or mental exertions bring on ‘extreme, prolonged exhaustion and sickness’.
  
  
• Problems with sleep, including unrefreshing sleep.

 

British Paediatric Surveillance Unit

"The BPSU is a world leading centre for rare paediatric disease surveillance. It enables doctors and researchers to investigate how many children in the UK and Republic of Ireland are affected by particular rare diseases, conditions or treatments each year."

The data looks sound, and even with a substantial under report, the numbers are notably low. The small gender differential is also interesting.

 

So PEM was optional?

 

Yup. All of item 1, and one of item 2, where PEM is. However one option in item 1 kind of describes PEM, so this is very confused:

Although I'm not even sure what I'm reading here:

Wut?

 

This says a lot about the limits of empirical evidence, how it's possible for thousands of professionals to be unable to see what's right in front of them, because an ideological model tells them otherwise. They simply cannot do this, one way or another they will follow a theory, no matter how wrong it is.

It's clear that without a scientific theory, medicine doesn't work at all, unless it's very simple and obvious. It's pretty much a requirement. Which makes decades of sabotage preventing a scientific theory from being developed as close as it gets to the banality of evil.

Not surprising, that a discipline based on science requires a scientific understanding, but the standard approach of refusing to do this in favor of "pragmatic"/empirical trial-and-error is very obviously a complete and total failure. And not just in execution, the entire premise is fatally flawed. It's a design failure that simply ignores reality as absurdly as a judicial system that would assume that judges only tell the truth and can be trusted to do just that.

The real failure is the combination of evidence-based medicine and the biopsychosocial ideology. Together they basically broke medicine, put a capstone on progress everywhere but advanced biomedical research. The rest, the basics, is basically stagnant, I could barely tell any significant difference between standard medical care today and how it was decades ago. The science moves but the art and practice are stuck in time.

 

Groundhog Day.

Cognitive dissonance really does know no bounds.

 

Where did this definition of 'severe' come from? Surely,

• 'Fatigue that is so severe that they are unable to attend school for more than 1 hour a week during the last 6 weeks of the school term.' ....Is outside any recognised definition?

 

Attend school....... If only

 

i don't understand the Item 1/Item 2 thing. If they have clinical diagnoses of ME/CFS, should meet the three Item 2 criteria. It doesn't make a lot of sense.

 

and shocking there is no discussion of long-term outcome/good vs short-term box-ticking, talk about being treated like objects as a risk.

I could at least understand when there was guff about 'kids wanting to not lose touch' because we've all been there with the acceptance and balancing physical/recovery needs with other needs but I find the line being about 'rehab' (which just seems a treat as an object term yet again, nothing to do with either medicine or the person's individual set of circumstances) so disingenuous and it gives me shivers to think of what it could lead to.

The job of pediatricians is/should be the health first and foremost so what on earth are they taking behavioural orders from Crawley who 'maybe met 33 of them' for, and where is the accountability she should have for discussing the potential harm or at least harm-benefit balance in her advice?

 

Where was Chalder involved in this?

 

The prevalence rate is remarkably low: 3.2 per million children (95%CI 2.2 to 4.5).

Perhaps this was due to underdiagnosis as the study asked paediatricians if they had cases of severe ME/CFS. It is a bit frustrating that the study doesn't more clearly report the response rate. It is not that clear how many pediatric centers were contacted and how many of those did not reply...

 

Authors =

Alexander Peter Royston, Manmita Rai, Amberly Brigden, Sarah Burge, Terry Y Segal, Esther M Crawley

 

And none of those names are Chalder, which is why I asked.

 

https://adc.bmj.com/content/early/2022/11/30/archdischild-2022-324319?rss=1

"Results
A total of 285 notifications were received (figure 1), of which 56 did not return questionnaires (though some were from referral centres and so reported separately); 20 were CYP older than 16; and 10 were duplicates. Of the 175 valid questionnaires received, 55 were provided by the two large referral centres, and the remainder were through BPSU notifications."

"Strengths and limitations
The BPSU provides excellent coverage of registered paediatricians and typically receives >90% response rates, making it well suited for screening for rare conditions.10 Despite this, 56 questionnaires were not returned following notifications, despite reminder emails/phone calls, and it is unclear whether these represent missed cases or duplicates (preidentified by reporters). This was higher than expected and may have been made worse as the study was conducted during a global pandemic. We were unable to conduct the 12 month follow-up because of the pandemic, which would have provided additional information about outcome and treatment. Use of the expert panel applying the analytical case definition provided an external view on case classification. The requirement for full blood test results meant that many CYP were excluded. This may mean our prevalence estimate is too low. We introduced additional methodology to capture CYP assessed by therapists in the two largest centres as therapists do not receive BPSU bulletins; however, these CYP had been assessed by paediatricians before referral for therapy. It is possible that we have missed CYP seen by therapists in other centres but, as far as we are aware, they are all assessed by paediatricians and therefore should have been reported. Ascertainment robustness has not been quantified in this study and a low rate of duplicates suggests cases may have been underestimated."

Even with the limitations, and accepting that the methodology draws the definition of 'severe' overly narrow it's difficult to see how the numbers can be orders of magnitude off. Perhaps there's two or three times the quoted number that are unaccounted for but it leaves severe ME/CFS in young people (ages are a cluster 13-16) in the UK as a rare condition.

 

Gah ! I've reached a point where Crawley/Chalder have merged into one !

 

If patients with severe ME/CFS don't see a pediatrician but another specialist instead or if the physicians don't diagnose these patients correctly, the estimate could be off by orders of magnitude.

 

My error there, corrected for Crawley - a special brain moment there clearly