Reprinted with the kind permission of Denise Lopez-Majano.
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.
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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.