Defining Pediatric CFIDS
Do We Need a CFS Case
Definition for Children Under 10?
Originally published in The CFIDS
Chronicle, Winter 1997
Karen Jordan, PhD is
project director of the chronic fatigue syndrome (CFS) epidemiology study at
DePaul University in Chicago. She moderated a panel at the October 1996 American
Association for Chronic Fatigue Syndrome’s research conference that addressed
the question, "Do we need a CFS case definition for children under 10?" All of
the panelists agreed that a specific set of criteria needs to be developed for
pediatric CFS, but they differed in their individual approaches to the
Dr. Bell argued for
specific pediatric case definitions for research and for clinical evaluation and
diagnosis of CFS in children. He
said there are two factors that need to be identified and separated in illness —
the physiological basis of illness, and the patient’s behavioral adaptation to
that physiological expression. He illustrated his point by saying that the
outcome of juvenile diabetes mellitus is influenced by the child’s decision
whether or not to take insulin.
Not having a definition for CFS can be
disastrous for the child with the disease, he said. Lacking a definition and
description of the physiological aspects of the illness, it is easy to assume
that the child’s problem is psychological.
Confusion results when the child is given
the message that there is no physiological abnormality, and that his or her
problem is the result of "some weakness in character." If the child is unable to
sort out whether he or she is really sick or is crazy like everyone says, it can
cause a crisis in identity formation that can have lifelong implications. A
specific CFS case definition for children, including a description of the
illness and of how to make the diagnosis, would establish the physiological
expression of the illness as well as the behavioral aspects, making it easier
for young people with CFS to grow up with healthy self-identities.
James Jones, MD
Dr. Jones said he agreed with Dr. Bell that a
specific definition is necessary if the diagnosis of CFS can be made in
children. One of the problems in
trying to define CFS in children (people 12 years of age and younger) is that a
child’s illness is usually reported or described by the child’s parents or other
caregivers, rather than by the child him or herself. The physician has to rely
on second-hand information.
The role of the caregivers in the
description of childhood illness needs to be carefully studied, Dr. Jones said.
He gave an example of a mother with CFS who thought her daughter also had CFS. A
thorough medical evaluation revealed that the daughter had Crohn’s disease, not
CFS. It was a case, he said, of the mother trying to explain the daughter’s
illness on the basis of her own symptoms. He asked, "Who is going to benefit by
assigning CFS to younger children — the kids or the doctors who take care of
He cautioned attendees about "vulnerable
child syndrome," a situation wherein a child with a family illness history
learns that response to illness is a way of life. He concluded by saying that
there is much to be learned about CFS in younger children.
Leonard A. Jason, PhD
Dr. Jason, the principal investigator for the
federally funded CFS epidemiology study at DePaul University, said that a great
deal of study will be required to determine if CFS exists in children under 10
years of age. In order to create a
valid definition, scientists need clear, objective signs and clear ways of
gathering information. A good definition is necessary, as is a good way of
assessing the definition. Test–retest reliability must be established, he said.
Dr. Jason shared data from the epidemiology
studies conducted by DePaul University researchers. The first study involved
random telephone calls to 1,000 people in Chicago asking if anyone in the
household had experienced fatigue lasting for six months or longer. Following
detailed examinations of those who reported CFS symptoms, two cases of CFS were
found. This translates to 200 cases per 100,000 adults, which is comparable with
current CDC estimates of 76–233 CFS cases per 100,000.
The second study, which is currently
underway, involves random phone calls to 26,000 people in the Chicago area. So
far, three to four percent of the sample meets all of the criteria for the 1994
CDC definition based on self-report of symptoms. Medical evaluations to rule out
other explanations for illness will follow.
Dr. Jason suggested the following
modifications to the 1994 case definition for use in children:
- Remove "persistent or relapsing
fatigue… that has not been life-long." This symptom may have been life-long
- Remove "fatigue is… not the result of
ongoing exertion." On a bad day, exertion in and of itself can cause
- Change "not substantially alleviated
by rest." Some CFS patients report that if they operate within the
"envelope" of the limitations imposed by the illness, they function fairly
- Change "post-exertional malaise
lasting more than 24 hours" because it is an arbitrary figure.
- Remove the exclusion for anorexia
nervosa and bulimia if the conditions have been resolved.
- Remove the exclusion for melancholic
Karen Jordan, PhD
Dr. Jordan proposed a starting point for a pediatric
case definition. Citing problems in applying the 1994 CDC definition to
children, she suggested additional modifications to those offered by Dr.
- Remove "occupational" from "reduced
- Use "frequent headaches" instead of
"headaches of a new type, pattern or severity."
- Add symptoms that are characteristic
of CFS in children — abdominal pain; rash; fever, chills or night sweats;
eye pain or light sensitivity; dizziness or lightheadedness — but keep the
required symptom minimum at four.
- Exclude childhood diseases such as
cystic fibrosis and muscular dystrophy.
- Do not exclude fibromyalgia, multiple
chemical sensitivities or school phobia.
Dr. Jordan shared some "pros and cons" for
developing a CFS case definition for children. Pros included:
- A standardized definition offers a
method of comparability between research studies, even if it is imperfect to
begin with. It can be built upon and adapted as necessary, she said.
- Naming and describing the illness is
beneficial for the patient. Not knowing what is going on leads to self-doubt
- A definition will help physicians and
parents help the child set realistic expectations for his/her activity
levels and limitations.
- A pediatric CFS definition might
become a "wastebasket," an easy diagnosis that satisfies patients and fails
to recognize the presence of other treatable illnesses.
- There is still confusion about the
true disease process in children.
- The stigma of chronic illness may be
hard for children to bear.
Dr. Jordan finished by offering the
following observations for future directions in research:
- The word "fatigue" is not used much by
parents to describe this symptom in their children.
- A thorough medical examination is
necessary to confirm the diagnosis of CFS.
- Longitudinal studies are important to
see what is happening to these children down the road.
The following comments and observations were made by
members of the audience and panel during the question and answer
- "Fatigue" is hard to define in adults,
let alone in children. It may mean sleepiness, malaise or muscle weakness.
Perhaps fatigue is a change in normal activity for kids, with less play,
more sleep and problems in school. Parents might not notice a symptom like
"fatigue" as much as they might notice that the child is not doing what they
- Dr. Bell said that of the 230 CFS
patients he treated in Lyndonville, 60 of whom were children, after 10 years
not a single patient has been subsequently diagnosed with another illness.
- There is concern about giving an
open-ended diagnosis of CFS to a child, lest the child "learn" to have CFS
over the years.
- Dr. Bell suggested subgrouping young
children who appear to be fatigued, children with a history of excessive
ear/sinus infections who eventually progress to a state resembling CFS, and
children who have illness as preschoolers, appear to recover, but develop a
CFS-like illness years later.
- It is the clinician’s job to rule out
other illnesses, and therefore it may not be necessary to list every
possible exclusion in the definition.
- The definition for children should be
"operational": doctors and parents should describe children according to
functional state rather than labeling them with CFS, which may not be in the
child’s best interest.