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A Pediatric Case Definition for Myalgic Encephalomyelitis and Chronic Fatigue Syndrome

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For a diagnosis of chronic fatigue syndrome (CFS), most researchers use criteria that were developed by Fukuda et al. (1994), with modifications suggested by Reeves et al. (2003). However, this case definition was established for adults rather than children. A Canadian Case Definition (ME/CFS; Myalgic Encephalomyelitis/CFS) has recently been developed, with more specific inclusion criteria (Carruthers et al., 2003). Again, the primary aim of this case definition is to diagnose adult CFS. A significant problem in the literature is the lack of both a pediatric definition of ME/CFS and a reliable instrument to assess it. These deficiencies can lead to criterion variance problems resulting in studies labeling children with a wide variety of symptoms as having ME/CFS. Subsequently, comparisons between articles become more difficult, decreasing the possibility of conducting a meta-analysis. This article presents recommendations developed by the International Association of Chronic Fatigue Syndrome Pediatric Case Definition Working group for a ME/CFS pediatric case definition. It is hoped that this pediatric case definition will lead to more appropriate identification of children and adolescents with ME/CFS.
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ORIGINAL RESEARCH
A Pediatric Case Definition
for Myalgic Encephalomyelitis
and Chronic Fatigue Syndrome
Leonard A. Jason, PhD Karen Jordan, PhD Teruhisa Miike, MD
David S. Bell, MD, FAAP Charles Lapp, MD Susan Torres-Harding, PhD
Kathy Rowe, MD Alan Gurwitt, MD Kenny De Meirleir, MD, PhD
Elke L. S. Van Hoof, Clin Psych, PhD
Leonard A. Jason (Chairperson), David S. Bell, Kathy Rowe, Elke L. S. Van Hoof,
Karen Jordan, Charles Lapp, Alan Gurwitt, Teruhisa Miike, Susan Torres-Harding,
and Kenny De Meirleir are members of IACFS (International Association for Chronic
Fatigue Syndrome).
Leonard A. Jason is affiliated with the Center for Community Research, DePaul
University, Chicago, IL, USA.
David S. Bell is Clinical Associate Professor of Pediatrics, State University of New
York at Buffalo, Buffalo, NY, USA.
Kathy Rowe is affiliated with the Department of General Paediatrics, Royal Chil-
dren’s Hospital, Parkville, Victoria, Australia.
Elke L. S. Van Hoof is affiliated with Cognitive and Biological Psychology, Faculty
of Psychological and Educational Sciences, Vrije Universiteit Brussel, Belgium.
Karen Jordan is in Private Practice, Seattle, WA, USA.
Charles Lapp is affiliated with Hunter-Hopkins Center, PA, Charlotte, NC, USA.
Alan Gurwitt is affiliated with Boston Psychoanalytic Institute and Society, MA, USA.
Teruhisa Miike is affiliated with the Department of Child Development, Faculty of
Medical and Pharmaceutical Sciences, Kumamoto University Graduate School, Kumamoto,
Japan.
Susan Torres-Harding is affiliated with the Center for Community Research,
DePaul University, Chicago, IL, USA.
Kenny De Meirleir is affiliated with the Department of Human Physiology, Faculty
of Physical Education and Physical Therapy, Vrije Universiteit Brussel, Belgium.
Address correspondence to: Leonard A. Jason, PhD, DePaul University, Center for
Community Research, 990 West Fullerton Avenue, Suite 3100, Chicago, IL 60614
(E-mail: Ljason@depaul.edu).
[Haworth co-indexing entry note]: “A Pediatric Case Definition for Myalgic Encephalomyelitis and
Chronic Fatigue Syndrome.” Jason, Leonard A. et al. Co-published simultaneously in Journal of Chronic Fa
-
tigue Syndrome (The Haworth Medical Press, an imprint of The Haworth Press, Inc.) Vol. 13, No. 2/3, 2006,
pp. 1-44; and: Pediatric Chronic Fatigue Syndrome (ed: Kenny L. De Meirleir, Neil R. McGregor, and Elke
L. S. Van Hoof) The Haworth Medical Press, an imprint of The Haworth Press, Inc., 2006, pp. 1-44. Single or
multiple copies of this article are available for a fee from The Haworth Document Delivery Service
[1-800-HAWORTH, 9:00 a.m. - 5:00 p.m. (EST). E-mail address: docdelivery@haworthpress.com].
Available online at http://jcfs.haworthpress.com
© 2006 by The Haworth Press, Inc. All rights reserved.
doi:10.1300/J092v13n02_01 1
SUMMARY. For a diagnosis of chronic fatigue syndrome (CFS), most
researchers use criteria that were developed by Fukuda et al. (1994), with
modifications suggested by Reeves et al. (2003). However, this case def
-
inition was established for adults rather than children. A Canadian Case
Definition (ME/CFS; Myalgic Encephalomyelitis/CFS) has recently
been developed, with more specific inclusion criteria (Carruthers et al.,
2003). Again, the primary aim of this case definition is to diagnose adult
CFS. A significant problem in the literature is the lack of both a pediatric
definition of ME/CFS and a reliable instrument to assess it. These defi
-
ciencies can lead to criterion variance problems resulting in studies la
-
beling children with a wide variety of symptoms as having ME/CFS.
Subsequently, comparisons between articles become more difficult, de
-
creasing the possibility of conducting a meta-analysis. This article pre-
sents recommendations developed by the International Association of
Chronic Fatigue Syndrome Pediatric Case Definition Working group for
a ME/CFS pediatric case definition. It is hoped that this pediatric case
definition will lead to more appropriate identification of children and ad-
olescents with ME/CFS.
doi:10.1300/J092v13n02_01 [Article copies avail-
able for a fee from The Haworth Document Delivery Service: 1-800-HAWORTH.
E-mail address: <docdelivery@haworthpress.com> Website: <http://www.
HaworthPress.com> © 2006 by The Haworth Press, Inc. All rights reserved.]
KEYWORDS. Pediatric CFS, definition, pediatric questionnaire
A PEDIATRIC CASE DEFINITION FOR ME/CFS
ME/CFS
1
is a persistent disabling disorder that is characterized by
severe, overwhelming fatigue along with a number of other symptoms
(Fukuda et al., 1994; Joyce et al., 1997). The origins and boundaries of
the syndrome are still unclear (Jason et al., 2003; Komaroff & Buchwald,
1998). Illnesses that are consistent with ME/CFS definitely occur in ad
-
olescents and children (Breau et al., 1999; Jordan et al., 1997; Marshal,
1999; Wright & Beverly, 1998). However, the case definition was de
-
veloped for adults (Fukuda et al., 1994) and may not be appropriate for
use with children and adolescents. Currently, no ME/CFS case defini
-
tion exists for children and adolescents. The lack of application of a
consistent pediatric definition of ME/CFS and the lack of a reliable in
-
strument to assess it (Jordan, Kolak, & Jason, 1997) might lead to stud
-
ies which inaccurately label children with a wide variety of symptoms
as having ME/CFS as well as possibly missing children who do have it.
2 PEDIATRIC CHRONIC FATIGUE SYNDROME
In The Netherlands, a case definition was proposed in order to in
-
crease coherence in child ME/CFS (De Jong et al., 1997). According to
this case definition, as with adult ME/CFS, no somatic or psychiatric
condition should be able to explain the symptom pattern presented by
the child/adolescent. In addition, there should be a distinct onset of the
symptom pattern. In contrast to adult ME/CFS, where patients need to
present a significant decrease in their physical functioning, children or
adolescents are not able to compare premorbid or morbid physical func
-
tioning due to their lack of reference and due to their flexibility. Both
are characteristics of the process of identify formation. Therefore, the
assessment of leisure, social and educational activities are indispens
-
able to being able to detect a decrease in their functionality.
There has been controversy over whether the assignment of a diagno-
sis of ME/CFS in children may lead to omissions or errors in the appro-
priate diagnostic evaluations of fatigued children (Harris & Taitz, 1989;
Jones, 1997; Lask, & Dillon, 1990). Some believe that the diagnosis of
ME/CFS in this age group should be considered only an interim diagno-
sis but not a definitive disease (Jones, 1997). Another concern about ap-
plying a ME/CFS diagnosis to a young child is the potential damage
inflicted on a child by conferring a diagnosis that is open-ended. How-
ever, this possibility must be weighed against the advantages of naming
the illness that may alleviate anxiety and uncertainty in both the child
and family as well as having a clarifying impact on the school environ-
ment. These arguments do not address the reality that many clinicians
are, indeed, faced with children with unexplained fatigue and other
symptoms for which exhaustive medical and psychosocial evaluations
have not revealed an acceptable explanation. In these children, no diag-
nosis may be made, often leading to the inappropriate assumption of
malingering or diagnosis of psychiatric disease. These incorrect diagno
-
ses may be more damaging than the diagnostic label of ME/CFS. It
seems clear that for any child with a chronic illness, an ongoing diag
-
nostic evaluation that involves looking for either primary causes of fa
-
tigue and other symptoms or complications of underlying process, is
essential (Carter & Marshall, 1995). There is now enough clinical
experience and research findings to put forward a case definition for
children and adolescents.
One of the main goals of classifying any disease or illness is to group
together patients who have an illness that may have many manifesta
-
tions, but a common underlying pathophysiological pathway (Hartz et
al., 1998). The benefit of classifying patients into diagnostic categories
is that it facilitates communication among clinicians/researchers, selec
-
Original Research 3
tion of appropriate treatment methods, and prediction of response to
treatment. Past experience has shown that even in cases where the un
-
derlying pathophysiological pathway has not been identified, research
on the etiology and treatment of the illness has been facilitated by sim
-
ply classifying these illnesses as syndromes of signs and symptoms
(e.g., systemic lupus erythematosus or tuberculosis). One of the greatest
sources of diagnostic unreliability is criterion variance, differences in
the formal inclusion and exclusion criteria used by clinicians to classify
patients into diagnostic categories (Spitzer et al., 1975). The addition of
specific criteria and standardized measures with scoring guidelines
would likely improve the reliability of diagnostic decisions by provid
-
ing clinicians with objective standards to follow when assessing the var-
ious features of this syndrome (King & Jason, 2005). Collecting very
careful clinical, family, and developmental histories is also important to
ensure a differential diagnosis.
In order to address these important classification and diagnostic is-
sues, this paper proposes a case definition for diagnosing ME/CFS in
children and adolescents. It is hoped that this case definition for children
and adolescents with ME/CFS will serve as a developmentally appro-
priate diagnostic tool for clinicians and researchers. Further, develop-
ment of a pediatric definition of ME/CFS will allow for the application
of consistent and objective criteria, and may serve to stimulate research
which will then not only further test the validity of this case definition
but also elucidate pathophysiology and improve treatment approaches.
DIAGNOSIS AND PROGNOSIS
It is critical that those conducting studies attempting to diagnose chil-
dren with ME/CFS carry out a thorough evaluation, including a com
-
prehensive medical and developmental history, physical examination,
and laboratory tests to confirm diagnosis. The history should involve
both of the parents as well as the child because children are still con
-
structing their identity. Subsequently, they do not have a reference to
which they can compare before and after situations.
Arav-Boger and Spirer (1995) describe the usual patient as being pre
-
viously athletic and ambitious, upper middle-class, and having close
relatives with ME/CFS. Similarly, while clinic and community samples
have found more female than male adults with ME/CFS (Gunn et al.,
1993; Jason et al., 1999), several studies involving children have shown
an equal representation of females to males (e.g., Jordan et al., 1998). A
4 PEDIATRIC CHRONIC FATIGUE SYNDROME
recent study by Van Hoof et al. (in press) shows the same gender repre
-
sentations compared to adults (80% females vs. 20% males) in adoles
-
cents. This finding might suggest that hormonal changes in adolescence
trigger this difference in gender prevalence. Another recent study by
Viner and Hotopf (2004) found that a higher risk of ME/CFS was asso
-
ciated with having a limiting longstanding condition in childhood, fe
-
male sex, and high social class in childhood. Higher levels of exercise in
childhood were associated with lower risk of ME/CFS.
Among adolescents, easy fatigability and disturbed learning and
memorization are several of the primary characteristics of this syn
-
drome (Miike et al., 2003). As is often so with adults, the fatigue may
be quite severe to the point that “exhaustion” would be a more apt de-
scription. Another striking feature of this illness is the individuality of
symptom patterns and unpredictability of symptom severity among
youngsters with ME/CFS. The unpredictable fluctuation of symptom
severity is one of the more stressful features for youth and family alike.
Children may have a few good days and then end up in bed. When oth-
ers see them on “good days,” they might become confused or skeptical
about the seriousness and debilitating effects of the illness. This can be-
come a serious matter as it can lead to rejection of the diagnosis by
school authorities and others and relentless pursuit of psychological ex-
planations even to the point of inappropriately diagnosing Munchausen-
by-proxy.
Children may experience different symptoms than adults with ME/
CFS (Jordan et al., 1997). Symptoms such as rashes and abdominal pain
may be frequently present in pediatric ME/CFS, but may not be as com-
mon in adults. Bell (1995b) reported that the three most common com-
plaints, besides fatigue, in children and adolescents with ME/CFS were
headaches, sleep disturbance, and cognitive difficulties. As children are
still learning effective coping skills, they frequently react upon their
complaints by increased irritability (Van Hoof & Maertens, 2002).
The prognosis for a child or adolescent diagnosed with ME/CFS has
been considered to be better than with adults with this diagnosis (Arav-
Boger & Spirer, 1995; Smith & Carter, 2003). While the condition has
not been found to be progressive nor life-threatening (Carter et al.,
1995), it is noteworthy that some children continue to experience signif
-
icant fatigue and disability. It is possible that children who do not show
any improvements over time have a more severe form of the illness or
differ in other important genetic or biological ways. Bell (1995a) notes
that this persistently disabled group tends to have fatigue and other
symptoms that are worse from onset and result in severe activity limita
-
Original Research 5
tion. Recent guidelines for the management of patients with this condi
-
tion have been published (Baumer, 2005).
DIFFERENTIATE BETWEEN DIAGNOSES
Formal psychological assessment may be useful in determining if a
child’s symptoms are attributable to factors such as a primary psychiat
-
ric disorder, school phobia, or family dysfunction (Jordan et al., 1998).
One study (Pelcovitz et al., 1995) that examined psychological factors
found that adolescent ME/CFS patients reported higher levels of inter
-
nalizing symptoms (i.e., fearful, depressed, and overcontrolled behav-
ior) than a comparison group of adolescent cancer patients. Another
study (Smith et al., 1991) found that one third of the children and ado-
lescents with ME/CFS met criteria for major depressive disorder as
diagnosed by clinical interview. Similarly, Walford et al. (1993) com-
pared three groups: children and adolescents with ME/CFS, cystic fi-
brosis and healthy controls. It was found that the ME/CFS group had
significantly higher depression scores than other groups. Furthermore,
significant social and academic impairment was present in the ME/CFS
group. Although recurrent, medically unexplained physical symptoms
are common in children and adolescents, somatization disorder that
meets DSM-IV criteria is rare in this age group. Many of these studies
have flaws within them, and they often do not clearly differentiate be-
tween pre-illness symptoms and post-illness symptoms, and therefore it
is conceivable that higher rates of psychological problems are secondary
to having ME/CFS.
In children and adolescents, school phobia or school refusal is an-
other diagnostic category to be considered in differential diagnosis.
However, school phobia can generally be distinguished from ME/CFS
after a comprehensive evaluation, as once the child with school phobia
is allowed to remain home, symptoms typically disappear, and there are
usually no complaints on weekends or holidays or during the summer.
Inquiring about hobbies/leisure activities is important in distinguishing
school phobia (or social phobia) and CFS. The latter will have abandoned
their hobbies and leisure activities. Some physicians have suggested
that ME/CFS in children and adolescents is a physical manifestation of
family dysfunction claiming that ME/CFS symptoms may be utilized
by the child for primary or secondary gain, to cope with developmental
issues or change, or to deal with family problems. However, Pelcovitz
et al. (1995) found no differences between families of adolescents with
6 PEDIATRIC CHRONIC FATIGUE SYNDROME
ME/CFS and families of adolescents with cancer and control families
on family functioning measures and marital problems indices.
It should be noted that for children ill with this syndrome, after 6
months or so, friends often stop calling or visiting the youngster. If there
are no peers, cousins or extended family or anyone close in age, the iso
-
lation can be devastating. Other changes and losses include no longer
being able to participate in normal activities with peers, loss of self-con
-
fidence and self esteem if teachers and physicians are overly skeptical.
School attendance then may not only be physically taxing but psycho
-
logically stressful as well.
PREVALENCE OF PEDIATRIC ME/CFS
Much of the epidemiological research to date has focused on adults,
with minimal focus on children and adolescents. A study that ME/
CFS-like illness, characterized by prolonged fatigue (= 1 month) ac-
companied by fever, decreased endurance with exertion, and pain
symptoms, occurs at a rate of 4.4% among adolescents seen in primary
care settings indicates that this syndrome is an important medical con-
cern among youth (Mears et al., 2004). Whether or not the syndrome oc-
curs as a readily recognizable illness in younger children is still an open
question. The main reason to be cautious in this regard is that the diag-
nosis was based on patient complaints and the self-reported conse-
quences of the illness.
Lloyd and associates (1990) included information regarding children
of all ages in their published prevalence estimates from an Australian
community population study. Prevalence estimates of 5.5 cases per
100,000 were determined for children ages 0 to 9 and 47.9 cases per
100,000 cases for children and adolescents aged 10 to 19. A major prob
-
lem with the study that limits the validity of prevalence estimates was
the low number of medical practitioners who participated and identified
cases in their practices (11 out of 50 doctors participated). This problem
may have been due to lack of information about the syndrome or doubts
about the validity of ME/CFS. Given that the population in this study
was obtained through physician referral, members of the community
that do not or cannot access medical care for their symptoms were not
included in the study.
The CDC has conducted several studies to estimate the prevalence of
pediatric ME/CFS in different geographical areas (Dobbins et al., 1997).
A surveillance study was performed in Atlanta, Reno, Grand Rapids,
Original Research 7
and Wichita (Gunn et al., 1993). Local physicians identified and re
-
ferred patients who fulfilled CDC diagnostic criteria for the syndrome.
Only 44% of eligible physicians agreed to participate in the study.
Based on the Holmes et al. (1988) definition, the authors estimated that
among adolescents aged 12-17, 8.7 cases per 100,000 showed chronic
fatigue symptomatology and 2.7 per 100,000 had ME/CFS. No inqui
-
ries regarding fatigue or ME/CFS symptoms were made regarding chil
-
dren under the age of 12.
Among a second generation of CDC studies was a community-based
investigation conducted in San Francisco (Dobbins et al., 1997). This
study employed random digit dialing to households as a means of iden
-
tifying children and adolescents with chronic fatigue and ME/CFS-like
illness. Estimates were made for children aged 2-11, indicating that
71.9 per 100,000 suffered symptoms of chronic fatigue, and 0 per
100,000 presented with ME/CFS-like symptoms. In adolescents aged
12-17, 465.7 per 100,000 were found to suffer chronic fatigue symp-
toms, and 116.4 per 100,000 were diagnosed with ME/CFS-like condi-
tions. Jones et al. (2004) performed a random digit dialing survey of the
residents of Wichita, Kansas. Adults identified fatigued adolescents in
the household and answered questions relating to the child’s health. Se-
lected adolescents were invited to attend a clinic with a parent/guardian.
After clinical evaluation they were classified as CFS or another fatigue
state as defined in the 1994 CFS definition. The survey contacted
34,018 households with 90,316 residents. Of 8,586 adolescents, 138
had fatigue for more than one month, and most (107 or 78%) had
chronic fatigue (more than 6 months) at some point during the 3 year
follow up. The baseline weighted prevalence of CFS-like illness was
338 per 100,000. However, because these studies did not include a med-
ical evaluation, the actual number of cases in that population could not
be determined, and thus, only “ME/CFS-like” illness could be diag
-
nosed.
The CDC conducted another study involving referrals from school
nurses from junior and senior high schools in Wichita, Kansas, and
Reno, Nevada. A prevalence of 24.0 per 100,000 ME/CFS was found
for the 12 to 17 year old age group (Dobbins et al., 1997). As in other
medical referral studies, the gatekeeper methodology, as well as reli
-
ance on previous diagnoses by physicians (rather than current evalua
-
tions), limited the reliability and generalizability of these findings. In a
community-based study that occurred in Wichita, Kansas, Jones et al.
(2004) estimated CFS-like pediatric prevalence rates to be 338 per
100,000, but no cases of CFS were found.
8 PEDIATRIC CHRONIC FATIGUE SYNDROME
In a community epidemiology study in Chicago (Jason et al., 1999), a
pediatric screening questionnaire was administered to the adult respon
-
dents at the completion of the adult ME/CFS screening questionnaire.
Follow-up interviews were conducted with children and adolescents
identified through the initial telephone screening process. Following the
psychiatric assessment, children and adolescents underwent a complete
physical examination with laboratory testing to diagnose the presence
of ME/CFS and rule out exclusionary medical conditions (Fukuda et al.,
1994). Physician reviews were completed on 34 screened positive cases
and 23 screened negative cases. Results of physician review revealed a
prevalence of .06%, or 60 cases per 100,000 (Jordan et al., 2006).
In addition, some of the published reports follow widely reported
“epidemics” or cluster outbreaks of the syndrome (e.g., Bell et al.,
1991). As the syndrome remains to be precisely defined, both in adults
and children, such epidemics may be considered unique events at this
time, as there is no conclusive evidence that the same illness process is
at work in both the cluster outbreaks and isolated cases.
CHANGES FROM THE ADULT DEFINITION
The adult definition (Fukuda et al., 1994) has been used to diagnose
pediatric samples. It is critical for future studies examining this disorder
in pediatric populations that a consistent definition, which has been
adapted from the adult definition to take into account special circum-
stances of children, be utilized. In addition to facilitating coherent re-
search on this population, a case definition adapted for children will
facilitate diagnosis and management by pediatricians and primary care
physicians confronted with unexplained, chronic fatigue in children.
The definition presented in Table 1 has elements of the Fukuda et al.
(1994) adult case definition, along with of recommendations of Reeves
et al. (2003). We have also incorporated the structure of a new clinical
case definition for ME/CFS that has been developed in Canada
(Carruthers et al., 2003). We believe that requiring certain symptoms
does provide more specification of critical symptoms for a case defini
-
tion. However, we have tried to limit the types of symptoms within each
of the Canadian criteria categories to allow investigators to more reli
-
ably categorize pediatric patients. We also believe that this case defini
-
tion does reduce the prominence of the symptom fatigue and more
explicitly highlights the importance of symptoms such as dizziness, de
-
creased endurance with symptoms, pain, and flu-like symptoms. Indi
-
Original Research 9
10 PEDIATRIC CHRONIC FATIGUE SYNDROME
TABLE 1. Definition of ME/CFS for Children
I. Clinically evaluated, unexplained, persistent or relapsing chronic fatigue over the past 3
months that:
A. Is not the result of ongoing exertion
B. Is not substantially alleviated by rest
C. Results in substantial reduction in previous levels of educational, social and per
-
sonal activities
D. Must persist or reoccur for at least three months
II. The concurrent occurrence of the following classic ME/CFS symptoms, which must have
persisted or recurred during the past three months of illness (symptoms may predate the
reported onset of fatigue).
A. Post-exertional malaise and/or post-exertional fatigue.
With activity (it need not be strenuous and may include walking up a flight of
stairs, using a computer, or reading a book), there must be a loss of physical or
mental stamina, rapid/sudden muscle or cognitive fatigability, post-exertional
malaise and/or fatigue and a tendency for other associated symptoms within
the patient’s cluster of symptoms to worsen. The recovery is slow, often taking
24 hours or longer.
B. Unrefreshing sleep or disturbance of sleep quantity or rhythm disturbance.
May include prolonged sleep (including frequent naps), disturbed sleep (e.g.,
inability to fall asleep or early awakening), and/or day/night reversal.
C. Pain (or discomfort) that is often widespread and migratory in nature. At least one
symptom from any of the following:
Myofascial and/or joint pain (Myofascial pain can include deep pain, muscle
twitches, or achy and sore muscles. Pain, stiffness, or tenderness may occur
in any joint but must be present in more than one joint and lacking edema or
other signs of inflammation.)
Abdominal and/or head pain (May experience eye pain/sensitivity to bright
light, stomach pain, nausea, vomiting, or chest pain. Headaches often de
-
scribed as localized behind the eyes or in the back of the head. May include
headaches localized elsewhere, including migraines.)
D. Two or more neurocognitive manifestations:
Impaired memory (self-reported or observable disturbance in ability to recall
information or events on a short-term basis)
Difficulty focusing (disturbed concentration may impair ability to remain on
task, to screen out extraneous/excessive stimuli in a classroom, or to
focus on reading, computer/work activity, or television programs)
Difficulty finding the right word
Frequently forget what wanted to say
Absent mindedness
Slowness of thought
Difficulty recalling information
Need to focus on one thing at a time
Trouble expressing thought
Difficulty comprehending information
Frequently lose train of thought
New trouble with math or other educational subjects
Original Research 11
E. At least one symptom from two of the following three categories:
1. Autonomic manifestations: Neurally mediated hypotension, postural ortho
-
static tachycardia, delayed postural hypotension, palpitations with or without
cardiac arrhythmias, dizziness, feeling unsteady on the feet–disturbed balance,
shortness of breath.
2. Neuroendocrine manifestations: Recurrent feelings of feverishness and
cold extremities, subnormal body temperature and marked diurnal fluctua
-
tions, sweating episodes, intolerance of extremes of heat and cold, marked
weight change-loss of appetite or abnormal appetite, worsening of symptoms
with stress.
3. Immune manifestations: Recurrent flu-like symptoms, non-exudative sore
or scratchy throat, repeated fevers and sweats, lymph nodes tender to palpita
-
tion–generally minimal swelling noted, new sensitivities to food, odors, or
chemicals.
III. Exclusionary conditions:
A. Any active medical condition that may explain the presence of chronic fatigue, such as:
1. Untreated hypothyroidism
2. Sleep apnea
3. Narcolepsy
4. Malignancies
5. Leukemia
6. Unresolved hepatitis
7. Multiple Sclerosis
8. Juvenile rheumatoid arthritis
9. Lupus erythematosus
10. HIV/AIDS
11. Severe obesity (BMI greater than 40)
12. Celiac disease
13. Lyme disease
B. Some active psychiatric conditions that may explain the presence of chronic
fatigue, such as:
1. Childhood schizophrenia or psychotic disorders
2. Bipolar disorder
3. Active alcohol or substance abuse–except as below:
a) Alcohol or substance abuse that has been successfully treated and
resolved should not be considered exclusionary.
4. Active anorexia nervosa or bulimia nervosa–except as below:
a) Eating disorders that have been treated and resolved should not be
considered exclusionary.
5. Depressive disorders
IV. May have presence of concomitant disorders that do not adequately explain fatigue, and
are, therefore, not necessarily exclusionary.
1. Psychiatric diagnoses such as:
a) School phobia
b) Separation anxiety
c) Anxiety disorders
d) Somatoform disorders
e) Depressive disorders
rectly, fatigue will accompany such infectious or neurological illnesses,
but may not be the main focus.
Several changes were made to adult case definition based on the con-
ception that the diagnosis of ME/CFS in children should be made based
upon the symptom complex present at the time of evaluation.
First, the adult definition requires that the fatigue not be lifelong and
that it be of a new and definite onset. In a revision of the Fukuda et al.
(1994) criteria, Reeves et al. (2003) state that only participants who re-
count having always felt severely fatigued should be excluded as having
“lifelong” fatigue. We also decided to not use this criterion in formulat-
ing the diagnostic criteria for children for two reasons. First, children
and their families may not be able to pinpoint a definite onset because,
in up to 25% of pediatric cases, the onset is insidious rather than sudden
(Bell, 1992). Second, children may not be able to compare their current
functioning with a healthy baseline as, due to developmental events and
progress, they may not have a comparable period with which to com
-
pare their current functioning. Further, children themselves may be un
-
able to judge onset because variations in cognitive development might
affect their ability to remember their functioning at previous points in
time. Finally, children may be more adaptable than adults and, conse
-
quently able to make accommodations for their fatigue and other symp
-
toms (Bell, 1995b). On the other hand, inquiring about hobbies, social
and leisure activities can provide an indication of the time wherein com
-
plaints started to significantly influence daily activities. Clinical prac
-
tice shows that ME/CFS patients abandon their hobbies, social and
leisure activities in order to keep up at school. This process starts when
the first school demonstrates a decline in performance. Subsequently,
parents will encourage their children to perform better and conse
-
12 PEDIATRIC CHRONIC FATIGUE SYNDROME
TABLE 1 (continued)
2. Other conditions defined primarily by symptoms that cannot be confirmed by
diagnostic laboratory tests, such as:
a) Multiple food and/or chemical sensitivity
b) Fibromyalgia
3. Any condition under specific treatment sufficient to alleviate all symptoms
related to that condition and for which the adequacy of treatment has been
documented.
4. Any condition, that was treated with definitive therapy before development of
chronic symptomatic sequelae.
5. Any isolated and unexplained physical examination, laboratory or imaging test
abnormality that is insufficient to strongly suggest the existence of an
exclusionary condition.
quently to spend more time in order to achieve higher levels of function
-
ing at school. Therefore, although no exact onset can be identified by
the child or parents, through the assessment process, it is often possible
to pinpoint the time point when the fatigue started to interfere with the
children’s daily functioning.
Similarly, the provision that the symptoms such as sore throat or
memory impairment not predate the fatigue has also been modified. It
has been found that, in children with an insidious onset, such symptoms
may in fact predate fatigue. Alternatively, children may have a history
of sore throats, ear infections, or upper respiratory infections as part of
the usual childhood series of illnesses. However, these illnesses may
make it difficult to tease out the onset of ME/CFS symptoms. In addi-
tion, parents and children may not be aware of the fatigue but may no-
tice other symptoms. Furthermore, children may also present as irritable
rather than fatigued, and it may be difficult to date the onset of this
symptom. The symptoms present at the time of diagnosis should, how-
ever, be associated in a complex of symptoms that occurs repetitively or
chronically.
Second, debate has also occurred regarding the six-month require-
ment for the fatigue and other symptoms (Kulig, 1991; Vereker, 1992).
The Canadian criteria suggest that children with symptoms lasting more
than three months duration can be diagnosed with the illness (Carruthers
et al., 2004). We agree with this notion, as Fowler et al. (2005) did not
find differences between 8-17 years olds with 3 versus 6 months of
chronic fatigue. Overall, the criterion regarding the duration of the
symptoms varies from two weeks to six months (Van Hoof & Maertens,
2002). Arbitrarily, we propose to diagnose a CFS-like condition after 1
to 2 months of duration. After three months, ME/CFS can be diagnosed.
In clinical practice, however, it often takes more than one year before a
ME/CFS diagnosis is given to children and adolescents (Van Hoof et
al., in press).
Third, the threshold number of four symptoms has been changed, as
we now adopt a similar system to that used with the Canadian ME/CFS
criteria of symptom clusters. Appendix A provides a copy of the Pediat
-
ric ME/CFS Questionnaire, which provides a way of gathering this
information to help diagnose pediatric ME/CFS. It is generally recom
-
mended that adolescents age 12 and older fill it out themselves, and par
-
ents can assist or fill it out for the children 11 and under, although
adherence to this guideline would vary depending on the comprehen
-
sion level of the individual child. It is possible that more than one person
(e.g., child only, parent only, both parents, other primary caregiver)
Original Research 13
could fill out the Questionnaire. Some clinicians feel that it is almost
never acceptable to only involve one parent in doing a child/adolescent
evaluation, because a limited and sometimes inaccurate view of the
child and family often results from questioning only one parent. Re
-
search by Jones et al. (2004) revealed that significant differences exists
between parental and children’s descriptions of the illness. Thus, when
-
ever possible, information from both parents should be collected. Fur
-
ther, it may be appropriate to gather information from grandparents,
extended family members, or other caregivers if they are in close con
-
tact with the young person.
For a diagnosis of pediatric ME/CFS, the following five classic ME/
CFS symptom categories must occur (see Table 1). Post-exertional mal-
aise, the first criteria symptom, must occur with loss of physical or men-
tal stamina, rapid muscle or cognitive fatigability (or easy distraction as
the behavioral component of cognitive fatigability). The second symp-
tom category is unrefreshing sleep, or disturbance of sleep quantity or
rhythm. The third symptom category requires that the young person ex-
hibit either 1. myofascial pain, 2. joint pain, 3. abdominal and/or 4. head
pain. The fourth symptom category is the occurrence of two or more
neurocognitive manifestations. Finally, the fifth symptom category
requires at least one symptom from two of the following three subcate-
gories: 1. Autonomic manifestations, or 2. Neuroendocrine manifesta-
tions, or 3. Immune manifestations. For those patients who do not have
the minimum duration of 3 or more months for the 5 classic ME/CFS
symptom categories, the diagnosis should be ME/CFS-like. In contrast,
there are a small number of patients with no pain or sleep dysfunction,
and some pediatric cases might have only 2 to 4 ME/CFS classic symp-
tom categories above. For these individuals, a diagnosis of atypical pe-
diatric ME/CFS can be given. Those individuals with pediatric ME/
CFS as well as atypical pediatric ME/CFS and pediatric ME/CFS-like
are important to study.
Some support for the inclusion of these symptoms emerged from
Rowe and Rowe’s (2002) confirmatory factor analysis, which found
muscle pain and fatigue, neurocognitive, abdominal head and chest
pain, neurophysiological, and immunological factors. Abdominal symp
-
toms have been added to the list, and this is supported by the work by
Rowe and Rowe (2002). Autonomic symptoms have also been added,
and this is supported by the Canadian case definition, (Carruthers et al.,
2003), which pointed to autonomic manifestations (neurally mediated
hypotension, light headedness). In addition, a study by Jason, Torres-
Harding et al. (2002) found a symptom currently not part of the Fukuda
14 PEDIATRIC CHRONIC FATIGUE SYNDROME
criteria, shortness of breath, did differentiate the groups in adults with
ME/CFS. Given that autonomic manifestations might play a role in pe
-
diatric neurally mediated hypotension, which has been connected to
ME/CFS (Poole et al., 2000), this symptom was also included. Overall,
De Becker et al. validated the importance of the general, cognitive and
musculoskeletal symptoms. These symptom factor scores were associ
-
ated with differentiation of both the Holmes and Fukuda defined CFS
patients from non-CFS patients (De Becker et al., 2001).
It is important that each of the symptoms should be either moderate
or severe, but this “severity index” has not been well defined in previous
criteria. We now specify that symptoms that are present be rated on the
following scale: 1 = not present, 3 = moderate, 7 = severe. Symptoms
need to be rated at moderate or severe (e.g., 5 or higher) to meet criteria.
The rating scale, rather than a simple yes/no dichotomy, will facilitate
accurate diagnosis in the research setting for several reasons. First, it
will eliminate false positives on a dichotomous symptom checklist, as
most of these symptoms are common to a variety of childhood illnesses,
although not to a moderate or severe degree. Second, it will assist the
rater, who when faced with a dichotomous choice, may choose “no” if
the symptoms are present sometimes or are not severe. Finally, the rat-
ing scale will increase the amount of information gathered about each
symptom. This will also allow better comparison over time, as symptom
severity may wax and wane. In addition, providing information con-
cerning the date of onset of the symptoms might allow investigators to
better understand the progression of this illness. This type scoring de-
sign allows a dimensional diagnostic evaluation based upon the pres-
ence of the symptoms and a traditional ICD-10 categorical diagnosis.
This scoring design is implemented because in diagnosing, it is important
to take into account both the presence of the symptoms as well as the
distress they create.
Fourth, the Canadian ME/CFS clinical case definition (Carruthers et
al., 2003) states that the concurrent occurrence of the symptoms must
have persisted or recurred during six or more consecutive months of ill
-
ness. We think it is better to indicate the past 3 months, as we are at
-
tempting to diagnose current ME/CFS in pediatric cases. In addition, it
is clear that the symptoms need not be continuous for the three-month
period and may predate the onset of the recognized fatigue. However, it
is unclear what is meant by “persisted or recurred” during the past three
months. This is a rather complex concept and can lead to unreliability
unless the criterion is better specified. We now operationalize this
phrase by assessing how often the patient has experienced the symptom
Original Research 15
over the past 3 months using the following 7 point scale, from 1 = hardly
ever to 7 = every day). To be counted as “persisted or recurred,” the
individual would have to indicate a score of at least 4.
In addition to including criteria to determine the meaning of substan
-
tial reductions in activity, associated criteria are needed to assess issues
of illness severity and remission states. Borrowing from Diagnostic and
Statistical Manual of Mental Disorders, Fourth Edition (DSM-IV)
(American Psychiatric Association, 1994) terminology and the CFS
clinical case definition developed by Lapp and Cheney (1995), patients’
severity of symptoms might be classified as follows: (A) minimal (just
enough symptoms to meet the diagnosis, particularly occurring with ex
-
ertion, usually able to attend school); (B) mild (few symptoms in excess
of those in the diagnosis, occurring even at rest, may be able to attend
school part of the time); (C) moderate (many symptoms in excess of
those in the diagnosis, moderate symptoms at rest that become severe
with effort, unable to attend school); (D) severe (often housebound or
bedbound); (E) in partial remission (full criteria for the syndrome were
previously met, but currently only a few symptoms remain with effort,
able to attend school regularly); and (F) in full remission (no longer any
symptoms, even with effort, able to attend school). For individuals who
require a more differentiated way of classifying patients’ severity of ill-
ness, we suggest using the AYME Functional Ability Scale (2005).
Fifth, exclusionary medical diagnoses include genetic and other dis-
orders usually first evident in childhood that would explain the fatigue
and symptoms, as well as those exclusionary medical diagnoses enu-
merated in the adult definition. Medical diagnoses that have been ade-
quately treated (e.g., Lyme disease) or that are not likely to cause
fatigue should not be considered exclusionary. One change, however,
revolves around the depression diagnosis. In the Fukuda et al. (1994)
adult definition, melancholic and psychotic depression are considered
exclusionary conditions, primarily due to the findings that melancholic
and psychotic processes represent distinct biological or endocrino
-
logical processes and may respond well to antidepressant or anti-psy
-
chotic medications (Robbins et al.,1989; Schulkin, 1994). Depression is
less common in childhood, compared with adolescence, and symptoms
may differ between these two age groups. Depressed pre-pubertal chil
-
dren are more likely to present with psychomotor agitation, symptoms
of phobic and separation anxiety, and somatic complaints. Adolescents
with depression, on the other hand, are more likely to present with
symptoms such as anhedonia, hypersomnia, weight loss or gain, hope
-
lessness, and lethal suicide attempts. The two groups of depressed youth
16 PEDIATRIC CHRONIC FATIGUE SYNDROME
do not differ on symptoms such as depressed mood, guilt, fatigue, or
negative self-image (Compas et al., 1993). As symptoms of depression
overlap with those of ME/CFS (Hawk et al., in press), a careful evalua
-
tion must be conducted by the physician, with close attention to the dif
-
fering developmental presentations (Jason, 1997). Inquiring about hobbies
and leisure activities is important in distinguishing depression and ME/
CFS. Those with the latter diagnosis will have abandoned their hobbies
and leisure activities. In particular, if adolescents are sick, not diag
-
nosed, and not believed, the youngsters could become depressed and
anxious. Further inquiring about depressive or anxious feelings will re
-
veal an underlying frustration as a result of loosing control rather than a
negative self-image. Furthermore, if children experience frequent ab-
sences, but no “diagnosis” and no extra help or support, they will have
to struggle to catch up when they have to return to school, and this can
also lead to depression and anxiety. Thus, depressive feelings should
not be considered necessarily exclusionary, as it may co-exist with ME/
CFS, particularly if it is a reactive depression to the losses incurred by
the illness. There could be a strong feeling of disappointment in ME/
CFS children towards their support and peer group as they can not ex-
plain the child’s condition. This differentiation may also be assisted by
evaluating the course of both the depressive symptoms and fatigue
symptoms, to determine whether depression and fatigue co-vary or ap-
pear to be separate diagnostic entities. However, depression is one of
the major entities to be considered during differential diagnosis, and,
when it may better explain the fatigue and child’s symptom patterns,
may be the appropriate diagnosis rather than ME/CFS.
Reeves et al. (2003) has recommended that major depressive disorder
with melancholic features, anorexia nervosa, or bulimia, not be consid-
ered exclusionary if these conditions have been resolved for more than 5
years before the onset of the current illness. We believe that pediatric
psychotic disorders of any variety continue to be exclusionary. In addi
-
tion, eating disorders (i.e., anorexia nervosa and bulimia nervosa) and
substance abuse have been qualified to be exclusionary only if the diag
-
nosis is current; a diagnosis of melancholic depression, substance abuse
or eating disorder that has been appropriately treated and resolved
should not be considered exclusionary.
Table 1 also lists disorders that should not necessarily be considered
exclusionary, although they may present comorbidly with ME/CFS.
Such disorders include school phobia, separation anxiety disorder, and
fibromyalgia. School phobia and separation anxiety disorder are two
disorders that should be carefully considered in the differential diagno
-
Original Research 17
sis. When school phobia or separation anxiety disorder predate the fa
-
tigue and other symptoms, it is possible that a diagnosis of ME/CFS is
inappropriate and that the symptoms are better explained by a psycho
-
logical disorder. Children with school phobia may be differentiated from
children with ME/CFS in that the former typically feel ill in the morning
but recover once allowed to remain home from school (Pilkington &
Piersel, 1991). In contrast, children and adolescents with ME/CFS
would experience symptoms not only at school, but in other settings.
Furthermore, with school phobia, symptoms are typically present only
on school days, not weekends or holidays. Similarly, familial distur
-
bance and dysfunction should be closely examined during the diagnos
-
tic process. In cases where a child’s symptoms are clearly the result of
such dysfunction (e.g., the child’s illness holding an unstable marriage
together), a diagnosis of ME/CFS would be inappropriate. However, it
is just as likely that a child may be truly ill with ME/CFS and be part of
an unhealthy family system.
CLARIFICATION OF DIAGNOSTIC CRITERIA
The criterion that the fatigue not be the result of ongoing exertion
(Criterion 1A) has been criticized as too vague. For the purposes of clar-
ification and consistency, in the case of a child who is active (e.g., par-
ticipates in extracurricular activities, sports, outings with friends) but
exhausted yet recovers quickly when activity is decreased, the fatigue
would be considered to be the result of ongoing exertion or activity and,
thus, would exclude a ME/CFS diagnosis. However, a child who partic-
ipates in very little activity (possibly to minimize ME/CFS symptoms)
when compared to his or her same-age peers, and becomes exhausted
upon minimal exertion would not be excluded from a ME/CFS diagno
-
sis due to the ongoing exertion clause. Inquiring about hobbies and lei
-
sure activities will reveal abandonment of their hobbies and leisure
activities. In summary, normal fatigue is not activity limiting, whereas
the fatigue present in ME/CFS limits the individual’s activity to varying
degrees.
Similarly, the provision that the fatigue is not substantially alleviated
by rest (Criterion 1B) requires clarification. Although a child with ME/
CFS may feel better after rest, he or she may get sick again quickly upon
minimal activity or exertion. Thus, in this case, the rest does not com
-
pletely eliminate the syndrome, although it may provide some relief,
and this symptom pattern should not exclude a ME/CFS diagnosis. The
18 PEDIATRIC CHRONIC FATIGUE SYNDROME
duration of the post-exertional fatigue is important. It can last for a con
-
siderable time and be accompanied by other symptoms such as cogni
-
tive downturn, muscle fatigability, as well as resurgence of other CFS
symptoms. Another symptom that frequently occurs is social with
-
drawal to minimize ME/CFS symptoms. Previously extraverted, easy-
going and happy children become introvert and quiet. Their parents talk
about the perceived desire of their children to withdraw from social
interactions and become less noticeable.
The criterion that the illness results in a substantial reduction in level
of academic, social, or personal functioning (Criterion 1C) requires
some clinical judgment. It may be difficult to determine changes from
previous level of functioning in very young children who may not be
able to recall, nor may their parents be able to recall, their previous ac-
tivity levels. School personnel school reports can provide an estimate of
the premorbid intellectual capacities of the ME/CFS child. It is possible
to assess premorbid functioning by reviewing teacher reports before
and after the onset of the symptoms. Clinical practice indicates that ME/
CFS patients had good premorbid functioning and were considered as
easy-going and motivated students. With onset of the illness, this level
of functioning decreases and it is reflected in the school reports.
In these cases, it is appropriate to compare the child’s daily function-
ing with what would be expected of a same-age peer. For example, in-
ability to attend school, difficulty attending to activities of daily living
(e.g., bathing, dressing, or feeding), or lack of participation in social ac-
tivities due to illness or symptoms such as dizziness should be consid-
ered when making the diagnosis. Where possible, there should be
validation of decreased activity level and other symptoms by outside
sources, such as teachers or school nurses who are familiar with ME/
CFS. The AYME Functional Ability Scale (2005) is a promising way of
differentiating patients’ functional abilities.
PSYCHOLOGICAL INSTRUMENTS
To assess comorbid neuropsychiatric conditions, instruments such as
the Diagnostic Interview for Children and Adolescents-Revised (DICA;
Herjanic & Reich, 1982) or the Structured Clinical Interview for the
DSM-IV for Children (KID-SCID; Hein) may be used to determine di
-
agnoses. Instruments such as the Children’s Depression Inventory (CDI;
Kovacs, 1992) or the Schedule for Affective Disorders and Schizophre
-
nia-Children’s Version (K-SADS; Chambers et al., 1985) may be used
Original Research 19
to assess level of depression. Minimal work has been done in the area of
assessment of children’s fatigue. Walford, Nelson, and McCluskey
(1992) report the satisfactory adaptation of the fatigue questionnaire by
Wessely and Powell (1989), later revised by Chalder et al. (1993), for
use with children. However, no psychometric data are available for this
scale in relation to the pediatric population. As Stouten (2005) recently
pointed out, many frequently used fatigue scales do not accurately rep
-
resent the severe fatigue that is characteristics of CFS (although this
problem is avoided with the Profile of Fatigue-Related Symptoms, Ray
et al., 1992).
Sleep disturbances can be assessed by using the Pittsburgh Sleep
Quality Index (Buysse, 1989), which has been effectively used in ele-
mentary school age samples (Tan, 2004). This Index measures sleep
disruptions and sleep quality. The McGill Pain Questionnaire is well
validated, is available in a short form (Melzack, 1975), and has been re-
liably used with pediatric samples (O’Rourke, 2004). Finally, chil-
dren’s functional status may be assessed using the Children’s Health
Questionnaire (Landgraf, Abetz, & Ware, 1996), an instrument that will
assess physical and psychosocial well-being. The 12 concepts mea-
sured by both forms of the CHQ include physical functioning, bodily
pain, general health perceptions, and self-esteem. As mentioned in the
manual, the Changed GHQ-score is most appropriate in chronic condi-
tions as children will incorporate their symptoms in their own ‘concep-
tual frame.’ The scale ‘no more than usual’ is included in the changed
GHQ scoring procedure.
INTERVENTIONS TO INCREASE QUALITY OF LIFE
In the case of children, day-to-day management of a chronic disease
and its psychological consequences becomes a family affair with par
-
ents in particular having a key role to play. Thus, it is not surprising that
the role of interventions in facilitating adaptation to the challenges of
chronic disease has received growing recognition. Information pre
-
sented to the child, parents and other primary caregivers should make
them equipped to play an active role in the daily management of their
illness. A number of interventions are drawn on theoretical concepts
such as self-efficacy and empowerment. At the individual level, key el
-
ements of empowerment include access to information, ability to make
choices, effective change in one’s life, assertiveness and self-esteem
(Rogers et al., 1997). Similarly, self-efficacy has been posited as a cen
-
20 PEDIATRIC CHRONIC FATIGUE SYNDROME
tral, mediating mechanism in human agency (Bandura, 1988), whereby
perceptions of capabilities to carry out the courses of action necessary to
meet situational demands influence choice of actions pursued, level of
motivation, thought pattern and emotional reactions experienced. There
is evidence of effectiveness for interventions incorporating cognitive-
behavioral techniques on variables such as self-efficacy, self-manage
-
ment of disease, family functioning, psychosocial well-being, reduced
isolation and social competence. Overall, psycho-educational interven
-
tions can take many forms including simple provision of information
via written materials, computer programs or the Internet.
Psycho-educational interventions for children and their families, how
-
ever, need to take account of developmental age as well as disease pro-
gression. Glasgow and colleagues (1999) suggest that problems with
self-care typically emerge during the first few years after diagnosis or
during early adolescence (13-15 years). Thus, educational and skills
training approaches may be particularly important for both child and
family at the time of diagnosis and for adolescents who are assuming re-
sponsibility for self-care. In CFS/ME area with pediatric samples, there
have been only a few implemented non-pharmacologic interventions,
and few had appropriate controls or long-term follow-up (Whiting et al.,
2001; Barlow & Ellard, 2004).
SUGGESTIONS FOR FUTURE RESEARCH
ON PEDIATRIC ME/CFS
The definition proposed here is provided as a starting point for facili-
tating consistent research on pediatric ME/CFS. This definition should
be subjected to rigorous scientific study to determine its efficacy. In
particular, reliability studies should be conducted to determine if the
definition facilitates consistent diagnosis (Jason et al., 1997). Rowe and
Rowe (2002) used 24 key symptoms and found one underlying syn
-
drome factor, suggesting that the syndrome complex can be legitimately
designated as a syndrome. In addition, Komaroff and colleagues (1996)
compared symptoms and fatigue characteristics of a large sample of
adult ME/CFS patients with three other groups (healthy controls, de
-
pressed subjects, and patients with multiple sclerosis) to determine the
validity of each symptom and its contribution to differential diagnosis.
Similar techniques would be useful in validating the pediatric defini
-
tion.
Original Research 21
Future research on pediatric ME/CFS should carefully word ques
-
tions aimed at identifying children’s level of fatigue, and the develop
-
mental context of the children and adolescents being assessed should be
taken into account. As children may present as irritable rather than com
-
plain of fatigue, questions that focus on school problems (e.g., learning
or memory problems) or other consequences of fatigue may need to be
asked in addition to questions regarding fatigue. In addition, as there
may be discrepancies between caregiver’s reports of the child’s func
-
tioning and the child’s own assessment, a comparison of these two
descriptions of symptomatology should be made.
Fukuda et al. (1994) recommended subgrouping adult ME/CFS pa
-
tients and similar efforts would be appropriate in the study of pediatric
ME/CFS. In addition, it is clear that the current cohort of individuals di-
agnosed with ME/CFS is a diverse group with varying disease course
and disability patterns, offering limited understanding of the etiology or
pathology of the illness and its components when considered together
(Jason et al., 2005). Patterns of illness course and duration are difficult
to decipher when using the current diagnostic criteria to identify indi-
viduals with this illness. Similar to disorders such as cancer, it is highly
likely that a number of distinct types of ME/CFS exist and that the cur-
rent method of grouping all individuals who meet diagnostic criteria
together complicates the identification of biological markers in these
subgroups.
The arguments against articulating a definition of ME/CFS specifi-
cally for pediatric patients revolve primarily around the harm of an inac-
curate diagnosis. These arguments include the uncertainty in diagnosing
children due to difficulty in obtaining an accurate self-report from
young children. In addition, premature diagnosis may prevent recogni-
tion of a treatable condition. Finally, some researchers and clinicians
doubt that ME/CFS exists as a clinical entity in children, particularly
young ones. However, a consistent case definition is needed to facilitate
research on pediatric ME/CFS and to assist in the identification of ho
-
mogenous groups. While some researchers have suggested that children
and adolescents have a higher rate of recovery from ME/CFS when
compared to adults, there is still a subgroup of pediatric cases whose ill
-
ness persists for extended periods of time (Arav-Boger & Spirer, 1995;
Smith & Carter, 2003). A consistent case definition would facilitate lon
-
gitudinal investigations into the prognosis of CFS and help identify risk
factors which may predict poorer prognosis, so that these higher risk
children and adolescents receive earlier intervention.
22 PEDIATRIC CHRONIC FATIGUE SYNDROME
The summary of symptoms in Table 1 could be referenced by pedia
-
tricians, school nurses, and even school teachers and staff responsible
for Individual Education Plan development and implementation. In ad
-
dition, the criteria list could be useful to the young person, parents or
others advocating for the youth with ME/CFS in need of accommoda
-
tions since it would give legitimacy to the child’s symptoms. The rapid,
accurate identification of cases of ME/CFS followed by comprehensive
and appropriate support and treatment might increases chances of re
-
covery for all children and adolescents with this debilitating illness.
AUTHOR NOTE
The authors appreciate the support from the Board members of the International As
-
sociation for Chronic Fatigue Syndrome. In addition, they thank the following people
who have provided them constructive feedback: Pat Fero, Jill McLaughlin, Eleanor
Stein, Mary Schweitzer, Michael Yogman, Kathleen Gale, Connie Vander Eb, and John
Herd.
NOTE
1. The acronym ME/CFS refers to Myalgic Encephalomyelitis and Chronic Fatigue
Syndrome, according to the Canadian Case Definition. The patient community has felt
that the term chronic fatigue syndrome trivializes the seriousness of this illness, as the
illness is typified by many severe symptoms in addition to fatigue, and fatigue is gener-
ally regarded as a common symptom experienced by many otherwise healthy individu
-
als in the general population. The term Myalgic Encephalomyelitis had been used prior
to the use of the term chronic fatigue syndrome (Acheson, 1959). Some individuals
have preferred to use the term Myalgic Encephalopathy rather than Myalgic Enceph
-
alomyelitis, as the former term does not suggest brain inflammation.
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24 PEDIATRIC CHRONIC FATIGUE SYNDROME
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doi:10.1300/J092v13n02_01
28 PEDIATRIC CHRONIC FATIGUE SYNDROME
APPENDIX A. Pediatric Health Questionnaire
INSTRUCTIONS
The DePaul Pediatric Health Screening Questionnaire (DPHQ) is used among chil
-
dren that are under the age of 18 years old. It can be administered to 12-17 year
olds as a self-report. If the child is too young or not able to fill out the questionnaire,
there is an adult version. For children younger than 12 years old, the adult guardian
should fill out the questionnaire along with the child. The adult guardian should re
-
cord the answers given by the child, unless the child is unable to recall information.
There are two types of reports, and only one is filled out:
Child version: Filled out by the child
Or
Adult version: Filled out together by the adult guardian and child as child report.
Adult fills it out alone if the child is not able to provide any information (too young or
too incapacitated).
When comparing the results of the two types of reports stated above (child-report
versus adult report), it is important to note that when the adult and child complete
the questionnaire, guardian parents may remember and recall the child’s experi-
ences and symptoms better than the child can. At the same time, they may also re-
member inaccurate or incomplete information that disagrees with the child’s
experiences and symptoms. Hence, it is important to emphasize mutuality and
agreement of reporting between child and adult guardian when completing the
questionnaire.
If the child is at a location away from adult guardians, such as a school or a tertiary
care center, an adult such as a nurse or a teacher may simply record the child’s re-
sponses to the questionnaire. Mutuality and agreement of reporting cannot exist in
such cases, but a child report is adequate, as long as it is based on an adult’s assis-
tance in administering the questionnaire to the child.
A limitation of the adult report is the need to have an adult assist the child in filling
out the questionnaire. Children of certain backgrounds may be under-represented if
they are unwilling or unable to fill out the questionnaire with an adult.
Many children under age 18 who have a chronic illness such as ME/CFS or cancer
might need the assistance of an adult, in order to reduce respondent burden and in
-
crease accuracy of reporting.
Original Research 29
SCORING
Scoring the DPHQ:
In order to determine a diagnosis of Pediatric ME/CFS, the child must report fa
-
tigue as well as classic CFS/ME symptoms in each of the following categories for
a duration of at least 3 months in addition to a frequency and severity rating of 4 or
above*:
I. Fatigue
Question Number(s) on DPHQ: 8(1) -AND- 22 -AND- 23
II. Classic CFS/ME
Symptom Category
Question Number(s) on DPHQ
a. Post-Exertional
Malaise
8(2) -AND 12
b. Sleep
At least one symptom from 8(3) to 8(7)
c. Pain At least one symptom from:
Myofascial pain, Joint pain, Abdominal, or Head pain 8(37) to
8(47)
d. Neurocognition
At least two symptoms from 8(8) to 8(16)
e. Other Category At least one symptom from two of the following three catego-
ries:
Autonomic manifestations: 8(17) to 8(20)
Neuroendocrine manifestations: 8(21) to 8(28)
Immune manifestations: 8(29) to 8(32)
DIAGNOSIS
______Pediatric ME/CFS
______Pediatric ME/CFS-like illness (meeting all classic symptom criteria except
for the 3 month duration)
______Atypical ME/CFS (missing one or more classic symptoms, but current ill
-
ness persists for at least 3 months)
______Other diagnosis (Please specify_________________________________)
30 PEDIATRIC CHRONIC FATIGUE SYNDROME
Name__________________________________________________
Date____________________
Pediatric Health Questionnaire (Child Version)
For all of the following questions, please provide or circle only one answer unless
otherwise asked.
1. How old are you? .................................................................................. ______
2. Are you male or female?
Male....................................................................................................... 1
Female .................................................................................................. 2
3. Are you of Latino or Hispanic origin?
Yes ........................................................................................................ 1
No.......................................................................................................... 2
4. To which of the following race(s) do you belong?
Black, African-American ........................................................................ 1
White ..................................................................................................... 2
American Indian or Alaska Native..........................................................3
Asian or Pacific Islander........................................................................ 4
Some other race (
Please write-in below
) ............................................5
_________________________________________________________
5. What grade are you in or what was the last grade that you completed?______
6. Do you attend school or do you have home-schooling/homebound instruction?
Attend School
........................................................................................ 1
OR
Home-school/Homebound Instruction (
Please write-in below
)............2
When did you start home-schooling/Homebound Instruction?__________
7. How many days of school do you usually miss in one month?______________
Original Research 31
8. Please fill out this chart (go from left to right)
Question #8...
Have you been
experiencing any of
the following
symptoms/problems
for at least
one month
?
CIRCLE ONE:
Y=YES...N=NO
If YES, then
answer the
rest of the
questions in
the chart
If NO, then go
to the next
symptom on
the list.
Duration:
When was
the first time
that you
had this
symptom?
Frequency:
How often do
you have this
symptom?
Please choose
a number
from 1-7:
Severity:
How much
does this
symptom
bother you?
Please choose
a number
from 1-7:
Month
or
Season
Year
1...
Hardly ever
2
3
4...
Half of the time
5
6
7...
Always
1...(no problem)
2
3
4...
(moderate
problem)
5
6
7...
(big problem)
1) Fatigue/Extreme
tiredness
YN
2) Feeling worse after
doing activities that
require physical or
mental effort
YN
3) Feeling tired after
you wake up in the
morning
YN
4) Need to nap every
day
YN
5) Problems falling
asleep
YN
6) Problems staying
asleep
YN
7) Waking up early in
the morning (like 3
a.m.)
YN
8) Problems
remembering things
YN
9) Difficulty paying
attention for a long
period of time
YN
10) Difficulty finding
the right word to say
YN
11) Difficulty
understanding things
YN
12) Only able to focus
on one thing at a time
YN
13) Frequently losing
your train of thought
YN
32 PEDIATRIC CHRONIC FATIGUE SYNDROME
Question #8...
Have you been
experiencing any of
the following
symptoms/problems
for at least
one month
?
CIRCLE ONE:
Y=YES...N=NO
If YES, then
answer the
rest of the
questions in
the chart
If NO, then go
to the next
symptom on
the list.
Duration:
When was
the first time
that you
had this
symptom?
Frequency:
How often do
you have this
symptom?
Please choose
a number
from 1-7:
Severity:
How much
does this
symptom
bother you?
Please choose
a number
from 1-7:
Month
or
Season
Year
1...
Hardly ever
2
3
4...
Half of the time
5
6
7...
Always
1...(no problem)
2
3
4...
(moderate
problem)
5
6
7...
(big problem)
14) Slowness of
thought
YN
15) Absent-
mindedness or
forgetfulness
YN
16) Recent trouble
with math or numbers
YN
17) Feel unsteady on
your feet, like you
might fall
YN
18) Shortness of
breath or trouble
catching your breath
YN
19) Dizziness Y N
20) Irregular heart
beats
YN
21) Losing or gaining
weight
YN
22) Not wanting to eat Y N
23) Sweating hands
YN
24) Night sweats
YN
25) Feel chills or
shivers
YN
26) Feeling hot or cold
YN
27) Feeling like you
have a high
temperature
YN
28) Feeling that your
temperature is low
YN
29) Sore throat Y N
Original Research 33
Question #8...
Have you been
experiencing any of
the following
symptoms/problems
for at least
one month
?
CIRCLE ONE:
Y=YES...N=NO
If YES, then
answer the
rest of the
questions in
the chart
If NO, then go
to the next
symptom on
the list.
Duration:
When was
the first time
that you
had this
symptom?
Frequency:
How often do
you have this
symptom?
Please choose
a number
from 1-7:
Severity:
How much
does this
symptom
bother you?
Please choose
a number
from 1-7:
Month
or
Season
Year
1...
Hardly ever
2
3
4...
Half of the time
5
6
7...
Always
1...(no problem)
2
3
4...
(moderate
problem)
5
6
7...
(big problem)
30) Tender/sore
lymph nodes
YN
31) Fever and sweats Y N
32) Some smells,
foods, or chemicals
make you feel sick
YN
33) Rash(es) Y N
(34) Allergies Y N
35) Mood changes Y N
36) Anxiety Y N
37) Pain or aching in
your muscles
YN
38) Muscle twitches Y N
39) Pain/stiffness/
tenderness in more
than one joint without
swelling or redness
YN
40) Eye pain Y N
41) Vomiting/throwing
up
YN
42) Nausea/feeling like
you might throw up
YN
43) Chest pain or
heartburn
YN
44) Upset stomach Y N
45) Abdomen/
stomach pain
YN
46) Ringing in ears Y N
47) Headaches** Y N
34 PEDIATRIC CHRONIC FATIGUE SYNDROME
**IF you have headaches now, do you get them more often, in a different place, or
do the headaches feel worse than they did in the past? (You may circle more than
one answer.)
Headaches happen more often ............................................................. 1
Headaches feel worse/more severe ......................................................2
Headaches are in a different place/spot ................................................3
9. Do you have any medical illness that might be causing your symptoms?
No.......................................................................................................... 1
Yes (
What medical illnesses do you have?
)......................................2
_________________________________________________________
10. Do you seem to catch illnesses more easily than other people your age?
No.......................................................................................................... 1
Yes ........................................................................................................ 2
11. Does it seem to take longer for you to get better after you are sick than other
people of your age?
No.......................................................................................................... 1
Yes ........................................................................................................ 2
12. How does being physically active (such as using stairs, walking, playing sports,
doing chores, getting dressed) make you feel for the rest of the day?
Much more tired than usual ................................................................... 1
More tired than usual............................................................................. 2
Has no effect ......................................................................................... 3
More energetic than usual..................................................................... 4
Much more energetic than usual ........................................................... 5
13a. Do you participate in any hobbies or activities outside of school?
Yes ........................................................................................................ 1
No.......................................................................................................... 2
13b. Are you currently able to carry out any of your hobbies or activities?
Yes ........................................................................................................ 1
No.......................................................................................................... 2
IF NO, when and why did you quit your hobbies/activities:___________
_________________________________________________________
Original Research 35
14. Have you been experiencing any problems with fatigue/extreme tiredness for
at least one month?
No (
Stop here
)
.................................................................. 1
Yes (
Continue to next page
) ............................................................... 2
IF YES, For about how many months? ____________
15. What do you think the cause of your fatigue or tiredness is?_____________
_____________________________________________________________
16. Do you think that your fatigue is caused by ongoing activity?
Yes ........................................................................................................ 1
No.......................................................................................................... 2
17. Did your fatigue illness start after you experienced_______? (Circle one or more.)
An infectious illness............................................................................... 1
An accident............................................................................................ 2
A trip or vacation ................................................................................... 3
An immunization (shot at doctor’s office)...............................................4
Surgery.................................................................................................. 5
Severe stress (bad or unhappy event(s)) ..............................................6
Other (
Please write in below
) .............................................................. 7
_________________________________________________________
18. How long did it take for your problem with fatigue or tiredness to get started?
Rapidly–within 24 hours ........................................................................ 1
Over 1 week .......................................................................................... 2
Over 1 month......................................................................................... 3
Over 2-6 months.................................................................................... 4
Over 7-11 months.................................................................................. 5
Over 1-2 years....................................................................................... 6
Longer than 2 years............................................................................... 7
I have always experienced fatigue.........................................................8
19. When you first became sick what were your worst 3 symptoms?
a. __________________________
b. __________________________
c. __________________________
36 PEDIATRIC CHRONIC FATIGUE SYNDROME
20. Right now, what are your worst 3 symptoms?
a. __________________________
b. __________________________
c. __________________________
21. Do your symptoms change over time?
No.......................................................................................................... 1
Yes ........................................................................................................ 2
22. Do you limit or cut back your activity levels to avoid feeling even more tired?
No.......................................................................................................... 1
Yes ........................................................................................................ 2
23. If you rest, does all of your fatigue go away, some of it go away, or none of it go
away?
All of it goes away (
Go to Question 24a
) .............................................1
Some of it goes away (
Go to Question 24a
) ........................................2
None of it goes away (
Go to Question 25
)...........................................3
24a. How long do you have to rest before your fatigue gets better?
__________________________________________________________
24b. Will your fatigue come back if you stop resting and start doing something?
No.......................................................................................................... 1
Yes ........................................................................................................ 2
25. How would you describe the way your fatigue illness is changing over time?
My fatigue is getting worse .................................................................... 1
I have good and bad periods ................................................................. 2
There is no change................................................................................ 3
My fatigue is getting better .................................................................... 4
Thank you for filling out the Pediatric Health Questionnaire
Original Research 37
Name__________________________________________________
Date____________________
Pediatric Health Questionnaire (Adult Version)
For all of the following questions, please provide or circle only one answer unless
otherwise asked.
1. What is your child’s age (in years)? ....................................................... _______
2. Is your child male or female?
Male....................................................................................................... 1
Female .................................................................................................. 2
3. Is your child of Latino or Hispanic origin?
Yes ........................................................................................................ 1
No.......................................................................................................... 2
4. To which of the following race(s) does your child belong?
Black, African-American ........................................................................ 1
White ..................................................................................................... 2
American Indian or Alaska Native..........................................................3
Asian or Pacific Islander........................................................................ 4
Some other race (
Please write-in below
) ............................................5
_________________________________________________________
5. What grade is your child in or what was the last grade that he/she completed?
___________
6. Does your child attend school or does he/she have home-schooling/homebound
instruction?
Attend School ........................................................................................ 1
OR
Home-school/Homebound Instruction (
Please write-in below
)............2
When did he/she start home-schooling/Homebound Instruction?________
7. How many days of school does your child usually miss in one month?
____________
38 PEDIATRIC CHRONIC FATIGUE SYNDROME
8. Please fill out this chart (go from left to right)
Question #8...
Has your child been
experiencing any of
the following
symptoms/problems
for at least
one month
?
CIRCLE ONE:
Y=YES...N=NO
If YES, then
answer the
rest of the
questions in
the chart
If NO, then go
to the next
symptom on
the list.
Duration:
When was
the first time
that your
child
had this
symptom?
Frequency:
How often
does your
child have this
symptom?
Please choose
a number
from 1-7:
Severity:
How much
does this
symptom
bother your
child?
Please choose
a number
from 1-7:
Month
or
Season
Year
1...
Hardly ever
2
3
4...
Half of the time
5
6
7...
Always
1...(no problem)
2
3
4...
(moderate
problem)
5
6
7...
(big problem)
1) Fatigue/Extreme
tiredness
YN
2) Feeling worse after
doing activities that
require physical or
mental effort
YN
3) Feeling tired after
he/she wakes up in
the morning
YN
4) Need to nap daily Y N
5) Problems falling
asleep
YN
6) Problems staying
asleep
YN
7) Waking up early in
the morning (like 3
a.m.)
YN
8) Problems
remembering things
YN
9) Difficulty paying
attention for a long
period of time
YN
10) Difficulty finding
the right word to say
YN
11) Difficulty
understanding things
YN
12) Only able to focus
on one thing at a time
YN
13) Frequently losing
his/her train of thought
YN
Original Research 39
Question #8...
Has your child been
experiencing any of
the following
symptoms/problems
for at least
one month
?
CIRCLE ONE:
Y=YES...N=NO
If YES, then
answer the
rest of the
questions in
the chart
If NO, then go
to the next
symptom on
the list.
Duration:
When was
the first time
that your
child
had this
symptom?
Frequency:
How often
does your
child have this
symptom?
Please choose
a number
from 1-7:
Severity:
How much
does this
symptom
bother your
child?
Please choose
a number
from 1-7:
Month
or
Season
Year
1...
Hardly ever
2
3
4...
Half of the time
5
6
7...
Always
1...(no problem)
2
3
4...
(moderate
problem)
5
6
7...
(big problem)
14) Slowness of
thought
YN
15) Absent-
mindedness or
forgetfulness
YN
16) Recent trouble
with math or numbers
YN
17) Feel unsteady on
his/her feet, like
he/she might fall
YN
18) Shortness of
breath or trouble
catching his/her
breath
YN
19) Dizziness Y N
20) Irregular heart
beats
YN
21) Losing or gaining
weight
YN
22) Not wanting to eat Y N
23) Sweating hands Y N
24) Night sweats Y N
25) Feel chills or
shivers
YN
26) Feeling hot or cold Y N
27) Feeling like he/
she has a high
temperature
YN
28) Feeling like he/she
has a low temperature
YN
29) Sore throat Y N
40 PEDIATRIC CHRONIC FATIGUE SYNDROME
Question #8...
Has your child been
experiencing any of
the following
symptoms/problems
for at least
one month
?
CIRCLE ONE:
Y=YES...N=NO
If YES, then
answer the
rest of the
questions in
the chart
If NO, then go
to the next
symptom on
the list.
Duration:
When was
the first time
that your
child
had this
symptom?
Frequency:
How often
does your
child have this
symptom?
Please choose
a number
from 1-7:
Severity:
How much
does this
symptom
bother your
child?
Please choose
a number
from 1-7:
Month
or
Season
Year
1...
Hardly ever
2
3
4...
Half of the time
5
6
7...
Always
1...(no problem)
2
3
4...
(moderate
problem)
5
6
7...
(big problem)
30) Tender/ sore
lymph nodes
YN
31) Fever and sweats Y N
32) Some smells,
foods, or chemicals
make your child feel
sick
YN
33) Rash(es) Y N
34) Allergies Y N
35) Mood changes Y N
36) Anxiety Y N
37) Pain or aching in
his/her muscles
YN
38) Muscle twitches Y N
39) Pain/stiffness/
tenderness in more
than one joint without
swelling or redness
YN
40) Eye pain Y N
41) Vomiting Y N
42) Nausea Y N
43) Chest pain or
heartburn
YN
44) Upset stomach Y N
45) Abdomen/
stomach pain
YN
46) Ringing in ears Y N
47) Headaches** Y N
Original Research 41
**IF your child has headaches now, does he/she get them more often, in a different
place, or do the headaches feel worse than they did in the past? (You may circle
more than one answer.)
Headaches happen more often ............................................................. 1
Headaches feel worse/more severe ......................................................2
Headaches are in a different place/spot ................................................3
9. Does your child have any medical illness that might be causing his/her symp
-
toms?
No.......................................................................................................... 1
Yes (
What medical illnesses does he/she have?
).............................2
_________________________________________________________
10. Does your child seem to catch illnesses more easily than other people his/her
age?
No.......................................................................................................... 1
Yes ........................................................................................................ 2
11. Does it seem to take your child longer to get better after he/she is sick than
other people his/her age?
No.......................................................................................................... 1
Yes ........................................................................................................ 2
12. How does being physically active (such as using stairs, walking, playing sports,
doing chores, getting dressed) make your child feel for the rest of the day?
Much more tired than usual ................................................................... 1
More tired than usual............................................................................. 2
Has no effect ......................................................................................... 3
More energetic than usual..................................................................... 4
Much more energetic than usual ........................................................... 5
13a. Does your child participate in any activities or hobbies outside of school?
Yes ........................................................................................................ 1
No.......................................................................................................... 2
13b. Is he/she currently able to carry any of them out?
Yes ........................................................................................................ 1
No.......................................................................................................... 2
IF NO, when and why did your child quit his/her activities:___________
_________________________________________________________
42 PEDIATRIC CHRONIC FATIGUE SYNDROME
14. Has your child been experiencing any problems with fatigue/extreme tiredness
for at least one month
?
No (
Stop here
)
................................................................... 1
Yes (
Continue to next page
) ............................................................... 2
IF YES, For about how many months? ____________
15. What do you think is the cause of your child’s fatigue or tiredness?__________
_____________________________________________________________
16. Do you think that your child’s fatigue is caused by ongoing activity?
Yes ........................................................................................................ 1
No.......................................................................................................... 2
17. Did your child’s fatigue illness start after he/she experienced______? (Circle
one or more.)
An infectious illness............................................................................... 1
An accident............................................................................................ 2
A trip or vacation ................................................................................... 3
An immunization (shot at doctor’s office)...............................................4
Surgery.................................................................................................. 5
Severe stress (bad or unhappy event(s)) ..............................................6
Other (
Please write in below
) .............................................................. 7
_________________________________________________________
18. How long did it take for your child’s problem with fatigue or tiredness to get started?
Rapidly–within 24 hours ........................................................................ 1
Over 1 week .......................................................................................... 2
Over 1 month......................................................................................... 3
Over 2-6 months.................................................................................... 4
Over 7-11 months.................................................................................. 5
Over 1-2 years....................................................................................... 6
Longer than 2 years............................................................................... 7
He/she has always experienced fatigue ................................................8
19. When your child first became sick what were his/her worst 3 symptoms?
a. __________________________
b. __________________________
c. __________________________
Original Research 43
20. Right now, what are your child’s worst 3 symptoms?
a. __________________________
b. __________________________
c. __________________________
21. Do his/her symptoms change over time?
No.......................................................................................................... 1
Yes ........................................................................................................ 2
22. Does your child limit or cut back his/her activity levels to avoid feeling even
more tired?
No.......................................................................................................... 1
Yes ........................................................................................................ 2
23. If yours child rests, does all of his/her fatigue go away, some of it go away, or
none of it go away?
All of it goes away (
Go to Question 24a
) .............................................1
Some of it goes away (
Go to Question 24a
) ........................................2
None of it goes away (
Go to Question 25
)...........................................3
24a. How long does your child have to rest before his/her fatigue gets better?
____________________________________________________________
24b. Will your child’s fatigue return if he/she stops resting and starts doing something?
No.......................................................................................................... 1
Yes ........................................................................................................ 2
25. How would you describe the way your child’s fatigue illness is changing over
time
His/her fatigue is getting worse ............................................................. 1
He/she have good and bad periods.......................................................2
There is no change................................................................................ 3
His/her fatigue is getting better..............................................................4
Thank you for filling out the Pediatric Health Questionnaire (Adult Version).
44 PEDIATRIC CHRONIC FATIGUE SYNDROME
... Inclusion and exclusion criteria for this study have been described in detail previously [35]. Participants included adolescents aged 13-18 years diagnosed with ME/CFS by a paediatrician specialising in ME/CFS at an Australian tertiary children's hospital using the Canadian Criteria adapted for paediatrics [36,37] and healthy adolescent controls aged 13-18 with no history of ME/CFS or other chronic illnesses. Exclusion criteria at study enrolment were insufficient English to complete the questionnaires, major depression or anxiety disorder, history of psychosis or bipolar disorder, pre-existing developmental disability or brain injury, and current use of any medication that may affect brain function. ...
... A short researcher-designed health questionnaire for the ME/CFS cohort was administered at baseline and follow-up, based on the diagnostic criteria for the paediatric case definition of ME/CFS and developed by the Pediatric ME/CFS Case Definition Working Group [36,37,52]. At baseline, the health questionnaire was completed by the ME/CFS patient's paediatrician who specialised in ME/CFS, and at follow-up the questionnaire completed by the adolescent with ME/CFS (some words were rephrased to be understood by a younger audience, see Supplementary Table S1 for comparison). ...
... Two main measures were obtained from the health questionnaire: (a) fulfilment of ME/CFS diagnostic criteria (including 'severe', 'moderate', or 'atypical' ME/CFS, [36,37,53], and (b) whether patients subjectively perceived they had ME/CFS at follow-up ('Do you still have ME/CFS? Yes or No.'). ...
Article
Full-text available
Background: The purpose of this study was to follow-up an Australian cohort of adolescents newly-diagnosed with ME/CFS at a tertiary paediatric ME/CFS clinic and healthy controls over a mean period of two years (range 1-5 years) from diagnosis. Objectives were to (a) examine changes over time in health and psychological wellbeing, (b) track ME/CFS symptomatology and fulfillment of paediatric ME/CFS diagnostic criteria over time, and (c) determine baseline predictors of ME/CFS criteria fulfilment at follow-up. Methods: 34 participants aged 13-18 years (25 ME/CFS, 23 controls) completed standardised questionnaires at diagnosis (baseline) and follow-up assessing fatigue, sleep quality and hygiene, pain, anxiety, depression, and health-related quality of life. ME/CFS symptomatology and diagnostic criteria fulfilment was also recorded. Results: ME/CFS patients showed significant improvement in most health and psychological wellbeing domains over time, compared with controls who remained relatively stable. However, fatigue, pain, and health-related quality of life remained significantly poorer amongst ME/CFS patients compared with controls at follow-up. Sixty-five percent of ME/CFS patients at baseline continued to fulfil ME/CFS diagnostic criteria at follow-up, with pain the most frequently experienced symptom. Eighty-two percent of patients at follow-up self-reported that they still had ME/CFS, with 79% of these patients fulfilling criteria. No significant baseline predictors of ME/CFS criteria fulfilment at follow-up were observed, although pain experienced at baseline was significantly associated with criteria fulfilment at follow-up (R = 0.6, p = 0.02). Conclusions: The majority of Australian adolescents with ME/CFS continue to fulfil diagnostic criteria at follow-up, with fatigue, pain, and health-related quality of life representing domains particularly relevant to perpetuation of ME/CFS symptoms in the early years following diagnosis. This has direct clinical impact for treating clinicians in providing a more realistic prognosis and highlighting the need for intervention with young people with ME/CFS at the initial diagnosis and start of treatment.
... Pediatric myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) is a debilitating disease characterized by persistent fatigue lasting for at least three months, a substantial reduction in activity, and an absence of a medical exclusionary condition causing fatigue [1,2]. Similar to adult ME/CFS, primary symptoms include post-exertional malaise, unrefreshing sleep, concentration problems, and bodily pain; however, symptoms more commonly found in children include abdominal pain and rashes [1]. ...
... Pediatric myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) is a debilitating disease characterized by persistent fatigue lasting for at least three months, a substantial reduction in activity, and an absence of a medical exclusionary condition causing fatigue [1,2]. Similar to adult ME/CFS, primary symptoms include post-exertional malaise, unrefreshing sleep, concentration problems, and bodily pain; however, symptoms more commonly found in children include abdominal pain and rashes [1]. ME/CFS negatively affects a range of critical life domains including social, emotional, school, and physical functioning [3][4][5][6]. ...
... Parent-child dyads with youth ages 5-17 were included in the sample as those with ME/CFS if the youth had no exclusionary medical conditions and met at least one of the following case definition criteria with six-month fatigue duration: Jason and colleagues' pediatric criteria [1], Fukuda criteria [23] or Institute of Medicine criteria [24], as diagnosed by two independent physicians assessing all the study measures and medical reports. Additionally, asymptomatic children and adolescents from the epidemiological study were selected and included as part of the control group [12]. ...
Article
Full-text available
Purpose Few studies have examined parent-child discrepancies on self-report measures of myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) symptomatology and health-related quality of life (HRQOL). The aim of this study was to investigate parent-child reporting discrepancies between a pediatric sample of diagnosed patients with ME/CFS and controls to better understand the role of children and adolescent reporting. Method Data for this study were drawn from a community-based epidemiological study of pediatric ME/CFS in the Chicagoland area. A total of 147 parent-child dyads (75 pairs with ME/CFS and 72 control pairs) completed measures assessing HRQOL and ME/CFS symptomatology. At the individual level, agreement was assessed using intra-class correlation coefficient (ICC) scores. Agreement was measured at the group level by a comparison of means using paired-sample t-tests. Results Intra-class correlations revealed varied agreement in both parent-child pairs of children who met at least one case definition of ME/CFS and in parent-child pairs in the control group. Conclusion The current study provides support for the existence of discrepancies between parent-child reports of ME/CFS symptomatology and HRQOL measures. Limitations and future directions are discussed.
... 1,2 Several case definitions have been developed in attempts to recognize the illness in adults and some case definitions have been geared towards children and adolescents. 3,4 There are multiple possible explanations regarding the cause of ME/CFS, and some studies have focused on immune dysfunction in adolescents with ME/CFS. For example, Sulheim et al. and Kristiansen et al. reported slightly elevated levels of C-reactive protein (CRP) in adolescents with ME/CFS, suggesting low-grade systemic inflammation. ...
Article
Objectives: Studies have demonstrated immune dysfunction in adolescents with Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS); however, evidence is varied. The current study used network analysis to examine relationships between cytokines among a sample of pediatric participants with ME/CFS. Methods: 10,119 youth aged 5-17 in the Chicagoland area were screened for ME/CFS; 111 subjects and controls were brought in for a physician examination and completed a blood draw. Youth were classified as controls (Cs, N = 43), ME/CFS (N = 23) or severe (S-ME/CFS, N = 45). Patterns of plasma cytokine networks were analyzed. Results: All participant groups displayed a primary network of interconnected cytokines. In the ME/CFS group, inflammatory cytokines IL-12p70, IL-17A, and IFN-γ were connected and included in the primary membership, suggesting activation of inflammatory mechanisms. The S-ME/CFS group demonstrated a strong relationship between IL-17A and IL-23, a connection associated with chronic inflammation. The relationships of IL-6 and IL-8 in ME/CFS and S-ME/CFS participants also differed from Cs. Together, these results indicate pro-inflammatory responses in our illness populations. Discussion: Our data imply biological differences between our three participant groups, with ME/CFS and S-ME/CFS participants demonstrating an inflammatory profile. Examining co-expression of cytokines may aid in the identification of a biomarker for pediatric ME/CFS.
... At the end of Stage 3, a team of physicians were responsible for making final diagnoses. Two reviewing physicians, aside from the examining physician, had access to all information gathered on each participant during each of the three phases of the study including results from the physical exam and used this information to make a final diagnosis of ME/CFS on the individuals in our study using the Fukuda et al. 9 criteria with revisions recommended by Reeves et al., 16 the pediatric ME/CFS criteria developed by an Canadian study group 17 and/or the Institute of Medicine 2015 clinical criteria. 10 Both reviewing physicians had to agree with each diagnosis. ...
Article
Full-text available
Objective: The prevalence of pediatric Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS) has been estimated from an ethnically and sociodemographically diverse community-based random sample of 10,119 youth aged 5–17. We assessed whether a salivary biomarker of fatigue could identify youth with ME/CFS. Study design: We examined the ratio of the concentrations of 2 peptide fragments in saliva, referred to as the Fatigue Biomarker Index (FBI), in participants from our study diagnosed with ME/CFS (n = 59) and matched controls (n = 39). Results: Significant overall differences were found in the FBI between those participants with severe ME/CFS and those with ME/CFS and the controls. Conclusions: If confirmed in other populations, the FBI could serve as an objective test to aid in the diagnosis of severe ME/CFS. Abbreviations: ANOVA: analysis of variance; BSA: bovine serum albumin; FBI: fatigue biomarker index; IM: infectious mononucleosis; ME/CFS: myalgic encephalomyelitis/chronic fatigue syndrome; OF: oral fluid; S-ME/CFS: severe myalgic encephalomyelitis/chronic fatigue syndrome
Article
Full-text available
Objectives: Children with chronic fatigue syndrome/myalgic encephalomyelitis (CFS/ME) experience a higher prevalence of depression and anxiety compared with age-matched controls. Our previous systematic reviews in 2015/16 found little evidence for effective treatment for children with CFS/ME with comorbid depression and/or anxiety. This review updates these findings. Design: A systematic review. We searched Cochrane library, Medline, Embase and PsycINFO databases from 2015 to 2020. We combined the updated results with our previous reviews in a narrative synthesis. Participants: Inclusion criteria: <18 years old; diagnosed with CFS/ME (using Centers for Disease Control and Prevention, National Institute for Health and Care Excellence or Oxford criteria); validated measures of depression and/or anxiety. Interventions: Observational studies or randomised controlled trials. Comparison: Any or none. Outcomes: Studies with outcome measures of anxiety, depression or fatigue. Results: The updated review identified two studies. This brings the total number of paediatric CFS/ME studies with a measure of anxiety and/or depression since 1991 to 16. None of the studies specifically targeted depression, nor anxiety. One new study showed the Lightning Process (in addition to specialist care) was more effective at reducing depressive and anxiety symptoms compared with specialist care alone. Previous studies evaluated cognitive-behavioural therapy (CBT); pharmacological interventions and behavioural approaches. CBT-type interventions had most evidence for improving comorbid anxiety and/or depressive symptoms but varied in delivery and modality. Other interventions showed promise but studies were small and have not been replicated. Conclusion: Very few paediatric CFS/ME intervention studies have been conducted. This review update does not significantly add to what is known from previous reviews. The evidence is of poor quality and insufficient to conclude which interventions are effective at treating comorbid anxiety and/or depression in paediatric CFS/ME. Prospero registration numbers: CRD42016043488 and CRD42015016813.
Article
Full-text available
Objectives To explore factors perceived as positive or negative among young people with chronic fatigue syndrome/myalgic encephalomyelitis (CFS/ME) in relation to school and everyday life. Design A qualitative study with semistructured individual interviews performed at the local hospital or at the informants’ homes between September 2017 and January 2018, with an additional telephone interview to collect data on experiences from the COVID-19 pandemic, conducted in September 2020. Data were analysed using a grounded theory approach. Setting The informants were recruited from two university hospitals that offer interdisciplinary assessments of young people with CFS/ME from various parts of Norway. Participants Five males and 13 females aged 13–21 years with CFS/ME diagnosed 3–56 months prior to the interviews participated. Results The informants were concerned about a lack of educational adaptations and missed social life at school. Educational and social adaptations could improve schooling and health among young people with CFS/ME. Negative experiences were related to a lack of knowledge about CFS/ME among school personnel and young people’s difficulties to limit activities. Online teaching as experienced during the COVID-19 pandemic was described as positive both for education and social life. Conclusions Young people with CFS/ME can benefit from better educational adaptations and increased social interaction with peers. From the participants’ view, factors that limit learning and socialisation include a lack of knowledge about CFS/ME among teachers and school personnel, expectations from teachers of doing more than they could manage at school, feeling alone coping with the disease and not recognising their own limitations regarding what they are able to do. Suggested factors perceived to enhance learning and socialisation were a better understanding of the disease among school personnel and peers, suitable educational adaptations and being able to socialise with peers.
Article
Full-text available
Introduction: Chronic fatigue syndrome (CFS/ME) is a disabling disease severely impacting school attendance, education, and social life in young students. Uncertainties surrounding CFS/ME etiology may impact the interpretation of CFS/ME in schools. Thus, school personnel need information from health care providers to make adequate adaptations to education and social life at school for these students. Objectives: To explore teachers, counselors, and school nurses' experiences with adapting education for students with CFS/ME aged 13–19 in secondary and high schools. Design: A qualitative study with focus group interviews and individual interviews performed face-to-face or digitally between November 2020 and March 2021. Data were analyzed using Systematic text condensation. Participants: Six teachers, two counselors, and four school nurses in secondary and high school participated. Results: Adapting education for students with CFS/ME was challenging, especially before the students received a diagnosis. The challenges were related to identifying the students' adaptational needs, maintaining a teacher-student relationship due to school absence, difficulties in maintaining continuity of education, and uncertainty regarding the diagnosis. Successful adaptations were related to quickly reacting to school absence, early referral to educational, psychological services, a close collaboration with the school management, and the development of digital teaching for students with CFS/ME. Interdisciplinary collaboration and a clear, constructive plan with adaptive measures, including maintained teacher-student communication and educational and social adaptations, may be useful in preventing the losses, young people, with CFS/ME experience. Conclusion: Early interdisciplinary collaboration to adapt education and social life at school for students with CFS/ME, may support teachers, counselors, and school nurses in their efforts to adapt education and prevent losses related to academic and social development in students with CFS/ME.
Article
Full-text available
Designed by a group of ME/CFS researchers and health professionals, the European Network on Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (EUROMENE) has received funding from the European Cooperation in Science and Technology (COST)—COST action 15111—from 2016 to 2020. The main goal of the Cost Action was to assess the existing knowledge and experience on health care delivery for people with Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS) in European countries, and to enhance coordinated research and health care provision in this field. We report our findings and make recommendations for clinical diagnosis, health services and care for people with ME/CFS in Europe, as prepared by the group of clinicians and researchers from 22 countries and 55 European health professionals and researchers, who have been informed by people with ME/CFS.
Article
Full-text available
Background Chronic fatigue syndrome (CFS) is defined according to subjective symptoms only, and several conflicting case definition exist. Previous research has discovered certain biological alterations. The aim of the present study was to explore possible subgroups based on biological markers within a widely defined cohort of adolescent CFS patients and investigate to what extent eventual subgroups are associated with other variables. Methods The Norwegian Study of Chronic Fatigue Syndrome in Adolescents: Pathophysiology and Intervention Trial (NorCAPITAL) has previously performed detailed investigation of immunological, autonomic, neuroendocrine, cognitive and sensory processing functions in an adolescent group of CFS patients recruited according to wide diagnostic criteria. In the present study, hierarchical cluster analyses (Ward’s method) were performed using representative variables from all these domains. Associations between clusters and constitutional factors (including candidate genetic markers), diagnostic criteria, subjective symptoms and prognosis were explored by standard statistical methods. Results A total of 116 patients (26.7% males, mean age 15.4 years) were included. The final cluster analyses revealed six clusters labelled pain tolerant & good cognitions, restored HPA dynamics, orthostatic intolerance, low-grade inflammation, pain intolerant & poor cognitions, and high vagal (parasympathetic) activity, respectively. There was substantial overlap between clusters. The pain intolerant & poor cognitions-cluster was associated with low functional abilities and quality of life, and adherence to the Canada 2003 diagnostic criteria for CFS. No other statistically significant cluster associations were discovered. Conclusion Within a widely defined cohort of adolescent CFS patients, clusters could be delineated, but no distinct subgroups could be identified. Associations between clusters and constitutional factors, subjective symptoms and prognosis were scarce. These results question the clinical usefulness of searching for CFS subgroups, as well as the validity of the most “narrow” CFS diagnostic criteria. Trial registration : Clinical Trials NCT01040429
Article
Full-text available
Chronic faligue syndrome (CFS) in children and adolescents presents unique challenges and opportunities to researchers. Issues specific to research conducted on children and adolescents with CFS ate discussed. Such issues include the importance of utilizing a consistent definition of CFS and ascertaining that all participants meet the criteria, the need for attention to wording of questions regarding fatigue, and the significance of medical evaluations as part of a research study. Considerations pertaining to research with minors, such as confidentiality and assent, are explored. Finally, suggestions for future research on children are made.
Article
Full-text available
A community-based screening of over 12,000 households was conducted in order to determine the prevalence of fatigue and CFS-like illness in a sample of 5- to 17-year olds. Results indicate that over 4% of the sample was fatigued and that 2.05% were diagnosed with CFS-like illness. Adolescents had a slightly higher rate of CFS-like illness (2.91%) than did pre-pubescent children (1.96%). Those with CFS-like illness were almost evenly divided between male (47.5%) and female (52.5%). Youngsters of Latino origin had the highest representation in the CFS-like group. Symptom data, family patterns, and other data are presented for both the CFS-like group and the entire sample.
Article
The chronic Epstein-Barr virus syndrome is a poorly defined symptom complex characterized primarily by chronic or recurrent debilitating fatigue and various combinations of other symptoms, including sore throat, lymph node pain and tenderness, headache, myalgia, and arthralgias. Although the syndrome has received recent attention, and has been diagnosed in many patients, the chronic Epstein-Barr virus syndrome has not been defined consistently. Despite the name of the syndrome, both the diagnostic value of Epstein-Barr virus serologic tests and the proposed causal relationship between Epstein-Barr virus infection and patients who have been diagnosed with the chronic Epstein-Barr virus syndrome remain doubtful. We propose a new name for the chronic Epstein-Barr virus syndrome—the chronic fatigue syndrome—that more accurately describes this symptom complex as a syndrome of unknown cause characterized primarily by chronic fatigue. We also present a working definition for the chronic fatigue syndrome designed to improve the comparability and reproducibility of clinical research and epidemiologic studies, and to provide a rational basis for evaluating patients who have chronic fatigue of undetermined cause.
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Background: Characteristics of persons with chronic fatigue syndrome (CFS) have previously been studied by comparing subjects with CFS to subjects with other conditions or no symptoms of fatigue- In the present study of subjects with idiopalhic chronic fatigue we examined the association between the number and severity of the features of CFS with other characteristics of the subjects. Methods: Data were obtained from a registry of persons over the age of 17 with fatigue for at least six months. All subjects in the registry completed an extensive questionnaire that provided information about fatigue, demographic characteristics, medical conditions, life style, sleeping habits, and psychological characteristics. The characteristics of the subjects were tested for an association with the number of CFS symptoms and the severity of individual CFS symptoms that are considered to be of fundamental importance and may identify more homogeneous subjects with chronic fatigue. Results: The number of CFS symptoms had a bell shaped distribution. This number was strongly associated with the severity of fatigue, the response of fatigue to mental and physical activity, and the following subject characteristics: a greater frequency of sinus and respiratory infections, a higher frequency of migraine headaches, a greater number of somatoform symptoms that were not included as criteria for CFS, and not drinking alcohol. These same subject characteristics were generally associated with at least one of the individual CFS symptoms but more weakly. Psychological complaints only had a statistically significant positive association with one feature of CFS, neurocognitive complaints. Conclusions: Persons with fatigue can be usefully characterized by the extent to which they meet the CFS criteria.