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Pediatric Case Definition
Where To Find The Pediatric Case Definition for CFS/CFIDS/ME
Pediatric Case Definition for CFS/CFIDS/ME
Other Stipulations
Conditions That May Coexist With Pediatric ME/CFS
Psychological Instruments Used In Assessment of ME/CFS
More Resources
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The Chronic Fatigue Syndrome/Chronic Fatigue and Immune Dysfunction Syndrome/Myalgic Encephalopathy/ Myalgic Encephalomyelitis (CFS/CFIDS/ME) is not an illness limited to adults. The illness also occurs in both children and adolescents. Although CFS/CFIDS/ME in both children and adults is the same illness syndrome, there are some variations in the symptoms and the course of illness between adults and children. Pediatric CFS/CFIDS/ME can range in severity from mild to moderate ("moderate symptoms at rest that become severe with effort-unable to attend school") to severe ("often housebound or bed-bound").

In a community-based study in Chicago using the 1994 Centers for Disease Control & Prevention (CDC) case definition (Jason et al., 1999; Jordan et al., 2006) children and adolescents were found to have CFS/CFIDS/ME at a rate of 60 cases per 100,000. A second study in 2006 (Jason) "...found the prevalence of pediatric ME/CFS among the general adolescent population to be about...1.8 out of each 1,000 American children." (CFIDS Chronicle, Summer 2007) This rate makes pediatric CFS/CFIDS/ME more prevalent than sickle cell anemia, pediatric heart disease, and Down's syndrome among children.

Most child and adolescent cases are endemic—that is, the illness strikes an individual and not a cluster of individuals in geographic or social proximity. However, epidemic cases do occur, notably an epidemic in Lyndonville, N.Y. from 1984-1987, in which many children in a small, upstate community became sick during the same time period. Sometimes, a sibling or parent would also become ill. (Much earlier episodic outbreaks before 1960 were often associated with epidemics of unusual versions of "atypical mild polio".)

Prior to 2006, especially before 2003 when the CFS/CFIDS/ME Canadian definition was published, children and adolescents were diagnosed in the United States by either the 1988 or the 1994 CDC adult definitions. In England, many children were, unfortunately, diagnosed according to the entirely faulty Oxford definition. In 1997, in the Netherlands, a case definition was proposed "in order to increase coherence in child ME/CFS" (DeJong et al.). The 2003 Canadian definition proposed, in a short section, that children could be diagnosed if their symptoms lasted more than 3 months—as opposed to the adult 6-month standard.

The Need for A Specific Pediatric Case Definition for Children and Adolescents

Despite the diagnostic improvement provided by the 2003 adult Canadian definition, the lack of a specific CFS/CFIDS/ME diagnostic criteria for children and adolescents continued to create very serious problems. Except for a few clinicians, including Dr. David Bell in Lyndonville, New York, CFS/CFIDS/ME researchers and clinicians knew even less about many of the specific aspects of pediatric CFS/CFIDS/ME than they knew about the adult illness.

The lack of any diagnostic criteria defining and substantiating the illness in children certainly did nothing to support the recognition of the existence of pediatric CFS/CFIDS/ME. The defining and naming of an illness gives validation to its existence—moreover such validation provides an incentive to medical research, and research provides further validation.

Dr. Leonard Jason (a pioneer in pediatric CFS/CFIDS/ME) and Nicole Porter in the CFIDS Chronicle Summer 2007 summed up the problems stemming from the lack of a specific pediatric case definition:

"Due to the lack of understanding and the long absence of a clear, pediatric-specific case definition for the illness, under-diagnosis or medical misunderstanding can lead to long-term developmental impediments not directly related to the physiological disorder."

"For example, without a clearly defined medical explanation for frequent absences and academic challenges, the skepticism, marginalization and punitive measures taken by many school districts and local judicial systems have caused additional problems for many families already dealing with the immediate difficulties associated with this illness."

"Disturbingly, these diagnostic problems sometimes lead to juvenile truancy charges and even allegations of parental abuse and neglect. This makes the misdiagnosis and under-diagnosing of pediatric ME/CFS a critical issue—forcing a family to negotiate the continuous compromises to the child's physical health, while also struggling against a system that doesn't understand and validate the illness and special issues the child faces."

In addition to these many difficulties, "The lack of application of a consistent pediatric definition of ME/CFS and the lack of a reliable instrument to assess it...might lead to studies which inaccurately label children with a wide variety of symptoms as having ME/CFS as well as possibly missing children who have it." (Jason et al., "A Pediatric Case Definition for ME/CFS", JCFS (2006) 13.(2/3).)

Misdiagnosis of children with CFS/CFIDS/ME as being "merely" depressed, as having a school phobia, psychosis, "attention needs", etc. is made even more probable without a validating diagnosis.

More Resources

Children and Youth


Where To Find The Pediatric Case Definition for CFS/CFIDS/ME

By 2006, the International Association of Chronic Fatigue Syndrome Working Group for a ME/CFS Pediatric Definition developed and published (in the Journal of Chronic Fatigue Syndrome, v. 13 n.2/3, 2006) a comprehensive "Pediatric Case Definition for Myalgic Encephalomyelitis and Chronic Fatigue Syndrome." The paper also included an instrument (a comprehensive questionnaire) that doctors and other professionals can use to specifically assess CFS/CFIDS/ME in children.

The International Association of CFS, (now the International Association of CFS/ME) is the international group of CFS/CFIDS/ME researchers and clinicians. Under its auspices, a number of the leading researchers and clinicians in the field of pediatric CFS/CFIDS/ME came together to develop the definition. These included: Dr. Leonard Jason, Dr. David Bell, Dr. Charles Lapp, Dr. Karen Jordan, Dr. Kenny de Meirlier, and Dr. Alan Gurwitt (a long-time member of the Massachusetts CFIDS/ME & FM Association), along with other experts.

The entire 2006 Pediatric case definition can be accessed (along with other case definitions) at the M.E. Society for America link:

http://www.cfids-cab.org/MESA/ccpc.html

You can also download a free copy of the pediatric CFS/CFIDS/ME assessment instrument, the DePaul Pediatric Health Questionnaire, for your physician at

http://www.cfids.org/bonus/pediatrictool.asp.

In 2008, a slightly revised version of the 2006 pediatric case definition was published:

Jason et al., "A Case Definition for Children with Myalgic Encephalomyelitis/Chronic Fatigue Syndrome," in Clinical Medicine: Pediatrics, 2008: 53-57.

This shorter and revised version can be accessed at the following link: http://www.la-press.com/a-case-definition-for-children-with-myalgic-encephalomyelitischronic-f-a1147 (The 2006 definition is the most comprehensive, along with its assessment instrument; but the 2008 version is probably more readable and usable for general pediatricians. Updated revisions in the 2008 version will be noted below.)

The 2006 Pediatric Case Definition includes sections: "Diagnosis and Prognosis", ‘Differentiate between Diagnoses", "Prevalence of Pediatric ME/CFS", "Changes from the Adult Definition", "Clarification of Diagnostic Criteria", "Psychological Instruments", "Interventions to Increase Quality of Life", and "Suggestions for Future Research on Pediatric ME/CFS". The entire article is 23 pages, although the diagnostic criteria list is 2 ½ pages. We will present a brief summary of the criteria below.

The definition itself contains elements of the 1994 CDC adult case definitions along with the 2003 Reeves et al. revisions. However, the structure of the 2003 Canadian adult ME/CFS definition is prominently incorporated into the new pediatric definition—thereby giving additional weight to symptoms based not exclusively on fatigue.

During the diagnostic process, the doctor "must carry out a thorough evaluation, including a comprehensive medical and developmental history, physical examination and laboratory tests to confirm diagnosis. The history should involve both parents as well as the child..."


Pediatric Case Definition for CFS/CFIDS/ME

Diagnostic criteria summary:

A child (person under 12-13) or adolescent (12-13 to adult) to be diagnosed with pediatric ME/CFS will meet the following criteria:

1. "Clinically evaluated, unexplained, persistent or relapsing chronic fatigue over the past 3 months that:

a) "Is not the result of ongoing exertion [this does not include the child with pediatric ME/CFS who may become exhausted after minimal exertion];"

b) "Is not substantially alleviated by rest [in the pediatric ME/CFS patient, rest may provide some relief, but not eliminate the syndrome—furthermore even minimal exertion may quickly lead to renewed sickness.]"

c) "Results in substantial reduction in previous levels of educational, social and personal activities"

d) "Must persist or recur for at least all three months." [This requirement is "operationalized" by assessing 'how often the patient has experienced the symptom over the past 3 months on a 7 point scale: 1=hardly ever to 7=every day; to meet the above requirement a score of at least 4 would be indicated."]

2. "The concurrent occurrence of the following classic ME/CFS symptoms, which must have persisted or recurred during the past 3 months of illness (symptoms may predate the reported onset of fatigue)."

a) "Post-exertional malaise...with loss of physical or mental stamina, rapid muscle or cognitive fatigability."

b) "Unrefreshing sleep, or disturbance of sleep quantity or rhythm."

c) "The young person has at least one symptom from any of the following:

1) myofascial pain, 2) joint pain, 3) abdominal and/or 4) head pain"

d) The occurrence of "two or more neurocognitive manifestations, including impaired memory, difficulty focusing, difficulty finding the right word, frequently forgetting 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."

e) "Finally, the fifth symptom category requires at least one symptom from two of the following 3 subcategories:

1) autonomic manifestations (neurally-mediated hypotension, postural orthostatic tachycardia, dizziness, shortness of breath, palpitations with or without cardiac arrhythmias, feeling unsteady on the feet—disturbed balance..."(see complete list via link)

2) "neuroendocrine manifestations (recurrent...feverishness and cold extremities, subnormal body temperature...sweating episodes, intolerance 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 palpitation—generally minimal swelling...new sensitivities to food, odors, or chemicals."

"Note: In about 25% of pediatric cases, the onset of the illness is insidious (slowly over time) rather than acute."


Other Stipulations

This case definition includes a number of further qualifications and quantifying criteria.

The diagnostic criteria stipulates that "each of the symptoms be either moderate or severe." The following rating scale is to be used by the diagnosing physician: "1= not present, 3= moderate, 7 = severe. Symptoms need to be rated at moderate or severe...to meet criteria."

Also, the definition includes a method for assessing "illness severity and remission states": "(A) minimal (just enough symptoms to meet the diagnosis, particularly occurring with exertion, usually able to attend school); (B) mild (few symptoms in excess of those of 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 bed bound); (E) in partial remission (full criteria...were previously met, but currently only a few symptoms remain with effort—able to attend school regularly); (F) in full remission (no longer any symptoms, even with effort—able to attend school)."

Exclusionary Conditions

The diagnostic criteria also note separate diagnoses that would exclude a finding of pediatric ME/CFS  (unless they had been separately and successfully treated).

a) These include: "untreated hypothyroidism, sleep apnea, narcolepsy, malignancies, leukemia, unresolved hepatitis, multiple sclerosis, juvenile rheumatoid arthritis, lupus erythematosus, HIV/AIDS, severe obesity... Lyme disease, celiac disease."

b) "Some active psychiatric conditions that may explain the presence of chronic fatigue such as: childhood schizophrenia or psychotic disorders; bipolar disorder; active alcohol abuse unless it has been successfully treated and resolved; active anorexia nervosa or bulimia nervosa, unless it has been treated and resolved."

c) "depressive disorders" -Special note: These are depressive disorders not related to, or secondary to the pediatric ME/CFS. These exclusionary depressive disorders must also adequately explain the physical symptoms. Please see differential diagnosis section below.


Conditions That May Coexist With Pediatric ME/CFS (co-morbid conditions)

The child or adolescent may have concomitant disorders "that do not explain fatigue, and are, therefore, not necessarily exclusionary: (a) psychiatric diagnoses such as school phobia, separation anxiety, anxiety disorders, somatoform disorders, depressive disorders."

Also, a child with pediatric ME/CFS may also concurrently have fibromyalgia and/or multiple food and chemical sensitivity. See the full pediatric definition for further concomitant illnesses.

Differential Diagnoses with Pediatric ME/CFS

Often pediatric ME/CFS is misdiagnosed as a psychiatric or behavioral disorder. These disorders include depression, anxiety, and school phobia—among others. However, a child or adolescent may actually have these disorders rather than pediatric ME/CFS.

It is critical for the diagnosing physician to determine if the child or adolescent actually has pediatric ME/CFS (even if these psychiatric or behavioral disorders coexist with the illness), or if the child does not have pediatric ME/CFS but is suffering exclusively with a psychiatric and/or behavioral disorder.

Psychiatric and Behavioral Diagnoses and ME/CFS

First, it should be noted that "somatization disorder that meets the DSM-IV criteria is rare in this age group", i.e., children and adolescents.

Depression: The pediatric criteria makes clear that depression of any diagnostic category may be a symptom of, or even co-exist, with childhood or adolescent ME/CFS. Depression is less common in childhood as opposed to adolescence, and symptoms may be different in the two age groups. Since symptoms of depression may co-exist with ME/CFS, the diagnosing physician must conduct a careful evaluation of the "differing developmental presentations...Inquiring about hobbies and leisure activities is important in distinguishing depression" and pediatric ME/CFS. Those with the physical illness will likely have abandoned their hobbies and leisure activities. Much of the concomitant depression in ME/CFS comes from "underlying frustration as a result of losing control rather than a negative self-image."

Falling behind in school with no recognition of their illness and with no extra help or support—and thereby having to struggle to catch-up—will often lead to depression and anxiety. Moreover, the isolation and loss of friendships and peer activity can lead to depression.

One primary way to differentiate between ME/CFS with depression, as opposed to a separate depressive illness, is to look carefully at symptoms. Depression itself is unlikely to cause multiple symptoms such as sore throats, swollen lymph nodes, fevers, etc. If a diagnosis of depression better explains the child's fatigue and symptom patterns, then depression may be the appropriate diagnosis as opposed to ME/CFS.

School phobia and separation anxiety: When these conditions predate the fatigue and other symptoms, it is possible that the child does not have ME/CFS and the symptoms are better explained by either of these diagnoses. "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 at home from school...In contrast, children and adolescents with ME/CFS would experience symptoms not only at school, but in other settings." Those with school phobia only would not usually have symptoms on weekends or school holidays.

The definition also contains a short discussion as to whether the child might be exhibiting symptoms due to family dysfunction as opposed to ME/CFS. A child with symptoms clearly aimed at holding a family together would not be diagnosed with ME/CFS—however a child truly ill with ME/CFS might be living in an unhealthy family system.


Psychological Instruments Used In Assessment of ME/CFS

The definition provides a discussion and listing of instruments used to assess comorbid psychiatric conditions, sleep disturbance, and physical and psychological well-being.

Pediatric Health Questionnaires

Appendix A in the definition provides a copy of the Questionnaires which are helpful in diagnosing pediatric ME/CFS. "It is generally recommended that adolescents age 12 and over fill it out themselves, and parents can assist or fill it out for children under 11..." More than one person may fill out the questionnaires—child, parents, caregivers, etc. Some clinicians feel that both parents should fill out the forms, since the view of one parent may be limited. Research has also shown that the parents' perceptions of the illness may vary from those of the child.

2008 Revised Pediatric Definition

In 2008, "A Case Definition for Children with Myalgic Encephalomyelitis/Chronic Fatigue Syndrome" was published in Clinical Practice: Pediatrics 2008 1: 53-57. Most of the articles' authors were the same who wrote the 2006 definition.

This 2008 definition is the same as the 2006 definition, except for a few small revisions.

In the revised criteria, a separate category was established for those children and adolescents who met "almost all the criteria, and they were classified with the Moderate ME/CFS Clinical criteria. To meet this criteria, the patient has to meet four out of the five classic ME/CFS symptoms with frequency and severity ratings of moderate or severe...In addition, for autonomic, neuroendocrine, and immune manifestations, adolescents had to have at least one symptom in any of the 3 categories..."

The revised definition also set up some other subcategories for those with idiopathic chronic fatigue—see the 2006 definition. One subtype in this category is Atypical ME/CFS, which is defined "as 3 or more months of fatigue, but missing more than one classic ME/CFS symptoms...Another category involves Pediatric ME/CFS-like illness, which is defined at meeting all classic ME/CFS symptom criteria, except for the 3 month duration or when one is lacking a medical evaluation."


More Resources

A Son's Road to Recovery

Developmental Issues in Adolescents with CFIDS - A Parent's Thoughts

On the Morbid Fascination with Psychiatric Morbidity

Planning Ahead for Next School Year

Teenage CFIDS - A Personal Story

 


 
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