Chronic Fatigue Syndrome/Myalgic Encephalomyelitis (CFS/ME), is widely recognized in adults. But it is not as well known that children and adolescents can have the illness. Recognition of CFS/ME in young people can be difficult because they are physically, emotionally and socially different from adults.

• About Pediatric CFS/ME
• Misdiagnosed - Undiagnosed
• What Do We Know From Research?
• What's Needed Next?
• Case Definition
• Development
• Symptoms
• Possible Causes
• Diagnosis
• Duration
• Treatment

Although CFS affects children and adolescents in many of the same ways it affects adults, there are some significant differences. From a clinical standpoint, one major difference is in onset.

In adults, gradual onset occurs in up to 75% of cases. In children with CFS, most of whom are adolescents, it's reversed, with around 75% experiencing acute onset. (Gradual onset, however, is more common in young children.)

There is variability of the illness in symptoms, which tend to migrate. For example, a child may experience sore throat and headache, followed the next day by lymph node pain. There is also considerable variability in symptom severity.

In adults, there are often a few symptoms that are worse than others. In children, certain symptoms may be worse one day, only to be replaced by other severe symptoms a few days or weeks later. This dynamic can be further complicated by the fact that children may have more difficulty recognizing and verbalizing their symptoms.

Dr. David Bell, a leader in the field of Pediatric CFS/ME believes:

The most striking thing about pediatric CFS is the ability of children to adapt to their symptoms. Yet that fact makes it more difficult to detect and treat the problem."

A teen may come home and sleep for three hours after school every day, but may not complain of tiredness because it has become the norm. Similarly:

"young children who grow up with CFS have become accustomed to the symptoms and are able to function despite persistent and sometimes severe discomfort."

The psychosocial aspects of the illness are another important difference for pediatric patients. Says Peter Rowe, MD, a researcher at Johns Hopkins University:

"One of the biggest challenges to pediatric patients results from the fact that the illness affects them before they've truly had an opportunity to identify what their aptitudes and strengths are in life, and to have established strong emotional relationships with a significant other. The accountant who develops CFS as an adult knows she was a capable, functioning contributor to society, and that the illness doesn't define her as a person."

CFS/ME is often misdiagnosed in children because the symptoms are not consistent. The child will experience varying symptoms at different times of the week or month. When the parents take their children to the doctor each time, the complete set of symptoms experienced over time are often not pieced together.

Another reason why CFS/ME is going undiagnosed in children is because the criterion developed by the CDC is for CFS/ME in adults. For children with CFS/ME, two types of onset patterns have been noted. In children ages 5 - 12, a gradual onset of symptoms is usually seen. In children 13 and older, it is more common to see an acute onset of symptoms.

The parents of these children often misunderstand their sick child as well. For young children with CFS, they may not even comprehend that they are sick because they have no knowledge of what normal and healthy feels like.

If you suspect your child has Chronic Fatigue Syndrome, he or she should be seen by a CFS/ME specialist. General practitioners are typically not knowledgeable in dealing with Chronic Fatigue Syndrome and if they are, they have probably dealt more with adults than children. Visit our doctor database for help in finding a doctor who specializes in CFS/ME treatment.

It's important to listen to your child and take their symptoms seriously. If your child complains a lot about being fatigued, do not just assume that they are lazy or unmotivated. If your child complains a lot about body pain, do not just attribute it to growing pains. Regardless of age, we all need validation when we are truly ill.

In spite of the paucity of research, studies have shed light on various aspects of pediatric CFS. The main studies show the following findings:

• CFS occurs in children and adolescents, though less frequently in children younger than age 10. The age at onset of Bell's pediatric CFS patients ranges from 11-18; for Rowe the age range is 10-21, with the average age at onset of 15. Both have seen younger CFS patients, but not frequently. This suggests age of onset may be connected to hormonal changes as children approach puberty, but this isn't confirmed.


• Pediatric CFS has quite a variable duration, from months to many years. Although many children get better, the illness can be more chronic than we first thought. For instance, a 13-year follow-up study of 35 pediatric cases by Bell indicated that as many as 20% don't recover, and of the 80% who reported a good functional outcome, only 37% considered themselves fully recovered.


• Comorbid psychiatric disorders such as anxiety and depression are not uncommon, but they are generally mild when present and often secondary to the effect of being ill. Many kids with CFS have no anxiety or depression, in contrast to prior views of CFS as a form of generalized anxiety disorder or atypical depression.


• There is a strong association between adolescent CFS and related circulatory disorders that are characterized by intolerance of prolonged upright posture. Rowe reports that in 90% of untreated pediatric patients, this worsening of CFS symptoms with standing is associated with abnormalities in the regulation of heart rate and blood pressure. The most common of these problems is neurally mediated hypotension (NMH), but postural tachycardia syndrome (POTS) is also common. These disorders are treatable, which has opened up new avenues for the rehabilitation of patients with CFS.


• A physical examination of children with CFS may not be normal, as was once claimed. In addition to the orthostatic intolerance just mentioned, adolescents with CFS are more likely to have joint hypermobility, a physical trait that is present before they become ill. Children with CFS are also more likely to exhibit movement restrictions such as restricted prone knee bend, ankle dorsiflexion and straight leg raise during the physical exam.


• Preliminary evidence suggests that certain treatments such as cognitive behavioral therapy and graded exercise may help pediatric patients manage CFS.

The variability of the illness in children makes it difficult to diagnose and identify treatments that would be beneficial. In the short run, this points to the critical need for individualized care for children with CFS. In the long run, it emphasizes the need for a much larger research initiative.

Rowe and Bell agree on the vital need to focus more research on pediatric CFS, not only because of the debilitating nature of the disease itself, but because of the critical period of life it disrupts.

As Rowe puts it:

"Chronic illness and its limitations on activities constrain a young person's ability to determine identity, discover skills and form strong emotional relationships with others. There is an inability to engage in the normal activities of adolescence that help with the healthy transition to adult life."

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 personal 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 often described 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

E. At least one symptom from two of the following three categories:

1. Autonomic manifestations: Neurally mediated hypotension, postural orthostatic 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 fluctuations, 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 palpitation, 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

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.

What happens to children and adolescents who develop CFS/ME? The majority of children, particularly adolescents, have an acute onset of symptoms that marks the beginning of CFS/ME. An acute onset is characterized by the sudden appearance of symptoms within a few days to weeks, usually with a flu-like or mononucleosis-like illness in a child who had previously been healthy.

Children who experience an acute onset can clearly describe their symptoms, such as the degree of fatigue or impairment in cognitive abilities, in comparison to their pre-illness state. These children may accumulate a bewildering array of diagnoses from their pediatricians, including childhood migraine syndrome, Crohn's disease, atypical epilepsy, school phobia, attention deficit disorder, rheumatoid arthritis, chronic rheumatic fever, irritable bowel syndrome and others.

Gradual Presentation

The gradual presentation of CFS is slightly different and seems to occur more often in younger (pre-adolescent) children. It is defined by the appearance of symptoms over several months or longer, or by mild symptoms suggestive of CFS (frequent sore throats, headaches or joint pains, relative inactivity, sleeping more than other children of the same age) prior to an acute episode.

Children and adolescents with CFS/ME are occasionally extremely pale. More often, however, they look completely well. The lack of obvious signs of illness may mean that the first sign of CFS/ME will be a marked limitation in either physical or mental activity. These limitations are usually first noticed by a parent or teacher.

Outward Appearance

The outwardly normal appearance of a CFS/ME child may lead to the accusation of School Avoidance Behavior (school phobia) or the citing of a parent for Munchausen's syndrome by proxy. The severity of CFS/ME varies.

Some children are severely disabled and bedridden. Others can go to school. A few are capable of playing sports. Most children fall between the two ends of this spectrum. As a rule, CFS/ME is more disabling than other chronic illnesses. The pattern and severity of the symptoms experienced by a child may change markedly from day to day or during the day. It is important to listen to what the child has to say about the severity of his/her symptoms. Remissions and relapses are common. Relapses may be caused by over-exertion or by other infectious illnesses.

Recovery Rates

Over time, slow improvement is likely, especially in the first four years. Recovery rates are uncertain but rates of up to 40 percent have been reported. Children whose health improves to near pre-illness levels are likely to find that they need more rest than their peers.

A student who becomes chronically ill may develop the additional problem of emotional conflict. The emotional conflict by itself can impinge upon a student's education. A teacher can better assist a chronically ill student once he/she realizes that the student is exhibiting one of the four behaviors associated with the emotional conflict:

• 1. Denial - I am not sick. I want to be like everyone else. (The student overdoes on good days, followed by severe relapses.)


• 2. Isolation - I cannot keep up with my peers. My peers ridicule me because some days I can keep up and other days I can do nothing.


• 3. Depression/Anxiety - I will never get well. I will never do what I want to do. I will never be who I want to be.


• 4. Resiliency - I am who/what I am. I will do what I can. My illness has taught me special things. I will be a different person than I imagined.

Psychotherapy

Psychotherapy can be helpful in working through the phases of this emotional conflict both for the patient and the family. For psychotherapy to be successful, the therapist needs to understand and be experienced with treating patients with illnesses like CFS/ME.

Symptoms of Chronic Fatigue Syndrome / Myalgic Encephalomyelitis (CFS/ME) include:

• Debilitating Fatigue
• Cognitive Difficulties
• Widespread Pain
• PostExertional Malaise
• Sore Throat
• Irritable Bowel Syndrome (IBS)
• Sleep Disorders

In adults with CFS/ME, these symptoms usually all occur at the same time at the onset of the illness. Children are often misdiagnosed because their symptoms will migrate. Due to the cognitive difficulties seen in this illness (short-term memory problems, lack of concentration), CFS children can be misdiagnosed and told they have attention deficit disorder (ADD).

Children with CFS/ME can appear normal and appear as though their activity levels are normal but upon closer evaluation, the parent will find that their child has to rest more after a sporting event, dance or other physical activity. Many people believe they had a milder case of CFS/ME as a child that exasperated when they got older. Many doctors attribute pain in children to "growing pains" when indeed they most likely had CFS/ME.

Pediatric patients with CFS/ME are less likely to have severe mood-related symptoms compared with patients who are suffering from clinical depression (eg, major depression). These mood-related symptoms include negative affect, suicidal ideation or previous suicidal behavior, externalizing or acting-out behaviors, problems with angry or aggressive behavior, low self-esteem, and feelings of reduced self-efficacy. Thus, research findings with pediatric patients suggest that children and adolescents with CFS/ME are not depressed in the conventional sense. Nevertheless, these patients are psychologically distressed when compared with healthy people of the same age.

Although social and academic activities may be disrupted, children with CFS/ME do not demonstrate the same level of decline in these areas experienced by children with clinical depression. Many children with CFS/ME are high achievers and report dissatisfaction even with above-average performance. Self-esteem and self-concept concerns are less likely to be issues than with depressed youth. Anecdotally, numerous investigators and clinicians have noted that pediatric patients with CFS/ME and their family members are inclined to deny the need for psychological assistance for their personal problems, to minimize the role of stressors in their symptoms, and to reject suggestions that psychological assistance may facilitate their recovery. They generally attribute their symptoms to infectious and immunological causes, which may be strongly reinforced by the lay literature.

Clearly, a disease this debilitating affects multiple aspects of functioning and development, as well as the life of the family as a whole. Many subjects reported regression via increased dependence in their relationship with their parents because of their physical limitations. The patients spent more time with their parents than the average adolescent or young adult and believed their health status made their parents overprotective and more cautious. At a time when many healthy peers are gaining more freedom and independence from their parents, these families displayed a level of involvement that may have delayed some of these common rites of passage, such as moving out of the home, going away to college, and working full-time.

In terms of their school functioning, many subjects in the study reported cutting back on the hours and days that they spent in class, despite previous successes achieved in the academic arena. Curiously, although many subjects believed that their grades had declined, they still believed that they performed at a level either commensurate with or superior to their classmates. Subjects reported a preference for quieter activities with smaller groups of friends and indicated that they received criticism and disbelief from some peers and teachers regarding the validity of their illness and lengthy absences.

The cause of CFS remains unknown. Many viral, bacterial and psychological causes have been considered and rejected, but the search continues.

From 10-15 percent of people with CFS have a family member who also has the illness, leading researchers to speculate that genetics may play a role, but more research is needed.

The diagnosis of CFS/ME is entirely clinical: a characteristic pattern of somatic symptoms (see Centers for Disease Control and Prevention (CDC) case definition) dominated by unexplained fatigue limiting normal activity. Laboratory testing should be used to exclude other possible causes for the symptoms, but by far the most effective tool to use when CFS/ME is suspected is an activity scale or daily diary that measures a child's overall normal activity levels.

A child with CFS/ME will have substantially reduced activity levels, but may appear to others to have a normal activity level. For example, a child with CFS/ME may be able to participate in sporting activities on the weekend (i.e., playing in a soccer game), giving the appearance that she is healthy. On closer examination, however, the child with CFS/ME who participated in the soccer game would have to rest for the remainder of the day and usually the next day.

School attendance is the most important indicator of both severity and prognosis of pediatric CFS/ME. If the CFS/ME symptoms are mild, the child can make it through a full day of school, perhaps even with gym class, but will have increased fatigue and may need to rest often. Moderate CFS/ME permits part-time school without gym or sports and requires the child to get extra rest every evening.

With severe forms of CFS/ME, school attendance may not be possible, and home tutoring should be used to maintain an appropriate grade level.

Comparing the child's activity levels on weekends and during the summer to their activity during the school week is also important. Children often report that they feel better in the summer, which is often considered an indication of school phobia. However, it may actually be because fewer sustained activities are required.

Measuring activity levels helps to rule out school phobia or depression. Depressed children do not feel overly tired and weak. A child with CFS/ME will be just as inactive on the weekend as during the school week or may actually maintain less activity during unrestricted times.

Pediatric CFS is frequently misdiagnosed as a behavioral or emotional problem, in particular school phobia. Unlike children with school phobia, children with CFIDS are typically ill on weekends as well as during the school week. Many children with CFS also have orthostatic intolerance, which causes inability to tolerate upright posture.

Many children (and adults) do recover from CFS. However, there is no standard duration for the illness. In the few published studies that have looked at outcomes of pediatric CFS this is what they have found:

• 8%-27% percent of children with CFS "recovered"


• 27%-46% percent improved


• 12%-29% percent remained unchanged from the onset of the illness


• The majority of children with CFS (up to 94%) experience worsening of their school performance due to the physical and cognitive symptoms.


• 20% to 44% percent of children with CFS must be home schooled because they are too ill to attend classes.

Children with CFS who cannot attend school miss out on important social development opportunities. They can be classified as disabled and may be entitled to educational services under the Individuals with Disabilities Education Act and/or Section 504 of the Rehabilitation Act of 1973.

To date, only a few studies have attempted to document the natural history and long-term outcome of pediatric CFS/ME, both in terms of health status and psychosocial morbidity. In general, the physical outcome of CFS/ME in these studies (ie, mean time since initial evaluation; range of 1.6-3.8 y) was favorable, with most patients reporting either resolution or improvement in their symptoms; however, a sizable proportion of these patients reported continued symptoms and significant functional limitations. As in many chronic diseases, children with CFS/ME face significant functional limitations (eg, frequent absences from school, disruptions in social activities, adjustments in family dynamics) surrounding the symptoms and treatment of their illness.

The first step in properly treating children with CFS is making the diagnosis. There is a great body of knowledge available about how children cope with chronic illness, and identifying a reason for their poor health will help them cope with it in an emotionally healthier way.

Failure to establish the diagnosis and lack of cooperation between professionals may lead to inaccurate impressions that the child is malingering or "faking." This may result in isolation, insecurity, sense of failure, family stress and even legal action against the family by school authorities. Further, an unresolved diagnosis may leave the child with uncertainty as to whether he or she is really experiencing the symptoms, or whether they are "all in the mind," as skeptics claim.

Otherwise, treatment for children varies little from that for adults and is intended primarily to relieve specific symptoms. Treatment must be carefully tailored to meet the needs of each patient. Sleep disorders, pain, gastrointestinal difficulties, allergies, dizziness, light-headedness, blood pressure irregularity and depression are some of the symptoms which physicians commonly attempt to relieve with prescription and over-the-counter medications. Children with CFS may have unusual responses to medications, so low dosages should be tried first and gradually increased as appropriate.

Treatment is otherwise largely supportive and responsive to symptomatology. This includes physical therapy and modest aerobic or anaerobic exercise (if possible) to avoid cardiovascular de-conditioning. Sleep may be addressed with medication; often, melatonin or night-time amitriptyline is helpful. If present and severe, pain is often addressed in a pain clinic. Headache may be successfully treated with anti-migraine therapy, and biofeedback regimens have been helpful at times. If concurrent with orthostatic intolerance, headache may be amenable to measures designed to improve blood flow and blood pressure. Fatigue and neurocognitive defects are harder to address. Some clinicians have used stimulants with some success.

Lifestyle changes, including increased rest, reduced stress, dietary restrictions, nutritional supplementation and light exercise (such as walking) also are frequently recommended. These changes will likely impact a young person's educational and social experiences, and their long-term impact must be considered. Supportive therapy, such as counseling, can also help a young person with CFS identify and develop effective coping strategies. Chronic illnesses such as CFS are traumatic for the child's family as well as the child, and support from school officials, physicians and friends is important.