Betrayal of the Severely Ill?
Appendix 10: CFS In Adolescents: Spectrum of Illness
Dr David Bell
Chronic fatigue syndrome (CFS) has been documented to occur in children and adolescents, but there has been ongoing confusion concerning incidence, clinical manifestations, and severity. The majority of paediatricians believe that if CFS exists at all, it is a short lived, perhaps trivial condition, and a manifestation of psychiatric illness. Many paediatricians would argue that CFS should not be diagnosed in children because the diagnosis will "cause" the illness to persist. This underlying assumption concerning the trivial nature of CFS and the minor impact on a child's life are not consistent with natural history of the illness as seen by clinicians.
The assumption of the benign nature of paediatric CFS has resulted in little attention paid to children and adolescents with this illness, and no paediatric diagnostic criteria have been developed. While it is possible that CFS can be mild and resolve completely in some young persons, there is a wide spectrum of illness severity, and severe cases exist. Five case histories of severe paediatric CFS are presented, each of which contradicts a commonly held belief about the illness, including severity, symptomatology and duration of illness. Through case presentations CFS can be seen as a serious, even life threatening illness, one that is not explained by psychiatric theory. The clinical course can cause severe functional limitation, pain and expose the child or adolescent to significant risks, such as the need for parenteral nutrition. The greatest emotional pain caused by the illness exists through the neglect or denial of both society and the medical profession.
There is an urgent need for increased attention to CFS among young persons. Studies concerning the clinical presentation, natural history, and long term prognosis should be undertaken. Specific diagnostic criteria should be developed, and clinicians should be educated to the possibility that CFS can extend into severe, perhaps lifelong, symptoms.
Modern medicine has been forced to confront numerous challenges over the past fifty years. Infectious disease, cancer, even HIV infection have been met by governmental agencies, specialty medical care and supportive primary care physicians, and while this may not have conquered the illnesses involved, those suffering have been shown that they have not been ignored.
The situation is quite different for chronic fatigue syndrome, an illness whose existence and even name are still disputed sixty years after the first modern clinical descriptions (1-3). It is not uncommon for a person with CFS to visit many physicians before a diagnosis is made, and even after diagnosis, patients state that they are unable to find a primary care physician who will look after them. It is disturbing to hear patients state that their doctor "does not believe in chronic fatigue syndrome", as if this condition were a religion or cult. CFS is not a religion, it is a medical condition that causes great hardship to those who experience it.
The crisis faced by those suffering from chronic fatigue syndrome is not due to the widespread assumption that it is a psychiatric disease. It is the result of the inability of society, governmental agencies, and the medical profession to accept that it exists at all. All illnesses exist as an interplay between mind and body. Diabetes mellitus can be interpreted as a psychiatric condition because emotions are important in the regulation of blood sugar. Schizophrenia has traditionally been considered a psychiatric condition, yet recent research suggests that it may be an organic disease. For the most part, modern medicine has coped well with both organic and psychiatric illness, affording compassionate and supportive care. But patients with CFS have been lost between arguing primary care physicians and specialists, generally experiencing neither support nor compassion. Medical care has traditionally been excellent in allowing the ill patient dignity, except in the case of chronic fatigue syndrome.
Basic to this problem has been that medical practitioners do not perceive CFS as an illness. The label of psychiatric disease has been used as a polite term for hypochondriasis. The harried primary care physician struggles to keep up with well described illnesses; there is little time left over for the complexities of an illness that does not show up on simple testing. It is easier to assume the patient is fabricating. An improvement in care would exist if CFS patients were to receive the same level of care afforded to patients with established psychiatric disease.
Children with CFS have been described in the medical literature (4,5), but their condition is more urgent than that of adults. They are less able to speak forcefully for themselves in front of an authority who tells them that they are imagining symptoms. Parents become spokespersons and are then accused of being overly involved. The most frequent response elicited in a child with CFS is to pretend to be well, a response that will meet with approval from the physician, the school, and even family. However this response, even in those children lucky enough to be able to attempt it, has its drawbacks, as it leads to somatic identity confusion. I have seen adults quite ill with CFS since childhood, who ignore half of their life because they are convinced they must be crazy.
Schools, along with most of society, complicate the situation for children with CFS. Rarely is the illness understood or accepted without physician support. Educational services are inadequate, and child abuse charges are filed for truancy. When home tutoring is afforded it is done so grudgingly, and only when the school feels the threat of legal action. In some children with mild CFS, I have written letters asking them to be excused from gym, as this activity jeopardises school attendance. School authorities comply, but make the children write unnecessary weekly compositions as a punishment.
It has always been my belief that CFS is an organic, not psychiatric illness. This view stems from my training in paediatric chronic disease and as a family therapist in addition to being a paediatrician. It also comes from being a primary care physician and knowing many of the children before they became ill. Furthermore, I do not feel that the symptoms pattern, which I see as being unique, as being consistent with a primary psychiatric disease. But I respect the dialogue with my psychiatric colleagues. Scientific dialogue should know no boundaries. But it should also be productive and result in improved care and support for the patients being discussed.
Despite nearly thirty years of attention on CFS, we really know very little about the condition in children. We do not know the cause or complications of the illness. The symptom presentation is subject to debate, particularly in children. The natural history of CFS is completely unknown with estimates ranging from it being a benign, self-limited illness to a persistent, progressive and disabling neurological condition. My own belief is that some children resolve the illness completely, some continue with mild to moderate symptoms, and some develop severe, perhaps lifelong illness.
The observation that some children with CFS have severe debilitating courses is not accepted by the medical profession, many of whom view the illness as trivial. In an attempt to expand this limited view of CFS, I would present five case histories of children who have had severe illness suggestive of CFS. It is not intended to imply that severe illness faces the majority of children with CFS, merely to describe the possibilities at this end of the clinical spectrum.
Case 1: OE
OE first became ill at the age of 13. Prior to her illness she has been a vigorous, healthy girl, an excellent student, and participated in numerous extra-curricular activities. On January 4th, 1984 she developed a fever to 102 and symptoms suggestive of bronchitis. Exhaustion was very severe from the day of onset. Her fever rose 105 for five days, then resolved, but she became febrile again to 105 for a further 8 days one week of slight improvement. She had been begun on antibiotics without effect. Fatigue and other symptoms persisted and she was unable to go to school. Her pattern of symptoms has been relatively constant, including persistent sore throat, occasional mouth ulcers, daily headache, inability to concentrate, feeling of mental "fogginess", severe abdominal pain, constant nausea, mild lymph node tenderness, diffuse muscle and joint pain, pallor with exertion, chest tightness and palpitations, light and noise sensitivity. Her condition has persisted without improvement for the next 14 years.
In August 1985 she had an episode of diffuse "shaking" for 45 minutes. There was no loss of consciousness, she had several other of these episodes in the next few months. In November 1985 she awoke from sleep with severe diffuse pain and nausea, She began to have jerking motions with her L arm "flying out and snapping back in"; soon in both arms, but L side more than R. She remained conscious but was unable to prevent the arm motions. Episode lasted 1/2 hour and subsided over several minutes. Repeated episodes occurred with parasthesiae for up to 2 hours prior to an episode. These seizure-like episodes have persisted, usually once or twice daily, and all studies have been negative; no treatment has been effective. While there is no change in consciousness, she feels faint after an episode.
By April 1987 she was unable to read simple material, and had occasional episodes of slurring of speech. She was confined to bed because of exhaustion, pain, and orthostatic intolerance. She was able to sit up to brush teeth only, and bathe with sponge bath. She developed paroxysmal atrial tachycardia with episodes up to 240 beats/minute. Her prodromal seizure symptoms were different, with tingling L hand but remaining conscious. She has had numerous extensive evaluations, most saying that the symptoms were psychosomatic, hypochondriasis or depression. Psychiatric examinations failed to find psychological or family abnormalities. One note records a description of her symptoms was precise, without a tendency to dramatise or exaggerate. There was no mood lability, hostility, or overt anxiety. I did not find her depressed, but saddened by her prolonged illness. The diagnosis of CFS was first made by Dr. Irena Brus in 1988.
Physical examination revealed fine tremor of R hand on several occasions, questionable ptosis of R eyelid. Extensive laboratory work-up over the years was essentially normal. The only positives were an elevated Raji cell assay, IL-2 receptor of 445 (nl 200-400), CD4/CD8 ratio of 0.93 with slight decrease of helper CD4.
The patient was unable to go to school from the onset of her illness. No diagnosis was made, and the school stated to the parents that "if there is no diagnosis, there is no illness". Therefore, within a few months of becoming ill, a PINS petition was filed (Person In Need of Supervision). The family retained counsel and appeared in court. However on a few occasions the child was unable to appear at the court hearings. She was found to be "truant" and the school district and court referred the matter to CPS (Child Protective Services) claiming that the parents were abusive in the form of "medical and educational neglect". The fact that at this point (4/84) the family had seen nearly twenty physicians was raised to show that the family was "doctor shopping." During the ongoing court action, the school district refused home tutoring, on the grounds that there was no illness present, and therefore unnecessary, even though the parents offered to pay for the tutor themselves. The child protective services argued that the patient should be removed from the home, placed in foster care, and returned to school, despite the fact that there were no abnormalities in family functioning found on numerous evaluations.
In 1987 at a final hearing after three years of ongoing court action, three physicians appeared before the court to testify that this young lady was medically ill and that there was no psychological or psychiatric disease present. Written expert psychiatric records and testimony were entered stating that there was no psychiatric disease. At that time the case was closed and the school system was ordered to begin home tutoring. The cost to the family from legal fees alone was in excess of $65,000 and no education was provided for over three years.
The difficulties experienced by this young lady have been experienced by many with CFS although the social complications are now better than they were in ten years ago. However, the continued lack of diagnostic criteria for children continues to force school systems to refer ill children for truancy and child abuse, an additional stress that is inappropriate.
Case 2: SP
SP was in good health until the age of 15 when he developed sinusitis, which did not respond to antibiotics. He had a progressive increase in fatigue, muscle and joint pain, abdominal pain with diarrhoea, light headedness, dizziness. One year after onset he was unable to stand or walk because of weakness although specific muscle strength testing was normal. He developed tremor and orthostatic intolerance to the degree that he was unable to sit up in bed. He developed severe gastrointestinal pain with absent intestinal motility and required tube feeding.
He was hospitalised and had extensive evaluations. Because of syncope on standing he was unable to stand and was transferred to a rehabilitation hospital where he remained for the next six months. During this time his maximum activity was to sit for 45 minutes. Extensive medical and psychiatric evaluations were unrewarding. Physical examination revealed pallor and complete absence of muscle contour. After six months of hospitalisation he was transferred to my office by ambulance for an evaluation without understanding that he was being discharged from the hospital. His insurance company then refused further medical or rehabilitation measures on the basis that they were not medically justified. Legal action was instituted but not resolved at the present time.
The hallmark of CFS is orthostatic intolerance. This youth had severe orthostatic intolerance to the degree that he was unable to sit for more than 45 minutes over a six-month period. Other symptoms such as abdominal pain, nausea, muscle and joint pain, disturbed thinking all present, but the activity is limited entirely by orthostatic intolerance, the inability to function in the upright position. After six months of hospitalisation the lack of objective medical evidence of physiologic or psychiatric disease lead to his having his insurance cancelled. He was subsequently found to have a positive tilt table test and reduced circulating blood volume to 76% of normal with reductions in both red blood cell mass (21.5 nL/Kg, normal = 23-27 mL/Kg) and plasma volume (33.7 mL/Kg, normal = 39-49mL/Kg) (6).
Case 3: DS
This young lady first became ill with an acute flu-like illness at the age of 11, which was remarkable for the severity of fatigue. She also had multi-joint pain, recurrent sore throat, abdominal pain with nausea and vomiting, light headedness, fainting, photophobia, flushing facial rash, frontal headaches. The symptoms persisted with severe fatigue and she was unable to go to school. Physical examination was generally unremarkable except for purple striae on her thighs and knees, which persisted for five months and gradually faded. Numerous laboratory evaluations were unremarkable, sedimentation rate was 2 mm/hr.
Positive immunological testing revealed intermittently elevated Interleukin -2 and mildly elevated Il-2 receptor at 570 (nl=200-400), and a minimally elevated Raji cell assay of 190 (nl=<5). Lymphocyte phenotype showed a CD4/CD8 ratio of 4.3 due to a mild decrease of CD8 lymphocytes (8% or 214/cu mm). Other testing including alpha interferon was normal.
She was treated symptomatically and remained out of school for three years. In September of 1990 she was improving and a limited school day was attempted. On her first day back in school she was teased by classmates and returned home where she took an overdose of medication including a family member's tricyclic antidepressants. She developed a ventricular arrhythmia and could not be resuscitated.
Suicide is clearly a complication of CFS, and its risk may be proportional to both the individual coping style of the patient and the amount of family and societal support. There have been no studies attempting to examine the incidence of suicide in CFS, as its presence establishes a diagnosis of depression. Clinically, I do not believe that the symptom pattern in this child, whom I followed closely for several years, was due to a primary psychiatric illness.
Case 4: AJ
AJ was born at 7 pounds after a pregnancy complicated by severe maternal migraines. His mother experienced symptoms suggestive of mild to moderate CFS for several years prior, and worsening of these symptoms during pregnancy. Delivery was uncomplicated but the child was irritable and cried on a nearly constant basis for the first year. He slept only six hours daily and was easily aroused, carrying a diagnosis of colic. At the age of two he was evaluated because of persistent fever to 101 but no specific cause was found. From age 2 1/2 to 3 he was relatively well, but then developed episodes of diarrhoea, fever to 103, joint pains, irritability and he stopped growing. He developed molluscum contageosum with nearly 100 lesions, but immunologic evaluation was unremarkable. At age 5 he developed headaches and worsening of joint pains without synovial thickening. He began to sleep frequently during the day and seemed lethargic; his growth returned to normal. Learning profiles were abnormal with attention deficit, poor short term recall both auditory and visual, but average to good intelligence. OT evaluation showed difficulties in space perception, tactile perception and poor motor skills particularly with midline crossing skills. He tired easily during testing. Diagnoses included juvenile rheumatoid arthritis, Crohn's disease, learning disability, emotional disorder, generalised allergies.
At the age of 7 he has generalised fatigue and sleeps four to five hours daily with episodes of near normal activity for up to several hours. He has continuous joint pain, muscle pain, headache, abdominal pain with diarrhoea and some constipation. Low grade fever, attention deficit and night sweats are also noted. Extensive medical evaluations have been unremarkable. Family and psychosocial evaluations have been unrewarding. His clinical condition at present is most consistent with the diagnosis of chronic fatigue syndrome.
This case is remarkable for several reasons, although unusual. In my experience, most children born to mothers with CFS have a normal childhood, and while there may be an increased incidence of CFS within families (7), the pattern experienced here is uncommon. The diagnosis of CFS is not possible in this child because he has had lifelong symptoms, a specific exclusion criteria by the CDC criteria.
Of particular concern is the relationship between chronic fatigue syndrome and learning disabilities. Adults with CFS and established learning patterns experience cognitive difficulties, and it should be no surprise that a child with CFS would have learning disabilities. If asked, this child would not complain of fatigue despite sleeping during much of the day; the term fatigue has little meaning as it has been a lifelong experience. The interrelationship between paediatric CFS, learning disabilities, and subgroups of JRA needs to be explored with specific research studies.
Case 5: SW
SW first became ill with a gradual onset of headache, muscle pain, frequent infections, and other symptoms at the age of 10. She developed hearing loss treated with hearing aids, the etiology of which was not ascertained. Three years after the hearing loss her hearing returned and she developed hyperaccusis. She continued with moderate symptoms, including fatigue until she developed mononucleosis at the age of 15, causing her to be bed-ridden for two months. For the next five years she had severe, disabling symptoms which caused persistent pain, and disrupted her education.
Among the worst symptoms was persistent nausea and abdominal pain. Because of persistent vomiting and a thirty pound weight loss, parenteral nutrition was started which stabilised her weight loss. She had complications with placement and infection of the catheter over two years. She was finally stabilised on jejunostomy tube feeding and TPN was weaned. Laboratory findings included an abnormal MRI scan with numerous high intensity lesions on T2 weighted images which were not felt to be consistent with multiple sclerosis. A mild elevation of spinal fluid protein to 65 was noted. Numerous psychiatric evaluations revealed no evidence for psychiatric disease.
Electrical studies of the gut revealed postprandial tachygastria, known to be correlated with delayed gastric emptying from ineffective contraction of the stomach. Antroduodenal manometry showed no spontaneous migration of motor complexes, which was reversed by somatostatin, leading to the diagnosis of gastroparesis without involvement of the small intestine. Symptoms of biliary dyskinesia were also present.
By May 96 at the age of 20 steady neurologic deterioration had occurred and she was bedridden with severe fatigue, pain, spasticity of the lower extremities, myoclonus, dysarthria, episodes of hearing loss. She developed a seizure disorder. Neurosurgery to correct a partial Arnold-Chiari malformation caused some improvement in neurologic symptoms but did not relieve the muscle and joint pain or the fatigue.
At the present time SW continues with severe symptoms. Despite these difficulties, she became pregnant which required restarting the TPN, complicated by numerous infections including infection of the TPN line. Oligohydramnios occurred but pregnancy continued. At 30 weeks, Cesarean section delivered a baby girl, and SW is a new mother.
The history of SW is typical for severe CFS in many regards. The onset began at the age of ten, and the symptoms were progressive, marked by episodes that clinically resembled mononucleosis. Severe CFS is associated with the same overall symptom pattern as the milder cases, but is more likely to have unusual neurologic symptoms, such as the hearing loss, seizures, and ataxia in this young lady.
Abdominal problems, such as irritable bowel syndrome, are common in most patients with CFS but are not recognised in the current diagnostic symptom criteria used in the United States (8). These symptoms are more prominent in children and adolescents with CFS, and are usually severe in those with the more severe courses. In this patient, the gastro-intestinal disturbances were well studied, and required treatment with both parenteral nutrition and tube feedings. Despite the many challenges of persistent illness, this young lady is facing her illness with great dignity and courage, deciding to experience the joys and challenges of parenthood.
It is remarkable that the five case histories described here, most and perhaps all would not be recognised as having chronic fatigue syndrome by current criteria. All have experienced not only severe symptoms, but severe social and educational hardships as a result of their illness. All have been accused of hypochondriasis and malingering at one time or another.
A central question which exists in the United States at the present time is whether it is appropriate to even make the diagnosis of CFS in children as this may obscure other medical and psychiatric diagnoses and encourage the development of an illness behaviour in children (9,10). The American Academy of Paediatrics, which has long defined its role as being an advocate for children, has remained silent on the issue.
At this time, it is crucial to begin approaching paediatric CFS with a systematic and scientific approach. Diagnostic criteria specific for children should be developed, along with guidelines to help educational management within school systems. The relationship between fatigue and learning. should be specifically addressed. Long term follow up studies should be begun along with adult studies to determine more accurately the incidence of progressive fatigue, arthralgias, irritable bowel and migraine during childhood. Most importantly, the medical community should take leadership in establishing dignified and compassionate care for children with chronic fatigue syndrome.
1. Krupp LB, Mendelson WB, Friedman R. An overview of chronic fatigue syndrome. J Clin Psychiatry 1991; 52: 403-10
2. Shafran S. The chronic fatigue syndrome. Amer J Med 1991; 90:730-739
3.. Klonoff DC. Chronic fatigue syndrome. Clin Infect Dis 1992; 15: 812-823
4. Bell DS. Chronic fatigue syndrome in children. Journal of Chronic Fatigue Syndrome 1995; 1: 9-33
5. Kulig JW. Chronic fatigue syndrome and fibromyalgia in adolescence. Adolescent Medicine:State of the Art Reviews 1991; 2: 473-484
6. Streeten DH, Bell DS. Blood volume and chronic fatigue syndrome. J Chronic Fatigue Syndrome in press.
7. Bell KM, Cookfair D, Bell DS, Reese P, Cooper L. Risk factors associated with chronic fatigue syndrome in a cluster of pediatric cases.Rev Inf Dis 1991; 13 (Suppl 1):S32-38
8. Fukuda K, Straus SE, Hickie I, Sharpe MC, Dobbins JG, Komaroff A, International Study Group. The chronic fatigue syndrome: a comprehensive approach to its definition and study. Ann Intern Med 1994; 121: 953-959
9. Jones JF. Chronic fatigue syndrome in adolescents - an opinion. J Chronic Fatigue Syndrome 1997; 3: 53-58 Plioplys AV. Chronic fatigue syndrome should not be diagnosed in children. Pediatrics 1997; 100: 270-271.
10. Plioplys AV. Chronic fatigue syndrome should not be diagnosed in children. Pediatrics 1997; 100: 270-271
Return to the AHMF Letter to the RACP.
Alison Hunter Memorial Foundation
PO Box 6132 North Sydney 2059 Australia
Phone/Fax +61 2 9958 6285
All material on the site © AHMF | Sitemap