Benign Myalgic EncephalomyelitisME InternationalMyalgic EncephalomyelitisME International

Chronic fatigue syndrome

Page 3(of 4)

Clinical practice guidelines - 2002

Produced by a Working Group convened under the auspices of the Royal Australasian College of Physicians
This supplement to the MJA was funded by a grant from the Commonwealth Department of Health and Ageing.
MJA 6 May 2002 176 (9 Suppl): S17-S55

What is the role of sleep management?

People with CFS experience a range of changes in sleep.[77,112,328-331,487-491] The most common features are reduced sleep efficiency, longer sleep onset, increased awakenings during sleep, increased total time in bed, and disturbance of circadian rhythm. There is a growing view that sleep disturbance in patients with CFS may be part of a wider abnormality of sleep-wake cycle function, and that restoration of the normal sleep-wake cycle, with consequent normalisation of circadian rhythm, should be an important goal of therapy.

In patients with CFS, behavioural approaches to sleep-wake cycle disturbance are likely to be more successful than pharmacological approaches, as the latter do not induce normal sleep. Cognitive and educational management approaches should be aimed at promoting an understanding of the role of disordered sleep, and dispelling any irrational fears or inappropriate beliefs about sleep. Relaxation training and stress management may be useful for some patients.

Sleeping for longer does not appear to improve physical or mental functioning in patients with CFS, and excessive periods of daytime sleep or frequent napping serve only to further disrupt circadian rhythm. The aim of sleep management is to establish a regular, normalised sleep-wake pattern:

  • try to avoid excessive night-time sleep periods;
  • avoid going to bed too early in the evening;
  • avoid stimulants during the evening period;
  • wake at a regular time in the morning (eg, 7am);
  • get out of bed at a regular morning time (eg, by 8am);
  • reduce (to less than 30 minutes) or abolish daytime naps; and
  • engage in daytime physical and mental activities (within the limits of the individual's functional capacity).

While the recommendations above are generally considered helpful for promoting good sleep in a range of sleep disorders, direct evidence for their benefit in CFS is currently lacking. If a patient with CFS has a concurrent primary sleep disorder (eg, sleep apnoea, restless leg syndrome, narcolepsy), this requires specific intervention.

3.2: Treatments for chronic fatigue syndrome for which scientific evidence is lacking*
Vitamin and mineral supplementsPhysical therapies
Vitamin CMassage
Vitamin EColonic irrigation
Vitamin B6Cold baths
Vitamin B12Feldenkreis
Coenzyme Q10Aromatherapy
l-GlutamineOxygen therapy
MagnesiumHydrogen peroxide
ZincHerbal treatments
AcupunctureEchinacea
HomoeopathyGarlic
NaturopathyDietary restrictions
Chiropractic"Hypoglycaemic" diet
Tai chi"Anti-candida" diet
MeditationLow salicylate, low preservative diet

* Not an exhaustive list.
Perspectives

"Each new proposed treatment might just be the one to set things moving in the right direction. They stretch from the sublime to the ridiculous, but you must try them all lest you risk the 'Don't you want to recover?' question. These treatments aren't always benign, often leaving you physically worse off than when you started, not to mention emotionally and financially."

- a person with CFS

"So far none of the alternative medicines have any scientifically proven benefit for people with CFS, although some individuals do seem to benefit from particular treatments they try. We also know that people who are desperate to get well may be exploited by practitioners offering unproven treatments. If a practitioner is offering alternative treatments to people with CFS, we believe that it is essential that they are informed of the cost and risks of the treatment, as well as whether there is any published scientific evidence to support its use."

- a patient support group

Unproven therapies in CFS

Given the variable clinical course of CFS, the likelihood of spontaneous improvement and the possibility of non-specific (placebo) responses, properly controlled clinical trials are essential for the evaluation of all proposed new treatments[153] (see 3.2). Any claim that a particular treatment can "cure" most people with CFS should be regarded with a high degree of scepticism, not least because patient cohorts in CFS treatment trials are generally heterogeneous[16,113] and hence unlikely to respond in a uniform fashion. It should be borne in mind that in some controlled trials over 30% of people with CFS may show improvement in the non-specific (placebo) treatment arm 153,450])

In general, evaluation of proposed new treatments for people with CFS requires:
  • a plausible scientific rationale for the agent or treatment method to be tested, and preliminary findings showing safety and potential efficacy (phase I data); and
  • extension of clinical studies beyond short-term, anecdotal or case-series approaches to randomised controlled trials that evaluate long-term treatment outcomes.[490,491]
The validity of the results of clinical trials is highly dependent on the quality of study design and analysis.[492,493] Critical methodological requirements are:
  • use of an internationally accepted case definition;[6,32]
  • random assignment to test or comparison groups;
  • adequate sample size;
  • use of well-characterised outcome measures494 and standardised self-report instruments for measuring fatigue, mood and other key symptoms;[32]
  • independent, blinded assessments of functional status at onset, completion of treatment, and three to six months later (to ensure durability of the treatment effect); and
  • reporting of refusal and drop-out rates, and of the type and frequency of adverse side effects.

Even with well-designed trials, positive results should be independently replicated[495] before a new treatment is widely promoted to the general public. The use of essential fatty acids for CFS is a case in point.[496] Doctors are encouraged to discuss frankly issues about evidence - including what is known and what is not known - to ensure that patients are able to make informed decisions about treatment.

4: CFS in children and adolescents

CFS in young people
  • CFS can occur in children and adolescents (Level I).
  • Clinical improvement is reported frequently in adolescents with CFS, with return to normal functioning over time in a significant proportion (Level III-2).
  • An individualised management plan should be developed in partnership with the young person and their family (Level IV).
  • The special needs of young people - social, educational and emotional - should be given high priority (Level IV).
Perspectives

"So often I am told I don't look sick. Most of the time now I don't tell people I'm sick when I go out. When you're in a support group; however, it's good not having to continually justify yourself. I've formed many strong friendships through my support group which I think will far outlive my battle with CFS."

- a young person with CFS

Prevalence

The prevalence of CFS in adolescents in Australia is not known, although in a prevalence study by Lloyd et al[30] 20% of those diagnosed with CFS were in the 10-19-years age range. In the United States, prevalence estimates among adolescents reported by Marshall[160] depended on the method of case acquisition. These ranged from 116 per 100 000 (telephone interview of CFS-like illness in San Francisco) to 22-26 per 100 000 (school nurses reporting diagnosis, Wichita, Reno) and 3 per 100 000 (physician surveillance: referred cases in four centres).[497] All socioeconomic groups are represented and females comprise 71%-74% of the total.[160,162,454,498]

Onset

The onset of the illness in children is typically reported to be abrupt, following a suspected or confirmed viral illness in 85%.[498] A seasonal variation with peak onset in autumn and winter has been reported in Australia, suggesting an infectious contribution.(498) Epstein-Barr virus (EBV) was the most common infection to be documented serologically at the onset of fatigue symptoms (12% of 290 cases); others were cytomegalovirus, Ross River virus, toxoplasmosis, chickenpox and Mycoplasma. Findings are similar in the US, with most adolescents reporting fatigue following a viral illness, most commonly EBV.[160,499]

Symptoms

An Australian series of 290 adolescents with CFS from a tertiary referral practice showed a clinical picture highly consistent with that characterised in adults. Prolonged fatigue after physical activity was present in all individuals. Headache, loss of ability to concentrate, the excessive need for sleep, excessive fatigue and myalgia following minor activity, nausea, abdominal pain, sore throat without coryza, and a feeling of disturbed balance were present in 87%, with symptoms rated as severe or moderately severe in over 50%. Feelings of depression and sadness were present in more than 50%. Twenty per cent of individuals met diagnostic criteria for fibromyalgia, a similar figure to that reported in other series.[157,158,500-502] The course of the illness also varies. About two-thirds of individuals report continuous symptoms with fluctuating levels of severity, and 15% have a relapsing-and-remitting course.[159]

Differential diagnosis in young people

The range of conditions that need to be excluded is less extensive than for adults. Recommended investigations are identical to those in adults, including a urinalysis, a full blood count, erythrocyte sedimentation rate, biochemical tests of kidney and liver function, and thyroid function tests.

Systemic lupus erythematosus (SLE) and other connective tissue disorders may present with lethargy and musculo-skeletal symptoms. Inflammatory bowel disease, coeliac disease, and gastrointestinal infection (eg, giardiasis) should be considered if there is abdominal pain, altered bowel habit or weight loss. A diet history should form part of the routine assessment. Occasionally, restrictive diets that are implemented in a search for alternative therapies have resulted in significant weight loss. Significant weight fluctuations, cessation of menses, altered body image and abnormal eating behaviour should raise the question of an eating disorder.

Adolescents with CFS often feel miserable, frustrated and angry, particularly after several months of illness. However, young people readily differentiate between feeling miserable meaning "fed-up" from miserable meaning "life is not worth living". Some groups report a higher rate of depression in young people with CFS compared with other chronic illnesses,(160) but the sample sizes were small and such series are susceptible to selection bias. When somatic symptoms characteristic of CFS are excluded from the commonly used depression scales, only a small proportion have major depression with anhedonia (7%). The rate is higher in adolescents with more severe CFS, particularly when there is a gradual onset and delay in diagnosis.

Anxiety about returning to the school situation is common in children and adolescents with CFS.[503] If extreme, however, other conditions that can mimic or complicate CFS should be considered (eg, depression, eating disorders, school refusal syndromes and, rarely, child abuse).[448]

Prognosis

Clinical improvement with return to normal functioning is frequently reported in adolescents with CFS, although the longest follow-up studies indicate that a small proportion remain unwell. The average duration of illness is 2-4 years with a range of 1-9 years until well.(159-163) Illness pattern varies - about two-thirds have continuous symptoms with fluctuating levels of severity, and 15% have a relapsing-and- remitting course.[159]

One follow-up study reported that improved functioning rather than return to completely normal health was a relatively common outcome after implementation of a structured management program.[162] This program consisted of educational support, graduated exercise, symptom relief, social contact and guidance on planned energy use. After an average of three years (range, 1-6 years) 30% were back to normal, and an additional 20% had mild symptoms following vigorous exercise, but with an otherwise normal activity level. Another 20% were functioning at less than 50% of their previous level of activity, including participation in school or work. Greater morbidity was linked with delay in diagnosis and in receiving assistance.

Management

For young people, CFS often poses special problems that relate to their development.[504,505] Some problems are specific to CFS, while others relate to the effect of chronic illness on the emotional and social aspects of adolescent development, examples of which are:

  • learning to become autonomous;
  • developing a sense of body image;
  • understanding and developing relationships;
  • making career plans;
  • dealing with sexual drives; and
  • developing value systems.

Loss of time from school, reduced stamina for writing, and difficulty concentrating (being slower to do things and being only able to concentrate for short periods of time) all contribute to significant educational disruption. Perhaps the most significant effect on schooling, however, relates to the loss of social contacts and access to social learning that plays such a large part in school life.

A management plan should be developed in partnership with the young person and his or her family.[448] The individual's illness pattern and severity should be taken into account when designing an individualised program. Although few randomised treatment studies have been performed in children, several strategies have been proposed as helpful.[161-163,503,506,507]

  • Symptom management should include treatment of headache, sleep disturbance, nausea, abdominal pain and dysmenorrhea, and muscle aches and pains.
  • Depression and anxiety symptoms should be recognised and treated.
  • Activities should be undertaken in a "paced" fashion and planned over a weekly period. The young person with CFS should be encouraged to balance social, physical and intellectual activities, and to make a commitment to undertake segments of each component regularly. This allows the individual to regain some control over their life. A gradual increase in physical activity with school attendance, or a graduated exercise program at home, can be incorporated into an overall weekly plan.
  • Maintaining social contact should also be given high priority. This may be through school or via extracurricular activities. Contact with support groups can also be helpful.
  • Liaison with the school is essential in order to design an education program involving attendance for particular subjects, or organising some school work by distance education with incorporation of social contact. Early planning and implementation of an educational program is desirable (ie, within 4-6 weeks of onset).

Once clinical improvement occurs, a "tailor-made" program for returning to school can be instituted. Returning to school can be anxiety provoking and stressful for young people with CFS because of remarks made by peers or teachers, as well as the resumption of physical, intellectual and social activities. A loss of confidence in social skills and intellectual ability is commonly reported.

Perspectives

"Support groups for adolescents and young adults with CFS have proved to be a great success wherever they have been established. They demonstrate the value of, and need for, social interaction with others in the same situation. People with CFS are no different from people with other chronic illnesses in this respect." - a patient support group

"One consequence of being chronically ill for years at a time is the isolation. As much as you try, it is very hard to keep up the old friendships from school, work and uni. People move on, but I have not been able to go out and socialise like before." - a person with CFS

Is referral to a specialist paediatrician necessary?

In a survey of adolescents with CFS, general practitioners were considered to be most helpful when they recognised the illness, acknowledged its effects, provided ongoing support, and monitored progress.[161,162] Specialist paediatricians were found to be helpful in:

  • confirming the diagnosis;
  • formulating and coordinating a plan of management;
  • providing suggestions for symptom management; and
  • providing documentation for and referral to education authorities (to arrange distance education, special consideration and special provision for Year 12 assessments, etc).

5: Social and legal issues

Implications of diagnosis
  • Making a diagnosis of CFS encourages appropriate treatment planning (Level IV).
  • A diagnosis of CFS does not establish a specific aetiology (Level I).
Perspectives

"Health professionals find it easier to label patients with depression, rather than recognise and acknowledge the natural grief reaction to the profound losses which occur with CFS - loss of health, disrupted family life, interrupted education and career, low self-esteem, etcetera. You can't dispense antigrief pills."

- a person with CFS

Diagnostic labelling of patients with fatigue

In the general population, fatigue states form a continuum in terms of severity and duration, and it is only in those with the most severe and persistent symptoms that a diagnosis of 'CFS' may be appropriate. Although the internationally accepted CFS case definition remains the "gold standard" for diagnosis, it is necessarily arbitrary, having been developed for the purpose of making valid comparisons between research studies carried out in different settings. As such, it creates an artificial boundary within the clinical continuum of fatigue states, giving the false impression that a specific clinical "entity" has thereby been delineated.[18,29,46,508]

In the absence of a clear understanding of aetiology and pathogenesis, the term CFS should be regarded as a descriptive label only.[46,508] Diagnostic boundaries are further blurred by the clinical overlap with other conditions such as fibromyalgia, irritable bowel syndrome, neurasthenia, anxiety and depression, in each of which fatigue can occur as a major symptom.[508] In each person with chronic fatigue the doctor must exercise clinical judgement in deciding whether CFS is an appropriate diagnostic label.[46]

What are the benefits of making a diagnosis of CFS?

A formal diagnosis of CFS can have positive implications for both the patient and the doctor. It allows the doctor to approach the patient with a greater degree of confidence, to explain the nature of the problem, to outline what treatments are appropriate, and to give a considered opinion as to what the outcome might be. Making a diagnosis of CFS should also mark the end of investigations to exclude other causes of illness (except at annual review - see Box 2.2).

From the patient's perspective, having a definitive diagnosis can go a long way towards relieving unwarranted fears and anxieties about the cause of symptoms.[510,511] Importantly, also, it validates the patient's experience of illness and suffering, making it easier to inform others of the nature of the illness, and legitimising the patient's entry into medical care. Once a patient has engaged with the doctor in this process, a series of personal, social and legal obligations result.[512,513] Family members, friends and employers can be expected to make appropriate allowances, and all concerned can be encouraged to make constructive contributions to the management plan. In the long term, this can help minimise morbidity.[451]

What are the disadvantages of a diagnosis of CFS?

As with many other chronic disorders, media reports, popular books and fundraising campaigns generally focus on the more extreme and dramatic end of the severity spectrum in CFS. In consequence, for the majority who are not so severely affected a diagnosis of CFS may conjure up alarming images of being confined to bed or a wheel chair, and life-long invalidism. In most cases, careful discussion with a knowledgeable practitioner can dispel such illusions, but some patients still harbour gloomy thoughts of a bleak future, with shattered dreams of family and career prospects.

The concern of some that "medicalisation" associated with providing a diagnostic label of CFS may create a self-fulfilling prophecy[514] is not usually borne out by experience.[510,511] In certain patients, however, the practitioner may consider it prudent to refrain from making a definitive diagnosis of CFS, or at least to be much more circumspect in applying the label. Thus, when the prognostic features are favourable (ie, younger age, less severe symptoms, shorter duration of illness) a more non-committal diagnosis, such as "post-infectious fatigue state", may be appropriate.

Importance of the doctor-patient relationship

Doctors who display the essential therapeutic characteristics of empathy, acceptance of their patient's suffering, non-judgemental style and a commitment to continued care are more likely to make an appropriate diagnosis[515] and to minimise the adverse effects of the illness experience.[452,513] Conversely, those who reject the patient's illness experience are likely to promote feelings of alienation and perpetuate ill health.[516] A qualitative study of people with CFS found that lack of perceived medical support and understanding was associated with increased seeking of alternative medicine.[517] Rejection by family, friends, peers and doctors leads many to experience CFS as a "delegitimising" illness.[518] Dismissing a patient's suffering as non-existent or imaginary is anti-therapeutic.[28]

Broaching the issue of psychological factors in causation should be done with caution and sensitivity, avoiding stereotypic value judgements. The hypothesised role of "somatisation"[110] is particularly problematic.[111] Outdated and simplistic notions of "psychogenesis", with their implications of "imaginary" illness and "unconscious malingering", leave patients feeling stigmatised, guilty and resentful. Pejorative terms reflecting a false dichotomy between "organic" and "functional" disease[519,520] are best avoided.

Unwarranted speculation about psychogenesis, based on the outcome of trials of cognitive behavioural therapy in CFS, should also be avoided. This is only likely to further alienate patients and cause resistance to potentially beneficial management strategies. If an effective therapeutic relationship is to develop, doctors must acknowledge that, despite the current lack of understanding of the underlying cause and mechanisms of chronic fatigue, the symptoms are real and the suffering and associated disability is genuine.(448)

Perspectives

"Currently, community services in Australia serve people with CFS, their families and carers very poorly. Services and support for people with other chronic and serious illness are generally provided without the ambivalence, relative ignorance and generally negative attitudes with which the support is provided to people with CFS, their families and carers."

- a patient support group

The role of patient support groups

Support groups have gained prominence in many areas of medicine, including in CFS, and they fill an important gap in areas that have traditionally been poorly catered for within the healthcare system. For individuals and families, they provide an opportunity to share experiences and exchange ideas on coping with practical day-to-day difficulties; they disseminate information on availability and quality of medical and government services, and news of research and treatment advances; and they can offer welcome relief from the sense of isolation that some patients feel. Support groups also have a more general advocacy role, for example in lobbying government agencies to improve funding for patient services, in fund-raising for ancillary services and research, and in promoting community awareness of the plight of sufferers (Box C).

Not all aspects of CFS support groups are necessarily positive. Inevitably, they tend to attract patients with the greatest functional impairment,[146] and this may inadvertently reinforce stereotypes of chronicity, disability and dependency. Moreover, the quality of advice can vary within and between groups, so it is important for practitioners to have ongoing knowledge of the activities and attitudes of local support groups.

Whenever possible, doctors should seek to work cooperatively with support groups. If effective dialogue is to be established and maintained, professionals must be sensitive to the concerns of patient groups, particularly in relation to the inappropriate use of pejorative and stigmatising terms.[521] Arrogant and dismissive professional attitudes, amplified by polarised press coverage, can contribute to the alienation of patients from traditional medicine.[517,522] Poor communication can also perpetuate misconceptions about aetiology, natural history and treatment rationales, which may themselves contribute to disability.[48,110,514,523]

Occupational issues

Many people with CFS struggle to continue working, despite their chronic illness, for reasons such as self-fulfilment, social identity, or economic necessity. The doctor can provide support by appreciating the specific difficulties experienced by CFS sufferers, and suggesting appropriate coping strategies. Limited energy, cognitive impairment, and memory lapses can impair work effectiveness, placing jobs in jeopardy. Arranging flexibility, prioritising work and compensating for deficits are commonly adopted mitigating strategies.[524]

Unpredictability resulting from the fluctuating nature of fatigue symptoms[525] is a significant problem in conforming to a work routine. Flexibility can often be negotiated in the form of shorter hours or a shorter working week, a variable work schedule with breaks for rest as needed, or discretionary task selection to match variations in capacity.[524]

Many patients choose to stop working, or are unable to continue, either temporarily or permanently. Practitioners should be supportive in helping patients make the most appropriate choices in relation to their own personal priorities. Those for whom "life is career", and whose struggle to keep working is proving unsuccessful, may become deeply depressed, whereas those who see the maintenance of family and social life as a higher priority may find giving up work a more rational and satisfying choice.[524]

Perspectives

"People with CFS seeking financial support from superannuation funds often experience drawn out applications, ill-informed and hostile review panels, further medical tests, lack of consultation with the treating doctor and the need to resort to legal action in an effort to obtain some financial support. This puts people with CFS under significant stress and may impede recovery."

- a patient support group

Medicolegal issues

Assessing a person with CFS for medicolegal purposes can be highly complex, and should be performed by a suitably qualified and experienced specialist. The role of the general practitioner is to provide factual information, such as details of consultations and referrals, investigations performed and treatment recommended.

In verifying a diagnosis of CFS, the current international diagnostic criteria (Box A) should be applied, including documentation of the characteristic symptoms, the lack of abnormalities on physical examination and results of the recommended laboratory investigations. A psychiatric evaluation may be indicated to document any psychological comorbidity.

Forming an opinion about the level of disability is a usual requirement in medicolegal assessment. Since CFS is a subjective illness, initial evaluation relies on a systematic review of the patient's self-reported functional capacity and an assessment of whether this is accurate. Corroborating information may be obtained from a partner or other family member, and from other practitioners with detailed knowledge of the patient.

A doctor acting as an assessor or expert witness may be asked to provide an opinion on causation. Uncertainties regarding the aetiology and pathogenesis of CFS should be acknowledged, and conclusions about the role of infection, chemical exposure or the emotional demands of the workplace should be appropriately tentative unless the clinical evidence is clear-cut and compelling.

Opinions about prognosis should be based on the known natural history, taking account of the duration, clinical course and severity of the individual's illness to date, and his or her progress in response to appropriate symptomatic and behavioural management measures. The notion of "permanent" disability is problematic, as most people with CFS improve gradually, and some eventually recover. In people who have been severely disabled and unable to work for more than five years, the probability of substantial improvement within 10 years is less than 10%-20%. This may be regarded as "permanent disability" for medicolegal purposes.

In the absence of evidence of malingering, speculative judgements about unconscious motivation should be avoided. The psychoanalytic concept of "secondary gain" has been misused in medicolegal settings and does not rest on a solid empirical base.[526,527] In evaluating patients with CFS, hypothesised secondary gains should be weighed against manifest secondary losses. The notion of "abnormal illness behaviour" is contentious,[528] and the term should not be used as a diagnostic label.

Acknowledgements

We thank the following individuals: Andrew Lloyd, Ian Hickie and Cristina Ricci for their comprehensive review of the published literature and preparation of preliminary draft 1997; Helen Lapsley (former chair of the Quality of Care and Health Outcomes Standing Committee of the National Health and Medical Research Council) for advice on guideline development and assessment of levels of evidence; Craig Ellis (Consumer Health Forum representative) for preparation of the Compilation of Submissions document and A CFS Health Consumer Perspective document; with assistance at various stages from Dr Joan Rothery, Annella Wheatley (typing), Jim Oakley, Judith Lovett, Bernhard Leidtke, Diana Clifton and members of the Launceston CFS support group; the Commonwealth Department of Health and Ageing for providing the federal grant; and Victoria Toulkidis for managing the project.

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