* Of the estimated 240,000 people in the UK with chronic fatigue syndrome or myalgic encephalopathy (CFSME), an increasing number are children, some as young as five

* Alongside fatigue, children with the illness commonly report dizziness, lightheadedness, abdominal pain, rash, fever and chills

* One study suggests that half of all long-term school absence is attributable to CFSME

* Eighty per cent of those with the illness will fall behind in their studies or even drop out of education altogether

* Scepticism was rife until January 2002 when the chief medical officer recognised ME as ‘a genuine illness’

* In 2004 the Department of Health earmarked pound;8.5 million for specialist services to deal with the disease

* Only a small percentage of sufferers return to normal health

Main text: David Newnham Illustration: Thea Brine Additional research: Sarah Jenkins

It incapacitates nearly a quarter of a million people in the UK, yet nobody knows what causes it. There is no way of testing for it, or of knowing how long it will last. Medicines have no effect, and although it is not considered fatal, many sufferers never recover. It can affect men and women from all social and ethnic groups. And now it is blighting the lives of more children than ever before. No wonder newspapers and medical journals are referring to ME as the new plague.

How does it feel?

Imagine that you feel so utterly drained of energy that you can no more climb into bed unaided than scale Everest. Imagine that sleep, when it finally comes, leaves you feeling no fresher, and that you feel like this not once or twice, after a busy day or a stressful night, but frequently and without warning, for weeks, months and even years. Imagine also that your head aches and your muscles hurt, that you feel cold in summer and hot on a chilly day, that your skin crawls, that bright lights and loud noises are intolerable, that your concentration is poor and even your memory fails you.

Finally, after endless tests, your doctor tells you that there appears to be nothing the matter with you. And then the doubts, the suspicions, the accusations begin. Are you making it up? Are you lazy? Or are you simply losing your mind? For thousands of people, this is the reality of the illness that dominates their lives, a complex and variable medical condition known either as myalgic encephalopathy (ME) or chronic fatigue syndrome (CFS).

What’s in a name?

Almost every aspect of CFSME is the subject of debate, starting with the name. The term myalgic encephalopathy (myalgic relates to muscles and encephalo to the brain, while pathy means sickness) describes an illness that involves muscular pain and neurological problems. Until recently, however, ME stood for myalgic encephalomyelitis, because it was wrongly thought to involve brain inflammation. The term chronic fatigue syndrome, on the other hand, can include a spectrum of fatigue diseases, one of which is ME.

The compound name CFSME seems to cover all bases. But does it? Since around two-thirds of cases, and particularly those involving young people, are thought to be triggered by viral infections, many prefer the terms post-viral fatigue syndrome (PVFS) or post-infection fatigue syndrome (PIFS); another name, chronic fatigue immune dysfunction syndrome (CFIDS), reflects abnormalities in the immune system detected in some sufferers. In the United States, a working party charged with finding an acceptable name has proposed the cumbersome chronic neuroendocrine immune dysfunction syndrome; an earlier suggestion, neuroendocrine immune disorder, having proved unpopular.

CFSME researchers in the UK recently pointed out that “medicine is the victim of fashion like any other human endeavour, and the naming of diseases is not exempt”. But, in this case, the debate about a name does suggest a more fundamental uncertainty about the nature of the condition or conditions in question.

Royal Free Disease

Before you can name an illness, you must first define it, and pinning down a set of symptoms common to all forms of a variable and unpredictable condition is fraught with difficulties. In 1955, nearly 300 members of staff at the Royal Free Hospital in London went down with a disease whose symptoms included profound malaise, headache, fever, sore throat, nausea, muscular pains and dizziness.

While the mysterious complaint was popularly known as “Royal Free Disease”, the cause of the outbreak was officially considered to be an epidemic form of ME. In the 1970s, two psychiatrists who reassessed the incident dismissed the outbreak as a form of hysteria. Subsequent research, however, established that people with ME, including a few still suffering the long-term effects of the Royal Free and other similar outbreaks that occurred around the same time, showed evidence of persistent viral activity as well as muscular abnormalities.

In 1986, Melvin Ramsay, a physician at the Royal Free, published a “definitive description” of ME. Onset, he said, could be sudden and without apparent cause, although it was “practically always” preceded by upper respiratory tract or gastrointestinal tract infection. Instead of recovering from these in the normal way, patients suffered persistent and profound fatigue, accompanied by “a medley of symptoms” and “a general sense of feeling awful”.

Beyond ME

Current definitions of CFS centre on unexplained persistent or relapsing chronic fatigue that is not substantially alleviated by rest, is present for at least six months, and which results in substantial reduction in previous levels of activity. Alongside fatigue, a diagnosis of CFS requires a number of other symptoms to be present, perhaps including impairment of short-term memory or concentration, mood swings, sore throat, tender lymph nodes, muscle and joint pain, headaches, unrefreshing or disturbed sleep and post-exertional malaise lasting more than 24 hours.

Opinions vary about different permutations of symptoms and their possible significance. But, on one point, there is now more agreement than ever before. ME and other forms of CFS are not imaginary conditions. Those who suffer from them - and it has been suggested that Florence Nightingale herself may have been incapacitated by ME on her return from the Crimean War in 1857 - are not hysterical, nor are their symptoms “all in the mind”.

A genuine illness

January 11, 2002 was a historic day in the history of CFSME. Launching an official report into the illness, Sir Liam Donaldson, the chief medical officer of England and Wales, said: “Until now, sufferers were ignored or not taken seriously, labelled as hypochondriacs, or urged to pull themselves together and get better on their own. From today that changes.”

Here was a report that, for once, did not mince words.

The report opened with the unequivocal statement: “Chronic fatigue syndrome (CFSME) is a genuine illness and imposes a substantial burden on the health of the UK population.” It went on to acknowledge that “patients, their carers, and healthcare professionals encounter different levels and varying manifestations of disbelief and prejudice against people affected by the condition”. And it reprimanded those professionals whose own scepticism had “done nothing to dispel public disbelief”.

The biggest problem facing both sufferers and doctors is undoubtedly the lack of any definitive diagnosis or test for the disease. Despite this, said the report, it is now “clinically recognisable”, and any clinician lacking the wherewithal to recognise it should get some training, and fast.

Last year, the Department of Health announced pound;8.5 million of ring-fenced funding for new specialist services to deal with the disease.

But for thousands of sufferers, the greatest leap forward must have been that official recognition afforded to MECFS two years previously.

Recognition and validation are essential steps along the road to recovery.

And never more so than when a young person is involved.

Suffer the children

Of the estimated 240,000 people in the UK with CFSME, an increasing number are school-age children, some as young as five. It is reckoned that one child in every 1,000 has the illness in one form or another, which works out at one per UK secondary school. In fact, one study has suggested that 51 per cent of all long-term school absence is attributable to a form of CFS. While children’s symptoms are generally similar to those of adults, children more commonly report dizziness, lightheadedness, abdominal pain, rash, fever and chills. Based on a survey of 445 of its 1,800 members, the Association of Young People with ME (AYME) believes that 78 per cent of children with the illness are severely affected at some stage, and that 80 per cent will fall behind with their studies or even drop out of education altogether.

Before and after

In order to flesh out these grim statistics, researchers recently interviewed dozens of parents of children with CFSME, gleaning from them a number of distressing “before and after” snapshots. One mother, who described her daughter prior to the illness as “a happy girl - a giggly teenager who used to have an infectious laugh and had everybody in stitches”, spoke of the devastating changes that occurred. Her daughter became “bed-bound, totally paralysed... cannot swallow, cannot open her hands, cannot move her feet apart, cannot turn in bed, cannot turn her head, doubly incontinent - totally dependent”.

Another girl had been very active, and “always laid-back and outgoing”.

“She went through a stage where she felt really dizzy, as though on a boat... at the very beginning the rooms used to go totally black and she felt like she was going to faint, which was quite scary.” The outgoing daughter’s speech became slurred and she felt so sick “from morning to going to bed”

that she was barely able to eat.

Can’t attend... or won’t?

It is noticeable how many of the parents emphasise that their children were happy at school before their illness - a natural response, say the researchers, to the frequent and hurtful accusations of “school phobia” and over-protective parenting. Such disbelief, all too often coupled with professional hostility, compounds the impact of the illness on both child and family, according to the chief medical officer’s report. For this reason, it says, diagnosis in the young must be “especially prompt, accurate, and authoritative”. In the absence of a diagnostic test, the process should be one of assembling clues from the child’s history and from examination, while ruling out alternative explanations such as hypothyroidism, musculoskeletal, neurological or cardiac disorders, depression, eating disorders and child abuse. Such is the potential impact of CFSME on a child’s physical, emotional and intellectual development, says the report, that the timescale for identifying the problem and deciding how to deal with it must be minimal.

And it adds: “When a child or young person has suffered excessive tiredness andor other symptoms leading to fragmentary school attendance or absence for at least 15 working days, active steps should be undertaken to identify the cause from a list of diagnoses that includes CFSME.”

Pace yourself

It is so difficult to get a doctor to diagnose CFSME that sufferers or their parents frequently regard success as a matter for celebration. Only then does it hit them that diagnosis, while helpful, is not an end in itself, and that a cure might not be forthcoming in the foreseeable future.

Like most other aspects of the condition, the subject of how to treat it has attracted heated debate - a state of affairs that the ME Association believes will continue “until more information on what we’re having to deal with is forthcoming”.

In the meantime, treatment is often aimed at reducing symptoms and can encompass mainstream and complementary methods, ranging through pharmaceutical drugs, homeopathy, behavioural therapies, acupuncture and diet changes.

Perhaps the most successful and therefore the most popular technique, and one which is being trialled by the Medical Research Council, is called Pacing. AYME describes this as an energy management strategy aimed at preventing patients falling into a “boom and bust” vicious circle of over-activity and relapse. “Patients alternate activity with rest, and switch between physical and mental tasks so that they do not become exhausted. Patients learn to spot the warning signs of impending exhaustion and stop activity before that point.” The fact that, for severely affected patients, “activity” may mean just sitting up in bed for a few minutes, is a measure of how devastating CFSME can be.

Never the same again

According to the ME Association, only “a fairly small percentage” of people who suffer from CFSME manage to return to completely normal health, and the time taken for this recovery may vary widely, from months toa number of years. According to the charity, the majority of sufferers will continue to fluctuate between good and bad periods of health, with relapses often precipitated by infections, operations, temperature extremes or stressful events.

For a significant minority, the outlook is even bleaker. They remain severely affected, and continue to require a great deal of medical, social and practical support. On a brighter note, most studies suggest that for young people, the prognosis may be better than for adults. Many children, it seems, are able to recover from CFSME even after long periods.

When school’s out

Children with ME may only be able to attend school part-time, if at all, in which case it will be important that they maintain links with their friends and have the support of their teachers. The charity Action For ME advises parents to have an individual education programme drawn up in collaboration with their child’s school, the LEA and the child.

If a child is well enough, it may be recommended that they try to attend school, possibly for short periods. When that happens, there is a greater likelihood of their rejoining friends in the same year if they have kept up with some of their school work. Here, a home tutor can be useful, and this should be arranged through the LEA.

However, Action For ME warns parents that study times should be paced, as with any other activity. “The amount of time they can study should only be increased very gradually, and reduced during bad days or relapses.”

Testing times As with many other illnesses, it seems likely that increased understanding of the role played by genes offers the best hope of a cure. Indeed, the discovery earlier this year of differences in gene expression in the immune cells of people suffering from CFSME has all the hallmarks of a breakthrough. A research team headed by Jonathan Kerr at St George’s University of London compared levels of gene expression (the process by which a gene’s information is converted into the structures and functions of a cell) in the white blood cells of 25 healthy individuals with those of 25 patients with CFS. Differences were found in 35 of the 9,522 genes they analysed, and subsequent analysis established that 15 of the genes were up to four times as active in people with CFS, while one was less active.

Several of the genes in question are known to be involved with mitochondria, which power our cells, and this would fit in with the fact that CFSME patients lack energy. The findings also suggested ways in which the illness could be triggered by viral infections, and how the immune system might be involved. Dr Kerr told journalists that he hoped the research “will open the door to development of pharmacological interventions”. In the short term, the discovery of changes in blood protein levels resulting from differences in gene activity will almost certainly lead to a blood test for the disorder, removing once and for all the credibility gap that for too long has added insult to injury for sufferers of CFSME.

Resources

* The Association of Young People with ME (AYME) has a website at www.ayme.org.uk, and its helpline can be contacted from Monday to Friday, 10am to 2pm, on 08451 23 23 89.

* ME Association: www.meassociation.org.uk; its helpline (0870 444 1836 for non-members) is open every day, 10am-noon, 2-4pm and 7-9pm.

* The Action For ME website offers fact files, a list of available publications and an overview of current research. Go to www.afme.org.ukinfocentre.asp.

* To download a copy of the 2002 report to the chief medical officer, visit the Department of Health website at www.dh.gov.uk and search “CFSME”.