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Chronic Fatigue Syndrome in children and young people
OCCASIONAL REVIEW
Chronic Fatigue Syndrome in children and young people
This in turn frequently contributes to a poor patient/carer experience with delay in diagnosis and not infrequent hostility. This is unfortunate as CFS is a serious illness causing significant school absence and has long-term consequences for the educational, social and psychological development of the child or young person if there is no appropriate intervention. It also has an impact on family function and in some instances may result in financial hardship. As specialist provision is patchy it is important that any professional working with children and young people with CFS has a thorough understanding of the condition and its management.
Carrie Mackenzie Alison Wray
Abstract Chronic Fatigue Syndrome/myalgic encephalomyopathy (CFS/ME) is a relatively common and serious condition in children and young people, having a significant impact on their physical, emotional and cognitive well being. Although some progress has been made in understanding this perplexing condition there is still a long way to go and controversies persist about terminology, aetiology and treatment. However, there is a general agreement that CFS is a heterogeneous condition and good evidence regarding management. This review explores the best practice approach to assessment, diagnosis and management of CFS/ME in children and young people. Early diagnosis and appropriate multidisciplinary intervention facilitate recovery. There remains a pressing need for research to improve understanding of the condition and thereby ensure all children and young people with CFS/ME are understood and receive effective treatment.
Definition The fact that the terminology associated with this condition has created such controversy over a number of years indicates the difficulties inherent in labelling such a heterogeneous group of individuals who present with a wide range of physical and emotional symptoms. However the persisting and disabling fatigue universal in this group of children and young people has lead to our team favouring the use of the term Chronic Fatigue Syndrome (CFS). We believe this reduces confusion and assists in acceptance of the diagnosis when used in conjunction with a sympathetic explanation of the very variable nature of the condition which includes both physical and psychological symptoms. The term favoured and adopted by the Royal College of Paediatrics and Child Health (RCPCH) is CFS/ME but in our experience the term ME carries with it a much more physical or medical basis which patients and carers often confuse with conditions such as Multiple Sclerosis (MS). Moreover there are still those who believe that CFS and ME are two separate and distinct entities which share features with other conditions such as “Medically Unexplained Symptoms” (MUS) or other functional somatic disorders and overlap with chronic pain syndromes. In making the diagnosis, it is crucial to also “demedicalize” the condition, drawing a line under all that has gone before in terms of investigating the constellation of presenting symptoms and reaching agreement with the affected child or young person and their carers that the way forward is by means of rehabilitation from the point they now find themselves at. Indeed if this does not happen it may prove difficult to engage the child and family fully in a treatment programme (Table 1).
Keywords Chronic Fatigue Syndrome; myalgic encphalopathy
Introduction Chronic Fatigue Syndrome (CFS) or Myalgic Encephalomyopathy (ME) is a relatively common and serious condition affecting between 0.1% and 2% of children and young people aged under 18. It received public recognition as a specific clinical condition in 2002 and understanding of the condition is slowly improving. However, it is a heterogeneous condition with no diagnostic tests so diagnosis can be a complex task, particularly as the child or adolescent can present with a range of signs and symptoms. The presentation and course of the condition is often seen to ebb and flow further exacerbating the difficulty in predicting, understanding and managing symptoms. Although NICE guidance recommends referral to a paediatrician after 6 weeks of symptoms, the time from onset of symptoms to initial consultation with an appropriate professional is extremely variable and for some children and young people never takes place. Inevitably the lack of positive investigative findings coupled with the on-going debate about the pathophysiological basis of the condition leads to considerable and confusing debate amongst professionals and lay groups alike.
Epidemiology Prevalence data for CFS are heterogeneous and confusing as a result of different study methodologies (e.g. settings and diagnostic criteria) and different age limits. A number of studies report prevalence rates from 0.1% to 2% of children under 18. Most studies focus on the condition in adolescents: those reporting prevalence rates in younger children show it to be markedly lower. There remains a distinct need for further research to truly understand the prevalence of CFS in the child and adolescent population. The evidence on gender remains inconclusive. Some studies report no significant gender difference whereas others report a female excess of 2:1. Our own figures based on 176 children and young people under 16 show a ratio of two boys to every three girls.
Carrie Mackenzie MBchB FRCPCH DCCH MD is Consultant Paediatrician in the Children’s Hospital, Sheffield, UK. Conflicts of interest: none declared. Alison Wray BA MPhil CPsychol is Principal Clinical Psychologist in the Dept of Clinical Psychology at the Children’s Hospital, Sheffield, UK. Conflicts of interest: none declared.
PAEDIATRICS AND CHILD HEALTH 23:1
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Ó 2012 Elsevier Ltd. All rights reserved.
OCCASIONAL REVIEW
history and often normal examination findings are key in making the diagnosis (see Table 2).
Definition of CFS in children and young people C
C
NICE guidance e CFS/ME involves a complex range of symptoms that includes fatigue, malaise, headaches, sleep disturbance, difficulties with concentration and muscle pain. The pattern and intensity of symptoms vary between people and during the course of each person’s illness. RCPCH e generalized fatigue persisting after routine tests and investigations have failed to identify an obvious underlying ‘cause’. The fatigue is likely to be associated with other ‘classical’ symptoms such as difficulty in concentrating and disturbed sleep patterns and is classically exacerbated by effort (both mental and physical).
Investigations As a minimum we require that the child or young person will have undergone the screening investigations set out in the NICE guidance and that the results of these tests are normal when seen for initial assessment by the specialist CFS team. Diagnosis marks the start of a long and challenging journey for the child or young person and their carers. It is important to demedicalize and demystify the condition and positively and confidently recognize the symptom clusters shared by CFS sufferers. A further key feature of the initial consultation is to explain the nature of the condition, acknowledge the difficulties they have encountered on their journey thus far and reassure the patients and their carers that the expectation is that their condition will improve and that in our experience the vast majority of children and young people with CFS recover and go on to achieve academic qualifications. 99% of young people aged 16 seen by our specialist team achieved some academic qualifications and were able to progress to the next stage of their education or training. The vast majority of children and young people will not require transition to adult CFS services. About 5% continue to have a persisting and disabling illness.
Table 1
Pathology and pathogenesis There has been a plethora of research into CFS but the very wide and varied nature of the hypotheses being pursued indicates the clear lack of understanding of the pathophysiological basis for this condition. Many of our children and young people can describe an intercurrent illness immediately prior to the onset of their fatigue but the exact nature of this usually relatively minor illness is often unclear and it is by no means a universal precursor. It may well be that some external “trigger” provokes an autoimmune process in genetically predisposed individuals but the evidence for this does not yet exist and it seems likely that the causation is multi-factorial. Various aetiological factors such as level of exercise taken, personal and maternal psychological well being, gender, socioeconomic status, birth weight, birth order, coexistence of atopic conditions, exposure to certain viruses, school attendance and achievement have all been considered but as yet their contribution, if any, remains unclear. Any hope of intervention to prevent CFS in future generations will demand a better understanding of the aetiological factors at play. However the once popular stereotypical picture of the affected individual being female, highly intelligent and high achieving is not borne out by our experience.
“I visited the clinic and was diagnosed with CFS. It was a relief to know that my debilitating symptoms had a name. After more than 9 months of feeling so terrible someone could finally tell me what was wrong.” “It helped when I knew I had CFS. It was far better knowing than being left worrying or trying to guess.” The fact that CFS is a heterogeneous group is now generally agreed and evidence is growing to support this. One study has identified three phenotypes in CFS in children that are differentially associated with severity; the musculoskeletal phenotype is
Diagnosis and differential diagnosis
Investigations
The diagnosis of CFS relies not only on the exclusion of other pathology by means of appropriately directed investigations but also on the presentation of symptom clusters shared by other children and young people with the condition in addition to persistent and disabling fatigue. These symptoms may classically include: malaise, sleep disorder, headache, dizziness, poor temperature control, abdominal pain, nausea, anorexia, sore throat, tender lymphadenopathy, arthralgia, myalgia and cognitive difficulties such as poor short-term memory, impaired concentration, anxiety and low mood. Since many of these symptoms overlap with diagnoses relevant to many disciplines such as: Gastroenterology, Rheumatology, Endocrinology, Neurology, Infectious Diseases and Immunology, Oncology and Haematology as well as Childhood and Adolescent Mental Health it is beholden upon us to ensure that appropriate paediatric subspecialists have been consulted and the necessary investigations undertaken. However in the absence of any other disease, the
PAEDIATRICS AND CHILD HEALTH 23:1
These tests should usually be done: C Urinalysis for protein, blood and glucose C Full blood count C Urea and electrolytes C Liver function C Thyroid function C Erythrocyte sedimentation rate or plasma viscosity C C-reactive protein C Random blood glucose C Serum creatinine C Screening blood tests for gluten sensitivity C Serum calcium C Creatine kinase C Serum ferritin Table 2
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Ó 2012 Elsevier Ltd. All rights reserved.
OCCASIONAL REVIEW
associated with muscle and joint pain and these children have worse fatigue. The migraine phenotype which is associated with headache, abdominal pain, nausea and dizziness and noise sensitivity is associated with a lower level of physical function, worse pain and lower school attendance. The sore throat phenotype is associated with sore throat and tender lymph nodes and is the least severe.
learn to self manage the condition. At the outset of treatment this commonly involves three aspects: Sleep hygiene Activity/energy management School liaison In the absence of a clear understanding of the cause of CFS we make use of a number of analogies which ‘fit’ well with patients’ experience of the condition. We ‘explain’ to the child that their body is like a battery that has lost its charge and now requires recharging. The ways to do this are good sleep hygiene and activity management or GET. We also find that the idea of vicious cycles arising as a consequence of the condition is a useful concept with which families can identify (Figure 1).
Impact of the condition “It is one of the loneliest illnesses in the world because we don’t have anything to show for it.” Everyone involved with CFS agree that the condition has a profound impact, not only on the child or young person, but also on their families. It is associated with considerable school absence, social isolation, loss, changes in family relationships and parental time off work.
Sleep hygiene Disordered sleep patterns are commonly found in CFS including day/night reversal, interrupted sleep, insomnia or hypersomnia. It is therefore important to establish effective and positive sleep routines which include: Establishing a regular waking time Avoiding prolonged sleep and day time sleeps Always sleep at night in own bedroom and avoid use of computer/TV prior to bed Relaxation to aid falling asleep and consistent bedtime routine Melatonin and/or amitriptyline are used in some case to support good sleep hygiene and improve the quality of sleep.
“My symptoms meant that I started to miss a lot of school e I just didn’t have the energy to make it through the day.” “My daughter lost all her spark.”
Management Once children and young people and their families have gained an understanding of the condition, the basis of treatment and an optimistically realistic expectation of what the future might hold, a co-ordinated multi-disciplinary approach to management is required in the majority of cases. Commonly, occupational therapist, physiotherapist, nurse specialist and clinical psychologist may need to be involved. Evidence based guidelines recommend the following:
Activity/energy management The young person needs to keep a diary to establish their baseline of activity. Activity meaning everything that they do from having a shower being on the computer to going to school. Realistic goals then need to be agreed with the young person so
Activity management: this is a person centred, goal directed approach to managing a child or young person’s symptoms. It uses analysis of activity and graded activity through learning the skills of pacing to improve physical and cognitive function. Cognitive behaviour therapy: this is an individualized psychological therapy which incorporates two major components: the cognitive element which focuses on the identification and modification of thoughts, beliefs and assumptions which may shape the child or young person’s understanding of their condition, and the behavioural element which aims to gradually and consistently introduce a change in behaviour e.g. an increase in activity or return to school. A CBT model can include treatment of accompanying anxiety or depression and can be tailored to include involvement of the family. Graded exercise therapy (GET): is a structured and supervised programme of exercise agreed between doctor, therapist (usually a physiotherapist) and the child or young person. It is based on their current level of ability. Intensity and duration of exercise begin at a very low level and are increased very slowly depending on progress. The aim of GET is to increase fitness and stamina. The primary aim of management is to re-establish the child or young person in a sustainable routine and then to help them
PAEDIATRICS AND CHILD HEALTH 23:1
Figure 1 Vicious cycle.
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Ó 2012 Elsevier Ltd. All rights reserved.
OCCASIONAL REVIEW
they avoid the boom and bust pattern of activity which hinders recovery. It can be useful to think in terms of the different energy demands of situations e.g. high or low. Increase in activity levels needs to take place gradually and be carefully monitored. We would recommend a 15% increase in activity e.g. if able to read for 30 minutes a day increase to 35 minutes.
Top tips for management from our team These patients take time: C in clinic so it is useful to book appointments at the end of clinic C to recover Stay confident when the inevitable relapse occurs Remember progress is always slower than you expect There is no quick fix Involve parents
School liaison CFS has a negative impact on education and many children and young people are unable to attend full time school. A crucial element of any successful management plan, therefore, is the establishment of a sustainable educational routine. This may involve the home tuition services initially, followed by a carefully managed plan for school re-integration. It is very likely to involve part-time school attendance. A recent study suggests that the factor most strongly associated with reduced school attendance is poor physical function. It is therefore important to ensure that the child or young person is fully engaged with treatment to improve physical function. However it is also necessary to be aware of the cognitive impact of CFS and to ensure teachers are informed of the effects of CFS on memory, information processing and concentration. Children with CFS are entitled to exam concessions when taking public exams if requested by a doctor. The key professional needs to be prepared to put considerable time into liaizing with educational services. In our experience this patient group needs more time for liaison than other children with a chronic illness.
Table 3
Provision of services The provision of services for children and young people with CFS remains patchy. Despite NICE guidance which recommends referral to specialist services immediately if severely affected, within 3 months if moderately affected and within 6 months if mildly affected, there are still only 13 specialist teams in Britain and these are often small with limited capacity. The burden of treating this condition for the majority therefore lies with paediatricians and local therapy services. However, the specialist teams now have considerable experience of this perplexing, enigmatic condition. We would encourage professionals working with CFS to consult with a specialist team. In recognition of the scarcity of resources we have been instrumental in setting up a clinical network in North and Central England for those working with CFS in order to improve knowledge and services.
Management of specific physical symptoms
Prevention of the primary disease and disease complications and disabilities
We find that as the child or young person learns to manage their fatigue effectively and their well being begins to improve so other troubling symptoms such as nausea, abdominal pain, myalgia and joint pain all decrease. We therefore tend to avoid specific pharmacological agents. Gastrointestinal symptoms can be managed through diet and ensuring healthy eating and adequate fluid intake avoiding caffeine and sugary drinks. Small frequent meals are often better than three heavy meals. Pain is managed with simple analgesics like paracetamol and/or ibuprofen. In addition, relaxation and cognitive behaviour techniques may be considered in parallel to improve pain.
In the absence of a recognized aetiology the search for a method of primary prevention of the condition seems futile. However prompt recognition of the condition and onward referral to a specialist service where available, with expertise in managing the condition seems likely to be in the best interest of the affected individuals and their families. Since CFS affects not only the child or young person but also those around them, the sooner appropriate education and support is put in place the more effective rehabilitation can be and the less likely physical and psychological co-morbidities are to become entrenched. The absence from school and lack of socialization with peers leave these children and young people at very real risk of long-term psychological and emotional difficulties which early recognition and intervention might go a long way to ameliorate or even prevent.
Role of the family The role of the family is a crucial in helping a child or young person learn to manage this condition. Family life is affected and families have to adjust their expectations of what they can do together. Some parents have to stop work in order to care for their child. Support and encouragement from family members is valued by children and young people; it is therefore important that parents understand the condition fully and are involved in treatment. The package of care we have developed involves an initial period of treatment of six sessions every 3e4 weeks. At the end of this we hold an MDT review and a further six sessions can be offered to consolidate activity management and continue with school liaison. Other contributing factors may have emerged by this stage such as anxiety, low mood or family factors. It is then appropriate to arrange CBT or family therapy (Table 3).
PAEDIATRICS AND CHILD HEALTH 23:1
Conclusion CFS is a disabling and serious condition affecting physical and mental function and compounded by the uncertain and unpredictable course of the illness. There is still much to understand about this enigmatic condition and both clinicians and researchers have a duty to develop understanding of the causes and course of the condition. However, it is clear that early diagnosis and appropriate management greatly facilitate recovery, and the majority of children and young people make good recoveries.
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OCCASIONAL REVIEW
“To me keeping positive and determination I think were really important in my recovery. I think it was really important to keep trying e you go through such bad times and good times with this condition it is important to know you will get there eventually.”
Practice points C
A
C
FURTHER READING Chalder T, Goodman R, Wessely S, Meltzer R. The epidemiology of fatigue in children. Br Med J 2003; 327: 654e5. Chalder T, Hussain K. Self-help for chronic fatigue syndrome. Blue Stallion Publications, 2002. Collingridge E. Severe ME/CFS: a guide to living association of young people with ME 2010. Crawley E, Sterne JAC. Association between school absence and physical function in paediatric chronic fatigue syndrome/myalgic encephalopathy. Arch Dis Child 2009; 94: 752e6. Davies S, Crawley E. Chronic fatigue syndrome in children aged 11 years old and younger. Arch Dis Child 2008; 93: 419e21. Garralda M, Chalder T. Practioner review: chronic fatigue syndrome in childhood. J Child Psychol Psychiatry 2005; 46: 1143e51. Haig-Ferguson A, Tucker P, Eaton N, Hunt L, Crawley E. Memory and attention problems in children with chronic fatigue syndrome or myalgic encephalopathy. Arch Dis Child 2009; 94: 757e62. Jelbert R, Stedmon J, Stephens A. A qualitative exploration of adolescents’ experience of chronic fatigue syndrome. Clin Child Psychol Psychiatry 2010; 15: 267e83. National Institute for Health and Clinical Excellence. Chronic fatigue syndrome/myalgic encephalomyelitis (or encephalopathy): diagnosis and management of CFS/ME in adults and children. Clinical guideline no 53. London: NICE, 2007. Royal College of Paediatrics and Child Health. Evidence based guidelines for the management of CFS/ME in children and young people. London: RCPCH, 2004. Van de Putte EM, Engelbert R, Kuis W, Sinnema G, Kimpen J, Uiterwaal C. Chronic fatigue and health control in adolescents and parents. Arch Dis Child 2005; 90: 1020e4. Viner R, Hotopf M. Childhood predictors of self reported chronic fatigue syndrome in adults: national birth cohort study. Br Med J 2004; 329: 941e3.
PAEDIATRICS AND CHILD HEALTH 23:1
C
C
C
C
C
C
C
C
C
Children and young people with CFS do recover with good outcomes CFS is a clinically heterogeneous syndrome characterized by persisting and disabling fatigue. It is usually associated with a cluster of other troubling symptoms Diagnosis is made by a paediatrician once other potential disorders have been excluded CFS is a genuine physical illness and this needs to be communicated to child and family CFS is increasingly being recognized as a cause of significant school absence/poor school attendance in children and young people Demedicalization of symptomatology is essential and beneficial and in particular the need to draw a line under previous investigations and management in order to move forward is crucial The absence of a “magic wand/pharmacological cure” requires to be explained from the outset Management requires an MDT approach and both CBT, GET and activity management are recommended treatments Commitment to the acceptance of the need for rehabilitation by means of energy management and the adoption of good routines on the part of patients and carers is very important Professionals need patience and confidence and to be prepared for relapses It is important to engage family members as well as the child or young person
Acknowledgements We are grateful to the young people and their parents for permission to use their comments in this review.
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Ó 2012 Elsevier Ltd. All rights reserved.
Chronic Fatigue Syndrome in children and young people
This in turn frequently contributes to a poor patient/carer experience with delay in diagnosis and not infrequent hostility. This is unfortunate as CFS is a serious illness causing significant school absence and has long-term consequences for the educational, social and psychological development of the child or young person if there is no appropriate intervention. It also has an impact on family function and in some instances may result in financial hardship. As specialist provision is patchy it is important that any professional working with children and young people with CFS has a thorough understanding of the condition and its management.
Carrie Mackenzie Alison Wray
Abstract Chronic Fatigue Syndrome/myalgic encephalomyopathy (CFS/ME) is a relatively common and serious condition in children and young people, having a significant impact on their physical, emotional and cognitive well being. Although some progress has been made in understanding this perplexing condition there is still a long way to go and controversies persist about terminology, aetiology and treatment. However, there is a general agreement that CFS is a heterogeneous condition and good evidence regarding management. This review explores the best practice approach to assessment, diagnosis and management of CFS/ME in children and young people. Early diagnosis and appropriate multidisciplinary intervention facilitate recovery. There remains a pressing need for research to improve understanding of the condition and thereby ensure all children and young people with CFS/ME are understood and receive effective treatment.
Definition The fact that the terminology associated with this condition has created such controversy over a number of years indicates the difficulties inherent in labelling such a heterogeneous group of individuals who present with a wide range of physical and emotional symptoms. However the persisting and disabling fatigue universal in this group of children and young people has lead to our team favouring the use of the term Chronic Fatigue Syndrome (CFS). We believe this reduces confusion and assists in acceptance of the diagnosis when used in conjunction with a sympathetic explanation of the very variable nature of the condition which includes both physical and psychological symptoms. The term favoured and adopted by the Royal College of Paediatrics and Child Health (RCPCH) is CFS/ME but in our experience the term ME carries with it a much more physical or medical basis which patients and carers often confuse with conditions such as Multiple Sclerosis (MS). Moreover there are still those who believe that CFS and ME are two separate and distinct entities which share features with other conditions such as “Medically Unexplained Symptoms” (MUS) or other functional somatic disorders and overlap with chronic pain syndromes. In making the diagnosis, it is crucial to also “demedicalize” the condition, drawing a line under all that has gone before in terms of investigating the constellation of presenting symptoms and reaching agreement with the affected child or young person and their carers that the way forward is by means of rehabilitation from the point they now find themselves at. Indeed if this does not happen it may prove difficult to engage the child and family fully in a treatment programme (Table 1).
Keywords Chronic Fatigue Syndrome; myalgic encphalopathy
Introduction Chronic Fatigue Syndrome (CFS) or Myalgic Encephalomyopathy (ME) is a relatively common and serious condition affecting between 0.1% and 2% of children and young people aged under 18. It received public recognition as a specific clinical condition in 2002 and understanding of the condition is slowly improving. However, it is a heterogeneous condition with no diagnostic tests so diagnosis can be a complex task, particularly as the child or adolescent can present with a range of signs and symptoms. The presentation and course of the condition is often seen to ebb and flow further exacerbating the difficulty in predicting, understanding and managing symptoms. Although NICE guidance recommends referral to a paediatrician after 6 weeks of symptoms, the time from onset of symptoms to initial consultation with an appropriate professional is extremely variable and for some children and young people never takes place. Inevitably the lack of positive investigative findings coupled with the on-going debate about the pathophysiological basis of the condition leads to considerable and confusing debate amongst professionals and lay groups alike.
Epidemiology Prevalence data for CFS are heterogeneous and confusing as a result of different study methodologies (e.g. settings and diagnostic criteria) and different age limits. A number of studies report prevalence rates from 0.1% to 2% of children under 18. Most studies focus on the condition in adolescents: those reporting prevalence rates in younger children show it to be markedly lower. There remains a distinct need for further research to truly understand the prevalence of CFS in the child and adolescent population. The evidence on gender remains inconclusive. Some studies report no significant gender difference whereas others report a female excess of 2:1. Our own figures based on 176 children and young people under 16 show a ratio of two boys to every three girls.
Carrie Mackenzie MBchB FRCPCH DCCH MD is Consultant Paediatrician in the Children’s Hospital, Sheffield, UK. Conflicts of interest: none declared. Alison Wray BA MPhil CPsychol is Principal Clinical Psychologist in the Dept of Clinical Psychology at the Children’s Hospital, Sheffield, UK. Conflicts of interest: none declared.
PAEDIATRICS AND CHILD HEALTH 23:1
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Ó 2012 Elsevier Ltd. All rights reserved.
OCCASIONAL REVIEW
history and often normal examination findings are key in making the diagnosis (see Table 2).
Definition of CFS in children and young people C
C
NICE guidance e CFS/ME involves a complex range of symptoms that includes fatigue, malaise, headaches, sleep disturbance, difficulties with concentration and muscle pain. The pattern and intensity of symptoms vary between people and during the course of each person’s illness. RCPCH e generalized fatigue persisting after routine tests and investigations have failed to identify an obvious underlying ‘cause’. The fatigue is likely to be associated with other ‘classical’ symptoms such as difficulty in concentrating and disturbed sleep patterns and is classically exacerbated by effort (both mental and physical).
Investigations As a minimum we require that the child or young person will have undergone the screening investigations set out in the NICE guidance and that the results of these tests are normal when seen for initial assessment by the specialist CFS team. Diagnosis marks the start of a long and challenging journey for the child or young person and their carers. It is important to demedicalize and demystify the condition and positively and confidently recognize the symptom clusters shared by CFS sufferers. A further key feature of the initial consultation is to explain the nature of the condition, acknowledge the difficulties they have encountered on their journey thus far and reassure the patients and their carers that the expectation is that their condition will improve and that in our experience the vast majority of children and young people with CFS recover and go on to achieve academic qualifications. 99% of young people aged 16 seen by our specialist team achieved some academic qualifications and were able to progress to the next stage of their education or training. The vast majority of children and young people will not require transition to adult CFS services. About 5% continue to have a persisting and disabling illness.
Table 1
Pathology and pathogenesis There has been a plethora of research into CFS but the very wide and varied nature of the hypotheses being pursued indicates the clear lack of understanding of the pathophysiological basis for this condition. Many of our children and young people can describe an intercurrent illness immediately prior to the onset of their fatigue but the exact nature of this usually relatively minor illness is often unclear and it is by no means a universal precursor. It may well be that some external “trigger” provokes an autoimmune process in genetically predisposed individuals but the evidence for this does not yet exist and it seems likely that the causation is multi-factorial. Various aetiological factors such as level of exercise taken, personal and maternal psychological well being, gender, socioeconomic status, birth weight, birth order, coexistence of atopic conditions, exposure to certain viruses, school attendance and achievement have all been considered but as yet their contribution, if any, remains unclear. Any hope of intervention to prevent CFS in future generations will demand a better understanding of the aetiological factors at play. However the once popular stereotypical picture of the affected individual being female, highly intelligent and high achieving is not borne out by our experience.
“I visited the clinic and was diagnosed with CFS. It was a relief to know that my debilitating symptoms had a name. After more than 9 months of feeling so terrible someone could finally tell me what was wrong.” “It helped when I knew I had CFS. It was far better knowing than being left worrying or trying to guess.” The fact that CFS is a heterogeneous group is now generally agreed and evidence is growing to support this. One study has identified three phenotypes in CFS in children that are differentially associated with severity; the musculoskeletal phenotype is
Diagnosis and differential diagnosis
Investigations
The diagnosis of CFS relies not only on the exclusion of other pathology by means of appropriately directed investigations but also on the presentation of symptom clusters shared by other children and young people with the condition in addition to persistent and disabling fatigue. These symptoms may classically include: malaise, sleep disorder, headache, dizziness, poor temperature control, abdominal pain, nausea, anorexia, sore throat, tender lymphadenopathy, arthralgia, myalgia and cognitive difficulties such as poor short-term memory, impaired concentration, anxiety and low mood. Since many of these symptoms overlap with diagnoses relevant to many disciplines such as: Gastroenterology, Rheumatology, Endocrinology, Neurology, Infectious Diseases and Immunology, Oncology and Haematology as well as Childhood and Adolescent Mental Health it is beholden upon us to ensure that appropriate paediatric subspecialists have been consulted and the necessary investigations undertaken. However in the absence of any other disease, the
PAEDIATRICS AND CHILD HEALTH 23:1
These tests should usually be done: C Urinalysis for protein, blood and glucose C Full blood count C Urea and electrolytes C Liver function C Thyroid function C Erythrocyte sedimentation rate or plasma viscosity C C-reactive protein C Random blood glucose C Serum creatinine C Screening blood tests for gluten sensitivity C Serum calcium C Creatine kinase C Serum ferritin Table 2
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Ó 2012 Elsevier Ltd. All rights reserved.
OCCASIONAL REVIEW
associated with muscle and joint pain and these children have worse fatigue. The migraine phenotype which is associated with headache, abdominal pain, nausea and dizziness and noise sensitivity is associated with a lower level of physical function, worse pain and lower school attendance. The sore throat phenotype is associated with sore throat and tender lymph nodes and is the least severe.
learn to self manage the condition. At the outset of treatment this commonly involves three aspects: Sleep hygiene Activity/energy management School liaison In the absence of a clear understanding of the cause of CFS we make use of a number of analogies which ‘fit’ well with patients’ experience of the condition. We ‘explain’ to the child that their body is like a battery that has lost its charge and now requires recharging. The ways to do this are good sleep hygiene and activity management or GET. We also find that the idea of vicious cycles arising as a consequence of the condition is a useful concept with which families can identify (Figure 1).
Impact of the condition “It is one of the loneliest illnesses in the world because we don’t have anything to show for it.” Everyone involved with CFS agree that the condition has a profound impact, not only on the child or young person, but also on their families. It is associated with considerable school absence, social isolation, loss, changes in family relationships and parental time off work.
Sleep hygiene Disordered sleep patterns are commonly found in CFS including day/night reversal, interrupted sleep, insomnia or hypersomnia. It is therefore important to establish effective and positive sleep routines which include: Establishing a regular waking time Avoiding prolonged sleep and day time sleeps Always sleep at night in own bedroom and avoid use of computer/TV prior to bed Relaxation to aid falling asleep and consistent bedtime routine Melatonin and/or amitriptyline are used in some case to support good sleep hygiene and improve the quality of sleep.
“My symptoms meant that I started to miss a lot of school e I just didn’t have the energy to make it through the day.” “My daughter lost all her spark.”
Management Once children and young people and their families have gained an understanding of the condition, the basis of treatment and an optimistically realistic expectation of what the future might hold, a co-ordinated multi-disciplinary approach to management is required in the majority of cases. Commonly, occupational therapist, physiotherapist, nurse specialist and clinical psychologist may need to be involved. Evidence based guidelines recommend the following:
Activity/energy management The young person needs to keep a diary to establish their baseline of activity. Activity meaning everything that they do from having a shower being on the computer to going to school. Realistic goals then need to be agreed with the young person so
Activity management: this is a person centred, goal directed approach to managing a child or young person’s symptoms. It uses analysis of activity and graded activity through learning the skills of pacing to improve physical and cognitive function. Cognitive behaviour therapy: this is an individualized psychological therapy which incorporates two major components: the cognitive element which focuses on the identification and modification of thoughts, beliefs and assumptions which may shape the child or young person’s understanding of their condition, and the behavioural element which aims to gradually and consistently introduce a change in behaviour e.g. an increase in activity or return to school. A CBT model can include treatment of accompanying anxiety or depression and can be tailored to include involvement of the family. Graded exercise therapy (GET): is a structured and supervised programme of exercise agreed between doctor, therapist (usually a physiotherapist) and the child or young person. It is based on their current level of ability. Intensity and duration of exercise begin at a very low level and are increased very slowly depending on progress. The aim of GET is to increase fitness and stamina. The primary aim of management is to re-establish the child or young person in a sustainable routine and then to help them
PAEDIATRICS AND CHILD HEALTH 23:1
Figure 1 Vicious cycle.
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Ó 2012 Elsevier Ltd. All rights reserved.
OCCASIONAL REVIEW
they avoid the boom and bust pattern of activity which hinders recovery. It can be useful to think in terms of the different energy demands of situations e.g. high or low. Increase in activity levels needs to take place gradually and be carefully monitored. We would recommend a 15% increase in activity e.g. if able to read for 30 minutes a day increase to 35 minutes.
Top tips for management from our team These patients take time: C in clinic so it is useful to book appointments at the end of clinic C to recover Stay confident when the inevitable relapse occurs Remember progress is always slower than you expect There is no quick fix Involve parents
School liaison CFS has a negative impact on education and many children and young people are unable to attend full time school. A crucial element of any successful management plan, therefore, is the establishment of a sustainable educational routine. This may involve the home tuition services initially, followed by a carefully managed plan for school re-integration. It is very likely to involve part-time school attendance. A recent study suggests that the factor most strongly associated with reduced school attendance is poor physical function. It is therefore important to ensure that the child or young person is fully engaged with treatment to improve physical function. However it is also necessary to be aware of the cognitive impact of CFS and to ensure teachers are informed of the effects of CFS on memory, information processing and concentration. Children with CFS are entitled to exam concessions when taking public exams if requested by a doctor. The key professional needs to be prepared to put considerable time into liaizing with educational services. In our experience this patient group needs more time for liaison than other children with a chronic illness.
Table 3
Provision of services The provision of services for children and young people with CFS remains patchy. Despite NICE guidance which recommends referral to specialist services immediately if severely affected, within 3 months if moderately affected and within 6 months if mildly affected, there are still only 13 specialist teams in Britain and these are often small with limited capacity. The burden of treating this condition for the majority therefore lies with paediatricians and local therapy services. However, the specialist teams now have considerable experience of this perplexing, enigmatic condition. We would encourage professionals working with CFS to consult with a specialist team. In recognition of the scarcity of resources we have been instrumental in setting up a clinical network in North and Central England for those working with CFS in order to improve knowledge and services.
Management of specific physical symptoms
Prevention of the primary disease and disease complications and disabilities
We find that as the child or young person learns to manage their fatigue effectively and their well being begins to improve so other troubling symptoms such as nausea, abdominal pain, myalgia and joint pain all decrease. We therefore tend to avoid specific pharmacological agents. Gastrointestinal symptoms can be managed through diet and ensuring healthy eating and adequate fluid intake avoiding caffeine and sugary drinks. Small frequent meals are often better than three heavy meals. Pain is managed with simple analgesics like paracetamol and/or ibuprofen. In addition, relaxation and cognitive behaviour techniques may be considered in parallel to improve pain.
In the absence of a recognized aetiology the search for a method of primary prevention of the condition seems futile. However prompt recognition of the condition and onward referral to a specialist service where available, with expertise in managing the condition seems likely to be in the best interest of the affected individuals and their families. Since CFS affects not only the child or young person but also those around them, the sooner appropriate education and support is put in place the more effective rehabilitation can be and the less likely physical and psychological co-morbidities are to become entrenched. The absence from school and lack of socialization with peers leave these children and young people at very real risk of long-term psychological and emotional difficulties which early recognition and intervention might go a long way to ameliorate or even prevent.
Role of the family The role of the family is a crucial in helping a child or young person learn to manage this condition. Family life is affected and families have to adjust their expectations of what they can do together. Some parents have to stop work in order to care for their child. Support and encouragement from family members is valued by children and young people; it is therefore important that parents understand the condition fully and are involved in treatment. The package of care we have developed involves an initial period of treatment of six sessions every 3e4 weeks. At the end of this we hold an MDT review and a further six sessions can be offered to consolidate activity management and continue with school liaison. Other contributing factors may have emerged by this stage such as anxiety, low mood or family factors. It is then appropriate to arrange CBT or family therapy (Table 3).
PAEDIATRICS AND CHILD HEALTH 23:1
Conclusion CFS is a disabling and serious condition affecting physical and mental function and compounded by the uncertain and unpredictable course of the illness. There is still much to understand about this enigmatic condition and both clinicians and researchers have a duty to develop understanding of the causes and course of the condition. However, it is clear that early diagnosis and appropriate management greatly facilitate recovery, and the majority of children and young people make good recoveries.
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“To me keeping positive and determination I think were really important in my recovery. I think it was really important to keep trying e you go through such bad times and good times with this condition it is important to know you will get there eventually.”
Practice points C
A
C
FURTHER READING Chalder T, Goodman R, Wessely S, Meltzer R. The epidemiology of fatigue in children. Br Med J 2003; 327: 654e5. Chalder T, Hussain K. Self-help for chronic fatigue syndrome. Blue Stallion Publications, 2002. Collingridge E. Severe ME/CFS: a guide to living association of young people with ME 2010. Crawley E, Sterne JAC. Association between school absence and physical function in paediatric chronic fatigue syndrome/myalgic encephalopathy. Arch Dis Child 2009; 94: 752e6. Davies S, Crawley E. Chronic fatigue syndrome in children aged 11 years old and younger. Arch Dis Child 2008; 93: 419e21. Garralda M, Chalder T. Practioner review: chronic fatigue syndrome in childhood. J Child Psychol Psychiatry 2005; 46: 1143e51. Haig-Ferguson A, Tucker P, Eaton N, Hunt L, Crawley E. Memory and attention problems in children with chronic fatigue syndrome or myalgic encephalopathy. Arch Dis Child 2009; 94: 757e62. Jelbert R, Stedmon J, Stephens A. A qualitative exploration of adolescents’ experience of chronic fatigue syndrome. Clin Child Psychol Psychiatry 2010; 15: 267e83. National Institute for Health and Clinical Excellence. Chronic fatigue syndrome/myalgic encephalomyelitis (or encephalopathy): diagnosis and management of CFS/ME in adults and children. Clinical guideline no 53. London: NICE, 2007. Royal College of Paediatrics and Child Health. Evidence based guidelines for the management of CFS/ME in children and young people. London: RCPCH, 2004. Van de Putte EM, Engelbert R, Kuis W, Sinnema G, Kimpen J, Uiterwaal C. Chronic fatigue and health control in adolescents and parents. Arch Dis Child 2005; 90: 1020e4. Viner R, Hotopf M. Childhood predictors of self reported chronic fatigue syndrome in adults: national birth cohort study. Br Med J 2004; 329: 941e3.
PAEDIATRICS AND CHILD HEALTH 23:1
C
C
C
C
C
C
C
C
C
Children and young people with CFS do recover with good outcomes CFS is a clinically heterogeneous syndrome characterized by persisting and disabling fatigue. It is usually associated with a cluster of other troubling symptoms Diagnosis is made by a paediatrician once other potential disorders have been excluded CFS is a genuine physical illness and this needs to be communicated to child and family CFS is increasingly being recognized as a cause of significant school absence/poor school attendance in children and young people Demedicalization of symptomatology is essential and beneficial and in particular the need to draw a line under previous investigations and management in order to move forward is crucial The absence of a “magic wand/pharmacological cure” requires to be explained from the outset Management requires an MDT approach and both CBT, GET and activity management are recommended treatments Commitment to the acceptance of the need for rehabilitation by means of energy management and the adoption of good routines on the part of patients and carers is very important Professionals need patience and confidence and to be prepared for relapses It is important to engage family members as well as the child or young person
Acknowledgements We are grateful to the young people and their parents for permission to use their comments in this review.
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