Joint Trust Guideline for the Management of: CFS/ME (Chronic Fatigue Syndrome / Myalgic Encephalopathy) in Children and Young People

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1 A Clinical Guideline For Use in: By: For: Division responsible for document: Key words: Name of document author: Job title of document author: Name of document author s Line Manager: Job title of author s Line Manager: Supported by: Assessed and approved by the: All out-patient and in-patient paediatric settings All paediatric medical and nursing staff. Allied health professionals particularly children s occupational therapists and physiotherapists Children and young people with CFS/ME Paediatrics CFS/ME Children Dr Kate Armon Consultant Paediatric Rheumatologist David Booth Date of approval: 16/10/2015 Ratified by or reported as approved to (if applicable): To be reviewed before: This document remains current after this 16/10/2018 date but will be under review To be reviewed by: Kate Armon Reference and / or Trust Docs ID No: JCG0051 id 1283 Version No: 2 Description of changes: Clinical Director - Paediatrics Ginny Garnet, Lead Nurse Children s Services Norfolk PCT, Dr Stephen Nirmal Consultant Paediatrician JPUH Clinical Guidelines Assessment Panel (CGAP). Accepted by James Paget University Hospital 09 October 2014 Clinical Standards Group and Effectiveness Sub-Board Minor changes Compliance links: (is there any NICE NICE related to guidance) If Yes - does the strategy/policy deviate from the recommendations of NICE? If so why? This guideline has been approved by the Trust's Clinical Guidelines Assessment Panel as an aid to the diagnosis and management of relevant patients and clinical circumstances. Not every patient or situation fits neatly into a standard guideline scenario and the guideline must be interpreted and applied in practice in the light of prevailing clinical circumstances, the diagnostic and treatment options available and the professional judgement, knowledge and expertise of relevant clinicians. It is advised that the rationale for any departure from relevant guidance should be documented in the patient's case notes. The Trust's guidelines are made publicly available as part of the collective endeavour to continuously improve the quality of healthcare through sharing medical experience and knowledge. The Trust accepts no responsibility for any misunderstanding or misapplication of this document. Copy of complete document available from: Trust Intranet Page 1 of 11

2 Quick reference guideline/s Presentation Unexplained fatigue >3/12 Post-exertional malaise/fatigue New onset Reduction in activity History & Examination Red flags (indicating other causes) Abnormal Neurology Features of anxiety/depression Weight loss Consider safeguarding issues, in particular Fabricated or Induced Illness (FII) Investigations Urinalysis FBC, ESR, CRP, Liver Function. Urea & Electrolytes, Glucose, Thyroid Function, Calcium, CK, Ferritin Coeliac Screen Others as indicated (Viral serology) Diagnosis Unexplained Fatigue >3/12 Significant impairment of functioning Not relieved by rest No other cause Cognitive difficulties Chronic pain Non-refreshing sleep Other Advice Rest & Relaxation Sleep hygiene Diet Graded Exercise Therapy (GET) Mutually developed programme Set achievable baseline activity Increase in intensity when able Management General Principles Individualised programme Child in control of their goals Needs led Matched to severity of symptoms Liaison with education Provide information (AYME) Regular review Develop a setback plan Cognitive Behaviour Therapy (CBT) Not currently available Objective of Guideline Copy of complete document available from: Trust Intranet Page 2 of 11

3 The objective of this guideline is to provide professionals with an evidence based approach for the diagnosis and management of CFS/ME in children. In particular it emphasises the importance of making an accurate diagnosis, ensuring that all the clinical features are investigated and the value of working in partnership with children and their families. Rationale for the recommendations This guideline was written to reflect and incorporate recent national guidance on the management of CFS/ME in children. The local services for children with CFS/ME are fragmented and not comprehensive. This guideline will serve as a standard by which these services should be reviewed and revised. The evidence base, in particular that reflected in the NICE guidance (1) Children s NSF Exemplar (2) and RCPCH guidance (3) are in concordance with one another and provided a sound framework for the local development of this guideline. Other than Cognitive Behaviour Therapy and Graded Exercise Therapy there is little evidence for the other management strategies. However the guidance in this document accurately reflects national guidance and the principles of sound paediatric practice. Broad recommendations The process by which some children develop CFS/ME is poorly understood. It probably arises through a combination of biological, psychological and social factors. Management of children and young people with CFS/ME may be difficult. Most children recover but many miss significant amounts of school and take months or years to regain full levels of activity. These recommendations are written to enable prompt diagnosis and effective management thereby promoting a quick recovery. General Children and their families should have the opportunity to make informed and shared decisions about their care at every stage. Engagement with the family should be supportive and collaborative. Professionals working with children with CFS/ME should Acknowledge the reality and impact of the condition and symptoms Provide information about the range of interventions and management Offer information about local and national self-help groups As with all aspects of working with children, professionals require a sensitive awareness of presentations that may suggest a child is being harmed. Fabricated and Induced Illness may present as a chronic health problem, this includes CFS/ME. These cases necessitate consultation with the named safeguarding professionals in the trusts and/or Children s Services. Copy of complete document available from: Trust Intranet Page 3 of 11

4 History, examination and investigations A child or young person with symptoms that suggest CFS/ME should be referred to a paediatrician. All consultant paediatricians are initially able to see children with chronic fatigue. A detailed history and examination are necessary to exclude other underlying pathology. An assessment of their psychological wellbeing is required. However a full psychiatric/psychological history is not initially required. The paediatrician should be alert to the potential emotional dimensions of the illness including family dynamics. The initial family history should enquire into chronic illness, and in particular CFS/ME or similar conditions in other family members. Red flag symptoms that indicate a different pathology include Abnormal neurological signs Weight loss Symptoms of anxiety and/or depression Clinical signs of arthritis Skin rashes Localised pain waking the child at night The following investigations should be completed prior to diagnosis Full blood count Urinalysis for protein, blood and glucose Urea and electrolytes Liver function Thyroid function ESR and CRP Random blood glucose Creatinine Coeliac screen Serum calcium Creatinine kinase Serum ferritin Other investigations may be necessary. Serological testing may be helpful if there is a history to suggest infection, in particular Epstein-Barr infection. Diagnosis The diagnosis of CFS/ME is made through careful and detailed history taking and examination. It is a diagnosis of exclusion. Fatigue and pain are common presenting features but there a range of clinical presentations. The symptoms may vary over time. A diagnosis should be made after other diagnoses have been excluded and the symptoms have persisted for 3 months. Other key suggestive features include Copy of complete document available from: Trust Intranet Page 4 of 11

5 Post-exertional fatigue or malaise Cognitive difficulties Sleep disturbance Chronic pain All paediatricians require an understanding of the management of CFS/ME, but they should be prepared to ask an experienced colleague for a second opinion if they, the child or family have doubts about the diagnosis. General Management Follow the links below for detailed guidance from NICE (1). Early engagement of the family, as well as maintaining a therapeutic alliance throughout the illness, is crucial for successful implementation of the management plan. Careful communication with the child and family is key, particular in the early stages to achieving a good outcome. A written contract between the child, family and professionals may be helpful for some in clarifying the commitment and expectations from all parties. (a) Sleep Management (see Appendix A) Providing general advice about sleep and sleep hygiene is important. The completion of an activity diary (Appendix B) is helpful in clarifying current sleep patterns. The sleep-wake cycle is often reversed. Changes to sleeping patterns should occur gradually. Give an advice sheet to the family (Appendix A). Amitriptyline at low dose at night, and Melatonin have both been used to help some children with CFS/ME and associated sleep difficulties but the evidence for their effectiveness in only anecdotal and not evidence based. Melatonin is unlicensed. (b) Rest Rest is an important component of the management plan, when a child /young person is neither sleeping nor engaged in physical or mental activity (including reading, watching TV, any social interaction, excludes listening to music). Limiting rest periods to 30 minutes at a time may be helpful and building regular periods of rest into daily routines is necessary. (c) Diet A well-balanced diet is important. Eating regularly is important. Some children benefit from 5-6 small meals a day rather than 3 large ones. This is particularly helpful in those who have associated nausea. There is no evidence that any exclusion diets improve the symptoms and outcomes for those with CFS/ME. Severely affected children will require dietician input. Breakfast is a meal that is often missed and it is helpful to identify its importance. (d) Pain Copy of complete document available from: Trust Intranet Page 5 of 11

6 Chronic pain is a common component of CSF/ME. Frequently young people are seen having escalated through analgesics without benefit. Polypharmacy and stronger analgesics often lead to side effects without alleviation of pain. If there has been no benefit, try to wean young people off regular medication and onto simple analgesia (paracetamol/ibuprofen) for exacerbations of pain only. Amitriptyline at low doses (starting at 10mg at night, building up to maximum of 50mg) can be very helpful for chronic pain and sleep disturbance. Education It is necessary to work closely with Children s Services (education) and the child s school to ensure a common understanding of the diagnosis and management goals. The statutory guidance from the Department for Children, Schools and Families on education for children and young people with medical needs should be followed. (4) The amount of time children go to school is not the only marker of progress. School should not be the only activity children undertake. There needs to be a balance between school, home and social activities. Cognitive Behaviour Therapy (CBT) There is evidence that CBT improves the outcome for children and young people with CFS/ME. (1) CBT should only be delivered by a healthcare professional with appropriate training. CBT helps children to understand and break down their problems into manageable and smaller parts. It is a process that identifies the situation or problem and links that with the child s thoughts, physical feelings, emotions and subsequent actions i.e. what they think about their fatigue affects how they feel about their fatigue and consequently what they do about it. CBT should be delivered by a professional with appropriate training. CBT is currently unavailable locally for children and young people with CFS/ME. Graded Exercise Treatment (GET) GET is best delivered by a suitably trained occupational therapist or physiotherapist. Locally GET is offered on a limited basis by the children s occupational therapy and physiotherapy teams. GET should be based on the child s current level of activity. That baseline should be easily achievable. An activity diary will help to establish the baseline (Appendix B). It is important to ensure the child is not in a boom/bust cycle before increasing exercise. Increases in exercise should be manageable, by 10% at most. GET should be based on the person s current level of activity. When planning GET professionals should: Identify the long term activity goals Ensure the increases in activity are gradual Copy of complete document available from: Trust Intranet Page 6 of 11

7 Recognise that progress may be slow When starting GET professionals should Agree with the child sustainable increases in activity Advise that the symptoms may deteriorate initially Management of setbacks/relapses Setbacks and relapses are common and should be anticipated. A setback management plan should be agreed early on in the management of CFS/ME. An example of a plan might be: Following a setback: A new baseline activity level will be determined All aspects of the management plan will be reviewed Activity should be increased again in a sustainable and managed way Despite a setback, it is important to continue with the same general principles of management described above. Review and ongoing management Regular, structured review should be undertaken. The review should: Assess the improvement or deterioration in symptoms. Review the diagnosis, if the signs or symptoms change significantly. Consider referral to other services (Child and adolescent mental health, specialist CFS/ME services) Assess any additional support needs. Identify and agree short term and long term goals. Support and self help groups Association of Young People with ME (AYME) Telephone: AYME 9a Vermont Place, Tongwell Milton Keynes MK15 8JA info@ayme.org.uk Copy of complete document available from: Trust Intranet Page 7 of 11

8 AYME is the largest charity for children and young people with CFS/ME. They offer a range of supportive services. Representatives from AYME were part of the RCPCH CFS/ME guideline development group. Clinical Audit Standards derived from guideline (a) The investigations completed prior to diagnosis (b) Availability and use of CBT in management (c) Availability and use of GET in management (d) Clear and documented management plans include how to manage setbacks. This could be in the form of a letter copied to parents following a consultation. Summary of development and consultation process undertaken before registration and dissemination The authors listed above worked with a guideline development group, which also included Katie Waghorn (Lead Children s Occupational Therapist) Norfolk PCT and Vicky Easton (Children s Physiotherapist) NNUH. They agreed the final content and drafted the guideline. During its development it was circulated for comment to: All Central Norfolk Paediatricians, Children s Therapy and Community Children s Nursing Team leads, The Bethel Child and Family Centre (Tier 3 CAMHS Service) and the clinical governance leads for both the Children and Maternity Service s directorate (NNUH) and Norfolk PCT Children s Services. Comments made were incorporated into this final draft. This version endorsed by the paediatric guideline group. Distribution list/ dissemination method 1. Norfolk & Norwich University Hospital Intranet 2. Norfolk PCT knowledge management website References/ source documents 1. Chronic fatigue syndrome/myalgic encephalomyelitis (or encephalopathy), NICE clinical guideline 53, Issue date: August Chronic fatigue syndrome/myalgic encephalopathy (CFS/ME), National Service Framework for Children, Young People and Maternity Services Exemplar, Publication date: December icyandguidance/dh_ Copy of complete document available from: Trust Intranet Page 8 of 11

9 3. Evidence Based Guideline for the management of CFS/ME in Children and Young People, Royal College of Paediatrics and Child Health, Publication date: December Access to education for children and young people with medical needs. Department for Children, Schools and Families, Publication date Copy of complete document available from: Trust Intranet Page 9 of 11

10 Appendix A Sleep Sleep problems are common in CFS/ME. It is important to have the correct expectation about how much sleep a child requires. Children require different amounts of sleep dependent on their age. 3-5 years hours 6-12 years hours years 9-9 ½ hours The following are important components of good sleep hygiene. (a) Bedtime routine. Routine is helpful. A detailed history can establish areas for improvement. Avoiding television and computer games in the hour before bedtime is important. Similarly caffeinated food and drink should be avoided. Incorporating a bath or shower in the routine may help. A sleep diary can be useful in identifying the child s usual bedtime and wake time. Anchoring these times is important. Children should be encouraged to fall asleep and wake at the same time 7 days a week. Any changes to these should be made gradually, particularly for those with day/night reversal. Gradual steps of waking up 30 minutes earlier are usually maintainable. The objective is for the child to wake at 7-8am. (b) Bedroom Children need dark and quiet bedrooms. Their bedrooms should be places in which they sleep. Periods of rest during the day are better in a different room. This enables the child to associate their bedroom as an environment in which they sleep well. Removing televisions and computers from their bedroom may be unpopular but is important. (c) Can t get to sleep If a child is unable to fall asleep after minutes they should get up and try their bedtime / wind down routine again. This allows them to preserve the association between their bedroom and sleep. Copy of complete document available from: Trust Intranet Page 10 of 11

11 Appendix B Activity Diary Monday Tuesday Wednesday Thursday Friday Saturday Sunday High Energy Activity Low Energy Activity Rest Sleep Copy of complete document available from: Trust Intranet Page 11 of 11