Stroke in childhood - Clinical guidelines for diagnosis, management and rehabilitation
Prepared by the Paediatric Stroke Working Group
The Clinical Effectiveness and Evaluation Unit
The Clinical Effectiveness and Evaluation Unit (CEEU) of the Royal College of Physicians has expertise in the develoment of evidence-based guidelines and the organising and reporting of multicentre comparative performance data. The work programme is collaborative and multiprofessional, involving the relevant specialist societies and patient groups, the National Institute for Clinical Excellence (NICE) and the Healthcare Commission. The CEEU is self-financing with funding from national health service bodies, the Royal College of Physicians, charities and other organisations.
Acknowledgement
The development of these guidelines was supported by funding from a variety of sources including: The Stroke Association, Different Strokes, Boehringer Ingelheim, Merck Sharp & Dohme, and Sanofi-Synthelabo
& Bristol-Myers Squibb.
The Royal College of Physicians is also pleased to acknowledge a grant from The Haymills Charitable Trust towards the cost of designing and printing the guidelines.
Front cover
Design by Merriton Sharp, London.
The cover photograph, of a young boy who suffered a stroke at 15 months but who has made an almost complete recovery, is used by kind permission of Derek and Jane Walker and Different Strokes.
Every effort has been made to ensure that the website addresses in this document are valid at the time of going to press. However, readers should be aware that they may be subject to change over time.
Contents
The Paediatric Stroke Working GroupForeword
The child and family perspective
Foreword
Stroke, in both adults and children, used to be something that happened but which medicine could do, or chose to do, little about. Over the last decade there has been a revolution in stroke care for adults, with the advent of specialist stroke units and evolving treatments. Publication of the first edition of the National clinical guidelines for stroke in 2000 and the National Sentinel Audit of Stroke stimulated local units to consider the quality of the care they were delivering and put improvements in place. Rehabilitative care after stroke for adults is now considered the norm, and the collaboration between physicians, nurses, therapists and patients in these projects has led to true multidisciplinary working – much to the benefit of patients.
Similar improvements, however, have not be seen in the treatment of childhood stroke which, although less common than adult stroke, is still a serious problem and one which anecdotal evidence suggests is prone to an even more variable quality of care. Thus, when the national guidelines were being revised by the Intercollegiate Stroke Working Party, a subgroup was formed to consider the paediatric aspects of stroke care. During development it became clear that little evidence existed for many areas. The gaps were filled using the expertise of the group and the views of patients, parents and carers, but it is clear that there is a need for more formal research.
This guideline is aimed at healthcare staff in all parts of the NHS and related services, but much of it may also be of value to patients, parents and families. It is hoped that it will help childhood stroke services to emulate the improvement that has occurred, and is continuing to occur, in the care of adults, bringing a consistency and a knowledge of best practice to an area marked until now by dislocated care and uncertain standards.
To the casual reader wondering if this is important – I would urge you to begin by glancing at the italicised quotes from participants in the child and carer workshop which are scattered through the document. They plainly show how much more should and can be done to ensure good acute management, rehabilitation and secondary prevention, and to help these young people and their parents and families adjust to and cope with the effects of their stroke. Children have a lifetime ahead of them – any benefits from improved care will also last a lifetime.
The child and family perspective
As part of the guideline development process a workshop was held for children affected by stroke and their families. It gave a sense of the issues children and families felt to be important. We particularly focused on these issues in developing the guidelines, and they are outlined here.
The child is part of a family and, therefore, any childhood illness will have effects on both the immediate and extended family. The diagnosis of stroke is an unexpected one in a child, which compounds the shock experienced by families at the time of diagnosis.
The lack of information for families of children affected by stroke was a strong theme in the workshop. Information on what has happened and what to expect could empower families to ensure that the immediate and long-term needs of their child can be met. Information for parents, carers and children should be designed to meet their specific needs. The same information pack is unlikely to be appropriate in all cases but the information leaflet provided with this document may form a useful starting point. It is important that the questions asked by parents, carers, and children are answered. The communication of information to children is often particularly neglected.
Parents and carers of children affected by stroke welcome support from those who have had similar experiences – they appreciate someone to talk to about what has happened and to help them look at the future. Children also welcome meetings with those in their own age group affected by stroke. Both parents and children explained that the diagnosis of stroke is traumatic and that they need help in adjusting. It is important for families to see that life goes on. At the end of this document there is a list of useful organisations, including support groups, which may be able to provide information and support.
Families commented on significant gaps in communication between the various professional teams involved in the care of a child affected by stroke, and, as discussed above, between professionals and the family. This is an area which is extensively addressed in the guidelines.
There are significant problems in accessing therapy following childhood stroke in some areas. It is important that there is a coherent plan for rehabilitation which takes into account all of the child’s needs and which can be met within local resources. Gaps in service provision should be highlighted and brought to the attention of service planners.
Introduction
Background
Childhood stroke is a neglected area, with both professionals and the general public lacking awareness of the problem and its potential consequences. Stroke affects several hundred children in the UK each year and is one of the top ten causes of childhood death (Fullerton et al 2002). Many children who have a stroke have another medical condition (such as a cardiac disorder or sickle cell disease) and, therefore, are already vulnerable to adverse neurodevelopmental effects (Lanthier et al 2000, Ganesan et al 2003). The prevalence of sickle cell disease varies widely within the United Kingdom. However, it is noteworthy that at least 10% of these children and young people will have a stroke during childhood. The burden of childhood stroke on the health services is, numerically, smaller than stroke in the elderly. However, the long-lasting physical, emotional and social effects of stroke on an individual near the beginning of their life affect not only the individual themselves, but also their family and society as a whole.
Many professional agencies can be involved in helping the affected child fulfil their potential and in providing support and advice to the family. These agencies may change in the course of the child’s life and it is important that they are all aware of the consequences of childhood stroke, and that their efforts are co-ordinated. The child’s cognitive, social and emotional needs are in constant evolution and the functional impact of childhood stroke may, as a consequence, vary over time.
We have taken a child-centred approach to formulating these guidelines, working in partnership with children, families and support groups, specifically seeking the views of children and families, and centring the guidelines on issues raised by them. Throughout this document use of the term ‘parents’ is intended to encompass the child’s parents and any other carers.
The large number of consensus statements and good practice points in these guidelines emphasise that research in the field of childhood stroke is urgently needed to provide definitive answers to many of the issues raised. There is an acknowledged need for multicentre collaboration in such research to enable the design of studies with sufficient power to produce definitive results. The networks necessary for this are beginning to be established and may lead to work which could provide a firmer evidence base for the care of children affected by stroke.
Stroke in childhood: clinical guidelines for diagnosis, management and rehabilitation
Scope of the guidelines
These guidelines will primarily address the diagnosis, investigation and management of acute arterial ischaemic stroke in children beyond the neonatal period (aged one month to
18 years at time of presentation), including acute presentation and management, rehabilitation and longer-term care. Many of the issues covered here, in particular those relating to rehabilitation, will also be relevant to children with other causes of stroke (for example cerebral venous infarction, neonatal stroke or intracranial haemorrhage).
Purpose of the guidelines
These guidelines are aimed at professionals working in primary care, secondary level acute and community paediatrics, tertiary level paediatric neurology and neurodisability, education, and social services. The aim of the guidelines is to provide evidence-based recommendations for clinicians.
Methodology
These guidelines were formulated in accordance with the principles specified by the Appraisal of Guidelines Research and Evaluation (AGREE) collaboration
Following the publication of the National Clinical Guidelines for Stroke in 2000 (Intercollegiate Working Party for Stroke 2000) – referred to hereafter, along with the second edition (Intercollegiate Stroke Working Party 2004), as the ‘adult guidelines’ – several parties approached the Royal College of Physicians inquiring about guidelines for childhood stroke. The British Paediatric Neurology Association instigated a working party to formulate guidelines. This work was done in collaboration with the Clinical Effectiveness and Evaluation Unit of the Royal College of Physicians. Potential members were identified through their recognised record of clinical and research activity in the field of paediatric stroke and also through their professional organisations. Representation was sought across a broad range of disciplines and two patient organisations (the Stroke Association and Different Strokes). The members of the working party are listed at the front of this book. Conflicts of interest were declared and monitored (and full statements held on file).
The working party began by constructing a list of headings using the existing adult stroke guidelines (Intercollegiate Working Party for Stroke 2000) as a reference. We also considered specific additional issues relevant to children (for example, return to school). For each area, the group decided on a list of specific questions that would be considered. Searches were done using key words relevant to these questions of available computerised databases from 1966 onwards: Medline, AMED, CINAHL and Embase. In addition, the Cochrane Collaboration database was searched and other national guidelines and publications were reviewed. Members of the working party brought their own expertise and knowledge of the literature, as well as information from their organisations and professional bodies.
Topics were divided and allocated to individual members for evaluation according to their expertise. These individuals had responsibility for appraising the evidence and drafting the recommendations. The Scottish Intercollegiate Guidelines Network (SIGN 50) guidelines appraisal checklists were used to assess the quality of published articles. Guidelines were written on the basis of the available evidence with grading of the strength of the recommendation and explanatory statements where necessary. All recommendations were then presented to the working party as a whole for discussion and agreement.
Selection of articles for inclusion was based on the following principles. Where evidence specifically relating to childhood stroke was available, this alone was used. However, such literature is extremely limited and, therefore, research from other paediatric neurological conditions was evaluated where these conditions were felt to be relevant to the issues being considered. If a recommendation was based on extrapolation from research in a different population to that covered by the guideline, the grade of recommendation was reduced by one level.
Where evidence from meta-analyses or randomised controlled trials (RCTs) was available, this was used. Where there was limited or no evidence from RCTs, then evidence from observational group studies or small-group studies was used. In general, evidence from single-case studies was not used, primarily because it is difficult to draw general conclusions from them. Where there was no evidence base to support guidelines in areas which were highly relevant to clinical practice, consensus statements from this working party, other working parties and professional bodies were used. Many recommendations are in line with those in the developing National Service Framework for Children
The strength of evidence and recommendations were graded using the scheme proposed by SIGN 50 and summarised in tables 1.1 and 1.2, overleaf. The ‘Evidence’ sections following guidelines give an indication of the nature and extent of the supporting evidence, together with key references. Lastly, for each topic, there is an evidence table or group of evidence tables giving further details of the main studies.
Children affected by stroke and their parents were invited to attend a structured workshop in order to identify areas they thought should be addressed within the guidelines. The findings were used to identify key themes, which were subsequently incorporated into the issues addressed by the guidelines.
All the guidelines have been peer reviewed by external reviewers, a group which included a range of stakeholders.
Guideline strength: levels of evidence
Type of evidence
High quality meta-analyses, systematic reviews of RCTs, or RCTs with a very low risk of bias
Well-conducted meta-analyses, systematic reviews of RCTs, or RCTs with a low risk of bias
Meta-analyses, systematic reviews of RCTs, or RCTs with a high risk of bias
High quality systematic reviews of case control or cohort studies; high quality case control or cohort studies with a very low risk of confounding, bias or chance and a high probability that the relationship is causal
Well-conducted case control or cohort studies with a low risk of confounding, bias or chance and a moderate probability that the relationship is causal
Case control or cohort studies with a high risk of confounding, bias or chance and a significant risk that the relationship is not causal
Non-analytic studies, eg case reports, case series
Expert opinion
Guideline strength: grades of recommendation
Grade of
Recommendatio Evidence
At least one meta-analysis, systematic review or RCT rated as 1++, and directly applicable to the target population; or, a systematic review of RCTs or a body of evidence consisting principally of studies rated as 1+, directly applicable to the target population and demonstrating overall consistency of results
A body of evidence including studies rated as 2++, directly applicable to the target population, and demonstrating overall consistency of results; or, extrapolated evidence from studies rated as 1++ or 1+
A body of evidence including studies rated as 2+, directly applicable to the target population and demonstrating overall consistency of results; or extrapolated evidence from studies rated as 2++
Evidence level 3 or 4; or, extrapolated evidence from studies rated as 2+
A tick after a guideline represents a ‘good practice point’ – the recommended best practice based on the clinical experience of the guideline development group
Context and use
These guidelines are intended to inform clinical decisions rather than to be rigidly applied.
Terminology and theoretical framework
A factor interfering with delivery of good stroke care for children is the lack of a widely accepted framework of care and universal vocabulary and terminology. Health professionals have widely varying experiences of childhood stroke, but the problem is exacerbated by the variation in expertise and preferences for intervention in the absence of an accepted framework. The revised World Health Organization (WHO) classification (International Classification of Functioning, Disability and Health (ICF)) is intended to include all aspects of the health of an individual throughout life. This is designed to replace the former ICIDH (International Classification of Impairments, Disabilities and Handicap), which was widely accepted and used in describing health.
The International Classification of Functioning, Disability and Health
ICF classifies health and health-related states. The unit of classification is ‘categories’ within health and health-related domains. It is important to note, therefore, that in the ICF persons are not the units of classification; that is, ICF does not classify people, but describes the situation of each person within an array of health or health-related domains. Moreover, the description is always within the context of environmental and personal factors. This interaction can be viewed as a process or a result depending on the user. For an example of how the ICF might be used in practice, see Appendix 3.
Overview of ICF components
In the context of health:
body functions are the physiological functions of body systems (including psychological functions)
body structures are anatomical parts of the body such as organs, limbs and their components
impairments are problems in body function or structure such as a significant deviation or loss
activity is the execution of a task or action by an individual
participation is involvement in a life situation
i activity limitations are difficulties an individual may have in executing activities
i participation restrictions are problems an individual may experience in involvement in life situations
Terminology and theoretical framework
Guidelines
Each team should use a consistent framework and terminology in providing care to the
child affected by stroke
It is recommended that the World Health Organization’s International Classification of
Functioning (ICF) terminology is used
Service organisation
Children affected by stroke make use of all levels of health, education and social services in the United Kingdom. From a medical perspective the patient journey can be considered in terms of acute medical care, and both acute and longer-term rehabilitation. Rehabilitation should be integrated with the child’s educational, social and emotional needs.
In contrast to adult stroke, where the model is to develop specialist stroke services, the relative rarity of childhood stroke means that existing primary, secondary and tertiary systems of child health will – appropriately – be involved. These services and their potential roles are outlined in Table 3.1, overleaf. Services for the rehabilitation and longer-term needs of children with any acquired brain injury, including stroke, are relatively underdeveloped in the United Kingdom; in fact, their care challenges services and processes in both health and education, which are typically built around the needs of children with much more stable, slowly changing requirements (eg those with cerebral palsy). Although the development of more accurately targeted services is being proposed, the following section will describe the potential roles, and involvement in the management of children affected by stroke, of services as they are currently structured. The wide variation in the nature and potential severity of the long-term effects of stroke means that it is difficult to propose a single approach which would be suitable for all children, and the applicability of each recommendation to the specific child and family should be considered.
It is our view that all children affected by acute stroke should be referred to a consultant paediatric neurologist. However, it may not always be appropriate for the child to be transferred to an acute paediatric neurology unit. If this is the case, the child’s management should be discussed with the tertiary level paediatric neurology service. At present many, but not all, tertiary paediatric neurology units have multidisciplinary teams with expertise in the evaluation of children with acquired neurological problems. However in other tertiary centres, and many secondary centres, formal acute-based teams do not exist and are convened on an ad hoc basis. Where specialist expertise is not available locally professionals are encouraged to liaise with, and obtain advice from, colleagues in specialist centres. The needs of the child must remain central to the consideration of which professionals to involve.
The lack of structured paediatric rehabilitation services could be attributed to i) a lack of research regarding long-term outcomes of acquired brain injury in childhood, ii) a misplaced optimism regarding the plasticity of the child’s brain and the potential for recovery, iii) a lack of appreciation of the developmental context, and the fact that effects not apparent immediately may emerge with time, iv) a lack of recognition of the ‘invisible’ consequences of brain injury (for example, cognitive or emotional effects). These factors all need to be taken into account when considering the services available to children affected by stroke. The proposed national working group to develop a paediatric rehabilitation policy would be highly relevant to children affected by stroke. At present there are only 50 specialist paediatric rehabilitation beds in the UK. This means that the rehabilitation of the majority of children affected by stroke will take place either in the community or on general paediatric wards.
Multidisciplinary assessment and co-ordination, and the provision of long-term care, are usually undertaken by community child health services, most often by the child development service. It is also at this level that ongoing liaison between health, social and education services should occur. In many areas there will be a specific team, usually based in a child development centre, responsible for children aged five and under with disabilities (Standards for child development services (RCPCH 1999)). The community child health service, alongside professionals in the education services, will also be involved in the management of school age children. It is important that services are not duplicated and that all those involved are clear on who is taking the lead.
Primary care services are usually involved in general health issues, and the child’s general practitioner should be routinely and regularly informed by tertiary and secondary services of a child’s health and the services they are using. The health visitor may play an important co-ordinating role within the multidisciplinary team.
Effective multi- and inter-agency working is essential to ensure comprehensive care in the rehabilitation of children with acquired brain injury. This is also emphasised in several documents, for example Together from the start (DfES 2002), and the Standards for child development services (RCPCH 1999). Developments in information sharing resources, such as the forthcoming Integrated Children’s System (DfES) should facilitate multi- and interagency working.
The aim of team-working is to provide a smooth, coordinated and integrated service for children and their families. A ‘team’ is defined as a group of people working towards a single goal or set of goals, but it is important that this is an interactive effort. The aim of the multidisciplinary team is to provide a holistic perspective of the child and family in planning or providing interventions, and to stop any duplication of questions, assessments or services.
The model of having a key worker for the child and family is controversial and has not been researched in this group of children. However, documents relating to the management of children with disability (for example Together from the start (DfES 2002) and Standards for child development services (RCPCH 1999)) as well as the recent green paper, Every child matters (DH 2003b), advocate such a model. Given the complex and evolving nature of the potential consequences of childhood stroke and the multitude of agencies which could be involved, we feel that a key worker is likely to increase the likelihood of delivering a co-ordinated care package. A key worker is defined as a person who ‘works in partnership with the family, with the function of co-ordinating service provision and serving as a point of reference for the family’ (Together from the start, DfES 2002). A further critical aspect of this role is that the key worker takes responsibility for ensuring delivery of the package of care. Any professional could take on the role of key worker, but it is likely to be most appropriate that this is a member of the secondary level team. Additional factors which should influence the choice of the key worker are the preference of the child and family and the key worker’s competencies. The family should be given clear information about the identity and role of their key worker.
Guidelines
All children with acute stroke should be referred to, or have their management discussed
with, a consultant paediatric neurologist
Where specialist expertise is not available locally, professionals from all disciplines are encouraged to liaise with, and obtain advice from, colleagues in specialist centres
regarding the acute assessment and management of the child affected by stroke
Care should be provided in an environment that is appropriate for the child’s age and developmental level (D)
The medical, social, emotional and educational needs of the child affected by stroke should be considered early and systematically assessed in a co-ordinated manner when planning their subsequent care (D)
All members of the healthcare team should work together with the child and family, using an agreed therapeutic approach (D)
The longer-term management of the child affected by stroke should be co-ordinated by
a consultant paediatrician
A multidisciplinary team with expertise in the care of children with neurological conditions should be involved in the management of the child affected by stroke. Whilst this may initially be at tertiary level, it is essential that the relevant secondary level child
development service is involved from an early stage
A key worker should be appointed to co-ordinate the package of care, ensure its delivery and to act as a central point of contact for the family (D). The key worker and their role should be explained to the family
Consent
Children, whatever their age, have a right to be consulted and informed about any proposed treatment. The UN Convention on Children’s Rights recognises the right of children to make informed decisions. Information (either verbal or written) needs to be accessible to children. Their dignity, self-respect, and rights to self-determination and non-interference should be preserved.
The Children Act 1989 and European Association for Children in Hospital charter require that children and parents participate in decision-making. Children’s feelings and wishes should be sought and taken into account, and any reasons for not following them should be explained. Religious persuasion, racial origin, culture and language should also be considered. Children should be protected from unnecessary treatment and interference. The Gillick Judgement (Gillick v West Norfolk Health Authority 1985) requires that consent be given by a child if they have ‘sufficient understanding and intelligence to enable understanding fully what is proposed, even if under the age of consent’.
Families and carers
The child and family perspective
‘I wanted to know… if I could use my hand normally, how long it would take to heal’ (participant in paediatric stroke workshop).
Parents of affected children had experienced ‘not being told what is going on’ and ‘being kept in the dark’. Parents and carers also reported feelings of helplessness, distress and guilt from witnessing their children’s pain and fear and being unable to help them: ‘I felt helpless, I couldn’t do anything for her’, ‘I rushed … to the hospital, all I could hear was my niece screaming
I cried, saying to myself she’s only 15 years old’, ‘Seeing your child suffering and feeling guilty’.
Parents found it hard when they felt that their knowledge of their child was ignored: ‘The staff at hospital who did not listen to mother’, ‘Not having your mothering/fathering instincts listened to’. Parents suggested that it would be helpful if doctors could talk to them using more accessible language when explaining what is wrong with their child: ‘Getting doctors to explain the child’s condition in layman terms, not “doctor speak”’.
Parents also reported significant emotional problems following their child’s stroke and
suggested that meeting other parents would be very beneficial: ‘We have at times both seriously contemplated suicide’, ‘Parents benefit from meeting other parents’.
The majority of parents felt that they have to constantly fight public services so their child can receive the care and treatment that they should be getting: ‘Having to constantly fight for the justice of your child’, ‘Having to fight/ask for help when you are so vulnerable’.
All quotes are from participants in the paediatric stroke workshop.
Communication with the child and their family
The recognition that the child is part of a family is central to paediatric care. Any childhood illness has an impact on the whole family, including parents, siblings and grandparents. Childhood stroke has been shown to have an adverse impact on parents’ emotional and physical health (Gordon et al 2002).
Stroke is a completely unexpected illness in a child and parents and children feel emotionally devastated by the diagnosis. This is compounded by the lack of awareness of childhood stroke amongst professionals, which means that it is often left to the child and family to pursue treatment, rehabilitation and appropriate educational support. All professionals should be aware of the stress associated with a diagnosis of stroke on the child and family from the outset. The importance of emotional support and sensitive and comprehensive communication at the time of diagnosis of a disorder with potential long-term developmental consequences is emphasised in Together from the start (DfES 2002) and the Standards for child development services (RCPCH 1999).
The Stroke Association and Different Strokes (see Appendix 4 for contact details) provide information and support for children affected by stroke and their families. Children, family members or carers need both factual and practical information at various stages, presented in a format appropriate to their needs (Rushforth 1999; Helps et al 2003). It should be recognised that parents have particular knowledge of their child and, therefore, their concerns should be addressed in planning the child’s care and educational placement.
Guidelines
Families/carers should be given factual information about their child’s condition as soon as possible after diagnosis (D). This should be simple and consistent, avoiding technical terms and jargon
Written information should be provided to the child and family regarding the child’s health and the statutory and voluntary services available (D)
Children should be given information about their condition at an appropriate level (D)
The child and family should be involved in making decisions about the child’s care, including rehabilitation and education (D)
The multidisciplinary health team at secondary level should provide co-ordinated care and liaise closely with education and social services through the key worker (D)
Definition
The World Health Organization defines stroke as ‘a clinical syndrome typified by rapidly developing signs of focal or global disturbance of cerebral functions, lasting more than 24 hours or leading to death, with no apparent causes other than of vascular origin’ (World Health Organization 1978). This definition is a clinical one and such a presentation has many potential underlying causes in childhood. Brain imaging is mandatory for accurate diagnosis, subsequent referral and, in particular, to exclude conditions requiring urgent neurosurgical intervention. Arterial ischaemic stroke, which is the main focus of these guidelines, can be defined as ‘a clinical stroke syndrome due to cerebral infarction in an arterial distribution’. Transient ischaemic attacks (TIAs) (where the neurological deficit resolves within 24 hours) may also occur in children. Although clinical symptoms may be transient, a significant proportion of children with this presentation have cerebral infarction. Terms such as ‘acute infantile hemiplegia’ are clinical descriptions, which do not identify the underlying aetiology; they should, therefore, be avoided.
The following sections will deal, firstly, with guidelines for establishing a diagnosis in a child presenting with an acute clinical stroke syndrome (section 5.2), and then discuss the further investigation of children with a diagnosis of arterial ischaemic stroke in order to establish underlying aetiology (section 5.3). It may be pragmatic to combine the initial (diagnostic) and subsequent investigations, especially in the case of imaging, and both sections should, therefore, be considered together.
Presentation and diagnosis
At the time of stroke children and families reported feelings such as ‘frightened’, ‘annoyed’, ‘angry’, ‘confused’, ‘devastated’.
Parents reported feeling concerned and frightened at the amount of time they had to wait for diagnosis, treatment and information about their child’s condition: ‘Sitting for hours in the emergency department, with —, before it was finally acknowledged she had had a stroke’ (parent participant in paediatric stroke workshop).
Recognition of clinical stroke may be difficult, particularly in infants and young children, and especially as neurological signs may be relatively subtle. If there is doubt, the child should be examined by a senior paediatrician. The most common clinical presentation of clinical stroke in childhood is with acute hemiparesis. Focal signs may be absent in neonates or young infants, in whom seizures may be the only manifestation of clinical stroke. Clinical symptoms and signs of arterial ischaemic stroke may be particularly subtle in children with sickle cell disease, and may be difficult to distinguish from painful crisis or the effects of treatment, for example treatment with opiates. Advice should be sought from a tertiary centre if there is concern about the acquisition and interpretation of imaging studies in a child with clinical stroke.
Guidelines
All children with a clinical presentation of stroke should be under the care of a consultant
paediatrician
Cross-sectional brain imaging is mandatory in children presenting with clinical stroke (C)
Brain magnetic resonance imaging (MRI) is recommended for the investigation of children presenting with clinical stroke (C)
Brain MRI should be undertaken as soon as possible after presentation. If brain MRI will not be available within 48 hours, computed tomography (CT) is an acceptable initial
alternative
Brain imaging should be undertaken urgently in children with clinical stroke who have a depressed level of consciousness at presentation or whose clinical status is
deteriorating
Any new neurological symptoms or signs in children with sickle cell disease should be
evaluated as potentially being due to stroke
All children with clinical stroke should have regular assessment of conscious level and
vital signs
Investigations
This section aims to provide some guidance about investigations in the evaluation of a child with arterial ischaemic stroke; it is not, however, intended to be comprehensive. There are many potential risk factors for arterial ischaemic stroke in children and the diagnostic process should be directed towards identifying as many of these as possible. The proportion of patients in whom no risk factors are identified has decreased as understanding of aetiology and investigation methods have improved. There is little information on the diagnostic sensitivity of individual investigations. Although the investigations discussed below should be undertaken in all cases, other investigations may be indicated in individual patients, and should be considered on a case-by-case basis. For a more complete discussion of this topic see Kirkham 1999. A clerking checklist is provided to highlight important aspects of the clinical history and examination.
Transfer to a tertiary centre may be necessary if facilities for definitive imaging or other investigations (eg echocardiography) are not available locally. As mentioned in relation to brain imaging, advice should be sought from a tertiary centre if there is concern about the acquisition and interpretation of paediatric echocardiography.
Non-invasive cerebrovascular imaging with techniques such as MR angiography (Husson et al 2003), CT angiography, ultrasound with Doppler techniques or a combination of such modalities can be applied in the first instance, and may be adequate. The existing research on paediatric arterial ischaemic stroke only includes studies limited to visualisation of the arterial vasculature between the distal common carotid artery and the circle of Willis. The value of imaging the aortic arch and its proximal main branches is unknown. It is acknowledged that, in some cases, non-invasive angiographic techniques alone will not provide sufficient information to enable the planning of subsequent management, and in these cases catheter cerebral angiography may also be required. Due to the lack of specific research evidence we have not made more detailed recommendations regarding imaging sequences but a helpful discussion of these can be found in the review article by Hunter (Hunter 2002).
If there are unusual features to the identified infarct, such as the anatomical location, the presence of excessive brain swelling and then the possibility of venous infarction or haemorrhage should be considered. More specific venous imaging investigations may then be applied by the radiologist as necessary. In the first instance, non-invasive options such as MR venography or CT venography are preferred over catheter angiography.
The yield of investigating children with arterial ischaemic stroke for thrombophilia is variable and will depend on factors such as ethnicity. Protein C deficiency and elevated lipoprotein(a) have been shown to be associated with an increased risk of recurrence (Strater et al 2002). Although the appropriate preventative treatment in affected patients is unknown, identification of a prothrombotic tendency may have other implications for the child’s more general health, for example risk of venous thrombosis. Additional specific investigations to be included when screening for thrombophilia should be discussed with the local haematology service, with consideration of the local prevalence of specific thrombophilia.
The importance of more conventional childhood stroke risk factors in children with sickle cell disease has not been evaluated. The clinical experience of the working party is that these may play a role in some patients and therefore we would not exclude children with sickle cell disease from the recommendations below.
Guidelines
Imaging of the cervical and proximal intracranial arterial vasculature should be performed in all children with arterial ischaemic stroke (C)
Imaging of the cervical vasculature to exclude arterial dissection should be undertaken
within 48 hours of presentation with arterial ischaemic stroke
Transthoracic cardiac echocardiography should be undertaken within 48 hours after
presentation in all children with arterial ischaemic stroke
All children with arterial ischaemic stroke should be investigated for an underlying prothrombotic tendency. This should include evaluation for protein C protein S deficiency, activated protein C resistance, increased lipoprotein (a), increased plasma homocysteine, factor V Leiden, prothrombin G20210A and MTHFR TT677 mutations and antiphospholipid antibodies (C)
Acute care
This section relates to all aspects of acute care. Medical care, early evaluation of disability and rehabilitation are equally important aspects. These guidelines assume that, as is usual in the United Kingdom, the early care of a child who has had a stroke will be undertaken in a specialist paediatric neurology or general paediatric ward.
The acute medical management of arterial ischaemic stroke can be divided into general care measures and measures aimed at limiting the extent of ischaemic damage or preventing early recurrence. The latter depend on the likely cause of stroke in each case. As with the adult guidelines, treatment of secondary complications or of associated diseases is not considered here.
Early multidisciplinary evaluation is vital to prevent complications and plan rehabilitation. If the child is in an acute paediatric neurology ward, he or she is likely to have access to a multidisciplinary team with expertise in paediatric neurology. However, on a general paediatric ward it may be necessary for any member of the team to seek advice from colleagues in a tertiary centre. There is no specific research relating to the evaluation and management of children affected by stroke, but principles relating to the evaluation and care of children with other acute neurological conditions and, where available, published guidelines have been applied in formulating these recommendations.
General care measures
There are no studies which have specifically examined the effect of disruptions in homeostasis on stroke outcome in children but we have highlighted the points below based on principles which would be applied to the care of any acutely ill child, as well as from the evidence base in adults affected by stroke.
Guidelines
Temperature should be maintained within normal limits (D)
Oxygen saturation should be maintained within normal limits (D)
Specific medical treatments
There are no studies specifically examining the efficacy of acute treatments for arterial ischaemic stroke in children. The following recommendations are based on the consensus opinion of the working party.
The use of anticoagulation in children with cardiac embolism is controversial as it involves balancing the risk of precipitating haemorrhagic transformation of the infarct with the potential to prevent further embolic events. The decision may be influenced by the cardiac pathology, time elapsed after the stroke and by neurological and imaging findings. In the absence of any evidence, we were unable to make a general recommendation, but felt that individual patient management should involve senior clinicians in paediatric cardiology and neurology.
The efficacy and optimal dose of aspirin in the treatment of children with acute arterial ischaemic stroke is unknown. The lowest dose recommended for treatment of other paediatric conditions, such as Kawasaki disease, in the paediatric formulary Medicines for children (Royal College of Paediatrics and Child Health 2003) is 5 mg/kg/day. This would approximate to the dose (300 mg) recommended for acute treatment of ischaemic stroke in adults (National clinical guidelines for stroke: second edition, Intercollegiate Stroke Working Party 2004) and therefore has been recommended below. The lowest effective dose for long- term prophylaxis may be lower, as discussed in the following section. Although children with sickle cell disease have been excluded from the first guideline, aspirin or anticoagulation may need to be considered if other risk factors, for example arterial dissection, are identified in individual patients.
There is currently no evidence to support use of thrombolytic agents such as tissue plasminogen activator (tPA) in the acute treatment of arterial ischaemic stroke in children.
Guidelines
Aspirin (5 mg/kg/day) should be given once there is radiological confirmation of arterial ischaemic stroke, except in patients with evidence of intracranial haemorrhage on
imaging and those with sickle cell disease
In children with sickle cell disease and arterial ischaemic stroke:
iurgent exchange transfusion should be undertaken to reduce HbS to <30% and raise haemoglobin to 10–12.5 g/dl
Acute care
if the patient has had a neurological event in the context of severe anaemia (eg splenic sequestration or aplastic crisis), or if exchange transfusion is going to be delayed for
more than four hours, urgent top-up blood transfusion should be undertaken
Providing there is no haemorrhage on brain imaging, anticoagulation should be considered in children with:
confirmed extracranial arterial dissection associated with arterial ischaemic
stroke
cerebral venous sinus thrombosis (C)
The decision to use anticoagulation in children with arterial ischaemic stroke who have a cardiac source of embolism should be discussed with a consultant paediatric
cardiologist and paediatric neurologist
Early neurosurgical referral should be considered in children with stroke who have depressed or deteriorating conscious level or other signs of raised intracranial
pressure
Secondary prevention of arterial ischaemic stroke in childhood
Child in paediatric stroke workshop: ‘What happens if you get two strokes?’
Stroke recurrence is a major concern for children and their families. Arterial ischaemic stroke recurs in between 6% and 20% of all children and in over 60% of children with sickle cell disease. The risk of recurrence is increased in children with multiple risk factors (Lanthier et al 2000) and in those with protein C deficiency, increased levels of lipoprotein
(a) and vascular disease (Sträter et al 2002). At present, there is very little evidence regarding the efficacy of secondary prevention strategies (Sträter et al 2001) but this is likely to change as the need for multicentre trials is gaining momentum. Thus we would emphasise that the consensus statements below are not long-term recommendations and will need to be updated as new evidence emerges.
Although widely used, the dose of aspirin to be used for secondary prevention of ischaemic stroke is undefined in childhood. Doses between 50–300 mg/day are recommended for adults (National clinical guidelines for stroke: second edition Intercollegiate Stroke Working Party 2004) and doses between 1–3 mg/kg/day have been recommended for secondary prevention in children (Nowak-Gottl et al 2003). Given the lack of evidence we have not been able to recommend a specific dosage; however, complications such as bruising may limit the dose which can be tolerated by the child.
Guidelines
Patients with cerebral arteriopathy other than arterial dissection or moyamoya syndrome
or those with sickle cell disease should be treated with aspirin (1–3 mg/kg/day)
Anticoagulation should be considered:
until there is evidence of vessel healing, or for a maximum of six months, in patients
with arterial dissection
if there is recurrence of arterial ischaemic stroke despite treatment with aspirin √
in children with cardiac sources of embolism, following discussion with the
cardiologist managing the patient
until there is evidence of recanalisation or for a maximum of six months after cerebral
venous sinus thrombosis
In children with sickle cell disease:
regular blood transfusion (every three to six weeks) should be undertaken to maintain the HbS% <30% and the Hb between 10–12.5 g/dl (C)
transfusion may be stopped after two years in patients who experienced stroke in the context of a precipitating illness (eg aplastic crisis) and whose repeat vascular imaging is normal at this time (C)
after three years a less intensive regime maintaining HbS <50% may be sufficient for stroke prevention (C)
those who cannot receive regular blood transfusions because of allo-immunisation, auto-antibody formation, lack of vascular access or non-compliance with transfusion or chelation may be considered for treatment with hydroxyurea (C)
Children with moyamoya syndrome (including those with sickle cell disease) should be referred for evaluation to a centre with expertise in evaluating patients for surgical revascularisation (D)
Children with sickle cell disease who have had a stroke should be referred to a specialist centre for consideration of bone marrow transplantation (B)
Advice should be offered regarding preventable risk factors for arterial disease in adult life, particularly smoking, exercise and diet (D)
Blood pressure should be measured annually to screen for hypertension
Patients who are found to have a prothrombotic tendency should be referred to a
haematologist
Early disability assessment and management
The philosophy of paediatric medicine is for care to be delivered, where possible, in the child’s home environment and to minimise the time a child spends in hospital. The inpatient period may be limited to the time when the child is acutely unwell or when there are outstanding diagnostic issues. It is, therefore, important that links to community child health services should be made from the outset in order for a package of care to be set up, and that the child is not discharged until this is in place. Ultimately this package may be delivered in a variety of settings, as discussed in chapter 3. Parents (and children when possible and appropriate) should be involved in planning long-term care.
Guidelines
As soon as possible after admission, following stroke all children should have an evaluation of:
swallowing safety (D)
feeding and nutrition
communication (D)
pain (D)
moving and handling requirements (D)
positioning requirements
risk of pressure ulcers (D)
All children affected by stroke should have a multidisciplinary assessment within
72 hours of admission to hospital
The professionals involved in the acute assessment and management of the child should initiate early liaison with their counterparts in the community to ensure a smooth
transition of care
Working party consensus
Working party consensus
Approaches to rehabilitation
Parent in paediatric stroke workshop: ‘When we came home from hospital it was as if you’re home now
– “Goodbye and get on with it”. That’s the way I feel and still feel. There’s been no physio or occupational therapy yet and she’s been home about eight weeks now. All I’ve had is phone numbers and names which are no help to me as there’s very little for younger people with strokes. They don’t cater for younger children only adults. So it feels as if you’re forgotten.’
Parent in paediatric stroke workshop: ‘After we came out of hospital the real problems began. The local hospital had little experience of strokes in children. We waited six weeks for physio locally, eight weeks for the OT to assess her. The problem seemed to be “resources” – or shall we say “Government funding”!’
Early rehabilitation of children with acquired brain injury is usually undertaken from the time of diagnosis in the setting of the acute ward; however, subsequent rehabilitation is usually carried out in the community or, in some cases, may be undertaken in a specialist children’s rehabilitation unit or on a general paediatric ward (see chapter 3). As previously stated, early liaison and communication between the professionals involved in rehabilitation from tertiary through to primary care services is critical to smooth transition. Rehabilitation interventions may include both ‘hands-on’ intervention and equipment provision, thus involving a variety of health professionals, education and social services agencies.
The impairments experienced by children affected by stroke, and their functional consequences, may evolve over time due to ongoing growth and development. Rehabilitation of children with limitation in functional skills requires consideration of the impact of sensory, perceptual, motor and cognitive impairments on the individual so that interventions are appropriate. The child and family should be encouraged to express their main concerns about re-integration to the home, community and school environments, and to have these concerns addressed. The impact of stroke on the psychological well-being of the family may adversely affect the child’s rehabilitation process. The rehabilitation professional should consult with specialist acute and community-based rehabilitation teams if this is indicated.
There is little evidence on the effectiveness of rehabilitation interventions specifically for children affected by stroke. It may appear in the following sections that there is undue emphasis on sensorimotor function; however, this is because there is a wider availability of research in this domain due to work undertaken in children with cerebral palsy. The reasons for including this literature have been discussed in the introduction. We accept that there are limitations in extrapolating from this population to children with acquired brain injury. Some important differences between the two groups include the level of pre-morbid functioning, age at injury and the rate of change in status. We would also emphasise that, as in children with traumatic brain injury, ‘invisible’ impairments are under-recognised and a source of significant morbidity in children affected by stroke.
Guidelines
Children affected by stroke should be offered advice on, and treatment aimed to achieve, play, self-care, leisure and school-related skills that are developmentally relevant and appropriate to their home, community and school environment (D)
Equipment which is appropriate in meeting rehabilitation aims should be assessed on an individual basis, provided in a timely manner, and regularly monitored by appropriate professionals (D)
Sensorimotor rehabilitation
Underlying approach to sensorimotor therapy
The aim of sensorimotor intervention is to improve motor control and physical independence, and to promote developmentally appropriate functional skills. These principles are practised in most therapy approaches. However, they vary according to the type of stimuli used, the emphasis on task-specific practice and/or the principles of learning which are followed. There is little evidence to support the superiority of one treatment approach over another.
Guidelines
Sensorimotor therapies should be practiced within a neurological framework, and
complement other interventions to improve functional skills
Rehabilitation activities should be task orientated and relevant to the individual’s life (C)
Therapy should be integrated into the child’s daily home and school activities (D)
Delivery of sensorimotor therapy
Issues regarding the frequency and intensity of contact with therapists are frequently raised by parents and older children. Play and daily living activities enable practice of functional skills and are, therefore, of therapeutic benefit. Collaborative working between parents, teachers or any other member of the multidisciplinary team can ensure that children are able to practise such activities in all their environments.
Goal-setting in therapy involves identifying measurable objectives of performance which are beyond the child’s current abilities, but which are considered achievable and relevant to the child’s life. Goal-setting may be a helpful adjunct to therapy, in addressing issues of concern to the child, family and therapists, and in measuring the outcome of intervention, and can be useful to record functional changes.
Guidelines
Children should be given as much opportunity as possible to practise skills (C)
Use of assessment measures
Assessment should aim to document a particular aspect of function, impairment, activity and participation as objectively as possible. This will enable the dissemination of information between professionals and services and facilitate the setting of measurable rehabilitation goals and measurement of progress. A wide range of assessment tools is available for the evaluation of children with neurological disorders, of which many are potentially useful in the evaluation of children affected by stroke. It is not possible to make recommendations regarding specific assessment tools as the most appropriate one will depend on many factors, such as the age and developmental level of the child and the resources and training of the team.
Guideline
The assessment tools selected should be appropriate for the child’s age and developmental
and functional level
Standardised and validated assessment tools should be used where possible
Motor impairment
Both central and peripheral components of motor impairment require consideration during rehabilitation. Hemiparesis is the most common pattern of motor impairment resulting from ischaemic stroke. Spasticity is also common; however, as the basal ganglia are commonly affected in childhood stroke, dystonia or a mixed pattern are seen frequently as well. Hemi-dystonia may have a delayed onset and may follow a period of initial good recovery after the stroke. The upper limb, particularly the hand, tends to be more severely affected than the lower limb in children with ischaemic stroke.
There are a wide variety of treatment interventions available to children with motor impairments but not all have a strong evidence base. These include therapies such as lycra splinting, inhibitive casting, treadmill training, constraint-induced movement therapy and functional electrical stimulation. However, this does not suggest that these treatments are not beneficial if used appropriately. The following guidelines are based on the interventions with an evidence base to support their use.
Muscle strengthening
Muscle weakness is now recognised as a component of disability and studies show that strength training does not increase spasticity and may, in fact, reduce it. Programmes to improve children’s muscle strength can lead to improved functional skills. Muscle shortening may also be linked with muscle weakness and interventions which immobilise muscles should be evaluated in terms of their impact on muscle strength.
Guidelines
Muscle strengthening should be used as part of the therapy programme to prevent or reverse contractures (D)
Muscle strengthening activities should be used to improve functional activity (D)
Management of spasticity
Botulinum toxin is a drug used in the treatment of spasticity. It is only effective on the dynamic component of muscle shortening and will not improve fixed contractures. Botulinum toxin is generally safe, has minimal side effects and can be targeted at individual muscles. However, it is an expensive intervention and its effects may reverse after three months. The long-term effects of repeated injections in children are not known. Its potential benefits are enhanced and maintained by concurrent therapy interventions. Currently there are no reliable conclusions on the use of botulinum toxin but a number of studies are underway.
Guidelines
If spasticity results in functional limitation or discomfort, botulinum toxin injection may be used to reduce muscle tone and improve range of joint motion (B)
The most effective dose for gastrocnemius injection is 20 u/kg to reduce the dynamic component of muscle shortening and increase active length (C)
Botulinum toxin should not be used in isolation from other therapy interventions
Ankle-foot orthoses
The use of ankle-foot orthoses (AFOs) may be considered in children with lower limb involvement. AFOs provide intimate control of the foot and ankle. They are used to maintain muscle length and improve gait patterns. Rigid and hinged AFOs extend to just below the knee with the latter being hinged to allow a limited range of ankle movement. Dynamic ankle-foot orthoses (DAFOs) have a contoured foot support and extend to just above the ankle. Continuous use of rigid orthoses may be implicated in reduced strength of the lower leg muscles.
Guideline
A rigid AFO could be considered to aid standing balance, swing through in gait and
prevention of foot and ankle contractures
A hinged or posterior leaf spring AFO should be used to facilitate normal gait patterns (D)
Sensory impairment
Clinical experience suggests children may experience disturbance of a variety of sensory modalities following stroke. Some of these may be more easily detectable than others, but all should be considered by health professionals assessing and working with the child as they have the potential to impact outcome and response to rehabilitation.
Somatosensory impairment
Clinical experience suggests that there may be transient or long-term alterations in tactile, proprioceptive and kinaesthetic awareness following childhood stroke. These difficulties may influence movement performance and sensorimotor rehabilitation. They may also influence body awareness and have implications for safety in functional daily activities. Somatosensory assessment relies on careful clinical observation in the absence of accepted valid assessment methods.
Guideline
Rehabilitation professionals should consider the presence of somatosensory impairment
and integrate this in planning and implementing rehabilitation
Hearing and vision impairment
There is no data on the incidence of vision or hearing impairment in children affected by stroke; however, as with any acquired brain injury, these are potential consequences. Assessment should be comprehensive and include evaluation of both perception and processing of visual and auditory stimuli.
Pain
Some children reported extreme pain at the time of stroke, for example one child at the workshop reported that when they had the stroke they felt pain which was ‘Awful, immense, thousand daggers in my eye, horrible, terrifying’.
Potential sources of pain after childhood stroke include headache, shoulder pain, dysaesthesia and abdominal pain due to gastro-oesophageal reflux. Shoulder pain in adults has been particularly associated with a prolonged hospital stay and poor recovery of arm function, and clinical experience with children would support this. Proprioceptive dysfunction may involve the whole body. Staff should be aware that children may experience pain and discomfort due to sensory disturbance, but can have difficulty expressing this.
Guidelines
Children affected by stroke should be assessed for the presence of pain using a validated paediatric pain tool (D)
All pain should be treated actively, using appropriate measures including positioning,
handling, and medication
In cases of intractable pain, the child should be referred to health professionals with
specialist expertise in pain management
Language and communication
The effects of childhood stroke on language and communication may be specific or global and short- or long-term. Stroke may impair function at any stage of language input (receptive aphasias), language processing (word-finding problems, grammatical problems and other aphasias), speech production (dysarthria) and written language (dyslexia). There may also be problems of social interaction and the willingness to communicate (mutism). Unlike in adults, speech, language and communication consequences may not be determined by which hemisphere is affected and is seen in children with both cortical and subcortical injuries. The child’s ability to communicate may also be adversely influenced by physical and/or behavioural problems, their general health, other aspects of their environment (for example, volume of ambient noise) or cognitive skills. Developmental speech and language problems may have been present prior to the stroke and these will need to be taken into consideration when planning management.
Communicating quality (2) (RCSLT 2001) states that a child with communication difficulties requires comprehensive assessment, and that where indicated a specialist speech and language therapist should be responsible for this. Whilst dramatic patterns of recovery have been documented in the early stages of rehabilitation, the prospect of long-term residual deficits, sometimes of a subtle or high level nature, should not be overlooked (Lees & Neville 1990, Lees 1997). Because language learning goes on through the first two decades of life, and the child needs to accommodate to the changing demands of different communication situations, difficulties may surface some time after the initial stroke.
Where children have restricted verbal output or are non-verbal following stroke, the use of alternative and augmentative communication (AAC) systems should be considered. This may include signing, symbols and simple or complex speech output devices.
Guidelines
Professionals working with children affected by stroke should be aware that language and communication skills may be affected (D)
If parents, professionals or the child’s educational assessment raise concerns regarding language or communication, the child should be referred to a specialist speech and language therapist (D)
A detailed assessment of the child’s communication abilities should be carried out in collaboration with the child, parents/carers, teachers and other therapists to identify the child’s strengths and weaknesses and plan intervention that aims to increase functional abilities (D)
A collaborative approach to the management of communication difficulties that includes working with an educational psychologist, other therapists, teachers and social workers should aim to equip the child with a language for life (D)
Cognitive effects
The cognitive consequences of stroke in childhood are often underestimated, yet they can impact on all aspects of functioning. Cognitive impairment is particularly likely to be underestimated in children without a physical disability and these children may be incorrectly perceived as not being disabled. Childhood stroke is known to affect both global and specific aspects of cognitive function. The severity of cognitive impairment associated with stroke is variable and, additionally, its impact has to be viewed in a developmental context. Unlike adults, cognitive consequences are not only determined by which hemisphere is affected. They may result from both cortical and subcortical injuries, although limited evidence suggests they are greatest in children with large lesions. Deficits have been
reported in intellectual functioning, language and verbal abilities, visual-motor and visual- spatial processing, performance, sequential memory and academic achievement. Specific patterns of impairment may be related to the underlying aetiology or site of injury and, additionally, may emerge during development. For example, attention and executive functioning difficulties have been found in children with sickle cell disease and stroke as these children are vulnerable to frontal lobe damage. Although the child’s functioning may still be within the normal range after a stroke, there may be a significant reduction in cognitive functioning and the child’s ability to access the curriculum may be altered. Computer-assisted learning may need to be considered to help the child access the curriculum, alongside other specialist interventions.
Cognitive impairment will impact on all aspects of daily functioning. Schools and teachers need to be aware of the potential for cognitive difficulties as these may not be apparent unless the child has a comprehensive assessment. Such an assessment should be carried out in collaboration with colleagues in educational and therapy services and should generate explicit recommendations to support the child both at home and at school. Studies of children with traumatic brain injury suggest that such injury affects the ability of the child to acquire new knowledge; this is also likely to apply to children affected by stroke. Developmental factors, as well as the changing expectations of the child, mean that the functional and educational impact of cognitive impairment is likely to change as the child gets older and therefore their cognitive functioning will need to be reviewed over time.
Children with sickle cell disease are at risk of progressive cognitive dysfunction due to the high incidence of stroke recurrence and‘silent’cerebral infarction. Services for these children should consider repeating cognitive assessments; however, there is no evidence on which to base recommendations about the frequency with which they should take place and the problems of repeated assessment should be considered.
Guidelines
Professionals working with children affected by stroke should be aware that cognitive function may be affected, both immediately and in the longer term (C)
A detailed psychological assessment of the child’s cognitive and functional abilities together with any wider family concerns should be carried out in collaboration with the child, parents/carers and teachers to identify any special educational needs (D)
Cognitive assessment should take account of the presence of any visual or hearing
deficits
Mood and behaviour
Parent in paediatric stroke workshop: ‘Because the brain is affected the child is in need of help physically, emotionally and mentally. They often are not the same people they were before the stroke. The parents cannot be expected to cope without the help of experts and the child/teenager definitely benefits if their parent is not always around as family tension/fears can be the cause of much distress at the time.’
Parent in paediatric stroke workshop: ‘My daughter also heard voices in her head after her stroke telling her the most distressing things. Stuff that made her feel anxious, guilty and afraid. She felt that her whole life had been turned upside down and that she was going mad. She suffered from severe anxiety attacks, exhaustion and her unhappiness led to a nervous breakdown five years after her stroke.’
There is emerging evidence that childhood stroke has effects on many aspects of behaviour, even in children without apparent difficulties in other domains. There is no specific research on the effects of childhood stroke on mood. However, studies of children with hemiplegia (a group which includes some children affected by acute stroke) has shown that these children experience an increased rate of emotional difficulties (Goodman & Yude 2000). Studies in children affected by stroke have shown that a third of parents feel that their child’s behaviour has altered following the stroke (Ganesan et al 2000). This observation has been confirmed by a high rate of emotional and behavioural difficulties detected using a behaviour screening questionnaire (Wraige et al 2003). In children with sickle cell disease, brain injury is associated with difficulties in decoding emotions and interpreting social situations which may not become evident until adolescence. Unidentified cognitive impairment may exacerbate mood or behaviour problems so both these areas should be evaluated together.
Guidelines
Families and professionals should be aware that stroke may have effects on mood and behaviour (D)
The psychological assessment of the child should include evaluation of mood and behaviour, including wider family concerns (D). This should be undertaken in conjunction with cognitive assessment
Mood and behaviour should be assessed if there is a change in the child’s functioning
in the home or school environment
If mood or behaviour problems are identified and are having an impact on the child’s functioning, the child should be referred to professionals with expertise in treating such
problems, such as the local child and adolescent mental health team
Activities of daily living
Motor, sensory and cognitive impairments resulting from a stroke may affect the child’s ability to engage in age-appropriate self-care, work and leisure activities. These may include activities such as dressing, bathing, toileting and feeding, and the abilities to move around the home or school environment, play and access the school curriculum. Assessment of the child’s ability to perform these activities in the home and school environment is important in facilitating the child’s return to community living.
Guidelines
Therapists working with a child affected by stroke should assess the child’s ability to
perform daily living activities
An occupational therapist should be involved in identifying therapeutic need in self- care, work/school and leisure activities and provision of intervention in this area if
indicated
Longer-term and community care
Return to school
Parent in paediatric stroke workshop: ‘ has been left both physically and mentally damaged! She is unable to keep up with her peers and my once quite bright little girl is now unable to concentrate, take her studies and pass exams.’
It is likely that a child affected by stroke will be out of school for a significant length of time. Whilst the child is an inpatient they may attend the hospital school; once discharged, the home teaching service may be involved in provision of education if they are unable to attend school. Access to education for children and young people with medical needs (DfES 2001) states that ‘all pupils should have access to as much education as their medical condition allows and that each child’s situation should be assessed and addressed in a co-ordinated manner by the child’s school and the agencies mentioned above’.
The timing of the return to school will vary according to the impact of stroke on the individual child. Return to school is a major milestone in the child’s recovery; a positive experience is likely to enhance reintegration. Before the child goes back to school, the local educational authority and school staff should be informed about relevant medical, physical, emotional and cognitive issues. The local education authority is responsible for ensuring that an individually tailored reintegration plan, with multi-agency approval, is in place for all pupils before return to school (Access to education for children and young people with medical needs DfES 2001). Although it is important that professionals familiar with the child are involved in liaison, parents should also be encouraged to raise their concerns. Communication is central and this should involve a named teacher (usually the special educational needs coordinator (SENCO)) and the child’s key worker.
As the child’s physical appearance may have altered as a result of the stroke (for example, if the child has a hemiparesis), staff and pupils should be encouraged to address this in a sensitive manner. The reaction of others to changes in physical appearance is something which many children are apprehensive about and the child should be involved in decisions about how they wish information regarding their illness to be presented to teachers and peers. Children often find it very tiring to return to a full school day after illness, and a graded return to school or the provision of rest periods should be considered. Classrooms are often noisy, with many potential distractions; this may exacerbate problems with attention or concentration. Simple measures, such as changing the child’s position in the
classroom, may be helpful but should only be undertaken after consultation with the child. Formal therapy programmes should be integrated with the child’s school programme.
‘Special educational needs’ are defined as learning difficulties or disabilities which require special educational provision. The Special educational needs code of practice (DfES 2002) and Special educational needs toolkit (DfES 2001) clearly outline the steps to identify, assess and meet children’s special educational needs. If children require additional input in order to access the national curriculum, the first stages of provision are termed ‘School Action’ and ‘School Action Plus’. The aim of School Action and School Action Plus is to develop individual plans for teaching and learning (termed ‘individual education plans’), additional to and different from those usually provided in the child’s educational setting, that will enable the child to access an appropriate curriculum. School Action Plus is introduced when changes for School Action have not had sufficient impact on the child’s progress. If School Action Plus does not provide appropriate or sufficient support to enable the child to access the curriculum, then a referral can be made to the local educational authority for a statutory assessment of the child’s special educational needs, which may lead to a ‘statement of educational needs’. If the child’s difficulties are very significant from the outset a referral for such an assessment can be made to the local educational authority by the school, parent or by any professional at any stage of the treatment.
Guidelines
Child health services, usually community child health services, should take responsibility for informing the local education authority of children who may have special educational needs as soon as possible after the stroke (D)
The child’s key worker should liaise with the special educational needs coordinator at the child’s school prior to school return (D)
A collaborative meeting should be undertaken to plan educational provision with appropriate assessment or support (D)
Health and school staff should agree procedures for communicating information
For children presenting with mobility difficulties, the school environment should be
assessed prior to return to school, ideally by an occupational therapist
It is recommended that all children affected by stroke are placed on a minimum of
School Action (see above) as many difficulties remain latent
Transition between paediatric and adult services
Childhood stroke may result in lifelong physical and cognitive impairments. The process of transition from paediatric to adult services will depend on the current and future needs of the individual, and will usually occur between 16 and 19 years of age. However, the exact timing will depend on the individual’s needs and should be discussed with the young person and family. Key aspects of transition are the transfer of responsibility for health care and the provision of education, training or employment after leaving school. A coordinated approach is critical and advance planning is essential to ensure smooth handover of any and all aspects. The period of transition is often a difficult time for young people and families. Vulnerable aspects of functioning, such as emotion or cognition, may be adversely affected by the stress associated with this if there is inadequate planning and support.
Children with active medical needs will require transfer of health care. These needs may relate to surveillance and management of an active condition (such as cardiac disease or sickle cell disease), as well as to the management of chronic disability. There are different models of transition (for example transfer from paediatric to adult sub-specialist or co- ordination of transfer by the general practitioner (Tuffrey & Pearce 2003)). The most appropriate model should take the young person’s needs, as well as the available services, into consideration. The medical team involved in overseeing the young person’s health care needs should be involved in planning and managing the handover of overall medical care. The Special educational needs code of practice (DfES 2001) provides information on the processes and agencies which should be involved in planning transition after school for young people with special educational needs. It is recommended that a multi-agency transition plan is developed to facilitate successful transition to post-school education, training or work.
If formal medical and therapy interventions have ceased and new problems attributable to the stroke or its aftermath become apparent, the young person’s general practitioner should refer them to an appropriate adult service. The paediatric services previously involved in the young person’s care should provide or share information. Primary health care professionals should be aware of the potentially wide-ranging effects of childhood stroke and, as emphasised elsewhere in this document, that functional difficulties may emerge after a ‘silent’ interval.
Guidelines
Paediatric general and specialty clinics and child development services should have a local policy on transition to adult services, which should be the responsibility of a named person (D)
A named professional should take responsibility for arranging an introduction to adult
health services
A flexible approach to the timing of this transfer needs to be considered which takes into account the young person’s readiness, current health status and links to other social transitions such as leaving school (D)
A multi-agency transition plan should be formulated for young people with special educational needs, with input from health, education and social services, and the young person, to plan transition into further education, training or employment (D)
A named professional should take responsibility for co-ordinating the transition plan and ensuring delivery of services (D)
Primary prevention
As the mechanisms and risk factors for arterial ischaemic stroke in children are not well understood at present, there is no research on effective primary prevention strategies other than in children with sickle cell disease. Although primary prevention of stroke in children in sickle cell disease is possible with regular prophylactic blood transfusion, this has many risks and disadvantages which should be clearly discussed with the child and family.
Guidelines
Children with haemoglobin SS or S° thalassaemia should be screened yearly from the age of three years for internal carotid artery or middle cerebral artery velocity >200 cm/s using appropriately trained personnel and transcranial Doppler ultrasound (B)
Children with sickle cell disease who have internal carotid artery/middle cerebral artery velocity >200 cm/s should be offered long-term blood transfusion (B)
Proposed audit criteria
These criteria have been proposed by the working party for clinical audit. They are divided into two sections – one dealing with acute care and another with longer-term issues. We recognise the importance of patient (child and family) and public involvement in the audit process Due to lack of resources it has not proved possible to include this perspective in the proposed criteria but this will be addressed in future editions.
Acute care
During acute management:
was the child referred to a consultant paediatric neurologist?
if not, was the management of the child discussed with a consultant paediatric neurologist?
How many hours after the onset of acute symptoms did the child undergo brain imaging (includes all children presenting with clinical stroke)?
In children with arterial ischaemic stroke what other imaging studies were carried out and when were these done:
MRI brain
MRA circle of Willis
imaging of cervical vessels (any modality)
cardiac echocardiogram?
When was the first documented assessment (state hours after admission) by:
a nurse
a physiotherapist
an occupational therapist
a speech and language therapist
a psychologist?
Was a member of the community child health team contacted prior to transfer to the community (state number of days before transfer and which professional was contacted)?
During the initial admission, was the family provided with written information regarding:
childhood stroke
statutory and voluntary agencies?
Longer-term care
Is the child under the care of a consultant paediatrician? If so, have they been seen in the last 12 months?
Is the child on treatment for secondary prevention of stroke?
When was the first documented contact (state weeks after transfer home) by:
a key worker
a nurse
a physiotherapist
an occupational therapist
a speech and language therapist
a psychologist?
Was a meeting held between professionals in health and education services and the child’s parents prior to the child’s return to school?
An example of the ICF
The components of the International Classification of Functioning, Disability and Health (ICF) might be used to describe the health of a nine-year-old boy affected by a right hemisphere ischaemic stroke in the following way.
Body functions and structures
Impairments include i) clinical findings of left sided hyperreflexia, hypotonia and persistent posturing of the left foot and ankle, ii) imaging findings of right middle cerebral artery territory infarction.
Functional and structural integrity includes the ability to swallow, speak and stand independently and the absence of neurological signs in the right side.
Activities and participation
Activity limitations include difficulties transferring from chair to standing and negotiating obstacles when walking. Short-term memory difficulties found on formal testing leading to difficulties in maintaining attention in school, thus affecting school grades.
Activities able to be performed independently may include self-feeding with the right hand, writing, managing buttons one-handed, and conversing with friends.
Participation restriction may include inability to rejoin the school football team (a major source of social contact with friends at weekends) due to running difficulties. Parents may report difficulty being sent on errands independently due to poor memory.
Participation that remains unaffected may include playing computer games with siblings, reading with parents, and going for walks with friends in the neighbourhood.
Contextual factors
Environmental: The child must climb two flights of stairs to reach his class every morning at school. He has difficulty walking quickly, especially when carrying a school bag. This is compounded by the distance between classes and the number of children crowding the corridor. The football coach is hesitant to allow the child to rejoin the football team in case
it places him at risk of injury. The boy’s friends welcome him back to school, but he not always able to keep up with playground activities and so is sometimes left to play alone.
Personal: The boy does not want to be singled out so does not agree to leaving class earlier than his peers in order to get to the next class on time. His sense of humour enables him to make friends easily, and thus he widens his social network to include other children for more variety of social contact at school.