Chapter 24. Children with complex motor disability
Jackie Parkes and Sonya Clarke
ABSTRACT
This chapter is divided into two broad parts. The first part describes the wider context related to children with complex motor disability – the numbers of children with severe disability, sources of information, the impact of having a child with a complex disability, the aims of services and parents’ expectations and need for those services. Reference is made to where the children’s nurse ‘fits in’ to the wider service context. The second part of the chapter deals with a small number of care needs that are commonly present in children with complex motor disability, and includes a description of a nursing assessment and possible interventions related to these problems.
LEARNING OUTCOMES
• Appreciate the perspectives of individual level and population-based approaches to assessment and planning care for children with complex motor disability.
• Describe the leading causes of complex motor disability in childhood.
• Recognise the relative contribution to be made by the medical and social models of disability.
• Be able to locate and interpret information (routine and other) on children with complex motor disability in the UK.
• Recognise the central role of parents as experts in the management of children with complex disability.
• Understand the role of the children’s nurse in identifying unmet need through competent nursing assessment of the child with complex motor disability and their family.
• Be able to critically apply proposed outlines for nursing assessment in the care of the child with complex motor disability and their families.
Introduction
The aim of this chapter is to focus on the care of the child with complex motor disability, and on the issues faced by their families, in the context of community and hospital. The chapter uses both an individual-level and a population-based perspective to explore both the needs of children with complex motor disability and the needs of their families. An individual level approach focuses on the needs and unique experiences of an individual – a child, his or her parents and siblings. This perspective is important in developing an individualised assessment of need and tailored plan of nursing care. It contrasts with a population-based approach, which considers the needs and experiences of groups of children and their families with similar conditions and usually living in the same geographic area (for example in a Health and Social Services Trust or one region of the UK). This perspective is important for prevention of disease, health promotion and planning and targeting services to those in greatest need. The children’s nurse has a role to play at both levels of assessment and intervention in relation to child health and more specifically with regard to children with complex motor disability.
Definitions, numbers, needs and services
Definition of complex motor disability
The term ‘complex motor disability’ is interpreted here to mean children whose primary problem is a significant disorder of movement and whose condition is affected and complicated by the presence of other associated impairments such as sensory deficits, problems with learning, communication or active seizures. Cerebral palsy is a good example of such a condition and most children with cerebral palsy have at least one additional, associated impairment in conjunction with problems related to movement. Cerebral palsy can be severe: approximately one in three children are unable to walk and one in four have no useful arm or hand function (Parkes et al 2001a). The impact of disability experienced is further increased by the presence of other severe associated impairments: one in four children with cerebral palsy have severe learning disability (IQ < 50) and one in ten have no useful vision (Parkes et al 2001a).
In addition, some children with complex motor disability will depend on technology to sustain their lives, for example requiring assisted ventilation, artificial feeding or care associated with having a colostomy, ileostomy, urostomy or catheterisation (Glendinning et al 2001). It should be noted that the terms ‘complex motor disability’, ‘complex healthcare needs’ and ‘technology-dependent children’ are not interchangeable, although many of the same children are common to all three groups.
Leading causes of motor disability
The numbers of children with disabilities and conditions requiring medical intervention has increased in the last 30 years (Gordon et al., 2000 and Kirk, 1999). Advances in medical care have helped to improve and prolong the life expectancy of some babies and children who would have previously died (Pharoah et al 1996). For example, neonatal intensive care has helped to improve the survival of extremely premature or low birthweight babies, who are at particularly high risk of developing problems like cerebral palsy (Johnson & King 1998, O’Shea et al., 1998 and Pharoah et al., 1996). Also, interventions like chemotherapy and ventilation, now available in the community, have improved the life span of children with degenerative conditions.
Although it is important for the children’s nurse to appreciate the underlying medical diagnoses and pathology of complex motor disabilities, medical diagnosis alone does not predict service needs (Gordon et al 2000, World Health Organization (WHO) 2001). For this reason, only a short overview of medical causes is presented here.
Activity
ActivityFor a comprehensive overview of causes, complications and resulting conditions leading to complex physical disability, see McCarthy et al (1992) pp 18–21.
The leading causes of complex motor disability that may give rise to complex needs in children include cerebral palsy, spina bifida, Duchenne muscular dystrophy and other degenerative and neuromuscular conditions like spinal muscular atrophy. Cerebral palsy is the leading cause of physical disability in childhood (Parkes et al 2001a, Surveillance of Cerebral Palsy in Europe (SCPE) 2000) and has been defined as ‘an umbrella term covering a group of non-progressive but often changing motor impairment syndromes secondary to lesions or anomalies of the brain arising in the early stages of its development’ (Mutch et al 1992 p 549). A number of rare metabolic disorders (e.g. Lesch–Nyhan syndrome) and genetic syndromes (e.g. Turner syndrome) can also result in neuromotor impairments and disability. For some children, the underlying medical diagnoses remain uncertain.
Some of these conditions will invariably lead to premature death (e.g. Duchenne muscular dystrophy), whereas others are likely to mean the child and family will have a life-time of disability requiring specialist help and support (e.g. cerebral palsy). Although conditions like cerebral palsy and spina bifida are not fatal per se, premature death is associated with more severe forms (Crichton et al 1995, Hutton et al 1994). It has been estimated that of those with the most severe disabling cerebral palsy (unable to walk, no useful arm or hand function and with intellectual impairment) 70% will survive to age 10; 50% to age 20; and 40% to 30 years (Hutton & Pharoah 1998).
Medical and social models of disability
As children’s nurses we need to be aware and appreciate the ‘larger’ issues around disability as a political, social and emerging academic discipline in its own right. Generally speaking, we are taught and tend to assume the ‘medical model’ in assessing and intervening in the care of children with disability and their families. The medical model supposes that disability is the result of pathology and impairment, resulting in deficit of function and rendering the person disabled and unable to participate fully in ‘normal’ life. Our interventions are aimed at the level of the individual and include pre- and postoperative surgical care, medication, rehabilitation and the provision of aids and appliances to help the individual adapt and ‘fit into’ mainstream society. Although it could be argued that this perspective is at least partially appropriate, the contrasting social model of disability has gained increasing validity and recognition.
The social model of disability conceives that impairments do not make a person disabled but rather that it is society who disables people by failing to accommodate their differences (Priestley 2003). The manifestation of this ‘failure to accommodate’ means that people with disabilities are excluded from mainstream life through segregated education, few and poorly paid employment opportunities, and difficulty gaining access to buildings and using public transport, among other things (Priestley 2003). In the social model of disability the level of intervention is aimed at changing societal attitudes and societal behaviour towards people with disability.
The distinction between these models is not merely an academic one because how we think about disability will influence our nursing practice. The children’s nurse has a clear responsibility to provide nursing care and meet the immediate physical and psychological needs of the child and family. We also have a wider role to play.
Seminar discussion topic
Seminar discussion topicCan children’s nurses act as advocates at the societal level, raising awareness about inclusion for children with disability in ‘mainstream’ society and helping to change attitudes?
The World Health Organization and disability
In 1980, the World Health Organization (WHO) published the much cited International Classification of Impairments, Disabilities and Handicaps (ICIDH). This defined impairment as ‘any loss or abnormality of psychological, physiological or anatomical structure or function’; disability as ‘any restriction or lack (resulting from impairment) of ability to perform an activity in the manner within the range considered normal for a human being’; and handicap as ‘a disadvantage for a given individual, resulting from an impairment or a disability, that limits or prevents fulfilment of a role that is normal’.
This framework has now been revised to form a more positive ‘components of health’ classification called the International Classification of Functioning, Disability and Health (or ICF; WHO 2002). Previously, the ICIDH was a ‘consequences of disease’ classification that focused on the impact of disease on the individual (WHO 1980). By contrast, the ICF focuses on health and measuring function regardless of the reason for the impairment (WHO 2002). The terms and concepts of ‘disability’ and ‘handicap’ have now been replaced by the terms ‘activity limitations’ and ‘participation restrictions’, respectively.
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wwwRead a beginner’s guide to the ICF called ‘Towards a common language for functioning, disability and health’ online at:
Click on ‘Beginner’s Guide’. In particular, see p 6 regarding ICF applications in service provision. Alternatively, for a more detailed account click on ‘Introduction’.
The ICF provides a conceptual framework for the ‘definition, measurement and policy formulations for health and disability’ (WHO 2002 p 2) for use in health care and at the levels of individual, institution and population. Finally, one of the additional unique features of the ICF is recognition and classification of the environmental factors (such as social attitudes and social structures), which are derived from the social model of disability. Assessment of these features is necessary to complete the ‘picture’ of health and disability.
In the context of children’s nursing it is important to appreciate the ICF because it is relevant and could be used in nursing practice in the following ways (adapted from WHO 2002):
Reflect on your practice
• To provide standardised descriptions and a conceptual framework of health and health-related states for individual children and their families. For example, it could be used in relation to assessing and planning care for children with complex motor disability.
• Use of standardised language to promote interdisciplinary communication (with physiotherapists, occupational therapists, paediatricians for example) and make comparisons about health, health care and health outcomes in children with complex needs within geographic areas and between geographic areas.
• More generally, to provide reliable summary measures of population health for surveillance purposes and monitoring health outcomes.
• The ICF promotes assessment of the ‘contextual factors’ or barriers that limit the participation of individual children with complex motor disability or populations of children with complex motor disability and their families.
• It provides a systematic coding scheme for health care information systems.
Reflect on your practiceWhat would be the advantages and disadvantages of including the ICF as part of routine nursing assessment in the care of children with complex motor disability?
Sources of information on children with complex motor disability
Information about the numbers of children with complex motor disability or other forms of disability have been described as ‘invisible’ from the routine statistics published by government (Gordon et al 2000). Most regions within the UK have a formal, computer-based system for monitoring child health – a child health surveillance system. The sorts of information recorded on child health surveillance systems include birth notifications, immunisations, preschool health checks and the identification of any special needs. These systems tend to be orientated towards provision of services and have limited use as complete sources of information on specific conditions like cerebral palsy (Parkes et al 1998). Furthermore, these routine information systems could, but generally do not, publish information on children with complex needs as part of routine statistics.
Some of the gaps in the routine information about children with special and complex needs have been filled by ad hoc (‘one-off’) surveys carried out in Great Britain and Northern Ireland. In Great Britain, the Office of Population Censuses and Surveys conducted surveys of childhood disability in 1985 and 1988 (Bone & Meltzer 1989), which were subsequently reanalysed by Gordon et al (2000). Glendinning et al., 1999 and Glendinning et al., 2001 also reported on the numbers of technology-dependent children living in the UK; Beresford (1995) on the numbers of parents caring for a severely disabled child at home (in England) and Roberts & Lawton (2001) on severely disabled children whose families have accessed the Family Fund.
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wwwThe Family Fund Trust is an independent organisation that provides financial support and information services to families caring for a severely disabled or ill child (under 16 years of age). The Family Fund Trust is government funded. Read more about the Family Fund at:
In Northern Ireland, sister OPCS surveys were conducted by the Policy, Planning and Research Unit in 1989 and 1990 (Duffy, 1995 and Smith et al., 1992). A survey was undertaken in Scotland to identify the numbers and service needs of children with motor impairment and their families (Gough et al 1993). The results of these surveys are summarised in Table 24.1. Some caution must be used in the interpretation of Table 24.1 because differences between surveys will be partly due to differences in the way ‘disability’ and ‘severe disability’ have been defined. It should be noted that the impact of disability may increase with age and studies of disability need to take this into account.
| Author and year | Geographical area | Definition used | Numbers and proportions of children with a disability (including children with severe/multiple problems where known) |
|---|---|---|---|
| OPCS Bone & Meltzer 1989 | England, Wales, Scotland | ‘Disabled’ as defined by the ICDIH (WHO); aged 0–15 years | 360,000 disabled children (3.2% of the child population in the UK) 327,000 disabled children in England & Wales |
| OPCS Gordon et al 2000 | As above | Re-analysed OPCS data and redefined disability and identified clusters of disability | 45,000 children with the most severe and multiple disabilities |
| PPRU Smith et al 1992, Duffy et al 1995 | Northern Ireland | ‘Disabled’ as defined by the ICDIH (WHO); aged 0–15 years | 14,600 disabled children (3.5% of the child population in NI) 2600 ‘severely disabled’ |
| Gough et al 1993 | Scotland | Motor impairment of central origin born 1985–86 and resident in Scotland 1991–92 | 244 children with a motor impairment of central origin (0.3% of child population in Scotland) Two-thirds had moderate–severe disability in relation to personal care; more than half had severe learning disability |
| Beresford 1995 | UK (using the Family Fund database) | Been helped by the Family Fund and been in recent contact with the Family Fund; children < 16 years | Collected data on 1100 families caring for a severely disabled child Estimate 150,000 families are caring for a severely disabled child |
| Glendinning et al 2001 | UK | Technology-dependent children | Estimates 6000 technology-dependent children in the UK (although some double counting may have occurred) 2800 children receiving artificial feeding (British Artificial Nutrition Survey cited by Glendinning et al) |
Some longitudinal studies of childhood motor disability do exist in the form of dedicated case registers of children with cerebral palsy. Currently, five such registers are actively recording cases in Northern Ireland, parts of England and Scotland. These registers fulfil an important function in providing unique information about the prevalence, severity and survival of children and young adults with cerebral palsy in the UK. The registers are also used to monitor trends over time and between areas and act as a sampling frame for research into causation and services. Table 24.2 summarises some of the literature on the numbers and prevalence of children with cerebral palsy in different parts of the UK, including information from the five registers referred to above.
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wwwThe five registers of children with cerebral palsy in the UK are part of a working collaboration. Read about the UK Collaboration of Cerebral Palsy Registers online at:
| Reference (alphabetical) | Geographical area | Method | Birth years | Number of children | Rate per 1000 live births |
|---|---|---|---|---|---|
| Colver & Mackie 1998 | North East of England | Case register | 1991–93 | 287 | 2.4 |
| Edmond et al 1989 | Great Britain | British Perinatal Mortality & Birth Survey | 1958, 1970 | 40, 41 | 2.5 |
| Ingram 1955 | Edinburgh | Ad hoc survey | 1938–52 | 208 | 1.9 |
| Jarvis et al 1985 | North East of England | Case register | 1960–75 | 421 | 1.6 |
| Johnson & King 1998 | Oxford | Case register | 1984–91 | 697 | 2.5 |
| MacGillivray & Campbell 1995 | Avon | Taken from a handicap register | 1969–88 | 489 | 2.1 |
| Parkes et al 2001b | Northern Ireland | Case register | 1981–93 | 784 | 2.2 |
| Pharoah et al 1990 | Mersey | Case register | 1980–84 | 309 | 1.9 |
| Pharoah et al 1998 | Mersey, Oxford & Scotland | Case registers | 1984–89 | 1649 | 2.1 |
| Williams et al 1996 | North East Thames | Case register | 1980–86 | 672 | 1.6 |
Although it could be argued that disability and complex motor disability remains relatively rare, the following extract written by a parent clearly highlights the impact on the child and family and their need for ongoing, intensive support to cope.
The impact of having a child with complex motor disability
The following ‘real life scenario’ written by a parent provides a unique and special insight into the parent’s feelings and experiences of having a child with complex needs. It is strikingly similar to other accounts by parents who have reiterated many of the same messages (see Beresford, 1994 and Kingdom and Mayfield, 2001).
In the account presented here, the parent does not elaborate on the precise ‘medical equipment’ she uses. However Beresford (1995) identified the following equipment being used by 1100 families for a severely disabled child at home: nasal feeding tubes (18%), suction equipment (16%), nebulisers and inhalers (10%), gastric feeding tubes (12%), a feeding pump (12%), catheters (7%), oxygen (9%), ventilators (7%), other things (14%). Clearly, parents have to develop advanced clinical skills to look after their child at home. The children’s nurse has a clear role to play in teaching families these skills, sourcing and providing equipment, anticipating and preventing potential problems and supporting parents at home to provide the highest quality care.
Scenario
ScenarioReal-life scenario by a parent of a child with complex motor disability
My son Daniel [a pseudonym] was born in 1998 and what should have been a wonderful event became a tragedy. Daniel has athetoid cerebral palsy. He is now 41⁄2 years old and is a 24-hour job.
Apart from physical exhaustion looking after a handicapped child, the mental torment is nearly worse. Watching your child day to day and wondering if he will see another one or if the next illness will be the last and you have to say ‘goodbye’.
The needs of the child are great and this can be where health services mainly focus. However, the needs of the parent often fall by the wayside. Practical help is required from the start in the form of advice, supplies and respite care. I ‘fell’ through the net and did not receive any real help until my son was 21⁄2 years old. Daniel was then assigned a community nurse who realised our plight and set out to help. She organised all supplies, including nappies and medical equipment. Something as simple as this eased the load and was something less to worry about. She was excellent for advice on Disability Living Allowance and Motability transport. She also arranged hospice and respite care, which are wonderful, and tried to make life that little bit easier.
Too many parents are struggling on their own with limited information and resources as all too often one parent has to give up work to be the carer. This is what happened to me. There are few day-care facilities who can deal with a child with a nasogastric tube, a tracheostomy and epilepsy.
Emotional support is rare. It does not have to be an expensive organisation. Someone to talk to, have a coffee with and who does not mind if you have a good cry to get things off your chest, is often all that is required, but it is not out there. If it does exist, many parents are not told.
This might all sound like ‘me, me, me’, however to give Daniel a good life, I have to be strong mentally as well as physically. To put Daniel in care if I have a breakdown would cost approximately £700 per week. Me caring for my son is free.
I cannot describe the mental torture of knowing my son will die. Only a mother who walks a day in my shoes could possibly understand the constant struggle for help and advice. The situation has not improved a great deal in the last 20 years.
Aim of services
In 1991 the UK government ratified the United Nations Convention on the Rights of the Child (United Nations General Assembly 1989) and by doing so undertook to commit to the principles laid out by the Convention. Several articles within the Convention are relevant to children with disabilities (Articles 2, 3 and 12). More specifically, Article 23 states:
… a mentally or physically disabled child should enjoy a full and decent life, in conditions which ensure dignity, promote self-reliance, and facilitate the child’s active participation in the community.
Article 23 should form the basis for providing services and interventions to the child with complex motor disability and his or her family. The overall aim of services to children with complex motor disability must also include early detection (and prevention where possible), early intervention and family support. The ultimate aim for all children with health or special needs must be to help them achieve the maximum potential and the best quality of life possible:
Seminar discussion topic
Children with profound and multiple disabilities and/or complex and continuing health care needs should enjoy the highest quality of life possible, receiving quality health, social and educational services which meet their needs and the needs of those who care for them, and respect their lifestyle and culture (Ward et al 2003)
Seminar discussion topicUse the real-life scenario presented above and the account provided by Kingdom & Mayfield (2001):
• What issues or ‘patient’ problems can you identify? Distinguish between child-centred problems and parent-centred problems.
• To what extent do the parents’ experiences and perceptions match the aims of services for children with disability specified above?
• Discuss the role of the children’s nurse in addressing each of the issues or problems identified.
• What other professionals might the children’s nurse interact with to help ensure the needs of this child and family are met?
Families’ experiences of services
As we have already heard from parents, help and support is available from a wide range of services and professionals, some of whom focus directly on helping the child, whereas others concentrate on assisting the parents and family. Sloper & Turner (1992) found that parents of severely disabled children were in contact with a large number of professionals, as many as ten in any 1 year, with a range of 5–17. Similarly, in a study of children with moderate to severe cerebral palsy, Parkes et al (2002) also found that families were in contact with an average of seven service providers in a 6-month interval, range 1–13. Not surprisingly, parents of children with disability have reported problems related to coordination of services and fragmentation of care. Bamford et al (1997) described this as ‘deficiencies in the total care concept’.
Sloper & Turner (1992) found that many parents reported that they had encountered problems in finding and coordinating services, particularly in the early days after diagnosis, as reported elsewhere (Bamford et al., 1991, Haylock et al., 1993 and Watson et al., 2002). In particular, parents have reported that professionals often duplicate work and seem unable to detect where the gaps in services are (Rees 1983). Haylock et al (1993) found school (i.e. special education) offered families a form of integration of care that they very much appreciated; again, this has been reported elsewhere (McConachie et al 1999).
The inadequacies of respite care for families with a child with special needs, both in terms of quantity and quality of services available, have been repeatedly identified in the literature (Beresford, 1994, Beresford, 1995, McConkey and Adams, 2000 and Stalker, 1990). The children’s nurse has a particular role to play here in terms of finding respite facilities, giving families good information and helping and preparing them psychologically to avail of services (Miller, 2002 and Ross and Parkes, 2004). Families with a child with complex problems that require nursing care (e.g. problems with active seizures requiring medication) may find it more difficult to find respite from both the formal and informal sector because of their child’s specialist requirements and the level of responsibility required of carers (Beale, 2002 and Parkes, 1998). Families of children with severe and complex needs are at higher risk of stress and feelings of being unable to cope (Sloper & Turner 1993).
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wwwRead about a model of good practice in relation to providing holistic care for children with complex healthcare needs and their families. See the Children’s Trust, Tadworth, Surrey, a charitable organisation providing specialist care, therapies and education for children with severe disabilities, at:
There are many examples of good practice in the care of children with complex motor disability both in the literature and in our local communities. Ross & Parkes (2004) outlined a model of good practice for the children’s community nurse in relation to the care of a child with complex motor disability and the child’s family. This paper highlights the unique role that the children’s nurse can play in coordinating a multidisciplinary response that helps to ‘cut down’ excessive visits by numerous professionals while at the same time tailoring services to meet with the child and family’s individual needs.
A number of ‘care interfaces’ and transitional phases may exist for the child with complex motor disability and their family, which the children’s nurse must be aware of and anticipate. These interfaces exist where the child and family ‘move’ from one service arrangement to another, for example from community to hospital or from child to adult services, as a result of changing need or developmental stage. These interfaces and transitional phases are times when the child and family may need extra help and support to cope and where service providers are ‘at risk’ of failing due to inadequate planning, poor cooperation or coordination between multiagencies.
The children’s nurse in collaboration with the child’s paediatrician, the family and other members of the multidisciplinary team, can act as coordinator of health and social care services and ensure that adequate advance planning takes place before discharge from hospital or transfer to another setting. Children with complex needs often receive hospital services at a number of hospital locations (McConachie 1997) and this highlights the importance of children’s nurses communicating with one another across institutional boundaries.
What families want from services
Based on research involving families with a disabled child, Baldwin & Carlisle (1994) identified the attributes of a quality service for children with disability. A number of their findings have been supported from research conducted by others. The results have implications for children’s nursing practice and are summarised below.
Evidence-based practice
Evidence-based practiceThe following has been taken from Baldwin & Carlisle (1994), whose findings are supported by other research (the references of which are included here). Families with a disabled child want:
• an individual, comprehensive assessment of the needs of each child and family. Sloper & Turner (1992) also found that services that focus purely on the child’s needs may put the family under more pressure
• regular review and assessment of need, particularly at times of transition. Hirose & Ueda (1990) identified the time of diagnosis, starting school, changing school and leaving school as times of stress for families with a child with disability
• an opportunity to make informed choices about services
• involvement in the design and delivery of a package of services
• a ‘key worker’ for each child and family, who maintains close contact, acts as a link person between the different agencies involved in their child’s care, acts as an advocate and represents their views (Audit Commission, 1994, Beresford, 1994 and Sloper, 1999).
Research evidence additional to Baldwin & Carlisle’s recommendations suggests that families need regular breaks that are tailored to their individual requirements (Beresford, 1994 and McConkey and Adams, 2000).
For a more detailed account of the evidence regarding service models to support parents of disabled children, see Sloper (1999).
Setting standards: the children’s national service framework
In 1998, the government launched a programme of National Service Frameworks (NSFs) for a number of key patient groups with the aim of ‘driving the modernisation agenda’ within the NHS. The aims of the NSFs are to set national standards for key interventions, develop strategies to support the implementation of interventions and to increase quality and decrease variation in service provision. An outline of the development of the Children’s NSF is provided by Smith (2003). The Standards for Hospital Services is the first part of the Children’s NSF and is available online.
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wwwFind the latest information on the development of the Children’s National Service Framework, including a downloadable copy of the Standards for Hospital Services, at:
There is also a Children’s NSF Bulletin Getting the right start, which is available at the same website address.
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