Palliative care: moving forward

Children with life-limiting conditions cannot yet expect the same access to specialist palliative care services that is available to adults. On the other hand, the paediatric approach to the care of patients already embodies much of what specialist palliative medicine can offer. Paediatric palliative care must, of course, develop from within paediatrics if we are to meet the needs of children. At the same time, we have much to learn from our colleagues in adult medicine, both corporately and individually. Adolescent palliative care, in particular, is an opportunity for collaboration.Specialist palliative care in children needs to be delivered in many different clinical locations. To achieve this, the consultant must take on an advisory and co-ordinating role as well as giving specialist advice. The exact role of the consultant in paediatric palliative care will depend more on the individual child than on the model. This article considers where we have reached, and where we are going in the development of this new subspecialty.     

Progress in palliative care for children in the UK

Paediatric palliative care has continued to develop as a philosophy of care and as a practical clinical service to children with life-limiting conditions. More doctors and nurses identify themselves as particularly interested in the subject, and there is a more modest increase in those considering themselves to be ‘specialists’. Where even five years ago even the phrase ‘palliative care’ was unfamiliar to many paediatricians, there is now an expectation that a specialist service should be available locally. This expectation is not yet met in practice; paediatric palliative care ‘networks’ have emerged in many parts of the country but, in general, these link existing services rather than expanding the service infrastructure. There are even areas in which innovative palliative care services to children have been cut or are under threat. This contribution examines the progress—and some of the regress—in children’s palliative care over the last five years, and considers some augurs for the future.     

Symptomatic palliative care for children with neurodisability

Paediatric palliative care and neurodisability are two relatively new, evolving paediatric sub-specialities that have increasing relevance in the current paediatric landscape. For many people palliative care has been synonymous with end of life care, but in paediatrics it encompasses much more and is for all children with life-threatening or life-limiting conditions, from the point of diagnosis. This breadth of focus is demonstrated well through the interface between paediatric palliative care and paediatric neurodisability. In this article we explore this unique interface through the three domains of complex symptom management, advanced care planning and end of life care. We describe the practicalities involved in all three areas and highlight the importance of early referral and the process of “dual” or “parallel” planning. We cover in more depth the specific management of the symptoms: dystonia/abnormalities of muscle tone, seizures, pain, agitation, secretions, respiratory failure, and gut failure.     

Paediatric palliative care: distinctive needs and emerging issues

Palliative medicine has developed as a specialized field of practice in recent decades but the focus has been very much on older adults with incurable malignancies. The needs of dying children have not been addressed adequately and the question of who is best placed to provide care to this group remains the subject of some contention. Although the principles of palliative care apply equally to children, a number of fundamental differences influence their application in the paediatric setting. These include a heterogeneous patient population, physiological factors, developmental issues, parental involvement in care giving and decision making and the desire of most paediatricians to maintain close involvement with their patients. Families generally prefer home care and even quite severe symptoms can be managed in this environment with appropriate planning, expertise and support. The delivery of effective palliative care in the paediatric setting is contingent upon overcoming barriers between hospital and community and sharing expertise between paediatricians and palliative care physicians. Research is also required to increase the evidence base for practice.     

Where should paediatric surgery be performed?

We have tried to review the evidence for the organisation of paediatric surgical care. Difficulties arise because of the lack of published data from district general hospitals concerning paediatric surgical conditions. Hence much of the debate about the surgical management of children is based on anecdotal evidence. However, at a time when the provision of health care is being radically reorganised to an internal market based on a system of purchasers and providers it is more important than ever to understand the issues at stake. Two separate issues have been discussed: the role of the specialist paediatric centre and the provision of non-specialist paediatric surgery in district general hospitals. There are arguments for and against large regional specialist paediatric centres. The benefits of centralisation include concentration of expertise, more appropriate consultant on call commitment, development of support services, and junior doctor training. The disadvantages include children and their families having to travel long distances for care, and the loss of expertise at a local level. If specialist paediatric emergency transport is available the benefits of centralisation far outweigh the adverse effects of having to take children to a regional paediatric intensive care centre. Specialist paediatric centres are aware of the importance of treating children and their parents as a family unit as highlighted by the Platt committee; this is an important challenge and enormous improvements have occurred to provide proper accommodation for families while their children are treated in hospital. To keep these arguments of large distances and separation from the home in context, one paediatric intensive care unit in Victoria, Australia, providing a centralised service to a region larger in are than England and with a similar admission rate, has a lower mortality rate than the decentralised paediatric intensive care provided in the Trent region of the UK. There is clear evidence that all neonatal surgery and anaesthesia should be conducted only by specialists. The debate now centres around the number of complex surgical cases a unit should treat to maintain its specialist status. The NHS executive, in its guidelines on contracting for specialist services, emphasises that "Sensible contracting needs to take into account the optimum population size not only for the stability of contracted referrals but also to give sufficient 'critical mass' for clinical effectiveness." Achieving this balance has consequences, not just for the maintenance of surgical expertise, but for the essential ancilliary services. There is clear evidence in anaesthesia that anaesthetists doing small numbers of neonatal procedures had significantly worse results. The same seems to be true in the fields of oncology, radiology, pathology, and intensive care. The reasons why the results of management of certain paediatric conditions are better at specialist centres are open to speculation. Presumably greater exposure to rare complex cases, concentration of expertise, more peer review, and a trickle down effect of the multidisciplinary approach all help to keep health care workers up to date with current world practice. In addition, it allows for appropriate specialist on call rotas and dedicated junior staff. If insufficient numbers of specialist surgical cases are being treated at a centre then the whole multidisciplinary team suffers. The 1989 NCEPOD report states "that paediatricians and general surgeons must recognise that small babies differ from other patients not only in size, and that they pose quite separate problems of pathology and management." The need for large centres of paediatric surgical expertise is now accepted by the Royal College of Surgeons of England, the British Association of Paediatric Surgeons, the Senate of Surgery of Great Britain and Ireland, the Royal College of Paediatrics and Child Health, the Royal College of Anaesthetists, the Audit     

Paediatric palliative medicine in the UK: past, present, future

Like any new specialty, paediatric palliative medicine is facing challenges as it establishes itself. While many of the required core skills have their roots in adult palliative medicine, its practitioners come from a range of paediatric backgrounds that include oncology, community paediatrics, neurodisability and acute pain. Such heterogeneity has been invaluable in bringing together the diverse set of skills and competencies needed by children and families facing life-limiting illness. At the same time, it brings its own challenges in establishing consistent standards of clinical expertise, education and research--essential if children are to have access to the same degree of medical expertise in palliative care already available to most adults. This article traces the origins of palliative care in children, examines its current strengths and challenges, and considers how those might shape its future.     

Predicting death in children

Rarely do paediatric palliative medicine physicians have to break the news of a diagnosis of a life-limiting condition. It is much commoner for us to be faced with the question: "how long?". This cannot be answered with certainty, and yet a great deal may depend on it. While palliative care should ideally be available from diagnosis, the need for "active" practical palliative care intervention will fluctuate during the course of a child's illness, often over months or years, sometimes decades. Typically, there will be several periods during which death seems likely before the final terminal episode, particularly among children with non-malignant life-limiting condition. Optimal management of all episodes depends on anticipating the child's needs, which in turn depends on recognising that such an episode has begun. Providing adequate palliative care critically depends on making a diagnosis of dying. In this article, we will consider why it is important to make a diagnosis of dying, briefly review some of the helpful tools available, and examine some of the evidence from published literature in children and adults.     

Paediatric palliative care in the NICU: A new era of integration

We are entering a new era of integration between neonatal medicine and paediatric palliative care, with increasing recognition that the role and skills of palliative care extend beyond care of only the terminally ill infant. This paper addresses the principles of paediatric palliative care and how they apply in the NICU, considers who provides palliative care in this setting and outlines the key components of care. We consider how the international standards of palliative care pertain to neonatal medicine and how a fully integrated approach to care may be realised across these two disciplines.Palliative care is so much more than end-of-life care, offering a proactive and holistic approach which addresses the physical, emotional, spiritual and social needs of the infant and family. This is a truly interdisciplinary endeavour, relying on a harmonisation of the skills from both the neonatal and palliative care teams to deliver high-quality coordinated care.     

Paediatric home care in the UK.

Paediatric home care services in the UK were ascertained in 1991 and 1992. Respondents from 209 (97%) UK health districts and boards identified 62 general and 124 specialist paediatric home care services by January 1993, 15% having opened in the previous year. Of all UK children, 30% lived in a district with a general home care service. Five health regions had only specialist services. Districts differed widely in the availability of home care for different disorders. The home care services were small, general services employing a mean (SD) of 2.5 (1.6) whole time equivalent (WTE) nurses, and specialist services 1.3 (0.8) WTE nurses. Few services were available 24 hours a day. Funding arrangements were diverse and some services had difficulties in obtaining consumables and equipment for home use. Despite rapid growth there remains considerable scope for the development of paediatric home care throughout the UK.     

Paediatric home care in the UK

Paediatric home care services in the UK were ascertained in 1991 and 1992. Respondents from 209 (97%) UK health districts and boards identified 62 general and 124 specialist paediatric home care services by January 1993, 15% having opened in the previous year. Of all UK children, 30% lived in a district with a general home care service. Five health regions had only specialist services. Districts differed widely in the availability of home care for different disorders. The home care services were small, general services employing a mean (SD) of 2.5 (1.6) whole time equivalent (WTE) nurses, and specialist services 1.3 (0.8) WTE nurses. Few services were available 24 hours a day. Funding arrangements were diverse and some services had difficulties in obtaining consumables and equipment for home use. Despite rapid growth there remains considerable scope for the development of paediatric home care throughout the UK.     

Promoting children’s emotional wellbeing

Promotion of emotional wellbeing should be a priority integral to all paediatric practice. The emotional environment may determine or merely influence children’s needs, but is always relevant.The root of children’s emotional state is the quality of their parental attachments, consideration of which should be routine. Children’s earliest experience of parental relationships determines their preconceptions of themselves and relationships, their foundations of verbal and non-verbal communication, and programming of the stress regulation system. It has a life-long influence on physical and psychological wellbeing, and on growth, development and behaviour.Recognition of the overriding priority for children of adequate attachment should ensure that establishment of secure foundations is a priority of neonatology, and that it is at the core of safeguarding children and support of recovery from neglect and abuse. Consideration of the psychological context of all paediatric problems, and seamless integration of paediatric and specialist mental health services, need to be achieved through training, practice and professional organization.Recognition of the reality of iatrogenic psychological harm should ensure that effort to prevent it is as high a priority as avoidance of physical harm.     

Variations in initial assessment and management of inflammatory bowel disease across Great Britain and Ireland

Background: There are no published data from Great Britain and Ireland detailing the initial management of children with inflammatory bowel disease (IBD). Aims: To prospectively record the initial investigation and treatment of children aged less than 16 years with newly diagnosed IBD. Methods: For 13 months, between June 1998 and June 1999, 3247 paediatricians, adult gastroenterologists, and surgeons across the UK and Ireland were prospectively surveyed each month and asked to report every newly diagnosed case of childhood IBD. Reporters subsequently completed a postal questionnaire about each case. Results: A total of 739 new IBD cases were reported from 172 institutions. Significant variations were observed in the investigation and treatment of these cases, when examined by number of cases reported per institution, or by the specialists providing care. There were wide regional variations in the proportion of children having access to paediatric gastroenterology services. Overall, one third of children received care from an adult service, and a tenth care exclusively from an adult gastroenterologist. Children with Crohn''s disease who had some or all of their care from adult services were more likely to receive systemic steroids and less likely to receive dietary therapy; those with ulcerative colitis were more likely to receive rectal steroids and to have surgery. Height and weight were also less likely to be recorded in those whose care involved adult services. Conclusion: Current specialist provision, and initial investigation and treatment of IBD, is heterogeneous. Optimisation of care is likely to be achieved by greater access to specialist paediatric gastroenterology services for all those with suspected IBD.     

Diabetes services in the UK: third national survey confirms continuing deficiencies.

AIMS: To determine the current level of diabetes services and to compare the results with previous national surveys. METHODS: A questionnaire was mailed to all paediatricians in the UK identified as providing care for children with diabetes aged under 16 years. Information was sought on staffing, personnel, clinic size, facilities, and patterns of care. Responses were compared with results of two previous national surveys. RESULTS: Replies were received from 244 consultant paediatricians caring for an estimated 17 192 children. A further 2234 children were identified as being cared for by other consultants who did not contribute to the survey. Of 244 consultants, 78% expressed a special interest in diabetes and 91% saw children in a designated diabetic clinic. In 93% of the clinics there was a specialist nurse (44% were not trained to care for children; 47% had nurse:patient ratio >1:100), 65% a paediatric dietitian, and in 25% some form of specialist psychology or counselling available. Glycated haemoglobin was measured routinely at clinics in 88%, retinopathy screening was performed in 87%, and microalbuminuria measured in 66%. Only 34% consultants used a computer database. There were significant differences between the services provided by paediatricians expressing a special interest in diabetes compared with "non-specialists", the latter describing less frequent clinic attendance of dietitians or psychologists, less usage of glycated haemoglobin measurements, and less screening for vascular complications. Non-specialist clinics met significantly fewer of the recommendations of good practice described by Diabetes UK. CONCLUSIONS: The survey shows improvements in services provided for children with diabetes, but serious deficiencies remain. There is a shortage of diabetes specialist nurses trained to care for children and paediatric dietitians, and a major shortfall in the provision of psychology/counselling services. The services described confirm the need for more consultant paediatricians to receive specialist training and to develop expertise and experience in childhood diabetes.     

Diabetes services in the UK: third national survey confirms continuing deficiencies

Aims: To determine the current level of diabetes services and to compare the results with previous national surveys. Methods: A questionnaire was mailed to all paediatricians in the UK identified as providing care for children with diabetes aged under 16 years. Information was sought on staffing, personnel, clinic size, facilities, and patterns of care. Responses were compared with results of two previous national surveys. Results: Replies were received from 244 consultant paediatricians caring for an estimated 17 192 children. A further 2234 children were identified as being cared for by other consultants who did not contribute to the survey. Of 244 consultants, 78% expressed a special interest in diabetes and 91% saw children in a designated diabetic clinic. In 93% of the clinics there was a specialist nurse (44% were not trained to care for children; 47% had nurse:patient ratio >1:100), 65% a paediatric dietitian, and in 25% some form of specialist psychology or counselling available. Glycated haemoglobin was measured routinely at clinics in 88%, retinopathy screening was performed in 87%, and microalbuminuria measured in 66%. Only 34% consultants used a computer database. There were significant differences between the services provided by paediatricians expressing a special interest in diabetes compared with "non-specialists", the latter describing less frequent clinic attendance of dietitians or psychologists, less usage of glycated haemoglobin measurements, and less screening for vascular complications. Non-specialist clinics met significantly fewer of the recommendations of good practice described by Diabetes UK. Conclusions: The survey shows improvements in services provided for children with diabetes, but serious deficiencies remain. There is a shortage of diabetes specialist nurses trained to care for children and paediatric dietitians, and a major shortfall in the provision of psychology/counselling services. The services described confirm the need for more consultant paediatricians to receive specialist training and to develop expertise and experience in childhood diabetes.     

A family clinic—optimising care for HIV infected children and their families

A family clinic providing specialist paediatric and adult medical, testing, counselling, and terminal care services for families living with HIV was set up at a paediatric tertiary care hospital in London in 1991. During the first five years, until April 1996,185 children from 149 families attended, including 119 infected children, of whom 32 have died. Only 5% of mothers were born in the UK; the rest were born in 24 different countries, the majority in sub-Saharan Africa. Less than a quarter of children were cared for by both parents, 61% by mothers alone, and 11% by guardians or foster parents. Of the adult attendees, 76% were women, and more than half were untested when they first attended the clinic. Provision of a family planning service within the family clinic was initiated as a result of women presenting with unplanned pregnancies. Shared care with local clinics is increasing, but with the complexity around the management of paediatric HIV infection, particularly with regard to antiretroviral treatments, there is need for continued specialist input. Coordination among specialist and locally based family services is required to provide flexible, accessible, and up to date care for families living with HIV infection in London.     

A family clinic--optimising care for HIV infected children and their families

A family clinic providing specialist paediatric and adult medical, testing, counselling, and terminal care services for families living with HIV was set up at a paediatric tertiary care hospital in London in 1991. During the first five years, until April 1996, 185 children from 149 families attended, including 119 infected children, of whom 32 have died. Only 5% of mothers were born in the UK; the rest were born in 24 different countries, the majority in sub-Saharan Africa. Less than a quarter of children were cared for by both parents, 61% by mothers alone, and 11% by guardians or foster parents. Of the adult attendees, 76% were women, and more than half were untested when they first attended the clinic. Provision of a family planning service within the family clinic was initiated as a result of women presenting with unplanned pregnancies. Shared care with local clinics is increasing, but with the complexity around the management of paediatric HIV infection, particularly with regard to antiretroviral treatments, there is need for continued specialist input. Coordination among specialist and locally based family services is required to provide flexible, accessible, and up to date care for families living with HIV infection in London.     

Paediatric palliative medicine: a unique challenge

For more than thirty years, adults suffering from life-threatening or terminal conditions have been able to access specialist skills in palliative medicine. It has taken much longer for children to start to have access to the same expertise. Paediatric palliative medicine adopts a robust and rational approach to symptom control, based on a thorough understanding of pathophysiology and therapeutics. At the same time, it recognises that physical issues are only one aspect of a child's quality of life and, by adopting a multi-dimensional approach, aims to address psychosocial and spiritual or existential concerns.     

Variations in initial assessment and management of inflammatory bowel disease across Great Britain and Ireland.

BACKGROUND: There are no published data from Great Britain and Ireland detailing the initial management of children with inflammatory bowel disease (IBD). AIMS: To prospectively record the initial investigation and treatment of children aged less than 16 years with newly diagnosed IBD. METHODS: For 13 months, between June 1998 and June 1999, 3247 paediatricians, adult gastroenterologists, and surgeons across the UK and Ireland were prospectively surveyed each month and asked to report every newly diagnosed case of childhood IBD. Reporters subsequently completed a postal questionnaire about each case. RESULTS: A total of 739 new IBD cases were reported from 172 institutions. Significant variations were observed in the investigation and treatment of these cases, when examined by number of cases reported per institution, or by the specialists providing care. There were wide regional variations in the proportion of children having access to paediatric gastroenterology services. Overall, one third of children received care from an adult service, and a tenth care exclusively from an adult gastroenterologist. Children with Crohn's disease who had some or all of their care from adult services were more likely to receive systemic steroids and less likely to receive dietary therapy; those with ulcerative colitis were more likely to receive rectal steroids and to have surgery. Height and weight were also less likely to be recorded in those whose care involved adult services. CONCLUSION: Current specialist provision, and initial investigation and treatment of IBD, is heterogeneous. Optimisation of care is likely to be achieved by greater access to specialist paediatric gastroenterology services for all those with suspected IBD.     

Kinderpalliativmedizin

Every year 3,500 children die in Germany from severe diseases. Since 1998 pediatric hospices and home hospice services, specialized palliative home care teams and one inpatient unit for pediatric palliative care have been established. In close collaboration with pediatricians in private practice and children??s hospitals, the multi-professional teams take care of the physical, psychosocial and spiritual needs of the children and their families. For symptom control, non-pharmacologic and drug-based interventions are employed synergistically. The aim of pediatric palliative care is to ease the suffering and to improve the quality of life of the child and its social environment. The burden and benefit of every examination and treatment must be thoroughly weighed with respect to the individual therapeutic goal.     

Dental disease in children with chronic illness.

This article aims to raise awareness among paediatricians and specialist paediatric services that poor oral health, in particular children with chronic illness, is a major cause of morbidity and can be a risk factor for severe, even life threatening complications. Good oral health and dentition is important for efficient mastication, speaking and of course, cosmetically for smiling. If left untreated, dental caries can lead to pain and infection. Chronic infection around one or more teeth can result in damage to localised structures, such as the developing permanent teeth. Children who are medically compromised (such as being immunocompromised from disease and/or therapy) however are at increased risk of developing systemic complications from dental infections, which may prove fatal. We focus on the role of the general paediatrician in promoting the importance of good dental health for all children and in particular those children "at risk". We present preventive measures, evidence based where available, that may improve dental care and promote the role of paediatric dental services in the multidisciplinary management of chronic disease.