10 - Children expressing themselves | Oxford Textbook of Palliative Care for Children (Liben, Oxford Textbook of Palliative Care for Children)

Editors: Goldman, Ann; Hain, Richard; Liben, Stephen

Title: Oxford Textbook of Palliative Care for Children, 1st Edition

> Table of Contents > Section 2 - Child and family care > 9 - Adolescents and young adults

Adolescents and young adults

Finella Craig

Young people with palliative-care needs form a distinct group with physical, emotional, psychological, and social needs that are significantly different from those of adults and children. They are neither large children nor small adults , but are in a continuum, during which their needs as children are evolving into those of adults. This evolution usually occurs between the ages of 13 24 years.

Better supportive care for conditions that manifest in childhood means that there are increasing number of young people surviving into early adulthood. The prevalence of life-limiting or life-threatening disorder in this group is estimated at 6000 10,000, with an annual mortality of 1.7 per 10,000 [1]. These young people have a wide spectrum of diseases and disorders, some presenting in early childhood (e.g. genetic and congenital conditions) and others apparent later in childhood or adolescence. Many reach a crisis in terms of physical deterioration in adolescence or early adulthood and many die in their late teens or early twenties. Within this group patients demonstrate a range of developmental levels and cognitive abilities, social, and emotional maturity, such that service provision for adolescents is a very distinct area of clinical health care.

Life-limiting and life-threatening conditions affecting young adults

The spectrum of life-limiting and life-threatening disorders encountered in young adults is considerable. While there is overlap with conditions encountered in the paediatric and adult populations, many conditions take on increasing significance in the adolescent years, as a consequence of natural disease progression, because of the physical and emotional changes of adolescence or simply because the child has outlived all expectations.

Most of the conditions encountered fall into one of the four groups identified in Table 1 (based on [1].)

Table 1 Life-limiting and life-threatening conditions affecting young adults

Group	Examples
Conditions where curative treatment is feasible but may fail	Cancer Organ failure for example, kidney, heart, liver
Conditions where there may be long periods of intensive treatment aimed at prolonging life, where premature death is still possible or inevitable	Cystic fibrosis Muscular dystrophies HIV/AIDS Some congenital heart diseases Pulmonary hypertension
Progressive conditions without curative options, where treatment is exclusively palliative	Batten disease Mucopolysaccharidoses Cruetzfeldt-Jakob disease
Severe neurological disability, which may cause weakness and susceptibility to health complications	Severe multiple disabilities following brain or spinal cord injury Severe cerebral palsy

One of the particular challenges of working with young adults is the wide spectrum of cognitive ability encountered. Some will have severe cognitive impairment related to their underlying disease, whereas others, such as those with congenital heart disease or cancer may have no cognitive delay. Those with progressive impairment, such as the young adults with Leigh's disease or Cruetzfeldt-Jakob disease, will have been mentally alert earlier in their lives and may still have some degree of cognitive autonomy, even if it cannot be expressed. These young adults may once have been relatively independent, able to organize parts of their own lives and make decisions regarding their care.

Adolescence as a transition phase

Adolescence is a period of rapid physical, emotional, social, and cognitive change, during which key developmental tasks

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must be achieved. These include the development of personal value systems and identity, independance from parents, taking responsibility for one's own behaviour, and achieving financial and social independence. Hormonal changes lead to alterations in physical appearance, changing body image, and sexual awakening. Even in the presence of deteriorating health, neurological and/or physical impairment, development will continue in some, if not all, of these areas.

Normal adolescent, development [2, 3]

In early adolescence, pubertal changes are usually accompanied by rapid growth, and mood swings. Cognitive skills develop, with improved abstract thought and greater ability to understand consequences and make plans for the future. Alongside these changes is the need to socialize and identify with a peer group.

During middle adolescence there is often an increase in risk-taking behaviour as young people develop autonomy through testing limitations and boundaries imposed by parents and authority figures. This is an essential process through which young people work out who they are and who they want to be, often challenging previously accepted family beliefs and values. As part of this process, the need to identify with a peer group intensifies. Sexual development continues with the emergence of physical needs and for many young people their first sexual experiences.

By late adolescence young adults are defining and understanding their functional roles in life with regard to lifestyles, careers, and relationships. They should have a sense of who they are, who they identify with, and the type of life they want to lead. This is a time of making plans for the future, establishing permanent relationships, developing increased financial independence, and spending time away from the family.

Difficulties of normal adolescent development faced by life-limited young people

For those with life-limiting illness, the complexity of achieving even some of the developmental tasks of adolescence is immense. Acute illness or progressive physical and/or cognitive impairment is often accompanied by increased dependence on parents, a requirement to conform to the values and beliefs of adult carers, and increasing isolation from healthy peers.

Chronic ill health may delay the onset of pubertal changes, including sexual development, and limit the young person's potential for growth, giving them the appearance of being younger than their age. Immediately they become more identifiably different from their peers. Normal adolescent mood swings may be harder to express and less well accommodated. A life limited young person may not have the physical ability to storm off or slam doors as an outlet to their frustrations. Additionally, they often have to spend more time with adults than their peers, and moodiness towards carers or parents can cause considerable tensions, especially when it is interpreted as rudeness.

Many life-limited young people, due to their underlying diagnosis, may not experience the development of cognitive skills that accompanies normal adolescence. Indeed, this may be a time when those with neurodegenerative disorders have an accelerated cognitive decline. Without maturity of abstract thought and the ability to understand consequences, they are less likely be allowed, or to achieve, some of the freedoms and responsibilities of their peers. Even those with mature cognitive development may experience difficulty achieving personal freedoms, perhaps due to the demands of medical care, physical limitations imposed by their disease, or parental involvement [4]. Without such freedoms, opportunities to socialize with peers, to test limitations, and to explore and experience sexuality are limited.

By late adolescence, most young people should have a sense of who they want to be and the lifestyle they want to follow. The freedom to socialize independently, to choose a peer group, to test limitations, and to explore and define sexuality are essential processes in determining who an individual will become. For the majority of life-limited young people adolescent development will have been affected by restrictions and limitations. Physical impairment, cognitive impairment and ill health, rather than individuality and freedom, determine, at least to some extent, who the young person will become. Making plans for the future, in the face of an inevitable early death, may not seem worthwhile or possible.

Developing independence

I am aware that I can never really be independent because I have special needs and cannot get a job. But it's difficult. I don't want to sit at home and watch telly and I can't go on the buses by myself. (Palliative Care for Young People aged 13 24 [1])

Achieving independence and aiming for personal goals can be extremely difficult for life-limited young people. Some, such as those with malignant disease, may have been progressing through normal adolescent development, with increasing independence, prior to receiving a life-limiting diagnosis. Others may have chronic conditions with varying degrees of life-long dependence, or progressive disorders where the changes of adolescence are accompanied by deteriorating physical health and mental ability. Whatever the nature of the underlying condition, the psychological impact of impaired, lost, or potentially unachievable independence can be immense, particularly when the young person sees healthy peers and siblings becoming increasingly independent.

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Conflict with parents is not unusual, especially when previously compliant children challenge parental control, and act-out risk-taking behaviour, such as refusing to comply with health-care needs. Achieving autonomy, independence and peer group relationships, the necessities of adolescent progression, can become an increasingly difficult struggle.

For parents, the steps towards independence can be extremely difficult, particularly if they have been heavily involved in all aspects of their child's life. Acknowledging that their child is growing up and could become less dependent is in itself a bereavement the loss of their child, and a change in their role as parents. Added to this is the anxiety that their child may not manage, that they may not want to be more independent, that they may come to harm or could experience failure and disappointment.

Not all young adults will seek or welcome increasing independence. This may be particularly true if they have been relatively isolated from healthy peers and have become dependent on parents for companionship as well as physical support. Some may feel threatened by the suggestion that they could take part in activities away from the security of their parents. They may also fear failure it may be better not to try than to prove it is impossible.

All young people, whatever the degree of physical and cognitive dependence, need to be given opportunities to become as independent as possible [5], as a necessary developmental transition from childhood. With good information and support even those with severe physical and cognitive impairment may be able to take over the organization of at least part of their own lives. Others may become fully independent or may be able to live away from home, if provided with an independent carer.

Professionals working with young people and their families must support and facilitate the transition to independence, while at the same time acknowledging difficulties faced by parents and their children. The potential of a young person is developed over a number of years, so the expectation of what may be possible should be introduced early. However, even the most independent young adults have a great need for their parents [1], and it is important to balance a young person's need for independence with encouragement to continue including their parents in at least some aspects of their life and decisions around health care [5].

Physical independence

Within the group of young people with life-limiting disorders the spectrum of physical dependency is wide. Some are heavily dependent on others for physical care, unable to escape from a parent or carer even for short periods. Others may retain considerable independence, becoming physically dependent only for some aspects of care when they are unwell. Whatever the situation, opportunities to maintain and develop independence must be optimized.

All life-limited young people should be assessed for physical aids and housing adaptations. Equipment that enables them to control their own environment (e.g. electric wheelchair, self-operated hoists) may create opportunities for independence and privacy at home, as well as reducing some of the burden for carers. Additionally, parents may feel more able to allow their child some independence, such as time on their own at home, if they are confident that they are using appropriate and safe equipment.

Forward planning is essential if equipment and house modifications are to be in place when needed. This should be possible with knowledge of the natural history of diseases, yet many families still find that equipment provision and replacement is difficult [6]. There may be delays because the young person is about to transfer to adult services, or because they have recently transferred and are now on a waiting list for an assessment. There may also be bureaucratic delays while teams determine whose budget is responsible for adaptations and aids. Good communication and clear budget allocation between the agencies (Health and Social Services in the UK) is essential so that families are not hampered by bureaucracy or forced to rely on crises intervention.

Independence in decision making [4, 5, 7, 8]

Parents often find it difficult to allow their child to take responsibility for their own life. To do this a young person must develop sufficient intellectual maturity to consider choices and the consequence of choices. Some parents may feel, appropriately, that their child does not have the intellectual maturity to make informed choices, particularly if the young person has a disorder associated with intellectual impairment. Other parents may be unable to recognize, or accept, that their child has developed sufficient intellectual maturity to make these choices.

Involving their child in decisions about health care can be particularly difficult, especially if parents have not included them in discussions about their diagnosis, prognosis, treatment, and future management. The young person needs to be given this information in order to make fully informed choices, whereas the parents may want to protect them from a full awareness of their prognosis. Some parents may feel that including the young person in health care decisions would be an unnecessary responsibility that should not be imposed on them. They may also feel that the consequences of decisions about health care are so big that their child, even if capable of autonomy in other areas of their life, should not be given autonomy in this area. Additionally, giving the young person

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more responsibility and autonomy could represent to the parents a loss of their role as parents and carers, which they are not yet prepared for.

The extent to which young people can be involved in health care decisions will vary within the group. Some will have, or will achieve, the emotional maturity to understand actual and hypothetical concepts and the consequences of possible actions. Others may not achieve, or may lose, the ability to think conceptually or to fully understand consequences. Some will need assistance in understanding the available options, future risks, and consequences of choices. This can be especially true for younger adolescents. Young people with learning difficulty should also be encouraged and enabled to take part in at least some aspects of decision making and it is suggested that they have independent advocates [1] to support them.

Some young adults, although capable of achieving independent decision-making, may have been content to let their parents maintain control in this area of their lives. They may need encouragement to start taking some responsibility for their own lives, as part of a natural and healthy transition to adulthood.

Professionals must be sensitive to the difficulties faced by parents and young adults when the balance of responsibility starts to shift, and should start to prepare them for this transition well in advance. The young person should be given opportunities to become increasingly involved in making decisions about their day-to-day life, as well as health care. The parents should be assisted to view this as a natural progression to independent adulthood, while the young person should be encouraged to continue including their parents in decision-making, although no longer as the sole decision-makers.

Restricting the young adult from participating in decision-making can clearly create unnecessary tension. However, professionals and families should be aware that including the young person in decision-making can also create tensions, particularly if the young person, parents and professionals express differing viewpoints. A young person may make an informed decision to discontinue a particular treatment, against the wishes of their parents, causing considerable family tension and emotional stress if the parents feel their child lacks the emotional or intellectual maturity to understand the consequences of such a decision. Parents may feel at times that their child, however intellectually mature, is making a particular decision for inappropriate reasons, and may fear that health professionals will inappropriately support their child's autonomy. An adolescent tantrum , where the best weapon is the refusal of life-prolonging medication, can have dramatic consequences if not handled appropriately. Health professionals must be able to help the young person identify the reasons for making certain choices and ensure that they fully understand the consequences of these choices. Explanation and negotiation, between parents, health professionals, and the young person, is essential.

At one point I decided not to take my calcium tablets they tasted like chalk. But then the doctor explained the pros and cons and I realized that they were right. I could then evaluate the situation and make a decision. Sometimes this information stage is missed out. (Palliative Care for Young People aged 13 24 years [1])

Financial independence

Financial independence for the young person with life-limiting illness is frequently impossible. While healthy school attenders often find part-time jobs, giving them a small independent income and valuable work experience, such opportunities are rarely available to those with life-limiting illness, especially where there are physical limitations to work access, treatment regimens that need to be accommodated and absences due to ill health. Long-term financial prospects are similarly poor. Interrupted schooling can hinder educational achievement and, in conjunction with a poor attendance record and ill health, limit employability. Access to higher education is difficult and limited and deteriorating physical or mental ability makes many career paths inaccessible. Many young people have high educational aspirations and should be given career advice, vocational training, and assistance to attend school or university. Comprehensive multidisciplinary support should be provided, but is rarely available [1].

Peer group identification

Opportunities for peer group identification are limited when poor health, physical appearance or impaired cognitive function identify a young person as different. At a time when a young person should be experiencing a new independence, the life-limited young adult faces medical treatments, ill health, isolation from their friends, and an increasing dependence on parents. This can be particularly difficult for previously healthy young people, such as those diagnosed with cancer during adolescence. Their appearance and priorities may change, such that they can no longer easily identify a peer group amongst their healthy friends, but identify with a new world of illness, treatment, and death.

It was so much easier to make friends in the hospital than it is anywhere else. (Nicky A, teenager with cancer: Nicky's Story [9])

For many life-limited young adults links with peers are lost through school absence, hospitilization, friends being overwhelmed by the illness, or friends moving on and developing new relationships. Increasing physical dependence further

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increases social isolation and impairs the young person's opportunities for independent interactions. Family and carers may inappropriately, but almost inevitably, become the young person's main source of social support and companionship. With the majority of carers being female, male companionship is frequently limited, further isolating young men in particular from peer identification.

She wants to be 15 and talk about 15 year-old things. It's not the same doing things with your mum. (Palliative Care for Young People aged 13 24 years [1])

Most young people want to be involved in leisure activities and a social scene, which should be considered as important as health care and education. When a young person is unable to attend school, alternative social (not just educational) provision should be made. Parents should be encouraged to enable their child to take part in independent social activities, with professionals ensuring they have appropriate physical aids to achieve this. Opportunities for group activities with other life-limited young people are important, but interaction with peers of all abilities is also essential to minimize feelings of separateness and isolation [1].

Sexual development

With progressive pubertal development comes the need for physical satisfaction and sexual relationships. Many life-limited young people will develop physical and cognitive sexual maturity alongside their peers, although others may have delayed sexual development as a consequence of chronic ill health. Those with cognitive impairment or impaired motor function may experience sexual maturity with physical needs that they cannot express or satisfy. There are also some young adults with cognitive impairment who reach physical sexual maturity and become inappropriately sexually active as they have insufficient cognitive development to understand that their behaviour is socially inappropriate.

Much normal adolescent activity and discussion is focused on sexual awareness, finding a partner, and sexual activity. Through peer group isolation, young people with life-limiting illness often miss out on this normal adolescent information and discussion, while parents and carers may be unaware or unwilling to accept that a child has sexual feelings. Even when parents and carers are aware of the young person's sexual maturity, the young person may not have the same freedom of discussion that they would with their peers.

Young people who develop sexual maturity alongside their peers may find physical disabilities, or anxieties associated with their disability, in addition to a lack of appropriate social contact and limited independence, impair their opportunity to experience a similar level of sexual activity. Those with delayed sexual development may find it increasingly difficult to fit into their peer group, both in terms of physical appearance and interests. Personal expectations relating to relationships and sexual experiences can become a major concern to the young person, who may have no one with whom they can safely discuss these issues. Some will mourn the fact that they may not live to experience sexual intimacy and others may become overtly sexually active in an attempt to achieve acceptance and normality. Without being able to join in the sexual arena of their friends, many life-limited young people can feel increasingly different and isolated.

Sex education is important for all young people, but especially those who may have limited access to education through school and peers. For several disorders more specific information about sexual activity and fertility is essential it is not unusual, for example, for young adults with cancer to presume they are infertile. Young people may also want information about masturbation, finding a sexual partner and accessing videos information that is usually provided by peers. In the absence of peers they may rely on parents and carers to provide this information, or let their questions (and needs) remain unaddressed. Young men may have particular difficulty accessing information of a sexual nature if they have only female carers. Providing opportunities for peer group interaction away from parents, which includes structured sexual education and emotional support, may be one way to address some of these issues.

Relationships with parents and siblings

I tended to push my mum away and shut her out I was scared to admit that I really needed her. (Nicky A, teenager with cancer)

Relationships with parents can be particularly complex for the life-limited young adult. Most are striving for, or have previously experienced, a degree of autonomy with physical and emotional independence. Declining health and/or impaired physical and cognitive ability requires them to need their parents more, for both emotional and practical support. They have to cope with two conflicting realities the need to be independent of parents and the need to be supported by parents.

Many young people also feel the need to protect their parents [10]. They may not want them to know if they are feeling unhappy, depressed or in pain, and may push them away rather than expose them to how they are feeling.

My mum stayed with me everyday but I would never tell her how I felt and I tended to push her away and shut her out I also thought I was mean for making her watch me get ill. (Nicky A, teenager with cancer)

Encouraging parents and young adults to share their feelings, however, can be helpful.

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I remembered my mum saying that's what families were for, to help and support each other when we were ill Now I like it when my mum stays with me because we had a long talk and I realized my mum was also suffering. I also agreed with my mum that I would stop shutting her out now we are closer than ever. (Nicky A, teenager with cancer)

Sibling relationships can be strong, but the burden on a healthy sibling can be tremendous [10]. They may be the main confident of the life-limited sibling, while worrying about their parents coping abilities and the conflicting need to pursue their own life [11]. They may appropriately complain of displacement, deprivation, anger, injustice, loneliness, and vulnerability, as parents have insufficient time for them [10]. Without appropriate support, these feelings can cause resentment and disruption of their relationship with the life-limited sibling.

Professionals caring for young people with life-limiting illness should be aware of, and should prepare the family for, some of the difficulties in family relationships, and dynamics that they may experience. It may be helpful if there is someone the family can talk to who is not part of the clinical team. Parent and sibling groups can also provide opportunities for family members to discuss how they are feeling and to feel less isolated. In the United Kingdom such groups can often be accessed through children's hospice services.

Psychological needs

With limitations in peer group interaction, employability, social and financial independence, and the opportunity to explore sexuality, it is difficult for young people to establish functional roles in life with regard to careers, relationships, and lifestyles. For most young people, the adolescent development of identity and autonomy is accompanied by a sense of immortality and the opportunity to determine and shape their own future. For many with life-limiting illness, mortality is a reality and the future is determined by limitations not possibilities. They cannot develop their own identity and autonomy from the same perspective as their peers, nor can they look to a seemingly endless and purposeful future of their own choosing. As their healthy peers establish permanent relationships, develop increased financial independence, spend time away from their family, and make plans for the future, the life-limited young person can feel increasingly isolated with no sense of purposeful future.

Many young people with life-limiting illness are mentally alert and fully understand their disease and prognosis and the implications for both themselves and their family. They may not, however, have established mechanisms to cope with the anxiety and uncertainty [4]. Feelings of anger and grief at being ill, anxiety about medical procedures, worries about family members, depression caused by separation from friends and normal activities, and fears of death are not uncommon [4, 5, 12, 13]. A physical appearance that the young person feels, identifies them as different from everyone else may cause feelings of being damaged and deviant in the outside world, further contributing to feelings of low self esteem. At a time when peer group acceptance and support is so important, they may feel increasingly ostracized. Some may grieve future losses, such as having a career and family, or act out risk taking behaviour in their struggle for autonomy or in an attempt to gain peer group acceptance.

It was a big relief that I was going back to hospital everyone is like me and no one looks at me like I am weird or a freak. (Nicky A, teenager with cancer)

Because the emotional demands of young adults are particularly complex, all professionals involved in their care should receive training that enables them to offer appropriate support. In addition to this, clinical psychology should be a core service in all care plans for young adults and should include the needs of parents, siblings, partners, and significant others [1]. Clinical psychologists should routinely work as part of the teams caring for life-limited young adults, offering interventions when needed and working with small groups. Many young adults are capable of discussing their prognosis, and the issues arising from this, but need to set the pace for these discussions. They also need opportunities to explore their feelings without fear of upsetting other members of their own family or professional carers. A neutral professional, who is not part of the clinical team, could take this role and may be someone with whom the young person can most easily voice fears and express anger.

Assessing the psychological needs of young adults with cognitive impairment and/or physical barriers to communication is particularly challenging.

Spiritual needs

As part of the quest to develop their own identity and value systems, adolescence is often a time when young people question the meaning of life and look for reasons for pain and suffering, unfairness, and punishment. With the prospect of an early death, the ideas and concepts of identity and who a person may become, gain increasing significance. Many will want to make their mark on the world, aware that they have limited time, and may be facing increasing physical and/or cognitive decline. Some may express hope for some kind of spiritual continuity, especially if they see little potential to achieve something before death.

Faith and religious belief may have played an important role in parental family values, but the young person may need

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to explore their own belief system and reasons for their illness and impending death [14]. They need opportunities to discuss and express their beliefs and for these to be acknowledged and respected, even where there is the potential for conflict if they choose to reject parental beliefs. Through this, their sense of individuality, autonomy, value, and continuity can be reinforced. Providing such opportunities for young adults with cognitive disability or physical barriers to communication can be especially difficult, as they may be unable to formulate or express spiritual needs. It is also important to be aware, particularly when working within units based on a specific religious ethic, that some young people may not have the maturity or confidence to refuse what is on offer.

Respite care

Young people living at home usually welcome the opportunity for respite away from the family, with opportunities for peer socialization [1]. Equally, caring for a life-limited young person, particularly with physical limitations and progressively deteriorating health, places a huge demand on parents. Appropriate respite should be available for young people both for scheduled breaks away from their family and in emergency situations. Unfortunately, facilities, for this age group are scarce [15] and for some young people respite may only be available in units that are primarily tailored to the needs of adults with learning disabilities and mental health problems.

Paediatric hospices may be able to offer respite admissions, usually if the young person is under 19 years of age, but this may be inappropriate for a mature young adult, who is, or has been, relatively independent. They may feel uncomfortable being admitted alongside much younger children or those with cognitive impairment, or into an environment with childish decorations, and activities.

Adult hospices can be equally inappropriate, particularly if the young person's cognitive age is below their chronological age, and more suited to a paediatric environment. Additionally, the environment of an adult hospice is unlikely to be geared towards accommodating more active young people, as the majority of users are often middle aged or elderly. Staff may not be aware of the specific needs of the young adult, particularly in relation to social activities, sexuality, privacy, and independence.

Young adults often want to stay up late at night and spend the day in bed. They want to be able to listen to their own music or to be noisy without fear of disturbing younger children or older adults. They want to be able to watch adult films and television programmes, perhaps not appropriate in a paediatric hospice environment. They want personal space and privacy to be alone when they feel like it. They want to be with people of their own age, with things to do in the evenings and weekends, and they want to fit in .

The very specific needs of young adults with regard to respite and hospice care are being increasingly recognized and the number of facilities for this age group is slowly increasing. In the UK, Acorns Hospice in the West Midlands and Helen House in Oxford have now opened units for young adults, having recognized the needs of the maturing paediatric users. Whatever the environment, respite admissions should be co-ordinated to maximize the young adult's opportunities for peer socialization and staff should be trained to support the developing and changing needs specific to this age group.

Terminal care

There is a large gap in the provision of terminal care for young adults. Paediatric teams may lack confidence in the symptom management of older patients, while adult community nurses and palliative care teams may feel less confident with patients who are much younger than they are used to. The adult teams may also feel less comfortable when working in partnership with parents, and may find it particularly difficult working with young adults whose parents have always taken a strong lead in care. Family doctors are also likely to have had little experience in the terminal care of this age group and may not have easy access to specialist support to advise on symptom management and drug doses. These difficulties can be compounded if the young adult is dying from a rare condition, particularly if it is one not usually encountered in adult patients. Where possible, the young adult and their family should be allowed to choose, who should take on the key co-ordination of their care, being able to choose from available paediatric and adult services. For some patients, partnership working between adult palliative care and paediatric teams may offer a solution [16], but such practice is not routinely available.

There are often practical difficulties when caring for young adult patients at home. Parents are often the main carers and may have difficulty with the physical demands of care. Nursing support from adult services may not be available if the young adult is still within the paediatric age range and, if the deterioration has been very rapid, appropriate aids may not be available in the home.

Finding an appropriate hospice to care for a terminally ill young adult can also be difficult, as discussed in the previous section. Even if an appropriate hospice is identified, there may be age restriction to admission, although these may sometimes be relaxed if the young adult is well known to the service. Young adults over the age of 19 years, however, are unlikely to

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have a first admission to a children's hospice, even if this would be the ideal location both for them and their family. While there are still insufficient hospices dedicated specifically to young adults, many will continue to be admitted to acute hospital wards for terminal care when this cannot be delivered at home.

Transition to adult services [17, 18, 19, 20, 21]

The transition to adult services can be incredibly difficult for young people, their families, and the health care teams involved in their care, for many reasons.

Families are likely to have used paediatric services for many years and feel secure in the familiar environment, dealing with professionals who know them well, and have long-term knowledge of the patient, the family, and the condition.

Paediatricians may feel reluctant to allow their patients and families to move to adult services. They may have a degree of emotional attachment, having worked hard for the child over a number of years, and watched them grow up.

It can often be difficult to identify the most appropriate team to take on the continuing care of a young adult. For those with rare disorders, particularly when they have outlived their predicted life expectancy, there may be no adult physicians or community services with sufficient experience and expertise.

Within adult services there is less tradition of a single general physician to oversee care and maintain a holistic quality, which is particularly needed for patients under the care of several different specialists. There is a fear that families are being transferred into new unfamiliar services, without a clear co-ordinator to guide them through and pull all the services together.

Families and paediatricians may perceive adult services to be inadequate, poorly co-ordinated, unsupportive, and impersonal. There is often an anxiety that parents and families will be excluded, as the adult teams will focus on the patient as an individual, rather than within the context of the family dynamic. Parents may feel that their child will be given too much autonomy in an adult service and will be allowed to behave irresponsibly, particularly if they are trying to assert their own identity through contradicting their parents'choices.

Transfer to adult services is usually determined by the age of a young person, not their clinical health. For young adults not expected to live beyond childhood, the transfer to adult services (i.e. an acknowledgement of adulthood) may signify that time is running out . Many will be reaching the end stages of their disorder, sometimes deteriorating as a consequence of the physical and hormonal changes of adolescence. They face deteriorating health in the care of people they do not know, who have the potential to deliver a less well co-ordinated service than they are used to. Many families and health care workers would prefer to sit it out in paediatric services rather than transfer care for what may be a short, but critical, period.

Age does not determine emotional readiness to transfer to adult services. Some young adults, particularly those with cognitive impairment, may not want, or be able, to take on the responsibility of more autonomous interactions with health care staff. They may not feel comfortable in a more adult environment, and may still want the security of the familiar child-oriented surroundings of the paediatric clinic. Additionally, even the most mature young adults may feel that they are being abandoned by the staff they have got to know and may suffer separation anxiety, loss of identity, loss of self esteem, and loss of significant relationships. Transfer can, for many, be a loss that is grieved.

Transition to adult services must be well planned and the young adult should be involved in the timing of the transition. It is essential that preparation for the transition starts well in advance, and that the young adult and their family are supported through the transition on both a practical and emotional basis. The transition should not be a black cloud hanging over their future, or something to be put off in the hope that the young person may die before transfer becomes necessary.

A long transition time is necessary for the family to adjust and look at alternatives to fill some of the gaps between paediatric and adult services. Association for Children with life-threatening or Terminal conditions and their family (ACT) recommends a framework for transition within which there should be scope for individual flexibility [1]. They recommend that a first stage might be identified ranging from 13 to 17/18 years with the goal of transferring to the adult service. Another goal could be set to increase the young person's independence by 25 years. The transition should be planned with the young person and set out for the patient, the family, and all professionals involved. Everyone must have a clear direction as an open-ended process can make it difficult for families and professionals to accept the change.

One useful way of making the transition gradually is to hold joint clinics with paediatric and adult staff, enabling the young person and family to build up confidence in the new team before completely transferring care. They should also be given written information about the adult unit (well in advance of transfer) and an introductory visit to the ward area, accompanied by a member of the paediatric team who knows them. It may be helpful to have a liaison nurse or another professional in a similar position to act as a link between the paediatric and adult teams, as well as joint visits by paediatric and adult nurses and therapists, so that there is a smooth

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handover and continuity. Psychological support, provided before and during the transfer, should be continued while the young person settles into the new environment.

Developing smooth systems for transfer to adult services should be possible for conditions where a relatively large proportion of paediatric patients reach adulthood. Some adult respiratory physicians, for example, have developed a special interest in the care of young adults with cystic fibrosis [22, 23] and there are also specialist clinics for adults with congenital heart disease [24]. The most difficult young people to place in adult services are those with disorders that do not fit easily into a disease-specific or system-specific category. For example, it can be difficult to identify the most appropriate team to take on a young adult with cerebral palsy, gastrooesophageal reflux, a gastrostomy and deteriorating lung function. They may be transferred to at least three different adult teams, with no obvious physician in overall charge of management. It may be that the family doctor is the most appropriate professional to oversee and co-ordinate the continuing care of these young adults, if they are willing and able to provide the extra time and commitment needed. The family doctor should continue to be supported by paediatric services during the transition.

It can be equally difficult to find an appropriate team to take on young adults with rare disorders, particularly when they have outlived their predicted life expectancy. Because of the relatively small numbers of patients involved it is unlikely that there will be adult physicians or community services with sufficient experience and expertise to manage them confidently. The development of central specialist services for these patients would enable them to receive care locally, with a central service and providing the specialist input to oversee their care [25].

The needs of professional staff and carers [1]

Young adults are a unique group to work with, yet in most countries there is no career structure in medicine, nursing or allied professions specifically for this age group. Professionals and carers may come from a range of backgrounds, with experience of working with children or adults or people with cognitive impairment or mental health problems. Some teams, for example, children's community nursing teams or adult palliative care teams, may only occasionally work with young adults. Where possible, there should be a dedicated member of the team who takes a specific interest in this age group, who can take a lead role in the care of the young person, educating and supporting other members of the team.

Whatever their background, staff working with young adults must have an interest in, and a commitment to, working with this age group, as well as the ability to communicate and empathize. Young people often like to be cared for by young staff, who they may find less threatening and easier to relate to. They also like to have a choice of male or female carers. Young men, particularly if they need assistance in their personal care, often value the availability of male staff.

Staff must have a wide range of basic knowledge and competencies to work with young adults. Recommendations from Palliative Care for Young People aged 13 24 years [1] include skills and understanding of:

The developmental stages of normal adolescence
The rights of young people, issues around consent, and ethical dilemmas
The effects of loss and bereavement
Work with reference to families, partners, and close friends
The needs of siblings
Cultural diversity
Spiritual needs religious and secular approaches
Liaison with school and college
Communication skills, focusing on particular issues, in addition to listening skills and non-verbal communication
Management of non-adherent behaviour
Management of emotional involvement
Counselling
Advocacy.

Essential experience for those working with young adults, recommended in Palliative Care for Young People aged 13 24 years [1], should include:

Experience in the community, to gain experience of care in different locations, an insight into family problems and the diversity of professionals involved.
Experience in a specialist unit, providing care for diseases such as muscular dystrophy, cystic fibrosis or cancer, where care is co-ordinated from the hospital.
Attachment to a children's hospice or an associated primary care team to gain experience of a wide cross-section of life-limiting conditions and to have the opportunity to spend time with the parents.
An opportunity to acquire counseling and communication skills (e.g. through attachment to psychology or to a palliative care team).
A training placement to gain knowledge of physical symptom control, perhaps with a paediatric or adult palliative care team.

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Individual and team support for those working with young adults is essential. The powerful feelings young people often need to explore, grieving future losses and trying to make sense of their life in the context of illness and premature death, can be emotionally draining for staff, especially if the young person chooses one member of staff to confide in. Staff may find the work raises questions about their own mortality and purpose in life. Young, less experienced staff may find it particularly hard to cope with the emotional demands of caring for this age group and will need regular support and supervision. Self-awareness and maintenance of professional boundaries is essential, to avoid responding to young people as social friends or, in the case of older staff, responding as though the young person was their own child.

Service development [1]

Palliative care strategies should take into account the wide range of disorders encountered in life-limited young adults. Those responsible for planning services should carry out a local needs assessment, specifically including and defining the care of young people with life-limiting conditions when developing their palliative care strategies. Services must be multi-disciplinary and multi-agency, but with a clearly identified professional to lead and co-ordinate services.

Each young adult should have a named key worker who has specialist knowledge and training to support their specific needs. This should include addressing the spiritual and psychological issues as well as the practical and medical concerns. The key worker should know what services the young person needs, their availability and how to access them. They should support the young person to be as independent as possible, with the ability to organize their own systems of clinical and social support.

The transition to adult service should be facilitated by early multidisciplinary planning that includes the young person. They should be supported through this transition by their key worker from the paediatric team and passed on to the worker who will maintain this role in the future. While there is a need for central specialist clinics for young adults with conditions rarely seen in adulthood, adult consultant medical physicians also need to develop expertise in the care of these young people. Primary care teams should have opportunities to receive training in palliative care and in supporting the transition to adult services.

Appropriate respite care needs to be more readily available, with clear and easy routes of access. Social opportunities should be provided, both within a respite setting and in the community. Each young person must be assessed and provided with appropriate aids for independence, planned in advance so that these are available when needed. A proper system of purchasing, maintaining, upgrading, and replacing aids and equipment must be in place and monitored.

Well co-ordinated multidisciplinary and multiagency support to achieve educational goals is essential. This should include career advice and assistance to attend higher education or access employment. Links between further education or employment and occupational therapists could be extremely helpful.

Above all, flexibility and choice in service provision is important, with the young person making decisions whenever possible.

Summary

The transition from childhood to adulthood occurs over a number of years and is characterized by hormonal, physical, emotional, and cognitive change. The purpose of this change is to develop an individual who is autonomous and functions effectively within a community. Even in the presence of life-limiting illness and deteriorating physical and/or cognitive ability many of these changes will continue and must be supported.

For the life-limited young person the transition to adulthood is fraught with difficulty. While cognitive and emotional changes drive them to pursue individuality and independence, their opportunities to experience and achieve this are limited. The adolescent sense of immortality and freedom that shapes one's future is often replaced by increasing dependence and the reality of what will never be achieved. It is important that they are viewed as young people first, and as being unwell second. They need opportunities to take part in normal adolescent activities, with people their own age and to socialize with healthy peers as well as others with life-limiting illness. This is essential to continuing development.

Staff working with young people must be dedicated to working with this age group and should have a wide range of basic knowledge and competencies. They should be able to support the young person through the transition to adulthood and to help them achieve as much autonomy as possible. Transfer to adult services is an important step in this transition, and should be well co-ordinated and planned in advance, identifying appropriate services to smoothly take on the continuing care. Alongside this, young people should be given the opportunity to take an increasing role in responsibility for, and decisions regarding, their own health care, as well as other aspects of their own life. It is important that staff are aware of the difficulties faced in normal adolescence and how life-limiting illness impacts on these, not only for the young person, but also for their family and carers. They must also be aware that many families and carers need support to allow the

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young person to make the transition to adulthood, recognizing what can be achieved as well as what can not.

Whatever a young person's abilities or potential, the physical and emotional changes of early adulthood must be acknowledged. In the face of life-limiting illness, well co-ordinated multidisciplinary and agency support is essential to help a young person achieve the goals of transition to adulthood, and to support them as they face the reality of goals that cannot be achieved.

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