Paediatric v. adult home parenteral nutrition

The prevalence of paediatric HPN is estimated to be 2·2 per million of the general population⁽⁷⁾. While children with short-bowel syndrome have a better chance of weaning off PN than an adult with a comparable diagnosis⁽Reference Guarino and De Marco³⁾, it is estimated that in the UK up to thirty adolescents are poised to transfer to adult services over the next 5 years⁽⁷⁾. Many of these adolescents will have been dependent on PN since early infancy (PN for ⩽6000 d). Of this cohort 40% have short-bowel syndrome secondary to neonatal causes, 30% have congenital enteropathies and a smaller number (15%) have severe motility problems⁽⁷⁾.

Linear growth may not always be completed by age 16–18 years when a patient might be poised to transfer to adult services, particularly if puberty is delayed. Adequate energy intake to allow a normal pubertal growth spurt is essential⁽Reference Johnson and Sexton⁸⁾. Requirements are assessed on an individual basis according to age and stage of growth, and a higher intake of intravenous fat than that required by an adult may be necessary to achieve adequate energy intake⁽Reference Koletzko, Goulet and Hunt⁹⁾. Flexibility in timing of transfer will ensure that the needs of individual young people are met. Children who miss out on development of oral feeding skills in the first year of life may have persistent eating problems that continue as young adults. Enteral intake may be reliant on gastrostomies or feeding tubes.

Intestinal failure-associated liver disease is more common in children (23% of patients receiving HPN)⁽Reference Colomb, Dabbas-Tyan and Taupin²⁾ than in adults on PN and carries a high mortality⁽Reference Colomb, Dabbas-Tyan and Taupin²⁾. Key risk factors are preterm birth⁽Reference Kelly^10, Reference Merrit¹¹⁾ and sepsis⁽Reference Sondheimer, Asturias and Cadnapaphornchai¹²⁾. Difficulties placing catheters for venous access cannot be underestimated and require experts familiar with placing catheters in unconventional sites⁽Reference Rodrigues, Van Mourik and Sharif¹³⁾. Intestinal transplantation has developed for those patients with complications of liver damage or loss of venous access, and children currently represent two-thirds of all candidates for intestinal transplantation⁽Reference Gupte, Beath and Protheroe^14, Reference Beath, Booth and Murphy¹⁵⁾.

Transition

Preparing patients for transition involves more than simply describing future events and raising awareness of transfer⁽Reference Viner¹⁶⁾. Paediatric care is family centred, focusing as much on the parents as on the children. This approach provides poor preparation for moving to the adult sector. Adult health services assume that patients have an extent of autonomy and have the capacity to negotiate the healthcare system. Shift from parent-managed to self-managed care is required⁽Reference Sawyer and Aroni^17, Reference McDonagh and Viner¹⁸⁾.

The standard definition of transition is ‘a purposeful, planned process that addresses the medical, psycho social and educational/vocational needs of adolescents and young adults with chronic physical and medical conditions as they move from child-centred to adult-orientated health care systems’⁽Reference Rosen, Blum and Britto¹⁹⁾. Key features of the transition are shown in Table 1.

Table 1.

Key features of transition (based on Viner⁽Reference Viner¹⁶⁾ and McDonagh et al.⁽Reference McDonagh, Shaw and Southwood⁴⁰⁾)Footnote *

* WHO defines ages 10–20 years as adolescence and the Department of Health recognises that health services for young people cover those individuals aged 16–19 years⁽²⁰⁾.

Adolescent medicine

Young people (the WHO defines ages 10–20 years as adolescence and the Department of Health recognises that health services for young people cover those individuals aged 16–19 years⁽²⁰⁾) are new independent users of health services. Provision of confidential healthcare that allows engagement with young people and promotes disease self-management behaviour (e.g. central line care) and engagement with the new HPN team is pivotal⁽Reference Kennedy and Sawyer²¹⁾. Screening for health risk behaviours (mental health, diet, exercise, cardiovascular risk, smoking) is as important in adolescents on HPN as in other young people in the population⁽Reference Goldenring and Rosen²²⁾. Opportunities exist for promoting health, disease knowledge and prevention (e.g. osteoporosis). There is growing evidence that skills training for young people with chronic illness can be associated with positive outcomes (e.g. improved information seeking, social competence and behaviour), which can maximise functioning and potential⁽Reference McDonagh, Southwood and Shaw^23, Reference Last, Stam and Onland-van Nieuwenhuizen²⁴⁾. There have been several reports of increased unemployment in young adults that is not always related to their disability or educational achievement⁽Reference McDonagh²⁵⁾.

Benefits of transition

Why is the transfer of healthcare so important? At worst, if it is not handled well, failure to engage with adult tertiary services makes routine clinic attendance less likely and quality of care may decline⁽Reference Dobbels, Van Damme-Lonbaert and Vanhaecke²⁶⁾. Young people who have experienced skills training in transition programmes and secure a strong relationship with the new healthcare team have better adherence to follow-up, improved disease control and quality of life⁽Reference Watson²⁷⁾.

Challenges: young people

While the young person with a chronic medical condition may have outgrown the paediatric healthcare environment, they may have delayed psychosocial milestones, reduced independence, social isolation and increased educational and vocational difficulties⁽Reference Stam, Hartman and Deurloo²⁸⁾. Management tasks may present greater obstacles and be more problematic in young people. Skill acquisition, e.g. setting up their own HPN, is variable and can be delayed in the context of chronic illness and be more problematic in young people as psychosocial milestones such as cognitive maturity and autonomy have not yet been achieved⁽Reference Sawyer, Drew and Yeo²⁹⁾.

Medical care might be rejected by adolescents as part of separation from parental control. They may be more prone to risk taking⁽Reference Sawyer, Drew and Yeo^29, Reference Bolton-Maggs³⁰⁾ or overwhelmed by the burden of healthcare issues and inappropriate expectations of clinicians.

Depression and anxiety are common problems in the daily life of adults on HPN and may arise as a result of lack of freedom, limitations in social life and being dependent. Somatic problems, fatigue and sleep disturbance impact on economic dependency⁽Reference Huisman-de Waal, Schoonhoven and Janeen³¹⁾. Quality of life is reduced in the presence of depression and narcotic dependency⁽Reference Winkler³²⁾. HPN teams should be familiar with these problems and facilitate psychosocial support and/or cognitive training.

Challenges: parents

Families of young people on HPN may be overprotective and have difficulties with transition⁽Reference Shaw, Southwood and McDonagh³³⁾, particularly if care appears discontinuous or they feel excluded. Demonstration that young people have developed independence in healthcare as well as other aspects of their lives allows parents (and their doctors) to ‘let go’.

Having a child on HPN is associated with physical and social stresses. Feelings of social isolation, loneliness and depression are reported in parents⁽Reference Huisman-de Waal, Schoonhoven and Janeen^31, Reference Sexton, Holden and Coad³⁴⁾. Young people and their families may be concerned about a reduction in services following transfer and a lack of preparation for such differences. Home-care services may be negotiated to support children and their families with nursing support⁽Reference Winkler³²⁾, and families may face a change of services with a change of provider as well as a new adult nutritional care team.

Transition: how to do it

There have been a number of recent publications on how to provide quality services for adolescents during transition, including the National Service Framework for Children, Young People and Maternity Services ⁽³⁵⁾ and the guidelines from the Royal College of Paediatrics and Child Health⁽³⁶⁾ and the Royal College of Nursing⁽³⁷⁾. They have been reviewed in the Department of Health's recent guidance in Transition: Getting it Right for Young People. Improving the Transition of Young People with Long Term Conditions from Children's to Adult Health Services ⁽³⁸⁾. Focus groups of adolescents have been helpful in developing models of care in rheumatology⁽Reference Shaw, Southwood and McDonagh³⁹⁾, with common themes emerging, e.g. need for multidisciplinary team, continuity and good coordination between professionals, clear sources of information, care that minimises the impact of the medical condition on their life and a less-formal environment. There is a need for information, and web sites devoted to health issues for adolescents are popular sources⁽Reference Bolton-Maggs³⁰⁾.

Planning

Health transitions are one part of the wider set of educational, family and social transitions that young people make. These transitions are outlined in Table 2.

Table 2.

Transitions undergone by the young personFootnote *

PN, parenteral nutrition.

* The Department of Health recognises that health services for young people cover those individuals aged 16–19 years⁽²⁰⁾.