Adolescence and transition post–liver transplantation

Published on 04/06/2017 by admin

Filed under Pediatrics

Last modified 04/06/2017

Print this page

rate 1 star rate 2 star rate 3 star rate 4 star rate 5 star
Your rating: none, Average: 2.6 (5 votes)

This article have been viewed 1044 times

!DOCTYPE html>

41

Adolescence and transition post–liver transplantation

MARIANNE SAMYN

41.1  Introduction

41.2  Adolescence and liver transplantation

41.3  Biological and psychosocial aspects of adolescence

41.4  Transition from paediatric to adult-centred health services

41.5  Conclusion

Further reading

References

41.1  INTRODUCTION

Thomas Starzl and colleagues from Denver, Colorado, reported the first series of liver transplants (LTs) in adults and children in December 1963 [1], sparking an upsurge in enthusiasm for the procedure in a number of centres throughout the world. Such programmes withered and ultimately failed on the lack of effective immunosuppression, and long-term survivors were rare. However, with the resumption in transplant activity during the 1980s, it is now clear that we are seeing successful paediatric cohorts of not only 10- but also 20-year survivors. There are reported 20-year survival rates of 52% from an American multicentre cohort transplanted between 1984 and 1988 [2] and 10- and 20-year survival rates of 83% and 80%, respectively, from a more recent Japanese paediatric living donor LT programme between 1989 and 2010 [2]. There is now a significant cohort of patients transplanted in childhood moving into adulthood, or as adolescents and young adults, where transplant outcome is poorer [3,4]. These young people deserve specialist attention and dedicated care to provide improved and excellent long-term outcomes. The onus of care has been on providing a good quality of life through the provision of appropriate transition from paediatrics to adult services.

41.2  ADOLESCENCE AND LIVER TRANSPLANTATION

Adolescence is characterised by a combination of biological, psychological and social challenges leading to the development of autonomy and self-identity and is often defined by the onset of puberty. Despite the excellent survival data post–liver transplantation, data on health-related quality of life (HRQL) in adolescents shows that they share many characteristics similar to those other chronic health conditions, rather than with the general population [5,6].

Whereas most of the current literature focuses on adolescence, it is now well demonstrated that structural and functional changes continue to take place in the brain into adulthood [7], and that the focus should be on ‘young people’, defined by the World Health Organization (WHO) as aged 10–24 years; this includes adolescents (aged 10–19 years) as well as young adults (aged 15–24 years), as a separate population who are neither ‘big children’ nor ‘small adults’ and deserve specialist care [8], shared by paediatric and adult professionals (Box 41.1).

41.3  BIOLOGICAL AND PSYCHOSOCIAL ASPECTS OF ADOLESCENCE

Growth impairment is common following LT, with the Studies of Pediatric Liver Transplantation (SPLIT) registry reporting that 20% of 8- to 18-year-old recipients have linear growth impairment [9]. Risk factors for growth impairment include prolonged steroid exposure, pretransplant failure to thrive, metabolic liver disease retransplantation and nonwhite race. Data on pubertal delay in young people with chronic liver disease is currently lacking; however, it is well documented in other chronic conditions, including inflammatory bowel disease, nephrotic syndrome, asthma and cystic fibrosis. of 353 children post-LT, pubertal delay was reported in 39% of girls and 42% of boys who had not reached Tanner 5 development between the ages of 16 and 18 years, compared with 100% of the general population [9]. The psychosocial effect on quality of life and long-term outcome should not be underestimated. Young people with pubertal and growth delay will find it more difficult to be managed in an age-appropriate fashion and struggle to become independent. Recombinant growth hormone has been used in the renal transplant population with a positive effect on linear growth and psychosocial well-being; however, larger studies are needed to assess its safety in the post-LT setting [10].

BOX 41.1 New definitions

Young people Aged 10–24 years

  Adolescents Aged 10–19 years

  Young adults Aged 15–24 years

World Health Organization

Transition

  ‘Purposeful, planned process that addresses the medical, psychosocial and educational/vocational needs of adolescents and young adults with chronic physical and medical conditions from child-centered to adult-orientated healthcare systems’.

Blum et al. (1993) [33]

Cosmetic side effects of medications such as steroids and cyclosporine (now less commonly used), as well as surgical scars, impact on body image and subsequently adherence to treatment; however, the literature is scarce on this topic. We recently investigated body image in a cohort of 80 patients aged between 16 and 24 years of whom 30% had undergone liver transplantation and found that body image perception was poorer in this group, although this did not appear to be associated with a surgical scar or side effects of immunosuppressive medication.

End-stage liver disease alters the normal physiology of the hypothalamic–pituitary–gonadal axis and disturbs oestrogen metabolism, affecting sexual function. Menstrual irregularities are common in women with chronic liver disease and typically improve following LT. A survey of menstrual function and obstetric history before and after LT reported irregular menses in 28% and amenorrhoea in 30% of 64 patients pretransplant, with 95% of 51 women younger than 46 years observing a normal menstrual cycle within the first year after LT [11].

With the median age at first intercourse being 16 years in developed countries, with 20%–30% of young people reporting sexual intercourse before the age of 15, comprehensive sex education is essential and even more important in young people with chronic health conditions. Sexuality and pregnancy should therefore be part of the consultation with young people with liver disease both pre- and post-LT.

Oestrogens, and typically the synthetically produced ethinylestradiol used in the combined hormonal preparations, are more potent, with a potential effect on the liver irrespective of the route of administration. Progestogens do not have receptors on liver cells and are commonly given at a lower dose and are well tolerated. Although not contraindicated in patients with compensated cirrhosis, in both pre- and post-LT settings, the current contraceptive recommendation is that progesterone-only preparations, such as the mini pill (e.g. Cerazette®), medroxyprogesterone injection or etonogestrol implants, and, if sexually active, a levonogestrol-releasing intrauterine system (e.g. Mirena®) [12], should be utilised.

Pregnancy following LT has been reported to have largely favourable outcomes for the allograft, mother and fetus (Box 41.2). We have reported on the outcome of 115 pregnancies in 84 patients, between 1988 and 2010 [13]. There were 71% live births, with prematurity reported in 34% and very low birth weight in 6%, which is higher than that of the general population. More recently, in a UK national cohort study between 2007 and 2012, live birth rates in LT recipients were 92%, with similar prematurity rates (42%) [14]. Maternal complications such as hypertension and preeclampsia were more common. In our series, graft rejection was noted in 16% and was considerably higher in patients conceiving within 12 months of LT, although there was no graft loss. Delivery by caesarean section is certainly more common. Current recommendations are to delay pregnancy until at least 1 year following liver transplantation and use appropriate contraception in the interim (Box 41.2) [12,15].

Liver transplantation will impact on the psychosocial aspects of adolescence and vice versa. Data available on cognitive development post-LT confirms an increased incidence of learning difficulties in this population. of 144 patients, aged 5–7 years, from the SPLIT registry who were >2 years posttransplant, mild to moderate cognitive delay was recorded in 26% and serious cognitive delay in 4%. The authors reported that 25% were having learning difficulties with reading and math skills and a relevant executive functioning deficit which would potentially affect independent management of their health condition in adult life [16]. Further research identified height centile at transplantation and genetic-metabolic conditions as having a high impact on long-term cognitive functioning. More studies are needed to assess cognitive function in young people long term after LT and its effect on outcome and quality of life.

BOX 41.2 Pregnancy and liver transplantation: recommendations

Delay pregnancy for at least 1 year following LT (if possible).

Immunosuppression (acceptable and safe)

  Tacrolimus

  Cyclosporine

  Prednisolone

  Azathioprine

Immunosuppression (avoid because of adverse fetal outcomes)

  Mycophenolate mofetil

  Sirolimus

Buy Membership for Pediatrics Category to continue reading. Learn more here