Challenges for Young People With Liver Disease Including Transition to Adult Services

Neurodevelopmental Changes During Adolescence

In terms of neurological development, adolescence is considered to stretch from the onset of puberty well into the mid-20s. It is a unique period, distinct from both childhood and adulthood, such that some characteristics, for example, spatial ability, actually decline with the onset of puberty, whereas others, like perception of danger, are temporarily altered during this period. Normal fears of danger are temporarily suppressed during adolescence, a shift evolutionarily rooted in the need to leave home and explore new habitats. Studies have found that adolescents fail to change their appraisal of risky situations even after being warned that the hazards are greater than they expect. This leads to the belief that they will escape the worst consequences of certain actions, a bias that only begins to diminish by the mid-20s. Steinberg describes adolescence as a period of “especially heightened vulnerability” resulting from potential disjunctions between the developing brain and the behavioral and cognitive demands placed on the young person. It is highly likely that this bias affects young people’s ability to adhere to lifestyle recommendations and medication.

Typically, during adolescence, emotional states run high, including anxiety levels. Young people feel the whole range of emotions as adults but have yet to develop the sophisticated executive functioning skills required to manage them. Attempting to make young people anxious about their condition by telling them off for poor adherence or management of their condition is more likely to lead to avoidance and subsequent disengagement with health professionals rather than to a change in their behavior.

Increases in impulsivity, delay discounting (the extent to which consequences decrease in effectiveness to control behavior as a function of there being a delay to their occurrence), reward seeking, and emotional reactivity are noted in adolescence, which make this period a time of heightened vulnerability of patients to taking risks with their health. This sense of being invincible and bulletproof can have helpful implications in the process of adolescence because it gives young people the courage to separate from parents and manage other changes in a developmentally appropriate and expected way (at least within Western models of adolescence). However, these feelings of invincibility can have more negative consequences for self-management of chronic illnesses. For example, adolescents who feel they are invincible would be more likely to believe that any negative consequences of missing medication will not happen to them.

Peer acceptance is key in adolescence, and young people consistently report a strong desire to feel normal at this age. Young people will go to great lengths to appear normal, and having a chronic condition impacts this desire and is perceived as unfair. This can explain why previously adherent adolescents suddenly stop taking their medication and engage in more risk-taking behaviors.

Growth and Puberty

Puberty involves significant changes to a young person’s body, which typically occur simultaneously with an increase in the importance of appearance and peer acceptance. The reciprocal interaction between chronic illness and puberty on various levels, including biological, psychological, and social, has been comprehensively illustrated by Suris and colleagues; however, it remains poorly investigated in the setting of liver disease and LT.

The Studies of Pediatric Liver Transplantation (SPLIT) registry found that 20% of a large cohort of pediatric liver transplantation (PLT) recipients had linear growth impairment at their last follow-up. With regard to pubertal development, only 61% of girls and 58% of boys aged 16 to 18 years had reached Tanner 5 pubertal stage (compared with 100% of a normative population). Even in those reaching Tanner stage 5, 11% had a linear growth impairment. In a more recent study, height at time of LT was the most important predictor of long-term growth, with catch-up growth only occurring within the first 2 years after transplant. Whereas pubertal delay is common in young people with chronic health conditions, its prevalence in chronic liver disease and LT recipients is not well documented. What is known in female adults is that end-stage liver disease alters the normal physiology of the hypothalamic-pituitary-gonadal axis and disturbs estrogen metabolism, which affects sexual function. This appears to settle after LT, as demonstrated in an adult study where 28% of 64 women with liver disease had irregular menses and 30% had amenorrhea before transplantation, whereas 95% of those under 46 years of age reported a normal menstrual cycle within the first year post-LT.

The psychological impact of both delayed or impaired puberty and growth should not be underestimated, especially considering how young people want to be normal and fit in. One study found an association between short stature (i.e., height for the lower 15% of the sample) and poorer body image in males but no association for females. This finding is consistent with that of a study by Tiggemann. A study from our center in 80 young people with liver disease (30% post-LT) demonstrated a poorer body image perception compared with the general population, and interestingly this did not appear to be related to having a surgical scar or perceived side effects of immunosuppressive medication, as body image was comparable across liver disease diagnoses.

Cognitive Development and Challenges in the Liver Transplant Setting

Cognitive ability in children and young people (CYP) is highly related to health behaviors and is predictive for educational attainment and subsequent occupational outcomes. Research indicates that just 18% to 44% of adult LT recipients return to employment post-transplant, which is considerably lower than other solid-organ transplant recipients and the national average. This highlights the importance of a better understanding of cognition and its mediating factors in this population to promote a better quality of life for young people entering adulthood post-LT.

In transplant recipients, cognitive outcomes appear to be negatively associated with longer illness duration or time spent on the transplant waiting list, and the most significant predictor for specialist education requirement post-transplant is the pre-transplant requirement for additional support. This suggests that most cognitive deficits requiring specialist education provisions originate before transplantation, and it highlights the importance of early exploration of cognition in all CYP with liver disease, including those pre-transplantation.

It is important to highlight that cognition goes beyond general intelligence, which has been the focus of most studies to date. Ee et al. demonstrated that, despite normal IQ scores, young LT recipients had difficulties in executive functioning, particularly in self-regulation, planning and organization, problem solving, and visual scanning, deficits that are also reflected in the adult literature.

Adolescence is a challenging period for young people, and in particular for those with a chronic condition. The second part of the chapter will focus on challenges such as adherence, mental health, and acquiring skills to manage a chronic condition.