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Considerable variations in growth hormone policy and prescription in pediatric end-stage renal disease across European countries – a report from the ESPN/ERA-EDTA registry Nephrol Dial Transplant 2015; https://doi org/10.1093/ndt/gfv105 44 Varni JW, Limbers CA, Burwinkle TM. Impaired health-related quality of life in children and adolescents with chronic conditions: a comparative analysis of 10 disease clusters and 33 disease categories/severities utilizing the PedsQL 4.0 Generic Core Scales Health Qual Life Outcomes 2007;5:43 45 Varni JW, Seid M, Rode CA. The PedsQL: measurement model for the pediatric quality of life inventory Med Care 1999;37:126–39 46 Warady BA, Fischbach M, Geary D, Goldstein SL. Frequent hemodialysis in children Adv Chronic Kidney Dis 2007;14:297–303 47 Wightman A, Bradford MC, Smith J. Health-related quality of life changes following renal transplantation in children Pediatr Transplant 2019; https://doi org/10.1111/petr.13333 48 World Health Organization https://www.who.int/ healthinfo/survey/whoqol-qualityoflife/en/ Transition and Transfer to Adult Care for Adolescents and Young Adults with Advanced Chronic Kidney Disease 41 Lorraine E. Bell and Dirk Bethe Introduction Clinical Vignettes Experiencing chronic illness during adolescence or young adulthood is a major challenge For patients with advanced chronic kidney disease (CKD), it can be overwhelming If the onset of CKD is during adolescence, hopes and dreams for the future may be shattered When it begins earlier, there may be lost opportunities for normal psychosocial milestones and development These children and adolescents often miss out on typical peer activities, socially, academically, and vocationally Generally, their transition to adult healthcare occurs during one of the most emotionally vulnerable stages of development, the period of emerging adulthood The clinical vignettes below illustrate a number of the challenges involved in transition In this chapter, we will examine these and other transition elements in greater detail Sally was diagnosed with pauci-immune glomerulonephritis and pulmonary hemorrhage 9 months ago at the age of 16 years Her treatment included plasma exchange, corticosteroids, and rituximab Although she stabilized clinically, her renal function is declining, and she’ll need dialysis within a month Prior to her illness, she had no health issues and was an excellent student and musician Her dream is to become a concert violinist Harry is 16 years old and has been on hemodialysis for 4 years He received a kidney transplant 5 years ago for end-stage renal disease (ESRD) due to focal segmental glomerulosclerosis (FSGS), but his disease recurred aggressively, and despite intensive multifaceted treatment, his graft failed within 9 months He lives in a foster family and is worried about what will happen when he turns 18 His dream is to be healthy and to have supportive friends and family Casey is almost 18 years old and has had chronic kidney disease (CKD) since infancy She received a kidney transplant at the age of 6 years and did well until mid-adolescence At the age of 16, she lost her transplanted kidney due to antibody- mediated rejection and has been on peritoneal dialysis since She is now highly sensitized Her home is 300 miles from the nearest pediatric dialysis and transplant center She struggles with taking her medication and with following her diet restrictions Two years ago she L E Bell Department of Pediatrics, Division of Nephrology, McGill University Health Centre, Montreal Children’s Hospital, Montreal, Québec, Canada e-mail: lorraine.bell@mcgill.ca D Bethe (*) Pediatric Nephrology Division, University Hospital Heidelberg, Heidelberg, Germany e-mail: dirk.bethe@med.uni-heidelberg.de © Springer Nature Switzerland AG 2021 B A Warady et al (eds.), Pediatric Dialysis, https://doi.org/10.1007/978-3-030-66861-7_41 793 L E Bell and D Bethe 794 dropped out of school, because it was too great an effort Her dream is to get a new kidney Jordan is 21 years old and recently transferred to an adult hemodialysis unit He often arrives late, and he is commonly very fluid overloaded While in pediatrics he was listed for a transplant but didn’t get an offer; now the adult transplant center is considering withdrawing him from the active list because of adherence concerns He finds it very difficult to be in a dialysis unit where patients are decades older and very ill-looking, and he avoids most social contact Simon is 18 years old and has had CKD since childhood He started peritoneal dialysis a year ago and was recently transferred to an adult unit His mother accompanies him to all of his healthcare appointments and provides most of the history When questioned directly, he is unable to describe the nature of his underlying kidney condition, as well as the names of or the reasons for most of the medications he takes The personal and treatment challenges illustrated in the above vignettes include: Coming to terms with the sudden onset of a life-threatening condition and complex treatment in the teenage years Dealing with a serious disease for which there are limited treatment options Treatment adherence Poor psychosocial support Living with kidney disease from an early age Missed educational opportunities Depression Lack of patient autonomy in the understanding of their disease and its treatment The change in environment from pediatric to adult care These elements and more need to be considered in transitioning a patient from pediatric to adult care Overview Transition is a purposeful multiyear process that begins during pediatric care and continues after transfer, until the patient is fully integrated into adult care The goal is to maximize lifelong functioning and potential through comprehensive patient-centered, flexible, and developmentally appropriate care that is continuous from adolescence to adulthood [1] For many patients with chronic illness, the process of transition may continue until they are in their mid-20s, for reasons discussed in section “Attention to Psychosocial Issues and Needs, Including Educational Challenges” A number of publications have looked at barriers to transition, across a wide range of conditions [2–5] Common themes are listed in Table 41.1 Beneficial aspects of transition to adult care have also been described by recently transferred youth; they appreciate the focus on responsibility and self-management, a business- like atmosphere, information relevant to adult issues, new treatment options, and the possibility to choose a hospital closer to home The primary determinant of a positive transition experience was the patient-centeredness of the adult-oriented provider and collaboration between the adult and pediatric services [6] Helping young people successfully transition into the adult world requires a multifaceted team approach that supports them from their early teenage years to at least their mid-20s Several excellent and comprehensive guidelines have been published on the adolescent to adult transition of care in the UK, USA, Canada, and other countries [7–12] An outstanding website for transition resources is available at Got Transition [13] However, successful implementation of guidelines requires support from multiple disciplines and very importantly from high-level hospital administration Resources are needed in terms of healthcare professionals, organizational support, and information technology 41 Transition and Transfer to Adult Care for Adolescents and Young Adults with Advanced Chronic… Table 41.1 Potential transition barriers [2–5, 9, 83] Patient and family related potential barriers Difficulty “letting go” of long-standing relationships with pediatric providers Fear of poor outcomes in relinquishing control of the condition to the young person Limited knowledge about their medication, illness, and the transition process Insufficient self-management skills Poor treatment adherence Limitations in neurocognition and/or developmental immaturity Difficulty developing trust in new adult site providers Feeling overwhelmed by the adult environment (described as impersonal, unwelcoming, and difficult to navigate) Psychosocial issues (e.g., depression, anxiety, denial of illness, mental health and substance issues, absent/unsupportive caregiver, unstable life circumstances) 10 Schooling and vocational issues 11 Competing life demands 12 Lack of peers with the same condition Key elements of all these published guidelines include the following: Having a named healthcare worker responsible for the patient’s transition process and a transition champion Starting early Timely attention to psychosocial issues and needs, including educational challenges, that patients may face Regular evaluation of adherence, including barriers and facilitators Patient and family engagement and empowerment Regular assessment of transition preparation using standardized tools and development of individualized transition plans Tracking progress of individual patients and of the clinic/program’s transition process as a whole Appropriate timing of transfer A succinct relevant patient transfer summary 10 A personalized health passport or summary document for the patient 11 Open bidirectional communication between the pediatric and adult teams 795 Provider and system related potential barriers Lack of institutional backing to effectively promote good transition practices Inadequate transitional care funding and resources Poor transition organization and support Lack of coordination for patients with need for several subspecialty services Inadequate communication from pediatric clinicians, including medical records and follow-up recommendations Lack of sharing of protocols between the pediatric and adult sites Limited availability of adult providers Insufficient training of adult providers in adolescent medicine and pediatric-onset conditions Shorter appointment times in adult healthcare system 10 Lack of mental health and support services in adult care 11 Change or loss of insurance coverage when reaching adult age 12 Recognition by the adult team of the young adult’s developmental needs 13 Continuation of the transition process after transfer to adult care, until the young person is fully integrated and able to function in the adult system 14 Education and training for healthcare professionals There is also a need for research, quality improvement assessment, consideration of cost, and advocacy for health policy change We will review each of these components in the following sections Named Healthcare Worker and Transition Champion The named worker can be someone with a formal position such as a patient navigator [12, 14–21] or transition coordinator or can be a designated nurse in the renal unit The importance of appointing someone to be responsible and providing them with sufficient time and resources cannot be overemphasized If not, there is an ongoing challenge during clinic visits, or in the dialysis unit, L E Bell and D Bethe 796 Table 41.2 Roles of transition navigatora or coordinator [12, 14–16, 18–21] Clinical Administrative • Track patients and outcome data • Transition readiness assessments • Develop and update educational material • Creation of medical passport • Facilitate group encounters (e.g., peer mentorship, • Patient education and self-management coaching educational evenings, etc.) • Screening for transition concerns such as • Participate in quality improvement psychosocial issues and comorbid diagnoses • Provide coaching on adherence • Encourage healthy lifestyle habits (diet, exercise) • Provide support for medical or mental health crisis management Navigational Patient advocacy • Evaluation of whether the patient will need • Help establish relationships with primary care community navigational support (e.g., providers and appropriate specialty care providers developmental services, mental health services, • Work with patient and healthcare providers to postsecondary education accommodations) promote continuity of care • Assist patients and families navigate financial • Assist with data sharing between pediatric and adult barriers to healthcare (e.g., health and medication service providers insurance changes at transition, transportation issues) • Facilitate and accompany patient to first adult site • Address concerns of autonomy and potential need appointment for surrogate decision-maker • Track follow-up appointments, medication refills, • Engage patients and families (get their input) in the and laboratory tests to flag early for nonadherence planning of transition interventions • Assist with health system navigation at and between appointments These tasks can be shared by several members of the multidisciplinary team, but a named person needs to coordinate and be responsible for the process a to balance planning for future events (transition and transfer) with day-to-day demands In nephrology, the transition navigator could be responsible for children on dialysis as well as those with kidney transplants, advanced CKD, or milder CKD combined with other complex conditions or needs Having one navigator for dialysis, transplant, and advanced CKD patients facilitates continuity of care during their transition preparation years Typical roles of a transition navigator are listed in Table 41.2 These multiple tasks could be shared by several people, but there must be someone to oversee and coordinate the process For programs with relatively small patient numbers, the coordinator or navigator position could be shared with other specialties, allowing for the development of expertise There is also a very important need for a transition champion, a leader who advocates for and promotes optimal transition practices, within individual programs and at the broader hospital level Often this person is a physician or nurse Starting Early Most guidelines recommend formally starting the transition process at around the age of 12 to 14 years [10, 11, 13, 22] However, thinking about this future task should begin much earlier; some would advise shortly after diagnosis [11] This is because young people with chronic disease need to integrate the care and responsibility for their health into their daily lives and develop confidence in their abilities Even small children can play an active role in their care, so that participation in their treatment becomes a natural part of their everyday life Their parents are integral to this process The active involvement of children and adolescents in their treatment also promotes their feeling of self-efficacy A longitudinal study of adolescents and young adults with a variety of chronic conditions showed that perceived self-efficacy is associated with a better healthrelated quality of life (HRQoL) [23] 41 Transition and Transfer to Adult Care for Adolescents and Young Adults with Advanced Chronic… ttention to Psychosocial Issues A and Needs, Including Educational Challenges Young people with advanced CKD begin adolescence, and thus transition, in the context of a very serious disease They and their family may have already had to deal with considerable psychosocial challenges before adolescence: these include incurability of the disease, threat to life, painful and unpleasant examinations and treatments, adherence to numerous medical prescriptions, instructions and restrictions, dependence on medical devices and healthcare providers, violation of body image, problems of social integration, neglect of parents’ individual and marital needs as well as of siblings’ needs, challenges with regular school attendance, cognitive delays associated with earlyonset renal failure [24, 25], burdens for some of additional disabilities and comorbidities, and socioeconomic hardships For other adolescent patients, advanced CKD and ESRD may seem to appear abruptly (e.g., patients with rapidly progressive glomerulonephritis or those with minimally symptomatic renal tubular and interstitial disease or dysplasia) They are suddenly struck with a serious disease and have not grown up with the idea that their life has limitations This group of patients may have major difficulties in adapting to illness and treatment and need special attention Studies have shown that HRQoL in pediatric patients with CKD, especially dialysis patients, is lower compared to healthy peers [26] but also compared to patients with other chronic diseases [27, 28] This is also reflected in mental health For example, Clementi et al [29] report increased rates of depression and anxiety disorders, lower selfesteem, and a higher level of social impairment in pediatric dialysis patients Therefore, patients and their families often carry a heavy burden when they enter adolescence and the transition process begins Growing up is a challenge for everyone Age- specific developmental tasks and challenges include identity formation, development of autonomy, identification with peer groups, emotional bonds, cognitive development, physical 797 Maturation Limbic reward areas Prefrontal cortex Risk phase Age Adolescence Fig 41.1 Nonlinear maturation processes of subcortical and prefrontal brain areas lead to an imbalance of neural networks in adolescence Age-typical risk-taking behavior and sensation-seeking are associated with this asynchronous maturation (Modified from Casey et al 2008 [30]; reproduced with permission of John Wiley and Sons) development, sexuality, forming one’s own philosophy of life, and school and vocational training Age-specific changes of brain structure and function have been shown in MRI studies The developmental changes in the brain continue beyond the age of 18, well into the third decade of life The brain develops asynchronously: the limbic system, associated with emotions, reward, impulsivity, and motivation, matures fairly early in adolescence, whereas the prefrontal cortex, involved in executive functions such as planning, problem-solving, and impulse control, doesn’t reach maturity until the middle of the third decade of life [30] Age-typical risk-taking behavior and sensation-seeking are associated with this asynchronous maturation (Fig. 41.1) Although these age-typical processes affect all young people, adolescents and young adults with a serious chronic disease such as CKD face particular challenges, including: dherence to Treatment, Despite A Asynchronous Brain Development In order not to endanger their health, they have the additional burden of adhering to their medical ... of 12 to 14 years [10, 11, 13, 22] However, thinking about this future task should begin much earlier; some would advise shortly after diagnosis [11] This is because young people with chronic disease... who advocates for and promotes optimal transition practices, within individual programs and at the broader hospital level Often this person is a physician or nurse Starting Early Most guidelines... doesn’t reach maturity until the middle of the third decade of life [30] Age-typical risk-taking behavior and sensation-seeking are associated with this asynchronous maturation (Fig. 41.1) Although