Transitioning in hydrocephalus: Current practices in

Paediatrics & Child Health, 2018, 1–6 doi: 10.1093/pch/pxy016 Original Article

Original Article

Transitioning in hydrocephalus: Current practices in Canadian Paediatric Neurosurgery centres Navneet Singh FRCSEd (Neuro.Surg) MSc PhD1, Patrick J. McDonald MD MHSc FRCSC1,2,3,4 Division of Neurosurgery, BC Children’s Hospital, Vancouver, British Columbia; 2BC Children’s Hospital Research Institute, Vancouver, British Columbia; 3Department of Surgery, University of British Columbia, Vancouver, British Columbia; 4National Core for Neuroethics, University of British Columbia, Vancouver, British Columbia 1

Correspondence: Patrick J. McDonald, Division of Neurosurgery, BC Children’s Hospital, 4480 Oak St. K3-216, Vancouver, BC V5Z 1B4. Telephone (604)875-2094, fax (604)875-3109, e-mail [email protected]

Abstract Introduction: Advances in perinatal care in the developed world have resulted in more children living into adulthood with complex chronic health conditions. Transition is a process to improve and maximize the functional status of adolescents via the provision of adequate and appropriate health services in adulthood. This process is frequently disjointed, fragmented and inconsistent and inadequate transition increases morbidity, hospital admissions and urgent interventions. Ten thousand children are diagnosed with hydrocephalus annually in North America. Most survive to adulthood yet there are few transitioning programs and little research data on successful programs for this population. Methods: An email survey of paediatric neurosurgical centres in Canada was carried out to establish current transition practices and attitudes for adolescents with hydrocephalus. Data were analyzed descriptively. Results: Eleven out of 12 centres responded. The age of transition ranged from 16 to 18 years. Four centres have access to a dedicated Adult Hydrocephalus Clinic. Referral practices vary between centres and we highlight inconsistencies in care to this cohort of patients in Canada. There is little satisfaction among neurosurgeons with current transition arrangements in Canada. Several suggestions were made on how to improve this process. Conclusion: We recommend research into the needs of patients with hydrocephalus in order to formalize appropriate standards for transitioning patients with a view to developing national guidelines to standardize the transition process. This will require input from patients, families and the wider medical and allied health professional groups. Keywords: CSF shunt, Hydrocephalus, paediatric, transitioning.

CHRONIC ILLNESS AND TRANSITION In high-income countries, 10% of adolescents suffer from a chronic medical condition (1). This rises to 15% among rural residents, lower social classes and the less educated (2). There is a 3.5 to 35.3% prevalence of chronic conditions in childhood (3) and one in five children aged 6 to 21 in the USA has a chronic disease (4). In the USA, chronic disease is responsible for 10% of paediatric hospital admissions (5). Improved diagnosis and management in health care including perinatal care has led to increased survival of neonates and infants into

adolescence and adulthood. It follows then that many of these patients with complex health care needs now need ongoing care into adulthood. In fact, in higher-income countries, up to 90% of children and young adults with special physical health care needs are now surviving to adulthood. This amounts to approximately half a million people annually in the USA and over one million if serious mental health issues are included (6). The American Academy of Pediatrics (AAP) defines the goal of transition as ‘a process to maximize lifelong functioning and potential through the provision of high quality, developmentally appropriate

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health care services that continue uninterrupted as the individual moves from adolescence to adulthood’ (7). The process can be broken down into a multistage pathway from preparation and engagement of young adults with their health care condition through to acceptance of responsibility for that condition and the actual transfer of care from paediatric to adult services and facilities (8,9). Failure of proper transitioning can lead to increased morbidity and increased hospital admissions and urgent interventions (10). Patients with neurocognitive disabilities may be at the highest risk from poor transition practices (11). Inadequate transition arrangements also lead to negative outcomes in terms of population health, cost and experiences with the health care system (12,13). They also identify a lack of progress in transition program development over the course of nearly a decade (14). Despite evidence of the importance of adequate transitioning, the transfer from paediatric to adult care is often disjointed, with fragmented services, gaps and inconsistencies in transition arrangements for a number of health conditions (1,15).

HYDROCEPHALUS Hydrocephalus, the pathological accumulation of intraventricular cerebrospinal fluid and accompanying ventricular dilatation, is a significant cause of morbidity and mortality in the paediatric population. It affects 6 in 10,000 live births with a 13% neonatal mortality rate prior to initial discharge (16). An estimated 10,000 children per year are diagnosed with hydrocephalus in the USA and Canada (17). Improved recognition and treatment of hydrocephalus, in conjunction with advances in other complex paediatric health conditions, has resulted in an

increase in children with treated hydrocephalus surviving into adulthood. Yet, unlike conditions such as cystic fibrosis or congenital heart disease, there are few existing programs for transitioning adolescents with hydrocephalus into adult care. Indeed, there is little research evidence to guide such programs or indicate what a successful program might look like. We report the results of a survey of paediatric neurosurgical centres across Canada, initially undertaken as part of a quality improvement initiative, to investigate transition practices for adolescents with hydrocephalus in their institutions and to consider whether formal, national guidelines are required to standardize the transition process.

METHODS AND RESULTS A six-question survey (Table 1) was emailed to neurosurgeons identified to have an interest in hydrocephalus at each of the 12 paediatric neurosurgery centres in Canada through the Canadian Pediatric Neurosurgery Study Group (CPNSSG). Two reminder emails were sent to the individual who had not completed the survey at 2 and 4 weeks after the initial contact e-mail. Alternative representatives were not sought after the final e-mail as only 1 of the 12 neurosurgeons did not respond. Answers were compiled and reviewed and common themes identified. Eleven of 12 (92%) centres responded (Table  1). The age of transitioning from paediatric to adult care ranged from 16 to 18  years (average 17.7). Four institutions were able to follow patients over 18  years old by special arrangement. Four centres had access to a dedicated Adult Hydrocephalus Clinic although one of these predominantly sees patients with normal

Table 1. Survey questions distributed to 12 peadiatric neurosurgery centres in Canada and a summary of responses

Question

Answer summary

1. At what age do patients at your institution transition to the adult care? 2. Do you continue to follow hydrocephalus patients as adults? 3. If answer to Q2 is no, is there an Adult Hydrocephalus Clinic you can refer patients to?

16–18 years

4. If answer to Q3 is no, how do you choose who to refer adult patients to? 5. Are you happy with how paediatric hydrocephalus patients are transitioned? 6. Do you have any suggestions on how we can make the transitioning process better for patients?

Four institutions follow paediatric patients into adulthood Four centres have access to a dedicated Adult Hydrocephalus Clinic • One of these predominantly sees patients with normal pressure hydrocephalus • One sees only local patients • Two see all paediatric patients • Two centres refer to specific adult colleague • Three centres refer to ‘adult neurosurgery’ • One centre refers the family doctor or paediatrician Three of 11 said yes

Eight of 11 said no See Table 2

Survey questions distributed to 12 paediatric neurosurgery centres in Canada and a summary of the answers.

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Paediatrics & Child Health, 2018, Vol. XX, No. XX pressure hydrocephalus (a condition largely confined to the elderly rather than children), one sees only local patients and two see all patients in their catchment area. Referral practices also varied. Two paediatric centres refer patients to a trusted adult colleague, three make a generic referral to ‘adult neurosurgery’ and one centre refers transitioning patients to their family doctor or primary care physician with advice to attend the emergency department if problems arise. Only 27% (3 of 11) of paediatric centres responding to the survey felt that their patients with hydrocephalus were adequately and appropriately transitioned into adult care. Several suggestions were made on how to improve this process. Recurring themes for improvement (Table 2) included adult hydrocephalus clinics run by a paediatric neurosurgeon, an adult neurosurgeon running a dedicated hydrocephalus clinic, referral to an adult neurosurgeon with an interest in hydrocephalus treatment and a joint paediatric/adult transitioning service staffed by both adult and paediatric neurosurgeons. It was suggested that proper transitioning requires clear documentation of clinical and shunt history including recent imaging, details of how the patient presents when their shunt fails as well as a protocol for follow-up intervals. One respondent felt that paediatric neurosurgeons needed to ensure better independence and ownership of health needs by patients and caregivers and was concerned that patients were too ‘babied’ in paediatric care, thus hampering their ability to take ownership of their condition.

DISCUSSION Transitioning and its challenges has been gaining attention as far back as 1989 at the Surgeon General’s conference on Growing Up and Getting Medical Care: Youth with Special Health Care Needs (15). Although for many adolescents with special health care needs transition does progress smoothly, for those with more complex needs or conditions affecting the nervous system, transition is not as optimal (11). The gold standard of care should be a smooth, seamless transition (15). In order to develop such a program, a picture of what an ideal transition looks like is required. A consensus statement from the American Academy of Pediatrics, the American Academy of Family Physicians and the American College of Physicians highlighted six critical steps to enable adequate transitioning (7). Five additional steps have subsequently been identified to create an 11-step plan for seamless transitioning (18). This includes, but is not limited to, identifying a designated transition co-ordinator and an adult provider, creating a portable, written medical summary, a plan for emergencies and discussions with the patient separately and together with their family. Young adults need to be empowered to acquire the knowledge and skills necessary to successfully navigate the adult health care system independently and to take on responsibility for their own health with a view to minimising complications and secondary illness (15). Transition should be a gradual process over time, appropriate for developmental stage rather than an abrupt change at a specific age (1).

Table 2. Proposed models of hydrocephalus transitioning (Common themes in bold, individual are in italics)

• Paediatric neurosurgeon running adult hydrocephalus service • Continued care into adulthood with the paediatric team in a congenital conditions clinic • Paediatric neurosurgeon who also has to do adult hydrocephalus and will be given 1 day a month of elective time and if wait list gets too big then more time • Monthly adult hydrocephalus clinic run by paediatric neurosurgeons • Adult neurosurgeon running adult hydrocephalus service • Adult neurosurgeon interested in hydrocephalus and other congenital neurosurgical conditions that present in adulthood (e.g., occult dysraphisms) • Adult hydrocephalus clinic with a genuinely interested adult neurosurgeon • Inviting the proposed adult surgeon to paediatric clinic to meet the transitioning patient in the patient’s familiar environment prior to transfer • Hybrid/joint service • Hydrocephalus clinic which would see adult and paediatric patients • Adult clinic with hydrocephalus and spina bifida multidisciplinary would be better • Hybrid practice—paediatrics and congenital adult neurosurgery. This would require adult and paediatric facilities to either be connected, or very close • Other • Part of our job is to get them prepared for the adult world where they are taking more responsibility for their health and to understand that adult neurosurgeons will only want to see you when you have a problem. • Having a ‘go to’ person or clinic is a necessity • Nurse practitioner/specialist-led clinic Proposed models of hydrocephalus transitioning. Common themes are in bold. Individual responses are in italics.

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4 Transitioning is a challenging time for adolescents, their families and also health care providers (19). Given that adolescence brings with it many challenges of its own (leaving home, moving to university, relationships and starting careers) preparation for clinical transition should commence early in adolescence (20). Inadequate transitioning affects up to 60% of adolescents with complex health needs (21) and the quality of transition processes can vary according to the underlying health issues and the specialists involved (22). A 2002 consensus statement described multiple deficiencies in transitioning arrangements (7). In particular, patients were not being prepared for transition, communication between specialty and primary or home care providers was inadequate and the adult system was not adequately resourced and was ill-prepared to deal with an influx of new, young adults with complex health needs. There was a lack of role clarity and expectations for adolescents and their families and a lack of familiarity in how to work in partnership with adult physicians (23). Other barriers include an unwillingness of paediatricians and parents to ‘let go’ and relinquish responsibility for clinical care of their patients and children (15,24). Equally, adolescents may struggle to break ties with their paediatrician and form a new relationship with an adult provider (25). Most studies on the transition from paediatric to adult centre health care focus on young people with cystic fibrosis, cancer, congenital heart disease, arthritis and diabetes—all conditions with existing specialists in the adult sector and now well-established transitioning guidelines (15). Patients with hydrocephalus require continuous access to adult neurosurgeons as shunts and endoscopic third ventriculostomies can fail at any time (26). Although almost all adult neurosurgeons treat adults with hydrocephalus, in the paediatric population, its causes and managements are different from that seen in adults and it is frequently seen as part of wider neurological or syndromic disease which requires a more holistic approach to management. With increasing subspecialization in neurosurgery, fewer adult neurosurgeons maintain experience in the management of childhood-onset conditions and may feel unable to adequately manage such patients in adulthood. Indeed, some comments in our survey highlighted this as an issue and had recommended hiring a paediatric neurosurgeon who also managed the adult hydrocephalus service to allow for improved continuity, or a hydrocephalus clinic that saw both adult and paediatric patients. Others highlighted the need for an adult neurosurgeon with a specific interest in hydrocephalus to take on transitioning patients in the adult centre. A lack of interested adult providers is an issue not only for hydrocephalus, but is a commonly cited barrier to transitioning throughout the literature, in particular the lack of training and experience in childhood-onset conditions among adult physicians (15,18,25,27,28). A perceived lack of time on the part of adult clinicians may exacerbate the shortage of providers in the

Paediatrics & Child Health, 2018, Vol. XX, No. XX adult sector. One institution has developed a successful transition program whereby comprehensive care for transitioned patients with hydrocephalus was managed by multidisciplinary teams including adult and paediatric physicians in a paediatric setting (26). In our survey, one respondent commented that since commencing an adult spina bifida clinic 11  years previously, the two top issues encountered were urological and hydrocephalus-related. A  model program for transitioning patients with spina bifida supports the notion that transition should be a multidisciplinary process involving social workers, physiotherapists, nurses, community paediatric and adult providers as well patients and their parents as well (1,20). Multidisciplinary clinics exist in the paediatric setting, but may be more fragmented in the adult sector. Parents and extended family networks can facilitate effective self-management of disease in adolescence in contrast to single parent families and those with lower socioeconomic status (29,30) while also providing emotional and practical assistance with issues such as transportation, medication and appointment reminders (19). Similarly, young adults benefit from the support offered by peers during the transition process reporting reduced anxiety (1). Very little data is available in the literature to inform optimal hydrocephalus transitioning and the heterogeneity of the different paediatric conditions that have been better studied may limit their generalizability to the hydrocephalus population. Equally, it is impractical and costly to devise separate transition programs for each and every childhood condition in every institution. Research data can still be used to guide transition principles and a practical approach may be to create core transitional care with supplementary specialty involvement depending on the systems involved. This, alongside paediatric and adult facilities working in partnership, may help provide a more patient-centred and personalized approach to care (28). However, questions remain on how this should be financed and delivered and how transitioning can be pushed higher in the priority list for health care leaders. A stronger evidence-based case for transition will support this. Potential areas to explore include how to measure transition outcomes, identifying the impact of failed transition, defining successful transition (15) and the factors that influence transition and effective self-care in adolescents (19). More importantly, the experiences of patients with hydrocephalus and their families need to be explored in order to develop a more patient-centred approach to the transitioning process. Although many programs lack a written transition guideline, those with younger paediatricians (those practising less than 20 years) are more likely to do so which may reflect the impact of modern residency training and increasing awareness around the issue of transitioning.

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CONCLUSION We present a snapshot of transitioning practices for patients with hydrocephalus in Canada and highlight inconsistencies in the way paediatric patients with hydrocephalus are transitioned to adult care, from the perspective of paediatric neurosurgeons in Canada. Most paediatric neurosurgery centres are not satisfied with the current transitioning arrangements and wish to streamline this process more effectively. We suggest that a detailed review into the needs of patients with hydrocephalus be undertaken with a view to developing formal guidelines and standards for transitioning patients. We recognize that any policy developments must also consider viewpoints of patients, families and the wider medical and allied health professional groups. The results of this survey provide an overview of current practices and opinion of practicing neurosurgeons and can be a platform on which to develop future guidelines.

Conflict of Interest None.

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