Gaps in transitional care to adulthood for patients with cerebral palsy: a systematic review

A systematic review was conducted using PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines [17] to investigate the current state of transitional care for young persons with CP and current healthcare attitudes. Three databases were searched in December 2020: PubMed MEDLINE (National Library of Medicine), Embase (Elsevier), and Scopus (Elsevier). Search strategies for Embase and Scopus were adapted from the PubMed search strategy shown in Supplementary Table 1. Date, study type, and language limits were not applied. Searches were conducted using controlled vocabulary (MeSH), keywords, and keyword synonyms.

Duplicates were removed after search completion using the automated deduplication feature in Endnote X9 (Clarivate, London, UK). The remaining eligible studies were screened by title and abstract for relevance. Those selected for full-text review were screened using predetermined inclusion and exclusion criteria. Two authors, DLM and EW, completed both stages of screening. Conflicts were reconciled by discussion to achieve consensus.

Inclusion criteria were publications in or translated into English, with full text and abstract available, studying a transition-age patient population (between 16 and 24 years old) with the diagnosis of cerebral palsy and assessing healthcare use, functional or social outcomes, perspectives on transitional care, or current implementations of transitional care practices. Studies reporting data on adult and pediatric patients or for cerebral palsy and additional pathologies were included if definite outcomes for transition-age patients with cerebral palsy could be identified. Exclusion criteria were as follows: abstracts only, secondary literature, not assessing transition-age patients with cerebral palsy, and not reporting on specified outcomes.

Articles selected for final inclusion were assessed for bibliographic data, study design, participants, intervention where applicable, and outcome data. Outcome measures were specified a priori. The first primary outcome measure was transition readiness, which was assessed qualitatively via healthcare utilization metrics, social and functional outcomes, and patient, caregiver, and provider interviews and surveys. Healthcare utilization metrics included rates of primary care visits, hospital stays, specialist referrals and visits, and use of rehabilitation services. Social outcomes were assessed via level of social or community engagement, participation in support groups and activities, and relationship development. Functional outcomes were assessed via employment status, housing, and capacity to live independently, with consideration of Gross Motor Function Classification System (GMFCS) level. Key themes of interviews were summarized. Surveys ranged in structure and content, using various validated questionnaires, scales, or both. The second primary outcome measure was efficacy of existing transitional care interventions, which was assessed qualitatively via survey responses from program administrators, providers, patients, and caregivers.

Data was analyzed descriptively. Means were calculated as weighted means and reported with range when available. As data was heterogenous by study design and execution, data were not pooled, and a meta-analysis was not conducted.

The income status for countries of study origin was determined using the World Bank designation [18]. Quality and risk of bias were assessed using the GRADE framework [19] and Cochrane ROBINS-I (Risk of Bias in Non-Randomized Studies of Interventions) tool [20] for each of the included studies. Overall risk of bias for this systematic review was inferred based on risk of bias of each included study.

The search identified 3151 articles, of which 27 were included (Fig. 1, Tables 1, 2, and 3) [21‐47]. Study designs included longitudinal cohort (3, 11.1%), cross-sectional survey (10, 37.0%), qualitative interview (10, 37.0%), retrospective analysis (3, 11.1%), and retrospective cohort (1, 3.7%). All included studies originated from high-income countries, including the USA (9, 33.3%), the UK (7, 25.9%), Canada (4, 14.8%), Sweden (3, 11.1%), Scotland (1, 3.7%), Denmark (1, 3.7%), France (1, 3.7%), and the Netherlands (1, 3.7%). Twenty-three of the included studies [21‐25, 27‐29, 31‐38, 40‐44, 46, 47] (85.2%) assessed transition-aged persons with CP and four studies [26, 30, 39, 45] (14.8%) assessed transitional care interventions. The quality of most studies was moderate (12, 44.4%). Most studies had a moderate risk of bias (12, 44.4%), resulting in this review having a moderate risk of bias as well.

The 23 included studies (85.2%) assessing transition-aged persons with CP consisted of 39,245 adolescent and young adult patients with cerebral palsy (Tables 1 and 2). The mean age of study participants at time of assessment ranged from 2 to 44 years old, with a weighted mean of 19.6 years. Nine studies [23‐25, 28, 33, 34, 36, 41, 43] (39.1%) did not report a mean age. Six studies [32, 34, 37, 41, 43, 44] (26.1%) classified participants by cerebral palsy subtype, of which 72.7% (614/844) were spastic, 10.5% (89/844) were dyskinetic, 3.5% (29/844) were ataxic, 9.8% (83/844) were classified as “other,” and 3.5% (29/844) were unclassifiable. Additionally, 12 studies [21, 22, 24, 27, 32, 37, 38, 40‐42, 44, 47] (52.2%) classified participants according to GMFCS level, of which 58.1% (757/1304) were levels I–III (mild/moderate impairment, ambulatory), 41.2% (537/1304) were level IV/V (severe impairment, non-ambulatory), and 0.8% (10/1304) were unclassifiable.

Forty-two percent of parents of transition-age youth with CP reported their doctors having discussed the changing healthcare needs of their child as they aged [25]. However, only 28–29% reported that their doctors had discussed the eventual need to see adult providers or had provided help in coordinating transitional care [25]. Nonetheless, 64% of participants were able to formulate their care demands themselves [32]. Higher level of motor functioning, higher level of education, participation in activities, parent involvement, satisfaction with services, and meeting the adult team before transfer were associated with increased participation and independence in formulating personal care plans [29, 32].

Adults with CP had a significantly higher rate of both GP visits and annual physicals compared to pre-transition young persons, but the total rate of physician visits was not significantly different between age groups [46]. Hospital utilization increased significantly among transitional age patients with CP in terms of number of annual discharges, inpatient days, and charges [33].

Comparatively, there was a significant decrease in both access and number of visits to specialists by young adults compared to pre-transition youth [37, 41, 46]. Specialists provided 28.4% of youth visits but only 18.8% of adult visits [46]. 34.7% of specialist referrals were unable to be made as such services did not exist for adult patients [34]. Adult physiotherapy and occupational therapy services were particularly under-provided [34]. Use of rehabilitation services decreased with age, independently of GMFCS status, while use of psychotropic and analgesic medication increased with age [32, 34, 36, 41, 43]. The highest areas of unmet needs in specialty care were for management of pain, bone or joint problems, and speech [42].

Young adults with CP significantly lagged non-CP peers in their development of housing, employment, and intimate relationships [32]. Use of formal respite services, support groups, and youth clubs was also relatively poor [37]. Social engagement decreased with age [43]. Living arrangements differed significantly among GMFCS levels, with 70% of participants with severe disability living with their parents [21]. Nonetheless, 55.9% of young adults with CP lived with their parents overall [21, 22]. Unmet needs in daily living healthcare and personal assistance were associated with increased severity of motor impairment (GMFCS) and attending non-specialist education [21, 42]. Unmet needs tended to increase over time but were not significantly related to whether the young person had transferred from child services [42]. One representative cohort of young adults with CP was 34.3% students, 19.6% employed, 35.2% participating in daily activities, and 8.8% unemployed, with 90% of employed participants having a GMFCS level of I–II [21].

Five studies [22, 23, 28, 35, 40] assessed attitudes of young adults with CP regarding their lived transition experiences. Shared themes included a lack of transition readiness and support, becoming a young adult and associated challenges, progression of physical disability, struggling to navigate the healthcare system, and feelings of isolation and abandonment.

Four studies [24, 31, 38, 43] assessed parents and caregiver attitudes towards their child’s transition from pediatric to adult healthcare services. Overarching themes included anxiety about service provisions, difficulty obtaining help and informational resources, poor communication and transparency from healthcare providers, the lack of coping skills, and a desire for community support. Parents also reported significantly lower satisfaction with current transition care processes than their children [38].

One study [27] included the provider perspective on transition challenges for young adults with CP. Themes included problems with accessibility, the suitability of primary care environments for caring for individuals with complex needs, gaps in seamless care, and limited time and funding when receiving or providing primary care services to young adults with CP.

Four studies [26, 30, 39, 45] assessed current implementations of transitional care practice (Table 3). The top three perceived barriers to successful transition of care included limited adult providers willing to accept CP patients, concern about the level of care in the adult healthcare system, and the lack of financial resources [26]. One survey [26] of 11 clinics across the USA found that 55% of clinics had structured transition programs, but only one program successfully transitioned 100% of their patients to adult providers by age 22. Forty percent of the clinics had transitioned < 25% of their patients with CP to adult providers by age 22 [26]. Only one clinic had an absolute upper age limit for seeing patients, and 36% of clinics accepted new patients older than 21 [26]. None of the responding providers were “completely satisfied” with their transition process [26].

Significant gaps existed between the advertised services and the experiences of young people utilizing them [30]. Proposed beneficial features of transition services included a written transition plan, holistic life-skills training, and having a key worker, which only 30, 43, and 49% of young people agreed were available to them, respectively [30]. Agreement was better for appropriate parent involvement, age-banded clinic, promotion of health self-efficacy, and coordinated team at 77, 77, 80, and 69%, respectively [30]. Comparatively, a retrospective evaluation [39] of another transition clinic demonstrated that more than 2/3 of families reported receiving services across seven categories: support primary care, specialty care, school, legal, community inclusion, healthcare financing, and providing medical care. Key areas identified for improvement included coordination and communication between and within health services [45].

Consistently, young adults with CP have expressed not feeling ready for the transition to adulthood [22‐24, 27, 28, 31, 35, 40, 43, 44], as well as a desire for a comprehensive transition approach that prioritizes capacity building and personal empowerment [22‐24, 28, 29, 31, 35]. However, the results of this review demonstrate that the current state of transitional care is insufficient to meet the complex needs of young adults with CP. While many transition-age persons with CP and their caregivers are actively seeking both primary and specialty care, there is poor access [25‐27, 30‐38, 40‐43]. Medical providers with the expertise, office and financial resources, and capacity or willingness to see young adults with CP are limited in quantity and location [25‐27, 30‐38, 40‐43]. Furthermore, such providers may be difficult to identify, have long waiting lists, or be financially inaccessible. As a result, these patients are frequently being seen in the emergency room instead of by trained specialists equipped to manage their complex care needs [33]. While hospital staff may be able to manage short-term, emergent care in these patients, long-term goals of care and management are rarely addressed in this setting [48].

The impact of opportunities for improvement in transitional care is further illustrated by the social and functional outcomes of study participants after aging out of the pediatric system. A lack of early intervention to promote social and career development leaves many young adults with CP unable to manage independent living, build careers, form intimate relationships, or engage socially [21, 22, 24, 32, 37, 40, 43]. These findings were magnified in those with more severe disabilities [21, 32]. However, current literature suggests that many adults with CP can live independently and have a high quality of life, especially if they have a strong support system and continuous specialty care [49]. As such, transitional care for young adults with CP should include means to address their social, emotional, and financial needs as they navigate adulthood.

Those few structured transition-programs that do exist are notable, and geographic location and financial resources restrict wider availability [26]. Many of these programs accept patients with a wide range of chronic illnesses, so management may be difficult to tailor to individuals’ disease-specific needs [50]. Most clinics in the USA are in major cities, affiliated with large, well-resourced, academic institutions, and thus primarily serve urban populations. These factors can create additional barriers to access, including physical distance or financial challenges for travel. Even those with access to these programs report low rates of satisfaction with the transition process and support services [26, 30, 45], a sentiment reflected by caregivers and providers alike [24, 26, 27, 30, 31, 39, 43]. There is a need for continued evaluation and reform in transitional healthcare.

Many young adults with CP are lost to follow-up upon graduating from the pediatric healthcare system. The level of care coordination and close monitoring provided during childhood is often not continued or available to adults or older age groups, even in countries with universal healthcare coverage [51]. Many patients and their families may struggle to leave pediatric providers and multidisciplinary care teams with longitudinal relationships they have been with for their entire life and establish trust with a new provider or ecosystem. Release of records may be delayed or forgotten altogether as the time interval between providers grows [35]. As a result, transition planning tools that may have been constructed with the patient’s pediatric team are not optimally utilized. In addition, frameworks for transition of care exist, so the application of transition planning is not prescriptive. For instance, GotTransition.org outlines 6 domains, without a specific detailed way of making it happen. The implementation is thus variable based on many factors, including individuals, providers, and health system factors, which leaves the outcome somewhat to chance.

Young adults with CP struggle to navigate the complex terrain of the adult healthcare system, which assigns, functions, and delivers healthcare in significantly different ways than the pediatric system. Cognitive delay and worsening physical disability can create additional challenges for patients in the domains of self-advocacy, scheduling appointments, and coordinating their own care [51]. Aging out of “dependent” status on parent or caregiver insurance plans leaves many young adults with CP on public aid, requiring them to adapt their healthcare needs to newly limited provider coverage and benefits [51]. There is also generally greater support from ancillary services in pediatric care, such as social work, nurse coordinators, and resources for caregivers. However, similar resources are largely limited in the adult healthcare system, leading to increased demands on caregivers, stress, burnout, and difficulty managing their adolescent’s changing needs [24, 27, 43].

Providers also face obstacles in providing care, emphasizing lack of time, resources, financial reimbursement, and training [27, 52, 53]. Providers may consider themselves ill-equipped to manage young persons with CP due to limited education on treating spasticity, dystonia, and complex orthopedic issues in this population [53‐55]. Additionally, persons with CP often require wheelchair-accessible entrances, exam rooms, exam equipment, and office personnel trained in proper patient handling techniques. These patients may also require longer appointments or have public insurance, which some providers may struggle to accommodate [27, 52, 53]. While the Americans with Disabilities Act (ADA) requires medical service providers in the USA to make their services accessible [56], similar statutes do not exist in every country or may be upheld to varying degrees [57].

Adults with CP require the expertise of both pediatric and adult-trained providers who are familiar with the pediatric and adult manifestations of the condition. As such, collaboration between pediatric and adult providers is crucial to transition success [26, 33]. Pediatricians should begin the discussion and planning for transition to adult healthcare early in adolescence, so as to identify possible barriers to care early on [25]. Additionally, pediatric providers can actively seek out adult care providers in their communities with an interest in caring for patients with CP to start a dialogue focused on potential barriers to care and work towards building local networks of CP providers [26]. Such a network could assist in facilitating patients meeting their adult teams prior to transfer, which has been significantly associated with increased participation in organizing their own care [29]. Convening a national focus group of pediatric and adult care providers to discuss and publish optimal transition processes could also be valuable [26].

Adult-training programs should continue to implement teaching on adult manifestations of pediatric conditions to accurately reflect the needs of today’s patient population. Mandating transition training at both the student and resident level, either via direct incorporation into a national curriculum or creation of a specialty certification program, is needed [55]. Essential elements of such a curriculum would include patient-family involvement in teaching, addressing critical issues regarding financing, and discussion of medical decision making for adults with varying degrees of dependence on caregivers and illness progression [58, 59]. Community providers with experience transitioning young persons with CP should serve as instructors and faculty mentors across specialties [55]. Certification could also be accessible via the completion of online training modules [58]. As transitional care for this population requires a multidisciplinary healthcare team, students and residents should practice providing and coordinating care in multidisciplinary settings [54, 59]. Mentors should assist students and residents interested in advocacy projects to identify opportunities for greater involvement and funding. In doing so, we can ensure there are ample adult providers with the comfort and competence to treat young adults with CP [25, 54].

Furthermore, transition of care protocols should be usable across various clinics and geographic areas, and not merely successful in one clinic or hospital system [26]. Once best practice guidelines have been established, standardized transition clinics designed around the complex care needs of adolescents and young adults with CP can be established. A comprehensive clinic would ideally address the health, vocational, and daily living needs of young adults with CP, including counseling, therapy, legal, and financial support services [22]. The provision of a “navigator” for parents and adolescents during their transition to adult healthcare services could be immensely helpful in facilitating use of multiple services [22, 24]. This intervention has been effective in transitional care for young adults with other chronic diseases of childhood, resulting in decreased loss to follow-up and significant improvement in mean transition readiness scores, disease knowledge, and confidence in both disease and pain management [1, 60]. Further quantitative and qualitative research, including possible trials and implementation science collaborations, are needed to elucidate the most effective interventions and program structure.

Recommendations for transitional care practices, based on the findings of this review, are summarized in Table 4.

There are several limitations to this review. As only published full-text studies were included, results are at risk for publication bias. Studies not written or translated into English were excluded from this review, potentially resulting in missed findings on the presence and efficacy of transition programs in other countries. Included studies had an overall moderate risk of bias given that the majority were descriptive or qualitative in nature. None of the included studies were randomized controlled trials, which are necessary to provide the highest-quality evidence on successful transition measures and associated outcome data. These elements limit our ability to detect potential differences in race, gender, or institution. Some of the included studies excluded children with varying degrees of cognitive impairment and/or functional status. Additionally, there was extensive variety in the measures and tools used to assess transition readiness across studies. Data from low- or middle-income countries were lacking. While many high-income countries have begun to implement healthcare transition-type programs, a notable worldwide gap still exists due to limited data from resource-limited areas [61, 62]. Despite these limitations, strict PRISMA guidelines were followed to systematically assess and provide a comprehensive analysis of the published literature. Future studies should develop and evaluate contextually appropriate, comprehensive, transitional readiness tools and care models [63‐66].