Pediatric chronic diseases: chronic diseases that occur in children under 15 years.

Transition support: assistance for transition from the pediatric health care system to the adult health care system.

This was a cross-sectional survey study of physicians and nurses providing transition support at pediatric institutions in Japan.

The study was conducted between August to November 2020. We sent pen-and-paper questionnaires to 494 medical institutions in Japan that specialize in pediatrics (pediatric specialty hospitals, pediatric cancer center hospitals, hospitals with specific functions that provide pediatric care, university hospitals, general hospitals, hospitals that provide home medical care for children, comprehensive perinatal care centers, regional perinatal care centers, etc.).

The eligibility criteria were physicians or nurses working at the above facilities and providing transition assistance at the time of the survey. The facility director selected respondents who met the eligibility criteria and gave them a survey form. Participants were assumed to have completed and directly returned the unmarked questionnaire themselves.

These items were generated based on a literature review, expert discussion, and interviews with three CNS in pediatrics (27). A Pilot Study was completed by sending the questionnaire to two pediatricians of HCT experts for content validity testing. Face validity was also tested on three similar participants. Alterations were made regarding feedback from the Pilot Study, such as changing the questions that were considered ambiguous. Then we formed the final version of the questionnaire for distribution to participants.

This study was in the initial discovery phase regarding actual transition support. The following data were included:

The questionnaire was anonymous to avoid bias in the participants' responses. The participants directly returned the questionnaires themselves.

An average score of 1 (not applicable at all), 2 (not very applicable), 3 (fairly applicable), and 4 (very applicable) was extracted to measure the factors preventing transition to adult care.

Descriptive statistics including frequencies, means, and standard deviations were used in the analysis.

When selecting potential research collaborators, return envelopes were distributed only to research participants to avoid the exercise of coercive power, the disclosure to outside parties of the presence or absence of replies, or the contents of the questionnaire. The questionnaires were unsigned to prevent the identification of the participants, and their responses to the questionnaire indicated that they had agreed to cooperate in the research. This study was conducted after obtaining approval from the ethical review committee of the researcher's institution (No. 19019).

The respondents included 199 (88.4%) physicians and 26 (11.6%) nurses, with 22 (9.8%) children's hospital respondents among them. A total of 153 (68.0%) had more than 10 years of clinical experience in transition support, 179 (79.5%) were in administrative positions, and 76 (33.8%) were Specialized Physicians or Certified Nurses/Clinical Nurse Specialists. The following were multiple responses: the places of care delivery for adolescents with chronic diseases included outpatient pediatrics clinics 204 (90.7%); neuromuscular diseases were the most common with 116 cases (51.6%), followed by syndromes involving chromosomal or genetic changes with 104 cases (46.2%) (Table 1).

Twenty (10.0%) had a specialty outpatient clinic and 61 (30.5%) had a full-time person in charge. The age at which the HCT program started was stated by 36 respondents (16.0%). Academic/career change and age were the most common reasons for starting support services, with 155 respondents (68.9%), followed by diseases outside the scope of pediatricians, with 135 (60.0%). Regarding collaboration with other agencies and departments, 80 (40.0%) respondents indicated that they collaborated with the local family physician, while 97 (48.5%) indicated that they collaborated with adult hospitals and departments. A total of 159 (79.5%) reported no use of educational and support tools for transition assistance, indicating that the guides were not widely used (Table 2).

Two hundred respondents indicated that they transferred adolescent patients to adult departments, with “transferring the patient” being the most common response. Of the transition planning, 155 (77.5%) of the respondents prepared medical summaries. Medical summaries included: disease name with 81 cases (40.5%), examination results with 78 cases (39.0%), treatment summary with 77 cases (38.5%), and prescribed medicine/care with 74 cases (37.0%).

More than 70% responded “No” to all five other content areas: establishing the transition policy with 180 (90%), tracking and monitoring transition progress with 179 (89.5%), assessing the patient's readiness for transition with 143 (71.5%), developing the transition plan with 169 (84.5%), following up with the patient and family with 140 (70%), and patient feedback with 177 (89.4%).

For the combination of the Six Core Elements, 33 (16.5%) practiced only “transferring the patient,” 70 (35.0%) practiced “transferring the patient” and “making a medical summary,” and 30 (15.0%) practiced four or more elements (Table 3).

The patient factors with the most scores were “very applicable” and “fairly applicable” were “Emotional dependence on pediatrics” with 80 cases (37.7%)/101 cases (47.6%) and “Patient's intellectual disability” with 98 cases (46.2%)/76 cases (35.8%), followed by “rare disease” with 75 cases (35.4%)/96 cases (45.3%) (Figure 1-1). Among family factors, “Emotional dependence on Pediatrics” was the highest at 82 cases (38.0%)/108 cases (50.0%), followed by “Over-involvement of patients” at 50 cases (23.1%)/102 cases (47.2%) and “Lack of information about adult departments” at 40 cases (18.5%)/113 cases (25.5%) (Figure 1-2).

The medical/infrastructure factor with the highest score was “Lack of adult medicine departments” with 86 cases (41.7%)/87 cases (42.2%). The followes were “Lack of understanding of patients and diseases by adult physicians” with 67 cases (32.5%)/100 cases (48.5%), “Lack of personnel to coordinate” with 53 cases (25.7%)/101 cases (49.0%), “Lack of collaboration between pediatricians and adult physicians” 47 cases (22.8%)/107 cases (51.9%), and “Lack of a collaborative system” with 51 cases (24.8%)/95 cases (46.1%) (Figure 1-3).

The most common item was related to academic institutions, with 166 (73.8%) selecting “sharing knowledge and support methods with adult medicine departments.” This was followed by “establishment of transitional care centers by policies and local governments” with 121 respondents (54.3%) and “securing an adult department to treat adolescent patients” with 114 respondents (51.1%), representing more than half of the sample (Table 4).

The survey's respondents were pediatricians and nurses in management positions at major pediatric institutions in Japan, and their views may reflect the principles and conditions of practice applicable to transition of care in adolescents and young adult health care in Japan.

In terms of specialties, “neuromuscular diseases” and “syndromes involving chromosomal or genetic changes” accounted for about half of the cases. Although a systematic approach to transitional support for neurological diseases is currently being developed in the U.S. (33), the current situation in Japan is not yet fully understood and programs must be developed to enhance support in the future.

Compared with previous surveys on HCT (34–37), the response rate for this survey (45%) was standard for national surveys that were not limited to board-certified physicians or medical departments. About half of the facilities that did not respond might understand HCT but did not provide support or did not have a sufficient understanding of the HCT available.

The results of this study showed that although more than 80% of adolescent patients were being transferred to adult departments, few departments and people were dedicated to transition support, and educational and informational tools were not being used. Regarding the HCT contents based on the Six Core Elements, the most common is transferring the patient, followed by the making of a medical summary, with 30–40% of the medical summaries containing information on the disease, and <5% related to patient's understanding of their disease or self-management. Regarding the HCT process based on the Six Core Elements, transfers accounted for about half of the cases. The purpose of the HCT program is not limited to transferring, but to provide seamless, high-quality, and developmentally appropriate medical services during the developmental process from adolescence to adulthood to maximize a person's role functioning and potential (29, 30).

Therefore, developing a transition plan with a medical summary in a transition program should not end with simply sending medical information to the adult department, but should include a transition summary (31). In the Transition to Adult Care program for sickle cell disease, the medical summary includes not only medical information, but also social, academic, and emotional content sent by the nurse case manager (38). The medical summary is also used as a tool to engage the patient or family in taking ownership of medical care (39). One method is an initiative that allows patients, pediatricians, and adult health care providers to share an electronic medical summary website (40). These may help bridge the gap between pediatric and adult care and are important in achieving a seamless transition. Development and research of tools that can be shared longitudinally and with patient families is needed in Japan.

About 15% of the facilities had HCT programs that combined four or more elements. The Six Core Elements are not a model of care, but a structured process. They can be customized for each hospital's use and can be applied to different types of transitional care models and settings (31). The structured HCT processes have shown positive results in reducing pre-transition patient anxiety and enhancing patients' experiences and satisfaction with their care, and interventions that had positive outcomes were described as having a combination of HCT activities (17, 41–43). These findings indicate a need for more widespread implementation of structured HCT programs in Japan.

In this study, the top factors that prevented patients from transitioning to adult care were intellectual disability and rare diseases among patient factors and emotional dependence on pediatrics among patient and family factors. Similar findings have been reported in Japan and other countries (25, 38, 44). Patient and family factors in transition barriers are said to include anxiety about transition, inadequate planning, and systemic problems (31), indicating that there are compounding factors. These considerations indicate that a combination of structured processes is needed to provide support.

As for rare diseases and intellectual disabilities, in Japan, the percentage of patients over the age of 20 with congenital metabolic disorders exceeds 35% (45), and children with rare diseases are reaching adulthood. The importance of supporting children with chronic illnesses who require these special considerations and the need for research is described (38), and the needs of patients and families (46, 47), barriers to transition (48), clinical reports on support (49–51), informational sites (52, 53), pediatric and adult department practices, and consensus on pediatric and adult medical care and support have been reported (54). Thus, it is necessary to study the current situation and support patients with special medical needs transitioning to adult care in Japan in the future.

Ochiai, in a survey of patients 15 years of age and older visiting a pediatric cardiology outpatient clinic of a children's hospital, described a lack of information about transfers and the need for continued attendance at a pediatric hospital (32). Based on such surveys, we developed the questionnaire regarding patient/family barriers, but they may not cover all of them.

The results of this survey showed that less than half of the respondents were collaborating with adult health care, and challenges to collaborating with adult health care were identified as a factor hindering transition. Also, the top expectations for transition support were related to collaborating with adult health care. The challenges in collaborating with adult and pediatric departments in Japan included the following: lack of communication/systems, lack of understanding and knowledge of pediatric care and patient characteristics by adult health care departments, lack of manpower/institution to coordinate, and difficulties in securing an adult department to treat adolescent patients. Research reports on barriers to transition in the health care system include communication and consultation gaps, knowledge and training limitations, lack of personnel and resources, and financial constraints (23, 31, 55, 56), similar to those found in this study.

One way to resolve these issues, at the policy and municipal levels, is to “establish transitional medical support centers.” This is a facility that provides comprehensive support for transition, including not only medical care but also welfare. In 2017, a model project was launched by the Ministry of Health, Labor and Welfare requiring each prefecture to secure at least one transitional medical support center (57), but as of 2021, there were only seven such centers nationwide. Their main roles include the following: (1) identification and publication of information on medical departments and medical institutions that can treat patients with chronic pediatric diseases in adulthood; (2) liaison, coordination, and communication support between pediatric medical institutions and adult medical institutions; and (3) promotion of support for patients' independence and autonomy (57). It is expected that the establishment of this support organization will be expanded in the future.

In Japan, various physicians' professional organizations developed guidelines for congenital heart disease (58), renal disease (59), type 1 diabetes (60), rheumatic diseases (61), and various other diseases. In the field of adult congenital heart disease in Japan, close affiliation and interaction with the International Society for Adult Congenital Heart Disease and European meeting in Adult Congenital Heart Disease exists, and a system of medical care and certified physicians has been introduced (62). In other countries, advanced practice nurses in charge can provide care that meets patients' needs (63), and they also improve patient care and family satisfaction (64). Thus, it is necessary to train specialists without being limited to a specific department to ensure that patients receive seamless transition support.

In Japan, few facilities have transition coordinators (36) and pediatricians are responsible for most of them. Although there are some projects and organizations that provide transition coordinator training programs (65) and ongoing training for pediatric nurses (66), there are no systematic educational programs. In other countries, there are already educational systems for pediatric and adult health care providers (67, 68). Therefore, in Japan, it is desirable to harmonize and improve the quality-of-care delivery through education and knowledge sharing in the future.

The results of this survey were mainly derived from physicians nationwide, and we believe that the status of transitional support in pediatric care is clear; however, because facilities that provide transitional support were more likely to respond, those that did not respond to the survey may not be providing adequate support. Therefore, the full scope of support, including that of adult departments and patients/families, might not have been captured. It would therefore be necessary to continue the survey by expanding its scope and considering specific transitional care and support.

The results of this survey were mainly derived from physicians nationwide, and we believe that the status of transitional support in pediatric care is clear. We found that about half of the facilities do not provide transition support due to barriers or insufficient understanding of specific support. As a future challenge, we believe it is necessary to make new contacts with facilities that did not respond to the survey and learn about their difficulties in promoting a systematic HCT program.