Allied health and nursing practices in pediatric solid organ transplantation: An international survey.

INTRODUCTION: With improved survival in pediatric solid organ transplantation (SOT) care has focused on optimizing functional, developmental, and psychosocial outcomes, roles often supported by Allied Health and Nursing professionals (AHNP). However, there is a scarcity of research examining frameworks of clinical practice. METHODS: The International Pediatric Transplant Association AHNP Committee developed and disseminated an online survey to transplant centers as a quality improvement project to explore AHNP practice issues. Participant responses were characterized using descriptive statistics, and free-text comments were thematically analyzed. Responses were compared across professional groups; Group 1: Advanced Practice Providers, Group 2: Nursing, Group 3: Allied Health. RESULTS: The survey was completed by 119 AHNP from across the globe, with responses predominantly (78%) from North America. Half of respondents had been working in pediatric transplant for 11+ years. Two-thirds of respondents were formally funded to provide transplant care; however, of these not funded, over half (57%) were allied health, compared to just 6% of advance practice providers. Advanced practice/nursing groups typically provided care to one organ program, with allied health providing care for multiple organ programs. Resource constraints were barriers to practice across all groups and countries. CONCLUSION: In this preliminary survey exploring AHNP roles, professionals provided a range of specialized clinical care. Challenges to practice were funding and breadth of care, highlighting the need for additional resources, alongside the development of clinical practice guidelines for defining, and supporting the role of AHNP within pediatric SOT. Professional organizations, such as IPTA, can offer professional advocacy.

Current approach to health care transition and integration into adult care for pediatric liver transplant recipients: A call for partnership.

Despite the increased risk of non-adherence, allograft rejection, and mortality following transfer from pediatric to adult care in liver transplantation (LT), there is no standardized approach to health care transition (HCT). Two electronic national surveys were developed and distributed to members of the Society for Pediatric Liver Transplantation and all adult LT programs in the United States to examine current HCT practices. Responses were received from 40 pediatric and 79 adult centers. Pediatric centers were more likely to focus on HCT noting the presence of a transition/transfer policy (60.2% vs. 39.2%), transition clinic (51.6% vs. 16.5%), and the routine use of transition readiness assessment tools (54.8% vs. 10.2%). Perceived barriers to HCT were similar among pediatric and adult respondents and included patient willingness to transfer and participate in care, failure to show for appointments, and lack of sufficient time and staffing. These results highlight the need for an increased awareness of HCT at both pediatric and adult LT centers. The path to improvement requires a partnership between pediatric and adult providers. Recognizing the importance of a comprehensive HCT program initiated in pediatrics and continued throughout young adulthood with ongoing support by the adult team is essential.

An international survey of allied health & nursing professionals during the COVID-19 pandemic: Perspectives on facilitators of & barriers to care.

BACKGROUND: Allied health and nursing professionals (AHNP) are integral members of transplant teams. During the COVID-19 pandemic, they were required to adapt to changes in their clinical practices. The goal of the present study was to describe AHNP perceptions concerning the impact of the pandemic on their roles, practice, and resource allocation. METHODS: An online survey was distributed globally via email by the International Pediatric Transplant Association to AHNP at transplant centers from September to December 2020. Responses to open-ended questions were collected using an electronic database. Using a thematic analysis approach, coding was conducted by three independent coders who identified patterns in responses, and discrepancies were resolved through discussion. RESULTS: The majority of respondents (n = 119) were from North America (78%), with many other countries represented (e.g., the United Kingdom, Europe, Australia, New Zealand, South Africa, and Central and South America). Four main categories of impacts were identified: (1) workflow changes, (2) the quality of the work environment, (3) patient care, and (4) resources. CONCLUSIONS: Participants indicated that the pandemic heightened existing barriers and resource challenges frequently experienced by AHNP; however, the value of team connections and opportunities afforded by technology were also highlighted. Virtual care was seen as increasing healthcare access but concerns about the quality and consistency of care were also expressed. A notable gap in participant responses was identified; the vast majority did not identify any personal challenges connected with the pandemic (e.g., caring for children while working remotely, personal stress) which likely further impacted their experiences.

Health Care Transition for Adolescents and Young Adults With Pediatric-Onset Liver Disease and Transplantation: A Position Paper by the North American Society of Pediatric Gastroenterology, Hepatology, and Nutrition.

Advances in medical therapies and liver transplantation have resulted in a greater number of pediatric patients reaching young adulthood. However, there is an increased risk for medical complications and morbidity surrounding transfer from pediatric to adult hepatology and transplant services. Health care transition (HCT) is the process of moving from a child/family-centered model of care to an adult or patient-centered model of health care. Successful HCT requires a partnership between pediatric and adult providers across all disciplines resulting in a transition process that does not end at the time of transfer but continues throughout early adulthood. Joint consensus guidelines in collaboration with the American Society of Transplantation are presented to facilitate the adoption of a structured, multidisciplinary approach to transition planning utilizing The Six Core Elements of Health Care Transition TM for use by both pediatric and adult specialists. This paper provides guidance and seeks support for the implementation of an HCT program which spans across both pediatric and adult hepatology and transplant centers.

Five Hundred Patients With Gut Malrotation: Thirty Years of Experience With the Introduction of a New Surgical Procedure.

OBJECTIVES: Define clinical spectrum and long-term outcomes of gut malrotation. With new insights, an innovative procedure was introduced and predictive models were established. METHODS: Over 30-years, 500 patients were managed at 2 institutions. Of these, 274 (55%) were children at time of diagnosis. At referral, 204 (41%) patients suffered midgut-loss and the remaining 296 (59%) had intact gut with a wide range of digestive symptoms. With midgut-loss, 189 (93%) patients underwent surgery with gut transplantation in 174 (92%) including 16 of 31 (16%) who had autologous gut reconstruction. Ladd's procedure was documented in 192 (38%) patients with recurrent or de novo volvulus in 41 (21%). For 80 patients with disabling gastrointestinal symptoms, gut malrotation correction (GMC) surgery "Kareem's procedure" was offered with completion of the 270° embryonic counterclockwise-rotation, reversal of vascular-inversion, and fixation of mesenteric-attachments. Concomitant colonic dysmotility was observed in 25 (31%) patients. RESULTS: The cumulative risk of midgut-loss increased with volvulus, prematurity, gastroschisis, and intestinal atresia whereas reduced with Ladd's and increasing age. Transplant cumulative survival was 63% at 10-years and 54% at 20-years with best outcome among infants and liver-containing allografts. Autologous gut reconstruction achieved 78% and GMC had 100% 10-year survival. Ladd's was associated with 21% recurrent/de novo volvulus and worsening (P > 0.05) of the preoperative National Institute of Health patient-reported outcomes measurement information system gastrointestinal symptom scales. GMC significantly (P ≤ 0.001) improved all of the symptomatology domains with no technical complications or development of volvulus. GMC improved quality of life with restored nutritional autonomy (P < 0.0001) and daily activities (P < 0.0001). CONCLUSIONS: Gut malrotation is a clinicopathologic syndrome affecting all ages. The introduced herein definitive correction procedure is safe, effective, and easy to perform. Accordingly, the current standard of care practice should be redefined in this orphan population.

A Learning Health System for Pediatric Liver Transplant: The Starzl Network for Excellence in Pediatric Transplantation.

OBJECTIVE: Learning health systems (LHS) integrate research, improvement, management, and patient care, such that every child receives "the right care at the right time...every time," that is, evidence-based, personalized medicine. Here, we report our efforts to establish a sustainable, productive, multicenter LHS focused on pediatric liver transplantation. METHODS: The Starzl Network for Excellence in Pediatric Transplantation (SNEPT) is the first multicenter effort by pediatric liver transplant families and providers to develop shared priorities and a shared agenda for innovation in clinical care. This report outlines SNEPT's structure, accomplishments, and challenges as an LHS. RESULTS: We prioritized 4 initial projects: immunosuppression, perioperative anticoagulation, quality of life, and transition of care. We shared center protocols/management to identify areas of practice variability between centers. We prioritized actionable items that address barriers to providing "the right care at the right time" to every pediatric liver transplant recipient: facilitating transparency of practice variation and the connection of practices to patient outcomes, harnessing existing datasets to reduce the burden of tracking outcomes, incorporating patient-reported outcomes into outcome metrics, and accelerating the implementation of knowledge into clinical practice. This has allowed us to strengthen collaborative relationships, design quality improvement projects, and collect pilot data for each of our priority projects. CONCLUSIONS: The field of pediatric liver transplantation can be advanced through application of LHS principles. Going forward, SNEPT will continue to unite patient advocacy, big data, technology, and transplant thought leaders to deliver the best care, while developing new, scalable solutions to pediatric transplantation's most challenging problems.

A learning health network for pediatric liver transplantation: Inaugural meeting report from the Starzl Network for Excellence in Pediatric Transplantation.

Learning Health Networks (LHN) improve the well-being of populations by aligning clinical care specialists, technology experts, patients and patient advocates, and other thought leaders for continuous improvement and seamless care delivery. A novel LHN focused on pediatric transplantation, the Starzl Network for Excellence in Pediatric Transplantation (SNEPT), convened its inaugural meeting in September 2018. Clinical care team representatives, patients, and patient families/advocates partnered to take part in educational sessions, pain point exercises, and project identification workshops. Participants discussed the global impact of transplant from both a population and individual perspective, identifying challenges and opportunities where the Starzl Network could work to improve outcomes at scale across a variety of transplant-related conditions.

Digital Wings: Innovations in Transition Readiness for Adolescent and Young Adult Transplant Recipients [corrected].

The Johns Hopkins University School of Medicine organized 2 multistakeholder symposia on February 2, 2018 and January 11, 2019 to address the problem of high graft failure in adolescent and young adult (AYA) solid organ transplant (SOT) recipients. Participants included international experts in transplantation, behavioral psychology, patient/parent advocacy, and technology. The objectives of the symposia were as follows: (1) to identify and discuss the barriers to and facilitators of effective transfer of care for AYA SOT recipients; (2) to actively explore strategies and digital solutions to promote their successful transfer of care; and (3) to develop meaningful partnerships for the successful development, evaluation, implementation, and dissemination of these digital solutions. Additionally, data were collected from 152 AYA SOT recipients demonstrating a substantial gap in how this population uses technologies for health-related activities, alongside an increased interest in an app to help them manage their transplant.

Adaptive functioning and its correlates after intestine and liver transplantation.

In this cross-sectional study, we compared levels of adaptive functioning and examined potential correlates of adaptive functioning in 18 pediatric intestine (ITX) and 22 liver (LTX) recipients transplanted between June 2003 and March 2009. Family caregivers completed the ABAS-II scale and provided socio-demographic information regarding recipients' age at transplantation, gender, ethnicity, time since transplantation, and caregivers' role, ethnicity, education, and family income. Overall adaptive functioning and all three adaptive functioning subdomain scores were significantly lower in ITX patients compared with LTX patients (p ≤ 0.04) and compared with the general population normative mean (p ≤ 0.003). Significant correlates of adaptive functioning after abdominal transplant included type of transplant procedure (r = -0.4, p = 0.02), gender (r = 0.4, p = 0.01), and educational level of caregiver (r = 0.5, p = 0.003) and together explained 45% of the variance in overall adaptive functioning. Findings provide new information regarding everyday functioning outcomes of ITX patients, add to existing data regarding non-medical outcomes for LTX patients, and highlight the need for ongoing monitoring and intervention following transplantation to enhance outcomes.

Long-term survival, nutritional autonomy, and quality of life after intestinal and multivisceral transplantation.

OBJECTIVE: To assess long-term survival, graft function, and health-related quality of life (QOL) after visceral transplantation. BACKGROUND: Despite continual improvement in early survival, the long-term therapeutic efficacy of visceral transplantation has yet to be defined. METHODS: A prospective cross-sectional study was performed on 227 visceral allograft recipients who survived beyond the 5-year milestone. Clinical data were used to assess outcome including graft function and long-term survival predictors. The socioeconomic milestones and QOL measures were assessed by clinical evaluation, professional consultation, and validated QOL inventory. RESULTS: Of 376 recipients, 227 survived beyond 5 years, with conditional survival of 75% at 10 years and 61% at 15 years. With a mean follow-up of 10 ± 4 years, 177 (92 adults, 85 children) are alive, with 118 (67%) recipients 18 years or older. Nonfunctional social support and noninclusion of the liver in the visceral allograft are the most significant survival risk factors. Nutritional autonomy was achievable in 160 (90%) survivors, with current serum albumin level of 3.7 ± 0.5 gm/dL and body mass index of 25 ± 6 kg/m(2). Despite coexistence or development of neuropsychiatric disorders, most survivors were reintegrated to society with self-sustained socioeconomic status. In parallel, most of the psychological, emotional, and social QOL measures significantly (P < 0.05) improved after transplantation. Current morbidities with potential impact on global health included dysmotility (59%), hypertension (37%), osteoporosis (22%), and diabetes (11%), with significantly (P < 0.05) higher incidence among adult recipients. CONCLUSIONS: With new tactics to further improve long-term survival including social support measures, visceral transplantation has achieved excellent nutritional autonomy and good QOL.

Most commonly asked questions from parents of pediatric transplant recipients.

Pediatric solid-organ transplant (SOT) recipients and their parents are often challenged to cope with new transplant regimens as well as common situations in the context of organ transplantation. Health care professionals will receive questions from parents and children regarding clinical transplant care as well as general pediatric concerns that seem unfamiliar to families now that their child has a transplant. The literature is limited in some areas of pediatric care after SOT, and there is little guidance for the health care practitioner. To help address gaps in the literature and provide guidance for health care professionals, this article reviews some of the most commonly asked questions regarding general care after SOT, parenting the child with a chronic illness, and growth and development. The answers provided stem from the literature in part but also the combined clinical experiences of transplant centers that over time have moved toward decreased limitations and full social integration.

The absence of chronic rejection in pediatric primary liver transplant patients who are maintained on tacrolimus-based immunosuppression: a long-term analysis.

BACKGROUND: Although the outcome of liver transplantation has improved significantly during the past two decades, graft loss caused by chronic rejection after liver transplantation still occurs in 2% to 20% of recipients. The overall incidence of chronic rejection is also reported to be low in adult recipients, and risk factors have been identified. Chronic rejection is associated with the inability to maintain baseline immunosuppression. Additionally, the diagnoses of primary biliary cirrhosis, primary sclerosing cholangitis, autoimmune hepatitis, hepatitis B virus, and hepatitis C virus, common indications for liver transplantation in adults, are associated with a higher incidence of chronic rejection. Fortunately, these diagnoses are rarely seen in children. Little is known about chronic rejection in long-term pediatric liver transplant survivors. The purpose of this longitudinal study was to examine the incidence of biopsy-proven chronic rejection in long-term survivors of primary pediatric liver transplantation under tacrolimus-based immunosuppression. METHODS: From October 1989 to December 1992, 166 children (boys=95, girls=71; mean age=5.0+/-2.9 years) received a primary liver transplant. These patients were followed until March 2000 with a mean follow-up of 9+/-0.8 (range, 7.4-10.4) years. All liver biopsy specimens and explanted grafts were evaluated for evidence of chronic rejection using the International Banff Criteria. RESULTS: The mortality rate during the follow-up period was 15% (n=25). Retransplantation was required in 11% (n=18) of recipients. Actuarial patient and graft survival rates at 10 years were 84.9% and 80.1%, respectively. There were 535 liver biopsy samples available for evaluation, including the 18 explanted allografts. Biopsy specimens of three other functioning allografts showed evidence of chronic rejection. Immunosuppression had been discontinued or drastically reduced in these recipients because of life-threatening infections, noncompliance, or both. On restoring baseline immunosuppression, all three children had normalized liver function and the allografts were maintained; the liver transplant patients who are alive currently have normal liver functions. CONCLUSION: The findings of this study suggest that chronic rejection does not occur in pediatric liver transplant recipients receiving tacrolimus-based immunosuppression, provided baseline immunosuppression is maintained.

Pediatric intestinal transplantation: historical notes, principles and controversies.

The development in technique and immunosuppressive management of the last 12 yr have made intestinal transplantation an effective treatment for children with intestinal failure. The information provided in this review support such a conclusion, but was more clearly validated by the March 2001 Medicare Report which provided a national coverage decision of the Social Security Act for intestinal transplantation. As of May 2001, there were 55 centers world-wide which have performed 696 intestinal transplants in 656 patients. (Intestinal Transplant Registry, http://www.lhsc.on.ca/itr) the majority of recipients have been children, and there has been a greater need for liver replacement in conjunction with the allograft intestine because of a higher incidence of TPN-induced cholestatic liver disease in children. Though overall long-term survival is approximately 50%, similar advances in surgical, clinical and immunosuppressive management since 1995 have improved patient survival to more than 70% in most experienced programs. Over 80% of survivors are enjoying nutrition-supporting intestinal function. The major causes of graft loss and patient demise continues to be rejection and infection. Tacrolimus remains the mainstay of immunosuppressive therapy. Further experience other induction protocols utilizing rapamycin and daclizumab, as well graft pretreatment protocols may further enhance results in the future.

Pediatric liver transplantation. A single center experience spanning 20 years.

BACKGROUND: Survival after liver transplantation has improved significantly over the last decade with pediatric recipients faring better than adults. The 20-year experience of pediatric liver transplantation at Children's Hospital of Pittsburgh is reported in terms of patient survival; graft survival in relation to age, gender, and immunosuppressive protocols; causes of death; and indications for retransplantation. METHOD: From March 1981 to April 1998, 808 children received liver transplants at Children's Hospital of Pittsburgh. All patients were followed until March 2001, with a mean follow-up of 12.2+/-3.9 years (median=12.6; range=2.9-20). There were 405 female (50.2%) and 403 male (49.8%) pediatric recipients. Mean age at transplant was 5.3+/-4.9 years (mean=3.3; range 0.04-17.95), with 285 children (25.3%) being less than 2 years of age at transplant. Cyclosporine (CsA)-based immunosuppression was used before November 1989 in 482 children (50.7%), and the subsequent 326 recipients (40.3%) were treated with tacrolimus-based immunosuppression. Actuarial survival was calculated using the Kaplan-Meier statistical method. Differences in survival were calculated by log-rank analysis. RESULTS: Overall patient survival at 1, 5, 10, 15, and 20 years was 77.1%, 72.6%, 69.4%, 65.8%, and 64.4%, respectively. There was no difference in survival for male or female patients at any time point. At up to 10 years posttransplant, the survival for children greater than 2 years of age (79.5%, 75.7%, and 71.6% at 1, 5, and 10 years, respectively) was slightly higher than those at less than 2 years of age (72.6%, 66.9%, and 65.3% at 1, 5, and 10 years, respectively). However, at 15 and 20 years posttransplant, survival rates were similar (>2 years=67.3% and 65.8%; <2 years=64.1% and 64.1%). A significant difference in survival was seen in CsA-based immunosuppression (71.2%, 68.1%, 65.4%, and 61%) versus tacrolimus-based immunosuppression (85.8%, 84.7%, 83.3%, and 82.9%) at 1, 3, 5, and 10 years, respectively (P=0.0001). The maximum difference in survival was noted in the first 3 months between CsA and tacrolimus; thus, indicating there may have been other factors (nonimmunological factors) involved in terms of donor and recipient selection and technical issues. The mean annual death rate beyond 2 years posttransplant was 0.47%, with the mean annual death rate for patients who received tacrolimus-based immunosuppression being significantly lower than those who received CsA-based immunosuppression (0.14% vs. 0.8%; P=0.001). The most common etiologies of graft loss were hepatic artery thrombosis (33.4%), acute or chronic rejection (26.6%), and primary nonfunction (16.7%). Of note, retransplantation for graft loss because of acute or chronic rejection occurred only in those patients who received CsA-based immuno-suppression. CONCLUSION: The overall 20-year actuarial survival for pediatric liver transplantation is 64%. Survival has increased by 20% in the last 12 years with tacrolimus-based immunosuppression. Although this improvement may be the result of several factors, retransplantation as a result of acute or chronic rejection has been completely eliminated in patients treated with tacrolimus.

Long-term nutritional outcome after pediatric intestinal transplantation.

BACKGROUND/PURPOSE: The aim of this study was to describe the long-term nutritional status of a large population of children after intestinal transplantation and to identify factors associated with nutritional outcomes. METHODS: Longitudinal anthropometric data are maintained in a database registry for all patients referred to our Intestinal Care Center (ICC). Z-scores for weight and height were calculated biannually over a maximum of 2 years, and associations between baseline and follow-up laboratory measures and growth were evaluated for patients greater than 6 months post intestinal transplant. RESULTS: Since the inception of the ICC in December 1996, 24 pediatric patients (18 boys, 18 white) received an isolated small bowel or small bowel/liver transplant (median age, 3.2 years). The majority of cases (75%) had been diagnosed with surgical short bowel syndrome and were dependent on total parenteral nutrition (TPN) at the time of transplant. Of the 23 patients who survived the initial postoperative period, 87% were weaned from TPN to an amino-acid or peptide-based enteral formula or solid food within 3 months. A positive trend in z-scores for weight and height/length was observed in only 30% and 26% of patients, respectively, during the follow-up period. Although mean albumin levels increased significantly from 2.8 to 3.1 mg/dl by 6 months posttransplant (P <.01) no difference in alkaline phosphatase was found over time. Steroid doses were weaned within 3 to 4 months after transplantation but not discontinued. The cumulative survival rate was 91% at 1 year and 86% at 2 years posttransplant, whereas those weaned from TPN achieved 100% and 94% survival, respectively. CONCLUSIONS: Attainment of positive linear growth remains a challenge in the pediatric transplant population despite successful liberation from TPN, protein anabolism, and high survival rates. Further investigation into alternative methods of nutritional evaluation and manipulation as well as the use of growth factors to enhance the growth process need to be investigated.