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Describe the 5-year outcomes of the first successful pediatric bilateral hand transplantation.The child underwent quadrimembral amputation at age two and received bilateral hand allografts at age eight. Rehabilitation included biomechanical, neurorehabilitation, and occupational approaches in acute and outpatient settings. Therapist observed outcomes, patient-reported measures, and parent-reported measures were repeated over a 5-year period.Observation assessments revealed functional dexterity skills and modified independence to full independence with self-care activities. The parent reported the child had moderate difficulty with upper extremity functioning 25-, 41-, and 48-months post-transplantation, and mild difficulty at 60-months; the child reported no difficulties in this domain at 41 months. Five years post-transplantation the child reported enjoying many age-appropriate activities, and high-quality peer relations were endorsed by both parent and child.The child developed hand movements for daily activities and was completing daily activities with improved efficiency. Health-related quality of life outcomes were favorable.

A position statement of the International Pediatric Transplant Association endorsing prioritizing pediatric recipients for deceased donor organ allocation, examining the key ethical arguments that serve as the foundation for that position, and making specific policy recommendations to support prioritizing pediatric recipients for deceased donor organ allocation globally.

The Pediatric Transplant Rating Instrument (P-TRI) is a 17-item scale developed to assess psychosocial risk factors for poor outcomes after solid organ transplantation. Research has identified the limitations of the original instrument and proposed revisions to improve clinical utility. This project examined patterns of risk in children being evaluated for kidney transplant using a revised P-TRI.A multidisciplinary kidney transplant team revised the P-TRI. A social worker and a psychologist collaboratively completed the modified instrument for 37 children after the psychosocial pretransplant evaluation. Electronic medical records were reviewed for transplant status (transplanted, active waitlist, inactive) 1 year later. Exploratory cluster analyses and chi-square tests examined patterns of risk and correlates with cluster membership.Three clusters were identified. The high-risk group (29.7%) had difficulties with medication and appointment adherence, strained relationships with the medical team, and the presence of parent psychiatric history. The medium-risk group (35.1%) had difficulties with parent knowledge, financial strain, and risk factors for medication nonadherence. The low-risk group (35.1%) demonstrated no difficulties with adherence or financial strain. Clusters were prospectively associated with transplant status, such that those in the high-risk group were less likely to be transplanted within 1 year post-evaluation.The revised P-TRI demonstrated good construct validity as risk level appeared to be associated with transplant listing status 1 year post-evaluation. These results suggest that standardized pretransplant psychosocial risk assessment tools may have value in optimizing transplant access if they can be paired with targeted, multidisciplinary interventions to address concerns early in the transplant process.

Professionals working in pediatric transplantation commonly encounter complex ethical dilemmas. Most ethical research in transplantation is related to adult practice. We aimed to gain insight into ethical issues faced by transplant professionals when dealing with pediatric transplant recipients.A two-stage study was designed; the first part was a questionnaire completed by 190 (80%) members of the International Pediatric Transplant Association (IPTA) from over 30 different countries. This was followed by a multidisciplinary focus group that explored the preliminary data of the survey.A total of 38% (56 of 149) respondents of the questionnaire had experienced an ethical issue between 2016 and 2018. Surgeons were more likely to have encountered an ethical issue as compared with physicians (60% vs. 35.7%, p = .035). Clinicians from Europe were more likely to have experienced an ethical issue in living organ donation compared with those from North America (78.9% vs. 52.5%, p = .005), with common ethical concerns being psychosocial evaluation and follow-up care of donors. The focus group highlighted the importance of a multidisciplinary approach to ethical issues.The results of this study can direct future research into pediatric transplantation ethics with the aim of producing educational resources, policies, and ethical guidelines.

Background: The first successful bilateral pediatric hand transplant was performed in 2015. Previous hand transplant decision analysis models have focused on the adult population. This model principally aimed to determine whether adverse outcomes associated with immunosuppression outweigh the benefits of performing bilateral hand transplant surgery in a pediatric candidate. The model also conceptualized the valuation of losing years of life and sought to determine the impact of that valuation on the surgical decision. Methods: A decision model compared undergoing bilateral hand transplant surgery with using prosthetics for an 8-year-old patient. The outcome measure used was quality adjusted life years (QALYs), and sensitivity analysis was performed on the immunosuppressive risks associated with the surgical decision, as well as the perceived valuation of aversion to life years lost. Results: The decision to perform surgery was marginally optimal compared to the prosthetic decision (50.11 QALY vs. 47.95 QALY). A Monte Carlo simulation revealed that this difference may be too marginal to detect an optimal decision (50.14 ± 8.28 QALY vs. 47.95 ± 2.12 QALY). Sensitivity analysis identified decision thresholds related to immunosuppression risks ( P = 29% vs. P = 33% modeled), and a trend of increasing risk as a patient is more averse to losing life years. Conclusions: The marginally optimal treatment strategy currently is bilateral hand transplant, compared to prosthetics for pediatric patients. Key determinants of the future optimal strategy will be whether immunosuppressive regimens become safer, with a reduced risk of losing life years due to immunosuppressive complications, and whether prosthetics become more acceptable and enable higher functioning.

BACKGROUND: Young adult heart transplant (HT) recipients transferring to adult care are at risk for poor health outcomes. We conducted a pilot randomized controlled trial to determine feasibility and test a transition intervention for young adults who underwent HT as children and transferred to adult care. METHODS: Participants were randomized to the transition intervention (4 months long, focused on HT knowledge, self-care, self-advocacy, and social support) or usual care. Self-report questionnaires and medical records data were collected at baseline and 3 and 6 months after the initial adult clinic visit. Longitudinal analyses comparing outcomes over time were performed using generalized estimating equations and linear mixed models. RESULTS: Transfer to adult care was successful and feasible (i.e., excellent participation rates). The average patient standard deviation of mean tacrolimus levels was similar over time in both study arms and < 2.5, indicating adequate adherence. There were no between group and within group differences in percent of tacrolimus bioassays within target range (>50%). Average overall adherence to treatment was similarly good for both groups. Rates of appointment keeping through 6 months after transfer declined over time in both groups. CONCLUSIONS: Feasibility of the study was demonstrated. Our transition intervention did not improve outcomes.

There are limited data describing the prevalence of mental health disorders (MHDOs) in patients with ventricular assist devices (VADs), or associations between MHDOs and resource use or outcomes. We used the Nationwide Emergency Department Sample administrative database to analyze 44,041 ED encounters for VAD-supported adults from 2010 to 2017, to assess the relationship between MHDOs and outcomes in this population. MHDO diagnoses were present for 23% of encounters, and were associated with higher charges and rates of admission, but lower mortality.

Introduction: There are minimal data describing the prevalence of mental health disorders (MHDO) in VAD-supported patients, or the association between MHDO and resource use or outcomes. Methods: This retrospective analysis utilizes all emergency department (ED) encounter-level data from the 2010-2017 Nationwide Emergency Department Sample (NEDS). Patients with VADs were identified using ICD-9/10 codes, as were associated MHDO diagnoses, which were categorized as depression/anxiety, suicide/self harm, bipolar/psychoses, and substance abuse. Outcomes and characteristics were compared for patients with and without a MHDO via descriptive statistics. Hospital admission and mortality occurring in ED or inpatient was investigated with logistic multivariable regression models adjusted for demographic and clinical characteristics. Results: Of the 47,543 encounters with VADs during the study period, 23% (n=11,103) had at least one MHDO. Depression/anxiety (82%) and substance abuse (22%) were the most prevalent MHDOs. Suicide attempt or self harm was the primary diagnosis for 20 (0.2%) encounters. Patients with MHDOs were more likely to be female (31% vs. 26%), younger (median age 59 vs. 61 years), and to have a non-MHDO chronic condition (77% vs 70%, P Discussion: 1 in 5 VAD-supported ED patient encounters also have a MHDO diagnosis. VAD-supported patients with a MHDO had higher resource use but lower mortality. These data underscore the need for greater attention to how MHDO may impact outcomes in VAD-supported patients.

Summary Background Although heterologous vascular composite allotransplantation has become a burgeoning treatment option for adult amputees, there have been no successful cases previously reported in children. Here, we describe the surgical, immunological, and neurorehabilitation details with functional outcomes 18 months after heterologous bilateral hand and forearm transplantation in an 8-year-old child with quadrimembral amputations and a previous kidney transplant. Methods 2 years of extensive preparation by medical and surgical teams preceded the hand–forearm transplantation of this child. The initial immunosuppressive protocol included thymoglobulin, tacrolimus, prednisone, and mycophenolate mofetil. In July, 2015, our vascularised composite allotransplantation team did the first bilateral hand and forearm transplantation in a child, an 8-year-old boy with previous living-related kidney transplantation. The surgery included four teams working simultaneously on the donor and recipient limbs, aided by customised cutting guides that aimed to reduce ischaemia time. Following an extended length of time in hospital, skin biopsies and close monitoring of renal function and drug concentrations occurred weekly for the first 3 months and were slowly tapered to monthly, and then quarterly. Skin biopsies were also done when tissue rejection was suspected. Paediatric-specific rehabilitation techniques were applied to promote patient engagement during rehabilitation. Progress was assessed by monthly sensory and motor function tests during routine clinic visits and with serial functional brain imaging studies, including structural brain MRI, magnetoencephalography and transcranial magnetic stimulation. Findings The surgery lasted 10 h and 40 min. Vascular revision of the ulnar artery was required a few hours postoperatively. There were no further immediate postsurgical complications. Rejection episodes occurred throughout the first year but were reversed. An increase in serum creatinine led to the addition of sirolimus at 3 months after transplantation with concomitant reduction in tacrolimus targets. Sensibility to light touch was present by 6 months after transplantation. Intrinsic hand muscle innervation was present by 7–10 months after transplantation. At 18 months, the child had exceeded his previous adapted abilities. As of 18 months after transplantation surgery he is able to write and feed, toilet, and dress himself more independently and efficiently than he could do before transplantation. He remains on four immunosuppressive medications and functional neuroimaging studies have shown motor and somatosensory cortical reorganisation. Interpretation Hand transplantation in a child can be surgically, medically, and functionally successful under carefully considered circumstances. Long-term data on the functional trajectory, neurological recovery, psychological sequelae, and the potential late effect of immunosuppression are still needed to support broader implementation of paediatric vascular composite allotransplantation. Funding The Children's Hospital of Philadelphia.

Young adult solid organ transplant recipients who transfer from pediatric to adult care experience poor outcomes related to decreased adherence to the medical regimen. Our pilot trial for young adults who had heart transplant (HT) who transfer to adult care tests an intervention focused on increasing HT knowledge, self-management and self-advocacy skills, and enhancing support, as compared to usual care. We report baseline findings between groups regarding (1) patient-level outcomes and (2) components of the intervention. From 3/14 to 9/16, 88 subjects enrolled and randomized to intervention (n = 43) or usual care (n = 45) at six pediatric HT centers. Patient self-report questionnaires and medical records data were collected at baseline, and 3 and 6 months after transfer. For this report, baseline findings (at enrollment and prior to transfer to adult care) were analyzed using Chi-square and t-tests. Level of significance was p

Background Adolescents often fare poorly after heart transplantation. However, whether the effect of age varies according to the etiology of heart failure is unknown. We tested the hypothesis that age-related heart transplantation outcomes are different in patients with myocarditis and congenital heart disease (CHD). Methods A retrospective analysis of the United Network of Organ Sharing database was performed for patients with myocarditis ( n = 709) and CHD ( n = 1,631) undergoing heart transplantation from 1987 to 2011. The effect of age on graft survival was assessed. Age was categorized as children (6–12 years), adolescents (13–18 years), younger adults (19–30 years), and older adults (31–50 years). Results For myocarditis, the median graft survival for adolescents was 6.9 years (95% confidence interval [CI], 5.6–9.6), which was significantly lower than other age groups (children: 14.1 [95% CI, 9.8–10.9] years, p = 0.004; younger adults: 11.8 [95% CI, 8.3–15.2] years, p = 0.172; older adults: 12.0 years [95% CI, 10.0–14.3 years], p = 0.033). For CHD, the median graft survival for adolescents was 7.4 years (95% CI, 6.8–8.6), which was not significantly different from other age groups (children: 9.0 [95% CI, 7.9–11.0] years, p = 0.737; younger adults: 11.2 [95% CI, 8.6–13.3] years, p = 0.744; older adults: 11.6 [95% CI, 9.2–15.3] years, p = 0.608). Multivariable analysis showed adolescent age was independently associated with worse graft survival for patients with myocarditis but not for CHD. Conclusions Adolescents with myocarditis have significantly worse graft survival after heart transplantation, but adolescents with CHD have similar outcomes to other patients with CHD. Further study is needed to improve outcomes in this vulnerable population.

There were 9,566 pediatric heart transplants reported to the ISHLT from 1982-2009.1 Every year, the ISHLT pediatric registry report survival statistics improve and, in addition, the number of children and adults undergoing transplantation for congenital heart disease continues to increase.2 With increasing numbers of pediatric patients surviving into adulthood with excellent quality of life,3 more young adult transplant recipients will receive care at adult centers. Transition programs are critical to support care for these young patients who are traditionally cared for at children’s hospitals. Transition is defined as a “complex set of beliefs, skills, and processes that facilitate the movement from pediatric care to adult centered care.”4 The goal of transition is to provide comprehensive, developmentally appropriate healthcare in a coordinated, uninterrupted, and seamless manner across centers and in partnership with patients.

Preparing patients for transitioning to self-managed care and subsequently transferring to the adult healthcare system has become a critical process for clinicians working with pediatric transplant recipients. This paper reviews several barriers to a successful transition. These include patient barriers, caregiver barriers, and considerations within pediatric and adult centers. To date, few approaches for improving the transition process have been empirically tested. This review details studies that have examined possible models including usage of a transition coordinator and transition clinics. Recommendations are offered to promote an optimal transition including the importance and content of preparation, assessing and addressing transition readiness, insuring the involvement of all stakeholders, and finally, at minimum providing services during the transfer period. Future directions are offered aiming to advance this important area of investigation.

Adjusting to life after transplant can be challenging to pediatric solid organ transplant recipients and their families. In this review, we discuss a number of important factors to consider during the first 2-3 yr after transplant (defined as the "early years"), including transitioning from hospital to home, returning to physical activity, feeding and nutrition, school reentry, potential cognitive effects of transplant, family functioning, and QOL. We highlight steps that providers can take to optimize child and family adjustment during this period.

Assessment of psychosocial functioning is an often-included component of the pretransplant evaluation process. This study reviews several domains of assessment that have been related to post-transplant outcomes across solid organ transplant populations. These include evaluation of patient and family past adherence, knowledge about the transplantation process, and their neurocognitive, psychological, and family functioning. To date, few comprehensive pretransplant evaluation measures have been standardized for use with children; however, several assessment measures used to evaluate the aforementioned domains are reviewed throughout the study. Additionally, this article discusses some developmental, illness-specific, and cultural considerations in conducting the psychosocial evaluation. We also discuss ethical issues specific to the pediatric psychosocial evaluation. Recommendations are advanced to promote a comprehensive evaluation that identifies family strengths and risk factors as they begin the transplant journey.

The purpose of this study was to assess the prevalence and correlates of acute stress disorder (ASD) and posttraumatic stress disorder (PTSD) in mothers and fathers, and postpartum depression (PPD) in mothers, of infants in the Neonatal Intensive Care Unit (NICU). 86 mothers and 41 fathers completed measures of ASD and of parent perception of infant medical severity 3-5 days after the infant's NICU admission (T1), and measures of PTSD and PPD 30 days later (T2). 35% of mothers and 24% of fathers met ASD diagnostic criteria at T1, and 15% of mothers and 8% of fathers met PTSD diagnostic criteria at T2. PTSD symptom severity was correlated with concurrent stressors and family history of anxiety and depression. Rates of ASD/PTSD in parents of hospitalized infants are consistent with rates in other acute illness and injury populations, suggesting relevance of traumatic stress in characterizing parent experience during and after the NICU.

The first successful organ transplant was a kidney transplant performed between identical twins in 1954. Since that time, major medical advances have been made to help improve survival rates for transplant recipients. In 2008, there were 1,964 solid organ transplants performed for children under age 18 (2007 Organ Procurement and Transplantation Network and the Scientific Registry of Transplant Recipients [OPTN/SRTR] Annual Report 1997–2006). Currently, approximately 1,830 pediatric patients are awaiting some type of solid organ transplant (2007 OPTN/SRTR Annual Report 1997–2006). Organ transplantation in children is relatively recent compared to other treatments for children with chronic illnesses. The focus over the first few decades has been on medical advances and improving survival rates for transplant patients. In the recent years, increasing attention has been given to the developmental, neurocognitive, and psychosocial outcomes prior to transplant and in the short-term period post transplant. Most chronic illnesses and acute traumatic medical events have implications for neurocognitive outcomes. End-stage disease of the liver, kidney, heart, and lung are all believed to affect intellectual, academic, and neurocognitive functions. Gross neurodevelopmental deficits have become less common due to early medical intervention (e.g., improved nutrition, surgical intervention, reduced exposure to aluminum (Warady 2002). Organ transplantation is believed to ameliorate the deleterious long-term developmental and neurocognitive effects, but this topic has received little attention in the literature, and the available results with regard to intellectual, academic, and neurodevelopmental results have been mixed. In a combined sample of solid organ transplant patients, 40% had clinically significant cognitive delays (Brosig et al. 2006). Examining the impact of different underlying disease processes and transplantation of each solid organ separately is critical. Thus, we discuss the neurocognitive outcomes of each organ group separately in this chapter. Neurocognitive outcomes can be assessed in a variety of ways depending upon the age of the child. Among infants and toddlers, neurocognitive functioning is measured by an assessment of motor function, social and environmental interaction, and language development. Assessment of older children may involve the evaluation of intelligence, academic achievement, emotional and behavioral functioning, and adaptive skills.

Purpose Mental health issues in the pediatric VAD population are poorly understood. Data from the adult VAD population suggests a high prevalence of psychiatric distress, often resulting in prophylactic or adjunctive treatment with psychiatric medications. We investigated the prevalence of psychiatric diagnosis and psychiatric medication usage in pediatric VAD recipients. Methods and Materials A retrospective study of the Pediatric Health Information System, a large administrative database of 43 US children’s hospitals, was performed for patients 6-20 years of age undergoing VAD placement from 2000-2010. Results 70 of 238 patients (29.4%) with VADs had documented psychiatric diagnoses, most commonly adjustment disorders (n=24). 59 of 238 (24.8%) patients with VADs received psychiatric medication (excluding benzodiazepines) during hospitalization, most commonly antidepressants (n=41). Of the patients who received psychiatric medication, 34 of them (56%) had documented psychiatric diagnoses. Of the 70 patients with documented psychiatric diagnoses, 33 of them (47%) received psychiatric medication. None of the patients who received antidepressant medication had documented diagnoses of depression. Patients who had a stroke were more likely to have been treated with psychiatric medications (p=0.04). Hospital length of stay (LOS) and total charges were greater among patients treated with psychiatric medications (p Conclusions One quarter of pediatric VAD patients received psychiatric diagnoses and one quarter were treated with psychiatric medications. Nearly half of patients receiving psychiatric medication did not have documented psychiatric diagnoses. Psychiatric diagnoses and medications were associated with longer hospital LOS and greater hospital charges. Further research is needed to understand patterns of and indications for psychiatric medication usage, as well as the burden and treatment of psychiatric disorders in this population.

Purpose Adolescents often fare poorly after heart transplantation (HT) compared to other age groups. However, it is unknown if the impact of age varies across diseases leading to HT. We tested the hypothesis that age-related HT outcomes are different in patients with congenital heart disease (CHD) vs myocarditis. Methods and Materials A retrospective analysis of the United Network of Organ Sharing database was performed for patients with myocarditis (n=709) and CHD (n=1,631) undergoing HT from 1987-2011, to assess the effect of age on graft survival. Age was categorized as children (6-12 years), adolescents (13-18 years), younger adults (19-30 years), and older adults (31-50 years). Results Adolescents comprised 28% of CHD patients and 14% of myocarditis patients (p Conclusions Adolescents with myocarditis have significantly worse graft survival after HT, but adolescents with CHD have similar outcomes to other patients with CHD. Further study is needed to improve outcomes in this vulnerable population.