New insights into family functioning and quality of life after pediatric liver transplantation

Denny B, Beyerle K, Kienhuis M, Cora A, Gavidia‐Payne S, Hardikar W. New insights into family functioning and quality of life after pediatric liver transplantation. Abstract: Thorough research of the medical aspects of pediatric liver transplantation has given way to recent interest in the impact of the transplantation process on the QOL of recipients and their families. In this cross‐sectional study, we compared the family functioning and QOL of children (n = 30) aged between three and 16 yr (M = 10.10, s.d. = 3.62) who had received a liver transplant in the previous 1–12 yr (M = 5.31, s.d. = 3.44) with non‐transplant children (n = 33), as reported via parent proxy. Results showed that parents of pediatric liver transplant recipients made significantly more adjustments to family routines to accommodate their children, particularly in relation to childcare. Impaired family functioning was also found to be associated with decreased QOL. These preliminary findings of relative deficits in family functioning may inform psychosocial interventions to assist pediatric liver transplant patients and their families. Further investigation beyond a single‐center study incorporating subjective information from pediatric patients and their parents is recommended.     

Referral of pediatric oncology patients for marrow transplantation and the process of informed consent

A survey of 24 pediatric oncologists from 21 institutions not performing bone marrow transplants found that 156 patients were referred for transplants in the years 1984/85; only 10 of these patients were not transplanted. No patient in good clinical condition whose disease was under control was denied transplantation after evaluation at a transplant center. The decision for transplantation was made by the families and the referring pediatric oncologists, almost always before the patient was seen at a transplant center. The fact that almost all patient and family evaluations at transplant centers take place after a family has decided to have a marrow transplant has obvious implications regarding informed consent. Referring pediatric oncologists must attempt to provide the best possible information to families when decisions regarding marrow transplantation are actually being made.     

Child and parental perspectives of multidimensional quality of life outcomes after kidney transplantation

Anthony SJ, Hebert D, Todd L, Korus M, Langlois V, Pool R, Robinson LA, Williams A, Pollock‐BarZiv SM. Child and parental perspectives of multidimensional quality of life outcomes after kidney transplantation.
Pediatr Transplantation 2010:14:249–256. © 2009 John Wiley & Sons A/S. Abstract: Kidney transplantation is an optimal therapy for pediatric patients with end‐stage kidney disease. This pilot study sought to examine multidimensional QOL outcomes after kidney transplant using VAQOL and General Health, the PedsQL 4.0, PedsQL End Stage Renal Disease Module, and Impact on Family Module. Sample included 12 adolescents aged 13–18 yr and their parent; three children aged eight to 12 yr and their parent; and six parents of children aged two to seven yr. All were 73 months post transplant. The median age at transplant was 9.3 yr and median time since transplant was 3.2 yr. VAQOL mean was 7.7/10 (child report) and 7.3/10 (parent report); the mean general health was 7.4/10. High levels of fatigue (≥5/10) were reported in 43%. PedsQL subscale mean values were lower than healthy reference scores. PedsQL Renal Module demonstrated great concern with physical appearance and physical symptoms (thirst and headaches), difficulty with peer and family interaction, and school disruption. Low scores on parental emotional function depict the negative impact of transplant on family functioning. Discordance exists between child and parental reports of QOL. Prospective studies are needed to explore multidimensional QOL to improve long‐term outcomes after pediatric kidney transplant.     

A survey of recommendations given to patients going home after bone marrow transplant.

A postal questionnaire was sent to 11 UK Children's Cancer Study Group bone marrow transplant centres asking them for details of their instructions to patients on discharge after either allogeneic or auto transplant; nine centres responded. There was no recommendation on which they all agreed. Though all centres gave prophylactic septrin, the times of starting and stopping treatment varied considerably. Three centres recommended lifelong penicillin after total body irradiation, one treated for two years and five gave no such prophylaxis. Four of nine centres gave routine acyclovir for herpes simplex prophylaxis. Most centres suggested prophylaxis against varicella after contact exposure for one year. However, three gave zoster immune globulin alone, one gave this together with acyclovir, and five gave acyclovir alone. No two centres recommended the same dose of acyclovir. Vaccinations were allowed from 6-18 months after transplant. One centre required documentation of recovery of immune function first. Four centres recommended a child stay off school for six months; others had 'common sense' approaches. Only one centre did not allow family holidays for the first six months but many imposed restrictions on these holidays. Dietary restrictions varied greatly between centres. It is concluded that there is a need for unified and scientifically justified guidelines after transplant for paediatric bone marrow transplant patients.     

Bone marrow transplantation

Summary Bone marrow transplantations have a definite role in treatment of leukemias and lymphomas. In acute myeloid leukemia and CML an allogeneic transplant using an HLA identical donor certainly provides a far superior survival than chemotherapy. Patients with Ph’ chromosome need to be transplanted in first remission if a suitable donor is available. In recurrent lymphomas the best results are chieved if the patient is transplanted in complete remission. Transplantation done using minor mismatched family donors or unrelated donors are still considered experimental and more data is needed before final recomendations can be made. Availability of supportive services is an absolute must prior to establishing transplant program. Selection of patients for transplantation should be done after carefully reviewing the indications and discussing with the family the emotional, financial and physical burden of the procedure. For selected indications in leukemias and lymphomas, BMT may be the only viable treatment option and therefore must be considered.     

Psychosocial assessment prior to pediatric transplantation: A review and summary of key considerations

Annunziato RA, Fisher MK, Jerson B, Bochkanova A, Shaw RJ. Psychosocial assessment prior to pediatric transplantation: A review and summary of key considerations.
Pediatr Transplantation 2010: 14: 565–574. © 2010 John Wiley & Sons A/S. Abstract: Prior to listing for transplantation, patients participate in a comprehensive, multidisciplinary evaluation. One component of this process, incorporated by the vast majority of transplant centers, is a psychosocial assessment conducted by a mental health professional. The primary objectives of a pre‐transplant psychosocial assessment are to identify risk factors for difficulty adjusting post‐transplant as well as behaviors that may compromise transplantation outcomes. This paper aims to provide a summary of key considerations for pediatric transplant teams describing what this assessment might include, when it should be performed, training requirements for the evaluators, how results of the evaluation might best be utilized and suggestions for optimal patient preparation. Our findings suggest that the evaluation, which can be conducted by a variety of professionals, should include assessment of patient knowledge and motivation for transplant, mental health and substance abuse history, presence or absence of family and social support, availability of financial resources, past history of treatment adherence, and the quality of the family’s relationship with the transplant team. Repeat assessments and utilizing the initial evaluation for outcome assessment should be considered. Finally, the evaluation offers a unique opportunity for better preparing patients and families for transplantation.     

An apparent case of undiagnosed donor Kawasaki disease manifesting as coronary artery aneurysm in a pediatric heart transplant recipient

We present a case of coronary ectasia and LAD coronary artery aneurysm with angiographic characteristics of Kawasaki disease in a three‐yr‐old girl two‐yr status post‐orthotopic heart transplant. Coronary anomalies were noted during initial screening coronary angiography two yr after transplant. Subsequent review of the donor echocardiogram revealed that the LMCA had been mildly dilated prior to transplant. In the absence of any symptoms consistent with Kawasaki disease in the transplant recipient, this appears to be a case of Kawasaki disease in the organ donor manifesting with coronary anomalies in the transplant recipient. The patient has done well clinically, and repeat coronary angiography has revealed partial regression of coronary anomalies. Given multiple reports in the literature of persistent abnormalities of coronary artery morphology and function after Kawasaki disease, close monitoring is warranted, with consideration of potential coronary protective medical therapies.     

Patients and their family members prioritize post‐transplant survival over waitlist survival when considering donor hearts for transplantation

Heart transplant providers often focus on post‐transplant outcomes when making donor decisions, potentially at the expense of higher waitlist mortality. This study aimed to assess public opinion regarding the selection of donor hearts and the balance between pre‐ and post‐transplant risk. The authors generated a survey to investigate public opinion regarding donor acceptance. The survey was shared freely online across social media platforms in April‐May 2019. A total of 718 individuals responded to the survey, with an equal distribution between patients and family members. Respondents consistently favored post‐transplant outcomes over waitlist outcomes. About 83.9% of respondents favored a hospital with longer waitlist times, worse waitlist outcomes, but excellent post‐transplant survival over a hospital with short waitlist times, a high waitlist survival, and inferior post‐transplant survival. This preference was no different between pediatric and adult populations (P = .7), patient and family members (P = .935), or those with a pre‐ vs post‐transplant perspective (P = .985). Patients and their family members consistently favor improved post‐transplant survival over waitlist survival when considering the risks of accepting a donor organ. These findings suggest that current practice patterns of donor selection align with the opinions of patients and family members with heart failure or who have undergone heart transplantation.     

Psychosocial responses of adolescent cystic fibrosis patients to lung transplantation

Abstract: What psychosocial issues do adolescent cystic fibrosis (CF) patients experience after undergoing lung transplantation (Tx)? The aim of this study was to determine, using an ethnographic study design, the common themes and emotional responses in post‐lung transplant adolescent CF patients of the Cardiothoracic Transplant Clinic at the Childrens Hospital Los Angeles. Nineteen CF lung transplant recipients were studied (eight males, 11 females: mean age at time of transplant, 15.7 ± 2.7 yr). The mean time interval from Tx to interview was 25.4 months (range 1–58 months). Sixteen patients had living donor lobar lung Tx while three patients received cadaveric lungs. A series of 25 questions was used to assess the psychosocial impact of Tx, and a semi‐structured interview focused on the following five domains: lifestyle, family functioning, social functioning, body image, and psychological functioning. The major themes identified by patients included: a strong desire to set and attain meaningful long‐range goals, the need to control as many aspects of their lives as possible while dealing with parental over‐protectiveness, and the adjustment to a new lifestyle. Common emotional responses included manageable fear/anxiety of lung rejection and uncertainty of the future, impatience with disruptions of daily routines caused by post‐transplant medical management and its effect on the attainment of set goals, and frustration with parental over‐protectiveness. In general, patients reported a positive outlook on life, with greater emphasis on sought‐after goals as well as inter‐personal relationships. This study demonstrates that adolescent CF transplant recipients develop long‐term goals and plans for independence. By identifying and anticipating the emotional needs of this population, health care providers can assist patients in improving the quality of their lives from a physiological, as well as a psychological, viewpoint.     

Isolated testicular leukemia following bone marrow transplant for acute lymphocytic leukemia. The need for pretransplant testicular biopsies

Bone marrow transplantation has become an accepted mode of treatment for children with acute myelocytic leukemia in their first remission and acute lymphocytic leukemia after their first bone marrow relapse. Two-year survival rates of 50% can be achieved in patients undergoing transplant during remission, in contrast to a 2-year survival of 15% in those undergoing transplant while still in marrow relapse. Recurrence of bone marrow leukemia relapse is a significant cause of marrow transplant failure. Overt or occult testicular relapse occurs in 10-15% of males with acute lymphocytic leukemia receiving or having completed standard therapy regimens for control of their disease and frequently leads to a subsequent bone marrow relapse. This paper describes a child with acute lymphocytic leukemia who received a successful marrow transplant following bone marrow relapse and developed testicular leukemia relapse approximately 20 months after transplant. The experience with this child suggests that bilateral testicular biopsies should be a mandatory part of the routine evaluation to screen for residual leukemia before bone marrow transplantation.     

Understanding the pathway between the transplant experience and health-related quality of life outcomes in adolescents

Abstract:  Developments in solid organ transplantation have resulted in improved survival for children with advanced kidney, liver, and heart disease; however, concerns have been raised regarding the quality of life of survivors. This study examined HRQOL in adolescent transplant recipients. We examined the influence of demographic, treatment regimen, and family factors on physical and mental health domains of HRQOL. The current single-center investigation involved 68 solid organ transplant recipients and their parents. All families participated in a structured interview to collect information on demographics, characteristics of the adolescents' disease and treatment regimen, family functioning, and HRQOL for parents and adolescents. Using hierarchical regression analyses, predictive models of physical functioning and mental health outcomes for adolescent transplant recipients were developed for parent-proxy and adolescent self-report. Perceived frequency of medication side-effects and family conflict significantly contributed to adolescent physical functioning and mental health outcomes. Taken together, transplant consequences and family environment significantly impact physical and mental health outcomes in adolescent transplant recipients. Our findings demonstrate the need for pharmacological considerations and psychological interventions to address these areas.     

Current status of heart transplantation in children: update 2003

Heart transplant is an effective therapy for children with end-stage heart disease. Success of this treatment depends on coordination and careful communication among the family, primary care physician, and transplant team. Primary care physicians play an essential role in the monitoring and management of the medical, nutritional, developmental, and psychosocial issues of pediatric heart transplant patients and their families (Box 3). Ongoing assessment of the child and parent's progress in adapting to transplant is crucial in order for appropriate referrals to occur. Relationships with the primary care team can improve medical outcomes for this complex group of patients and provide a framework for improved adherence to care.     

Best practices in the pediatric pretransplant psychosocial evaluation

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.     

Peripheral eosinophilia and eosinophilic gastroenteritis after pediatric liver transplantation

Reports indicate peripheral eosinophilia (PE) and gastrointestinal eosinophilic inflammation can occur after pediatric liver transplantation. The incidence of these conditions, potential risk factors, and the impact of PE and gastrointestinal eosinophilic inflammation on liver transplant outcome were determined in this pediatric liver transplant program. Medical records of liver transplant recipients from 1 to 97 and from 12 to 99 were reviewed. Fifty-seven transplants on 54 patients were performed during the study period. Fifty-three patients were evaluated; all had normal pre-transplantation peripheral eosinophil counts. PE of > 10% developed in 28% of patients. Using this definition, all such identified patients had absolute eosinophil counts of > 350/mm3. History of immediate hypersensitivity did not differ between patients with or without eosinophilia. Gastrointestinal endoscopy and biopsy was performed in 23 patients with gastrointestinal complaints. Of those, six had eosinophilic gastroenteritis and all six had PE. Compared with patients without eosinophilia, those with PE were younger at the time of transplantation (p < 0.05), had more frequent rejection (p < 0.01), were more commonly managed with tacrolimus-based immunosuppression (p < 0.001), and experienced more frequent episodes of detectable EBV viral load (p < 0.04). Patients with eosinophilic gastroenteritis were more frequently retransplanted (p < 0.006). PE associated with symptomatic eosinophilic gastroenteritis is common after pediatric liver transplantation. Age at transplant, frequency of rejection episodes, tacrolimus-based immunosuppression, and EBV viral load may be associated with the development of this condition. There may be higher rates of graft loss in such patients. Whether innate immune responsiveness or an acquired immune dysregulation accounts for these findings merits further evaluation.     

Intestinal transplantation in children

Intestinal transplant is now considered a viable option for children with irreversible intestinal failure suffering life limiting complications of parenteral nutrition. Identifying the possible candidates and referring them early to the transplant centre allows consideration of transplantation before the clinical situation deteriorates, and ensures the establishment of communication pathways between the family, transplant centre and the patient's local team. This encourages optimal care both before and after transplantation, and has been shown to improve long-term outcome.There are different types of intestinal transplants that may be performed according to the indication. With improvements in surgical techniques, immunosuppression, early identification of rejection and good communication between professionals and families, outcomes for patients undergoing these procedures continue to improve.     

Family strain and its relation to psychosocial dysfunction in children and adolescents after liver transplantation

Parental functioning is essential to children's development. Therefore, this cross‐sectional single‐center study examined the prevalence of family strain in 181 parents and its associations to psychosocial functioning in their children after LT. Median age at LT was one yr. Mean time elapsed since LT was 5.8 yr. The IFS, and the SDQ were applied to parents. Family strain in the present sample was comparable to that in the German normative group of families with a chronically ill or disabled child, but families of LT recipients showed a significantly higher financial impact, impact on coping, and impact on siblings (p < 0.001). Younger age of patients at survey, a more severe clinical course, child's restrictions, and financial losses following LT were determined as significant predictors of family strain (R² = 0.42). Parents reported less family strain after living‐related compared with deceased donation. Family strain was significantly correlated to psychosocial dysfunction in children post‐LT. Present findings demonstrate a risk of maladjustment to the post‐LT condition in families. They emphasize the importance of psychological assessment of parents and patients during transplant and follow‐up to ensure the best achievable long‐term outcome of patients.     

Multidimensional assessment of quality of life of children and problems of parents in Indian society after pediatric renal transplant: Beyond the conventional thoughts

This study evaluated multidimensional QoL after renal transplant to find the physical, psychosocial, and economic issues after pediatric transplant. Sixty‐two patients under the age of 18 at the time of assessment were asked to complete WHOQOL questionnaires. Assessment of behavioral, emotional status of child, problems of parents, and SEC of family were also performed. The beneficial effect of transplantation was observed across all domains of QoL (physical, psychological, social, environmental, and overall QoL). The greatest change was observed in QoL domains that dealt with overall satisfaction (81.28±15.76 vs 45.32±10.98; P<.0001). The domain dealing with environmental factors showed the least variation after transplantation (65.58±17.45 vs 51.34±17.81; P<.0001). Feelings of happiness and peer group socialization were reported in 81% and 69% of patients, respectively. There was no marital disharmony in 52% of families. However, SEC deteriorated in 16% of families and 59% of the families availed financial assistance. Sixty‐nine percent of children had not attended school after one year of transplantation, but return to school after transplant was reduced to 8% at 5 years. Pre‐ and post‐transplant social and psychological support may help these patients and their families adjust in society and have a positive outlook for their future.     

School and sports participation post‐transplant

Pediatric recipients of life‐saving organ transplants are living longer, with improved graft and overall survivals. After successful transplant, children are encouraged to return to “normal life,” with school attendance and participation in age‐appropriate physical activities. This transition may cause stress to the recipients, parents, teachers, and other participating caregivers and staff. Planning for school reentry and assuring education for and open lines of communication with the school staff can help alleviate some of this discomfort and ease the process for the patient and the family. Cardiovascular disease has emerged as the leading cause of death in survivors of pediatric transplantation and is contributed to by modifiable risk factors such as obesity, hypertension, and the MS. Physical activity is a proven tool in decreasing surrogate markers of this risk. Sports participation is an important way to promote an enjoyment of physical activity that can ideally persist into adulthood, but conflicting advice and opinions exist regarding type and participation in physical activity. Moreover, specific recommendations are likely not applicable to all recipients, as certain degrees of rehabilitation may be needed depending on degree and length of illness. In general, a program of rehabilitation and increased physical activity has been shown to be safe and effective for most pediatric transplant recipients. Focusing on optimizing the “normal” childhood activities of going to school and participating in sports can improve the physical, social, cognitive, and mental health outcomes of this population after transplant and should be prioritized.