Long-term survival after pediatric solid organ transplantation is now the rule rather than the exception for increasing numbers of children with end-stage organ diseases. While transplantation restores organ function it does not necessarily return one to a normal life. Therefore, it is prudent to focus on assessment of not only traditional biologic outcomes but also the quality life for these children and their families. This article gives a brief overview of current definitions, conceptualizations, approaches to measurement of, and unique considerations in the evaluation of quality of life in children who have undergone solid organ transplant. Current understanding of quality of life in children who have undergone solid organ transplantation is reviewed, followed by limitations of current knowledge. Clinical implications are discussed and future research directions suggested.
Long-term survival after pediatric solid organ transplantation is now the rule rather than the exception for increasing numbers of children with end-stage kidney, heart, lung, liver, and small bowel diseases. Although life-saving, solid organ transplantation is not curative; a fatal disease has been replaced by a chronic condition with its own associated morbidities, which may evolve or be present despite well-preserved allograft function. While transplantation restores organ function, it does not necessarily return one to a normal life. Therefore, it is prudent to now focus on assessment of not only traditional biologic outcomes but also the quality of life of these children and their families. Health-related quality of life provides a more comprehensive evaluation than do disease parameters alone of the impact of an illness and its treatment on functioning and well-being. Improving long-term outcomes after solid organ transplantation must consider not only the quantity but also the quality of life years survived. This article gives a brief overview of current definitions, conceptualizations, approaches to measurement, and unique considerations in the evaluation of quality of life in these children. Current understanding of quality of life in children who have undergone kidney, heart, lung, liver, or small bowel transplantation is reviewed, followed by limitations of current knowledge. Clinical implications are discussed and future research directions suggested.
Quality of life: definitions and conceptualization
In the World Health Organization’s (WHO) 1948 landmark definition of health, the traditional considerations of physiologic factors and the absence of disease were expanded to encompass the broader multidimensional domains of “physical, mental, and social well-being.” While this commonly cited definition of health has remained the cornerstone of the quality of life construct, many varying definitions of quality of life have been proposed over the years. In excess of 100 definitions of the term ‘quality of life’ have been identified in the literature. Although a precise and universally accepted definition of quality of life has yet to be framed, there is growing consensus around two fundamental concepts that are inherent in most definitions. First, quality of life is primarily subjective, and should therefore be assessed from the patient’s perspective whenever possible. Individuals have their own unique perspective on quality of life, which depends on present lifestyle, past experience, hopes for the future, dreams, and ambitions. Second, quality of life is generally conceptualized as a multidimensional construct encompassing several broad domains, with the most widely used domains reflecting those articulated by the WHO definition: physiologic, social, and psychological factors.
Physiologic Factors
These factors include clinical indicators of health, energy level, and functional status (the ability to perform tasks of daily living). Objective clinical indicators of health remain the cornerstone of medicine and provide standardization; however, individual factors such as tolerance of pain and individual perceptions can influence evaluations of well-being even in the face of normal physiologic parameters. Indeed, low levels of energy and limitations in functional health status can negatively influence ratings of global quality of life by creating, feelings of ineffectiveness, social restriction, and emotional distress. Although there is a general reliance by clinicians on objective assessments, these frequently correlate poorly with subjective ratings of patients’ well-being. Results from patient-driven tools may therefore better reflect functional status and quality of life.
Social Factors
These factors include both social health and social support. Social health is the ability to perform normal social roles, how one gets along with others and how others react to them, and incorporates personality, social skills, and one’s available social supports. Social supports can directly influence physical health by easing the emotional or tangible burden of physical symptom, and can attenuate the effects of stressful life events and reduce severity of disease. Measurement of social support typically includes self-administered rating scales, and interpreting results must reflect diverse social norms across individuals, cultures, and social strata.
Psychological Factors
These factors include emotional status, self-esteem, body image, and cognitive variables. Evidence indicates that psychological factors affect health and physiologic responses to illness and treatments. For example, anxiety and depression can affect adherence to treatments, hasten disease progression, and negatively affect one’s quality of life. These variables may be directly amenable to the development of interventions to alleviate suffering, improve treatment adherence, and in turn improve perceptions of quality of life and enhance long-term outcomes. The relationship between physical health and psychological states is complex, with an impact that may be currently underappreciated due to a tendency toward a medicalized nature and focus of care.
Approaches to quality of life measurement
Meaningful assessment of patient quality of life mandates the ability to reliably and accurately assess well-being. Numerous generic and disease-specific indices and indicators have been developed that attempt to assess children’s quality of life. Within the context of pediatric transplantation, there remains no gold standard instrument with the ability to measure concerns which may be specific to this particular patient population. As a result, it is very difficult to compare, generalize, or replicate findings. Important methodological considerations that require attention are discussed here.
Generic Versus Disease-specific Tools
Generic quality of life measures are multidimensional, consisting of, at minimum, the physical, psychological, and social health dimensions delineated by the WHO definition of health. Generic measures are designed to be broadly applicable across many types of diseases, treatments, and groups of individuals, and permit comparisons across interventions and diagnostic conditions. In addition, data can be compared with general pediatric population norms to determine the impact of disease on quality of life. Disease-specific measures, on the other hand, take into account aspects of disease and treatment relevant to a specific medical condition such as transplant-related issues (eg, medication side effects, worry about graft rejection, graft loss, and need for retransplant). Such measures may be more sensitive to changes in a child’s condition, tend to be more effective at detecting treatment effects, and therefore may have greater salience for clinicians. At present the use of both generic and disease-specific measures is likely to provide valuable and complementary information to further understand the quality of life in children who have undergone transplantation.
Self-report Versus Proxy Report
Given the subjective nature of quality of life, there are strong arguments in favor of eliciting data directly from children wherever possible, given data showing that information provided by proxy respondents is not equivalent to child self-report no matter how well-intentioned or informed the adult. In a meta-analysis evaluating the agreement between child and proxy reports in chronically ill populations, researchers found greater agreement between children’s and parents’ reports for observable behaviors such as physical functioning than for nonobservable functioning such as emotional or social quality of life.
Although the perspectives of children in evaluating quality of life are extremely important, direct assessment may be limited by age, medical condition, and developmental ability for comprehension, hence requiring a parent or caregiver to act as a proxy for the child. When it is not possible to elicit quality of life information from a child, the perspectives of parents are valuable in enabling the assessment of every child in population-based studies. Although there may be a degree of distortion in parental responses about their child’s quality of life, parents nonetheless are usually a knowledgeable source of information about their child.
Adult Versus Pediatric Measurement
Although published studies consistently report overall improvement in global quality of life for adults who have undergone transplantation, the focus on adult-specific issues such as employability, medical leave from work, marital relationships, and sexuality means these findings cannot be extrapolated onto the lives of children who have undergone transplantation. Issues that may be salient for children include school attendance, exercise capacity and sport participation, peer groups, bullying, growth retardation, and weight and body image. The challenge with measurement of pediatric quality of life relates to the wide developmental spectrum across the pediatric age group. Each developmental period is characterized by unique sets of issues, milestones, and developmental and psychosocial considerations. What might be important in the life of a young child may no longer apply once the individual reaches adolescence. Furthermore, adolescence is a particularly unique period, distinct from adulthood, and often described as emotionally turbulent and in transition. Issues unique to adolescents such as puberty, sexual development, search for autonomy and transition to adulthood have strong relevance for the study of quality of life and have been inadequately addressed in the literature.
In summary, traditional posttransplantation outcomes such as number of biopsy-proven rejection episodes, graft function, hospitalization days or infections, or patient mortality rates do not adequately measure the impact of children’s day-to-day life following transplant. Optimizing the future success of solid organ transplantation in children is defined by more than just excellent patient or graft survival rates. Further attention to strategies to achieve the goal of a health state that is desirable and a marked improvement from the pretransplant condition are needed. Assessment of quality of life will permit health care professionals to hear the voice and perspective of the patient, and has been found to predict, often better than physiologic measures, future health status, mortality, and resource use, which are critical issues in the care of children worldwide who have undergone, or will require, kidney, heart, lung, liver, or small bowel transplantation. The findings are now summarized.
Quality of life following pediatric kidney transplantation
Pediatric kidney transplantation is widely used, both with deceased and living donors. Among living donor recipients, 1- and 5-year patient survival rates exceed 98% and 96%, and deceased donor survival rates are 97% and 96% at 1 and 5 years, respectively. Current literature conveys excellent long-term outcomes for children who have undergone kidney transplant. However, these results are mostly limited to patient and graft survival, and freedom from rejection and morbidities. Quality of life research in pediatric kidney transplant patients remains scarce, with most existing studies relying on cross-sectional assessment or measurement of single dimensions of quality of life such as physical or social aspects.
Kidney transplant recipients experience better overall health and well-being after transplant as measured by a wide range of instruments of varying degrees of validity. Variables used to assess psychosocial adjustment after transplantation include return to school and level of education attained, neurodevelopmental outcome, prevalence of psychological disorders, and later employment status, as well as more specific quality of life measurement tools including the Pediatric Quality of Life Inventory Generic Scale and End Stage Renal Disease Module, Child Health Questionnaire, and the Child Behavior Checklist, to name a few. These studies almost universally have reported good long-term psychosocial outcome after transplantation. However, many physical and psychosocial issues remain salient after transplant. It has been shown that physical complaints such as headaches and fatigue are common in children after kidney transplant, as well as negative impacts of medications on growth (eg, short stature), weight gain, and general health. Physical effects of medications can also influence behavioral adjustment. In particular, research has demonstrated a strong relationship between medication side-effects and nonadherence with medical regimens. Medication adverse effects also have been shown to impact on social and psychological domains. It has been reported that somatic complaints and social problems are more common in boys after transplant, and that children have increased emotional problems when there are overt signs of illness/treatment related to immunosuppressive medications (eg, short stature, weight gain, mouth sores).
Issues pertaining to school function are of paramount importance. Difficulty with school re-entry and prolonged absence from school due to treatment-related issues impact on academic achievement, which then can lead to a detrimental effect on sustaining peer relationships, poor self-concept and self-esteem, all of which negatively influence quality of life. Studies have shown that most children are enrolled in regular schools; however, research has demonstrated varying degrees of problems with learning and memory, inferior school performance on standardized achievement tests, and attention problems that require intervention to maximize academic success in pediatric kidney transplant recipients.
Children living with end-stage kidney disease are at risk for anxiety and depression. In general, psychosocial factors such as depressive symptoms and anxiety, strained social relationships, and adjustment issues improve after organ transplantation, but they continue to warrant attention over the long term. While several studies have assessed domains of psychological well-being, there is minimal research on actual rates of anxiety and depression in pediatric kidney transplant recipients; most available studies use screening tools rather than diagnostic criteria and therefore do not properly ascertain the true incidence and implications of these factors in children. Recent research compared 40 adolescent renal transplant patients with healthy peers and chronic kidney disease patients using psychiatric diagnostic interviews, and the Child Behavior Checklist self-rating scale. The investigators demonstrated that 65% of transplant recipients met DSM-IV ( Diagnostic and Statistical Manual of Mental Disorders-IV ) criteria for a lifetime psychiatric disorder compared with 60% of chronic kidney disease and 37.5% of controls.
A recent exploratory study investigating multidimensional aspects of quality of life in 23 pediatric renal transplant recipients and their parents using the Pediatric Quality of Life Inventory Generic and End Stage Kidney Disease Modules found that children reported good overall quality of life, yet results were lower than healthy children samples across all subscales. Parent ratings were lower across all domains especially on emotional, social, school, and physical summary scales. This study identified concerns in the following four areas: (1) physical symptoms (eg, fatigue and thirst); (2) body image and weight concern in adolescents; (3) school disruption due to medical follow-up; and (4) strained relationships with family and peers.
Much of the research on quality of life in kidney transplant reflects long-term effects of an earlier era whereby higher levels of immunosuppression were used. Many kidney transplant programs now emphasize steroid minimization and steroid-free protocols, as well as more targeted immunosuppression. The impact of this on quality of life remains to be seen, but it can be speculated that kidney transplant recipients in the more recent era should expect better growth trajectories and less adverse drug effects, both of which are strongly linked to higher quality of life perceptions. Primary health care providers can actively screen for emotional distress, depression, and anxiety as well as the availability of social supports and adequate school function. Assessing for the presence of physical effects of medications such as headaches and fatigue will provide opportunities for interventions and surveillance of adverse medication effects to improve the long-term quality of life of transplant recipients.
In summary, children who undergo kidney transplant can experience excellent survival and quality of life outcomes, yet there remain important issues regarding physical and psychosocial aspects after transplant that warrant further attention over the long term.
Quality of life following pediatric liver transplantation
Long-term survival after pediatric liver transplantation is now the rule rather than the exception. One-year and 5-year survival statistics are high, approximately 95% and 85%, respectively. More than 11,000 children in the United States (UNOS Web site, accessed October 2009) and Canada have received a liver transplant for the spectrum of indications, as discussed elsewhere in this issue. Advancing the understanding of health-related quality of life is an important issue to address to further improve the long-term well-being, health, and survival of this specific patient population. Before 2000, most reports on quality of life in pediatric liver transplant recipients were cross-sectional investigations using several generic and some transplant-specific tools with varying degrees of validity. However, more recent single-center studies have used well-validated instruments, which have enabled the comparisons of quality of life in pediatric liver transplant recipients with other chronic disease groups. These validated generic tools have included the PedsQL 4.0 Generic Core Scales, Child Health Questionnaire Parent Report Form 50, Child Health Questionnaire Child Form 87, Health Utilities Index Mark 3, Infant Toddler Quality of Life Instrument, PedsQL Multidimensional Fatigue Scale, and PedsQL Cognitive Functioning Scale. Important findings from single-center studies as well as available published data from multicenter collaborations are highlighted here.
Quality of life in children and adolescents after liver transplantation has been evaluated in several single-center experiences and reviewed in a recent publication. This critical systematic review of 11 of the most important published single-center studies, representing 395 children and adolescents who received liver transplants between 1981 and 2002, summarized that pediatric liver transplant recipients have (1) decreased quality of life compared with healthy children peers in the specific domains of physical, psychological, social, and family functioning; and (2) equal, or better, quality of life than comparison groups of children and adolescents with other chronic illnesses, such as pediatric juvenile arthritis, asthma, and epilepsy. Decreased psychosocial function also identified concerns in the area of school function. One study assessing the academic outcome of a cohort of 50 children alive 3 to 9 years after pediatric liver transplantation revealed that almost 20% had an intelligence quotient score of less than 70. It is as yet unknown whether an increased prevalence of developmental delays or learning disabilities exist in this population. Further, it is also possible that increased school absenteeism because of illness or doctor visits may be important contributors. School findings in a multicenter cohort of pediatric liver transplant recipients are discussed by Kamath and Olthiff elsewhere in this issue.
Subsequently, more recent single-center studies evaluating quality of life after pediatric liver transplantation have verified similar domain findings and raised additional relevant concepts. Attention to the adolescent-age population have reiterated the previously noted discrepancy between self-report and proxy reports, with adolescents self-rating their physical, emotional, and social functioning as normal whereas their parent proxy did not. A second single-center experience with adolescent survivors of liver and kidney transplantation demonstrated similar self-reported physical and psychological function to healthy controls, in contrast to parent-proxy reports viewing general health and physical function of these same patients to be poorer than the normative population. This contrast in viewpoint supports the concept that ratings of both parents and patients should be considered to gain a fuller picture of quality of life outcomes. Finally, assessment of family functioning identified no difference between families with a pediatric liver transplant recipient compared with both a normative sample and families with a child with a chronic illness.
Understanding of the factors affecting quality of life for children after liver transplantation has been explored. Pretransplant factors associated with improved quality of life include a primary diagnosis of biliary atresia, Caucasian race, and maternal education level, whereas older age at time of liver transplant has been associated with decreased quality of life. Posttransplant variables associated with better quality of life include fewer hospitalizations in the preceding year, improved height parameters, longer time since liver transplant, and higher self-esteem, while not surprisingly, variables of medication side effects, headaches, the development of a second comorbid condition, and parental conflicts were associated with decreased quality of life.
Realizing the limitations with even the most well-designed single-center experiences, the pediatric liver transplant community has capitalized on a research infrastructure called the Studies of Pediatric Liver Transplantation (SPLIT). Since its inception in 1996, SPLIT has worked to sustain a prospective collection of comprehensive pre- and posttransplant medical data on pediatric liver transplant candidates and recipients from pediatric liver transplant programs in the United States and Canada. Results from a large cross-sectional analysis of 873 pediatric liver transplant recipients from 20 pediatric liver transplant programs in the United States and Canada were reviewed by Alonso and colleagues on behalf of the SPLIT Functional Outcomes Group (FOG). A subsequent preliminary subanalysis of 110 children with fulminant liver failure (from within the entire FOG study cohort) who underwent emergency liver transplant revealed that the patients themselves self-report similar quality of life to healthy children controls on the 23-item PedsQL Generic Core Scales self-report except in the domain of school functioning. This result is in contrast to their parents, who proxy reported impaired health-related quality of life across all domains compared with healthy controls, emphasizing the need to assess qualitative outcomes directly from the child’s perspective whenever possible.
Further to single-center experiences studying family function, Alonso and colleagues conducted a 5-center cross-sectional analysis of quality of life and family function dynamics in a homogeneous population of 2-year survivors of pediatric liver transplantation, which showed that younger survivors (age <2 years) had lower scores in the domains of global health, general health perceptions, and change in health as compared with older transplant recipients (patient age >5 years at time of assessment) as reported by the completed parent-proxy reports of the Infant Toddler Quality of Life Instrument and the Child Health Questionnaire Parent Form 50, who had lower scores in the domains of physical health and general health. Median scores from the parent-proxy report suggest that younger survivors had fewer issues with discomfort and pain compared with the control group created for the study. Mean family assessment device scores indicated that families of a liver transplant recipient do not have increased levels of family dysfunction.
While physical function, mental health, cognitive development, and daily living skills are good examples of domains that can be assessed by all pediatric health care providers involved in the long-term follow-up for survivors of pediatric liver transplantation, it is also important to highlight dimensions that are uniquely relevant to pediatric liver transplant recipients. Qualitative 1-to-1 interviews conducted with 63 children or adolescent liver transplant recipients and 84 parents from Canada and the United Kingdom identified a range of common and diverse childhood experiences and perceptions of quality of life following pediatric liver transplant. These item generation interviews identified many generic items relevant to children with chronic medical conditions, and include physical health (restrictions), social (peer relationships, bullying, and teasing), emotional (fears and worries about future health, sadness due to knowledge of their parents worrying about them, and self-blame for imposing worry on family members), and school (attendance, ability to concentrate) domains. In addition, many disease-specific domains were identified as particularly relevant to a patient who has undergone liver transplantation as a child, which are not currently captured in available validated generic tools. Highlights of the top 9 disease-specific domains are: (1) vulnerability to specific posttransplant complications; (2) altered body image (living with a scar from the liver transplant surgery); (3) fear of infection; (4) thoughts about the future (rejection, retransplantation); (5) altered lifespan because of having a liver transplantation as a child; (6) living with a liver that is not the native one; (7) treatment-related challenges (side effects unique to immunosuppression medications, difficult procedures); (8) lifelong needs (medical team care, transition to adult-care teams), and (9) behavioral responses (compliance and nonadherence to surveillance and treatment regimens). To date, there is no published validated disease-specific health-related quality of life tool for use in pediatric liver transplantation patients, although work is ongoing toward this goal, so that in the near future such a tool is likely to become available. Completion of psychometric testing is necessary to ensure broad cross-cultural applicability of an eventual health-related quality of life instrument for liver transplant recipients, thus ensuring that the assessment of quality of life in this population is culturally sensitive and will lead to a more nuanced understanding of the factors that influence transplant recipients’ well-being.
Quality of life following pediatric heart transplantation
In the last decade heart transplantation has proven to be an effective and accepted therapy in pediatric patients with lethal heart disease. One-, 5-, and 10-year survival statistics are high, approximately 95%, 75%, and 65%, respectively. Since 1982 more than 7750 pediatric patients have received heart transplants, with an annual transplant rate of around 400. In pediatric heart transplantation the investigative issues that have been most emphasized in past years pertain to mortality risk factors and morbidity, yet with improved medical outcomes, current research has shifted toward understanding and enhancing quality of life. Although data regarding survival have justified continued use of heart transplantation in children, there is a paucity of published data regarding the quality of life of these children. To date there is little literature available about the psychosocial effects on children who undergo heart transplantation, and their long-term psychological and social functioning remains unclear.
Early studies using functional status to evaluate the quality of life of pediatric heart transplant recipients indicated that quality of life after transplant improved and transplant recipients experienced a much improved physical status after recovery from the transplant process. The literature reported that postoperatively, children were active and could generally return to age-appropriate developmental, educational, social, and recreational activities. In early retrospective clinical chart reviews, researchers reported that 89% to 100% of pediatric heart transplant recipients participated in or were capable of participation in normal activities for age and “lead an active and satisfying life.” In a multicenter study of 49 pediatric patients, it was revealed that most children experienced a dramatic improvement in functional status following heart transplant, with 93% attending school and participating in recreational activities including swimming, biking, skating, skiing, baseball, volleyball, and tennis. Research examining the behavior and cognitive functioning of 65 and 81 children who had undergone heart or heart-lung transplantation found that most children had returned to activities appropriate for their age and had settled back into school, with academic performance attainments, evaluated by the British Ability Scales, as well as the Schonell graded spelling test, within normal limits.
With a focus more on psychosocial outcomes as proxies for quality of life, a study examining the psychological functioning of 23 pediatric heart transplant recipients, which used clinician ratings on the Children’s Global Assessment Scale, found that the majority of patients (78.3%) demonstrated improved psychological functioning after their transplant. In a longitudinal follow-up study, improvement in psychological functioning was maintained over a decade, with 73% patients having improved emotional and psychological functioning. Similarly, employing semistructured interviews as well as several standardized questionnaires including the Global Assessment of Functioning Scale, Children’s Behavior Questionnaire Scale, and the Piers & Harris Self-Concept Scale, an assessment of psychosocial outcomes in 23 heart and 21 heart-lung transplant recipients found significant improvement in physical health and psychosocial functioning 12 months after transplant. These results are consistent with 2 prospective evaluations of heart or heart-lung transplant recipients, which revealed that following transplantation overall physical and psychosocial functioning improved. In 2003 Pollock-BarZiv and colleagues examined the self-perceived quality of life of 8 heart transplant recipients, aged 10 to 18 years, and results demonstrated excellent overall quality of life and psychological well-being. A rare qualitative study found that most children described their lives in positive terms, such as “mostly good” or “fun,” and valued the normal aspects of life.
Despite significant improvements in functional status, quality of life, and psychosocial outcomes, results of some studies suggest that a significant minority of patients do experience psychological difficulties at some stage after transplantation. In a review of psychological outcomes of heart transplantation in children, Todaro and colleagues reported that a proportion of recipients are at risk for psychological difficulties following heart transplantation. Results indicated that pediatric heart transplant recipients are at increased risk of experiencing psychological distress, such as anxiety, depression, behavioral disorders, and poor self-concept, compared with normal, healthy peers. There appears to be a higher prevalence of psychological difficulties and stress among pediatric heart transplant recipients, resulting in symptoms of negative affect, decreased social competence, and behavior problems. These findings suggest that for some patients, there may be psychological difficulties coping with the demands or stressors of pediatric heart transplant.
It may be concluded that while problems do remain, the overall results of these studies support the adaptive potential of children and adolescents following heart transplantation, and indicate that the majority of pediatric recipients appear capable of healthy physical and psychological functioning after heart transplant.
Quality of life following pediatric lung transplantation
Since the first successful lung transplant was performed in the early 1980s, ongoing advances in surgical methods and improved medical management have led to 1- and 5- year patient survival rates of 83% and 50%, respectively. The most recent report of the International Society for Heart and Lung Transplantation (ISHLT) registry reveals nearly 1000 pediatric lung transplants to date. The most common reason for lung transplant in children is cystic fibrosis, but other etiologies include pulmonary hypertension, interstitial disease, congenital diseases, and other causes ( http://www.unos.org ).
While lung transplantation is now widely used for advanced lung disease, research examining quality of life in lung transplant recipients has only begun to surface in the past decade. Numerous clinical and registry studies demonstrate improved survival after lung transplant, yet studies focusing on quality of life remain scarce, particularly within pediatrics. In fact, due to the small numbers of pediatric lung transplants it is uncommon to find a study dedicated to quality of life in this cohort, and most published data to date in children are found embedded within adult studies, or along with research on heart transplant populations.
Issues specific to pediatric lung transplantation include a high need for mechanical ventilation before transplant, challenges in surveillance of rejection and chronic rejection using spirometry in younger children, and increased complications related to infection both before and after transplant. Research thus far shows improved overall quality of life after lung transplantation. A comprehensive literature review between 1980 and 1999 found only 13 published reports addressing quality of life after lung transplant, with most emanating from Canada, the United States, and the United Kingdom. Additional studies have since emerged, yet limited published data exist that focuses on quality of life issues in pediatric lung transplant recipients. The recent ISHLT registry reported no activity limitations in 80% of children surviving 7 years following transplant ; as yet, the long-term psychosocial status remains unknown. A study by Durst and colleagues used qualitative interviews to examine psychosocial issues after lung transplantation in adolescents with cystic fibrosis. The study reported that after transplant patients were able to develop long-term goals, and a wish to reclaim control over their lives as much as possible and adjust to a new lifestyle; yet common emotional responses included fear and anxiety over rejection, uncertainty over the future, and frustration with parent over protectiveness. These findings represent a cohort that has been chronically ill and has endured years of hospitalization and treatments, so results may not necessarily translate onto other pediatric lung diagnoses after transplant. Future studies are urgently required to decipher quality of life issues among short- and long-term survivors of pediatric lung transplantation.
Quality of life after pediatric small bowel transplant
Intestinal transplantation has become a standard treatment for intestinal failure in patients with life-threatening complications of parenteral nutrition. Over the last decade, 1- and 5- year patient survival rates are as high as 80% and 50%, respectively, with survival rates in high-volume centers in the United States and Europe even better, as reviewed in the article by Avitzur and colleagues elsewhere in this issue.
The quality of life after small bowel transplantation is probably equal to or better than quality of life on parenteral nutrition, and children report quality of life similar to normal school children, although these results require longer follow-up. The achievement of oral tolerance in children who previously were dependent on parenteral nutrition is a disease-specific item unique to children with intestinal failure who survive intestinal transplantation. Indeed, a general assessment of quality of life by means of oral tolerance and the Karnofsky score, a general performance status tool, showed that the majority of surviving recipients tolerate enteral feeding and are capable of normal activities as recorded in the Intestine Registry ( Intestinal Registry Report , Bologna, 2009).
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