"Transition from children's to adult services for adolescents/young adults with life-limiting conditions: developing realist programme theory through an international comparison".

BACKGROUND: Managing transition of adolescents/young adults with life-limiting conditions from children's to adult services has become a global health and social care issue. Suboptimal transitions from children's to adult services can lead to measurable adverse outcomes. Interventions are emerging but there is little theory to guide service developments aimed at improving transition. The Transition to Adult Services for Young Adults with Life-limiting conditions (TAYSL study) included development of the TASYL Transition Theory, which describes eight interventions which can help prepare services and adolescents/young adults with life-limiting conditions for a successful transition. We aimed to assess the usefulness of the TASYL Transition Theory in a Canadian context to identify interventions, mechanisms and contextual factors associated with a successful transition from children's to adult services for adolescents/young adults; and to discover new theoretical elements that might modify the TASYL Theory. METHODS: A cross-sectional survey focused on organisational approaches to transition was distributed to three organisations providing services to adolescents with life-limiting conditions in Toronto, Canada. This data was mapped to the TASYL Transition Theory to identify corresponding and new theoretical elements. RESULTS: Invitations were sent to 411 potentially eligible health care professionals with 56 responses from across the three participating sites. The results validated three of the eight interventions: early start to the transition process; developing adolescent/young adult autonomy; and the role of parents/carers; with partial support for the remaining five. One new intervention was identified: effective communication between healthcare professionals and the adolescent/young adult and their parents/carers. There was also support for contextual factors including those related to staff knowledge and attitudes, and a lack of time to provide transition services centred on the adolescent/young adult. Some mechanisms were supported, including the adolescent/young adult gaining confidence in relationships with service providers and in decision-making. CONCLUSIONS: The Transition Theory travelled well between Ireland and Toronto, indicating its potential to guide both service development and research in different contexts. Future research could include studies with adult service providers; qualitative work to further explicate mechanisms and contextual factors; and use the theory prospectively to develop and test new or modified interventions to improve transition.

Transition of Care in Congenital Heart Disease: Ensuring the Proper Handoff.

BACKGROUND: With great advances in medical and surgical care, most congenital heart disease patients are living in to adulthood and require lifelong surveillance and expert care for adult onset complications. Care lapse and lack of successful transfer from pediatric to adult care put young adults at risk for increased morbidity and premature death. Hence, transition and transfer from pediatric to adult care is a crucial and critical process to provide access to specialized care and lifelong surveillance. PURPOSE OF REVIEW: The aim of this article is to describe barriers to successful transition and transfer and to share practical strategies and concepts to overcome these barriers in order to successfully implement a transition program. RECENT FINDINGS: There are patient-specific, local, and institutional specific barriers to establish a successful transition program which involves many stakeholders. Collaboration of the Pediatric and Adult Congenital Heart Disease programs is paramount; the understanding of the benefit and the need of a structured transition program, dedication, and a proactive approach are essential. Youth- and family-centered education improves healthcare knowledge, self-management, self-advocacy, and appropriate interdependence and helps young adults to take ownership of their health. Nurses play an integral role within the multidisciplinary team in supporting seamless, successful transition and transfer of CHD patients from pediatric to adult care thereby reducing loss to follow-up and lapses in care. Most experiences and recommendations are based on retrospective studies and expert consensus. It is imperative to evaluate the impact of structured and planned transition/transfer programs on the outcomes. Hence, prospective, randomized trials are required to document if implementation of structured intervention transition programs improve knowledge, patient experiences, and outcomes of congenital heart defect survivors.

Tools for addressing systems issues in transition.

Moving from pediatric to adult healthcare is a time of stress and opportunity for adolescents with special healthcare needs (ASHCN) and their families. With over 90% of children with special healthcare needs surviving into young adulthood, there is an increasing imperative to actively engage youth in preparing for the adult system (Betz and Smith 2011; Pai and Schwartz 2011). The goal of transition care is to provide young people with a coordinated, uninterrupted and developmentally appropriate transfer to adult healthcare (Kaufman and Pinzon 2007). This is often complicated by the complex medical, social and psychological tasks and requirements that adolescents experience as they navigate their healthcare and their lives as young, developing people.

Functional health status of adolescents after the Fontan procedure -- comparison with their siblings.

BACKGROUND: Studies have suggested that patients who have undergone the Fontan procedure experience lower functional health status and diminished exercise capacity compared with other children. OBJECTIVES: To compare the functional health status of Fontan patients with and without siblings, assess whether there are any differences between Fontan patients and their siblings, and determine associated factors. METHODS: A cross-sectional, single-centre, observational study was performed on Fontan patients 10 to 20 years of age, and their sibling closest in age, followed in a tertiary pediatric hospital. Functional health status was measured by the Child Health Questionnaire Child Form and the Pediatric Quality of Life Inventory. RESULTS: A total of 68 patients and 38 siblings were enrolled. Patients with siblings scored significantly lower on numerous domains of physical functional status than those without siblings. Compared with their matched siblings, Fontan patients reported significantly lower scores in all domains of the Pediatric Quality of Life Inventory and on physical (but not psychosocial) domains of the Child Health Questionnaire Child Form. Factors associated with increased patient-sibling differences included younger patient age, female sex, intracardiac lateral tunnel Fontan connection and lower ejection fraction at the time of study enrollment. CONCLUSIONS: Adolescents with Fontan physiology reported a lower functional health status in physical domains than their siblings, but had similar status in psychosocial domains. Having a sibling was associated with lower reported functional health status, suggesting an important effect of self-perceived physical limitations over true limitations.

Impact of increasing adiposity in hyperlipidemic children.

Despite lifestyle management, children with high-risk hyperlipidemias may become overweight, and this may further adversely impact their lipid profile. Regression analysis was used to determine changes over time in adiposity and their association with lipid profiles and other risk factors for hyperlipidemic children followed in a lipid disorder clinic. 184 patients were included. Median age at presentation was 7 years (2-17 years), and median duration of follow-up was 9 years (5-20 years). Mean initial total cholesterol was 6.9+/-1.6 mmol/L, low-density lipoproteins were 5.2+/-1.7 mmol/L, high-density lipoproteins were 1.2 +/- 0.4 mmol/L, triglycerides were 1.1+/-0.8 mmol/L, and body mass index z score was +0.4+/-1.0. A significant increase in body mass index z score (+0.032/year, P< .001) was observed. There was an associated significant increase in total cholesterol and triglyceride levels and decrease in high-density lipoprotein levels over time. Worsening adiposity is prevalent in hyperlipidemic children and adversely affects their lipid profiles and cardiovascular risk.

Association between physical activity, adiposity, and lipid abnormalities in children with familial hyperlipidemia.

BACKGROUND: The benefits of physical activity in children have been studied extensively; however, its role in children with familial hyperlipidemia (FH) is unknown. OBJECTIVE: To determine associations between physical activity, adiposity, and lipid profiles in children with FH. DESIGN: A physical activity questionnaire was completed by 147 children with FH. Correlations between activity levels, body mass index (BMI), and fasting lipid profiles were determined. RESULTS: The mean age of patients was 12.5+/-3.2 years with a mean total cholesterol of 6.17 mmol/l (238 mg/dl), low-density lipoprotein-cholesterol of 4.43 (171), high-density lipoprotein-cholesterol of 1.08 (42), and triglyceride levels of 1.51 (134). Patients had greater weight for height indices than normal, with a mean BMI z score of +0.90+/-1.30 SD (P<0.001 versus normal), and with 21% of the participants being more than 2 SD above normal. Higher BMI z scores significantly correlated with higher triglyceride levels (r=0.33; P<0.0001) and greater time spent in sedentary pursuits (r=0.24; P=0.004), in particular watching television (r=0.26; P=0.003). The increased time that other family members spent in physical activity significantly correlated with a lower BMI z score (r=-0.21; P=0.01) of the patient and greater time spent in physical activity (r=0.24; P=0.003). There was no association between patients' physical activity levels and lipid profile or BMI. CONCLUSION: Similar to the general population, children with FH are also at risk of becoming overweight. Increased adiposity significantly correlated with the greater sedentary activities of the patient, lower physical activities of the family, and higher triglyceride levels. Physical activity levels of the patient correlated with family activity levels.

Quality of life of children after repair of transposition of the great arteries.

BACKGROUND: We sought to assess quality of life of children with transposition of the great arteries (TGA) enrolled during transition in management strategy from atrial to arterial switch operation. METHODS AND RESULTS: Neonates enrolled by the Congenital Heart Surgeons Society in a prospective study of TGA between 1985 and 1989 were eligible. A Child Health Questionnaire was sent for completion by the child between February and June 2000. Data were compared with published normative values. Child Health Questionnaires were completed by 306 of 708 survivors at a mean age of 13+/-1 years. Diagnosis included TGA (n=202, 66%), TGA/ventricular septal defect (VSD) (n=84, 27%), and TGA/VSD/pulmonary stenosis (n=20, 7%). Repair type was arterial switch (n=189, 62%), atrial switch (n=105, 34%; Senning=58, Mustard=47), or Rastelli (n=12, 4%). Children with TGA scored significantly higher than published norms in all categories except self-esteem. TGA/VSD/pulmonary stenosis was associated with lower scores than TGA and TGA/VSD in physical functioning (P=0.002), general health perceptions (P=0.012), and mental health (P=0.048). Arterial repair was associated with higher scores than atrial or Rastelli repair in physical functioning (P<0.001), pain (P=0.004), mental health (P=0.019), self-esteem (P=0.004), and general health perceptions (P<0.001). By multivariable analyses, the most common independent factors impacting scores were repair type, perfusion parameters, and gender. CONCLUSIONS: Quality of life and health status as perceived by children 11 to 15 years after TGA repair is excellent when compared with published normative data and is better after arterial switch operation than after atrial repair.

A randomized crossover trial of combination pharmacologic therapy in children with familial hyperlipidemia.

We sought to determine whether a low-dose combination of a bile acid-binding resin (colestipol) with an hydroxymethylglutaryl CoA reductase inhibitor (pravastatin) would result in improved acceptability, compliance, and effectiveness in lipid-lowering compared with conventional therapy with a higher dose of a bile acid-binding resin only, with fewer side effects. We performed a randomized, crossover open-label clinical trial with two 18-wk medication regimens separated by an 8-wk washout period in 36 children and adolescents with familial hypercholesterolemia or familial combined hyperlipidemia. The regimens included colestipol 10 g/d (10 pills) versus a combination of colestipol 5 g/d with pravastatin 10 mg/d (six pills). All patients were maintained on a fat-reduced diet. Acceptability was better with the combination regimen. Mean compliance was similar and suboptimal (approximately 60%) with all medication components. Mean relative LDL cholesterol lowering was significantly better with the combination regimen (-17 +/- 16% versus -10 +/- 13%; p = 0.045), although insufficient to achieve recommended target values in the majority of patients on either regimen. Both regimens were equally free of adverse effects, with no important effect on chemistry or hematologic values. Patient-reported adverse effects were more common with the conventional-dose colestipol-only regimen. Compliance with medication regimens using the bile acid-binding resins is suboptimal, although combination with a low dose of a statin may result in better lipid lowering.

Impact of the characteristics of patients and their clinical management on outcomes in children with homozygous familial hypercholesterolemia.

OBJECTIVE: To relate clinical factors to the development of cardiovascular atherosclerosis for patients with homozygous familial hypercholesterolemia. BACKGROUND: Homozygous familial hypercholesterolemia is associated with extreme elevations in levels of cholesterol causing aggressive atherosclerosis. METHODS: We reviewed 10 children, 8 of whom were male, assessed at a single institution. We found that individual characteristics, levels of lipid, cardiovascular investigations, and management were related to the activity of low density lipoprotein receptors. RESULTS: Activity of low density lipoprotein receptors was defined as absent, being less than 2% of normal, in 4 patients who presented at the ages of 0.3, 1.4, 1.8, and 4.5 years, respectively. The activity was minimal, representing 5%-30% of normal, in another 4 patients presenting at the ages of 6.1, 9.6, 9.9, and 12 years, and was undetermined in 2 patients who presented at the ages of 3.5, and 12.1 years. Levels of low density lipoprotein cholesterol at presentation ranged from 12.2 to 24 millimoles per litre. Plasmapheresis was performed bi-weekly in 9 patients. Patients with absence of receptor activity were less likely to have a serial decrease in the levels of low density lipoprotein cholesterol prior to plasmapheresis, and one of these patients was increased to weekly plasmapheresis. In addition, they had more aggressive cardiovascular involvement of the coronary arteries, aortic valve and aorta, requiring surgical intervention at the age of 8 and 12 years in 2 patients, with sudden death at the age of 3.1 years in one patient. In contrast, 1 patient with minimal receptor activity had surgical intervention at the age of 16.6 years and another patient died suddenly at the age of 33.6 years. CONCLUSION: Complete cardiac assessment is recommended at presentation. The frequency of plasmapheresis should be adjusted according to the activity of low density lipoprotein receptors and the individual response of the patient.