Getting ready for transition to adult care: Tool validation and multi-informant strategy using the Transition Readiness Assessment Questionnaire in pediatrics.

BACKGROUND: Transitioning from pediatric to adult healthcare can be challenging and lead to severe consequences if done suboptimally. The Transition Readiness Assessment Questionnaire (TRAQ) was developed to assess adolescent and young adult (AYA) patients' transition readiness. In this study, we aimed to (1) document the psychometric properties of the French-language version of the TRAQ (TRAQ-FR), (2) assess agreements and discrepancies between AYA patients' and their primary caregivers' TRAQ-FR scores, and (3) identify transition readiness contributors. METHODS: French-speaking AYA patients (n = 175) and primary caregivers (n = 168) were recruited from five clinics in a tertiary Canadian hospital and asked to complete the TRAQ-FR, the Pediatric Quality of Life Inventory™ 4.0 (PedsQL™ 4.0), and a sociodemographic questionnaire. The validity of the TRAQ-FR was assessed using confirmatory factor analyses (CFA). Agreements and discrepancies were evaluated using intraclass correlation coefficients and paired-sample t tests. Contributors of transition readiness were identified using regression analyses. RESULTS: The five-factor model of the TRAQ was supported, with the TRAQ-FR global scale showing good internal consistency for both AYA patients' and primary caregivers' scores (α = .85-.87). AYA patients and primary caregivers showed good absolute agreement on the TRAQ-FR global scale with AYA patients scoring higher than primary caregivers (ICC = .80; d = .25). AYA patients' age and sex were found to be contributors of transition readiness. CONCLUSIONS: The TRAQ-FR was found to have good psychometric properties when completed by both AYA patients and primary caregivers. Additional research is needed to explore the predictive validity and clinical use of the TRAQ-FR.

Becoming unique: A qualitative study of identity development of adolescent kidney recipients.

Teenagers who receive a renal organ transplant have to take up the double challenge of identity development, the primary task of adolescence, and of overcoming the complexities of their illness. Previous qualitative studies found that adolescents felt that the organ transplant and its treatments mainly defined who they are. The relationship to the donor can be a source of concern for some of them, especially for those who received from a parent and feel an obligation to be obedient and grateful. While donor parents are known to interpret their gesture as giving life for a second time, no research to date has described how this particular gesture may influence adolescent development. The present article aims to examine and describe identity development of teenage kidney recipients in a context of parental or deceased donation. We used a qualitative design involving individual interviews with 10 adolescents. Five of them received from a donor parent, five from a deceased donor. Data were analyzed using IPA. Results suggest that identity development is influenced by similar concerns for all adolescents regardless of donor source: body image, social relationships, and anxiety about the future. One aspect that stood out from the discourse of those who received from a parent was feelings of guilt towards the donor when engaging in behaviors that could comprise graft survival, which was a challenge for identity development. Receiving the transplant freed teens from the struggle of just managing their illness and was a catalyst for exploration and engagement, which are crucial for identity development.

Mixed-methods evaluation of a transition and young adult clinic for kidney transplant recipients.

The aims of the present study were to describe the experiences of kidney transplant patients attending a young adult clinic or a regular adult clinic, to explore similarities and differences between the groups, and to conduct an evaluation of the clinical and psychosocial outcomes of the young adult clinic, by comparing these outcomes to those of the regular adult clinic. A mixed-methods design combining qualitative and quantitative data was used. Empirically validated questionnaires measuring self-determination theory variables, quality of life, and adherence were distributed to all consenting patients attending the YAC (n = 17) and RAC (n = 16). Semi-structured interviews were conducted with a subsample of the first (n = 10) and second group (n = 8), and analyzed using thematic analysis. Clinical outcomes were retrieved from medical records. Descriptive, correlational, and comparative analyses were performed. We found clinically significant differences on tacrolimus blood levels variability, self-reported adherence, and physical quality of life. Small and medium effect sizes were detected. No statistical differences were found. Statistically significant correlations were found between self-determination theory variables and both physical quality of life and different measures of adherence. Four themes characterized patients' experiences: resilience; relational needs and the therapeutic alliance; quest for balance; and quest for normalcy. The young adult clinic seems to meet its initial objectives and to make a difference particularly in the early period post-transition, but over time what matters most for patients is therapeutic alliance. Mental health issues need to be better addressed, and special attention should be paid to youths transplanted in an adult setting.

Resolution of diagnosis and parental attitudes among parents of adolescent kidney recipients.

The diagnosis of a chronic illness is described as an upsetting event that implies an emotional crisis for parents. Some are able to come to terms with their child's chronic condition and feel a sense of resolution, but for others, strong negative emotions persist through time. The present study examines diagnostic resolution among parents of teenagers with a transplant. The design was qualitative and involved individual interviews with nine parents. Five were donor to their child. Data were analyzed according to the principles of IPA. Early reactions to the diagnosis suggest that parents with an unresolved status experienced trauma. Many factors seem to contribute to diagnostic resolution such as good communication between spouses, positive relationship with the medical staff, and being the parent donor. For all parents, concerns over adherence are central to their relationship with their youth. Results call attention to the support needs of all parents and particularly those with an unresolved status.

Conversion from twice- to once-daily tacrolimus in pediatric kidney recipients: a pharmacokinetic and bioequivalence study.

BACKGROUND: The objectives of this study were to investigate pharmacokinetic and pharmacogenetic parameters during the conversion on a 1:1 (mg:mg) basis from a twice-daily (Prograf) to once-daily (Advagraf) tacrolimus formulation in pediatric kidney transplant recipients. METHODS: Twenty-four-hour pharmacokinetic profiles were analyzed before and after conversion in 19 stable renal transplant recipients (age 7-19 years). Tacrolimus pharmacokinetic parameters [area under the concentration-time curve (AUC0-24), minimum whole-blood concentration (Cmin), maximum whole-blood concentration (Cmax), and time to achieve maximum whole-blood concentration (tmax)] were compared between Tac formulations and between CYP3A5 and MDR1 genotypes after dose normalization. RESULTS: Both AUC0-24 and Cmin decreased after conversion (223.3 to 197.5 ng.h/ml and 6.5 to 5.6 ng/ml; p = 0.03 and 0.01, respectively). However, the ratio of the least square means (LSM) for AUC0-24 was 90.8 %, with 90 % CI limits of 85.3 to 96.7 %, falling within bioequivalence limits. The CYP3A5 genotype influences the dose-normalized Cmin with the twice-daily formulation only. CONCLUSIONS: Both tacrolimus formulations are bioequivalent in pediatric renal recipients. However, we observed a decrease in AUC0-24 and Cmin after the conversion, requiring close pharmacokinetic monitoring during the conversion period.

Assessment of identity and quality of life in diabetic and renal transplant adolescents in comparison to healthy adolescents.

Identity development represents a central task of adolescence. Identity achievement is characterized by a coherent sense of who one is following a period of exploration and can help navigate the challenges of adulthood. This study examined identity within a quality of life (QOL) context in 85 adolescents with a renal transplant or with Type 1 diabetes in comparison to 90 healthy controls. Results revealed significant differences in ideological identity, with patients showing higher levels of diffusion and controls showing higher levels of foreclosure. No differences with respect to interpersonal identity, QOL, perceived control over the QOL domains, and perceived opportunities for growth and development were found. Future research should assess identity and QOL over a longer period of time to determine whether differences between chronically ill and healthy young adults can be detected.

Transition from Paediatric to adult health services: Aspirations and practices of human flourishing.

BACKGROUND: Transition from paediatric to adult care is challenging for youths with a chronic condition. Most transition programmes place high value in autonomy and independence. We undertook a qualitative study to: (1) identify the needs and aspirations of youths and (2) better understand the well-being and flourishing of youths. METHODS: Semi-structured interviews were conducted with youths, parents of youths and healthcare professionals recruited from four clinics. Thematic analysis focused on: (1) perceptions of transition; (2) key aspects of human flourishing during transition; and (3) salient concerns with respect to the transition and dimensions of human flourishing. RESULTS: 54 interviews were conducted. Perceptions of transition clustered around: (1) apprehension about adult care; (2) lack of clarity about the transition process; (3) emotional attachment to paediatric healthcare professionals; (4) the significance of the coinciding transition into adulthood. Fourteen salient concerns (e.g., Knowledge and information about the transition, Parental involvement in healthcare) were identified with corresponding recommendations. Salient concerns related to important dimensions of human flourishing (e.g., environmental mastery, autonomy). DISCUSSION AND CONCLUSION: The flourishing of youths is affected by suboptimal transition practices. We discuss the implications of our findings for environmental mastery, contextual autonomy, and the holistic and humanistic aspects of transition.

Psychological profile of adolescents with a kidney transplant.

To describe the psychological profile of renal transplant adolescents compared to healthy peers and to adolescents with CKD, three groups of adolescents aged 12-18 yr were selected: TX, CX, and adolescents with CKD. Psychiatric symptoms and disorders were evaluated through direct interviews (K-SADS-PL) and self-report questionnaires (YSR and CBCL). Forty TX (14 LRD and 26 DD transplant recipients), 40 CX and 20 CKD were included. Twelve of 40 (30%) TX, three of 20 (15%) CKD, and three of 40 (7.5%) CX had a history of learning difficulties (p = 0.03). Compared to CX, TX had lower total YSR competencies score (p = 0.028) and lower total CBCL competencies score (p = 0.003). Twenty-six of 40 (65%) TX, 12 of 20 (60%) CKD and 15 of 40 (37.5%) CX (p = 0.038) met DSM-IV diagnostic criteria for lifetime psychiatric disorder, with rates of depressive disorder of 35% among TX and CKD compared to 15.2% among CX (p = 0.043). Eight of 40 (20%) TX had a history of simple phobia. Nine of 40 (22.5%) TX met diagnostic criteria for ADHD as compared to one of 20 (5%) CKD and three of 40 (7.5%) CX. In the TX group, we found no significant differences in educational and psychiatric variables between LRD and DD. In conclusion, we found a high prevalence of psychiatric morbidity (depression, phobia, ADHD), educational impairment and social competence problems in the TX group. CKD scored in between TX and CX on most measures.

Prevention of CMV disease in pediatric kidney transplant recipients: evaluation of pp67 NASBA-based pre-emptive ganciclovir therapy combined with CMV hyperimmune globulin prophylaxis in high-risk patients.

A new prevention strategy for CMV infection was evaluated in our pediatric kidney transplant unit. This approach comprises a pre-emptive therapy, based upon the monitoring of CMV pp67 mRNA in whole blood by the qualitative NASBA, combined with prophylactic CMV-IG in high risk (R-/D+) children. Thirty-one kidney transplant children were followed for six months with serial measurements of CMV pp67 mRNA in the blood. The R-/D+ patients were given prophylactic CMV-IG for the first 16 wk after transplantation. I.v. ganciclovir was administered upon CMV detection by NASBA and was discontinued after two consecutive negative results. CMV infection, detected by NASBA, developed in 11 (35%) recipients: one (33%) of the R+/D- patients and 10 (72%) of the R-/D+ patients. CMV disease developed in 9.6% of the patients (3/31), exclusively in the R-/D+ group. These three patients presented concurrently with CMV viremia and disease. It is noteworthy that two of the three patients could not receive a complete course of CMV-IG, and one of the latter two subjects had been treated for acute rejection 15 days before CMV infection. Ganciclovir was given for the 11 cases of primary infection, and for three cases of relapsed CMV infection. pp67 NASBA-based pre-emptive ganciclovir therapy, combined with prophylactic CMV-IG in high-risk patients leads to a lower rate of CMV disease, as long as a complete course of CMV-IG has been administered and ganciclovir is given during the period of treatment for acute rejection in high-risk populations.

Hemolytic uremic syndrome: late renal injury and changing incidence-a single centre experience in Canada.

Aims. To assess trends in the incidence of pediatric diarrhea-associated hemolytic uremic syndrome (D(+) HUS) and document long-term renal sequelae. Methods. We conducted a retrospective cohort study of children with D(+) HUS admitted to a tertiary care pediatric hospital in Montreal, Canada, from 1976 to 2010. In 2010, we recontacted patients admitted before 2000. Results. Of 337 cases, median age at presentation was 3.01 years (range 0.4-14). Yearly incidence peaked in 1988 and 1994-95, returning to near-1977 levels since 2003. Twelve patients (3.6%) died and 19 (5.6%) experienced long-term renal failure. Almost half (47%) The patients required dialysis. Need for dialysis was the best predictor of renal sequelae, accounting for 100% of severe complications. Of children followed ≥1 year (n = 199, mean follow-up 8.20 ± 6.78 years), 19 had severe and 18 mild-to-moderate kidney injury, a total sequelae rate, of 18.6%. Ten years or more after-HUS (n = 85, mean follow-up 15.4 ± 5.32 years), 8 (9.4%) patients demonstrated serious complications and 22 (25.9%) mild-to-moderate, including 14 (16%) microalbuminuria: total sequelae, 35.3%. Conclusions. Patients with D(+) HUS should be monitored at least 5 years, including microalbuminuria testing, especially if dialysis was required. The cause of the declining incidence of D(+)HUS is elusive. However, conceivably, improved public health education may have played an important role in the prevention of food-borne disease.

[Physician-patient communication in nephrology]. / La communication médecin-malade en néphrologie.

Training the nephrologist-to-be to physician-patient communication (PPC) has several objectives: to guide his/her history taking in order to make a diagnosis, of course, but also to help him/her establish a long-lasting relationship, inform and motivate the patient, and prepare him/her to break bad news and to deal with end-of-life issues. PPC rests on specific strategies and on the ethical grounds of a human encounter, the success of which expresses itself in care. An effective PPC offers many benefits to the patient, but also to the clinician. Talking about renal failure and dialysis, about kidney transplantation, and about withdrawing or not starting therapy: these are a few of the communicational challenges awaiting the nephrologist in training, mostly with patients requiring long-term follow-up. PPC also requires specific skills in the field of pediatric nephrology, where one deals alternately with children, adolescents and parents. Whatever the personal abilities of the nephrologist-to-be, PPC is a competency that can be learned and improved, for the benefit of the patient, and also of the physician.

[A specialized and integrated outpatient clinic for the care of children with chronic kidney disease: experience of CHU Sainte-Justine]. / Organisation des soins spécialisés et intégrés en ambulatoire pour la prise en charge de la maladie rénale chronique en pédiatrie: expérience du CHU Sainte-Justine.

The management and optimal care for the pediatric patient with chronic kidney disease requires attention not only to medical management, but also special focus on the psychosocial and developmental factors of children which is complicated by the presence of other disease-related complications. In recent years, specialized chronic kidney disease and predialysis clinics have been set up to facilitate and improve the quality of care of these patients with a multidisciplinary organisation and coordinated management approaches of a renal team. We present our experience in establishing such a renal management clinic named "Prévoir" for children with chronic kidney disease at Sainte-Justine Hospital.

Efficacy of plasma therapy in atypical hemolytic uremic syndrome with complement factor H mutations.

Atypical hemolytic uremic syndrome (aHUS) frequently results in end-stage renal failure and can be lethal. Several studies have established an association between quantitative or qualitative abnormalities in complement factor H and aHUS. Although plasma infusion and exchange are often advocated, guidelines have yet to be established. Long-term outcome for patients under treatment is still unknown. We describe a patient who, at 7 months of age, presented with aHUS associated with combined de novo complement factor H mutations (S1191L and V1197A) on the same allele. Laboratory investigations showed normal levels of complements C4, C3 and factor H. Plasma exchanges and large-dose infusion therapy resulted in a resolution of hemolysis and recovery of renal function. Three recurrences were successfully treated by intensification of the plasma infusion treatment to intervals of 2 or 3 days. This patient showed good response to large doses of plasma infusions and her condition remained stable for 30 months with weekly plasma infusions (30 ml/kg). Long-term tolerance and efficacy of such intensive plasma therapy are still unknown. Reported secondary failure of plasma therapy in factor H deficiency warrants the search for alternative therapeutic approaches.

Duration of extracorporeal therapy in acute maple syrup urine disease: a kinetic model.

Maple syrup urine disease (MSUD, MIM 248600) can be complicated by metabolic crises necessitating extracorporeal removal therapy (ECRT). Since leucine levels are usually not immediately available during therapy, an accurate kinetic model of leucine plasma levels during removal would be useful to establish the duration of ECRT. Such a kinetic model is available for neonates undergoing continuous ECRT (CECRT) with a leucine clearance>or=35 ml min-1 1.73 m-2. The current study tests the validity of this model in older children. Plasma leucine levels were obtained from eleven ECRT sessions [seven CECRT and four intermittent hemodialysis (HDi) sessions] in seven children aged 1-14 years. No hemodynamic instability or neurological complications were observed during treatment. HDi provided a higher leucine clearance and required shorter sessions than CECRT (5.4+/-0.6 vs. 17.1+/-6.0 h). All patients regained precrisis neurological status except for one patient who had severe neurological damage (severe cerebral edema) at the time of dialysis and subsequently died despite efficient leucine removal. A leucine clearance>or=50 ml min-1 1.73 m-2 is required to obtain a kinetic model similar to that reported in neonates, both with CECRT and HDi. This model should be helpful in predicting the duration of therapy needed to attain desired leucine levels.

Circulating granulocyte colony-stimulating factor, C-X-C, and C-C chemokines in children with Escherichia coli O157:H7 associated hemolytic uremic syndrome.

Leukocytes are implicated in the pathogenesis of diarrhea-associated hemolytic uremic syndrome (D(+) HUS). We hypothesized that increased circulating levels of granulocyte colony-stimulating factor (G-CSF), and the chemokines epithelial cell-derived neutrophil-activating protein-78 (ENA-78), growth related oncogen-alpha (GRO-alpha), macrophage inflammatory protein-1beta (MIP-1beta), and monocyte chemotactic protein-1 (MCP-1) are related to the severity of illness in Escherichia coli O157:H7 infections. We compared the circulating concentrations of these mediators in the course of E. coli O157:H7 enteritis, hemorrhagic colitis, and HUS. Our data show that, on admission, children with HUS presented 10-fold abnormally increased levels of G-CSF (p < 0.007), 3-fold increased MIP-1beta concentrations (p < 0.001), and 2-fold lower values of ENA-78 (p < 0.0001). One week later, a further 4-fold decrease in ENA-78 concentration was noted (p < 0.0001) whereas MIP-1beta levels returned to normal. HUS patients requiring peritoneal dialysis showed 6-fold increased G-CSF (p < 0.001) and 5-fold decreased ENA-78 (p < 0.001) levels. On admission, children with uncomplicated O157:H7 hemorrhagic colitis (HC) presented 3-fold abnormally increased concentrations of G-CSF (p < 0.001) and MIP-1beta (p < 0.0001). Those with O157:H7 enteritis but no bloody stools showed higher rates of abnormal GRO-alpha, MIP-1beta, and MCP-1 measurements than children with O157:H7 HC or HUS: GRO-alpha (50% enteritis, 36% HC, 17% HUS; p < 0.06), MIP-1beta (40% enteritis, 22% HC, 11% HUS; p < 0.02), MCP-1 (77% enteritis, 20% HC, 18% HUS; p < 0.0001). The data indicates that GRO-alpha, MIP-1beta, and MCP-1 are produced during E. coli O157:H7 enteritis, whether or not HC or HUS develops. Our data suggest that children with O157:H7 associated HUS may present abnormally increased circulating levels of G-CSF and decreased ENA-78 concentrations. The mechanisms responsible for leukocytes recruitment in O157:H7 infections are unclear and await further studies.