Medical and developmental impact of transition from subcutaneous insulin to oral glyburide in a 15-yr-old boy with neonatal diabetes mellitus and intermediate DEND syndrome: extending the age of KCNJ11 mutation testing in neonatal DM.

Mutations in the KCNJ11 gene, which encodes the Kir6.2 subunit of the ATP-sensitive potassium channel, often result in neonatal diabetes. Patients with this mutation have been successfully transitioned from insulin to sulfonylurea (SU) therapy without compromise in their glycemic control. Among patients with neonatal diabetes due to KCNJ11 mutations, approximately 25% have neurological findings including developmental delay, motor dysfunction, and epilepsy, known as DEND syndrome. There have been rare cases of juvenile patients with intermediate DEND syndrome (iDEND) reporting variable improvement in neurological function following transition from insulin to SU treatment. We describe the response to glyburide in a 15-yr-old boy with severe global developmental delays resulting from the KCNJ11 mutation V59M. The patient was discovered to have diabetes mellitus at 11.5 months of age, making this the oldest age at diagnosis of a KCNJ11 mutation-related case of neonatal diabetes. Because consensus has been to screen patients for this mutation only if younger than 6 months at the time of diagnosis, we suggest that all patients under the age of 12 months at diagnosis should receive genetic testing for monogenic causes of diabetes.

Assessing regimen adherence of adolescents with type 1 diabetes.

OBJECTIVE: The purpose of this study is to evaluate two updated measures of diabetes regimen adherence. The Diabetes Self-Management Profile (DSMP) is a widely used, structured interview. Limitations include a substantial interviewer and respondent time burden and the need for well-trained interviewers to use appropriate prompts and score the open-ended responses. The Diabetes Behavior Rating Scale (DBRS) is a self-administered, fixed-choice survey. RESEARCH DESIGN AND METHODS: Both measures were administered to 146 youth with type 1 diabetes (aged 11-18 years) and their parents. Items were added to the DBRS to allow for both flexible and conventional regimens, and the DSMP was modified to use standardized wording across items, accommodate flexible regimens, and permit administration by nonmedical interviewers. RESULTS: Both measures had good evidence of internal consistency (for the DSMP: parent 0.75 and youth 0.70; for the DBRS: parent 0.84 and youth 0.84). Scores on the DSMP and the DBRS were significantly related (r = 0.72 for parents and 0.74 for youth). There was moderate agreement between parent and youth (DSMP, r = 0.51; DBRS, r = 0.48). The measures were correlated with HbA1c for both parent (DSMP, r = -0.35; DBRS, r = -0.35) and youth (DSMP, r = -0.36; DBRS, r = -0.34) reports. CONCLUSIONS: Both measures exhibit good psychometric properties and good criterion validity but varied in terms of respondent and interviewer burden, issues that should be considered in selecting assessment procedures.

Self-efficacy, outcome expectations, and diabetes self-management in adolescents with type 1 diabetes.

The goal of this research was to develop and evaluate measures of adolescent diabetes management self-efficacy and outcome expectations that reflect developmentally relevant, situation-specific challenges to current diabetes regimens. Self-efficacy for diabetes management, expected outcomes of adherence, adherence to the diabetes regimen, and glycemic control were assessed in 168 adolescents (ages 10-16 years) with type 1 diabetes. Factor analyses indicated a single scale for self-efficacy and two distinct factors representing positive and negative outcome expectations. Reliability and predictive validity of the new scales were supported. In regression analyses, self-efficacy and the interaction of self-efficacy with expectations of positive outcomes were significantly associated with diabetes self-management adherence and glycemic control in older adolescents. The effect of self-efficacy was greatest when adolescents had stronger beliefs in the beneficial outcomes of adherence. These brief measures can be used to identify youths at risk of poor diabetes self-management. Interventions targeting self-efficacy may lead to improved diabetes self-management.

Safety and effectiveness of insulin pump therapy in children and adolescents with type 1 diabetes.

OBJECTIVE: To evaluate the safety and effectiveness of insulin pump therapy in children and adolescents with type 1 diabetes. RESEARCH DESIGN AND METHODS: All 95 patients who began insulin pump therapy at Johns Hopkins Hospital between January 1990 and December 2000 were included in the study. The mean age was 12.0 years (range 4-18), and 29% of the patients were <10 years old. Data were obtained by chart review beginning 6-12 months before pump start. The median duration of follow-up was 28 months. RESULTS: There was a small but significant decrease in HbA(1c) at 3-6 months after pump start (7.7 vs. 7.5%; P = 0.03). HbA(1c) levels then gradually increased and remained elevated after 1 year of follow-up; however, this association was confounded by age and diabetes duration, both of which were associated with higher HbA(1c) levels. After adjusting for duration and age, mean HbA(1c) after pump start was significantly lower than before pump start (7.7 vs. 8.1%; P < 0.001). The number of medical complications (diabetic ketoacidosis, emergency department visits) was similar before and after pump start. There were fewer hypoglycemic events after pump start (12 vs. 17, rate ratio 0.46, 95% CI 0.21-1.01). CONCLUSIONS: This study suggests that pump therapy is safe and effective in selected children and adolescents with type 1 diabetes.

Perspectives from before and after the pediatric to adult care transition: a mixed-methods study in type 1 diabetes.

OBJECTIVE: Among the many milestones of adolescence and young adulthood, transferring from pediatric to adult care is a significant transition for those with type 1 diabetes. The aim of this study was to understand the concerns, expectations, preferences, and experiences of pretransition adolescents and parents and posttransition young adults. RESEARCH DESIGN AND METHODS: Participants completed questionnaires and responded to open-ended qualitative questions regarding self-management, self-efficacy, and their expectations and experiences with pediatric and adult care providers across the transition process. RESULTS: At a mean age of 16.1 years, most pretransition adolescents had not yet discussed transferring care with their parents or doctors. Although many posttransition young adults reported positive, supportive interactions, several described challenges locating or establishing a relationship with an adult diabetes care provider. Qualitative themes emerged related to the anticipated timing of transfer, early preparation for transition, the desire for developmentally appropriate interactions with providers, the maintenance of family and social support, and strategies for coordinating care between pediatric and adult care providers. CONCLUSIONS: Standardizing transition preparation programs in pediatric care and introducing transition-oriented clinics for late adolescents and young adults prior to adult care may help address patients' preferences and common transfer-related challenges.

Long-term maintenance of treatment outcomes: diabetes personal trainer intervention for youth with type 1 diabetes.

OBJECTIVE: To describe a 2-year follow-up of A1C outcomes of a self-regulation intervention for youth with type 1 diabetes. RESEARCH DESIGN AND METHODS: A total of 81 youths with type 1 diabetes ages 11-16 years were randomized to usual care versus a diabetes personal trainer intervention consisting of six self-monitoring, goal-setting, and problem-solving sessions with trained nonprofessionals. A1C data were obtained from medical records 2 years postintervention, and ANCOVA adjusting for age and baseline A1C was conducted. RESULTS: An overall intervention effect on A1C (8.93% control vs. 8.43% intervention; F = 8.24, P = 0.05) and a significant intervention-by-age interaction (F = 9.88; P = 0.002) were observed, indicating a greater effect among older than younger youths. Subgroup analyses demonstrated no treatment group differences among pre-/early adolescents but a significant difference in A1C among middle adolescents (9.61% control vs. 8.46% intervention; F = 7.20, P = 0.011). CONCLUSIONS: Findings indicate maintenance of intervention effects on A1C observed at 1-year follow-up.

Diabetes personal trainer outcomes: short-term and 1-year outcomes of a diabetes personal trainer intervention among youth with type 1 diabetes.

OBJECTIVE: To assess the social-cognitive, behavioral, and physiological outcomes of a self-management intervention for youth with type 1 diabetes. RESEARCH DESIGN AND METHODS: A total of 81 youth with type 1 diabetes aged 11-16 years were randomized to usual care versus a "diabetes personal trainer" intervention, consisting of six self-monitoring, goal-setting, and problem-solving sessions with trained nonprofessionals. Assessments were completed at baseline and multiple follow-up intervals. A1C data were obtained from medical records. ANCOVA adjusting for age and baseline values were conducted for each outcome. RESULTS: At both short-term and 1-year follow-up, there was a trend for an overall intervention effect on A1C (short-term F = 3.71, P = 0.06; 1-year F = 3.79, P = 0.06) and a significant intervention-by-age interaction, indicating a great effect among older than younger youth (short-term F = 4.78, P = 0.03; 1-year F = 4.53, P = 0.04). Subgroup analyses demonstrated no treatment group difference among younger youth but a significant difference among the older youth. No treatment group differences in parent or youth report of adherence were observed. CONCLUSIONS: The diabetes personal trainer intervention demonstrated significant effects in A1C among middle adolescents.

Identification of distinct self-management styles of adolescents with type 1 diabetes.

OBJECTIVE: Using a profile-based approach to the assessment of diabetes management, the purpose of this study was to identify and evaluate an empirically derived classification system of distinct self-management styles. RESEARCH DESIGN AND METHODS: Youth with type 1 diabetes (n = 156) aged 10-16 years and their parents were administered a modified version of the Diabetes Self-Management Profile (DSMP). Cluster analyses were performed independently on parent and youth report forms to categorize patients based on their patterns of scores in five diabetes self-management areas. RESULTS: Cluster analyses revealed three self-management styles that emerged from both youth and parent report: a "methodical style" (33%) with an emphasis on careful meal planning and correct insulin administration; an "adaptive style" (46%), characterized by high rates of blood glucose testing, exercise, and self-care adjustments; and an "inadequate style" (21%) with moderate rates of self-care adjustments and otherwise low DSMP scores. Convergence between parent and youth report classifications was moderate (Cohen's kappa = 0.47, P < 0.0001). A1C was 1.6% higher in the inadequate style group than in both other groups (P < 0.0001), and the classification significantly accounted for differences in A1C above what was explained by an overall DSMP score. CONCLUSIONS: The findings provide support for recognizing subgroups of patients with unique multidimensional patterns of self-care behaviors. The assessment of self-management styles may prove useful for customized treatments that are targeted directly to the patients' needs.