Implementation of IT supported standardization of individualized hydrocortisone management for treatment of patients with adrenal insufficiency.

OBJECTIVES: Hydrocortisone stress dosing guidelines for children with adrenal insufficiency (AI) recommend a wide range of acceptable stress doses. This has led to variability in dosing recommendations resulting in confusion among endocrine, non-endocrine providers and patient families. This quality improvement project sought to standardize documentation and hydrocortisone stress dosing within our pediatric endocrine division to optimize communication regarding AI management. METHODS: Plan-Do-Study-Act (PDSA) cycle one aimed to address documentation of components important in AI management including body surface area (BSA), home daily dose, home stress dose, in-patient stress dose, procedure dose and crisis dose using a smart phrase within the electronic health record (EHR). To automate the process, PDSA cycle two introduced two smart buttons within the endocrine notes. PDSA cycle three focused on standardizing hydrocortisone stress doses. RESULTS: Initial documentation targets were met for all AI management components except for the crisis dose. The second target was only met for the home stress dose. Implementing the smart buttons aided in reaching the second target for home daily and home stress doses. Dose standardization targets were achieved in all categories except for the on-going crisis dose. A follow up survey after an in-service for non-endocrine providers showed increased knowledge of locating hydrocortisone stress dosing recommendations within the EHR. CONCLUSIONS: With the assistance of technology, this quality improvement project ultimately enhanced communication through the standardization of documentation and individualized hydrocortisone stress dosing for children with AI. Although not all secondary targets were met, there was meaningful improvement in documentation and stress dose standardization compliance.

Electronic Dashboard to Improve Outcomes in Pediatric Patients With Type 1 Diabetes Mellitus.

BACKGROUND AND OBJECTIVES: Incidence of type 1 diabetes mellitus (T1DM) is increasing, and these patients often have poor glycemic control. Electronic dashboards summating patient data have been shown to improve patient outcomes in other conditions. In addition, educating patients on T1DM has shown to improve glycated hemoglobin (A1C) levels. We hypothesized that using data from the electronic dashboard to monitor defined diabetes management activities to implement population-based interventions would improve patient outcomes. METHODS: Inclusion criteria included patients aged 0 to 18 years at Phoenix Children's Hospital with T1DM. Patient data were collected via the electronic dashboard, and both diabetes management activities (A1C, patient admissions, and visits to the emergency department) and patient outcomes (patient education, appointment compliance, follow-up after hospital admission) were analyzed. RESULTS: This study revealed that following implementation of the electronic dashboard, the percentage of patients receiving appropriate education increased from 48% to 80% (Z-score = 23.55, P < .0001), the percentage of patients attending the appropriate number of appointments increased from 50% to 68.2%, and the percentage of patients receiving follow-up care within 40 days after a hospital admission increased from 43% to 70%. The median A1C level decreased from 9.1% to 8.2% (Z-score = -6.74, P < .0001), and patient admissions and visits to the emergency department decreased by 20%. CONCLUSIONS: This study shows, with the implementation of an electronic dashboard, we were able to improve outcomes for our pediatric patients with T1DM. This tool can be used at other institutions to improve care and outcomes for pediatric patients with T1DM and other chronic conditions.

A quality improvement project for managing hypocalcemia after pediatric total thyroidectomy.

Objectives Hypocalcemia following total thyroidectomy (TT) is relatively common. It may result in significant morbidity, prolonged hospital stay, and increased costs. Treatment with intravenous (IV) calcium gluconate may also carry significant risks. In pediatrics, management consensus guidelines are lacking. Methods At Phoenix Children's Hospital, a team of pediatric endocrinologists, surgeons and otolaryngologists developed a clinical pathway for patients undergoing TT. It was a Quality Improvement (QI) project with the primary aim of decreasing IV calcium gluconate use from a baseline of 68% to less than 40% over 15 months. Secondary aims included reducing hypocalcemia and length of hospitalization. Interventions included sending weekly pathway reminder emails, starting pre-operative calcium, and pathway implementation into the electronic health record. Results Twenty-seven patients underwent TT over 15 months. IV calcium gluconate use dropped to 48%. Hypocalcemia and length of hospitalization were 96% and 52.7 h (range 21.1-115.7) respectively. Pathway adherence improved after targeted interventions. Eleven (73%) of the 15 patients whose post-operative parathyroid hormone (PTH) nadir was below 15 pg/mL required IV calcium gluconate vs. two (17%) out of 12 with levels above this threshold. Conclusions Standardizing care allowed for objective outcome analysis. We learned that post-operative serum PTH level was the main risk factor for requiring IV calcium gluconate. Implementing the pathway as a QI project allows for revisions based on outcomes, ultimately resulting in a pathway that best utilizes our infrastructure to optimize care. Other pediatric institutions may face similar challenges and can potentially learn from our experience.

Severe early onset obesity and hypopituitarism in a child with a novel SIM1 gene mutation.

SUMMARY: Single-minded homolog 1 (SIM1) is a transcription factor that plays a role in the development of both the hypothalamus and pituitary. SIM1 gene mutations are known to cause obesity in humans, and chromosomal deletions encompassing SIM1 and other genes necessary for pituitary development can cause a Prader-Willi-like syndrome with obesity and hypopituitarism. There have been no reported cases of hypopituitarism linked to a single SIM1 mutation. A 21-month-old male presented to endocrinology clinic with excessive weight gain and severe obesity. History was also notable for excessive drinking and urination. Endocrine workup revealed central hypothyroidism, partial diabetes insipidus, and central adrenal insufficiency. Genetic evaluation revealed a novel mutation in the SIM1 gene. No other genetic abnormalities to account for his obesity and hypopituitarism were identified. While we cannot definitively state this mutation is pathogenic, it is notable that SIM1 plays a role in the development of all three of the patient's affected hormone axes. He is now 6 years old and remains on treatment for his pituitary hormone deficiencies and continues to exhibit excessive weight gain despite lifestyle interventions. LEARNING POINTS: Mutations in SIM1 are a well-recognized cause of monogenic human obesity, and there have been case reports of Prader-Willi-like syndrome and hypopituitarism in patients with chromosomal deletions that contain the SIM1 gene. SIM1 is expressed during the development of the hypothalamus, specifically in neuroendocrine lineages that give rise to the hormones oxytocin, arginine vasopressin, thyrotropin-releasing hormone, corticotropin-releasing hormone, and somatostatin. Pituitary testing should be considered in patients with severe obesity and a known genetic abnormality affecting the SIM1 gene, particularly in the pediatric population.