Nancy L. Swigonski

A continuous quality improvement curriculum for residents: addressing core competency, improving systems.

Purpose. To describe the development, implementation, and evaluation of a residency continuous quality improvement (CQI) curriculum. Method. Forty-four medicine and pediatrics residents participated in a CQI curriculum. Resident-designed projects were scored for CQI construct skills using a grading tool. Pre- and post-tests evaluated knowledge, perceived knowledge, interest, and self-efficacy. Results. Differences between pre- and post-test perceived knowledge and self-efficacy were highly significant (p < .001). The mean project score was 81.7% (SD 8.3%). Higher knowledge was associated with higher ratings of self-efficacy. There was no correlation of measured knowledge with project score or interest. Conclusions. Resident education and learning in CQI served to produce innovative and creative improvement projects that demonstrated individual residents’ competency in practice-based learning and improvement.

Screening for Celiac Disease in Asymptomatic Children With Down Syndrome: Cost-effectiveness of Preventing Lymphoma

BACKGROUND. Studies demonstrate an increased prevalence of celiac disease in persons with Down syndrome, leading some organizations and authors to recommend universal screening of children with Down syndrome. However, many children with Down syndrome are asymptomatic, and the long-term implications of screening are unknown. The complication of celiac disease that leads to mortality in the general population is non-Hodgkins lymphomas. OBJECTIVES. The purpose of this research in asymptomatic children with Down syndrome was to (1) calculate the number needed to screen to prevent a single case of lymphoma and (2) present a cost-effectiveness study of screening. METHODS. We constructed a decision tree using probabilities derived from the published literature for Down syndrome or from the general population where Down syndrome-specific data were not available. Celiac disease was determined by serologic screening and confirmation with intestinal biopsy. Sensitivity analysis was used to alter probability estimates affecting the cost of preventing lymphoma. RESULTS. Using our baseline values, the no-screen strategy is dominant; that is, screening not only costs more but also results in fewer quality-adjusted life-years. A screening strategy costs more than

Prevalence, attitudes, and behaviors related to the use of nonsteroidal anti-inflammatory drugs (NSAIDs) in student athletes

500000 per life-year gained. Screening all asymptomatic children with Down syndrome for celiac disease costs almost

Outcomes of Children With Severe Bronchopulmonary Dysplasia Who Were Ventilator Dependent at Home

5 million to prevent a single case of lymphoma. CONCLUSION. These analyses do not support the cost-effectiveness of screening, and more data are needed before recommendations to screen asymptomatic children with Down syndrome for celiac disease can be made.

Hearing screening of high risk newborns.

PURPOSE To describe NSAID use among football players and examine differences in attitudes and behaviors associated with reports of daily use. Data are from 681 self-administered questionnaires. Independent variables were race (white), insurance status (private), attitudes (whether NSAIDs helped performance), and behaviors (decided independently vs. asked an adult when to take an NSAID, what type, or dose; use of NSAIDs to treat pain vs. block pain). Among NSAID users, independent variables were entered into a logistic regression to determine their effects on the probability of daily use. Athletes were male, 90% white, mean age 15.8 years, and 88% had private insurance. Four-hundred-fifty-two (75%) used NSAIDs in the past 3 months, and 90 (15%) were daily users with no significant difference in age, race, or insurance between users and nonusers. Those who perceived better performance owing to NSAIDs (adjusted odds ratio [AOR] = 2.4), who decided on their own when to take NSAIDs (AOR = 2.2), and who used NSAIDs prophylactically were more likely to use daily NSAIDs (AOR = 2.5) after controlling for other variables in the model. NSAID use in high school football players is highly prevalent. Although package inserts caution against chronic use, nearly one of seven players uses NSAIDs daily. Daily users were more likely to decide independently and to use NSAID prophylactically, raising issues about the need for adult supervision and education of athletes regarding side effects and appropriate use.

Measuring the Severity of Infantile Hemangiomas: Instrument Development and Reliability

OBJECTIVE: To describe the incidence and outcomes of children with chronic respiratory failure secondary to severe bronchopulmonary dysplasia (BPD) on chronic positive pressure ventilation (PPV) via tracheostomy at home. METHODS: We retrospectively reviewed medical charts of patients with severe BPD who were PPV dependent at home and who were enrolled in a university-affiliated home ventilator program between 1984 and 2010. We excluded patients with other comorbidities that could contribute to the development of chronic respiratory failure. We reported the incidence of these children in Indiana and cumulative incidences of survival, liberation from PPV, and decannulation. RESULTS: Over 27 years, 628 children were cared for in our home ventilator program. Of these, 102 patients met inclusion criteria: 83 (81.4%) were alive and 19 (18.6%) were deceased. Sixty-nine patients (67.6%) were liberated from PPV, and 97.1% of them were weaned before their fifth birthday, with a median age at liberation of 24 months (interquartile range, 19–33). Similarly, 60 patients (58.8%) were decannulated, of which 96.7% completed this process before their sixth birthday, with a median age at decannulation of 37.5 months (interquartile range, 31.5–45). The incidence of children with chronic respiratory failure secondary to BPD who were PPV-dependent at home in Indiana was 1.23 per 100 000 live births in 1984 and increased to 4.77 per 100 000 live births in 2010. CONCLUSIONS: Although extreme prematurity associated with severe BPD necessitating PPV at home carries significant risks of morbidity and mortality, successful liberation from mechanical ventilation and decannulation are likely to occur.

Assessing the Health, Functional Characteristics, and Health Needs of Youth Attending a Noncategorical Transition Support Program

Prospective screening of an extremely high risk group of 137 infants cared for in the Newborn Intensive Care Unit of the James Whitcomb Riley Hospital for Children was undertaken during 1983. Auditory brain stem responses (ABR) were obtained utilizing a clinical evoked potential system (Madsen 2250). Patients were selected for screening prior to discharge or transfer to the referring hospital on the basis of one or more of the following criteria: birth weight <1250 grams; birth weight <1500 grams and ventilatory support; significant depression at birth (Apgars <3 and 6 at 1 and 5 minutes, respectively); seizures, meningitis, and/or sepsis. Of the original 137 infants tested, 82 passed the initial ABR, 22 conditionally passed, and 34 failed. Eighty-two infants had follow-up behavioral and audiometric testing while 20 infants died and 35 were lost to follow-up. Four infants had severe sensorineural hearing loss, each of whom had failed the initial ABR. None of the infants who initially passed or conditionally passed the ABR had sensorineural hearing loss on followup testing. High risk factors for sensorineural hearing loss in the neonatal period included: intraventricular/periventricular hemorrhage, apnea, family history, major malformations of the head and neck, and possibly hyperbilirubinemia and congenital infection. No relationship of sensorineural hearing loss with very low birth weight, hyponatremia, infection, seizures, or medications was found. On the basis of these data, it is suggested that electrophysiologic hearing screening of a high risk population may be delayed until 3 to 6 months of age to improve specificity of testing.

Development of the Adult PedsQL™ Neurofibromatosis Type 1 Module: Initial Feasibility, Reliability and Validity

OBJECTIVES To develop instruments that measure the severity of infantile hemangiomas (Hemangioma Severity Scale [HSS]) and the complications of infantile hemangiomas for longitudinal use (Hemangioma Dynamic Complication Scale [HDCS]). DESIGN Instrument development and reliability study. SETTING Academic research. PARTICIPANTS The HSS and the HDCS were developed through the collaborative effort of members of the Hemangioma Investigator Group Research Core, an expert multi-institutional research group. After development of the scales, 13 pediatric dermatologists used the HSS to score 20 different hemangiomas. In addition, 12 pediatric dermatologists used the HDCS to score hemangioma-related complications for 24 clinical scenarios. Interrater and intrarater reliability was measured for both scales. MAIN OUTCOME MEASURES Interrater and intrarater reliability. RESULTS For the HSS, interrater reliability and intrarater reliability exceeded 99%. Similarly, the HDCS had a high rate of interrater agreement; for individual items, agreement among raters was 67% to 100%, with most clinical scenarios demonstrating greater than 90% agreement. Intrarater reliability was excellent for all individual items of the HDCS. CONCLUSION The HSS and the HDCS are reliable scales that can be used to measure the severity of infantile hemangiomas, including the severity of complications for longitudinal use.

Health-Related Quality of Life in Publicly Insured Children With Special Health Care Needs

PURPOSE To assess the health, functional characteristics, and health care service needs of youth and young adults with special health care needs attending a comprehensive, noncategorical transition program. METHODS A self-administered survey was developed from national health surveys and clinical experience to assess concepts identified as important for successful transition to adulthood. Surveys were mailed to 198 parents of youth and young adults with special health care needs attending the transition clinic. Parents were asked about the youths health, functional status, and health care services needed. The clinical database provided demographic and patient health characteristics. Results were compared against the 2005-2006 National Survey of Children with Special Health Care Needs. RESULTS Forty-four percent of surveys were returned. Average age of youth was 17.5 (11-22) years old and diagnoses included cerebral palsy (36%), spina bifida (10%), developmental delay or Down syndrome (17%), and autism (6%). Most youth needed assistance with personal care (69%) and routine needs (91%) and used assistive devices (59%). Compared with the 2005-2006 National Survey of Children with Special Health Care Needs, parents reported higher needs for all services except mental health care and tobacco or substance use counseling. Forty three percent reported at least one unmet health need. Few parents reported the need for counseling on substance use (1%), sexual health screening (16%), nutrition (34%), and exercise (41%). CONCLUSIONS Youth attending our transition program had more functional limitations, poorer reported health status, different diagnosis distribution, and higher levels of needed health services. Few parents identified needs for other recommended adolescent preventive services. Transition programs should assess patient health characteristics and service needs to design effective patient-centered services.

The health-related quality of life of children, adolescents, and young adults with neurofibromatosis type 1 and their families: Analysis of narratives

BackgroundNeurofibromatosis type 1 (NF1) is a common autosomal dominant genetic disorder with significant impact on health-related quality of life (HRQOL). Research in understanding the pathogenetic mechanisms of neurofibroma development has led to the use of new clinical trials for the treatment of NF1. One of the most important outcomes of a trial is improvement in quality of life, however, no condition specific HRQOL instrument for NF1 exists. The objective of this study was to develop an NF1 HRQOL instrument as a module of PedsQL™ and to test for its initial feasibility, internal consistency reliability and validity in adults with NF1.MethodsThe NF1 specific HRQOL instrument was developed using a standard method of PedsQL™ module development – literature review, focus group/semi-structured interviews, cognitive interviews and experts’ review of initial draft, pilot testing and field testing. Field testing involved 134 adults with NF1. Feasibility was measured by the percentage of missing responses, internal consistency reliability was measured with Cronbach’s alpha and validity was measured by the known-groups method.ResultsFeasibility, measured by the percentage of missing responses was 4.8% for all subscales on the adult version of the NF1-specific instrument. Internal consistency reliability for the Total Score (alpha =0.97) and subscale reliabilities ranging from 0.72 to 0.96 were acceptable for group comparisons. The PedsQL™ NF1 module distinguished between NF1 adults with excellent to very good, good, and fair to poor health status.ConclusionsThe results demonstrate the initial feasibility, reliability and validity of the PedsQL™ NF1 module in adult patients. The PedsQL™ NF1 Module can be used to understand the multidimensional nature of NF1 on the HRQOL patients with this disorder.