Medicine and Health Sciences Commons^™

Increasing Annual Eye Exam Completion For Children With Cystic Fibrosis Receiving Cftr Modulators, Paula Capel, Claire Elson, Stephanie Duehlmeyer, Jessica Haynes, Amanda Conway, Christopher M. Oermann

Posters

Background: CFTR modulators are life-changing therapy for people with CF. During preclinical studies of ivacaftor, cataracts were identified in juvenile rat pups. Subsequently, noncongenital cataracts have been reported in children receiving CFTR modulator therapy [1], so baseline and annual slit lamp eye examinations for cataract development are recommended for children with CF (aged <18). At Children’s Mercy Kansas City (CMKC), our standard practice is to conduct baseline and annual ophthalmologic examinations to assess for cataracts in children with CF prescribed CFTR modulator therapy, but there was no formal process for tracking adherence to this standard and ensuring that examinations were completed. A quality improvement project was undertaken in May 2022 to determine the rate of completion of annual eye exams and increase adherence to our annual monitoring standard.

Methods: To improve adherence to annual monitoring standards, a quality improvement taskforce including CF care team nurses and pharmacists was developed. A database was used to document baseline and follow-up eye examinations. A standardized communication process was developed and deployed that included an eye exam brochure (Figure …

Evaluating The Accuracy And Quality Of The Information On Pulmonary Rehabilitation Videos Shared On Youtube Amidst The Covid-19 Pandemic, Payaum Fotovat-Ahmadi, Bilal M. Ali, Bhavinkumar Dalal

Posters

YouTube is a visual library that is growing at incredible pace, especially with the rise of COVID-19 pandemic. Pulmonary rehabilitation refers to the rehabilitation treatment of patients with chronic pulmonary diseases. YouTube has made it possible to practice remote rehabilitation, the effectiveness of which have been proven non-inferior to traditional approaches.

Evaluation Of Phenobarbital In The Treatment Of Alcohol Withdrawal In The Intensive Care Unit, Sienna J. Ringgenberg, Vishal K. Patel

Posters

Alcohol withdrawal syndrome (AWS) is typically managed using the Clinical Institute Withdrawal Assessment (CIWA) protocol which includes benzodiazepines, supportive care, and close clinical monitoring. Alternatives to benzodiazepines such as phenobarbital provide several advantages including less frequent dosing, longer tapering off of doses, and additional glutamate inhibition. The purpose of this study is to evaluate the effectiveness and potential benefit to using phenobarbital in addition to CIWA protocol in the treatment of AWS at Beaumont, Royal Oak.

Impact Of Structured Reporting Template On The Quality Of Hrct Radiology Reports For Interstitial Lung Disease, Han G. Ngo, Girish B. Nair, Sayf Al-Katib

Posters

This QI study compared the completeness of HRCT radiology reports before and after the implementation of a disease-specific structured reporting template for suspected cases of interstitial lung disease (ILD).

A Study To Compare Baseline Functional Residual Capacity And Forced Vital Capacity As Predictors Of Mortality And Hospitalization In A Cohort Of Mild To Moderate Interstitial Lung Disease, Olga R. Gomez Rojas, Ehsun Naeem, Filip Ionescu, Edward Castillo, Girish B. Nair

Posters

Forced Vital Capacity (FVC) is a common parameter used to assess disease progression in patients with Interstitial lung disease (ILD) and acts as a surrogate for mortality. Previous studies suggest Functional Residual Capacity (FRC) may be an earlier predictor of disease progression in ILD. In this study, we compared predictive ability of baseline FRC to FVC to predict one-year-mortality and respiratory related hospitalization in patients with mild to moderate ILD

A Rare Case Of Suspected Generalized Arterial Calcification Of Infancy (Gaci) In An Infant Presenting With Respiratory Failure In An Infant Presenting With Respiratory Failure And Arterial Calcification, Lilah Melzer, Catharine Kral, Bonnie Sullivan, Eric T. Rush, Erin Khan

Posters

No abstract provided.

Recurrent Primary Spontaneous Pneumothorax Masquerading As A Congenital Pulmonary Airway Malformation In A Young Female, Chandra Swanson, Justin Sobrino, Tolulope A. Oyetunji Md Mph, Erin Khan

Posters

Introduction: Spontaneous pneumothoraxes in children are uncommon, may be idiopathic or associated with underlying pulmonary disease, and can present management challenges. We present a 12-year-old female with recurrent right sided spontaneous pneumothorax in the setting of an asymptomatic SARS-Co-V2 (COVID) infection and imaging concerning for congenital lobar overinflation (CLO) versus congenital pulmonary airway malformation (CPAM), prompting surgical intervention. Case: A 12-year-old pre-menstrual female with remote history of eczema, asthma, and environmental allergies presented from an outside facility with four-days of progressive chest pain and dyspnea on exertion and diagnosis of right-sided spontaneous pneumothorax, improving after pigtail chest tube placement. Physical …

Optimizing Oral Glucose Tolerance Test Completion At A Pediatric Cystic Fibrosis Care Center: A 10-Year Continuing Quality Improvement Effort, Christopher M. Oermann, Paula Capel, Jessica Haynes, Michelle Fischer, Jill Kohmetscher

Posters

Background: Cystic fibrosis–related diabetes (CFRD) is a common comorbidity among people with CF (PwCF). It is associated with weight loss, protein catabolism, lung function decline, and increased mortality. Nutritional status and pulmonary function begin to decline in PwCF several years before the diagnosis of CFRD. Early CFRD detection and aggressive insulin therapy have been shown to reduce the mortality gap between PwCF who have CFRD and those who do not. The Clinical Care Guidelines for Cystic Fibrosis–Related Diabetes recommend annual screening for people with CF starting at age 10 [1]. Methods: In 2011, team members at Children’s Mercy Kansas City …

Vancomycin Auc Monitoring In Individuals With Cystic Fibrosis At A Pediatric Institution, Christopher M. Oermann, Stephanie Duehlmeyer, Ellen Meier, Claire Elson

Posters

Vancomycin AUC monitoring in individuals with cystic fibrosis at a pediatric institution S. Duehlmeyer1, C. Oermann1, E. Meier1, E. Elson1. 1Pulmonology, Children’s Mercy Kansas City, Kansas City, USA Background: Antibiotic therapy is essential for the treatment of cystic fibrosis (CF) lung infections. Methicillin-resistant Staphylococcus aureus (MRSA) infects 20% to 25% of people with CF (PwCF) and is associated with increased morbidity. Treatment of pulmonary exacerbations (PEs) often requires hospitalization including respiratory treatments and intravenous (IV) antimicrobials. IV vancomycin, which is commonly used for MRSA infections, requires serum concentration monitoring to ensure efficacy and minimize toxicity. Previous guidelines recommended trough concentrations …

Covid-19 Vaccination In Individuals With Cystic Fibrosis At A Pediatric Cystic Fibrosis Center, Christopher M. Oermann, Claire Elson, Ellen Meier, Paula Capel, Jessica Haynes, Michelle Fischer, Stephanie Duehlmeyer

Posters

Background: Observational data suggest that most people with cystic fibrosis (PwCF) who contract COVID-19 have outcomes similar to those of the general population, although PwCF who are older or have CF-related diabetes, poor lung function, or a history of lung transplantation may be at greater risk for more severe disease. Therefore, the CF Foundation advocates for PwCF to discuss vaccination with care teams. At present, the FDA has authorized emergency use of 3 COVID-19 vaccines. ACIP/CDC guidance allows each state to determine vaccine distribution based on an individual’s exposure and risk for severe disease. This study describes the attitudes of …

Development And Interim Analysis Of A Cystic Fibrosis-Specific Antibiogram, Claire Elson, Ellen Meier, Douglas Swanson, Rangaraj Selvarangan, Megan Gripka, Christopher M. Oermann

Posters

Antimicrobial therapy is essential to treat cystic fibrosis (CF) lung infections. Empiric antimicrobial selection is generally based on previous culture information and, if available, an institution-specific antibiogram (ABGM). Most institutions antibiograms exclude cultures from individuals with cystic fibrosis, imposing challenges with empiric antimicrobial selection and monitoring susceptibility patterns over time. A cystic fibrosis-specific antibiogram may help drive population-specific antimicrobial selection and improve antimicrobial stewardship.

Development Of Drive-Through And Mail-In Systems For Obtaining Surveillance Respiratory Specimens In A Pediatric Cystic Fibrosis Center, Ellen Meier, Paula Capel, Christopher M. Oermann

Posters

Routine surveillance cultures are an essential part of health maintenance for people with cystic fibrosis (CF). The Cystic Fibrosis Foundations (CFF) recommends that respiratory cultures be obtained every three months. The CF Care Center at Children’s Mercy Kansas City (CMKC) also obtains respiratory cultures when there is an acute change in respiratory symptoms. The COVID-29 pandemic resulted in ambulatory clinic closures and an inability to obtain surveillance respiratory cultures. A creative solution for obtaining respiratory cultures was needed during the pandemic.

Standardizing Aminoglycoside Induced Ototoxicity Monitoring, Claire Elson, Christopher M. Oermann, Stephanie Duehlmeyer, Ellen Meier

Posters

Aminoglycoside (AG) antibiotics are essential for the treatment of cystic fibrosis (CF) lung infections. Pseudomonas aeruginosa. Nontuberculous mycobacteria. Monitoring is critical secondary to potential nephrotoxicity and ototoxicity. Children’s Mercy Kansas City (CMKC), standardized nephrotoxicity monitoring in 2016. Observed variable ototoxicity monitoring practices. Prevalence of ototoxicity, 2016 CFF Patient Registry, 1.1% in pediatric patients (less than or equal to 18 years). 2.2% in pediatric and adult population. National Institute of Deafness and Other Communication Disorders, 13% in US population equal to or less than 12 years old. A standardized AG induced ototoxicity monitoring algorithm (AIOA) was developed and implemented at CMKC …

A Multifaceted Approach To Improve Quarterly Visit Rates At A Pediatric Cystic Fibrosis Care Center, Paula Capel, Jessica Banks, Micaela Mckenna, Ashley Andrews, Christopher M. Oermann

Posters

Standard quality improvement methodology was used to improve quarterly visit rates among cystic fibrosis patients at Children's Mercy Kansas City Cystic Fibrosis Center.

• A family-centered, team-based approach was adopted
• A change in culture led to sustained improvement

Improved quarterly visit rates should drive improvement in outcomes including pulmonary function and nutritional status.

Improving Pneumococcal Vaccination Rates In High Risk Patients Across Multiple Specialty Divisions, Rachel Moran, Julia G. Harris, Claire Olsen, Rana El Feghaly, Liset Olarte, Douglas Blowey, Luke A. Harris

Posters

Pediatric patients with deficient immune systems or certain chronic medical conditions have an increased risk of acquiring invasive pneumococcal disease.

The 23-valent pneumococcal (PPSV23) vaccine provides protection against 23 pneumococcal serotypes and is recommended for patients aged 2 years or older who are high-risk for invasive pneumococcal disease.

Unfortunately, many high-risk patients are not properly vaccinated due to lack of provider knowledge or understanding of accountability between primary care and specialty providers.

The goal of this project was to improve PPSV23 vaccination rates by 10-20% across multiple Children's Mercy Kansas City specialty divisions.

Dehydrated Hereditary Stomatocytosis Causing Sever Ascites Leading To Pulmonary Hypoplasia And Respiratory Insufficiency In A Neonate, Michael D. Mcdowell Jr, Christopher M. Oermann

Posters

Care Presentation: EM is a 32.5-week EGA infant who was born with sever congenital ascites (following repeated prenatal paracentesis) identified on prenatal ultrasound. Initial chest-abdomen imaging is shown in Figure 1. He required CPAP at delivery but was weaned of high flow nasal cannula with intermittent episodes of prolonged intubation during repeat paracentesis. Paracentesis demonstrated simple ascites rather than chylous fluid. Lymphangiograms demonstrated transient lymphatic leakage with resolution. Genetic evaluation revealed a pathogenic mutation in the PIEZO 1 gene for autosomal dominant DHS. This disorder had reduced penetrance and variable expressivity and symptoms range for absence of clinical features to …

Implementation Of A Guideline-Based Nontuberculous Mycobacteria Management Algorithm, Claire Elson, Ellen Meier, Douglas Swanson, Christopher M. Oermann

Posters

Nontuberculous mycobacterial (NTM) disease is a challenge to manage in patients with cystic fibrosis (CF). Diagnosis of NTM pulmonary disease is complex. Effective treatment requires long term, multi-drug therapy delivered by several routes. Consensus recommendations published in 2016 were developed to guide CF providers in NTM screening, diagnosis and management. Primary Outcomes. Children’s Mercy-Kansas City (CMKC) developed an NTM working group to facilitate implementation of standardized NTM management. Methods. NTM working group: Pulmonologist (CF Center Director), Infectious Diseases specialist, nurse practitioner (CF Center Coordinator), and pharmacist. Sought expertise from the Director of Microbiology Laboratory regarding susceptibility testing. Developed NTM Management …

Sustainability And Outcomes Of A Standardized Aminoglycoside Induced Ototoxicity Monitoring Algorithm, Claire Elson, Christopher M. Oermann, Michelle Weltman, Ellen Meier

Posters

No abstract provided.

Improving Pneumococcal Polysaccharide Vaccination In Children With Cystic Fibrosis, Adam Van Mason, Wendy Estrellado-Cruz, Kristi Williams, Ellen Meier, Elizabeth Elson, Stephanie Duehlmeyer, Paula Capel, Jessica Banks, Christopher M. Oermann

Posters

No abstract provided.

Implementation Of A Multi-Institution, Multistate Cystic Fibrosis Transition Program, Christopher M. Oermann, D Polineni, A Andrews, A Moog, Paula Capel, T Bomstedt, Ellen Meier, J Mermis

Posters

Background: Children’s Mercy-Kansas City (CMKC) is an independent children’s hospital in Kansas City, MO. Its CF Care Center (CFCC) provides care for 250 patients. The University of Kansas Medical Center (KUMC) is an academic medical center in Kansas City, KS. Its CFCC includes adult/pediatric programs (230/50 patients). Interactions between CMKC and KUMC were minimal prior to 2013. CMKC provided care through adulthood despite CF Foundation mandates requiring transition of patients aged 18-21 years. Transition only occurred per patient request. Methods: The CMKC CFCC underwent restructuring and staff expansion in 2013-2014. KUMC underwent staff expansion in 2012, adding a nurse, respiratory …