Lung T1 MRI assessments in children with mild cystic fibrosis lung disease.

RATIONALE: Lung T1 MRI is a potential method to assess cystic fibrosis (CF) lung disease that is safe, quick, and widely available, but there are no data in children with mild CF lung disease. OBJECTIVE: Assess the ability of lung T1 MRI to detect abnormalities in children with mild CF lung disease. METHODS: We performed T1 MRI, multiple breath washout (MBW), chest computed tomography (CT), and spirometry in a cohort of 45 children with mild CF lung disease (6-11 years of age). MAIN RESULTS: Despite mean normal ppFEV1 values, the majority of children with CF in this study exhibited mild lung disease evident in lung clearance index (LCI) measured by MBW, chest CT Brody scores, and percent normal lung perfusion (%NLP) measured by T1 MRI. The %NLP correlated with chest CT Brody scores, as did LCI, but %NLP and LCI did not correlate with each other. Analysis of the Brody subscores showed that %NLP and LCI largely correlated with different Brody subscores. CONCLUSIONS: T1 MRI can detect mild CF lung disease in children and correlates with chest CT findings. The %NLP from T1 MRI and LCI correlate with different chest CT Brody subscores, suggesting they provide complementary information about CF lung disease.

Clinical and genetic risk factors for cystic fibrosis-related liver disease in Egyptian CF children: A single-center experience.

BACKGROUND: Cystic fibrosis (CF) is an autosomal recessive disease. It affects multiple organ systems, including the liver, leading to CF-related liver disease (CFLD). It was noted that CFLD in Egyptian children with CF is more common than in non-Egyptian people with CF (pwCF). This study aimed to determine the incidence of CFLD and the potential risk factors for developing CFLD in Egyptian children. The correlation between CFLD and the various genotypes prevalent in Egyptian CF children will be discussed. In addition, comparison of CFLD in Egyptian and non-Egyptian CF patients will be presented. METHODS: This cross-sectional study included 50 pwCF from Ain Sham University's Pediatric Pulmonology Clinic in Children's Hospital, Cairo, Egypt. The sweat chloride test and genetic studies were done at the time of diagnosis. Additionally, all subjects underwent detailed history taking, laboratory investigations, clinical assessment, and pelvic abdominal ultrasound for evaluation of hepatic involvement. RESULTS: One-third of the Egyptian children with CF were found to have liver disease. The following independent risk factors for developing CFLD were identified as: male sex, severe genetic mutation (class I and II), long duration of CF disease, early onset of the CF, pancreatic insufficiency, as well as history of meconium ileus. In addition, diabetes mellitus and severe lung disease were proven to significantly increase the risk of developing CFLD. CONCLUSION: CFLD is common in Egyptian pwCF. CFLD's risk factors are similar to other reported research from other countries in the region.

Complications and Practice Variation in the Use of Peripherally Inserted Central Venous Catheters in People With Cystic Fibrosis: The Prospective Study of Peripherally Inserted Venous Catheters in People With Cystic Fibrosis Study.

BACKGROUND: Peripherally inserted central catheters (PICCs) are used commonly to administer antibiotics to people with cystic fibrosis (CF), but their use can be complicated by venous thrombosis and catheter occlusion. RESEARCH QUESTION: Which participant-, catheter-, and catheter management-level attributes are associated with increased risk of complications of PICCs among people with CF? STUDY DESIGN AND METHODS: This was a prospective observational study of adults and children with CF who received PICCs at 10 CF care centers in the United States. The primary end point was defined as occlusion of the catheter resulting in unplanned removal, symptomatic venous thrombosis in the extremity containing the catheter, or both. Three categories of composite secondary outcomes were identified: difficult line placement, local soft tissue or skin reactions, and catheter malfunction. Data specific to the participant, catheter placement, and catheter management were collected in a centralized database. Risk factors for primary and secondary outcomes were analyzed by multivariate logistic regression. RESULTS: Between June 2018 and July 2021, 157 adults and 103 children older than 6 years with CF had 375 PICCs placed. Patients underwent 4,828 catheter-days of observation. Of the 375 PICCs, 334 (89%) were ≤ 4.5 F, 342 (91%) were single lumen, and 366 (98%) were placed using ultrasound guidance. The primary outcome occurred in 15 PICCs for an event rate of 3.11 per 1,000 catheter-days. No cases of catheter-related bloodstream infection occurred. Other secondary outcomes developed in 147 of 375 catheters (39%). Despite evidence of practice variation, no risk factors for the primary outcome and few risk factors for secondary outcomes were identified. INTERPRETATION: This study affirmed the safety of contemporary approaches to inserting and using PICCs in people with CF. Given the low rate of complications in this study, observations may reflect a widespread shift to selecting smaller-diameter PICCs and using ultrasound to guide their placement.

Implementation of standardized cystic fibrosis care algorithm to improve the center data-quality improvement project international collaboration.

BACKGROUND: A collaboration between the University of Michigan (U of M) Cystic Fibrosis Center (CFC) and Marmara University (MU) CFC was initiated to improve the health status of people with cystic fibrosis (pwCF) at MU through implementing Quality Improvement (QI) initiatives. The main aim was to improve lung function in children with FEV1pp <80. The secondary aim was to assess the changes in health related quality of life. METHODS: Included in the project were pwCF who received cystic fibrosis (CF) care at the MU CFC and were 6-18 years of age with an FEV1pp <80. Flow charts were created and a standardized CF care algorithm was implemented. Weekly case review were done to develop individualized treatment plans. Appropriate intervention was applied and patient data were assessed at baseline, 3, 6, 9 and 12 months. The Cystic Fibrosis Revised Questionnaire (CFQ-R) was completed. RESULTS: 55 pwCF were included (mean age:11.8 ± 3.3 years). Mean FEV1pp (SD) at baseline, 6 and 12 month was 63.7 (14.6), 66.9 (16.6), 70.4 (19.2), respectively, with a relative increase of 5.0% in 6 months (p:0.002) and 10.5% in 12 months compared to baseline (p<0.001). Physical functioning, eating problems and respiratory symptoms domains of the CFQ-R questionnaire were improved at the end of the one year for 6-13 (p = 0.024, p = 0.009, p = 0.002) and 13-18 year olds (p = 0.013, p = 0.002, p = 0.038). CONCLUSION: There was significant improvement in pwCF with FEV1<80%pp after implementing this QI project. The processes and assessments used can be adopted by other low-middle income countries to improve similar measures.

Improvements in body mass index of children with cystic fibrosis following implementation of a standardized nutritional algorithm: A quality improvement project.

BACKGROUND: A collaboration between the University of Michigan (UM) Cystic Fibrosis Center (CFC) and Marmara University (MU) CFC was initiated in MU through conducting Quality Improvement projects (QIP). The global aim was to improve nutritional status of children with CF (cwCF), with a specific aim to increase the mean BMI percentile (BMIp) for cwCF by 10 percentile points in 12 months. METHODS: Body mass index (BMI) percentiles of cwCF were categorized as: nutritionally adequate (BMIp ≥ 50%); at risk (BMIp 25%-49%); urgently at risk (BMIp 10%-25%); critically at risk (BMIp < 10%). Appropriate interventions were made according to BMIp category every three months. Forced expiratory volume in one-second percent predicted (FEV1pp), and health-related quality of life (HRQoL) were evaluated. RESULTS: One hundred and eight-two cwCF with a mean age of 9.1 ± 4.3 years were included in the project. Baseline BMIp increased from 25.6 to 37.2 at the 12th month (p < 0.001). In the critically at-risk group BMIp increased from 3.6 to 20.5 (p < 0.001), in the urgently at risk group from 15.9 to 30.8 (p < 0.001), in the at risk group from 37.0 to 44.2 (p < 0.079) and in the nutritionally adequate group the increase was from 66.8 to 69.5 (p < 0.301). FEV1pp also improved significantly, from 81.3 ± 20.6 to 85.9 ± 20.8 (p < 0.001). Physical functioning, eating problems, and respiratory symptoms domains of the HRQoL evaluation improved (p < 0.05). CONCLUSION: This project has led to significant improvements in BMIp, FEV1pp and HRQoL of cwCF; similar projects could easily be implemented by centers in other developing countries.

Clearing up the smoke: Physical and mental health considerations regarding cannabis use in adolescents with cystic fibrosis.

The cannabis plant is the most used federally illegal drug in the United States and is widely used by adolescents. Cannabis has complex effects on the body and mind. All health professionals who take care of adolescents with cystic fibrosis (CF) should be aware of the factors impacting cannabis use in CF. Given limited evidence regarding the benefits of cannabis and the significant risks, clinicians have the responsibility to identify risk of cannabis use early, counsel patients about the risks, provide a safe space for ongoing conversations about cannabis use in the context of CF care, and deliver evidence-based interventions.

Missed Cystic Fibrosis Newborn Screening Cases due to Immunoreactive Trypsinogen Levels below Program Cutoffs: A National Survey of Risk Factors.

Testing immunoreactive trypsinogen (IRT) is the first step in cystic fibrosis (CF) newborn screening. While high IRT is associated with CF, some cases are missed. This survey aimed to find factors associated with missed CF cases due to IRT levels below program cutoffs. Twenty-nine states responded to a U.S-wide survey and 13 supplied program-related data for low IRT false screen negative cases (CFFN) and CF true screen positive cases (CFTP) for analysis. Rates of missed CF cases and odds ratios were derived for each factor in CFFNs, and two CFFN subgroups, IRT above ("high") and below ("low") the CFFN median (39 ng/mL) compared to CFTPs for this entire sample set. Factors associated with "high" CFFN subgroup were Black race, higher IRT cutoff, fixed IRT cutoff, genotypes without two known CF-causing variants, and meconium ileus. Factors associated with "low" CFFN subgroup were older age at specimen collection, Saturday birth, hotter season of newborn dried blood spot collection, maximum ≥ 3 days laboratories could be closed, preterm birth, and formula feeding newborns. Lowering IRT cutoffs may reduce "high" IRT CFFNs. Addressing hospital and laboratory factors (like training staff in collection of blood spots, using insulated containers during transport and reducing consecutive days screening laboratories are closed) may reduce "low" IRT CFFNs.

Transitions of Care in Cystic Fibrosis.

The development of formal transition models emerged to reduce variability in care, including cystic fibrosis (CF) responsibility, independence, self-care, and education (RISE), which provides a standardized transition program, including knowledge assessments, self-management checklists, and milestones for people with CF. Despite these interventions, the current landscape of health care transition (HCT) remains suboptimal, and additional focused attention on HCT is necessary. Standardization of assessment tools to gauge the efficacy of transfer from pediatric to adult care is a high priority. Such tools should incorporate both clinical and patient-centered outcomes to provide a comprehensive picture of progress and deficiencies of the HCT process.

Collaboration between two CF centers; one in USA and one in Turkey before and during CoV2 pandemic.

To address the discrepancy in the quality of care and outcomes between cystic fibrosis centers (CFCs) in high-income countries and limited resources countries (LRCs), a collaboration between our team at the University of Michigan CFC (UMCFC) and a CF center in Turkey (Marmara University CFC [MUCFC], Istanbul) was established. The collaboration included evaluation of all aspects of care and initiation of quality improvement (QI) measures. Teaching and implementing QI tools has led to start of improvement in MUCFC care. Close monitoring and sharing resources like UMCFC algorithms, protocols, and QI processes were done.

Five cases of missed cystic fibrosis heterozygous mutations identified after a positive newborn screen on a sibling.

In Michigan (MI), NBS for CF was started in October 2007 using the IRT/DNA protocol. In 2016, a component of the Hologic molecular test kit used by the MI NBS lab was recalled (40 CF mutation 2nd tier test). This recall had a major impact on states using the Hologic test kits in their NBS programs. Michigan specimens were sent to another state's NBS Lab for 2nd tier testing using the Luminex 60 mutation test kit until the Luminex kit could be procured and validated in MI. In this report, we present five cases born during this time period. These cases were initially reported out as having normal NBS results for CF but had heterozygous F508 del (c.1521_1523delCTT) mutations later identified. Of the five cases, one was diagnosed with CF (Case1), one with CF related metabolic syndrome (CRMS), and the other three were carriers.

Risk factors for obstructive sleep apnea in cystic fibrosis.

INTRODUCTION: Despite emerging data that suggest a high frequency and severity of obstructive sleep apnea (OSA) among patients with cystic fibrosis (CF), few of them are referred for polysomnography. Little is known about which patients with CF are at increased risk for OSA and which sleep symptoms merit investigation. METHODS: A single-center retrospective analysis of clinical and polysomnographic data from 2009, January 1 to October 31, 2020 in referred children and adults with CF. RESULTS: Among 74 patients (42 children, 32 adults) with CF, 39 (53%) had OSA. No age or sex differences emerged in OSA frequency. Mean apnea-hypopnea index (AHI) was higher among overweight/obese adults (n = 16) as compared with adults of normal weight or underweight (11.4 vs. 6.2; p = 0.005). Adults with (n = 10) versus without a crowded oropharynx had 13.0 times greater odds of OSA (95% confidence interval (CI): 1.4, 121.4; p = 0.02). Children with (n = 24) versus without tonsillar hypertrophy had a higher risk for OSA (OR = 5.2; 95% CI: 1.4, 19.8; p = 0.02), as did children with (n = 10) versus without symptomatic chronic sinusitis (OR = 5.8; 95% CI: 1.1, 32.1; p = 0.04). Neither snoring, excessive daytime sleepiness, nor lung disease severity were associated with OSA. CONCLUSION: Key risk factors for OSA may differ between children and adults with CF: upper airway pathology appears important in children and overweight/obesity or a crowded oropharynx in adults. Given the lack of sensitivity of snoring, daytime sleepiness, and lung disease severity, detection of OSA may require a low threshold for polysomnographic assessment in this vulnerable population.

The effects of nebulizer hygiene training on the practices of cystic fibrosis patients and caregivers.

BACKGROUND: Nebulizers can be contaminated with microorganisms and may be a source of infection in the lower airways in patients with cystic fibrosis (CF). OBJECTIVE: Primary aim of this study was to determine the level of knowledge regarding nebulizer hygiene and adherence to CF foundation infection prevention and control (IPC) measures of CF patients in our center. We also evaluated the effect of a standardized training program on nebulizer cleaning and disinfection practises with pre and posttest. METHODS: Caregivers of 173 CF patients followed at Marmara University CF Center filled a questionnaire (pretest) regarding nebulizer hygiene and received didactic education including pictures and videos based on the cystic fibrosis foundation (CFF) IPC guidelines, patients were also provided educational materials. Posttest was performed 1-3 months after the education session. RESULTS: Following standardized training, usage of appropriate methods according to CFF IPC guidelines improved significantly. Frequency of nebulizer cleaning after each use increased from 58.4% to 78% (p < .01) and disinfection frequency after each/daily usage increased from 33.6% to 75.7% (p < .01). Additionally, methods of cleaning and storage of the nebulizer, also improved significantly (p < .01, p < .01). CONCLUSION: Education was highly effective to increase the rate of proper practices for nebulizer hygiene. The necessity of cleaning, disinfection, careful drying, correct storage of the nebulizer parts, and changing the nebulizer equipment within recommended time should be emphasized to CF families regularly.

Screening of depression and anxiety in adolescents with cystic fibrosis and caregivers in Turkey by PHQ-9 and GAD-7 questionnaires.

BACKGROUND: Depression and anxiety symptoms in patients with cystic fibrosis (CF) and their caregivers are 2-3 times higher than in the normal population. This study aims to evaluate the frequency and severity of depression and anxiety symptoms and to determine possible risk factors in CF patients and their mother and/or fathers at Marmara University CF center. METHODS: The study included 132 CF patients who were followed up at our CF center. Patient Health Questionnaire (PHQ-9) and the Generalized Anxiety Disorder Questionnaire (GAD-7) were used to screen depression and anxiety. The questionnaires were completed by 50 CF patients (aged 12-17 years) and 132 parents of patients (aged 0-17 years). RESULTS: While moderate to severe depressive symptoms were seen in 26% of patients, 33.7% of mothers and 14.6% of fathers; moderate to severe anxiety were present in 18%, 21.8% and 8.5%, respectively. None of the demographic characteristics was identified as a predictor of depression or anxiety. GAD-7 scores have shown a higher prevalence of anxiety in mothers of patients with chronic methicillin-resistant Staphylococcus aureus (p = .034). Additionally, hospitalization in the last 12 months was significantly correlated with higher PHQ-9 scores in fathers (p = .043). Analysis of patients' adherence to medical treatment and airway clearance showed higher depression and anxiety in mothers of the nonadherent group (p = .002). CONCLUSION: Depression and anxiety were common in CF patients and their parents. These results illustrate the importance of depression/anxiety screening and psychosocial support for the CF patient and their parents.

Assessing the Utility of an Outpatient Exercise Program for Children With Cystic Fibrosis: A Quality Improvement Project.

Children with cystic fibrosis (CF) (cwCF) suffer from inadequate weight gain, failure to thrive, and muscle weakness. The latter may be secondary to disuse atrophy (muscle wasting or reduction in muscle size associated with reduced physical activity and inflammation). Handgrip strength (HGS) is a reliable surrogate for muscle strength and lean body mass. Data from our CF center have shown an association between low HGS and forced expiratory volume in 1 s (FEV1) in cwCF. High-intensity interval training (HIIT) improves physical strength. Therefore, we devised a project to assess implementing a HIIT exercise program in the home setting, in order to improve physical strength in cwCF with HGS ≤ 50th percentile. Patients were instructed to complete 3-5 sessions of HIIT exercises per week. Wilcoxon matched-pairs signed-rank tests were used to compare HGS, FEV1, and body mass index (BMI) percentile at baseline and at a follow-up clinic visit. Follow-up was limited due to the COVID pandemic. Adherence to the HIIT regimen was poor. A total of twenty-nine cwCF participated in the program. However, a total of 13 individuals reported some form of moderate activity at follow-up and therefore constituted our final study population. There was a statistically significant increase in absolute grip strength (AGS) and FEV1 for these individuals. Even though the home HIIT protocol was not followed, the project demonstrated that moderate physical activity in cwCF can lead to significant improvement in HGS and overall physical strength.

Development of an Interdisciplinary Telehealth Care Model in a Pediatric Cystic Fibrosis Center.

Background: People with cystic fibrosis (PCF) have unique physical and emotional needs, which are best met through interdisciplinary care (IDC). In the midst of the pandemic, our center aimed to begin a telehealth care model with an objective to increase successful care visits from baseline of 0-95% by June 26, 2020, including meeting cystic fibrosis (CF) care standards of IDC visits that are coproduced through agenda setting with PCF. Methods: Shifting IDC for pediatric CF patients to telehealth was part of a quality improvement initiative. Our team used asynchronous virtual visits (VVs), with the IDC team members' VVs done on different days than the physician's. Multiple plan-do-study-act cycles were completed to address evolving telehealth needs, including IDC team member flow logistics, communication with PCF, and surveying PCF for the patient perspective. Rates of IDC and agenda setting were measured from March 16, 2020 to June 26, 2020. Results: IDC VVs were at 86% in March 2020 with fluctuations until mid-May when we reached 100% and achieved sustainability. Agenda setting was reached at 100% and maintained. With continued effort, an additional 46.3% of PCF registered for the patient portal, totaling 90.6% with access. Our survey revealed 100% of PCF were able to see IDC team members that they needed to, with 87% "extremely satisfied" and 13% "somewhat satisfied" with their telehealth experience. Conclusions: Successful telehealth in pediatric CF IDC can be achieved through continuous communication, optimal utilization of available technologies, and may help foster unique opportunities to help improve health outcomes.

Addressing nutritional failure in a cystic fibrosis patient using nutritional and psychological intervention.

BACKGROUND: Malnutrition is associated with Cystic Fibrosis (CF) disease progression. That contributes to decreased lung function, poor quality of life, and increased morbidity and mortality. Appetite stimulants (AS) have been used to help increase caloric intake. When caloric intake is inadequate, gastrostomy tube (GT) feeding is offered and has shown to improve weight gain. In some patients, both AS and GT placement might not be adequate to insure weight gain. That could be due to the underlying psychopathological issues like anxiety and depression that are common in CF. AIM: To address malnutrition in CF. METHOD: Since increasing oral caloric intake was inadequate to improve weight gain. AS and GT feeding were discussed and offered. AS was started, but was not used regularly. However, GT was still needed to improve weight gain. Because of patient's hesitations, ongoing psychological counseling was done to help patient accept the intervention. RESULTS: Addressing patient's depression and anxiety and the failure to gain adequate weight at the same time helped improving patient's nutritional status. CONCLUSION: The presented case report demonstrates the improvement in weight gain and body mass index in a CF patient after addressing both the patient's psychological needs and increasing caloric intake through gastrostomy tube feeding.

The Association of Grip Strength, Body Mass Index, and Lung Function in Youth with Cystic Fibrosis.

Compared with body mass index (BMI), lean body mass and fat-free mass are strongly associated with lung function in children and adolescents with cystic fibrosis (CF). Methods of measuring body composition in youth with CF are often unreliable, expensive, or not clinically feasible. Grip strength (GS), a measure of muscle function, is used as a surrogate for muscle mass and is an indicator of nutrition status. This quality improvement project explored the feasibility of measuring GS in medically stable youth with CF, aged 6-21 years. A total 361 GS measurements were performed by using a digital hand dynamometer in youth from a single CF center. Using reference tables that were created for this project by merging data from the 2011-2012 and 2013-2014 National Health and Nutrition Examination Surveys, youth with CF were found to be weaker than age- and gender-matched peers, even when controlled for differences in size. A positive association (P < .001) was found between GS percentile and lung function, as measured by forced expiratory volume in 1 second percent predicted (FEV1pp). Statistical analysis revealed that both BMI percentile and absolute GS (AGS) percentile were positively associated with FEV1pp and with each other, primarily at the lower levels of BMI percentile (<50%) and AGS percentile (<50%). GS may provide a reliable, less expensive, and clinically feasible alternative to body composition measurements in monitoring nutrition status in youth with CF, especially in youth whose BMI is in the <50th percentile.

Sleep-disordered breathing in cystic fibrosis.

INTRODUCTION: Cystic fibrosis (CF) is a life-shortening, genetic disease that affects approximately 30,000 Americans. Although patients frequently report snoring, mouth breathing, and insomnia, the extent to which sleep-disordered breathing (SDB) may underlie these complaints remains unknown. METHODS: Single-center retrospective review of polysomnography results from referred patients with and without CF individually-matched (1:2) for age, gender, race, and body mass index (BMI). RESULTS: Mean ages were 8.0 ± 5.2 (sd) and 35.9 ± 12.9 years, among 29 children and 23 adults with CF respectively. The CF and non-CF groups were well-matched in age and BMI. Subjects with vs. without CF had three times greater odds of moderate-severe SDB (apnea-hypopnea index (AHI) ≥ 5 in children, ≥ 15 in adults) (p = 0.01). Nocturnal oxygen saturation nadir (Minimum SpO2) was lower among CF vs. non-CF groups (p = 0.002). For every 1-unit increase in AHI, the decline in Minimum SpO2 was larger for subjects with vs. without CF (p = 0.05). In subjects with CF, forced expiratory volume in 1 s percent predicted (FEV1 PPD) was associated with Minimum SpO2 (Pearson r = 0.68, p < 0.0001) but not AHI (r = -0.19, p = 0.27). For every 1-unit increase in AHI, magnitude of decline in Minimum SpO2 was larger for those with low vs. normal FEV1 PPD (p = 0.01). CONCLUSION: Severity of SDB may be worse among referred patients with vs. without CF. The SDB may modify the relationship between CF lung disease and nocturnal hypoxemia. Markers of lung disease severity including lung function do not predict SDB severity, suggesting the need for routine polysomnography to screen for this sleep disorder.

Quality improvement initiative to improve pulmonary function in pediatric cystic fibrosis patients.

BACKGROUND: Our Cystic Fibrosis (CF) Center initiated a Quality Improvement (QI) project in November 2017 with the goal of improving our patients' forced expiratory volume in 1 second (FEV1) percent predicted (pp) and continued for 1 year. Our specific aim was to increase the relative mean FEV1 pp by 5% in 12 months for CF patients 6 to 21 years old with FEV1 ≤ 80 pp. METHODS: We identified patients with FEV1 ≤ 80 pp, developed cause and effect diagrams (fishbones) to identify contributing factors to FEV1 ≤ 80 pp, and created flowcharts to address barriers. The barriers to adherence that may result in FEV1 ≤ 80 pp were studied using a fishbone. A standardized approach across providers was implemented to individualize care for each patient. Each discipline developed a flowchart to address barriers to improving FEV1. RESULTS: Forty patients were identified (43% male). Their mean age was 16.8 years (range 8.2-21.5 years). Mean FEV1 pp at baseline was 58.6 (range 30-80). The fishbone identified needs for continuing education for patients/families, and providing a treatment plan at each clinic visit. After 6 months of implementation, patients had an improvement in mean FEV1 pp by 6.4% (CI, 0.4%-12.9%). At 12 months, mean FEV1 pp had improved by 14% (CI, 6.5%-21.4%), which exceeded our goal of 5%. CONCLUSION: Through this ongoing project, team members, patients, and families partnered to improve lung function in pediatric CF patients. Flowcharts facilitated a standardized approach across providers to develop individualized treatment plans for patients, which resulted in improved lung function.