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.

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.

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.

Impaired glucose tolerance, body mass index and respiratory function in patients with cystic fibrosis: A systematic review.

OBJECTIVES: This systematic review aims to evaluate body mass index (BMI) and forced expiratory volume in one second (FEV1 ) in patients with cystic fibrosis (CF) diagnosed with impaired glucose tolerance (IGT) compared to those with normal glucose tolerance (NGT). The significance of this topic stems from concern that individuals with CF and IGT may have an overall worse clinical status as indicated by BMI and FEV1 . DATA SOURCE: An exhaustive literature search was completed between July 2017 and September 2017 using PubMed, CINAHL, Web of Science, Dissertations & Theses, PsycINFO, and Open Grey. Studies were limited to human subjects with CF. There were no restrictions on publication date, study design or language. STUDY SELECTION: Included studies examined BMI and FEV1 as outcome measures in individuals with CF and IGT compared to those with NGT. After screening for inclusion criteria, 12 observational studies met the specified conditions. RESULTS: Two studies showed a significantly worse BMI and FEV1 in subjects with CF and IGT compared to those with NGT. The remaining 10 studies found no significant associations with BMI and FEV1 in subjects with CF and IGT compared to NGT. CONCLUSION: Although this review does not prove cause and effect, BMI and FEV1 are important outcome measures in patients with CF. Considering the inconclusive findings, practitioners should individualize care for this patient population. Additional research should focus on clinical status and interventions/treatment for individuals with CF and IGT.