Outcomes

The mission of the Cystic Fibrosis Foundation is to cure cystic fibrosis and to provide all people with the disease the opportunity to lead full, productive lives by funding research and drug development, promoting individualized treatment, and ensuring access to high quality, specialized care.

The Cystic Fibrosis Foundation's Patient Registry captures a broad range of data on the health of those living with CF, providing critical information to help advance many important initiatives - from improvements in the quality of CF care and the development of care guidelines to the design of clinical trials to test new CF therapies.

The below information are excerpts from the 2018 Patient Registry Report for our center, with the information regarding lung function and nutritional status highlighted.

Pulmonary function is an important clinical health indicator for people with CF. FEV1 is a measure of lung function. It is the forced exhale volume of air in the first second of an exhaled breath after a maximal inhalation. It is shown as a percent predicted, based on the FEV1 of healthy, non-smoking people of the same age, height, gender and race. The below graphs show our center's median FEV1 percent predicted, displayed in red, ranking for 2018 in patients 6 - 12 of age and 13 - 17 years of age and also our center's trend from 2009 - 2018 according to the CFF Patient Registry.

Our center is ranked 5th in the nation for lung function in patients 6 - 12 years old among all centers
Our center is ranked 9th in the nation for lung function in patients 13 - 17 years old among all centers
Our center is ranked 1st in the nation for lung function in patients 6 - 17 years old in centers with >100 patients.

Nutritional Outcomes are a key indicator of health in cystic fibrosis patients. Research suggests that maintaining a healthy weight is also important to lung function. Children and teens with CF need adequate nutrition to grow and stay healthy. Patients less than 24 months of age are assessed using the WHO Nutritional Outcomes. Children and adolescents age 2 to 19 years of age are assessed using BMI percentile. BMI percentile matches a child's BMI to other children in the United States of the same age and gender. The below graphs show our center's ranking for 2018, displayed in red, and also our center's trend from 2009 - 2018 according to the CFF Patient Registry.

Current QI Projects

Bridging the Gap Between Inpatient and Outpatient Cystic Fibrosis Respiratory Care

The goal is to improve the quality and consistency of respiratory care provided to our CF patients regardless of patient setting and to improve patient satisfaction. This project was developed based on feedback from our patients and families. Through educational skills day, pre and post Education Surveys, and educational “cheat sheets” for easy access to information, we plan to provide all respiratory therapists with consistent care information and guidelines to ensure proper and consistent respiratory care is provided to all CF patients.

iPad Educational Video Project

The goal for our educational video project was to develop videos on various topics for patients, parents, and caregivers to access while either in clinic (via iPads in each clinic room) or from the website to learn more about lung function testing, exercise and airway clearance techniques (ACT’s). While written instruction is helpful and informative, the importance of actual testing and therapy demonstration through video by a healthcare provider and patient is vital for understanding of the proper technique. Patients and families receive accurate, relevant information directly from their healthcare team. Time together in clinic can be limited, so developing and sharing condition-specific videos can improve clinic work flow, make time between patient and family and provider more personal and improve the overall healthcare experience.

Respiratory Therapy Education Follow-up for Newly Diagnosed Cystic Fibrosis (CF) Infants

The goal of this project is to provide parents and caregivers respiratory education for their child and to then follow-up via phone within a few days of instruction and then again in person at the next clinic visit to assess proper technique and knowledge of performing the respiratory treatments and to have them self-assess their comfort level at performing these treatments. Parents of newly diagnosed CF children have an increased amount of information and specific care expectations placed on them, over and above the demands of caring for a newborn. The hope is that this increased communication and support will help lessen the learning curve and provide the support needed for our parents, while also ensuring that the respiratory care is being done properly and as prescribed.

The Correlation between Mental Health, Physical Health and Adherence in Pediatric CF

The mental health team has been formally screening patients 12 and older for symptoms of anxiety and depression using the PHQ9 and GAD7. This study has found that teenagers in our clinic report very high rates of anxiety (29%) and depression (24%). These rates are much higher than the CFF had reported in a national study of adolescents with CF.

Standardized Protocol for Nutrition and Growth (SPrNG) for Children Aged 0-23 Months

Our Aim is to improve the weight-for-length percentiles during the first 24 months of life in children with CF by implementing a standardized protocol for nutrition and growth in our outpatient CF clinic. Optimized nutrition and appropriate growth are associated with improved lung function and survival in later life. Standardized, comprehensive nutrition management during the first two years of life is a potential opportunity to normalize growth and optimize future pulmonary outcomes.

LLC III and CF FUN to OneCF LLC

LLC III

We are participating in a quality improvement program which lasts from June 2017- October 2018. LLC3 provides us with fundamental improvement knowledge, skills, and practice to improve CF care processes and clinical outcomes for people with CF with a primary focus on pulmonary and nutrition. Throughout the program we have attended multiple learning sessions and a benchmarking site visit in which we learn different skills such as effective communication, co- production, patient and family experiences and team dynamics and relationships. During our benchmarking site visit we strive to gain new knowledge by observing, sharing and learning about processes at a high performing CF center.

CF FUN to OneCF LLC

We have recently gotten accepted to a new quality improvement program which will last from July 2018- October 2019, focusing on transition. CF FUN to OneCF LLC will convene interprofessional improvement teams in the Pediatric and Adult program to assess and improve care processes and outcomes for people with CF. Similar to LLC3, we will attend multiple learning sessions and a benchmarking site visit.