Most people don’t give breathing much thought, but cystic fibrosis can make this essential and typically ordinary act a challenge. Cystic fibrosis is a genetic disease that causes a thick, sticky buildup of mucus. It can trap bacteria, clogging the airways and leading to recurring infections that damage the lungs and make breathing increasingly difficult over time. The heavy mucus impacts other organs, including the pancreas, where it impedes digestive enzymes and prevents the body from effectively breaking down food and absorbing nutrients. Both situations can be particularly troublesome for a child.
While there is no cure for CF, effective management can make all the difference. CHoR is home to central Virginia’s only Cystic Fibrosis Foundation Accredited CF Care Center. In addition to providing testing, education, care and advocacy, our CF team leads efforts to improve symptoms and quality of life through clinical research.
“Cystic fibrosis affects each person differently, but quality and consistent care makes a significant positive difference,” said Michael Schechter, MD, chief of pulmonary medicine and director of CHoR’s CF Care Center. “Improvements in daily function and overall quality of life can be accomplished relatively rapidly using quality improvement principles including interdisciplinary team goal setting, standardized and proactive approaches to address areas of concern, and the use of data to follow the effectiveness of the process.”
Most recently the team, which includes nurses, physicians, dietitians, social workers, physical therapists, respiratory therapists, psychologists and geneticists, has focused on establishing quality improvement protocols specific to two important measures in CF care – forced expiratory volume in one second (FEV1) and body mass index.
FEV1 is measured during a pulmonary function test and shows the amount of air a person can forcefully exhale in one second. Typically, lower FEV1 scores indicate more severe illness. Our team has developed a systematic process to calculate and track the changes in FEV1 at every visit and define the appropriate course of action to address issues promptly.
Similar to the efforts in tracking FEV1, the team has developed a protocol for monitoring BMI. Because CF can impact nutrition and growth, consistently tracking and responding to small falloffs in BMI percentiles allows for addressing issues in a timely manner rather than waiting for yearly summary reports, during which time complications can compound. When falloffs are identified, the children and parents get immediate action plans, adjusted dosages of medications/supplements, weight gain goals and advice on increasing caloric intake. They also receive consistent follow-up.
Five years ago, our CF care team recognized opportunities for improvement in the treatment of patients with CF and in how their outcomes compared to those at other CF centers.
“Following the introduction of this new and systematic approach to care, the average pulmonary and nutritional status of our patients is now among the best in the United States. When we have high expectations of ourselves and our patients, we are driven to work toward and achieve great outcomes,” Dr. Schechter.
In addition to building this detailed system for consistent tracking and treatment, the team is working on bundling and sharing the program, including data, charting and action plans, with other CF Centers in a way that is easy to adopt and adapt to their own settings. Dr. Schechter has been invited to present and discuss his team’s approach to care at a number of these centers around the world who wish to adopt it. The ultimate goal is to help all children, whether here at CHoR or on the other side of the globe, focus on playing, learning and just being kids, not on limitations imposed by CF.
Following CF guidelines helps us help Tyler succeed in daily life and on the court.