Cystic Fibrosis Centers Engage Patients, Improve Health

A network of accredited cystic fibrosis centers stretches across the U.S., and it is a tight-knit community where everyone knows everyone’s business. They chat about quality improvement initiatives, offer support for implementing evidence-based care, seek best practices, and air outcomes for all to see.

For the past seven years, the Cystic Fibrosis Foundation has nurtured this culture of knowledge-sharing by offering training grants for CF care centers nationwide to participate in Learning and Leadership Collaboratives (LLC). While various learning collaboratives in many health care settings have existed for several decades, the CF community’s success at methodically spreading the science of quality improvement has stood out, according to Marjorie M. Godfrey, MS, RN, co-director of the Dartmouth Institute Microsystem Academy which provides the curriculum for the LLCs.

“You can’t just send out a bunch of materials and expect teams to fix problems,” Godfrey said. “You must engage them in the developmental process. … The CF Foundation has promoted and created conditions that foster amazing learning.”

More than half of the CF care centers accredited by the CF Foundation have taken advantage of the yearlong LLCs. Each center usually identifies two or three interdisciplinary team members to take part in the action-learning series that uses face-to-face classes, interactive electronic and phone meetings, and a coaching model. Each team chooses a project based on the CF Foundation’s national benchmarking data and work to improve care of CF patients through systematic, targeted interventions.

“The centers who have participated have benefited because you’re given tools to look at what is in your own back yard, and then you can set forth ways to correct the problems,” said Robert Zanni, MD, FAAP, CPI, director of the Cystic Fibrosis Center at Monmouth Medical Center, Long Branch, N.J. He participated in a LLC in 2006 and is now a Clinical Microsystem coach. “We want to give the best care we can give.”

Foundation for quality improvement

The importance of taking a standardized approach to quality improvement hit home for many CF centers when Bruce Marshall, MD, joined the CF Foundation as vice president of clinical affairs in 2002. Back then, analysis of patient registry annual data revealed that accredited CF centers “were all over the place” when crucial patient outcomes such as lung function, nutrition, and survival were compared, Dr. Zanni said. Dr. Marshall’s leadership laid the framework for the LLCs by encouraging CF centers to continuously review medical outcomes and practice patterns and to obtain greater input from patients.

Clinicians from the Johns Hopkins Cystic Fibrosis Center, Baltimore, took advantage of the LLCs’ formal quality improvement training six years ago. Since then they have constantly re-examined their practices to identify areas that can be improved. Each quality improvement project they initiate has champions within the CF center who focus on the individual care process, identify current therapies, optimize their use, and get better outcomes.

“The system you have now is designed to get the results you have now,” said Peter J. Mogayzel Jr., MD, PhD, who has been director of the Johns Hopkins CF Center since 2002. “If you want to get different results, you have to change the system.”

One of Johns Hopkins CF Center’s quality improvement projects reshaped how the CF community looks at vitamin D supplementation. They found that existing recommendations set by the CF Foundation for treating vitamin D deficiency in children with CF were too low. Johns Hopkins amended its treatment protocol and now treats both adult and pediatric CF patients who have vitamin D deficiency with 50,000 IU daily for four weeks. After Johns Hopkins published their findings in 2008, other CF centers also began treating their patients with vitamin D deficiency more aggressively.

Factors unique to CF centers’ success

The vitamin D project is only one of the many interventions originating from the LLCs that have accelerated the rate of advancement in CF care. What gives these CF quality improvement efforts such momentum?

Accredited CF centers’ commitment to transparency is one reason, Godfrey said. The centers routinely send updates to the CF Foundation’s electronic patient registry, which anonymously tracks the health status of more than 26,000 CF patients. The CF Foundation then analyzes and shares CF center aggregate performance data to facilitate quality improvement. For example, a CF team who wants to improve patients’ lung function can easily find out who the top performing center is and explore what they did to achieve high FEV1 values. If you look at other learning collaboratives, they often don’t have transparent access to all patient outcomes, Godfrey said.

The role of coaching support is also a unique component of the LLCs. Dartmouth develops CF clinicians to help interdisciplinary teams as they integrate an improvement model into all aspects of their frontline CF care. The coaches provide support, answer questions, and give gentle reminders to help teams meet project deadlines and goals. “Hands down, the coaches are moving this forward,” Godfrey said.

As a coach, Dr. Zanni stays in touch with his designated team through conference calls several times a month. “It’s also a great opportunity to keep me engaged in the quality improvement process,” he said. “Someone always has another twist on how to do something better.”

Patient and family involvement

But the clinicians’ perspective is only half the story. CF centers who want to improve care delivery always must consider the recipients of the system they want to put in place: patients and families. “The idea that families and patients need to be involved at the very beginning of a quality improvement project is beginning to take hold,” Dr. Mogayzel said.


At Monmouth Medical’s CF Center, for example, a family advisory board worked with clinicians to design a quality improvement project that focused on improving adherence to daily airway clearance therapies. An initial survey showed that 53 percent of their patient population was non-adherent. The quality improvement team met with patients and families to brainstorm about possible roadblocks – time management, not feeling the immediate effects of therapy, boredom from using a particular device, etc.

Kerryann Silvestri, whose 7-year-old son, AJ, has cystic fibrosis, participates in the Monmouth Medical CF Center’s family advisory board. She found it valuable to have an honest dialogue with other families and clinicians about her son’s busy treatment schedule and what sometimes gets in its way.

“I think it’s important to get families’ perspective because the reality is that as dedicated as a physician or CF team can be, they have the ability to turn CF off at the end of the day,” Silvestri said. “We bring real-life experience to the table.”

The quality improvement team used these insights to implement a patient educational program called REACT (Re-Education of Airway Clearance Techniques). All patients were asked to come into the center and took additional surveys to pinpoint aspects of their airway clearance regimen where they might need some assistance.

Respiratory therapists reviewed patients’ equipment to ensure machines were up to date and evaluated their therapy delivery techniques to identify and correct trouble spots. The RTs provided additional coaching for patients who had fallen into bad habits and sometimes provided them with a choice of alternative techniques to regain their motivation to maintain a regular airway clearance schedule.

“This simple redirection made a dramatic change in how patients were doing,” Dr. Zanni said. Prior to the REACT quality improvement initiative, patients at the Monmouth Medical CF Center had median FEV1 scores of 85 percent. Two years after implementing the REACT program, patients improved an average of 10 percentage points. The challenge now, Dr. Zanni reminded, is for the CF center staff to remain vigilant and continue to engage families through yearly re-education so that they continue a sense of ownership of their airway clearance therapy.

As more CF centers create family advisory boards, Silvestri encourages patients and families to become part of the formal quality improvement process. “It’s important that they can ask or say anything,” she said. “It’s OK to say, ‘We’re not the most perfect center in the world. We’re in a constant state of quality improvement.’”

Contact Sharlene George at [email protected].