Through the internationally recognized center of excellence for cystic fibrosis care, University of Minnesota Health providers use research, teamwork and education to improve quality of life for patients and their families from birth through adulthood.
Cystic fibrosis (CF) requires rigorous treatment throughout a patient’s life. Parents of children with CF must focus on nutrition and keep track of an extensive list of medications, all while carrying out nebulizer treatments and airway clearance therapies.
“Patients and families can spend hours every day managing the disease,” says Jordan M. Dunitz, MD, Adult Cystic Fibrosis Program Director and Co-director of the Minnesota Cystic Fibrosis Center. “It’s a very labor-intensive condition.”
To assist patients and families with the numerous responsibilities associated with managing CF, the Minnesota Cystic Fibrosis Center pediatric and adult care teams work to lessen the burden of treatment so that people living with CF can put life’s other responsibilities and joys ahead of the disease.
Taking Early Action
The prognosis for CF patients greatly improves when aggressive treatment is pursued early, which is why children are screened for CF at birth in every state in the U.S. If a child screens positive for CF, a confirmatory sweat test and follow-up appointment should be scheduled with a genetic counselor at a Cystic Fibrosis Foundation-accredited care center, such as the Minnesota Cystic Fibrosis Center, during the child’s first few weeks of life.
“A newborn with a positive newborn screen for CF needs to have a definitive sweat test to confirm the diagnosis,” says Terri Laguna, MD, MSCS, Pediatric Cystic Fibrosis Program Director and Co-director of the Minnesota Cystic Fibrosis Center. “If this sweat test is positive, then we see the patient and family that same day in our clinic to start treatment as soon as possible.”
In this initial meeting, families are introduced to the wide range of professionals who make up their pediatric CF team at the Minnesota Cystic Fibrosis Center. Because CF is so rare — only 25 to 30 infants are diagnosed with CF in the state of Minnesota each year — parents may not know what to expect. Education provides the foundation for these patients and families to learn how to take control of this disease.
From this point, regular appointments are kept with the entire pediatric multidisciplinary team at the Minnesota Cystic Fibrosis Center. Infants are seen every month until they are 6 months old. Afterward, they are seen every two months until they are 1 year old. CF patients are then seen quarterly for the rest of their lives. Appointments increase in frequency as necessary if patients experience illnesses or complications.
Treatment begins early, with nutritional support, airway clearance therapy, nebulized medications and chest physiotherapy beginning when children are 2 months old.
“There is a large body of evidence that suggests maintaining good nutrition has a dramatic impact on life expectancy,” Dr. Laguna says. “We take an aggressive approach to preventive care to make sure our patients live longer and have better quality lives.”
A Team Effort
Though the pediatric and adult programs at the Minnesota Cystic Fibrosis Center are led by pulmonologists, the disease affects multiple systems in the body and requires a comprehensive team approach to properly treat patients.
Patients’ core care teams consist of dietitians, genetic counselors, social workers, respiratory therapists, nurses, genetic counselors, pharmacists and patients’ primary care providers. The CF team also collaborates closely with gastroenterologists and endocrinologists, several of whom are internationally recognized for their work in pediatric CF and gastrointestinal complications of CF.
“People automatically think of lungs when talking about CF,” Dr. Laguna says. “However, children with CF can develop complications with their liver, growth, nutrition and more. These factors need to be prioritized early on.”
The pediatric dietitian at the Minnesota Cystic Fibrosis Center stays in close contact with parents, ensuring that patients are gaining weight and receiving the proper nutrition. If a complication arises regarding the patient’s GI tract, an M Health gastroenterologist works with the CF management team to address it.
It’s vital for an adult and pediatric CF team with many members to stay in continual communication. To accomplish this, both CF care teams meet weekly to review patient cases from both the previous and upcoming weeks, allowing them to formulate plans for care and adjust goals depending on patient situations.
“Our goal is to help patients live full lives. We encourage them to pursue their dreams of higher education, college, careers, marriage and families.”
— Jordan M. Dunitz, MD, Adult Cystic Fibrosis Director and Co-director, Minnesota Cystic Fibrosis Center
“All CF care centers should have multidisciplinary teams — that’s the standard of care,” Dr. Dunitz says. “However, we think of our teams as very horizontal. Our pulmonologists are the leaders, but everyone’s thoughts and positions are important, and we take them into account.”
Outside of regularly scheduled meetings, team members stay in contact through email and personal interaction.
“The ability to do internal consultations is a great advantage,” Dr. Dunitz says. “If I ever have a question, all I have to do is walk down the hall to find an expert.”
Because of their open lines of communication, team members can reinforce each other’s treatment recommendations and quickly relay information to the proper specialist. For example, while a pulmonologist might develop a treatment plan for a patient, a social worker may take the lead in implementing the plan within the patient’s daily life. If a patient brings up a gastrointestinal issue while meeting with the endocrinologist, the endocrinologist can immediately notify the gastroenterologist of the problem.
Leaders in CF Care
Every year, the pediatric and adult CF teams attend the North American Cystic Fibrosis Conference (NACFC). Scientists, multidisciplinary team members, clinicians and caregivers from around the globe attend the conference to discuss CF research, treatment, medicines and care. In 2016, both the adult and pediatric programs at the Minnesota Cystic Fibrosis Center were recognized with the Cystic Fibrosis Foundation’s Quality Care Award, highlighting excellence in clinical care and quality improvement work.
“Every member of my team either presents research or leads a workshop or roundtable at the NACFC,” Dr. Laguna says. “I’m very proud of my team, and it’s a great sign to be acknowledged for their expertise.”
Dr. Laguna herself is a member of the Cystic Fibrosis Foundation’s Communication Steering Committee, working to help improve communication between CF care centers, patients’ families and team members. She was also invited to speak at the Brazilian Cystic Fibrosis Conference in 2017.
Family members and patients are vital members of CF care teams, as they manage or live with the condition on a day-to-day basis.
“We have a deeply entrenched commitment to listening to parents,” Dr. Laguna says. “We want to have open lines of communication and education so that everyone understands the treatment plan and goals.”
Parents are given the contact information for every member of the pediatric care team during their initial meeting.
“Parents have constant access to all members of the team, 24 hours a day,” Dr. Laguna says. “An important piece of our care is being available for them at all times.”
The Role of Research
Research is an integral part of patient care — and a key factor in improving CF treatment for everyone. Helping expand the number of research studies available to patients, the Minnesota Cystic Fibrosis Center is a member of the Therapeutics Development Network (TDN), which is made up of over 70 CF programs that form a clinical research pipeline to study new medications and therapies.
“Centers have to work together in order to gather enough patients to conduct clinical trials because there are so few CF patients in the country,” Dr. Laguna says. “That’s why being a member of the TDN is so important. It allows us to give patients access to new treatments that are being tested.”
The Cystic Fibrosis Foundation often selects leaders from the University of Minnesota Health pediatric and adult CF programs to develop initiatives and participate in pilot research projects that, in the future, may be implemented across the country. Participation from patients and families is vital to this research.
“We have patients who are extremely interested in research,” Dr. Dunitz says. “They are highly thoughtful and cooperative in terms of providing samples and ideas and helping us move treatments forward.”
In addition to being a member of TDN, the Minnesota Cystic Fibrosis Center has also developed its own separate research program focused on the development of lung disease in infants and children. Experts like Dr. Laguna have obtained grant funding from the National Institutes of Health and the Cystic Fibrosis Foundation to develop their own research projects. Through the participation of patients and families, Dr. Laguna is working to better understand the early infections in infants and toddlers with CF.
A highlight of the Minnesota Cystic Fibrosis Center is that it includes both pediatric and adult care programs. Pediatric patients can take advantage of a smooth transition between the two teams as they reach adulthood, since they are already familiar with the M Health care facilities and processes.
“The Minnesota Cystic Fibrosis Center has the only adult CF program in the area,” Dr. Dunitz says. “About half of the patients we see are from our own pediatric program.”
The pediatric and adult care teams meet once a month to discuss patients who are in the process of transitioning to the adult care team. They share records and details to ensure the adult team is prepared to take on the new patient. The pediatric team can also consult with the adult team on cases that warrant advanced care planning, guaranteeing a continuity of care throughout a patient’s life.
Education also plays a vital role in the transition from pediatric to adult care.
“We start the transition process early to make sure patients are independent by the time they need adult care,” Dr. Laguna says. “We educate them on what their medications are and how to make appointments, get refills and take care of themselves.”
“I knew I wanted to be a pediatric lung physician from the time I was a child because I’ve had asthma my entire life. Eventually, I decided to devote my career to cystic fibrosis care and research. The Minnesota Cystic Fibrosis Center offered me the opportunity to do both.”
— Terri Laguna, MD, MSCS, Pediatric Cystic Fibrosis Program Director and Co-director, Minnesota Cystic Fibrosis Center
The transition process is similar for pediatric patients who receive treatment outside of M Health locations.
“We gather records from outside providers as necessary and arrange telephone calls with their physicians,” Dr. Dunitz says. “Just as we would with a patient transitioning from our own pediatric program, we introduce patients to the members of the adult care team over time, starting with the pulmonary CF physician and introducing a few specialists each visit so as not to overwhelm them.”
Physicians with the Minnesota Cystic Fibrosis Center are also affiliated with University of Minnesota Health, giving their patients access to a full range of care. If patients require a transplant during the course of treatment, their physicians can connect them to and play a role in the transplant program.
“We offer lung transplants, which is a service that is not offered by all CF centers and programs,” Dr. Dunitz says. “We cover other transplants that are associated with the disease as well, such as liver transplants.”
Preparing for the Future
Because the Minnesota Cystic Fibrosis Center is associated with the University of Minnesota Medical School, the institution helps shape the training of new generations of CF physicians. An accredited pediatric pulmonology fellowship program is offered through the University of Minnesota, and members of the Minnesota Cystic Fibrosis Center perform outreach on campus.
“I help develop collaborations with members of the university across a wide range of departments, from microbiology and biochemistry to engineering and dentistry,” Dr. Dunitz says. “We want to take advantage of the great research strength of the University of Minnesota and engage these talented people who may not realize that their knowledge can be applied to CF.”
Dr. Laguna became the director of the pediatric CF program four years ago. From the helm, she will continue to guide the Minnesota Cystic Fibrosis Center’s trend of excellence.
“Other CF programs visit the Minnesota Cystic Fibrosis Center and ask us for our expertise,” Dr. Laguna says. “We have assembled a phenomenal team, and I want to keep that momentum moving forward. I want to keep our patients as healthy as possible for as long as possible.”