Six years ago, Jodi Guilford’s daughter, Mackenzie, who was 16 months old at the time, tested positive for cystic fibrosis (CF). Jodi still remembers that day and how the medical staff at the University of Michigan Cystic Fibrosis Center supported her and her family. “The team was wonderful,” Jodi recalls. “We met with a physical therapist, a social worker, a dietitian, and Dr. Nasr (Director of the University of Michigan Cystic Fibrosis Center). They were very supportive.” Even though the Guilfords live in Defiance, Ohio, which is a two-hour drive from Ann Arbor, and there are other CF centers closer to home, they made the decision to have Mackenzie receive medical treatment at U-M. Jodi says, “It is a decision we have never once regretted. We are 100 percent comfortable with our decision, and the fact that we are getting the best care possible.”

Approximately 30,000 children and adults in the United States have this genetic disease that primarily affects the respiratory system. In patients with CF, the airway mucus becomes very thick and sticky, blocking air passages to the lungs. The disease also affects the pancreas as well as the sweat glands. People with cystic fibrosis are unable to digest fats, and their sweat is abnormally salty. CF is most common in Caucasians, but it isn’t limited to that racial group.

Under the leadership of Samya Nasr, M.D., Director, Cystic Fibrosis Center, and Director of Clinical Services, Pediatric Pulmonology, the University of Michigan Cystic Fibrosis Center has been recognized by the Cystic Fibrosis Foundation as a national leader in the care of patients with CF. The center’s state-of-the-art approach, including the use of infant lung function testing, chest CT scans, and bronchoscopy to detect infection, as well as vigorous antibiotic treatment and nutritional supplementation, is improving the quality of life and lifespan for patients with the disease.

One reason people choose U-M’s CF center is the opportunity to participate in clinical trials of new drugs for treatment of the disease. “A CF center should be strong in research as well as patient care, so you can give parents the whole picture of what is going on with CF,” says Nasr. Jodi appreciates Nasr’s aggressive treatment of her daughter’s disease. “Dr. Nasr is really up on her research,” she says.

Jodi credits the whole medical team with being very informed and updated about the latest CF information. Patients visit the CF clinic every three months and see a physician, nurse, dietitian, social worker, physical therapist, and respiratory therapist throughout the course of their care. U-M has six physicians who are involved in CF care, which is more than many centers.

The CF Foundation recommends that patients see a dietitian once a year. At U-M, Mary Hope O’Leary, R.D., M.S., Pediatric Nutrition Specialist, sees patients at almost every visit, providing much closer monitoring than most other CF centers.

CONTINUITY OF CARE

The comprehensive care provided to CF patients extends to the inpatient unit (5-West) at University of Michigan C.S. Mott Children’s Hospital. “The nurses on 5-West have been unbelievably committed to CF care. They have been tremendously helpful in keeping kids healthy,” Nasr notes.

Another important part of inpatient care is offered by Child and Family Life, which provides therapeutic programs and play to help ease the stress of hospitalization on patients and their families. “Child & Family Lifehas been very important to patient care,” Nasr says. “They do a lot of things for the patients to keep them busy. Because of Child & Family Life, there are some patients who love to come to the hospital!”

EXTRA BENEFITS

The team provides many special opportunities for families throughout the year including a CF education night, a support group for parents, and a newsletter. Wendy Carmack, who has three sons, two of whom have CF, describes the support group meetings as a great resource. “Every parent with a child with CF should go to these meetings,” she recommends.

Social workers Carol Campbell Welsch, M.S.W., A.C.S.W., Coordinator, CF Center, and Donna Hurle, M.S.W., run the support group and organize the newsletter as well as working with patients and families in the clinic, during inpatient stays, and over the phone. Social workers provide important support and advocacy for patient/family needs.

Welsch also helps patients transition to the adult CF clinic when they are ready. With the life expectancy of cystic fibrosis patients increasing, the number of adult patients with the disease is growing.

The social workers and the rest of the team maintain a positive perspective and help patients focus on what they can do to stay healthy. Jodi appreciates that positive outlook, and the team’s frequent reminders to focus on what is going well. That is just one more factor in helping the Guilford family know that they made the right decision when they chose the U-M CF Center six years ago.