Team-based care has made all the difference for cystic fibrosis (CF) patients, said Debbie Toder, MD, assistant professor of pediatrics. Because CF affects the respiratory and digestive systems, treatment requires expertise from pediatric and medical pulmonologists, respiratory therapists, dietitians and gastroenterologists. Without a centralized treatment center, cystic fibrosis patients may overlook certain complications or not get the appropriate level of care.

Dr. Toder is director of the Cystic Fibrosis Center at Children’s Hospital of Michigan, where the CF team treats approximately 140 children and 80 adult patients. It is one of five CF centers in the state, and the only one in metro Detroit.

Since graduating from medical school at Penn State in 1985, Dr. Toder says she has seen CF patients live longer, healthier lives, due in large part to the aggressive treatment that comes with comprehensive care. “When patients have coordinated health care, they are able to successfully manage the various aspects of their disease,” said Dr. Toder.

Symptoms and complications of CF are numerous. A defective protein allows too much salt to leave the body, requiring CF patients to seek nutritional counseling to increase weight gain and prevent cardiac problems and shock. Since the mucus in their body lacks water, it becomes too thick, clogging the lungs and causing serious infections. The mucus may then obstruct the pancreas, preventing enzymes from reaching the intestines to break down and digest food. If these symptoms go untreated, patients may develop liver disorders, lung damage, pancreas shutdown or respiratory failure. If these symptoms are treated, however, a more normal life span may be possible.

The center is part of a clinical trials network which evaluates multiple drugs and therapies for CF. Wayne State researchers are currently working on better enzymes that improve digestion and growth, oral supplements that provide substantial nourishment and respiratory therapies that increase lung function. For instance, Dr. Ibrahim Abdulhamid, associate director of the center, is using zinc supplements to help decrease the severity and frequency of lung infections in patients with CF, thereby stalling the progression of pulmonary disease.

Dr. Abdulhamid was also part of a team that examined the validity of a new scale to measure CF patients’ “quality of well-being.” Typically, successful treatment is measured in clinical terms including increased weight gain, decreased coughing and sputum production, and improved pulmonary function. As aggressive therapies increase life expectancy, patients and physicians want more information about how those treatment modalities affect quality of life. So far, the research has shown that patients treated at comprehensive centers have better outcomes overall.

“We are no longer solely concerned with acute exacerbations, but also with how well the patient does during nonacute phases,” said Dr. Abdulhamid. “It is important for us to have information about the impact of disease and treatments on a patient’s social and physical functioning and other relevant domains.”  

This consideration of the total patient is the foundation for the Cystic Fibrosis Center at WSU and the Detroit Medical Center. It has been in existence since 1961, and continues to make great advances in research and patient care.