Located at Nebraska Medicine, the adult program of the Nebraska Regional Cystic Fibrosis (CF) Center is the only comprehensive program of its type in the region.
The program uses a multidisciplinary approach to care and includes dedicated physicians, nurses, respiratory therapists, dietitians, social workers and research coordinators. It also includes a pediatric program which typically transitions patients to the adult program by age 21.
In 2007, the adult program was identified as one of eight benchmark centers in a study sponsored by the national Cystic Fibrosis Foundation. In addition, last year the adult and pediatric programs were awarded the Cystic Fibrosis Quality Care Award in recognition of its quality improvement work.
Cystic fibrosis is an inherited condition that affects the lungs and digestive system of about 30,000 people in the United States. A genetic defect causes the body to produce mucus that is thick and sticky rather than loose and watery. Instead of sweeping out bacteria from the lungs, it clogs the lungs and leads to lung damaging infections and inflammation. The mucus also blocks the pancreas, preventing natural enzymes from breaking down and absorbing food.
New medical treatments and aggressive disease management introduced over the last 30 years have greatly improved survival rates for people with cystic fibrosis with many people living well into their 30s, 40s and beyond.
Many patients have mild symptoms of cystic fibrosis, which can make the disease difficult to diagnose. Even in milder forms of the disease, cystic fibrosis still progresses and causes lung damage. If you have any of the following symptoms and have not been diagnosed, you may want to seek a physician that specializes in cystic fibrosis. These include:
- Salty-tasting skin
- Persistent cough that may be accompanied with phlegm
- Frequent lung infections
- Wheezing of shortness of breath
- Poor growth/weight gain
- Frequent greasy, bulky stools or difficulty in bowel movements
There are several ways to diagnosis cystic fibrosis. Diagnostic methods include:
- A newborn screening
- This screening is required by all states. While not definitive, it can lead to further testing to confirm a diagnosis.
- Sweat test
- This is a simple test that measures the concentration of salt in a person’s sweat.
- Genetic carrier testing
- More than 10 million Americans are carriers of the defective cystic fibrosis gene but have no symptoms. To have cystic fibrosis, a child must inherit a copy of the defective gene from each parent. A blood test can be done that will detect carriers.
Studies show that patients who are treated at an accredited cystic fibrosis center, such as the one here at Nebraska Medicine, are able to manage their disease with the greatest success and have the longest survival rates.
Getting early and proper care is essential to achieve the best outcomes. The goal of treatment is to reduce damage to the lungs by regularly clearing the thick cystic fibrosis mucus from the airways. This helps reduce inflammation, the severity and frequency of lung infections and subsequent lung damage. Good management of the disease may help minimize recurring illnesses and infections and progression of lung disease.
The treatment of CF involve several strategies that include the following:
- Daily airway clearance
These techniques are practiced daily and help loosen and clear mucus from the lungs. They include:
- Mechanical vest – used at least twice a day to shake the mucus loose, which helps clear the airways.
- Postural drainage and percussion – Involves sitting, standing or lying in a variety of positions while someone pounds on the chest to help free up mucus.
- Inhaled medications
Inhaled medications allow drugs to reach the airways more quickly. These may include:
- Mucolytics - medications that thin the mucus
- Antibiotics to treat infection
- Hypertonic saline to draw more water into the airways and make it easier to cough out mucus
- Antibiotics are used to fight infection-causing bacteria.
- Good nutrition is vital to disease management and directly impacts lung function.
- Peter Murphy, MD, pulmonologist, director
- Austin Thompson, MD, pulmonologist, associate director
- Jill Fliege, APRN, coordinator
- Jennifer Rounds, RN
- Andrea White, RN
- Jane Matsui, RRT, respiratory therapist
- Brigid Mordeson, RD, dietitian
- Laura Romero, LMSW, social worker
- Shandalle Fertig, CCRC, clinical certified research coordinator
For clinic location and hours use the Find a Physician link. Make an appointment with a cystic fibrosis specialist by calling 800-922-0000.
- Peter Murphy, MD, pulmonologist
- Austin Thompson, MD, pulmonologist
- John Colombo, MD
- Paul Sammut, MD
- Heather Thomas, MD
- Carrie Barker, MD