Cystic Fibrosis

Many patients have mild symptoms of cystic fibrosis initially, 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 doctor who 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

Why come to Nebraska Medicine for diagnosis or treatment?

We use a variety of methods to diagnose whether you actually have it, including

Newborn Screening

This screening is required by all states. While not definitive, it can lead to further testing to confirm a diagnosis.

A 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. This means that both parents must be carriers of the gene. If this is the case, each child born to those parents has a one in four chance of having cystic fibrosis. A blood test can be done that will detect carriers. 

Your Diagnosis and Treatment Decisions Made by a Team of Experts

In other words, you will have the best minds in the area collaborating on your treatment. Our program incorporates several doctors with different specialties to come up with a treatment plan. This allows you to have a dedicated group of pulmonologists (doctors who specialize in lung conditions), nurses, respiratory therapists, dietitians, social workers and research coordinators who all work together to achieve the best patient experience and care plan. We even have doctors who specialize in treating diabetes cases related to Cystic Fibrosis.

Continuous Care from Child through Adulthood

The pediatric program smoothly transitions patients to the adult program by age 21. Patients can grow up within the clinic, know the faces and spaces, and receive exemplary care through all their ages and stages of the disease.

Nebraska Medicine is proud to offer opportunities to patients who qualify to trial the newest therapies and medicines in the pipeline. The ground-breaking research paired with doctors, nurses and staff who genuinely care for each patient make Nebraska Medicine a great home for those living with cystic fibrosis.

How We Treat Cystic Fibrosis:

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.

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

Antibiotics are used to fight infection-causing bacteria. Good nutrition is vital to disease management and directly impacts lung function.