Learn more about living with cystic fibrosis.

Cystic fibrosis (CF) is an inherited disease that worsens over time. Caused by a genetic mutation, the disease causes the glands that secrete mucus and sweat to function improperly, leading mucus to build up like thick glue in the lungs, pancreas, liver, and other organs. There’s no cure for CF, which causes serious health problems including chronic infections, lung damage, and respiratory failure.

To learn about how lung transplantation can benefit someone with cystic fibrosis, talk to your doctor and contact the Lung Transplant Program at UPMC.

What Causes Cystic Fibrosis?

Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene can cause CT. If you have CF, you inherited a defective CFTR gene from each parent. Both parents must have the genetic mutation for their child to get the disease. People with only one faulty one normal CFTR gene are considered carriers because they don’t have the disease but can pass the gene on to their children.

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Symptoms of Cystic Fibrosis

Signs and symptoms of cystic fibrosis vary from person to person, but may include:

  • Persistent cough with phlegm.
  • Frequent lung infections, such as pneumonia or bronchitis.
  • Wheezing or shortness of breath.
  • Salty tasting skin.
  • Poor growth rate.
  • Weight loss.
  • Difficult bowel movements.
  • Male infertility.

More than 30,000 people in the United States have cystic fibrosis, with more than 70,000 affected worldwide, according to the Cystic Fibrosis Foundation. Over 75 percent of those affected by the disease are diagnosed by age two.

Living with Cystic Fibrosis

Life with cystic fibrosis varies, as each person’s body may experience different symptoms and side effects. The typical life expectancy for someone with CF is mid-30s. As treatments have improved over the years, patients with CF are now living into their 40s and beyond.

Cystic fibrosis may cause mucus to clog lung airways, leading to difficulty breathing. The mucus also can trap bacteria, causing serious lung infections, including pneumonia. Additionally, it can cause the pancreas to stop producing digestive enzymes that help break down food and allow the body to absorb nutrients. In the liver, mucus can block bile ducts and contribute to liver disease.

A series of techniques known as postural drainage and chest percussion can help loosen and remove mucus from the lungs.

By placing the body in certain positions, gravity can help drain the mucus into the mouth. Clapping the chest with a cupped hand causes vibrations that move mucus into larger airways where it can be coughed up. A doctor will recommend how often to perform these techniques.

For those who are well enough, doctors may recommend exercise to loosen mucus, encourage coughing, and improve airflow into your lungs. Exercise also may help increase the strength and endurance of your lungs.

How to Avoid Complications

To help prevent complications and infections, people who have CF should try to avoid germs, which can cause serious infections. Here are some helpful tips for CF patients:

  • Avoid contact with other people with cystic fibrosis or anyone who is sick.
  • Keep a six-foot distance from anyone who is coughing or sneezing.
  • Wash hands often.
  • Disinfect your nebulizer frequently.
  • Avoid sharing personal items, such as straws or utensils.
  • Avoid frequent contact with dust or dirt.
  • Get vaccinated.

Lung Transplant for Patients with CF

For some with CF, damage to the lungs may become so severe that a lung transplant is a viable option. Lung transplants are risky and do not improve symptoms in other parts of your body. Your doctors will perform tests to determine if you are a good candidate for transplantation.

If you are deemed a candidate for lung transplantation, you will be placed on an organ donor wait list. For the average CF patient, the wait can be a few months. With a full lung transplant, your diseased lungs are replaced with a healthy pair of donor lungs.

About 60 percent of people live at least five more years after a lung transplant. As with any transplant surgery, the procedure comes with the risk of infection and rejection of the organ. You will have to take immunosuppressant medicines for the rest of your life.

To learn more about this option, talk to your doctor or contact the Lung Transplant Program at UPMC. You can review what qualifies someone for the procedure and how to be referred.

Cystic fibrosis is a serious but manageable condition when you have the proper care and treatment. Make sure you find a doctor who specializes in cystic fibrosis treatment to help you formulate a plan to live as healthy a life as possible with your condition.

 

Editor's Note: This article was originally published on , and was last reviewed on .

About Transplant Services

For more than four decades, UPMC Transplant Services has been a leader in organ transplantation. Our clinicians have performed more than 20,000 organ transplant procedures, making UPMC one of the foremost organ transplant centers in the world. We are home to some of the world’s foremost transplant experts and take on some of the most challenging cases. Through research, we have developed new therapies that provide our patients better outcomes — so organ recipients can enjoy better health with fewer restrictions. Above all, we are committed to providing compassionate, complete care that can change – and save – our patients’ lives. Visit our website to find a provider near you.