Cystic fibrosis (CF) is an inherited disease, caused by a genetic mutation, that worsens over time. 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 at 412-648-6202. You can also review what qualifies someone for the procedure and how to be referred.
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, like 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
Living 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 can also trap bacteria, causing serious lung infections like pneumonia. Additionally, it can cause the pancreas to stop producing digestive enzymes, which 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 can cause vibrations, moving 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 may also help increase the strength and endurance of your lungs.
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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, like 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 at 412-648-6202. You can also 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.
About Transplant Services
Established in 1981, UPMC Transplant Services is one of the foremost organ transplant centers in the world. Our clinicians have performed more than 20,000 organ transplant procedures, including liver, kidney, pancreas, single and double lung, heart, and more. We are home to some of the world’s foremost transplant experts and have a long history of developing new antirejection therapies—so organ recipients can enjoy better health with fewer restrictions.