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Contents:
  1. Discovery Shows How Mucus Build-up, Not Infections, Triggers Cystic Fibrosis Lung Damage
  2. Research for Your Health
  3. Webinars | Pulmonary Fibrosis Foundation
  4. Women with cystic fibrosis proudly pose to raise awareness of their disease

One of the first systems to show signs of CF is the respiratory system.

Discovery Shows How Mucus Build-up, Not Infections, Triggers Cystic Fibrosis Lung Damage

The lungs become susceptible to repeated infections and over time, it becomes difficult for a child with CF to breathe. More than 75 percent of cases are diagnosed by the time a child is 2 years old. Cystic fibrosis-related diabetes CFRD can lead to glucose intolerance and insulin-dependent diabetes. Symptoms often first become noticeable during puberty. In rare cases, CFLD can lead to cirrhosis. Our Center is staffed by an experienced, multidisciplinary team of clinicians, all of whom have specialized training in the care of patients with cystic fibrosis.

For Patients. Contact the Cystic Fibrosis Center International Cystic Fibrosis. What is cystic fibrosis? Your doctor may diagnose cystic fibrosis based on your signs and symptoms and results from certain tests, such as genetic and sweat tests that are done to confirm screening tests. To diagnose cystic fibrosis, your doctor may recommend some of the following tests and procedures:.

The sweat test detects a higher amount of chloride—a component of salt that is made of sodium and chloride—in the sweat of people who have cystic fibrosis. In order to make sweat for this test, a colorless, odorless chemical and a little electrical stimulation are applied to a small area of an arm or leg.

The sweat is collected and sent to a hospital lab for testing. While there is not yet a cure for cystic fibrosis, advances in treatment are helping people live longer, healthier lives. After early diagnosis , the goal is proactive treatment to slow down lung disease as much as possible.

You or your child will work with cystic fibrosis specialists. In newborns with a positive screening result, treatment may begin while the diagnosis is being confirmed. Treatment for cystic fibrosis is focused on airway clearance, medicines to prevent and fight infections, and surgery, if needed. Your healthcare team will likely include a cystic fibrosis specialist. This is a doctor who is familiar with the complex nature of cystic fibrosis.

Your doctor may work with a medical team that specializes in cystic fibrosis, often at major medical centers. Airway clearance techniques help loosen lung mucus so it can be cleared, reducing infections and improving breathing. The techniques include special ways of breathing and coughing, devices used by mouth and therapy vests that use vibrations to loosen mucus, and chest physical therapy. These techniques are often used along with medicines such as bronchodilators and mucus thinners.

Medicines to treat cystic fibrosis include those used to maintain and improve lung function, fight infections, clear mucus and help breathing, and work on the faulty CFTR protein.


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Your doctor may prescribe some of the following medicines to treat cystic fibrosis:. Surgery may be an option for people with advanced conditions. See Living With for more information. If you or your child has been diagnosed with cystic fibrosis, it is important that you continue your treatments, follow up with your doctors, and learn how to manage the condition. The goal of cystic fibrosis care is to control symptoms and prevent complications.

Regular checkups with your doctor may be part of your follow-up and treatment. How often your regular checkups take place will depend on your age.

Younger patients, and those who have just been diagnosed, will have more frequent visits. As adults, you may see your doctor less often, perhaps every three months and then once a year for an evaluation. Return to Treatment to review possible treatment options for your cystic fibrosis. In addition to more frequent regular checkups, you may need to see your doctor for additional tests and evaluations, which may include the following:.

Staying healthy is an extremely important part of cystic fibrosis care. Your medical team will work with you to develop a plan for lifestyle changes that can become part of your everyday life. These include the following:. To help you prevent complications and reduce the risk of infections, your doctor may recommend the following:. Cystic fibrosis may have serious complications. Call your doctor if you believe you have any of the following:. Return to Signs, Symptoms, and Complications to review complications.

We are committed to advancing science and translating discoveries into clinical practice to promote the prevention and treatment of heart, lung, blood, and sleep disorders, including cystic fibrosis. Learn about current and future NHLBI efforts to improve health through research and scientific discovery. Learn about the following ways in which the NHLBI continues to translate current research and science into improved health for people who have cystic fibrosis. In support of our mission , we are committed to advancing cystic fibrosis research in part through the following ways:.

We lead or sponsor many studies on cystic fibrosis. See if you or someone you know is eligible to participate in our clinical trials. To learn more about clinical trials at the NIH Clinical Center or to talk to someone about a study that might fit your needs, call the Office of Patient Recruitment Learn more about participating in a clinical trial. View all trials from ClinicalTrials. Visit Children and Clinical Studies to hear experts, parents, and children talk about their experiences with clinical research.

After reading our Cystic Fibrosis Health Topic, you may be interested in additional information found in the following resources. Cystic Fibrosis. Also known as CF. Cystic fibrosis CF is a genetic condition that affects a protein in the body. Mucus is normally slippery and protects the linings of the airways, digestive tract, and other organs and tissues.

Research for Your Health

People who have cystic fibrosis make thick, sticky mucus that can build up and lead to blockages, damage, or infections in the affected organs. Inflammation also causes damage to organs such as the lungs and pancreas. Some people who have cystic fibrosis have few or no signs or symptoms, while others experience severe symptoms or life-threatening complications.

The most serious and common complications of cystic fibrosis are problems with the lungs, also known as pulmonary or respiratory problems, which may include serious lung infections.

Webinars | Pulmonary Fibrosis Foundation

People who have cystic fibrosis often also have problems maintaining good nutrition, because they have a hard time absorbing the nutrients from food. This is a problem that can delay growth. Your doctor may recommend treatments to improve lung function and manage other complications.

Early treatment can improve your quality of life and help you live longer. Explore this Health Topic to learn more about cystic fibrosis, our role in research and clinical trials to improve health, and where to find more information.


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  6. Causes - Cystic Fibrosis. What gene mutations cause cystic fibrosis? Learn more about what the CFTR protein does in your body. Read more. Read less. How is cystic fibrosis inherited? Inheritance Pattern for Cystic Fibrosis. This image shows how CFTR genes are inherited. A person inherits two copies of the CFTR gene, one from each parent. If each parent has a normal CFTR gene and a mutated CFTR gene, each child has a 25 percent chance of inheriting two normal genes, a 50 percent chance of inheriting one normal gene and one gene with a mutation and being a cystic fibrosis carrier, and a 25 percent chance of inheriting two genes with mutations and having cystic fibrosis.

    Look for. Treatment will discuss medicines, supplements, and lifestyle changes that your doctors may recommend if you are diagnosed with cystic fibrosis. Risk Factors - Cystic Fibrosis. Family history and genetics. Race or ethnicity. Screening and Prevention - Cystic Fibrosis. Carrier screening to detect CFTR mutations.

    Women with cystic fibrosis proudly pose to raise awareness of their disease

    Review who is more likely to be a CF carrier in Risk Factors. Prenatal screening. Newborn screening. Prevention strategies.