Cystic fibrosis - resources

After getting detailed information about the symptoms and your family's medical history, your healthcare professional may order tests to help with diagnosis and plan treatment. Your healthcare professional also may ask questions, such as: What symptoms are you or your child having? When did the symptoms start? Does anything make the symptoms better or worse? Has anyone in your family ever had cystic fibrosis? Has growth been average and weight been stable? Cystic fibrosis. National Heart, Lung, and Blood Institute. https://www.nhlbi.nih.gov/health/cystic-fibrosis. Accessed Aug. 8, 2024. Bronchiectasis. National Heart, Lung, and Blood Institute. https://www.nhlbi.nih.gov/health/bronchiectasis. Accessed Aug. 8, 2024. FAQs: Cystic fibrosis: Prenatal screening and diagnosis. American College of Obstetricians and Gynecologists. https://www.acog.org/womens-health/faqs/cystic-fibrosis-prenatal-screening-and-diagnosis. Accessed Aug. 8, 2024. Ferri FF. Cystic fibrosis. In: Ferri's Clinical Advisor 2025. Elsevier; 2025. https://www.clinicalkey.com. Accessed Aug. 8, 2024. Kliegman RM, et al., eds. Cystic fibrosis. In: Nelson Textbook of Pediatrics. 22nd ed. Elsevier; 2025. https://www.clinicalkey.com. Accessed Aug. 8, 2024. Kellerman RD, et al. Cystic fibrosis. In: Conn's Current Therapy 2024. Elsevier; 2024. https://www.clinicalkey.com. Accessed Aug. 8, 2024. Lockwood CJ, et al., eds. Respiratory diseases in pregnancy. In: Creasy and Resnik's Maternal-Fetal Medicine: Principles and Practice. 9th ed. Elsevier; 2023. https://www.clinicalkey.com. Accessed Aug. 8, 2024. Ong T, et al. Cystic fibrosis: A review. JAMA. 2023; doi:10.1001/jama.2023.8120. Rubin R. Tackling the misconception that cystic fibrosis is a "white people's disease." JAMA. 2021; doi:10.1001/jama.2021.5086. Care centers. Cystic Fibrosis Foundation. https://www.cff.org/managing-cf/care-centers. Accessed Aug. 8, 2024. Trikafta (prescribing information). Vertex Pharmaceuticals Inc.; 2023. https://www.trikafta.com/. Accessed Aug. 8, 2024. Symdeko (prescribing information). Vertex Pharmaceuticals Inc.; 2023. https://www.symdeko.com/. Accessed Aug. 8, 2024. Orkambi (prescribing information). Vertex Pharmaceuticals Inc.; 2023. https://www.orkambi.com/. Accessed Aug. 8, 2024. Kalydeco (prescribing information). Vertex Pharmaceuticals Inc.; 2023. https://www.kalydeco.com/. Accessed Aug. 8, 2024. Pilewski JM. Update on lung transplantation for cystic fibrosis. Clinics in Chest Medicine. 2022; doi:10.1016/j.ccm.2022.07.002. Fridell JA, et al. Pancreas transplantation for cystic fibrosis: A frequently missed opportunity. Clinical Transplantation. 2021; doi:10.1111/ctr.14371. Wadsworth LE, et al. Non-invasive ventilation is associated with long-term improvements in lung function and gas exchange in cystic fibrosis adults with hypercapnic respiratory failure. Journal of Cystic Fibrosis. 2021; doi:10.1016/j.jcf.2021.05.011. Allscripts EPSi. Mayo Clinic. Medical review (expert opinion). Mayo Clinic. Oct. 18, 2024.

If you or your child has symptoms of cystic fibrosis — or if someone in your family has CF — talk with your healthcare professional about testing for the condition. Make an appointment with a doctor who has skills and experience in treating CF. CF requires regular follow-up with your healthcare professional, at least every three months. Call your healthcare professional if you have new or worsening symptoms, such as more mucus than usual or a change in the mucus color, lack of energy, weight loss, or severe constipation. Get medical care right away if you're coughing up blood, have chest pain or trouble breathing, or have severe stomach pain and bloating. Call 911 or your local emergency number or go to the emergency department at a hospital if: You're having a hard time catching your breath or talking. Your lips or fingernails turn blue or gray. Others notice that you're not mentally alert.

Cystic fibrosis (CF) is a genetic disorder, which means you get it from your parents at birth. It affects your lungs, pancreas, and other organs. CF changes the way chloride (salt) moves through the cells of your body. This causes the mucus (which should be thin and slippery) in various organs to become thick and sticky. Over time, this thick mucus builds up inside your airways, making it hard to breathe. The mucus traps germs and leads to infections and inflammation. It can also cause severe, long-term damage to the lungs and lead to respiratory failure (inability to breathe normally) and death. In the pancreas, the thick mucus caused by CF prevents the release of digestive enzymes when you eat. This leads to malnutrition and poor growth. CF can also cause liver disease, reproductive problems, and cystic fibrosis-related diabetes (CFRD). More than 40,000 people in the U.S. live with CF. Doctors diagnose about 1,000 new cases each year. Today, more than half of the CF population is aged 18 or older, and new treatments have expanded the life expectancy by decades.

Based on the current clinical studies, Alyftrek is a safe and effective treatment for people with cystic fibrosis. The Cystic Fibrosis Foundation published a CFTR modulator therapy care guideline in 2018. Alyftrek is not included in these guidelines since it was approved by the FDA after these guidelines were published.

The symptoms of cystic fibrosis vary. Some children will have symptoms at birth, while others may not have symptoms for weeks, months, or even years. The severity of symptoms also varies, with some children showing only mild digestive and lung problems and others having severe food-absorption problems and life-threatening breathing complications. The most common symptoms of cystic fibrosis are: Salty-tasting skin , which parents notice when they kiss their child Frequent coughing , wheezing , or bouts of pneumonia or sinusitis Difficulty breathing that keeps getting worse Big appetite but poor weight gain Bulky, smelly, greasy bowel movements Over time, the symptoms of cystic fibrosis can worsen and may include: Chronic productive cough, recurrent lung infections Obstructive lung disease ( emphysema ) Chronic nasal congestion and sinus infections Pancreatitis , a painful inflammation of the pancreas Liver disease Diabetes Gallstones