Cystic Fibrosis: What You Need to Know
Cystic fibrosis (CF) is a genetic condition that mainly affects the lungs and digestive system. People with CF produce thick, sticky mucus that clogs airways and traps bacteria, causing repeated lung infections and breathing problems. It also makes the pancreas less able to release enzymes, which leads to poor weight gain and malnutrition.
Symptoms often show in infancy or early childhood, but some people get a milder form and are diagnosed later. Watch for persistent cough, salty-tasting skin, poor growth, and frequent greasy stools. If a newborn screen is positive or symptoms match, doctors use a sweat test and genetic testing to confirm the diagnosis.
Treatment focuses on keeping airways clear, preventing infections, and supporting nutrition.
Airway clearance techniques include chest physiotherapy, breathing exercises, and devices that vibrate the chest to loosen mucus. Inhaled medicines — bronchodilators, mucolytics, and hypertonic saline — help thin mucus and make it easier to cough up. Regular airway clearance and inhaled meds reduce flare-ups and hospital visits.
Antibiotics treat infections when they appear and sometimes as long-term therapy to control chronic bacteria. Many people with CF use inhaled antibiotics to target lung bugs directly. When lung function drops significantly, doctors may recommend a course of IV antibiotics or consider lung transplant in severe cases.
A big change in recent years is CFTR modulators — medicines that target the faulty protein behind CF. Drugs like ivacaftor, lumacaftor/ivacaftor, tezacaftor/ivacaftor, and elexacaftor/tezacaftor/ivacaftor can improve lung function and reduce hospital stays for people with certain genetic mutations. Not every person with CF is eligible, so genetic testing matters.
Nutrition care is part of everyday life with CF.
Pancreatic enzyme replacements help digest food, and high-calorie diets make up for higher energy needs. Fat-soluble vitamin supplements (A, D, E, K) are commonly needed. Regular monitoring by a dietitian helps keep growth and weight on track.
Exercise is simple but powerful: it helps clear mucus, improves fitness, and supports mood. Even walking, swimming, or cycling for 30 minutes most days can help.
People with CF should avoid close contact with other people who have CF because infections can pass between them. Routine vaccinations and flu shots reduce risks. Seeing a specialized CF care team — pulmonologist, dietitian, physiotherapist, and social worker — makes a big difference in managing daily needs.
If you or a family member is newly diagnosed, ask about genetic counseling, clinical trials, and patient support groups. These resources offer practical help and the latest treatment options. Keeping updated with your care team helps you make choices that fit your life.
Life expectancy and daily health have improved a lot. Many adults with CF work, study, and raise families. Still, lung health can decline over time, so early treatment matters. Ask your team about mental health support and home therapies that fit your schedule. For trustworthy info, check national CF foundations, your clinic, and peer support forums. New treatments and trials arrive each year, so staying informed can change outcomes. Talk openly with your team and loved ones.
