Cystic Fibrosis
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Understanding Cystic Fibrosis
Pensacola Lung Group is proud to be an accredited adult CF program and the only accredited center in the local area. Our team provides specialized care designed to meet the complex needs of adults living with CF.
Table Of Contents
Causes and Risk Factors
CF is caused by mutations in the CFTR (cystic fibrosis transmembrane conductance regulator) gene, which affects the movement of salt and water in and out of cells. Key risk factors include:
- Genetics: CF is inherited in an autosomal recessive pattern, meaning a child must inherit two faulty CFTR genes (one from each parent) to develop the condition.
- Family History: Individuals with a family history of CF are at a higher risk.
Symptoms and Diagnosis
CF symptoms vary in severity and can affect multiple organs. Common respiratory and digestive symptoms include:
- Persistent cough with thick mucus
- Frequent lung infections
- Wheezing or shortness of breath
- Poor weight gain and growth despite adequate food intake
- Greasy, bulky stools or difficulty digesting food
- Nasal polyps and chronic sinus infections
Diagnosis typically involves:
- Newborn Screening: Identifies CF soon after birth using a blood test.
- Sweat Test: Measures the amount of salt in sweat, a key indicator of CF.
- Genetic Testing: Confirms the presence of CFTR gene mutations.
- Lung Function Tests: Evaluates how well the lungs are working.
Treatment and Management
CF treatment focuses on managing symptoms, reducing complications, and improving lung function. Common approaches include:
- Airway Clearance Techniques: Chest physiotherapy and specialized devices help remove mucus from the lungs.
- Medications:
- Mucus-thinning drugs to improve airway clearance
- Bronchodilators to open airways
- Antibiotics to treat or prevent lung infections
- CFTR modulators to target the defective CFTR protein (for certain mutations)
- Nutritional Support: Enzyme supplements, high-calorie diets, and vitamins improve digestion and overall health.
- Pulmonary Rehabilitation: Structured exercise and breathing techniques enhance lung function.
- Lung Transplant: In severe cases, a lung transplant may be an option when other treatments are no longer effective.
Living with Cystic Fibrosis
Daily management is key to improving quality of life for individuals with CF. Strategies include:
- Following a prescribed treatment plan and attending regular checkups
- Staying active with appropriate exercise to help clear mucus from the lungs
- Practicing good hygiene to reduce infection risk
- Eating a balanced diet with adequate nutrition to support lung and digestive health
- Seeking support from healthcare providers, CF care centers, and patient advocacy groups
Frequently Asked Questions
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Can cystic fibrosis be cured?
There is no cure for CF, but treatments continue to advance, improving life expectancy and quality of life.
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How long do people with CF live?
Life expectancy varies based on disease severity and treatment, but advances in care have significantly increased survival rates.
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Is cystic fibrosis contagious?
No, CF is a genetic condition and cannot be spread from person to person.
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What are the complications of CF?
Potential complications include respiratory failure, diabetes, and digestive issues, but early treatment can help manage these risks.
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How can someone with CF reduce the risk of infections?
Frequent handwashing, avoiding exposure to germs, and staying up to date on vaccinations (which can help prevent certain infections that can be particularly dangerous for individuals with CF) can help lower infection risk.
