Cystic fibrosis is a genetic condition caused by a mutation in the cystic fibrosis transmembrane regulator (CFTR) gene. The mutation leads to the production of abnormal CFTR protein or no CFTR protein at all. This disrupts the flow of salts and water across cell membranes, causing a build-up of thick, sticky mucus in the lungs, kidneys, pancreas, and other organs.

The accumulation of thick mucus in the lungs is a typical characteristic of CF, causing lung infections and other respiratory system symptoms.

Lung involvement

Thick, sticky mucus in the lungs creates an environment that is favorable for the growth of bacteria. A healthy lung is capable of clearing the infection, but due to the thick mucus these infection-causing agents persist in CF, leading to chronic lung infections or pneumonia.

The most common bacteria associated with CF lung infections is Pseudomonas aeruginosa. Although usually treatable with antibiotics, P.aeruginosa have evolved, and there are strains that have become increasingly resistant to most antibiotics. Persistent infection with bacteria such as P.aeruginosa can lead to further inflammatory damage in the lungs.

The thickened sticky mucus also can clog the airways in the lungs, hindering airflow and causing breathing problems and bronchitis. This can result in symptoms such as wheezing (shortness of breath) and a persistent cough. 

Blood flow through the arteries can be blocked in mucus-clogged lungs, causing an increase in blood pressure and resulting in pulmonary hypertension and further deteriorating lung function. High blood pressure also may lead to heart problems in the long term.

As CF progresses, the risk of developing severe respiratory complications such as pneumothorax (collapsed lung) increases significantly.

Upper respiratory tract involvement

Sinusitis (inflammation of sinus cavities in the nose) is common in children with CF.

The lining of the nose also can become inflamed in CF patients, causing small non-cancerous growths called nasal polyps. These may form in the sinuses, but can extend to the nostrils and the throat, causing breathing difficulties. Medication can shrink nasal polyps, and in some instances, doctors may need to remove them surgically.

Treatment of respiratory system symptoms

Several treatment options are available to lower and manage the respiratory symptoms of CF. Anti-inflammatory medications and inhaled bronchodilators can aid in breathing. Similarly, antibiotics to clear lung infections and prevent their reoccurrence can help improve lung function. Advanced therapies such as CFTR modulators that target the underlying cause of CF also can clear the airways, ease breathing and improve patients’ quality of life. Physiotherapy may help loosen the mucus in the lungs, making it easier to cough it out.

Respiratory symptoms and lifestyle changes

Lifestyle changes, such as quitting smoking and increasing fluid intake, can help manage the respiratory system symptoms of CF.

Nutrition plays a vital role in improving patients’ quality of life. Eating a healthy diet, and including supplements in the diet to facilitate nutrient absorption, is critical for patients with CF.

Regular physical exercise to support lung function and practicing breathing techniques that help loosen the mucus can help alleviate respiratory system symptoms.

Nutritional changes should be made only after consulting with a doctor and following that recommendation. Similarly, any exercise and physical activity should be performed under the guidance of a doctor and experienced physiotherapist.

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Cystic Fibrosis News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health providers with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.

Vijaya Iyer is a freelance science writer with BioNews Services. She has contributed content to their several disease-specific websites, including cystic fibrosis, multiple sclerosis, muscular dystrophy, and more. She received her PhD in Microbiology from Kansas State University. Her research focused on molecular biology, bacterial interactions, metabolism, and animal models to study bacterial infections. Following her PhD, Dr. Iyer went on to complete three postdoctoral fellowships at Kansas State University, University of Miami and Temple University. She joined BioNews Services to utilize her scientific background and writing skills to help patients and caregivers remain abreast with important scientific breakthroughs.
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Vijaya Iyer is a freelance science writer with BioNews Services. She has contributed content to their several disease-specific websites, including cystic fibrosis, multiple sclerosis, muscular dystrophy, and more. She received her PhD in Microbiology from Kansas State University. Her research focused on molecular biology, bacterial interactions, metabolism, and animal models to study bacterial infections. Following her PhD, Dr. Iyer went on to complete three postdoctoral fellowships at Kansas State University, University of Miami and Temple University. She joined BioNews Services to utilize her scientific background and writing skills to help patients and caregivers remain abreast with important scientific breakthroughs.
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