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.
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.
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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