Cystic fibrosis (CF) is a genetic condition that affects the lungs, digestive system, and other body organs by damaging the cells that produce sweat and mucus in the body. People with this condition produce sticky, thick mucus that accumulates and obstructs tubes, ducts, and airways all over your body, causing infections and making it difficult to digest meals or breathe.
Symptoms vary from person to person, depending on which organs are damaged and the severity of the condition. Some people have little or no symptoms, while others have severe ones. Early detection, treatment, and close monitoring can help manage symptoms, decrease the progression of CF, and lead to improved lung health.
What are the symptoms of cystic fibrosis?
Symptoms that people with CF may experience include:
- Skin with a strong salty flavor
- Shortness of breath or wheezing
- Nasal polyps
- Infertility in men
- Having difficulty gaining weight
- Coughing that lasts and produces mucous
- Clubbed or enlarged fingertips and toes
- Chronic constipation, greasy stools, and rectal prolapse
- Recurrent sinus infections (such as sinusitis) or lung infections (such as pneumonia)
What are the causes of cystic fibrosis?
Cystic fibrosis is caused by a mutation in the CFTR (cystic fibrosis transmembrane conductance regulator) gene, which controls and regulates the passage of salt and fluids into and out of your cells. If the CFTR gene does not function properly, the respiratory, digestive, and reproductive systems produce thick, sticky mucus and sweat that contains more salt.
To develop CF, children must inherit one copy of the gene from both parents. Children will not show symptoms if they inherit one copy, but they will be carriers of the disease and may be able to pass it down to their children.
How is cystic fibrosis diagnosed?
During your consultation, the pulmonologist will perform a physical examination and run various tests to diagnose CF. These tests may include a DNA test to check for CFTR gene mutations or a blood test to measure the amount of immunoreactive trypsinogen in the body, which is found in increased concentrations in the blood of CF patients.
The doctor may also perform a sweat test to calculate the amount of salt in your sweat and see whether it is higher than normal. They may also request X-rays of the chest or sinuses or a lung function test, which involves taking a deep breath in and then pushing the inhaled air into the spirometer’s mouthpiece.
How is cystic fibrosis treated?
Cystic fibrosis has no cure; however, various treatments can help manage symptoms and prevent complications, including:
Cystic fibrosis medications
- Antibiotics to prevent or treat lung infections.
- Anti-inflammatory drugs (such as Ibuprofen) to treat inflammation in your lungs’ airways.
- Inhaled bronchodilators to open the airways and clear your lungs.
- Inhaled mucus thinners to help thin mucus and get it out of your airways.
- Stool softeners to avoid constipation or bowel obstruction.
- CFTR modulators to help you gain weight and improve lung function.
- Gene-targeting medications
- Oral pancreatic enzymes to help with nutrient absorption in the digestive tract
Techniques for clearing the airway
- Oral devices that use vibrations to loosen mucus and make coughing easier.
- Chest physical therapy (CPT). This treatment requires you to move into specific positions and another person to clap their palm on your chest and back. CPT can help clear mucus from the airways and minimize inflammation.
- Pulmonary rehabilitation. Physical activity and breathing strategies can help improve your lung function and result in the removal of mucus and improved breathing.
Cystic fibrosis surgeries
- Surgery on the nose or sinuses to remove nasal polyps and treat recurrent or chronic sinusitis.
- A double lung transplant (for bronchiectasis, a condition that causes enlarged and infected airways) or a liver transplant (for liver damage).
- A feeding tube that can be surgically implanted in your abdomen to help with nutritional absorption and digestion.
Schedule your appointment at Novomed today!
If you have symptoms of cystic fibrosis, make an appointment with our expert pulmonologist in Dubai to learn more about your condition and treatment options.
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