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Cystic Fibrosis

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Cystic Fibrosis

Cystic fibrosis, or CF, is a genetic disorder involving the lungs, pancreas, and other organs. The condition is caused by mutations in the CFTR g...
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Cystic Fibrosis: Symptom, Causes, Diagnosis and Treatment

Cystic fibrosis, or CF, is a genetic disorder involving the lungs, pancreas, and other organs. The condition is caused by mutations in the CFTR gene. These mutations lead to the production of abnormally thick, sticky mucus that can block the lungs, pancreas, and other organs. CF is a lifelong disease usually detected in childhood. It requires ongoing treatment to manage symptoms and complications. Manipal Hospitals offers multidisciplinary care for patients with  CF.

Symptoms of Cystic Fibrosis

CF symptoms vary but commonly include persistent cough with thick mucus, recurrent lung infections, wheezing, poor weight gain, and frequent respiratory illnesses. Patients may also experience digestive difficulties, greasy stools, or salty-tasting skin.

How We Diagnose Cystic Fibrosis

CF diagnosis includes a sweat chloride test that measures the amount of chloride (salt) in sweat. High chloride levels strongly suggest CF. Genetic testing may be used to detect mutations in the CFTR gene. Newborn screening tests are recommended to detect CF in infants. Diagnostic testing is then performed to confirm the diagnosis.

Treatment Options

CF treatment is mainly symptomatic and preventive. Treatments may include mucus-thinning drugs, airway clearance techniques, antibiotics to treat lung infections, and pancreatic enzyme replacement therapy. Nutritional support is critical to ensure adequate growth and health.

What to Expect

CF management at Manipal Hospitals requires long-term multidisciplinary care. This involves an experienced team of pulmonologists, gastroenterologists, nutritionists, and physiotherapists. Treatment is mainly focused on maintaining lung function, preventing infections, and supporting adequate nutrition.

Airway clearance forms an important part of CF treatment. These therapies help mobilise thick mucus from the lungs. This allows easier breathing and reduces the risk of respiratory infections. Bronchodilators, mucolytic agents, and inhaled antibiotics may be prescribed to improve airway function and control respiratory infections.

Doctors recommend pancreatic enzyme supplements for many people with CF to assist with nutrient absorption. They also provide nutritional guidance to support normal growth and weight maintenance. This may include a high-calorie diet and vitamin supplementation.

Routine monitoring helps doctors assess lung function, detect infections, and modify treatment when required. For some patients, specialised drugs called CFTR modulators help improve the function of the defective CFTR protein. Proper monitoring and appropriate treatment can help patients with CF lead active and fulfilling lives.

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