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Cystic Fibrosis Symptoms

The signs and symptoms of cystic fibrosis vary, depending on how severe the disease is and the degree of bacterial infection. Almost always, a cough is present. Cough is usually worse in the morning and after exertion and usually produces very thick, yellow-, green-, tan-, or brown-colored mucus. The most common symptoms are: very salty-tasting skin; persistent coughing, wheezing or pneumonia; excessive appetite but poor weight gain; and bulky stools.

To understand cystic fibrosis symptoms a bit better, you need to know that sweat cools the body and that mucus lubricates the respiratory, digestive, and reproductive systems, and prevents tissues from drying out, protecting them from infection. People with cystic fibrosis lose excessive amounts of salt when they sweat, which can upset the balance of minerals in the blood, causing abnormal heart rhythms, shock, and other life-threatening symptoms. Patients with cystic fibrosis accumulate the thick mucus in their intestines and lungs. The result is malnutrition, poor growth, frequent respiratory infections, breathing difficulties, and eventually permanent lung damage. Lung disease is the usual cause of death in most patients.

There are periods when the symptoms of lung infection increase for those with cystic fibrosis. During this time, patients with cystic fibrosis may feel short of breath or fatigued; they may also have increased cough and/or sputum production. They may also experience sinus pain or tenderness, fever, weight loss and abnormal lung sounds such as wheezing and crackles. Crackles are lung sounds that occur during inspiration or when a breath is taken in. Just like their name, a crackling sound is produced by air entering the distal breathing passages. This is often associated with pneumonia, or heart failure.

As the chronic obstructive lung disease advances, the chest takes on a barrel-shaped appearance and clubbing of the finger and toenails occurs. Problems stemming from the gastrointestinal tract are the next most common feature. This might include difficulty passing stools, rectal prolapse; large, smelly, greasy stools; and failure to gain weight (even with a large appetite). Frequent hospitalizations are needed to treat recurring respiratory, gastrointestinal, and nutritional problems.

Cystic fibrosis is associated with various other medical problems. These include sinusitis (inflammation of the nasal sinuses, which are cavities in the skull behind, above, and on both sides of the nose), nasal polyps (fleshy growths inside the nose), clubbing (rounding and enlargement of fingers and toes), pneumothorax (rupture of lung tissue and trapping of air between the lung and the chest wall), hemoptysis (coughing of blood), cor pulmonale (enlargement of the right side of the heart), abdominal pain and discomfort, gassiness (too much gas in the intestine), and rectal prolapse (protrusion of the rectum through the anus). Liver disease, diabetes, inflammation of the pancreas, and gallstones also occur in some people with CF.

 

2024 American Association for Respiratory Care