Muscle Loss in Cystic Fibrosis

People with cystic fibrosis experience difficulty breathing. Often, this is because of severe infection in the lungs or progressive damage to lung tissue due to inflammation. However, breathing problems can be worsened by muscle loss in the diaphragm – the muscle used for breathing.

Muscle loss in people with CF had been thought to be a result of poor nutrient absorption from food and a lack of exercise during long periods of ill health. However, a study by Dr. Basil Petrof at McGill University in Montreal shows that muscle loss and weakness in people with CF may sometimes be a result of the same genetic mutation that causes CF. It seems that CFTR – the gene responsible for CF – also plays a role in keeping muscle cells healthy, and when this gene is altered, as in cystic fibrosis, muscle cells can become weak. Lung infection and inflammation can make the problem worse.

These findings may help researchers develop new therapies that target muscle loss in people with CF. By keeping the diaphragm strong, people with CF may better survive periods of respiratory distress.

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Reviewed/updated: 2010-02-09