This chapter provides a comprehensive update on the prevention, recognition, and treatment of low bone mineral density in people with CF. As life expectancy improves, the extra-pulmonary complications of CF are becoming increasingly important to quality of life. Up to 25 per cent of CF patients have reduced bone mineral density in adulthood, leading to the development of fragility fractures which cause pain, thereby interfering with airway clearance and predisposing to pulmonary infection. Osteoporosis can be a relative contraindication for lung transplantation.
Other important musculoskeletal issues including CF arthropathy, growth, and urinary incontinence are covered. CF arthropathy is a non-erosive episodic sero-negative arthritis, often difficult to treat and which may require specialist input. Urinary incontinence is common girls and women with CF and has a negative impact on quality of life and ability to complete therapies. The pathophysiology and management of urinary incontinence are discussed.