Update on maintaining bone health in cystic fibrosis

Abstract

The high prevalence of decreased bone density in adults with cystic fibrosis is now well recognized, and guidelines for screening and treatment of cystic fibrosis-related bone disease have recently been published. This review summarizes the current best practices for optimizing bone health in cystic fibrosis and highlight recent findings that provide insight into the etiology of cystic fibrosis-related bone disease. Recent research suggests that cystic fibrosis-related bone disease actually starts during childhood, when individuals with cystic fibrosis fail to demonstrate normal bone calcium accretion. The failure to reach peak bone mass is made worse by increased osteoclast activity and bone resorption. This combination results in decreased bone density and an increased risk of fracture. Recent clinical studies suggest that multiple contributing factors need to be addressed in cystic fibrosis to optimize bone health: malnutrition, vitamin and mineral malabsorption, recurrent infections, inadequate sex hormones, and lack of exercise. Oral bisphosphonates have been demonstrated to be effective in cystic fibrosis and should be used when osteoporosis or progressive osteopenia is present. Research suggests cystic fibrosis-related bone disease actually begins during childhood, and guidelines now exist to aid in identifying and treating those with decreased bone density.