Treatment of bone disease in cystic fibrosis

As individuals with cystic fibrosis (CF) have experienced marked improvements in longevity over the last three decades, bone disease has emerged as a new problem. Bone disease in CF has not been previously reviewed in this journal. Therefore, this review will give a brief overview of bone disease in CF and then concentrate on treatment options.

In some series, as many as three fourths of adults with CF have low bone density. Decreased absorption of fat-soluble vitamins due to pancreatic insufficiency, altered sex hormone production, chronic inflammation, physical inactivity, and glucocorticoid treatment are some of the factors that contribute to this problem. Vitamin D depletion most likely contributes to bone disease, but identifying the safest and most efficacious vitamin D supplementation has yet to be resolved. Calcium and vitamin K supplementations are important if the diet contains less than the recommended amounts. Treatment of delayed puberty and adult hypogonadism with hormone replacement is recommended to achieve peak bone mass and maintain bone density. Bisphosphonates, including pamidronate and alendronate, are beneficial in improving bone mineral density before and after transplantation in CF adults. Bisphosphonates have not been studied in CF children.

Although much progress has been made in our understanding of the pathogenesis, natural history, and clinical manifestations of bone disease in CF, treatment options are still evolving. More attention to nutrition, in terms of the maintenance of lean body mass and vitamin D and calcium supplementation, is likely to decrease bone complications. Bisphosphonates can be of value in CF adults with low bone density. Several clinical trials are under way to help optimize the treatment of CF bone disease.