Guide to Bone Health and Disease in Cystic Fibrosis

Abstract

Cystic fibrosis (CF) is the most common genetic disease within the Caucasian population and leads to premature respiratory failure. Approximately 60,000 individuals are currently living with CF in North America and Europe, 40% of whom are adults. The life span of these patients has increased from approximately 2 to 32 yr of age over the last three decades. Bone disease has emerged as a common complication in long-term survivors of CF. Some studies have observed that 50-75% of adults have low bone density and increased rates of fractures. Prevention and treatment of CF-related bone disease must address the myriad risk factors (decreased absorption of fat-soluble vitamins due to pancreatic insufficiency, altered sex hormone production, chronic lung infection with increased levels of bone-active cytokines, physical inactivity, and glucocorticoid therapy) for poor bone health. This review is a condensed and updated summary of the Guide to Bone Health and Disease in Cystic Fibrosis: A Consensus Conference, a statement that evolved from a meeting convened by the Cystic Fibrosis Foundation in May 2002 to address the pathogenesis, diagnosis, and treatment of bone disease in CF. The goal of this conference was to develop practice guidelines for optimizing bone health in patients with CF.

Failure of High-Dose Ergocalciferol to Correct Vitamin D Deficiency in Adults with Cystic Fibrosis

“…These efforts culminated in the 2005 publication of the guidelines from the CFF Consensus Conference on Bone Health in which detailed recommendations for screening and treatment of vitamin D deficiency were outlined (17). Conference leaders based treatment recommendations on experience in individuals without CF and stated that the recommendations may need to be revised as CF-specific data became available.…”

“…Because of the growing concern about bone disease in CF, the U.S. Cystic Fibrosis Foundation (CFF) convened a panel of experts to develop consensus treatment guidelines to optimize CF bone health, and these recommendations were recently published (17). The guidelines contained several specific recommendations regarding vitamin D. First, greater attention to maintaining adequate serum 25-OHD levels should occur, with levels being checked annually in the late fall with the goal of maintaining serum 25-OHD between 30 and 60 ng/ml (75-150 nmol/L).…”

Lactose malabsorption is a risk factor for decreased bone mineral density in pancreatic insufficient cystic fibrosis patients

“…To some extent, this does seem to be justified, because BMD reflects bone health over the lifetime of the patient, and current vitamin D concentrations may fluctuate from season to season. 1,25 On the other hand, both parameters are simply related to LM. Nonetheless, the use of logistic regression for analysis of the study results has shown the strong relationship of BMD with both body weight (Po0.025) and LM (Po0.044).…”

“…Approximately 60 000 individuals with CF are currently living in North America and Europe, 40% of whom are adults. 1 Although bone disease in CF was first described in 1979, 2,3 it has only recently received the proper attention as a result of the increase in life expectancy of CF patients. Their life span has increased from approximately 2 to 37 years of age over the past 3 decades, 4 a time period which allows for the occurrence of bone disease.…”

“…Severe bone disease may result in exclusion in qualification for lung transplantation, a life-saving treatment for individuals with CF. 1,5 Patients with CF have many risk factors for reduced BMD. Important contributing conditions include: malabsorption of vitamin D, 6 poor nutritional status, 7,8 low physical activity, 9 glucocorticoid therapy 7 and delayed pubertal maturation or early hypogonadism.…”