A recent study emphasizes the importance of monitoring bone health in individuals with cystic fibrosis.
Mutations in the CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) gene are the major cause of this mucus build-up. The CFTR protein helps shuttle chloride ions in and out of cells, balancing salt and water in the cells that line the lungs. When CFTR is mutated, there is an impairment in this process, leading to the overproduction of thick mucus in the lungs.
Although improvements in breathing and reduction in lung mucus is a major focus of treatment for cystic fibrosis, bone health can also be impacted by the disease. According to the report, authored by Malcolm Marquette and Charles Haworth of the Adult Cystic Fibrosis Centre at Papworth Hospital in England, bone mineral density is often low in cystic fibrosis patients due to effects caused by problems with the CFTR gene.
The authors also suggest that cystic fibrosis effects the pancreas, which can, in turn, impact bone health by reducing bone density. Pulmonary infections can add to the impact on bone resorption (remodeling that normally occurs in bones), further decreasing bone density.
The authors concluded in their report that, “Strategies to improve bone health in cystic fibrosis include optimizing general cystic fibrosis nutritional and pulmonary care and the judicious use of bisphosphonates in selected patients. CFTR correctors/potentiators may have positive impact on bone metabolism in people with CF.”