Cystic fibrosis (CF) is a condition known to be linked to lung problems, but bone health can also be impacted in people with the disease. A new paper from researchers in the United Kingdom focuses on this problem. The report, “Bone health and disease in cystic fibrosis,“ appeared in the journal Pediatric Respiratory Reviews.
Cystic fibrosisis one of the most commonly occurring chronic diseases of the lungs in children and young adults, and can be a life-threatening disorder. Breathing is often difficult for people with cystic fibrosis, due to a sticky mucus that builds up in the lungs and all too often leads to serious bacterial lung infections that can be fatal.
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.”
Bisphosphonates prevent bone loss and are the most commonly prescribed drugs used to treat osteoporosis.
The study emphasizes the importance of monitoring bone health in individuals with cystic fibrosis.