Pediatric endocrinologist Malinda Wu discusses a session she moderated on cystic fibrosis-related bone disease during the 2023 North American Cystic Fibrosis Conference. Cystic fibrosis is most known for causing lung disease, but it affects most organs in the body, including the pancreas and the skeletal system. People with cystic fibrosis are at higher risk for having fractures with low trauma than people without cystic fibrosis.
Hello, my name is Melinda Wu. I am a pediatric endocrinologist at Johns Hopkins at the 2023 North American Cystic fibrosis conference. I moderated a session about cystic fibrosis related bone disease. I was joined by Sarah Hendrix from M US and Caroline Chaloner from Maine Medical Center. Cystic fibrosis is a genetic disorder caused by defective CFTR channels. CF is most commonly known for causing lung disease but affects most organs in the body including the pancreas and the skeletal system. People with cystic fibrosis are at higher risk for having fractures with low trauma than people without cystic fibrosis. In some reports, young adults with cystic fibrosis were 100 times more likely to have a vertebral compression fracture than the general population. Osteoporosis in people with CF is diagnosed much like pediatric and young adult osteoporosis based on a combination of low bone density for age and a significant fracture history. Even before being diagnosed with osteoporosis. Healthy people with CF already have different bone micro architecture, including a thinner cortex, decreased trabecular cross sectional area and thinner trabecula with a lower calculated failure load compared to healthy people without CF. Bones are a dynamic organ, continuously undergoing formation and resorption. In childhood. There is more formation as people are accruing bone before they reach their peak bone mass. In young adulthood, people with CF reach a lower peak bone density than people without CF. We reviewed recommendations for screening Children with CF with risk factors for CF, bone disease including chronic high dose glucocorticoid use poor lung function being underweight, delayed puberty and a history of fractures. The screening test recommended for Children is a dexa scan, measuring bone mineral density at the lumbar spine and total body less head. We reviewed recommendations for optimizing nutrition and physical activity to optimize bone health and also highlighted the need for research specific to optimizing bone health of people with cystic fibrosis. As many of the recommendations have been extrapolated from people without CF. In addition to adequate calcium and vitamin D intake, many people with CF are pancreatic, insufficient and thus unable to absorb vitamin D without taking pancreatic enzymes, protein intake and maintaining lean muscle mass are also key to maintaining bone health and people with CF have increased protein needs than people without CF, adequate intake of vitamins K A and C and magnesium are also important for bone formation. Weight bearing exercise is a well known potent stimulus for bone formation. Children with CF would benefit from high impact weight bearing exercise several times per week. In addition to regular moderate intensity exercise, adults with CF may benefit from a combination of strength training and aerobic activity to maintain their bone and muscle mass bisphosphonates are the most well studied anti osteoporosis medication for people with CF. But other osteoporosis agents, including ones that promote bone formation have been used to increase bone density in people with CFCFTR modulators which are a CF specific treatment have not consistently shown improvements in people's bone density. More study is needed to understand risk factors and predictors for fracture risk in people with CF. Thank you.
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