Cystic Fibrosis-Related Bone Disease in Youth: High-Risk Population With Low Screening Adherence

Cystic fibrosis (CF)-related bone disease (CFBD) is characterized by low bone mineral density and increased fracture risk. The U.S. CF Foundation recommends using dual-energy x-ray absorptiometry (DXA) to screen all adults and at-risk children 8 to <18 years. We aimed to establish the proportions of children who meet CFBD screening criteria, are ordered a DXA, and complete DXA screening.

The medical charts of children seen in our pediatric CF clinic in 2022 were reviewed to identify who met criteria for CFBD screening by DXA per CF Foundation guidelines: prior fracture, delayed puberty, chronic glucocorticoid use, underweight, or poor lung function. The medical charts of all patients followed in our pediatric clinic were reviewed to extract childhood fracture history.

In this cohort of 118 children aged 8 to <18 years, 28% met criteria for CFBD screening by DXA. Only one child who met criteria for DXA screening in 2022 had a DXA ordered. Fracture history was the most common reason to meet screening criteria. For all patients followed in the clinic, the estimated cumulative incidence of childhood fracture was 53%.

Children with CF are experiencing fractures throughout their childhood and should undergo further evaluation for skeletal fragility, beginning with detailed fracture history. However, few children are completing DXA scans according to national guidelines. In the future, barriers to pediatric DXA screening must be identified and overcome to improve adherence to national guidelines. Addressing these gaps will be vital to optimizing bone health management in children with CF.