The largest nonpulmonary cause of death in cystic fibrosis is a cystic fibrosis-related liver disease (CFLD) (CF). Researchers assessed and compared the illness load and nonrespiratory comorbidities of people with and without severe CFLD. A countrywide longitudinal study of severe CFLD patients compared to non-CFLD controls. A total of 166 patients with severe CFLD and 166 with no CFLD were found. Across all ages, the percentage of projected forced expiratory volume in 1 second was substantially lower in CFLD than in noCFLD. For respiratory indications, gastrointestinal indications, and other indications, hospitalizations per patient per year were greater in CFLD than in noCFLD. There was an increase in the usage of nasogastric and gastrostomy nutritional supplements in the CFLD cohort. Furthermore, the CFLD group had a greater prevalence of bone disorders, such as osteopenia and osteoporosis, as well as CF-related diabetes.

Patients with severe CFLD have a higher disease burden, including more hospitalizations (for both respiratory and nonrespiratory reasons), dietary treatments, and a higher risk of CF-related bone disease and diabetes.