Managing the costs of CF vary widely, are likely underestimated

Most studies report healthcare system costs, such as hospitalizations

Steve Bryson, PhD avatar

by Steve Bryson, PhD |

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The total cost of managing cystic fibrosis (CF) — healthcare costs, patient and caregiver costs, as well as costs to society — varied widely per patient per year in a review of published studies.

Most studies only reported costs to the healthcare system, such as hospitalizations and healthcare visits, but out-of-pocket expenses to the individual seldom were, meaning the total cost of CF is likely underestimated, the researchers said.

Details of the cost analysis were published in the Journal of Cystic Fibrosis, in the study, “Measuring the burden of cystic fibrosis: A scoping review.”

The buildup of sticky and thick mucus in various organs, including the lungs, digestive tract, and reproductive system, is a feature of the inherited disease. Its  symptoms and complications vary from person to person and can ease or worsen over time.

Although CF is a rare disease, it places a substantial economic burden on healthcare systems due to frequent hospitalizations, regular clinic visits, various procedures, and medications. Patients and caregivers also incur costs in time and money, and productivity losses can burden society as a whole.

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An illustration of healthcare costs, showing a cracked piggy bank with a bandage and a bottle of prescription medication.

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Understanding costs of CF

Several CF cost-of-illness studies conducted in different countries have been reported, but the findings have been inconsistent, leading researchers in Canada to review studies published between 1990 and 2022 that described the cost of illness related to CF.

“Understanding the economic impact of CF is crucial for planning resource allocation,” wrote the researchers, who analyzed 39 studies that included between five and more than 6,000 participants. Among them, 22 (56.4%) were conducted in Europe, 15 (38.5%) in North America, and two (5.1%) in Australia. More than half (59%) were published in the last 10 years.

Most estimated CF-related costs using a bottom-up approach (59%), with data collected from individual patients. Others followed a prevalence-based approach (46.2%), whereby the total costs were estimated during a given period.

The most frequently reported direct costs (87.2%) were from medication, followed by inpatient care (84.6%), then outpatient care (79.5%). Less frequently reported costs were for diagnostic tests and added health professionals, such as dieticians and therapists, along with out-of-pocket expenses incurred by patients or caregivers. Direct non-healthcare costs reported were transportation (30.8%), childcare, housekeeping or domestic help (23.1%), and vitamins and supplements (17.9%).

Just under a third of the studies examined CF costs from three perspectives — individual costs, healthcare system (payer), and society, with about half reporting costs from the healthcare system only. Indirect costs, such as productivity losses, job or school absenteeism, and time lost for treatments or travel, were included in a fifth of the studies.

As reported in 2022 U.S. dollars adjusted for inflation, the total cost of CF varied widely, ranging from $415 to $160,000 per person per year. The lowest cost reported was related to outpatient care and diagnostic tests, whereas the highest was related to treatment with Kalydeco (ivacaftor), an approved CFTR modulator.

Costs for adults outmatched those for children in all but one study.

“Whereas the direct costs of CF are consistently greater in adults,” one study showed that “indirect costs of caregiving are much higher for caregivers of children with CF than adults,” the researchers said.

Costs were higher among those with more severe lung disease. Comparisons between sex and/or gender were inconsistent across studies, with some finding no difference and others showing higher costs for either males or females.

“Cost of illness studies are important to capture and summarize the burden of CF for individuals, healthcare systems and society,” the researchers said. “Most studies only reported costs to the healthcare system (i.e., hospitalizations and healthcare encounters), which likely underestimates the total costs of CF. The lack of standardized tools and reporting of costs makes it difficult to compare studies or generalize findings.”