A study published by Abhinav Agrawal
and colleagues in Intractable Rare Disease Research
indicates that as the management of cystic fibrosis has dramatically improved, the advancement and progression has come at a significant financial cost.
CF is a chronic genetic disorder due to a faulty or missing a cystic fibrosis transmembrane conductance regulator (CFTR) protein that results in lung and digestive system damage. CF patients are very susceptible to infections due to the thick and sticky mucus that tends to inhabit their lungs and GI tract. When infections occur, problems become amplified because the underlying condition leading to CF exacerbations can require hospitalizations.
Ironically, as management of CF has improved, the number of pulmonary exacerbations has increased for one simple reason: CF patients are living longer, meaning there are more of them. In 1986, most CF patients did not live long into adulthood and adults only accounted for 29% of the patient population. In 2013, 51.6% of CF patients are adults.
Additionally, the median predicted survival of patients with CF in 1970 was 16 years, but increased to 40 years by 2013.
As CF patients age, there is a higher risk of CF exacerbations; with that comes hospitalizations, which is where the costs of care have skyrocketed.
Using data from the National Inpatient Sample Database (NIS)
in the United States,
Agarawal et al determined there were 8,328 total hospital stays involving CF patients in 2003 and 12,590 in 2013 (P
< .001). The cost of each hospital stay increased from a mean of $60,051 in 2003 to $94,664 in 2013, even though length of each stay remained moderately steady during those 2 years (mean length of stay in 2003 was 10.1 days compared to 10.4 days in 2013).
Based on these statistics, the researchers calculated that the aggregate cost of hospital visits increased from $500,105,727 in 2003 to $1,191,819,760 in 2013 for patients with CF.
The authors concluded that the “Rising hospital costs associated with the care of CF patients necessitates future studies analyzing the diagnostic modalities, algorithms and treatment practices of physician's treating CF patients.”
Agrawal A, Agarwal A, Mehta D, et al. Nationwide trends of hospitalizations for cystic fibrosis in the United States from 2003 to 2013. Intractable Rare Dis Res