A new study has shown that cystic fibrosis (CF)-related mortality rates in the United States have declined over the past two decades, while the median age of death among CF patients has increased. The study, conducted by researchers from the Medical College of Wisconsin, analyzed CF-related mortality trends from 1999 to 2020 using data from the U.S. Centers for Disease Control and Prevention WONDER database.
The findings reveal that age-standardized mortality rates for CF decreased significantly, dropping from 1.9 to 1.04 per million population. The decline was especially prominent in recent years. This positive trend was observed in both sexes. Furthermore, the median age of death among CF patients increased from 24 to 37 years.
The study also looked at demographic factors and found decreases in mortality rates across various groups, including sex, White race, non-Hispanic ethnicity, census regions, and urbanization status. However, the trends were not consistent among patients of non-White race and Hispanic ethnicity. Additionally, it was discovered that the median age of death was lower among women, non-White races, and Hispanic ethnicity.
Interestingly, in 2020, a small percentage of CF decedents had severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection as the primary cause of death. This highlights the potential impact of the COVID-19 pandemic on CF patients, as the virus can pose a significant threat to individuals with underlying health conditions.
While the study acknowledges the observational nature of the data and cannot make definitive causal statements, the researchers speculate that the introduction of cystic fibrosis transmembrane conductance regulator modulator therapy may have played a role in the declining CF mortality rates. This therapy has shown promise in improving lung function and reducing lung infections in CF patients.
Cystic Fibrosis transmembrane conductance regulator (CFTR) modulators are drugs that target the underlying genetic defect in CF. These medications can improve the CFTR protein’s function, which regulates the movement of salt and fluid in and out of cells. By restoring CFTR protein function, these drugs help to alleviate some of the symptoms and complications associated with CF.
The introduction of CFTR modulator therapy has significantly advanced the treatment of CF. These drugs, such as ivacaftor, lumacaftor/ivacaftor, and lumacaftor/ivacaftor, have demonstrated efficacy in improving lung function, reducing exacerbations, and improving the overall quality of life for CF patients. Their ability to target the underlying cause of CF has provided new hope for those living with the disease.
Although the study highlights positive trends in CF-related mortality rates, it is essential to note that there are still many challenges and unmet needs in managing the disease. CF is a chronic and complex condition requiring comprehensive care and multidisciplinary approaches.
The findings of this study underscore the importance of continued research and advancements in CF treatment. As we unravel the complexities of the disease, it is crucial to develop new therapies that can further improve outcomes and extend the lives of CF patients. With ongoing efforts and advancements, we can aim for even more significant reductions in CF-related mortality rates and further increase the median age of death among CF patients.
Sources:
- https://www.nhlbi.nih.gov/health/cystic-fibrosis
- https://www.nhlbi.nih.gov/grants-and-training/funding-opportunities/cystic-fibrosis