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International comparison of survival in Cystic Fibrosis between Canada, France and Australia.
Very nice work, Adele. This is really interesting. 1) Do you have some hypotheses as to why CF patients in France may have longer survival? Do you think it’s related to newborn screening? 2) What do you make of the fact that the KM curve for France crosses over the other ones around 75 years?
Thank you very much Dr Martinu, these are great questions. 1) We are showing very preliminary results, however we do have several suggestions on why France may have longer survival. As you suggested, newborn screening may indeed play an important role, since even the less severe patients are being captured. However, Australia also has had NBS for a very long time similarly to France, but survival in Australia is comparable to Canada. We still need to do further analysis. France has a high emergency lung transplant program that allows for CF patients who are eligible to receive a lung transplant very quickly. 2) The crossover would result in the fact that there are no deaths that occurred after the age of ~70 yrs in Canada and Australia (the curve suggests that there is no more dropping after that age). Also, in France, the cohort is much larger and patients are living longer therefore we capture more deaths at a later age.
This is very interesting, Adele. Do you know the % of people with different CFTR mutations? Do you think they may contribute to the differences in median age of survival among the countries?
Great question Kenneth, we have not done this analysis yet, it is definitely in our plans. We do think it might contribute because it seems that in France, there is a higher proportion of “other mutations”, compared to Canada and Australia, and we suggest that the “other mutations” group represent less severe patients.
Very nice work. What if you look at patients with the same mutations – i..e just deltaF homozygotes. Do you see the same difference? There seemed to be fewer delta homozygotes in France. Interesting that there seemed to be more underweight patients in France. Do they have a different approach? Do you think newborn screening leads to better care or to detection of mild cases that might otherwise have gone undiagnosed?
Thank you Dr Binnie for this great question. In fact, there are fewer delta homozygous patients in France. We did look at survival in delta homozygous patients only, although we still observed a similar trend, the difference in median survival was much less important (48.4 Canada, 45.5 Australia and 52.2 France). It was striking to see that the proportion of underweight patients in France was high compared to Canada and Australia. Nutritionally, I believe they have a different approach, but I should confirm with the French (it is a great point to look into). However, it should be noted that in the general non-CF population, French people are “skinnier” than the general Canadian and Australia population (we actually compared statistics from each country). I believe that yes, newborn screening should lead to better care and certainly detection of mild cases that might otherwise have gone undiagnosed or diagnosed much later in life. We are still doing analysis to determine the impact of NBS on survival between countries.
Also what are the results if you exclude cepacia?
Another important question that we will definitely be looking into in the upcoming analysis, considering the negative impact of cepacia on survival in CF patients. Great question!
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