Altered life expectancy?

[just1more]

Ok, at risk of getting in trouble for being a bit too honest/realistic I have read this thread several times and can't help but respond.

First of all yes, I do think that some of the improvements have improved lifespan. There is no doubt our ability to manage infections better has prolonged life. Likewise, the increased availability and technology for transplants has helped extend life in the period that would otherwise be the 'end-stage'.

However I work with numbers and statistics for a career. And because of that we need to be careful as statistics only say what you want them to say. A median life exectancy of 36 (per cff) means that of those diagnosed with CF, 1/2 will die before 36 and 1/2 after 36. It makes NO estimate for any single person or group of persons.

While we still lose way too many in their 20's, a median of 36 is much better than it was a few decades ago, for several reasons.

Some of those are things such as Pulmozyme & Tobi; the introduction of the vest to improve the effectiveness of CPT; more agressive treatment of infections and complications; and increased options for tx (which alone can add years).

However, as I said statistics are not 100%. The reason I say this is that 20 years ago, only the most 'typical' cystics were diagnosed and thus the statistics were based primarily upon those with early symptoms and classic progression. With the increased awareness of CF, and the introduction of genetic testing, allowing for 'a-typical' or adult diagnoses, we have increased the CF population being used.

While I feel it is great that we are better able to identify and help ALL cystics, not just thoses dx'd as a baby, everytime someone is dx'd in their 30's or 40's we are adding someone who 20 years ago would have never been considered to have cf.

Unfortunately, in my opinion, this makes comparisons of a statistics on life-expectancy a poor determinant of progress in treatment so we must focus on the quality of life and providing the best we can. Ultimately, nobody knows how long any of us will live, so live each day like it is your last and have no regrets.

 

[just1more]

Ok, at risk of getting in trouble for being a bit too honest/realistic I have read this thread several times and can't help but respond.

First of all yes, I do think that some of the improvements have improved lifespan. There is no doubt our ability to manage infections better has prolonged life. Likewise, the increased availability and technology for transplants has helped extend life in the period that would otherwise be the 'end-stage'.

However I work with numbers and statistics for a career. And because of that we need to be careful as statistics only say what you want them to say. A median life exectancy of 36 (per cff) means that of those diagnosed with CF, 1/2 will die before 36 and 1/2 after 36. It makes NO estimate for any single person or group of persons.

While we still lose way too many in their 20's, a median of 36 is much better than it was a few decades ago, for several reasons.

Some of those are things such as Pulmozyme & Tobi; the introduction of the vest to improve the effectiveness of CPT; more agressive treatment of infections and complications; and increased options for tx (which alone can add years).

However, as I said statistics are not 100%. The reason I say this is that 20 years ago, only the most 'typical' cystics were diagnosed and thus the statistics were based primarily upon those with early symptoms and classic progression. With the increased awareness of CF, and the introduction of genetic testing, allowing for 'a-typical' or adult diagnoses, we have increased the CF population being used.

While I feel it is great that we are better able to identify and help ALL cystics, not just thoses dx'd as a baby, everytime someone is dx'd in their 30's or 40's we are adding someone who 20 years ago would have never been considered to have cf.

Unfortunately, in my opinion, this makes comparisons of a statistics on life-expectancy a poor determinant of progress in treatment so we must focus on the quality of life and providing the best we can. Ultimately, nobody knows how long any of us will live, so live each day like it is your last and have no regrets.

 

[just1more]

Ok, at risk of getting in trouble for being a bit too honest/realistic I have read this thread several times and can't help but respond.

First of all yes, I do think that some of the improvements have improved lifespan. There is no doubt our ability to manage infections better has prolonged life. Likewise, the increased availability and technology for transplants has helped extend life in the period that would otherwise be the 'end-stage'.

However I work with numbers and statistics for a career. And because of that we need to be careful as statistics only say what you want them to say. A median life exectancy of 36 (per cff) means that of those diagnosed with CF, 1/2 will die before 36 and 1/2 after 36. It makes NO estimate for any single person or group of persons.

While we still lose way too many in their 20's, a median of 36 is much better than it was a few decades ago, for several reasons.

Some of those are things such as Pulmozyme & Tobi; the introduction of the vest to improve the effectiveness of CPT; more agressive treatment of infections and complications; and increased options for tx (which alone can add years).

However, as I said statistics are not 100%. The reason I say this is that 20 years ago, only the most 'typical' cystics were diagnosed and thus the statistics were based primarily upon those with early symptoms and classic progression. With the increased awareness of CF, and the introduction of genetic testing, allowing for 'a-typical' or adult diagnoses, we have increased the CF population being used.

While I feel it is great that we are better able to identify and help ALL cystics, not just thoses dx'd as a baby, everytime someone is dx'd in their 30's or 40's we are adding someone who 20 years ago would have never been considered to have cf.

Unfortunately, in my opinion, this makes comparisons of a statistics on life-expectancy a poor determinant of progress in treatment so we must focus on the quality of life and providing the best we can. Ultimately, nobody knows how long any of us will live, so live each day like it is your last and have no regrets.

 

[just1more]

Ok, at risk of getting in trouble for being a bit too honest/realistic I have read this thread several times and can't help but respond.

First of all yes, I do think that some of the improvements have improved lifespan. There is no doubt our ability to manage infections better has prolonged life. Likewise, the increased availability and technology for transplants has helped extend life in the period that would otherwise be the 'end-stage'.

However I work with numbers and statistics for a career. And because of that we need to be careful as statistics only say what you want them to say. A median life exectancy of 36 (per cff) means that of those diagnosed with CF, 1/2 will die before 36 and 1/2 after 36. It makes NO estimate for any single person or group of persons.

While we still lose way too many in their 20's, a median of 36 is much better than it was a few decades ago, for several reasons.

Some of those are things such as Pulmozyme & Tobi; the introduction of the vest to improve the effectiveness of CPT; more agressive treatment of infections and complications; and increased options for tx (which alone can add years).

However, as I said statistics are not 100%. The reason I say this is that 20 years ago, only the most 'typical' cystics were diagnosed and thus the statistics were based primarily upon those with early symptoms and classic progression. With the increased awareness of CF, and the introduction of genetic testing, allowing for 'a-typical' or adult diagnoses, we have increased the CF population being used.

While I feel it is great that we are better able to identify and help ALL cystics, not just thoses dx'd as a baby, everytime someone is dx'd in their 30's or 40's we are adding someone who 20 years ago would have never been considered to have cf.

Unfortunately, in my opinion, this makes comparisons of a statistics on life-expectancy a poor determinant of progress in treatment so we must focus on the quality of life and providing the best we can. Ultimately, nobody knows how long any of us will live, so live each day like it is your last and have no regrets.

 

[just1more]

Ok, at risk of getting in trouble for being a bit too honest/realistic I have read this thread several times and can't help but respond.
<br />
<br />First of all yes, I do think that some of the improvements have improved lifespan. There is no doubt our ability to manage infections better has prolonged life. Likewise, the increased availability and technology for transplants has helped extend life in the period that would otherwise be the 'end-stage'.
<br />
<br />However I work with numbers and statistics for a career. And because of that we need to be careful as statistics only say what you want them to say. A median life exectancy of 36 (per cff) means that of those diagnosed with CF, 1/2 will die before 36 and 1/2 after 36. It makes NO estimate for any single person or group of persons.
<br />
<br />While we still lose way too many in their 20's, a median of 36 is much better than it was a few decades ago, for several reasons.
<br />
<br />Some of those are things such as Pulmozyme & Tobi; the introduction of the vest to improve the effectiveness of CPT; more agressive treatment of infections and complications; and increased options for tx (which alone can add years).
<br />
<br />However, as I said statistics are not 100%. The reason I say this is that 20 years ago, only the most 'typical' cystics were diagnosed and thus the statistics were based primarily upon those with early symptoms and classic progression. With the increased awareness of CF, and the introduction of genetic testing, allowing for 'a-typical' or adult diagnoses, we have increased the CF population being used.
<br />
<br />While I feel it is great that we are better able to identify and help ALL cystics, not just thoses dx'd as a baby, everytime someone is dx'd in their 30's or 40's we are adding someone who 20 years ago would have never been considered to have cf.
<br />
<br />Unfortunately, in my opinion, this makes comparisons of a statistics on life-expectancy a poor determinant of progress in treatment so we must focus on the quality of life and providing the best we can. Ultimately, nobody knows how long any of us will live, so live each day like it is your last and have no regrets.

 

[Lex]

Great post, Just1more. That's an angle I never thought of before.

 

[Lex]

Great post, Just1more. That's an angle I never thought of before.

 

[Lex]

Great post, Just1more. That's an angle I never thought of before.

 

[Lex]

Great post, Just1more. That's an angle I never thought of before.

 

[Lex]

Great post, Just1more. That's an angle I never thought of before.

 

[NYCLawGirl]

Tom, what a great and insightful post. I have been thinking about that as well - the number of CF cases diagnosed as adults now (i.e., people living without any treatments at all into adulthood with lesser-known mutations) has dramatically increased. The CFF's life expectancy numbers are taken from clinic statistics, and most adults diagnosed later begin attending CF clinics post-daignosis, meaning they begin to be factored into statistics (as well they should). So you do need to take those numbers with a huge grain of...um...salt <img src="">

Another thought to add: transplant has recently become so much more widespread than it was even 10 years ago. I had a number of CF friends who died as we reached our later teen years, and very few of them even considered the option of transplant. Now, it has reached the point where most end-stage CFers consider transplant as a last-resort treatment, and that means that a good number of us are now "outliving" our old CF lungs. It's not a CF treatment, per se, in the same way that DNase or TOBI are, but it definitely has an impact on life expectancy.

It's undeniable that we've made progress. So many of us, myself included, have WAY outlived the ages our parents were told to expect, and there's no reason not to believe that a good number of CFers will continue to do so even as that number goes up. So basically I would say be optimistic, plan for the future, and be extremely grateful for all the major advances...but it's still not nearly enough!

 

[NYCLawGirl]

Tom, what a great and insightful post. I have been thinking about that as well - the number of CF cases diagnosed as adults now (i.e., people living without any treatments at all into adulthood with lesser-known mutations) has dramatically increased. The CFF's life expectancy numbers are taken from clinic statistics, and most adults diagnosed later begin attending CF clinics post-daignosis, meaning they begin to be factored into statistics (as well they should). So you do need to take those numbers with a huge grain of...um...salt <img src="">

Another thought to add: transplant has recently become so much more widespread than it was even 10 years ago. I had a number of CF friends who died as we reached our later teen years, and very few of them even considered the option of transplant. Now, it has reached the point where most end-stage CFers consider transplant as a last-resort treatment, and that means that a good number of us are now "outliving" our old CF lungs. It's not a CF treatment, per se, in the same way that DNase or TOBI are, but it definitely has an impact on life expectancy.

It's undeniable that we've made progress. So many of us, myself included, have WAY outlived the ages our parents were told to expect, and there's no reason not to believe that a good number of CFers will continue to do so even as that number goes up. So basically I would say be optimistic, plan for the future, and be extremely grateful for all the major advances...but it's still not nearly enough!

 

[NYCLawGirl]

Tom, what a great and insightful post. I have been thinking about that as well - the number of CF cases diagnosed as adults now (i.e., people living without any treatments at all into adulthood with lesser-known mutations) has dramatically increased. The CFF's life expectancy numbers are taken from clinic statistics, and most adults diagnosed later begin attending CF clinics post-daignosis, meaning they begin to be factored into statistics (as well they should). So you do need to take those numbers with a huge grain of...um...salt <img src="">

Another thought to add: transplant has recently become so much more widespread than it was even 10 years ago. I had a number of CF friends who died as we reached our later teen years, and very few of them even considered the option of transplant. Now, it has reached the point where most end-stage CFers consider transplant as a last-resort treatment, and that means that a good number of us are now "outliving" our old CF lungs. It's not a CF treatment, per se, in the same way that DNase or TOBI are, but it definitely has an impact on life expectancy.

It's undeniable that we've made progress. So many of us, myself included, have WAY outlived the ages our parents were told to expect, and there's no reason not to believe that a good number of CFers will continue to do so even as that number goes up. So basically I would say be optimistic, plan for the future, and be extremely grateful for all the major advances...but it's still not nearly enough!

 

[NYCLawGirl]

Tom, what a great and insightful post. I have been thinking about that as well - the number of CF cases diagnosed as adults now (i.e., people living without any treatments at all into adulthood with lesser-known mutations) has dramatically increased. The CFF's life expectancy numbers are taken from clinic statistics, and most adults diagnosed later begin attending CF clinics post-daignosis, meaning they begin to be factored into statistics (as well they should). So you do need to take those numbers with a huge grain of...um...salt <img src="">

Another thought to add: transplant has recently become so much more widespread than it was even 10 years ago. I had a number of CF friends who died as we reached our later teen years, and very few of them even considered the option of transplant. Now, it has reached the point where most end-stage CFers consider transplant as a last-resort treatment, and that means that a good number of us are now "outliving" our old CF lungs. It's not a CF treatment, per se, in the same way that DNase or TOBI are, but it definitely has an impact on life expectancy.

It's undeniable that we've made progress. So many of us, myself included, have WAY outlived the ages our parents were told to expect, and there's no reason not to believe that a good number of CFers will continue to do so even as that number goes up. So basically I would say be optimistic, plan for the future, and be extremely grateful for all the major advances...but it's still not nearly enough!

 

[NYCLawGirl]

Tom, what a great and insightful post. I have been thinking about that as well - the number of CF cases diagnosed as adults now (i.e., people living without any treatments at all into adulthood with lesser-known mutations) has dramatically increased. The CFF's life expectancy numbers are taken from clinic statistics, and most adults diagnosed later begin attending CF clinics post-daignosis, meaning they begin to be factored into statistics (as well they should). So you do need to take those numbers with a huge grain of...um...salt <img src="">
<br />
<br />Another thought to add: transplant has recently become so much more widespread than it was even 10 years ago. I had a number of CF friends who died as we reached our later teen years, and very few of them even considered the option of transplant. Now, it has reached the point where most end-stage CFers consider transplant as a last-resort treatment, and that means that a good number of us are now "outliving" our old CF lungs. It's not a CF treatment, per se, in the same way that DNase or TOBI are, but it definitely has an impact on life expectancy.
<br />
<br />It's undeniable that we've made progress. So many of us, myself included, have WAY outlived the ages our parents were told to expect, and there's no reason not to believe that a good number of CFers will continue to do so even as that number goes up. So basically I would say be optimistic, plan for the future, and be extremely grateful for all the major advances...but it's still not nearly enough!

 

[MargaritaChic]

<div class="FTQUOTE"><begin quote><i>Originally posted by: <b>LouLou</b></i>
The good news is that denufusol and vx-770 both are in this direction.</end quote></div>

Don't forget PTC124 (for those with Stop mutations).

 

[MargaritaChic]

<div class="FTQUOTE"><begin quote><i>Originally posted by: <b>LouLou</b></i>
The good news is that denufusol and vx-770 both are in this direction.</end quote></div>

Don't forget PTC124 (for those with Stop mutations).

 

[MargaritaChic]

<div class="FTQUOTE"><begin quote><i>Originally posted by: <b>LouLou</b></i>
The good news is that denufusol and vx-770 both are in this direction.</end quote></div>

Don't forget PTC124 (for those with Stop mutations).

 

[MargaritaChic]

<div class="FTQUOTE"><begin quote><i>Originally posted by: <b>LouLou</b></i>
The good news is that denufusol and vx-770 both are in this direction.</end quote>

Don't forget PTC124 (for those with Stop mutations).

 

[MargaritaChic]

<div class="FTQUOTE"><begin quote><i>Originally posted by: <b>LouLou</b></i>
<br />The good news is that denufusol and vx-770 both are in this direction.</end quote>
<br />
<br />Don't forget PTC124 (for those with Stop mutations).
<br />
<br />