tough topic- portrayed life expectancy

imported_Momto2

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<p style="font-weight: normal;" class="MsoNormal">OK, I know
this is a loaded subject.    I'm a 40
year old DDF508, diagnosed at 3 months after severe failure to thrive, pneumnia, pneumothorax, etc, etc.  
Over the years, I have met about 20 people with CF, and with the
exception of 2, all were all younger than I.   
At this point, they have all passed away prior to age 34, and I am the
last of my childhood cohort.   What I do not understand is how many pamphlets and websites state that "people with CF
can now live relatively healthy and normal lives" and that the "life expectancy
is now 37 and climbing".    How can this
possibly be true?   Are they talking
about children born today have that life expectancy?   Or due to genetic testing are they
including people diagnosed at age 50 or even 60 into the stats to make them
look a heck of a lot better?    These
statements go against everything I have seen and experienced.  Now, I've never met someone with "mild CF",
although I know from the internet that they certainly exist.   It seems much more honest to have CF broken up into a
few categories of mild, moderate, severe, and give the stats for that.  I look at the pamphlets, read the stuff, and shake my head.   I know the powers that be dont want to scare kids and new CF parents, but I think they often trivialize the disease.  I have seen plenty of parents of young kids and teens who are really struggling.   Anyone have an opinion on this?  Similar experiences, similar questions?  I dont know who else to ask..........<p style="font-weight: normal;" class="MsoNormal"><br>
 

imported_Momto2

New member
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<p style="font-weight: normal;" class="MsoNormal">OK, I know
this is a loaded subject. I'm a 40
year old DDF508, diagnosed at 3 months after severe failure to thrive, pneumnia, pneumothorax, etc, etc.
Over the years, I have met about 20 people with CF, and with the
exception of 2, all were all younger than I.
At this point, they have all passed away prior to age 34, and I am the
last of my childhood cohort. What I do not understand is how many pamphlets and websites state that "people with CF
can now live relatively healthy and normal lives" and that the "life expectancy
is now 37 and climbing". How can this
possibly be true? Are they talking
about children born today have that life expectancy? Or due to genetic testing are they
including people diagnosed at age 50 or even 60 into the stats to make them
look a heck of a lot better? These
statements go against everything I have seen and experienced. Now, I've never met someone with "mild CF",
although I know from the internet that they certainly exist. It seems much more honest to have CF broken up into a
few categories of mild, moderate, severe, and give the stats for that. I look at the pamphlets, read the stuff, and shake my head. I know the powers that be dont want to scare kids and new CF parents, but I think they often trivialize the disease. I have seen plenty of parents of young kids and teens who are really struggling. Anyone have an opinion on this? Similar experiences, similar questions? I dont know who else to ask..........<p style="font-weight: normal;" class="MsoNormal"><br>
 

imported_Momto2

New member
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<p style="font-weight: normal;" class="MsoNormal">OK, I know
this is a loaded subject. I'm a 40
year old DDF508, diagnosed at 3 months after severe failure to thrive, pneumnia, pneumothorax, etc, etc.
Over the years, I have met about 20 people with CF, and with the
exception of 2, all were all younger than I.
At this point, they have all passed away prior to age 34, and I am the
last of my childhood cohort. What I do not understand is how many pamphlets and websites state that "people with CF
can now live relatively healthy and normal lives" and that the "life expectancy
is now 37 and climbing". How can this
possibly be true? Are they talking
about children born today have that life expectancy? Or due to genetic testing are they
including people diagnosed at age 50 or even 60 into the stats to make them
look a heck of a lot better? These
statements go against everything I have seen and experienced. Now, I've never met someone with "mild CF",
although I know from the internet that they certainly exist. It seems much more honest to have CF broken up into a
few categories of mild, moderate, severe, and give the stats for that. I look at the pamphlets, read the stuff, and shake my head. I know the powers that be dont want to scare kids and new CF parents, but I think they often trivialize the disease. I have seen plenty of parents of young kids and teens who are really struggling. Anyone have an opinion on this? Similar experiences, similar questions? I dont know who else to ask..........<p style="font-weight: normal;" class="MsoNormal"><br>
 

hmw

New member
See, CFF is a bit misleading on their whole 'life expectancy' thing. They don't actually say that the average life expectancy is 37... they say the PROJECTED median age of survival, the age at which half of those with 37 may be expected to live, is 37. This applies to those born now, not those who have died in the last few years (those who have died recently have had a much lower average age at death, although the CFF doesn't make that statistic readily available.)

I agree with you on many of your points. I have never heard of someone with two severe mutations (ddf508, other class 1 or 2 mutations) being dx'ed well into adulthood. We need to know the 'over all' picture of CF, but we also need to see it divided into subsets- those who have followed a more typical course (symptomatic from childhood) and those who escape dx for many many years and somehow- even if sicker than a 'typical' person- enjoy some measure of QOL. We can't deal effectively with this disease without a realistic picture of what we are fighting.

And yes, the disease is trivialized and painted a whole lot rosier than it should be oftentimes. Speaking as a parent of a child with cf- this does no one any good. It only hurts the kids to let the parents live with rose-colored glasses, and definitely hurts all of us when all the public sees is something that isn't a big deal... when the only time we pull out the 'serious stuff' is at fund raising time.
 

hmw

New member
See, CFF is a bit misleading on their whole 'life expectancy' thing. They don't actually say that the average life expectancy is 37... they say the PROJECTED median age of survival, the age at which half of those with 37 may be expected to live, is 37. This applies to those born now, not those who have died in the last few years (those who have died recently have had a much lower average age at death, although the CFF doesn't make that statistic readily available.)

I agree with you on many of your points. I have never heard of someone with two severe mutations (ddf508, other class 1 or 2 mutations) being dx'ed well into adulthood. We need to know the 'over all' picture of CF, but we also need to see it divided into subsets- those who have followed a more typical course (symptomatic from childhood) and those who escape dx for many many years and somehow- even if sicker than a 'typical' person- enjoy some measure of QOL. We can't deal effectively with this disease without a realistic picture of what we are fighting.

And yes, the disease is trivialized and painted a whole lot rosier than it should be oftentimes. Speaking as a parent of a child with cf- this does no one any good. It only hurts the kids to let the parents live with rose-colored glasses, and definitely hurts all of us when all the public sees is something that isn't a big deal... when the only time we pull out the 'serious stuff' is at fund raising time.
 

hmw

New member
See, CFF is a bit misleading on their whole 'life expectancy' thing. They don't actually say that the average life expectancy is 37... they say the PROJECTED median age of survival, the age at which half of those with 37 may be expected to live, is 37. This applies to those born now, not those who have died in the last few years (those who have died recently have had a much lower average age at death, although the CFF doesn't make that statistic readily available.)
<br />
<br />I agree with you on many of your points. I have never heard of someone with two severe mutations (ddf508, other class 1 or 2 mutations) being dx'ed well into adulthood. We need to know the 'over all' picture of CF, but we also need to see it divided into subsets- those who have followed a more typical course (symptomatic from childhood) and those who escape dx for many many years and somehow- even if sicker than a 'typical' person- enjoy some measure of QOL. We can't deal effectively with this disease without a realistic picture of what we are fighting.
<br />
<br />And yes, the disease is trivialized and painted a whole lot rosier than it should be oftentimes. Speaking as a parent of a child with cf- this does no one any good. It only hurts the kids to let the parents live with rose-colored glasses, and definitely hurts all of us when all the public sees is something that isn't a big deal... when the only time we pull out the 'serious stuff' is at fund raising time.
 

duke1234

New member
As far as I'm concerned there is no "mild" cf. I wasn't diagnosed until age 4 at age 8 I was flown to childrens healthcare of Philadelphia to be part of a "well cf" study where they tried to did out what made some kids do better. Try wanted 100 kids, they were only able to find 90 kids healthy enough. I was in the top 90 best cf patients, mine was called "mild" and "well" I didn't go to the hospital for a tuneup until I was 12 and all the nurses thought I must has just moved to the state since they didn't know me, I was considered best of the best and now I'm 17 and fev1 18%. Cf looks for a weakness and attacks. Whether it takes 5 or 50 years cf finds a weakness eventually. The life expectancy seems a little messed up to me to, But it might be right, there are a lot of patients on this board older than 35.
 

duke1234

New member
As far as I'm concerned there is no "mild" cf. I wasn't diagnosed until age 4 at age 8 I was flown to childrens healthcare of Philadelphia to be part of a "well cf" study where they tried to did out what made some kids do better. Try wanted 100 kids, they were only able to find 90 kids healthy enough. I was in the top 90 best cf patients, mine was called "mild" and "well" I didn't go to the hospital for a tuneup until I was 12 and all the nurses thought I must has just moved to the state since they didn't know me, I was considered best of the best and now I'm 17 and fev1 18%. Cf looks for a weakness and attacks. Whether it takes 5 or 50 years cf finds a weakness eventually. The life expectancy seems a little messed up to me to, But it might be right, there are a lot of patients on this board older than 35.
 

duke1234

New member
As far as I'm concerned there is no "mild" cf. I wasn't diagnosed until age 4 at age 8 I was flown to childrens healthcare of Philadelphia to be part of a "well cf" study where they tried to did out what made some kids do better. Try wanted 100 kids, they were only able to find 90 kids healthy enough. I was in the top 90 best cf patients, mine was called "mild" and "well" I didn't go to the hospital for a tuneup until I was 12 and all the nurses thought I must has just moved to the state since they didn't know me, I was considered best of the best and now I'm 17 and fev1 18%. Cf looks for a weakness and attacks. Whether it takes 5 or 50 years cf finds a weakness eventually. The life expectancy seems a little messed up to me to, But it might be right, there are a lot of patients on this board older than 35.
 

just1more

New member
<div class="FTQUOTE"><begin quote><i>Originally posted by: <b>Momto2</b></i>

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<p style="font-weight: normal;" class="MsoNormal">OK, I know
this is a loaded subject. </end quote></div> Yes it is, I'm going to tread lightly.<p style="font-weight: normal;" class="MsoNormal"><br><p style="font-weight: normal;" class="MsoNormal"><div class="FTQUOTE"><begin quote> At this point, they have all passed away prior to age 34, and I am the
last of my childhood cohort.   What I do not understand is how many pamphlets and websites state that "people with CF
can now live relatively healthy and normal lives" and that the "life expectancy
is now 37 and climbing".    How can this
possibly be true?   Are they talking
about children born today have that life expectancy?   Or due to genetic testing are they
including people diagnosed at age 50 or even 60 into the stats to make them
look a heck of a lot better? </end quote></div><p style="font-weight: normal;" class="MsoNormal"><br><p style="font-weight: normal;" class="MsoNormal">First, the stat you are seeing quoted is not what most would consider life expectancy.  Assuming you are referring to the CFF, the age they quote is what they ESTIMATE to be the in which 1/2 of the current CF population will die.  It doesn't evaluate quality of life, age at diagnosis, etc.<p style="font-weight: normal;" class="MsoNormal"><br><p style="font-weight: normal;" class="MsoNormal">That said, you also were correct, this includes people diagnosed at 45 and 50.  There are CF'rs such as yourself that beat the odds, we had a very well loved one here that at DDF508 and passed away 18mhts ago in her 40's.<p style="font-weight: normal;" class="MsoNormal"><br><p style="font-weight: normal;" class="MsoNormal">However, as you have noted, for the most part we lose the vast majority of our community between early 20's and mid 30's.<p style="font-weight: normal;" class="MsoNormal"><br><p style="font-weight: normal;" class="MsoNormal"><div class="FTQUOTE"><begin quote>Now, I've never met someone with "mild CF",
although I know from the internet that they certainly exist.   It seems much more honest to have CF broken up into a
few categories of mild, moderate, severe, and give the stats for that.</end quote></div><p style="font-weight: normal;" class="MsoNormal">This is an ongoing debate, the main difference is that most with 'mild' CF are diagnosed by genetic testing, whereas when you were a child the only people with the CF label showed clear symptoms that screamed 'classic CF'.  However, it is hard to draw lines as to who fits in which group and what impact does it really have? Treatments, quality of life, insurance costs????  Ultimately it is a very slippery slope.<p style="font-weight: normal;" class="MsoNormal"><br><p style="font-weight: normal;" class="MsoNormal"><div class="FTQUOTE"><begin quote>  I look at the pamphlets, read the stuff, and shake my head.   I know the powers that be dont want to scare kids and new CF parents, but I think they often trivialize the disease.  I have seen plenty of parents of young kids and teens who are really struggling.  </end quote></div> <br>I don't believe it is an attempt to trivialize, however, I do know that many feel that way (myself sometimes included).  Much effort and $$ has gone into research and treatment of CF in the 40 years since you were born.  There have been great strides and things are better, but how much better is hard to judge.  There are so many uncertainties with CF, and with life itself, many chose to look at the positive as long as they can.<br>
 

just1more

New member
<div class="FTQUOTE"><begin quote><i>Originally posted by: <b>Momto2</b></i>

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<p style="font-weight: normal;" class="MsoNormal">OK, I know
this is a loaded subject. </end quote> Yes it is, I'm going to tread lightly.<p style="font-weight: normal;" class="MsoNormal"><br><p style="font-weight: normal;" class="MsoNormal"><div class="FTQUOTE"><begin quote> At this point, they have all passed away prior to age 34, and I am the
last of my childhood cohort. What I do not understand is how many pamphlets and websites state that "people with CF
can now live relatively healthy and normal lives" and that the "life expectancy
is now 37 and climbing". How can this
possibly be true? Are they talking
about children born today have that life expectancy? Or due to genetic testing are they
including people diagnosed at age 50 or even 60 into the stats to make them
look a heck of a lot better? </end quote><p style="font-weight: normal;" class="MsoNormal"><br><p style="font-weight: normal;" class="MsoNormal">First, the stat you are seeing quoted is not what most would consider life expectancy. Assuming you are referring to the CFF, the age they quote is what they ESTIMATE to be the in which 1/2 of the current CF population will die. It doesn't evaluate quality of life, age at diagnosis, etc.<p style="font-weight: normal;" class="MsoNormal"><br><p style="font-weight: normal;" class="MsoNormal">That said, you also were correct, this includes people diagnosed at 45 and 50. There are CF'rs such as yourself that beat the odds, we had a very well loved one here that at DDF508 and passed away 18mhts ago in her 40's.<p style="font-weight: normal;" class="MsoNormal"><br><p style="font-weight: normal;" class="MsoNormal">However, as you have noted, for the most part we lose the vast majority of our community between early 20's and mid 30's.<p style="font-weight: normal;" class="MsoNormal"><br><p style="font-weight: normal;" class="MsoNormal"><div class="FTQUOTE"><begin quote>Now, I've never met someone with "mild CF",
although I know from the internet that they certainly exist. It seems much more honest to have CF broken up into a
few categories of mild, moderate, severe, and give the stats for that.</end quote><p style="font-weight: normal;" class="MsoNormal">This is an ongoing debate, the main difference is that most with 'mild' CF are diagnosed by genetic testing, whereas when you were a child the only people with the CF label showed clear symptoms that screamed 'classic CF'. However, it is hard to draw lines as to who fits in which group and what impact does it really have? Treatments, quality of life, insurance costs???? Ultimately it is a very slippery slope.<p style="font-weight: normal;" class="MsoNormal"><br><p style="font-weight: normal;" class="MsoNormal"><div class="FTQUOTE"><begin quote> I look at the pamphlets, read the stuff, and shake my head. I know the powers that be dont want to scare kids and new CF parents, but I think they often trivialize the disease. I have seen plenty of parents of young kids and teens who are really struggling. </end quote><br>I don't believe it is an attempt to trivialize, however, I do know that many feel that way (myself sometimes included). Much effort and $$ has gone into research and treatment of CF in the 40 years since you were born. There have been great strides and things are better, but how much better is hard to judge. There are so many uncertainties with CF, and with life itself, many chose to look at the positive as long as they can.<br>
 

just1more

New member
<div class="FTQUOTE"><begin quote><i>Originally posted by: <b>Momto2</b></i>

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<p style="font-weight: normal;" class="MsoNormal">OK, I know
this is a loaded subject. </end quote> Yes it is, I'm going to tread lightly.<p style="font-weight: normal;" class="MsoNormal"><br><p style="font-weight: normal;" class="MsoNormal"><div class="FTQUOTE"><begin quote> At this point, they have all passed away prior to age 34, and I am the
last of my childhood cohort. What I do not understand is how many pamphlets and websites state that "people with CF
can now live relatively healthy and normal lives" and that the "life expectancy
is now 37 and climbing". How can this
possibly be true? Are they talking
about children born today have that life expectancy? Or due to genetic testing are they
including people diagnosed at age 50 or even 60 into the stats to make them
look a heck of a lot better? </end quote><p style="font-weight: normal;" class="MsoNormal"><br><p style="font-weight: normal;" class="MsoNormal">First, the stat you are seeing quoted is not what most would consider life expectancy. Assuming you are referring to the CFF, the age they quote is what they ESTIMATE to be the in which 1/2 of the current CF population will die. It doesn't evaluate quality of life, age at diagnosis, etc.<p style="font-weight: normal;" class="MsoNormal"><br><p style="font-weight: normal;" class="MsoNormal">That said, you also were correct, this includes people diagnosed at 45 and 50. There are CF'rs such as yourself that beat the odds, we had a very well loved one here that at DDF508 and passed away 18mhts ago in her 40's.<p style="font-weight: normal;" class="MsoNormal"><br><p style="font-weight: normal;" class="MsoNormal">However, as you have noted, for the most part we lose the vast majority of our community between early 20's and mid 30's.<p style="font-weight: normal;" class="MsoNormal"><br><p style="font-weight: normal;" class="MsoNormal"><div class="FTQUOTE"><begin quote>Now, I've never met someone with "mild CF",
although I know from the internet that they certainly exist. It seems much more honest to have CF broken up into a
few categories of mild, moderate, severe, and give the stats for that.</end quote><p style="font-weight: normal;" class="MsoNormal">This is an ongoing debate, the main difference is that most with 'mild' CF are diagnosed by genetic testing, whereas when you were a child the only people with the CF label showed clear symptoms that screamed 'classic CF'. However, it is hard to draw lines as to who fits in which group and what impact does it really have? Treatments, quality of life, insurance costs???? Ultimately it is a very slippery slope.<p style="font-weight: normal;" class="MsoNormal"><br><p style="font-weight: normal;" class="MsoNormal"><div class="FTQUOTE"><begin quote> I look at the pamphlets, read the stuff, and shake my head. I know the powers that be dont want to scare kids and new CF parents, but I think they often trivialize the disease. I have seen plenty of parents of young kids and teens who are really struggling. </end quote><br>I don't believe it is an attempt to trivialize, however, I do know that many feel that way (myself sometimes included). Much effort and $$ has gone into research and treatment of CF in the 40 years since you were born. There have been great strides and things are better, but how much better is hard to judge. There are so many uncertainties with CF, and with life itself, many chose to look at the positive as long as they can.<br>
 
C

cindylou

Guest
To clarify:

A patient whose FEV1 is greater than 60% (I believe) is classed as "Mild."

A patient whose FEV1 is 40-60% is "Moderate."

A patient whose FEV1 is >40% is "Severe."

"Mild" CF refers ONLY to the amount of actual lung damage in a given moment. It has no bearing on whether or not that person is going to catch a killer cold that will turn into pneumonia, cause a massive hemoptysis, and put them on the vent. I am classified as "mild" right now, but I in the past 3 years I've been in the hospital 10 times (an average of every 3 months). I know people who have lung functions around 40% and are living a much "healthier", more "normal" life than I am. I also have friends who bounce between "mild" and "severe" lung involvement regularly!

So, I think it's dangerous to say there is "mild CF" and "regular CF," because clinically, "mild" only deals with current lung function. CF is a hugely unpredictable and changeable disease, and there is no way to say whether or not someone has had a truly unusually "mild" case until their life is over! Like Duke said, you can have several years (or even decades) of fabulous health and then go downhill in six months.

There are definitely people who are diagnosed later in life, and it's usually because they aren't having as many day-to-day problems, but quite often people diagnosed later have just never found the right doctor and have extremely damaged lungs as a result of their late diagnosis.

Sara, have you ever tried the listserv Cystic-L? It's one of the oldest online CF communities and there are a number of over 40 CFers in there.
 
C

cindylou

Guest
To clarify:

A patient whose FEV1 is greater than 60% (I believe) is classed as "Mild."

A patient whose FEV1 is 40-60% is "Moderate."

A patient whose FEV1 is >40% is "Severe."

"Mild" CF refers ONLY to the amount of actual lung damage in a given moment. It has no bearing on whether or not that person is going to catch a killer cold that will turn into pneumonia, cause a massive hemoptysis, and put them on the vent. I am classified as "mild" right now, but I in the past 3 years I've been in the hospital 10 times (an average of every 3 months). I know people who have lung functions around 40% and are living a much "healthier", more "normal" life than I am. I also have friends who bounce between "mild" and "severe" lung involvement regularly!

So, I think it's dangerous to say there is "mild CF" and "regular CF," because clinically, "mild" only deals with current lung function. CF is a hugely unpredictable and changeable disease, and there is no way to say whether or not someone has had a truly unusually "mild" case until their life is over! Like Duke said, you can have several years (or even decades) of fabulous health and then go downhill in six months.

There are definitely people who are diagnosed later in life, and it's usually because they aren't having as many day-to-day problems, but quite often people diagnosed later have just never found the right doctor and have extremely damaged lungs as a result of their late diagnosis.

Sara, have you ever tried the listserv Cystic-L? It's one of the oldest online CF communities and there are a number of over 40 CFers in there.
 
C

cindylou

Guest
To clarify:
<br />
<br />A patient whose FEV1 is greater than 60% (I believe) is classed as "Mild."
<br />
<br />A patient whose FEV1 is 40-60% is "Moderate."
<br />
<br />A patient whose FEV1 is >40% is "Severe."
<br />
<br />"Mild" CF refers ONLY to the amount of actual lung damage in a given moment. It has no bearing on whether or not that person is going to catch a killer cold that will turn into pneumonia, cause a massive hemoptysis, and put them on the vent. I am classified as "mild" right now, but I in the past 3 years I've been in the hospital 10 times (an average of every 3 months). I know people who have lung functions around 40% and are living a much "healthier", more "normal" life than I am. I also have friends who bounce between "mild" and "severe" lung involvement regularly!
<br />
<br />So, I think it's dangerous to say there is "mild CF" and "regular CF," because clinically, "mild" only deals with current lung function. CF is a hugely unpredictable and changeable disease, and there is no way to say whether or not someone has had a truly unusually "mild" case until their life is over! Like Duke said, you can have several years (or even decades) of fabulous health and then go downhill in six months.
<br />
<br />There are definitely people who are diagnosed later in life, and it's usually because they aren't having as many day-to-day problems, but quite often people diagnosed later have just never found the right doctor and have extremely damaged lungs as a result of their late diagnosis.
<br />
<br />Sara, have you ever tried the listserv Cystic-L? It's one of the oldest online CF communities and there are a number of over 40 CFers in there.
 

imported_Momto2

New member
Thanks for discussing this folks. I havent known any adults with CF in the past 15 years. It can feel really isolating as I am sure many of you know. Its nice to know others share some of my frustrations and I also get to hear new perspectives and experiences. Both are MUCH appreciated and I know this is a really touchy (and distressing/sad) subject. Thank you, thank you all, for chiming in.
 

imported_Momto2

New member
Thanks for discussing this folks. I havent known any adults with CF in the past 15 years. It can feel really isolating as I am sure many of you know. Its nice to know others share some of my frustrations and I also get to hear new perspectives and experiences. Both are MUCH appreciated and I know this is a really touchy (and distressing/sad) subject. Thank you, thank you all, for chiming in.
 

imported_Momto2

New member
Thanks for discussing this folks. I havent known any adults with CF in the past 15 years. It can feel really isolating as I am sure many of you know. Its nice to know others share some of my frustrations and I also get to hear new perspectives and experiences. Both are MUCH appreciated and I know this is a really touchy (and distressing/sad) subject. Thank you, thank you all, for chiming in.
<br />
<br />
<br />
 

StillFighting

New member
Sara,

I have always questioned the statistics as well and how the data is accumulated. You raise a valid point. If so many have passed away (on this site alone) before the age of 37 and we use this site as a representative population, then how can the median age actually be 37?

It would be interesting to challenge these claims. Perhaps CFF can offer an explanation to these statistics.

Regards,

Ana
 

StillFighting

New member
Sara,

I have always questioned the statistics as well and how the data is accumulated. You raise a valid point. If so many have passed away (on this site alone) before the age of 37 and we use this site as a representative population, then how can the median age actually be 37?

It would be interesting to challenge these claims. Perhaps CFF can offer an explanation to these statistics.

Regards,

Ana
 
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