Round table Discussion of Atypical CF at National CF Conference in Anaheim..

[Deb]

I have been labeled "atypical CF" several times in the last few years and honestly I don't like it. I agree with some of the others that CF is CF.
Yes, I was diagnosed later in life but I attribute that more to lack of physician knowledge. My sweat test was well above normal at 100+. I have one DF508 and one R117H. Although I do not have pancreatic insufficiency I have had more than my share of lung infections. Two types of mycobacterial infections have cost me one lung. I am now living with one infected lung and although I am trying hard to stay healthy I battle CF every day of my life.

 

[Deb]

I have been labeled "atypical CF" several times in the last few years and honestly I don't like it. I agree with some of the others that CF is CF.
Yes, I was diagnosed later in life but I attribute that more to lack of physician knowledge. My sweat test was well above normal at 100+. I have one DF508 and one R117H. Although I do not have pancreatic insufficiency I have had more than my share of lung infections. Two types of mycobacterial infections have cost me one lung. I am now living with one infected lung and although I am trying hard to stay healthy I battle CF every day of my life.

 

[Deb]

I have been labeled "atypical CF" several times in the last few years and honestly I don't like it. I agree with some of the others that CF is CF.
<br />Yes, I was diagnosed later in life but I attribute that more to lack of physician knowledge. My sweat test was well above normal at 100+. I have one DF508 and one R117H. Although I do not have pancreatic insufficiency I have had more than my share of lung infections. Two types of mycobacterial infections have cost me one lung. I am now living with one infected lung and although I am trying hard to stay healthy I battle CF every day of my life.

 

[Mistyjo]

My daughter has been diagnosed as cystic fibrosis metabolic syndrome. I didn't think that was any different than atypical cf.

 

[Mistyjo]

My daughter has been diagnosed as cystic fibrosis metabolic syndrome. I didn't think that was any different than atypical cf.

 

[Mistyjo]

My daughter has been diagnosed as cystic fibrosis metabolic syndrome. I didn't think that was any different than atypical cf.

 

[windex125]

I am 56 and was diagnosed as a infant 1955 then undiagnosed then re-diagnosed. I finally started getting treament at the age of 25 with a CF center, my 20/30's were constant lung infections and in 1999 my CF center thought I shd go to UNC for their nasal chloride test which confrimed postive for CF prior to that my CF center labeled me Atypical my sweat tests were borderline as well. I am PS but I have now only one functioing lung at this time and my PFT's are in the high 40's. Bill like you I have MAC and also chronic bronchestesis my mutaions are D508/V920M. Pat 56/CF and a bunch of other issues as well

 

[windex125]

I am 56 and was diagnosed as a infant 1955 then undiagnosed then re-diagnosed. I finally started getting treament at the age of 25 with a CF center, my 20/30's were constant lung infections and in 1999 my CF center thought I shd go to UNC for their nasal chloride test which confrimed postive for CF prior to that my CF center labeled me Atypical my sweat tests were borderline as well. I am PS but I have now only one functioing lung at this time and my PFT's are in the high 40's. Bill like you I have MAC and also chronic bronchestesis my mutaions are D508/V920M. Pat 56/CF and a bunch of other issues as well

 

[windex125]

I am 56 and was diagnosed as a infant 1955 then undiagnosed then re-diagnosed. I finally started getting treament at the age of 25 with a CF center, my 20/30's were constant lung infections and in 1999 my CF center thought I shd go to UNC for their nasal chloride test which confrimed postive for CF prior to that my CF center labeled me Atypical my sweat tests were borderline as well. I am PS but I have now only one functioing lung at this time and my PFT's are in the high 40's. Bill like you I have MAC and also chronic bronchestesis my mutaions are D508/V920M. Pat 56/CF and a bunch of other issues as well

 

[amyr]

Windex,
Please feel free to share your story on my blog. I am gathering as much info on this subject as part of my work with the CFF. All email addresses are private and will not show up on my blog.

http://www.connections4life.com/blog.html

 

[amyr]

Windex,
Please feel free to share your story on my blog. I am gathering as much info on this subject as part of my work with the CFF. All email addresses are private and will not show up on my blog.

http://www.connections4life.com/blog.html

 

[amyr]

Windex,
<br />Please feel free to share your story on my blog. I am gathering as much info on this subject as part of my work with the CFF. All email addresses are private and will not show up on my blog.
<br />
<br />http://www.connections4life.com/blog.html
<br />
<br />

 

[bmombtoo]

<P>Brad, </P>
<P><FONT color=#000000>My son's symptoms sound a lot like yours. He was considered atypical when he was diagnosed at age 12 back in 2006. The atypical meant that his presentation was not "normal". By age two he had been hospitalized twice for lung infections (bronchitis) and high temperatures. He had lots of asthma like symptoms and sinus infections and needed sinus surgery in 2006. They found polyps and referred him to the CF center at Hopkins.  He was positive on one sweat test and on a nasal differential test. His pancreas works well. Since being treated for CF he has done well until recently. This spring his lung functions dropped in the seventies and by June they were at 60% and he was hospitalized and needed a central line. Six weeks after that he had his first pseudomonas and was place on TOBI. His lung functions are now at 82%.</FONT></P>
<P><FONT color=#000000> </FONT></P>
<P><FONT color=#000000>Gene testing has not been successful. We are African American which adds to the rarity. He has an older brother with some similar symptoms and I have the sinus issues and take xopenex by nebulizer 2 or 3 times a day. When the gene is identified we can't test the rest of the family. My nasal potential differential is very similar to his and this spring I had pneumonia again. My mother lost two of her brothers when they were in their early twenties back in the thirties.   </FONT></P>
<P> </P>

 

[bmombtoo]

<P>Brad, </P>
<P><FONT color=#000000>My son's symptoms sound a lot like yours. He was considered atypical when he was diagnosed at age 12 back in 2006. The atypical meant that his presentation was not "normal".By age two he had been hospitalized twice for lung infections (bronchitis) and high temperatures. He had lots of asthma like symptoms and sinus infections and needed sinus surgery in 2006. They found polyps and referred him to the CF center at Hopkins. He was positive on one sweat test and on a nasal differential test. His pancreas works well. Since being treated for CF he has done well until recently. This spring his lung functions dropped in the seventies and by June they were at 60% and he was hospitalized and needed a central line. Six weeks after that he had his first pseudomonas and was place on TOBI. His lung functions are now at 82%.</FONT></P>
<P><FONT color=#000000></FONT></P>
<P><FONT color=#000000>Gene testing has not been successful.We areAfrican American which adds to the rarity. He has an older brother with some similar symptoms and I have the sinus issues and take xopenex by nebulizer 2 or 3 times a day. When the gene is identified we can't test the rest of the family.My nasal potential differential is very similar to his and this spring I had pneumonia again. My motherlost two of herbrothers when they were in their early twenties back in the thirties. </FONT></P>
<P></P>

 

[bmombtoo]

<P><BR>Brad, </P>
<P><FONT color=#000000>My son's symptoms sound a lot like yours. He was considered atypical when he was diagnosed at age 12 back in 2006. The atypical meant that his presentation was not "normal".By age two he had been hospitalized twice for lung infections (bronchitis) and high temperatures. He had lots of asthma like symptoms and sinus infections and needed sinus surgery in 2006. They found polyps and referred him to the CF center at Hopkins. He was positive on one sweat test and on a nasal differential test. His pancreas works well. Since being treated for CF he has done well until recently. This spring his lung functions dropped in the seventies and by June they were at 60% and he was hospitalized and needed a central line. Six weeks after that he had his first pseudomonas and was place on TOBI. His lung functions are now at 82%.</FONT></P>
<P><FONT color=#000000></FONT></P>
<P><FONT color=#000000>Gene testing has not been successful.We areAfrican American which adds to the rarity. He has an older brother with some similar symptoms and I have the sinus issues and take xopenex by nebulizer 2 or 3 times a day. When the gene is identified we can't test the rest of the family.My nasal potential differential is very similar to his and this spring I had pneumonia again. My motherlost two of herbrothers when they were in their early twenties back in the thirties. </FONT></P>
<P></P>

 

[musclemania70]

Brad--my mother diagnosed me too. Good thing for caring, alert, and insightful mothers.
Especially since I am the only 1 of 4 children to have cf!!!!!

 

[musclemania70]

Brad--my mother diagnosed me too. Good thing for caring, alert, and insightful mothers.
Especially since I am the only 1 of 4 children to have cf!!!!!

 

[musclemania70]

Brad--my mother diagnosed me too. Good thing for caring, alert, and insightful mothers.
<br />Especially since I am the only 1 of 4 children to have cf!!!!!