Double Delta F508

[ElizabethlovesChris]

My hubby is a ddf508 as well. He just turned 30 years old, was diagnosed at 8 days old due to meconium ileus. He has been a fairly severe case for most of his life, with some years being worse than others. Pre-puberty he was extremely small and thin for his age, but has gained weight better as an adult. He's 5'7" and about 138 lbs.

His last PFTs were 2 weeks ago and his best blow was 57%, and he blew a miracle 72% in Jan. 07 (we have no clue where it came from!). Normally been in high 50s, low 60s for the last couple of years.

Our main fear is his severe hemoptysis which has been occurring over the last 2 years. He has had 4 (or maybe 5?) embolizations for that, and it's been BY FAR the scariest thing because you truly have no control over it and it truly feels like the end when all that pure blood is rushing out.

He has had 24 separate polypectomy sinus surgeries in his lifetime (starting at age 3) has had a g-tube in and out in childhood, a fundoplication at age 9, diagnosed with liver fibrosis and splenomegaly at age 24, and in recent years episodes with kidney stones. No diabetes (knock on wood).

Best of luck to all of you, regardless of your mutations - screw CF!

 

[ElizabethlovesChris]

My hubby is a ddf508 as well. He just turned 30 years old, was diagnosed at 8 days old due to meconium ileus. He has been a fairly severe case for most of his life, with some years being worse than others. Pre-puberty he was extremely small and thin for his age, but has gained weight better as an adult. He's 5'7" and about 138 lbs.

His last PFTs were 2 weeks ago and his best blow was 57%, and he blew a miracle 72% in Jan. 07 (we have no clue where it came from!). Normally been in high 50s, low 60s for the last couple of years.

Our main fear is his severe hemoptysis which has been occurring over the last 2 years. He has had 4 (or maybe 5?) embolizations for that, and it's been BY FAR the scariest thing because you truly have no control over it and it truly feels like the end when all that pure blood is rushing out.

He has had 24 separate polypectomy sinus surgeries in his lifetime (starting at age 3) has had a g-tube in and out in childhood, a fundoplication at age 9, diagnosed with liver fibrosis and splenomegaly at age 24, and in recent years episodes with kidney stones. No diabetes (knock on wood).

Best of luck to all of you, regardless of your mutations - screw CF!

 

[lmattaway]

Can I be in the club too? I always found it interesting that a lot of texts refer to DDF508 as producing a more severe case of CF b/c that has not been my experience. I typically have FEV1 in the 100% range and I've only ever been hospitalized for the removal of nasal polyps. I have more problems with my nose and my digestion than I do with my lungs. What a crazy gene!
~Lindsay

 

[lmattaway]

Can I be in the club too? I always found it interesting that a lot of texts refer to DDF508 as producing a more severe case of CF b/c that has not been my experience. I typically have FEV1 in the 100% range and I've only ever been hospitalized for the removal of nasal polyps. I have more problems with my nose and my digestion than I do with my lungs. What a crazy gene!
~Lindsay

 

[lmattaway]

Can I be in the club too? I always found it interesting that a lot of texts refer to DDF508 as producing a more severe case of CF b/c that has not been my experience. I typically have FEV1 in the 100% range and I've only ever been hospitalized for the removal of nasal polyps. I have more problems with my nose and my digestion than I do with my lungs. What a crazy gene!
~Lindsay

 

[lmattaway]

Can I be in the club too? I always found it interesting that a lot of texts refer to DDF508 as producing a more severe case of CF b/c that has not been my experience. I typically have FEV1 in the 100% range and I've only ever been hospitalized for the removal of nasal polyps. I have more problems with my nose and my digestion than I do with my lungs. What a crazy gene!
~Lindsay

 

[lmattaway]

Can I be in the club too? I always found it interesting that a lot of texts refer to DDF508 as producing a more severe case of CF b/c that has not been my experience. I typically have FEV1 in the 100% range and I've only ever been hospitalized for the removal of nasal polyps. I have more problems with my nose and my digestion than I do with my lungs. What a crazy gene!
~Lindsay

 

[lmattaway]

Can I be in the club too? I always found it interesting that a lot of texts refer to DDF508 as producing a more severe case of CF b/c that has not been my experience. I typically have FEV1 in the 100% range and I've only ever been hospitalized for the removal of nasal polyps. I have more problems with my nose and my digestion than I do with my lungs. What a crazy gene!
~Lindsay

 

[CFMOM65]

my daughter 18 mnths has the combo she has had some pneumonias caused from aspirating when she was very little but besides that she has had alot of digestive issues , rectal prolapse, consipation all the time, big belly she has had failure to thrive a few times thats actually how they diagnosed her w/ cf but right now her weight is doing super she is very short 29 inches. she has also had alot of sinus problems they took out her adnoids wich seems to b helping tremendously. she has had trobles w/ her liver since she was 2 mnths old and takes actigall and has a vit k deffeciancy


cfmom

daughter 9 yrs old w/o cf & baby girl 18 mnths w/ cf

 

[CFMOM65]

my daughter 18 mnths has the combo she has had some pneumonias caused from aspirating when she was very little but besides that she has had alot of digestive issues , rectal prolapse, consipation all the time, big belly she has had failure to thrive a few times thats actually how they diagnosed her w/ cf but right now her weight is doing super she is very short 29 inches. she has also had alot of sinus problems they took out her adnoids wich seems to b helping tremendously. she has had trobles w/ her liver since she was 2 mnths old and takes actigall and has a vit k deffeciancy


cfmom

daughter 9 yrs old w/o cf & baby girl 18 mnths w/ cf

 

[CFMOM65]

my daughter 18 mnths has the combo she has had some pneumonias caused from aspirating when she was very little but besides that she has had alot of digestive issues , rectal prolapse, consipation all the time, big belly she has had failure to thrive a few times thats actually how they diagnosed her w/ cf but right now her weight is doing super she is very short 29 inches. she has also had alot of sinus problems they took out her adnoids wich seems to b helping tremendously. she has had trobles w/ her liver since she was 2 mnths old and takes actigall and has a vit k deffeciancy


cfmom

daughter 9 yrs old w/o cf & baby girl 18 mnths w/ cf

 

[CFMOM65]

my daughter 18 mnths has the combo she has had some pneumonias caused from aspirating when she was very little but besides that she has had alot of digestive issues , rectal prolapse, consipation all the time, big belly she has had failure to thrive a few times thats actually how they diagnosed her w/ cf but right now her weight is doing super she is very short 29 inches. she has also had alot of sinus problems they took out her adnoids wich seems to b helping tremendously. she has had trobles w/ her liver since she was 2 mnths old and takes actigall and has a vit k deffeciancy


cfmom

daughter 9 yrs old w/o cf & baby girl 18 mnths w/ cf

 

[CFMOM65]

my daughter 18 mnths has the combo she has had some pneumonias caused from aspirating when she was very little but besides that she has had alot of digestive issues , rectal prolapse, consipation all the time, big belly she has had failure to thrive a few times thats actually how they diagnosed her w/ cf but right now her weight is doing super she is very short 29 inches. she has also had alot of sinus problems they took out her adnoids wich seems to b helping tremendously. she has had trobles w/ her liver since she was 2 mnths old and takes actigall and has a vit k deffeciancy


cfmom

daughter 9 yrs old w/o cf & baby girl 18 mnths w/ cf

 

[CFMOM65]

my daughter 18 mnths has the combo she has had some pneumonias caused from aspirating when she was very little but besides that she has had alot of digestive issues , rectal prolapse, consipation all the time, big belly she has had failure to thrive a few times thats actually how they diagnosed her w/ cf but right now her weight is doing super she is very short 29 inches. she has also had alot of sinus problems they took out her adnoids wich seems to b helping tremendously. she has had trobles w/ her liver since she was 2 mnths old and takes actigall and has a vit k deffeciancy


cfmom

daughter 9 yrs old w/o cf & baby girl 18 mnths w/ cf

 

[karismom]

WELL I HAVE TWO DAUGHTERS W DDF508.......AND KARI (26) IS POST TX X 2 AND HAS DIABETES, BOWEL OBSTRUCTIONS HAD MECONIUM ILEUS, SINUS SURGERIES OSTEOPOROSIS............................... AND HOLLI JADE (21) HAS BEEN HOSP THREE TIMES HAS HAD BOWEL DIFFICULTIES AND TWO SINUS SURGERIES.............................. SO IT IN MY OPINION HAS NOTHING TO DO WITH YOUR OUTCOME!!!!! AND I WILL DEFEND THAT THEORY TIL THE DAY I DIE!!!!!!!!!!!!!!!!!!!!!!!!! SO ANYONE WANTS TO DISPUTE IT WITH ME DO NOT BOTHER. I HAVE LIVED IT FOR 21 YEARS. THANKS FOR LISTENING.



peace.

lisa <img src="i/expressions/rose.gif" border="0">

 

[karismom]

WELL I HAVE TWO DAUGHTERS W DDF508.......AND KARI (26) IS POST TX X 2 AND HAS DIABETES, BOWEL OBSTRUCTIONS HAD MECONIUM ILEUS, SINUS SURGERIES OSTEOPOROSIS............................... AND HOLLI JADE (21) HAS BEEN HOSP THREE TIMES HAS HAD BOWEL DIFFICULTIES AND TWO SINUS SURGERIES.............................. SO IT IN MY OPINION HAS NOTHING TO DO WITH YOUR OUTCOME!!!!! AND I WILL DEFEND THAT THEORY TIL THE DAY I DIE!!!!!!!!!!!!!!!!!!!!!!!!! SO ANYONE WANTS TO DISPUTE IT WITH ME DO NOT BOTHER. I HAVE LIVED IT FOR 21 YEARS. THANKS FOR LISTENING.



peace.

lisa <img src="i/expressions/rose.gif" border="0">

 

[karismom]

WELL I HAVE TWO DAUGHTERS W DDF508.......AND KARI (26) IS POST TX X 2 AND HAS DIABETES, BOWEL OBSTRUCTIONS HAD MECONIUM ILEUS, SINUS SURGERIES OSTEOPOROSIS............................... AND HOLLI JADE (21) HAS BEEN HOSP THREE TIMES HAS HAD BOWEL DIFFICULTIES AND TWO SINUS SURGERIES.............................. SO IT IN MY OPINION HAS NOTHING TO DO WITH YOUR OUTCOME!!!!! AND I WILL DEFEND THAT THEORY TIL THE DAY I DIE!!!!!!!!!!!!!!!!!!!!!!!!! SO ANYONE WANTS TO DISPUTE IT WITH ME DO NOT BOTHER. I HAVE LIVED IT FOR 21 YEARS. THANKS FOR LISTENING.



peace.

lisa <img src="i/expressions/rose.gif" border="0">

 

[karismom]

WELL I HAVE TWO DAUGHTERS W DDF508.......AND KARI (26) IS POST TX X 2 AND HAS DIABETES, BOWEL OBSTRUCTIONS HAD MECONIUM ILEUS, SINUS SURGERIES OSTEOPOROSIS............................... AND HOLLI JADE (21) HAS BEEN HOSP THREE TIMES HAS HAD BOWEL DIFFICULTIES AND TWO SINUS SURGERIES.............................. SO IT IN MY OPINION HAS NOTHING TO DO WITH YOUR OUTCOME!!!!! AND I WILL DEFEND THAT THEORY TIL THE DAY I DIE!!!!!!!!!!!!!!!!!!!!!!!!! SO ANYONE WANTS TO DISPUTE IT WITH ME DO NOT BOTHER. I HAVE LIVED IT FOR 21 YEARS. THANKS FOR LISTENING.



peace.

lisa <img src="i/expressions/rose.gif" border="0">

 

[karismom]

WELL I HAVE TWO DAUGHTERS W DDF508.......AND KARI (26) IS POST TX X 2 AND HAS DIABETES, BOWEL OBSTRUCTIONS HAD MECONIUM ILEUS, SINUS SURGERIES OSTEOPOROSIS............................... AND HOLLI JADE (21) HAS BEEN HOSP THREE TIMES HAS HAD BOWEL DIFFICULTIES AND TWO SINUS SURGERIES.............................. SO IT IN MY OPINION HAS NOTHING TO DO WITH YOUR OUTCOME!!!!! AND I WILL DEFEND THAT THEORY TIL THE DAY I DIE!!!!!!!!!!!!!!!!!!!!!!!!! SO ANYONE WANTS TO DISPUTE IT WITH ME DO NOT BOTHER. I HAVE LIVED IT FOR 21 YEARS. THANKS FOR LISTENING.



peace.

lisa <img src="i/expressions/rose.gif" border="0">

 

[karismom]

WELL I HAVE TWO DAUGHTERS W DDF508.......AND KARI (26) IS POST TX X 2 AND HAS DIABETES, BOWEL OBSTRUCTIONS HAD MECONIUM ILEUS, SINUS SURGERIES OSTEOPOROSIS............................... AND HOLLI JADE (21) HAS BEEN HOSP THREE TIMES HAS HAD BOWEL DIFFICULTIES AND TWO SINUS SURGERIES.............................. SO IT IN MY OPINION HAS NOTHING TO DO WITH YOUR OUTCOME!!!!! AND I WILL DEFEND THAT THEORY TIL THE DAY I DIE!!!!!!!!!!!!!!!!!!!!!!!!! SO ANYONE WANTS TO DISPUTE IT WITH ME DO NOT BOTHER. I HAVE LIVED IT FOR 21 YEARS. THANKS FOR LISTENING.



peace.

lisa <img src="i/expressions/rose.gif" border="0">