Effecacy of Kalydeco: Pancreatic sufficiency as an indicator?

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jmiller

New member
So I heard through the local CF chapter grapevine that they are considering using pancreatic sufficiency as an indicator of the effectiveness of Kalydeco in other mutations.... I suppose this is because sufficiency (or partial sufficiency) might indicate some CFTR function. In the past my docs have commented on the low level of enzymes that my sister and I take -- and neither of us have struggled with weight -- so I'm wondering if some of my pancrease is still functioning. I have DF508 and 2184delA. Not much is known about the second. I have heard previously that it is considered to be associated with "classic CF" but then again, we seem to be re-writing the books on these mutations....

So my question -- what dosage of enzymes do you take? (if you do?) I take one MT20 (21000 units of lipase) with meals/snacks and don't seem to have any issues. Is this low? normal?
 
J

jmiller

New member
So I heard through the local CF chapter grapevine that they are considering using pancreatic sufficiency as an indicator of the effectiveness of Kalydeco in other mutations.... I suppose this is because sufficiency (or partial sufficiency) might indicate some CFTR function. In the past my docs have commented on the low level of enzymes that my sister and I take -- and neither of us have struggled with weight -- so I'm wondering if some of my pancrease is still functioning. I have DF508 and 2184delA. Not much is known about the second. I have heard previously that it is considered to be associated with "classic CF" but then again, we seem to be re-writing the books on these mutations....

So my question -- what dosage of enzymes do you take? (if you do?) I take one MT20 (21000 units of lipase) with meals/snacks and don't seem to have any issues. Is this low? normal?
 
S

saveferris2009

New member
I take a TON of enzymes 5 zenpep with each meal and my FEV1 has been over 90% until this past fall (I'm 30). I've never had issues gaining weight after I went through puberty. So given this hypothesis, I'm not sure if I really fit in.....?
I know several PS CFers whose lungs aren't in great shape in their 20s. So I'm not sure if there's a correlation, but as always, I'm eager to learn more about what they find out. Please keep us posted!
 
S

saveferris2009

New member
I take a TON of enzymes 5 zenpep with each meal and my FEV1 has been over 90% until this past fall (I'm 30). I've never had issues gaining weight after I went through puberty. So given this hypothesis, I'm not sure if I really fit in.....?
I know several PS CFers whose lungs aren't in great shape in their 20s. So I'm not sure if there's a correlation, but as always, I'm eager to learn more about what they find out. Please keep us posted!
 
K

kaphi72

New member
That would be great. I am basically PS, I do not need to take any enzyme whatsoever. Never had any problems gaing wait. I have always been on the heavy side of the scale for a CF, going up to 160 for a 5f7 - My lungs aren't in good shape - I am on a canadian lung transplant waiting list FEV1 theses days in the 45% range - It has gone up dramatically since I started using Cayston. From 25% to up to 48%. I am 39 years old.
 
K

kaphi72

New member
That would be great. I am basically PS, I do not need to take any enzyme whatsoever. Never had any problems gaing wait. I have always been on the heavy side of the scale for a CF, going up to 160 for a 5f7 - My lungs aren't in good shape - I am on a canadian lung transplant waiting list FEV1 theses days in the 45% range - It has gone up dramatically since I started using Cayston. From 25% to up to 48%. I am 39 years old.
 
C

cindylou

Guest
There definitely is not a correlation for me! I practically don't have an exocrine pancreas anymore. I take 8 Zenpep 20s with meals and snacks, and have asked to be able to take more but was told that was the highest dose allowed for my weight! Even with that many, I have huge problems with digestion. My lung disease, however, has always been unstable but fairly mild (pfts currently in the 80s). I'm about to start Kalydeco (HOORAY!) and would love it if it woud help me cut down a few enzymes, but I'm not banking on it. I figure my pancreas is probably pretty shot at this point! <img src="i/expressions/face-icon-small-wink.gif" border="0">
 
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cindylou

Guest
There definitely is not a correlation for me! I practically don't have an exocrine pancreas anymore. I take 8 Zenpep 20s with meals and snacks, and have asked to be able to take more but was told that was the highest dose allowed for my weight! Even with that many, I have huge problems with digestion. My lung disease, however, has always been unstable but fairly mild (pfts currently in the 80s). I'm about to start Kalydeco (HOORAY!) and would love it if it woud help me cut down a few enzymes, but I'm not banking on it. I figure my pancreas is probably pretty shot at this point! <img src="i/expressions/face-icon-small-wink.gif" border="0">
 
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moxie1

Guest
I am homozygous DF508 and take very few enzymes. I didn't take any for a long, long time, but then again I ate a low-fat diet so that I wouldn't need enzymes.
I take 2 MT20's with meals and zero with snacks, usually because I don't feel like getting them out. On occasion I run out of enzymes in my pill container that I carry in my purse and can still eat fatty foods like pizza without any problems.
I do not have any weight issues and would actually like to be a little skinnier. I'm 5'4" and 140 lbs. FEV1 is 70%
 
M

moxie1

Guest
I am homozygous DF508 and take very few enzymes. I didn't take any for a long, long time, but then again I ate a low-fat diet so that I wouldn't need enzymes.
I take 2 MT20's with meals and zero with snacks, usually because I don't feel like getting them out. On occasion I run out of enzymes in my pill container that I carry in my purse and can still eat fatty foods like pizza without any problems.
I do not have any weight issues and would actually like to be a little skinnier. I'm 5'4" and 140 lbs. FEV1 is 70%
 
L

LouLou

New member
I think everyone when they speak of pancreatic sufficiency should not only mention if they take enzymes or not but also if they take ADEKs. I have been in the study for Kalydeco since 2008 and have not heard any researcher say definitively that anyone thinks this is drug is going to change anyone's pancreatic status since our pancreatic condition is caused by scarring, right? That being said, most people gain weight (10 lbs average on the drug) and let's face it many of us adult cfers don't need to gain any weight. I'm afraid people may gain the weight and then figure they are PS when in fact they are still losing necessary nutrients from their foods out in their stools. It's important people don't take Kalydeco and then skip their enzymes so they can continue to eat like crap. Wise up people, if Kalydeco is going to give your body the ability to function "normally" you may have to eat like "normal" people do...hopefully better since we are so wise :)
I'd be curious those people with classic cf mutations that don't seeem to require enzymes have you had a fecal fat elastase test? Are your ADE and K vitamin levels checked annually ....and if so are they normal? If they are only normal because you take an ADEK vitamin doesn't that constitute PI?

I take 3 Zenpep MT 20s with meals. I can't snack much anymore now on Kalydeco or I pork out. My weight is 132 lb. at 5'3" and my stools are about the same as before Kalydeco (still that funny cf tan color) but they are less frequent only once a day! I continue to take ADEKs twice a day and for the first time ever my Vit D level has dropped below normal. I did the high dose once a week for a month regimen but on recheck it is still low (28).
 
L

LouLou

New member
I think everyone when they speak of pancreatic sufficiency should not only mention if they take enzymes or not but also if they take ADEKs. I have been in the study for Kalydeco since 2008 and have not heard any researcher say definitively that anyone thinks this is drug is going to change anyone's pancreatic status since our pancreatic condition is caused by scarring, right? That being said, most people gain weight (10 lbs average on the drug) and let's face it many of us adult cfers don't need to gain any weight. I'm afraid people may gain the weight and then figure they are PS when in fact they are still losing necessary nutrients from their foods out in their stools. It's important people don't take Kalydeco and then skip their enzymes so they can continue to eat like crap. Wise up people, if Kalydeco is going to give your body the ability to function "normally" you may have to eat like "normal" people do...hopefully better since we are so wise :)
I'd be curious those people with classic cf mutations that don't seeem to require enzymes have you had a fecal fat elastase test? Are your ADE and K vitamin levels checked annually ....and if so are they normal? If they are only normal because you take an ADEK vitamin doesn't that constitute PI?

I take 3 Zenpep MT 20s with meals. I can't snack much anymore now on Kalydeco or I pork out. My weight is 132 lb. at 5'3" and my stools are about the same as before Kalydeco (still that funny cf tan color) but they are less frequent only once a day! I continue to take ADEKs twice a day and for the first time ever my Vit D level has dropped below normal. I did the high dose once a week for a month regimen but on recheck it is still low (28).
 
T

Taylersmom

New member
Im not sure if my son's enzymes are regular or not.. What is the norm?? He has no problem gaining weight, he eats lots of fatty foods, almost 4-5 full meals a day and lots of snacks. I can't remember him, not being hungry. He only takes 2 Creon 24's with meals and none with snacks. He is more of a lung issue type... And no he does not take Adeks, he has tried in the past and it gave him a horrible stomache ache. He has his vit levels checked annually and they are normal. Does anyone else take creon 24000?
 
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Taylersmom

New member
Im not sure if my son's enzymes are regular or not.. What is the norm?? He has no problem gaining weight, he eats lots of fatty foods, almost 4-5 full meals a day and lots of snacks. I can't remember him, not being hungry. He only takes 2 Creon 24's with meals and none with snacks. He is more of a lung issue type... And no he does not take Adeks, he has tried in the past and it gave him a horrible stomache ache. He has his vit levels checked annually and they are normal. Does anyone else take creon 24000?
 
J

JENNYC

New member
My baby girl takes 4 creon 12,000 with meals and 2 with snacks, so sounds like your son Taylersmom takes the same as my Abby. And I think her dose is probably a little high just to be on the safe side. They say it's better to take more than less.
 
J

JENNYC

New member
My baby girl takes 4 creon 12,000 with meals and 2 with snacks, so sounds like your son Taylersmom takes the same as my Abby. And I think her dose is probably a little high just to be on the safe side. They say it's better to take more than less.
 
I

inna1977

New member
Here is a blog from someone on 770/661 trial and she is doing amazing.

http://www.cfgirlseyeofthetiger.com/blog/2012/03/20/Halfway-through-.aspx
 
I

inna1977

New member
Here is a blog from someone on 770/661 trial and she is doing amazing.

http://www.cfgirlseyeofthetiger.com/blog/2012/03/20/Halfway-through-.aspx
 
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