CF Liver Disease - Any Therapies in the Pipeline

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BlueTurtle

New member
You can GET people to that age...but without a proper digestive system...it won't matter. We are talking QUALITY of life as well. And how do you know the CF foundation doesn't see it this way? I cannot believe they do not have a DIGESTIVE research team, that would be completely insane...it is a HUGE part of this disease. Most doctors will tell you that an unhealthy digestion can affect everything in the body, including the immune system. Something CFers need with all the antibiotics and therapies they are on.
 
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dasjsmum

New member
It's just the way it is, Amy cant change it. It's the lung disease that leads to death and causes loss of quality of life, if my kids could be cured of their lung disease putting up with the digestive side for the rest of their lives would be no problem. It would be nice to see more emphasis on diet etc., but obviously the money goes where it's needed most.
 
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dasjsmum

New member
It's just the way it is, Amy cant change it. It's the lung disease that leads to death and causes loss of quality of life, if my kids could be cured of their lung disease putting up with the digestive side for the rest of their lives would be no problem. It would be nice to see more emphasis on diet etc., but obviously the money goes where it's needed most.
 
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dasjsmum

New member
It's just the way it is, Amy cant change it. It's the lung disease that leads to death and causes loss of quality of life, if my kids could be cured of their lung disease putting up with the digestive side for the rest of their lives would be no problem. It would be nice to see more emphasis on diet etc., but obviously the money goes where it's needed most.
 
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AnD

New member
I understand your frustration- I have a "fatty liver" and have had problems with bile stones. I have a great new GI doctor (he's pediatric) who used to work at our local children's hospital, but now he and his partner have a private practice, and he has agreed to see the adult cfs! He is such an answer to prayer! <img src="i/expressions/face-icon-small-happy.gif" border="0"> Lots of Ursidoil and water, plus meds (if I understand right) if I get chronic inflamation... he also diagnosed me with H Pylori and treated that, which cut down on my stomach aches wonderfully! And he put me on Nexium (which works better than my OTC ones I tried and quit because of....stomach aches). Other than that... I have wondered, "Can I not have a Pulmozyme pump or something for my liver? (LOL, but only half kidding <img src="i/expressions/face-icon-small-wink.gif" border="0"> ).

After my last appointment, he called me himself to tell me the good news that my inflamation had gone down wonderfully! He's the only GI who hasn't been convinced from the minute he saw my chart that my stomach aches were enzyme (lack there of) related. Kinda a "whole person" kind of doctor! It seems that with cf, that can be kinda rare...
 
A

AnD

New member
I understand your frustration- I have a "fatty liver" and have had problems with bile stones. I have a great new GI doctor (he's pediatric) who used to work at our local children's hospital, but now he and his partner have a private practice, and he has agreed to see the adult cfs! He is such an answer to prayer! <img src="i/expressions/face-icon-small-happy.gif" border="0"> Lots of Ursidoil and water, plus meds (if I understand right) if I get chronic inflamation... he also diagnosed me with H Pylori and treated that, which cut down on my stomach aches wonderfully! And he put me on Nexium (which works better than my OTC ones I tried and quit because of....stomach aches). Other than that... I have wondered, "Can I not have a Pulmozyme pump or something for my liver? (LOL, but only half kidding <img src="i/expressions/face-icon-small-wink.gif" border="0"> ).

After my last appointment, he called me himself to tell me the good news that my inflamation had gone down wonderfully! He's the only GI who hasn't been convinced from the minute he saw my chart that my stomach aches were enzyme (lack there of) related. Kinda a "whole person" kind of doctor! It seems that with cf, that can be kinda rare...
 
A

AnD

New member
I understand your frustration- I have a "fatty liver" and have had problems with bile stones. I have a great new GI doctor (he's pediatric) who used to work at our local children's hospital, but now he and his partner have a private practice, and he has agreed to see the adult cfs! He is such an answer to prayer! <img src="i/expressions/face-icon-small-happy.gif" border="0"> Lots of Ursidoil and water, plus meds (if I understand right) if I get chronic inflamation... he also diagnosed me with H Pylori and treated that, which cut down on my stomach aches wonderfully! And he put me on Nexium (which works better than my OTC ones I tried and quit because of....stomach aches). Other than that... I have wondered, "Can I not have a Pulmozyme pump or something for my liver? (LOL, but only half kidding <img src="i/expressions/face-icon-small-wink.gif" border="0"> ).

After my last appointment, he called me himself to tell me the good news that my inflamation had gone down wonderfully! He's the only GI who hasn't been convinced from the minute he saw my chart that my stomach aches were enzyme (lack there of) related. Kinda a "whole person" kind of doctor! It seems that with cf, that can be kinda rare...
 
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BlueTurtle

New member
Have you had your gall bladder removed? Also, when did you experience problems with your liver. Has the liver gotten progressively worse or better? Were your bile stones in the liver? When did you start using ursodol? Thanks for the information...this part of CF is too important to ignore.
 
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BlueTurtle

New member
Have you had your gall bladder removed? Also, when did you experience problems with your liver. Has the liver gotten progressively worse or better? Were your bile stones in the liver? When did you start using ursodol? Thanks for the information...this part of CF is too important to ignore.
 
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BlueTurtle

New member
Have you had your gall bladder removed? Also, when did you experience problems with your liver. Has the liver gotten progressively worse or better? Were your bile stones in the liver? When did you start using ursodol? Thanks for the information...this part of CF is too important to ignore.
 
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AnD

New member
This has been fairly recent, so I don't have a whole lot of info for you <img src="i/expressions/face-icon-small-wink.gif" border="0"> . I had my gallbladder out 19 years ago, and told them I was still having the same stomach aches, and that I had seen something on tv that said it could be garbage still left in the bile duct. They told me that that couldn't be it; have some enzymes. Worse stomach aches.
Fast forward 16 years: I'm in for a tune up after having our baby, and they send me to get some tests and scans since I am there anyways and my liver levels came back slightly elevated. Lo and behold- stone in my bile duct and panceratitis. Get that taken out and the panceratisits taken care of. Go on ursidol.

Last year I went to the er with stomach pains- liver infection and- stones. 8 days in ICU, 13 in the hospital. Go to my present GI doctor, he ups my ursidiol (600 mg?2x/day, lessens the # of times I have to take it, diagnosises me with the H Pylori, says I have some inflamation according to the numbers he got back from my blood test, but they are numbers he can live with. Tells me to drink more water. Calls back after my follow up appointment, and says inflamation is much improved <img src="i/expressions/face-icon-small-wink.gif" border="0"> . That's about it. I did find in my reading that a lot more cf's have liver disease than realize it, because once it finally shows up in the blood tests, it is pretty far along. I have another appointment in April; I'll let you know how it goes <img src="i/expressions/face-icon-small-wink.gif" border="0"> .
 
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AnD

New member
This has been fairly recent, so I don't have a whole lot of info for you <img src="i/expressions/face-icon-small-wink.gif" border="0"> . I had my gallbladder out 19 years ago, and told them I was still having the same stomach aches, and that I had seen something on tv that said it could be garbage still left in the bile duct. They told me that that couldn't be it; have some enzymes. Worse stomach aches.
Fast forward 16 years: I'm in for a tune up after having our baby, and they send me to get some tests and scans since I am there anyways and my liver levels came back slightly elevated. Lo and behold- stone in my bile duct and panceratitis. Get that taken out and the panceratisits taken care of. Go on ursidol.

Last year I went to the er with stomach pains- liver infection and- stones. 8 days in ICU, 13 in the hospital. Go to my present GI doctor, he ups my ursidiol (600 mg?2x/day, lessens the # of times I have to take it, diagnosises me with the H Pylori, says I have some inflamation according to the numbers he got back from my blood test, but they are numbers he can live with. Tells me to drink more water. Calls back after my follow up appointment, and says inflamation is much improved <img src="i/expressions/face-icon-small-wink.gif" border="0"> . That's about it. I did find in my reading that a lot more cf's have liver disease than realize it, because once it finally shows up in the blood tests, it is pretty far along. I have another appointment in April; I'll let you know how it goes <img src="i/expressions/face-icon-small-wink.gif" border="0"> .
 
A

AnD

New member
This has been fairly recent, so I don't have a whole lot of info for you <img src="i/expressions/face-icon-small-wink.gif" border="0"> . I had my gallbladder out 19 years ago, and told them I was still having the same stomach aches, and that I had seen something on tv that said it could be garbage still left in the bile duct. They told me that that couldn't be it; have some enzymes. Worse stomach aches.
Fast forward 16 years: I'm in for a tune up after having our baby, and they send me to get some tests and scans since I am there anyways and my liver levels came back slightly elevated. Lo and behold- stone in my bile duct and panceratitis. Get that taken out and the panceratisits taken care of. Go on ursidol.

Last year I went to the er with stomach pains- liver infection and- stones. 8 days in ICU, 13 in the hospital. Go to my present GI doctor, he ups my ursidiol (600 mg?2x/day, lessens the # of times I have to take it, diagnosises me with the H Pylori, says I have some inflamation according to the numbers he got back from my blood test, but they are numbers he can live with. Tells me to drink more water. Calls back after my follow up appointment, and says inflamation is much improved <img src="i/expressions/face-icon-small-wink.gif" border="0"> . That's about it. I did find in my reading that a lot more cf's have liver disease than realize it, because once it finally shows up in the blood tests, it is pretty far along. I have another appointment in April; I'll let you know how it goes <img src="i/expressions/face-icon-small-wink.gif" border="0"> .
 
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mom2leila

New member
Hey Blue,

I'm with you on this issue. I wish there was more to do, too. Our daughter presented with liver issues at 2 months of age. She was terribly jaundiced. Her acute liver disease resolved with ursodiol (which she takes twice daily for the rest of her current liver's life) and time, but her liver enzyme numbers are far from normal. Right now they are elevated but unchanged. We get abdominal ultrasounds every 6-12 months to check for a change in blood flow in the liver. We also have had a liver biopsy a couple years ago and are due for another this summer. Her spleen is already enlarged from blood flow restriction. Other kids tease her about her big tummy. Our GI doc said on our last visit he "hopes we have another 10-20 years" before we see serious problems like esophageal varices. After that we will no doubt be looking at transplant. So for us, the digestive issues might take us down before the pulmonary ones. I seem to remember reading that 20% have liver issues? Amy's right, that's not very many. Doesn't make it any easier if you're in the 20% though.
 
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mom2leila

New member
Hey Blue,

I'm with you on this issue. I wish there was more to do, too. Our daughter presented with liver issues at 2 months of age. She was terribly jaundiced. Her acute liver disease resolved with ursodiol (which she takes twice daily for the rest of her current liver's life) and time, but her liver enzyme numbers are far from normal. Right now they are elevated but unchanged. We get abdominal ultrasounds every 6-12 months to check for a change in blood flow in the liver. We also have had a liver biopsy a couple years ago and are due for another this summer. Her spleen is already enlarged from blood flow restriction. Other kids tease her about her big tummy. Our GI doc said on our last visit he "hopes we have another 10-20 years" before we see serious problems like esophageal varices. After that we will no doubt be looking at transplant. So for us, the digestive issues might take us down before the pulmonary ones. I seem to remember reading that 20% have liver issues? Amy's right, that's not very many. Doesn't make it any easier if you're in the 20% though.
 
M

mom2leila

New member
Hey Blue,

I'm with you on this issue. I wish there was more to do, too. Our daughter presented with liver issues at 2 months of age. She was terribly jaundiced. Her acute liver disease resolved with ursodiol (which she takes twice daily for the rest of her current liver's life) and time, but her liver enzyme numbers are far from normal. Right now they are elevated but unchanged. We get abdominal ultrasounds every 6-12 months to check for a change in blood flow in the liver. We also have had a liver biopsy a couple years ago and are due for another this summer. Her spleen is already enlarged from blood flow restriction. Other kids tease her about her big tummy. Our GI doc said on our last visit he "hopes we have another 10-20 years" before we see serious problems like esophageal varices. After that we will no doubt be looking at transplant. So for us, the digestive issues might take us down before the pulmonary ones. I seem to remember reading that 20% have liver issues? Amy's right, that's not very many. Doesn't make it any easier if you're in the 20% though.
 
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Uli

New member
Hi,

has anybody with severe liver problems ever been tested for lack of Alpha-1-Antitrypsin? Thats a genetic disease also (but on another gene than cf) and it affects the lungs and the liver.
So if somebody has already been diagnosed with cf, it could easily be that no doctor thinks of this other disease, also you can have both if you are unlucky and the lack of Alpha-1-Antitrypsin needs different medication. I hope I´ve got the right name for that disease in english...it can be tested with a simple blood-test.

Uli,44, Germany, cf/cfrd
 
U

Uli

New member
Hi,

has anybody with severe liver problems ever been tested for lack of Alpha-1-Antitrypsin? Thats a genetic disease also (but on another gene than cf) and it affects the lungs and the liver.
So if somebody has already been diagnosed with cf, it could easily be that no doctor thinks of this other disease, also you can have both if you are unlucky and the lack of Alpha-1-Antitrypsin needs different medication. I hope I´ve got the right name for that disease in english...it can be tested with a simple blood-test.

Uli,44, Germany, cf/cfrd
 
U

Uli

New member
Hi,

has anybody with severe liver problems ever been tested for lack of Alpha-1-Antitrypsin? Thats a genetic disease also (but on another gene than cf) and it affects the lungs and the liver.
So if somebody has already been diagnosed with cf, it could easily be that no doctor thinks of this other disease, also you can have both if you are unlucky and the lack of Alpha-1-Antitrypsin needs different medication. I hope I´ve got the right name for that disease in english...it can be tested with a simple blood-test.

Uli,44, Germany, cf/cfrd
 
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