CF patients thrive

MaeFlower · Sep 17, 2010

My daughter met with her pediatrician today & we discussed all of her current issues (pneumonitis & mucusy stools), I again asked about the possibility of a CF diagnosis. He told me that CF patients don't normally "thrive" like Vivien is doing now (her initial diagnosis was "failure to thrive," yet has since had a nissen & a g-tube placed & is gaining weight)...

My question is, do some CF patients thrive normally depending on what mutation of CF they have, whether it's a-typical or not?

MaeFlower · Sep 17, 2010

My daughter met with her pediatrician today & we discussed all of her current issues (pneumonitis & mucusy stools), I again asked about the possibility of a CF diagnosis. He told me that CF patients don't normally "thrive" like Vivien is doing now (her initial diagnosis was "failure to thrive," yet has since had a nissen & a g-tube placed & is gaining weight)...

My question is, do some CF patients thrive normally depending on what mutation of CF they have, whether it's a-typical or not?

MaeFlower · Sep 17, 2010

My daughter met with her pediatrician today & we discussed all of her current issues (pneumonitis & mucusy stools), I again asked about the possibility of a CF diagnosis. He told me that CF patients don't normally "thrive" like Vivien is doing now (her initial diagnosis was "failure to thrive," yet has since had a nissen & a g-tube placed & is gaining weight)...
 
 My question is, do some CF patients thrive normally depending on what mutation of CF they have, whether it's a-typical or not?

Mommafirst · Sep 17, 2010

Not all cf patients are destined for failure to thrive issues. My daughter was a chubby baby who eventually fell into the ftt category after a year of age. But some cf patients are pancreatic sufficient and have little issue with weight or are overweight. Plus, my daughter's ftt was resolved with the feeding tube that she still has. So saying your child with a tube isn't a cf kid because she isn't ftt anymore seems silly.

Mommafirst · Sep 17, 2010

Not all cf patients are destined for failure to thrive issues. My daughter was a chubby baby who eventually fell into the ftt category after a year of age. But some cf patients are pancreatic sufficient and have little issue with weight or are overweight. Plus, my daughter's ftt was resolved with the feeding tube that she still has. So saying your child with a tube isn't a cf kid because she isn't ftt anymore seems silly.

Mommafirst · Sep 17, 2010

Not all cf patients are destined for failure to thrive issues. My daughter was a chubby baby who eventually fell into the ftt category after a year of age. But some cf patients are pancreatic sufficient and have little issue with weight or are overweight. Plus, my daughter's ftt was resolved with the feeding tube that she still has. So saying your child with a tube isn't a cf kid because she isn't ftt anymore seems silly.

hmw · Sep 17, 2010

My daughter wasn't dx'ed with cf until she was 7. She was chubby until she was 3... then started falling off the growth charts and by the time she was dx'ed was in a state of growth failure. She was clearly pancreatic sufficient the first few years of her life- she ate normally, no enzymes, etc. She now has a feeding tube and is gaining weight very rapidly.

It was explained to us that the right type of nutrition can be much easier to digest, even for kids with cf- since she seems to need fewer enzymes for it than she would regular meals- and is gaining a lot more weight than she ever did with an equivalent amount of 'regular food' calories.

I would definitely continue to push for testing- it doesn't make sense to rule out the dx based on this criteria.

hmw · Sep 17, 2010

My daughter wasn't dx'ed with cf until she was 7. She was chubby until she was 3... then started falling off the growth charts and by the time she was dx'ed was in a state of growth failure. She was clearly pancreatic sufficient the first few years of her life- she ate normally, no enzymes, etc. She now has a feeding tube and is gaining weight very rapidly.

It was explained to us that the right type of nutrition can be much easier to digest, even for kids with cf- since she seems to need fewer enzymes for it than she would regular meals- and is gaining a lot more weight than she ever did with an equivalent amount of 'regular food' calories.

I would definitely continue to push for testing- it doesn't make sense to rule out the dx based on this criteria.

hmw · Sep 17, 2010

My daughter wasn't dx'ed with cf until she was 7. She was chubby until she was 3... then started falling off the growth charts and by the time she was dx'ed was in a state of growth failure. She was clearly pancreatic sufficient the first few years of her life- she ate normally, no enzymes, etc. She now has a feeding tube and is gaining weight very rapidly.
 
 It was explained to us that the right type of nutrition can be much easier to digest, even for kids with cf- since she seems to need fewer enzymes for it than she would regular meals- and is gaining a lot more weight than she ever did with an equivalent amount of 'regular food' calories.
 
 I would definitely continue to push for testing- it doesn't make sense to rule out the dx based on this criteria.

LouLou · Sep 18, 2010

I was a chubby baby 30 years ago who was not diagnosed until 30 months despite 3 positive sweat tests! Despite being a healthy size I was and am pancreatic insufficient. Like Hariett said, we are able to get the nutrition out of some food better than others and breast milk is one of the things that many of us thrived on.

Cf patients are not always failure to thrive and isn't the reason your daughter is thriving now because of all the interventions she's had? How does her doctor justify her needing a g-tube?

My son is PS. Genetic information is below.

Please insist on genetic sequencing which will test her for over one thousand mutations.

I read your post to my husband. We commented how we think it's time you found a new doctor. Sorry to be so blunt but it seems he's in the dark ages about cf knowledge. You are in charge of your daughters care. Did the sweat testing get completed at an accredited cf center? You should be working with them not your pedi for further testing.

LouLou · Sep 18, 2010

I was a chubby baby 30 years ago who was not diagnosed until 30 months despite 3 positive sweat tests! Despite being a healthy size I was and am pancreatic insufficient. Like Hariett said, we are able to get the nutrition out of some food better than others and breast milk is one of the things that many of us thrived on.

Cf patients are not always failure to thrive and isn't the reason your daughter is thriving now because of all the interventions she's had? How does her doctor justify her needing a g-tube?

My son is PS. Genetic information is below.

Please insist on genetic sequencing which will test her for over one thousand mutations.

I read your post to my husband. We commented how we think it's time you found a new doctor. Sorry to be so blunt but it seems he's in the dark ages about cf knowledge. You are in charge of your daughters care. Did the sweat testing get completed at an accredited cf center? You should be working with them not your pedi for further testing.

LouLou · Sep 18, 2010

I was a chubby baby 30 years ago who was not diagnosed until 30 months despite 3 positive sweat tests! Despite being a healthy size I was and am pancreatic insufficient. Like Hariett said, we are able to get the nutrition out of some food better than others and breast milk is one of the things that many of us thrived on.
 
 Cf patients are not always failure to thrive and isn't the reason your daughter is thriving now because of all the interventions she's had? How does her doctor justify her needing a g-tube?
 
 My son is PS. Genetic information is below.
 
 Please insist on genetic sequencing which will test her for over one thousand mutations.
 
 I read your post to my husband. We commented how we think it's time you found a new doctor. Sorry to be so blunt but it seems he's in the dark ages about cf knowledge. You are in charge of your daughters care. Did the sweat testing get completed at an accredited cf center? You should be working with them not your pedi for further testing.

MaeFlower · Sep 18, 2010

Thanks ladies. Vivi's first test was completed at a credited CF testing center, yet they are not accredited to treat CF patients...not sure how that works. The test was borderline of 44. Her initial genetic test came back that she was not a carrier for the most common mutations of CF. She has had pneumonia/pneumonitis 4 times since birth. She has mucusy stools & is on Elecare 24calorie, for 16 hours a day. So I too believe she doesn't have much of a choice but to gain (yet, I may be misguided in believing this).

She has another sweat test scheduled this Tuesday with an accredited CF Pulmonologist. Depending on what that test holds, the CF center may continue to pursue the full panel.

Thanks so much for the feedback. I truly believe you are all the experts to go to because the medical professionals only get a small glimpse of what it is like, whereas you all/we all live it everyday.

MaeFlower · Sep 18, 2010

Thanks ladies. Vivi's first test was completed at a credited CF testing center, yet they are not accredited to treat CF patients...not sure how that works. The test was borderline of 44. Her initial genetic test came back that she was not a carrier for the most common mutations of CF. She has had pneumonia/pneumonitis 4 times since birth. She has mucusy stools & is on Elecare 24calorie, for 16 hours a day. So I too believe she doesn't have much of a choice but to gain (yet, I may be misguided in believing this).

She has another sweat test scheduled this Tuesday with an accredited CF Pulmonologist. Depending on what that test holds, the CF center may continue to pursue the full panel.

Thanks so much for the feedback. I truly believe you are all the experts to go to because the medical professionals only get a small glimpse of what it is like, whereas you all/we all live it everyday.

MaeFlower · Sep 18, 2010

Thanks ladies. Vivi's first test was completed at a credited CF testing center, yet they are not accredited to treat CF patients...not sure how that works. The test was borderline of 44. Her initial genetic test came back that she was not a carrier for the most common mutations of CF. She has had pneumonia/pneumonitis 4 times since birth. She has mucusy stools & is on Elecare 24calorie, for 16 hours a day. So I too believe she doesn't have much of a choice but to gain (yet, I may be misguided in believing this).
 
 She has another sweat test scheduled this Tuesday with an accredited CF Pulmonologist. Depending on what that test holds, the CF center may continue to pursue the full panel.
 
 Thanks so much for the feedback. I truly believe you are all the experts to go to because the medical professionals only get a small glimpse of what it is like, whereas you all/we all live it everyday.

hmw · Sep 18, 2010

EleCare is an elemental formula that would be exceptionally easy to digest... the broken down proteins and type of fat it's made with are easier for those with cf to absorb. Emily is also on an elemental formula and she's gaining so much weight. I discussed this quite a bit with her dr and nutritionist when deciding what to use for her tube feeds because I wasn't sure how the way we were giving her enzymes would help her through an entire night's feed and they explained that the broken down proteins and the type of fat in elemental formulas were so much easier for those with cf to absorb that the need for enzymes was often reduced. So I am not surprised at all that your baby is gaining more weight, esp since pancreatic insufficiency is not always as severe in young infants as it will be later in life. The fact that her stools continue to look the way they do, though, is one way to tell that if she has cf she would definitely benefit from taking enzymes.

I'm glad she's seeing an accredited dr. I would push HARD for genetic sequencing- don't wait for the dr to pursue it. Your daughter has so much going on this needs to be ruled out. Esp in an infant, a sweat test of 44 is high enough to be quite concerned; babies often test lower than older children. I would ask for the 'Ambry CF Amplified w/ deletions and duplications' test. That test has been pretty much the most thorough in genetic testing for CF for quite some time now. If Ambry is not an option, ask for the Quest sequencing test ('Cystic Fibrosis Complete Rare Mutation Analysis, w/ deletions and duplications.') The other sequencing tests out there are not quite as comprehensive as these.

We aren't medical professionals so can only offer advice based on our own experiences... but I feel like you do... other parents living it day in and day out can help so much! We are here to answer as many of your questions and be of as much support as we can.

hmw · Sep 18, 2010

EleCare is an elemental formula that would be exceptionally easy to digest... the broken down proteins and type of fat it's made with are easier for those with cf to absorb. Emily is also on an elemental formula and she's gaining so much weight. I discussed this quite a bit with her dr and nutritionist when deciding what to use for her tube feeds because I wasn't sure how the way we were giving her enzymes would help her through an entire night's feed and they explained that the broken down proteins and the type of fat in elemental formulas were so much easier for those with cf to absorb that the need for enzymes was often reduced. So I am not surprised at all that your baby is gaining more weight, esp since pancreatic insufficiency is not always as severe in young infants as it will be later in life. The fact that her stools continue to look the way they do, though, is one way to tell that if she has cf she would definitely benefit from taking enzymes.

I'm glad she's seeing an accredited dr. I would push HARD for genetic sequencing- don't wait for the dr to pursue it. Your daughter has so much going on this needs to be ruled out. Esp in an infant, a sweat test of 44 is high enough to be quite concerned; babies often test lower than older children. I would ask for the 'Ambry CF Amplified w/ deletions and duplications' test. That test has been pretty much the most thorough in genetic testing for CF for quite some time now. If Ambry is not an option, ask for the Quest sequencing test ('Cystic Fibrosis Complete Rare Mutation Analysis, w/ deletions and duplications.') The other sequencing tests out there are not quite as comprehensive as these.

We aren't medical professionals so can only offer advice based on our own experiences... but I feel like you do... other parents living it day in and day out can help so much! We are here to answer as many of your questions and be of as much support as we can.

hmw · Sep 18, 2010

EleCare is an elemental formula that would be exceptionally easy to digest... the broken down proteins and type of fat it's made with are easier for those with cf to absorb. Emily is also on an elemental formula and she's gaining so much weight. I discussed this quite a bit with her dr and nutritionist when deciding what to use for her tube feeds because I wasn't sure how the way we were giving her enzymes would help her through an entire night's feed and they explained that the broken down proteins and the type of fat in elemental formulas were so much easier for those with cf to absorb that the need for enzymes was often reduced. So I am not surprised at all that your baby is gaining more weight, esp since pancreatic insufficiency is not always as severe in young infants as it will be later in life. The fact that her stools continue to look the way they do, though, is one way to tell that if she has cf she would definitely benefit from taking enzymes.
 
 I'm glad she's seeing an accredited dr. I would push HARD for genetic sequencing- don't wait for the dr to pursue it. Your daughter has so much going on this needs to be ruled out. Esp in an infant, a sweat test of 44 is high enough to be quite concerned; babies often test lower than older children. I would ask for the 'Ambry CF Amplified w/ deletions and duplications' test. That test has been pretty much the most thorough in genetic testing for CF for quite some time now. If Ambry is not an option, ask for the Quest sequencing test ('Cystic Fibrosis Complete Rare Mutation Analysis, w/ deletions and duplications.') The other sequencing tests out there are not quite as comprehensive as these.
 
 We aren't medical professionals so can only offer advice based on our own experiences... but I feel like you do... other parents living it day in and day out can help so much! We are here to answer as many of your questions and be of as much support as we can.

Fancymushroom · Sep 18, 2010

Hi,

I fully agree with everything the other posters have said. It's often not till later in life that the digestion gets worse and even then, there are quite a few people who get enough calories from what they eat to maintain a good weight. I have a friend who who literally has NO pancreas funtion at all and severe digestive problems and yet doesn't find it difficult to maintain her weight. (She eats alot though). I was diagnosed at the age of 19 which is obviously very late, as my lungs have always been my main problem. However, I had horrible digestion before dx, only ate foods with little or no fat, have always been short and slim, and was constantly eating (carbs, carbs, carbs!!) back then. I was slim but not terribly underweight, yet I was dxed with a sweat test of over 100.
These days (nearly 3 years on) I take as many enzymes as the next person wcf, have to do night feeds to maintain my weight at a reasonable level, and get blockages occasionally. Which goes to show how much things can change in a short space of time. Your baby's sweat test should probably be ringing some alarm bells even if it's not diagnostic. Have they tested her stool for enzymes/fat? x

Fancymushroom · Sep 18, 2010

Hi,

I fully agree with everything the other posters have said. It's often not till later in life that the digestion gets worse and even then, there are quite a few people who get enough calories from what they eat to maintain a good weight. I have a friend who who literally has NO pancreas funtion at all and severe digestive problems and yet doesn't find it difficult to maintain her weight. (She eats alot though). I was diagnosed at the age of 19 which is obviously very late, as my lungs have always been my main problem. However, I had horrible digestion before dx, only ate foods with little or no fat, have always been short and slim, and was constantly eating (carbs, carbs, carbs!!) back then. I was slim but not terribly underweight, yet I was dxed with a sweat test of over 100.
These days (nearly 3 years on) I take as many enzymes as the next person wcf, have to do night feeds to maintain my weight at a reasonable level, and get blockages occasionally. Which goes to show how much things can change in a short space of time. Your baby's sweat test should probably be ringing some alarm bells even if it's not diagnostic. Have they tested her stool for enzymes/fat? x

CF patients thrive

New member

New member

New member

Mommafirst

Guest

Mommafirst

Guest

Mommafirst

Guest

New member

New member

New member

New member

New member

New member

New member

New member

New member

New member

New member

New member

New member

New member