Question about progression

[Justinsmom]

<P>Hi,</P>
<P> </P>
<P>We are just going through the diagnostic process for my 7 year old son.  He began with pancreatic insufficiency right after turning 6.  He started with frequent loose stools (8+ a day).  In late January his Fecal Elastase number was 148, by early March it was 70 and by July it was 25.  He also developed intollereance to other foods (frutose).  He started on Creon and has gained 3 pounds (after 1 1/2 years of no weight gain) in 2 months.  As his body has healed, he has lost much of the intollerance, as our CF-GI hoped he would.  He now can eat without pain and bloating.  His genetic test showed 1 mutation and we will be retesting with Ambry to look for the second.  </P>
<P> </P>
<P>My question is, for parents whose children showed first with pancreatic issues, how long until the lung issues started.  I understand every case is different.  Justin has had mild asthma (does well while on Singulair) and only has needed his inhaler a couple times a year.  This year, however, he has had a dry cough every morning for the past week.  It gets worse if he is running around.  We have used his inhaler and then he is fine for the rest of the day.  Does this sound like asthma or early signs of lung involvement?  Do the two go together?  Do people ever get only pancreatic and not lung or is it progessive?  Does the fact that he has pancreatic involvement tend to indicate a more "severe" presentation?  Does the pancreas usually decline that quickly? </P>
<P> </P>
<P>Thank you for sharing your experiences.  </P>
<P> </P>
<P>Josette </P>
<P>Going through diagnosis process for bright, energetic, spirited boy who may have CF</P>

 

[Justinsmom]

<P>Hi,</P>
<P></P>
<P>We are just going through the diagnostic process for my 7 year old son. He began with pancreatic insufficiency right after turning 6. He started with frequent loose stools (8+ a day). In late January his Fecal Elastase number was 148, by early March it was 70 and by July it was 25. He also developed intollereance to other foods (frutose). He started on Creon and has gained 3 pounds (after 1 1/2 years of no weight gain) in 2 months. As his body has healed, he has lost much of the intollerance, as our CF-GI hoped he would. He now can eat withoutpain and bloating. His genetic test showed 1 mutation and we will be retestingwith Ambry to look for the second. </P>
<P></P>
<P>My question is, for parents whose children showed first with pancreatic issues, how long until the lung issues started. I understand every case is different.Justin has had mild asthma (does well while on Singulair) and only has needed his inhaler a couple times a year. This year, however, he has had a dry cough every morning forthe past week. It gets worse if he is running around. We have used his inhaler and then he is fine for the rest of the day. Does this sound like asthma or early signs of lung involvement? Do the two go together? Do people ever get only pancreatic and not lung or is it progessive? Does the fact that he has pancreatic involvement tend to indicate a more "severe" presentation? Does the pancreas usually decline that quickly? </P>
<P></P>
<P>Thank you for sharing your experiences. </P>
<P></P>
<P>Josette </P>
<P>Going through diagnosis process for bright, energetic, spirited boy who may have CF</P>

 

[Justinsmom]

<P><BR>Hi,</P>
<P></P>
<P>We are just going through the diagnostic process for my 7 year old son. He began with pancreatic insufficiency right after turning 6. He started with frequent loose stools (8+ a day). In late January his Fecal Elastase number was 148, by early March it was 70 and by July it was 25. He also developed intollereance to other foods (frutose). He started on Creon and has gained 3 pounds (after 1 1/2 years of no weight gain) in 2 months. As his body has healed, he has lost much of the intollerance, as our CF-GI hoped he would. He now can eat withoutpain and bloating. His genetic test showed 1 mutation and we will be retestingwith Ambry to look for the second. </P>
<P></P>
<P>My question is, for parents whose children showed first with pancreatic issues, how long until the lung issues started. I understand every case is different.Justin has had mild asthma (does well while on Singulair) and only has needed his inhaler a couple times a year. This year, however, he has had a dry cough every morning forthe past week. It gets worse if he is running around. We have used his inhaler and then he is fine for the rest of the day. Does this sound like asthma or early signs of lung involvement? Do the two go together? Do people ever get only pancreatic and not lung or is it progessive? Does the fact that he has pancreatic involvement tend to indicate a more "severe" presentation? Does the pancreas usually decline that quickly? </P>
<P></P>
<P>Thank you for sharing your experiences. </P>
<P></P>
<P>Josette </P>
<P>Going through diagnosis process for bright, energetic, spirited boy who may have CF</P>

 

[Ratatosk]

DS mainly had digestive issues. Was diagnosed shortly after he was born. His doctor immediately had us begin chest physiotherapy 3-4 times a day with bronchiodialators to keep his lungs healthy. Baseline is no cough -- if he does cough, usually means he's coming down with something. Despite no symptoms, he has cultured typical CF bugs in the past -- psuedomonas, steno. maltophilia, H. flu....

 

[Ratatosk]

DS mainly had digestive issues. Was diagnosed shortly after he was born. His doctor immediately had us begin chest physiotherapy 3-4 times a day with bronchiodialators to keep his lungs healthy. Baseline is no cough -- if he does cough, usually means he's coming down with something. Despite no symptoms, he has cultured typical CF bugs in the past -- psuedomonas, steno. maltophilia, H. flu....

 

[Ratatosk]

DS mainly had digestive issues. Was diagnosed shortly after he was born. His doctor immediately had us begin chest physiotherapy 3-4 times a day with bronchiodialators to keep his lungs healthy. Baseline is no cough -- if he does cough, usually means he's coming down with something. Despite no symptoms, he has cultured typical CF bugs in the past -- psuedomonas, steno. maltophilia, H. flu....

 

[Rebjane]

Hi Josette,

Cystic Fibrosis is a complicated disease. It also is very individualized, meaning just because one person has certain symptoms does not mean that another person will have those same exact symptoms.

Are you going to an CF center? The Cystic Fibrosis Foundation at www.cff.org has a listing of CF centers near you as well as information about Cystic Fibrosis.

CF is a progressive disease, however, like I said everyone is different. At this point, someone who is diagnosed with CF, will eventually have respiratory symptoms. The goal is to keep those lungs as healthy as possible until a cure is found, or better treatments come to market.

My daughter is 8(will be 9 in a few months) She has severe pancreatic insufficiency. She has been taking enzymes since she was a newborn. She does have respiratory involvement as well. However, our family is diligent with her breathing treatments and VEST therapy. She had not been in the hospital since she was 2 and a half years old(knock wood and everything else). My daughter does to public school, plays soccer(is actually one of the "better" players on the team), she sings, plays baseball, skies.

There are SO many things we can do to keep our CF children well; however they will get sick. It is the nature of the beast. Our family tries to do our best and control what we can and make our lives and our kids lives as full as we can.

Good luck!

You have asked lots of good questions..Keep asking away.

 

[Rebjane]

Hi Josette,

Cystic Fibrosis is a complicated disease. It also is very individualized, meaning just because one person has certain symptoms does not mean that another person will have those same exact symptoms.

Are you going to an CF center? The Cystic Fibrosis Foundation at www.cff.org has a listing of CF centers near you as well as information about Cystic Fibrosis.

CF is a progressive disease, however, like I said everyone is different. At this point, someone who is diagnosed with CF, will eventually have respiratory symptoms. The goal is to keep those lungs as healthy as possible until a cure is found, or better treatments come to market.

My daughter is 8(will be 9 in a few months) She has severe pancreatic insufficiency. She has been taking enzymes since she was a newborn. She does have respiratory involvement as well. However, our family is diligent with her breathing treatments and VEST therapy. She had not been in the hospital since she was 2 and a half years old(knock wood and everything else). My daughter does to public school, plays soccer(is actually one of the "better" players on the team), she sings, plays baseball, skies.

There are SO many things we can do to keep our CF children well; however they will get sick. It is the nature of the beast. Our family tries to do our best and control what we can and make our lives and our kids lives as full as we can.

Good luck!

You have asked lots of good questions..Keep asking away.

 

[Rebjane]

Hi Josette,
<br />
<br />Cystic Fibrosis is a complicated disease. It also is very individualized, meaning just because one person has certain symptoms does not mean that another person will have those same exact symptoms.
<br />
<br />Are you going to an CF center? The Cystic Fibrosis Foundation at www.cff.org has a listing of CF centers near you as well as information about Cystic Fibrosis.
<br />
<br />CF is a progressive disease, however, like I said everyone is different. At this point, someone who is diagnosed with CF, will eventually have respiratory symptoms. The goal is to keep those lungs as healthy as possible until a cure is found, or better treatments come to market.
<br />
<br />My daughter is 8(will be 9 in a few months) She has severe pancreatic insufficiency. She has been taking enzymes since she was a newborn. She does have respiratory involvement as well. However, our family is diligent with her breathing treatments and VEST therapy. She had not been in the hospital since she was 2 and a half years old(knock wood and everything else). My daughter does to public school, plays soccer(is actually one of the "better" players on the team), she sings, plays baseball, skies.
<br />
<br />There are SO many things we can do to keep our CF children well; however they will get sick. It is the nature of the beast. Our family tries to do our best and control what we can and make our lives and our kids lives as full as we can.
<br />
<br />Good luck!
<br />
<br />You have asked lots of good questions..Keep asking away.

 

[Mommafirst]

I think your questions are good ones, I wish there were good answers. I'm one who uses a lot of deductive logic, and it would seem to make sense that if the CFTR has stopped working enough to cause a sudden onset of pancreatic insufficiency, that other CFTR related symptoms would follow.....but I don't believe that is NECESSARILY the case. MOST kids with CF are born with severe pancreatic insufficiency, and the onset of lung disease STILL varies for them. My daughter, on the other hand, was born pancreatic sufficient and still seems to be so (though borderline -- numbers not high enough to be considered normal, not low enough to be insufficient), though we are all prepared for the eventual decline in this area. She has been hospitalized for IV antibiotics 4 times (she's 5) because the staph she has always cultured really can kick her butt and doesn't respond well to oral abx. But even with those hospitalizations, she is considered to be doing very well (for a kid with CF :/ )

I know it is so hard to just let the disease run its course and not want to know where it is going and how fast it will get there. Its miserable to not know the future....hopefully someday there will be more knowledge to answer your questions and better predict who progresses faster and why, as its really hard to cure a disease when you don't really know why.

 

[Mommafirst]

I think your questions are good ones, I wish there were good answers. I'm one who uses a lot of deductive logic, and it would seem to make sense that if the CFTR has stopped working enough to cause a sudden onset of pancreatic insufficiency, that other CFTR related symptoms would follow.....but I don't believe that is NECESSARILY the case. MOST kids with CF are born with severe pancreatic insufficiency, and the onset of lung disease STILL varies for them. My daughter, on the other hand, was born pancreatic sufficient and still seems to be so (though borderline -- numbers not high enough to be considered normal, not low enough to be insufficient), though we are all prepared for the eventual decline in this area. She has been hospitalized for IV antibiotics 4 times (she's 5) because the staph she has always cultured really can kick her butt and doesn't respond well to oral abx. But even with those hospitalizations, she is considered to be doing very well (for a kid with CF :/ )

I know it is so hard to just let the disease run its course and not want to know where it is going and how fast it will get there. Its miserable to not know the future....hopefully someday there will be more knowledge to answer your questions and better predict who progresses faster and why, as its really hard to cure a disease when you don't really know why.

 

[Mommafirst]

I think your questions are good ones, I wish there were good answers. I'm one who uses a lot of deductive logic, and it would seem to make sense that if the CFTR has stopped working enough to cause a sudden onset of pancreatic insufficiency, that other CFTR related symptoms would follow.....but I don't believe that is NECESSARILY the case. MOST kids with CF are born with severe pancreatic insufficiency, and the onset of lung disease STILL varies for them. My daughter, on the other hand, was born pancreatic sufficient and still seems to be so (though borderline -- numbers not high enough to be considered normal, not low enough to be insufficient), though we are all prepared for the eventual decline in this area. She has been hospitalized for IV antibiotics 4 times (she's 5) because the staph she has always cultured really can kick her butt and doesn't respond well to oral abx. But even with those hospitalizations, she is considered to be doing very well (for a kid with CF :/ )
<br />
<br />I know it is so hard to just let the disease run its course and not want to know where it is going and how fast it will get there. Its miserable to not know the future....hopefully someday there will be more knowledge to answer your questions and better predict who progresses faster and why, as its really hard to cure a disease when you don't really know why.

 

[Kaethe108]

Hi Josette,
with a few of your questions I can at least tell you the answers we got from our CF centre.
Our daughter was pancreatic insufficient since she was born but that does not mean that her case will be more severe. Also how much the pancreas is involved does not say anything about how severe the respiratory involvement will be. The pancreas is simply quite vulnerable and in many cases gets destroyed pretty soon.
This also depends on the combination of mutations. With our combination we can hope that lung involvement starts only in teenage age. In most cases with the same combination it was like this, but: Every case is different. It depends on many other factors. E.g. our professor said the daily treatment routine makes about 50-60% of the disease's progression.
I know how you feel! I would also like somebody tell us, when and what we have to expect. But nobody can... So with every little cough I get scared that NOW "it" starts...
The only thing that is SURE is that we can have a huge influence on how our kids will develop. And this is our task...
I hope you are in close contact with a specialized CF centre. And please read something about the new medicaments that are already in the pipeline and available quite soon. This will give you hope and strength. At least it does with me.

All the best,
Kaethe

 

[Kaethe108]

Hi Josette,
with a few of your questions I can at least tell you the answers we got from our CF centre.
Our daughter was pancreatic insufficient since she was born but that does not mean that her case will be more severe. Also how much the pancreas is involved does not say anything about how severe the respiratory involvement will be. The pancreas is simply quite vulnerable and in many cases gets destroyed pretty soon.
This also depends on the combination of mutations. With our combination we can hope that lung involvement starts only in teenage age. In most cases with the same combination it was like this, but: Every case is different. It depends on many other factors. E.g. our professor said the daily treatment routine makes about 50-60% of the disease's progression.
I know how you feel! I would also like somebody tell us, when and what we have to expect. But nobody can... So with every little cough I get scared that NOW "it" starts...
The only thing that is SURE is that we can have a huge influence on how our kids will develop. And this is our task...
I hope you are in close contact with a specialized CF centre. And please read something about the new medicaments that are already in the pipeline and available quite soon. This will give you hope and strength. At least it does with me.

All the best,
Kaethe

 

[Kaethe108]

Hi Josette,
<br />with a few of your questions I can at least tell you the answers we got from our CF centre.
<br />Our daughter was pancreatic insufficient since she was born but that does not mean that her case will be more severe. Also how much the pancreas is involved does not say anything about how severe the respiratory involvement will be. The pancreas is simply quite vulnerable and in many cases gets destroyed pretty soon.
<br />This also depends on the combination of mutations. With our combination we can hope that lung involvement starts only in teenage age. In most cases with the same combination it was like this, but: Every case is different. It depends on many other factors. E.g. our professor said the daily treatment routine makes about 50-60% of the disease's progression.
<br />I know how you feel! I would also like somebody tell us, when and what we have to expect. But nobody can... So with every little cough I get scared that NOW "it" starts...
<br />The only thing that is SURE is that we can have a huge influence on how our kids will develop. And this is our task...
<br />I hope you are in close contact with a specialized CF centre. And please read something about the new medicaments that are already in the pipeline and available quite soon. This will give you hope and strength. At least it does with me.
<br />
<br />All the best,
<br /> Kaethe

 

[Printer]

Josette:

I was dx at age 47 after many years of Acute Pancreatitis. I am completly PI (10 Creon 24 with every meal) but still have very good lung function FEV1 @ 74%.

Bill

 

[Printer]

Josette:

I was dx at age 47 after many years of Acute Pancreatitis. I am completly PI (10 Creon 24 with every meal) but still have very good lung function FEV1 @ 74%.

Bill

 

[Printer]

Josette:
<br />
<br />I was dx at age 47 after many years of Acute Pancreatitis. I am completly PI (10 Creon 24 with every meal) but still have very good lung function FEV1 @ 74%.
<br />
<br />Bill