Pancreatic Enzyme Levels ???

E

Edna0312

New member
Hi everyone,

My daughter (4 ?cf) was diagnosed at age 2 with PI. She had an endoscopy with pancreatic function test 1 1/2 years ago, and had normal lipase levels, slightly low amylase and elastase?, and very low trypsin and chromotrypsin?. (I'm not sure I spelled those right)

Anyway, she just had the endo w/panc. function redone to compare to her earlier test. The Dr. thought her levels would probably go up and she would outgrow this. This time, however, all of her enzyme levels were next to nothing! Even the lipase which had been normal was severely insufficient. Her GI dr. is following her closely and repeating these endos to keep track of what is going on with her. He does not believe she has cf based on the neg sweat tests.

Have any of you had these test repeated and what kind of results have you found?

BTW, she has had 3 negative sweat tests, and neg basic cf screening. She has had recurrent pneumonia, has had rickets from malabsorption, has had improper tooth enamel formation which has caused extensive dental work, and has fevers on a regular basis for seemingly no reason.

The docs are driving me crazy by not wanting to take this further and find out what is wrong!

Also, my 2yr old son has had more pronounce PI than my daughter. And, more pneumonias. He also has had Developmental Delays. (Not cognitive, but motor -- sitting up, crawling, walking, and talking.)

Any help or insight would be appreciated.

One more thing, please pray for us. I might have my ped. ready to send for the comprehensive Ambry test. Should find out next week.

Thanks,

Edna
(dau - 7yr; dau - 4yr ?cf; son - 2yr ?cf)
and 1 more due in Sept!
 
E

Edna0312

New member
Hi everyone,

My daughter (4 ?cf) was diagnosed at age 2 with PI. She had an endoscopy with pancreatic function test 1 1/2 years ago, and had normal lipase levels, slightly low amylase and elastase?, and very low trypsin and chromotrypsin?. (I'm not sure I spelled those right)

Anyway, she just had the endo w/panc. function redone to compare to her earlier test. The Dr. thought her levels would probably go up and she would outgrow this. This time, however, all of her enzyme levels were next to nothing! Even the lipase which had been normal was severely insufficient. Her GI dr. is following her closely and repeating these endos to keep track of what is going on with her. He does not believe she has cf based on the neg sweat tests.

Have any of you had these test repeated and what kind of results have you found?

BTW, she has had 3 negative sweat tests, and neg basic cf screening. She has had recurrent pneumonia, has had rickets from malabsorption, has had improper tooth enamel formation which has caused extensive dental work, and has fevers on a regular basis for seemingly no reason.

The docs are driving me crazy by not wanting to take this further and find out what is wrong!

Also, my 2yr old son has had more pronounce PI than my daughter. And, more pneumonias. He also has had Developmental Delays. (Not cognitive, but motor -- sitting up, crawling, walking, and talking.)

Any help or insight would be appreciated.

One more thing, please pray for us. I might have my ped. ready to send for the comprehensive Ambry test. Should find out next week.

Thanks,

Edna
(dau - 7yr; dau - 4yr ?cf; son - 2yr ?cf)
and 1 more due in Sept!
 
E

Edna0312

New member
Hi everyone,

My daughter (4 ?cf) was diagnosed at age 2 with PI. She had an endoscopy with pancreatic function test 1 1/2 years ago, and had normal lipase levels, slightly low amylase and elastase?, and very low trypsin and chromotrypsin?. (I'm not sure I spelled those right)

Anyway, she just had the endo w/panc. function redone to compare to her earlier test. The Dr. thought her levels would probably go up and she would outgrow this. This time, however, all of her enzyme levels were next to nothing! Even the lipase which had been normal was severely insufficient. Her GI dr. is following her closely and repeating these endos to keep track of what is going on with her. He does not believe she has cf based on the neg sweat tests.

Have any of you had these test repeated and what kind of results have you found?

BTW, she has had 3 negative sweat tests, and neg basic cf screening. She has had recurrent pneumonia, has had rickets from malabsorption, has had improper tooth enamel formation which has caused extensive dental work, and has fevers on a regular basis for seemingly no reason.

The docs are driving me crazy by not wanting to take this further and find out what is wrong!

Also, my 2yr old son has had more pronounce PI than my daughter. And, more pneumonias. He also has had Developmental Delays. (Not cognitive, but motor -- sitting up, crawling, walking, and talking.)

Any help or insight would be appreciated.

One more thing, please pray for us. I might have my ped. ready to send for the comprehensive Ambry test. Should find out next week.

Thanks,

Edna
(dau - 7yr; dau - 4yr ?cf; son - 2yr ?cf)
and 1 more due in Sept!
 
O

okok

New member
Wow i am so sorry to hear you are going through this. Most cases of pancreatic insuffiency in childern are due to CF. Shawaman diamond syndrome also causes PI but it is very rare (1 in 50,000 wheras CF is 1 in 2500 for caucasions) PI caused by CF does gradually worsen over time because the thick secretions plug up the pancreatic ducts over time. This process does not happen instantly. It also does not discriminate between the types of enzymes the pancreas produces though. Although some enzymes may be more resilent and able to funtion at lower concentrations.

One thing that is interesting about your situation (and i am not sure if this normally happens in CF) is that your daughter's lipase levels are normal. It is very common for CFers to malaborb fats. Do you know if your daughter malabsorbs fat at all??? In CFers, fat absorbtion takes a double hit because not only do they not have enough available lipase, bile, which is required to emulsify fats, gets clogged by the thick mucus secrections. Even with enzyme supplementation CFers still malabsorb i think about 10% of their dietary fat.

I am sure your doc has tested for shwaman diamond syndrome, right???
If i were you i would ask that your doctor do a full panel genetic testing on your childern. Also ask him to sit down with you and explain IN DETAIL what lab work your daughter has had and how it fits in with the CF picture. I know there are some lab tests that can be suggestive of CF.

I hope this helps.
 
O

okok

New member
Wow i am so sorry to hear you are going through this. Most cases of pancreatic insuffiency in childern are due to CF. Shawaman diamond syndrome also causes PI but it is very rare (1 in 50,000 wheras CF is 1 in 2500 for caucasions) PI caused by CF does gradually worsen over time because the thick secretions plug up the pancreatic ducts over time. This process does not happen instantly. It also does not discriminate between the types of enzymes the pancreas produces though. Although some enzymes may be more resilent and able to funtion at lower concentrations.

One thing that is interesting about your situation (and i am not sure if this normally happens in CF) is that your daughter's lipase levels are normal. It is very common for CFers to malaborb fats. Do you know if your daughter malabsorbs fat at all??? In CFers, fat absorbtion takes a double hit because not only do they not have enough available lipase, bile, which is required to emulsify fats, gets clogged by the thick mucus secrections. Even with enzyme supplementation CFers still malabsorb i think about 10% of their dietary fat.

I am sure your doc has tested for shwaman diamond syndrome, right???
If i were you i would ask that your doctor do a full panel genetic testing on your childern. Also ask him to sit down with you and explain IN DETAIL what lab work your daughter has had and how it fits in with the CF picture. I know there are some lab tests that can be suggestive of CF.

I hope this helps.
 
O

okok

New member
Wow i am so sorry to hear you are going through this. Most cases of pancreatic insuffiency in childern are due to CF. Shawaman diamond syndrome also causes PI but it is very rare (1 in 50,000 wheras CF is 1 in 2500 for caucasions) PI caused by CF does gradually worsen over time because the thick secretions plug up the pancreatic ducts over time. This process does not happen instantly. It also does not discriminate between the types of enzymes the pancreas produces though. Although some enzymes may be more resilent and able to funtion at lower concentrations.

One thing that is interesting about your situation (and i am not sure if this normally happens in CF) is that your daughter's lipase levels are normal. It is very common for CFers to malaborb fats. Do you know if your daughter malabsorbs fat at all??? In CFers, fat absorbtion takes a double hit because not only do they not have enough available lipase, bile, which is required to emulsify fats, gets clogged by the thick mucus secrections. Even with enzyme supplementation CFers still malabsorb i think about 10% of their dietary fat.

I am sure your doc has tested for shwaman diamond syndrome, right???
If i were you i would ask that your doctor do a full panel genetic testing on your childern. Also ask him to sit down with you and explain IN DETAIL what lab work your daughter has had and how it fits in with the CF picture. I know there are some lab tests that can be suggestive of CF.

I hope this helps.
 
O

okok

New member
I just thought of another thing....Usually ricketts is caused by a vit D defiency right...? Vit D is a fat soluble vitamin so if your daughter is malabsorbing fats then that could be related to the rickets. However, most CFers do not get rickets probably because they get exposed to enough sunlight that their bodies can produce enough vit D to prevent the disease. Do you guys live in a northern climate or a very rainy place...? does your daughter get exposed to sunlight?
 
O

okok

New member
I just thought of another thing....Usually ricketts is caused by a vit D defiency right...? Vit D is a fat soluble vitamin so if your daughter is malabsorbing fats then that could be related to the rickets. However, most CFers do not get rickets probably because they get exposed to enough sunlight that their bodies can produce enough vit D to prevent the disease. Do you guys live in a northern climate or a very rainy place...? does your daughter get exposed to sunlight?
 
O

okok

New member
I just thought of another thing....Usually ricketts is caused by a vit D defiency right...? Vit D is a fat soluble vitamin so if your daughter is malabsorbing fats then that could be related to the rickets. However, most CFers do not get rickets probably because they get exposed to enough sunlight that their bodies can produce enough vit D to prevent the disease. Do you guys live in a northern climate or a very rainy place...? does your daughter get exposed to sunlight?
 
E

Edna0312

New member
Thanks for your insight.

Let me clarify...my daughter's lipase was on the low end of normal a year and a half ago. She is now <u>completely</u> insufficient of all pancreatic enzymes. She definitely has fat malabsorption.

Yes, she and my son both have been tested for everything from intestinal infections to immune system disorders. They have both also been tested for Schwaccman Diamond. Everything is neg.

I said that my daughter had rickets, but let me clarify that. At one year she started walking. Within a couple of months you could tell that her legs were bowing badly. By 18 mos. it was so bad that the ped sent us to the Children's spine center to be evaluated. He thought she would probably outgrow it and she did. My son (also with PI) did the same thing. Now my oldest daughter is fine and had no problems. I also know that sometimes bowed legs are genetic. There is NO history of anything in either my or my husband's family. My theory is that malabsorption of vitamins and nutrients caused her bones not to form properly.

I have asked for my GI to put them on ADEK, and for some reason he hasn't. Should I push harder for that??? Are all of your children with PI on ADEK??

Thanks,

Edna
 
E

Edna0312

New member
Thanks for your insight.

Let me clarify...my daughter's lipase was on the low end of normal a year and a half ago. She is now <u>completely</u> insufficient of all pancreatic enzymes. She definitely has fat malabsorption.

Yes, she and my son both have been tested for everything from intestinal infections to immune system disorders. They have both also been tested for Schwaccman Diamond. Everything is neg.

I said that my daughter had rickets, but let me clarify that. At one year she started walking. Within a couple of months you could tell that her legs were bowing badly. By 18 mos. it was so bad that the ped sent us to the Children's spine center to be evaluated. He thought she would probably outgrow it and she did. My son (also with PI) did the same thing. Now my oldest daughter is fine and had no problems. I also know that sometimes bowed legs are genetic. There is NO history of anything in either my or my husband's family. My theory is that malabsorption of vitamins and nutrients caused her bones not to form properly.

I have asked for my GI to put them on ADEK, and for some reason he hasn't. Should I push harder for that??? Are all of your children with PI on ADEK??

Thanks,

Edna
 
E

Edna0312

New member
Thanks for your insight.

Let me clarify...my daughter's lipase was on the low end of normal a year and a half ago. She is now <u>completely</u> insufficient of all pancreatic enzymes. She definitely has fat malabsorption.

Yes, she and my son both have been tested for everything from intestinal infections to immune system disorders. They have both also been tested for Schwaccman Diamond. Everything is neg.

I said that my daughter had rickets, but let me clarify that. At one year she started walking. Within a couple of months you could tell that her legs were bowing badly. By 18 mos. it was so bad that the ped sent us to the Children's spine center to be evaluated. He thought she would probably outgrow it and she did. My son (also with PI) did the same thing. Now my oldest daughter is fine and had no problems. I also know that sometimes bowed legs are genetic. There is NO history of anything in either my or my husband's family. My theory is that malabsorption of vitamins and nutrients caused her bones not to form properly.

I have asked for my GI to put them on ADEK, and for some reason he hasn't. Should I push harder for that??? Are all of your children with PI on ADEK??

Thanks,

Edna
 
S

Samsmom

New member
Edna I wish I had answers for you. It sounds like you are doing everyting you can to get answers for your children. I'm sorry you are going through all of this. The one thing that I keep thinking is that if your daughter is completely insufficient of all pancreatic enzymes then she is either not producing them or they cannot get out of the pancreas. I wonder how many conditions/diseases could cause that?? Sounds like a mystery diagnosis case. Keep pushing until you find the answers. Best of luck.
 
S

Samsmom

New member
Edna I wish I had answers for you. It sounds like you are doing everyting you can to get answers for your children. I'm sorry you are going through all of this. The one thing that I keep thinking is that if your daughter is completely insufficient of all pancreatic enzymes then she is either not producing them or they cannot get out of the pancreas. I wonder how many conditions/diseases could cause that?? Sounds like a mystery diagnosis case. Keep pushing until you find the answers. Best of luck.
 
S

Samsmom

New member
Edna I wish I had answers for you. It sounds like you are doing everyting you can to get answers for your children. I'm sorry you are going through all of this. The one thing that I keep thinking is that if your daughter is completely insufficient of all pancreatic enzymes then she is either not producing them or they cannot get out of the pancreas. I wonder how many conditions/diseases could cause that?? Sounds like a mystery diagnosis case. Keep pushing until you find the answers. Best of luck.
 
S

sweetwhite30

New member
I will add switch doctors.They probably think your inflicting some this to her so they are not to worried it seems...Take her somewhere else to be seen,you need a second opionion in this case,and fast.....
 
S

sweetwhite30

New member
I will add switch doctors.They probably think your inflicting some this to her so they are not to worried it seems...Take her somewhere else to be seen,you need a second opionion in this case,and fast.....
 
S

sweetwhite30

New member
I will add switch doctors.They probably think your inflicting some this to her so they are not to worried it seems...Take her somewhere else to be seen,you need a second opionion in this case,and fast.....
 
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