Pancreatic sufficient, but losing weight

[ByGrace]

Quick recap: My DS, 12 years old, was diagnosed with CRMS because of one mutation & one variant (DF508 & 5T/12TG) and borderline sweat tests (40/41). He has chronic constipation and wasn't gaining weight steadily on a growth curve so his Dr prescribed enzymes as a "trial" to see if they would help. He did begin to gain weight but I couldn't help but wonder if he was just catching up with a normal growth spurt or if the enzymes were actually doing something. The Dr that started him on enzymes left her practice and the new Dr agreed that we could do a little testing to see if he actually needed them or not. We did the one-time fecal elastase test and it came back normal at 380 (normal being >200). The Dr said he was PS so we stopped taking the enzymes. That was one week ago and he hasn't had any new GI symptoms like pain, diarrhea, or greasy stool (he's never had any of those), he is still constipated and taking Miralax daily, but now I have noticed a weight loss. We had finally gotten him up to 82 pounds (4'11" tall), and now after a week he is at 79 pounds, so maybe the enzymes were helping him after all. Here is my question: If he truly is PS, why would taking enzyme supplements help him to gain weight? In theory, his pancreas is producing all the enzymes he needs, so how would introducing more help anything? I'm so confused.

 

[Aboveallislove]

I thought I read somewhere a certain amount of time when off the enzymes before testing the stool??? How long did they wait? Maybe some googling on that issue to see if the text wasn't accurate? I'd also call the doctor and explain and see her thoughts.

Quick recap: My DS, 12 years old, was diagnosed with CRMS because of one mutation & one variant (DF508 & 5T/12TG) and borderline sweat tests (40/41). He has chronic constipation and wasn't gaining weight steadily on a growth curve so his Dr prescribed enzymes as a "trial" to see if they would help. He did begin to gain weight but I couldn't help but wonder if he was just catching up with a normal growth spurt or if the enzymes were actually doing something. The Dr that started him on enzymes left her practice and the new Dr agreed that we could do a little testing to see if he actually needed them or not. We did the one-time fecal elastase test and it came back normal at 380 (normal being >200). The Dr said he was PS so we stopped taking the enzymes. That was one week ago and he hasn't had any new GI symptoms like pain, diarrhea, or greasy stool (he's never had any of those), he is still constipated and taking Miralax daily, but now I have noticed a weight loss. We had finally gotten him up to 82 pounds (4'11" tall), and now after a week he is at 79 pounds, so maybe the enzymes were helping him after all. Here is my question: If he truly is PS, why would taking enzyme supplements help him to gain weight? In theory, his pancreas is producing all the enzymes he needs, so how would introducing more help anything? I'm so confused.

 

[ByGrace]

Good catch on that, Aboveallislove, I was trying to keep my post shorter and thought I'd leave out all the little details.
The Dr that ordered the test suggested 1 week with no enzymes before testing. The research I did about the test said that the porcine enzymes would not effect the test results since they only test for human enzymes. Still, he went off for 3 days and he did have some diarrhea and undigested food so I took a sample then so we could get him back on the enzymes. When the results came back normal the Dr said we didn't need them, but we could stay on them if we wanted to. I told her that he was only off the enzymes for 3 days, in case she thought that did have an effect on the result, but said that retesting wasn't necessary. I thought maybe I just over-reacted before and didn't give his body enough time to readjust or something. This time when he stopped taking them he didn't have any change in his stool, so I'm not sure what happened last time; a fluke maybe. I'm not sure.

 

[Rebjane]

Curious as to why the doctor wanted to take him off the enzymes if they were helping your son gain weight? Is this a CF doctor?

 

[ByGrace]

They were never sure if the enzymes were what caused the weight gain or if it was just coincidental. DS sees a CF pulmonologist but a gastroenterologist who does not specialize in CF. The GI is the one that ordered the elastase test, per my request. I guess she assumed that since his results came back normal that meant he didn't need the enzymes. I've sent a message to GI letting them know what's going on and see if they can give me some answers. We'll see.

 

[lauryn.tubes]

Hi ByGrace - my daughter is CRMS as well and her caloric consumption does not at all correlate with her low weight gain. She's not losing weight - but the girl eats like a horse and is tiny.

So i've done some research on this. "Normal" for a fecal elastase is above 500...anything below 200 is pancreatic insufficient. So what does the 380 mean? I don't really know the answer to that question but I think these CRMSers may not be absorbing food like they're supposed to be; they're not at bad as a classic CFer...but they're still not normal. If he's losing weight, or if his vitamin levels aren't good...I (personally) would have a serious conversation about enzymes. Here's the article where i got my data:

https://www.gdx.net/core/support-guides/CDSA-CDSA2-combo-Support-Guide.pdf

 

[ByGrace]

lauryn.tubes, thank you so much for that article! I will show this to his Dr and hopefully we can agree that based on his results being <500 and his weight loss that he does not have "optimal pancreatic function". That really does help answer the question for me. I've restarted the enzymes with him and I'm sure we'll see some weight gain soon.

 

[Ank]

Hi.
I think enzyme should not be stoped.As u said he had undigested food in stools .it means he need enzyme
Slowly our pancrease produces less human enzyme and therfore u need more artifical enzyme
Increasing enzyme might help...
more ever weight loss can be due liver problems and diabtese

Intially when one is young 3 meals a day cn b sufficient but as age increase u need 5 to 6 meals with energy drinks



Also i read sm ppl are benifited by pertzye(pancrelipase with hydrogen carbonate)

As basic medium is need by enzyme to digest food
Hydrogen carbone might help


As fat digestion is difficult u cn hv mre protein ..by eatin soy,egg white ..

 

[Printer]

I will answer based upon personal experience. I am 100% PI. If I skip, or take too few enzymes i will have diarrhea, not constipation. If he were not digesting his food properly, there would be an excess of fat in his stools (look for stools that float). The only time that I discuss CF with a Doctor outside of the CF Clinic is to educate them. If I were you, I would have this discussion with the Doctor in the CF Clinic, only.

Constipation CAN BE caused by swallowing mucus. This mixes with food and adheres to the colin walls, creating a blockage. Look for stools with a smaller diameter than one would expect. Merilax will help with this. Otherwise ask your Doctor to order a belly x-ray.

 

[ByGrace]

Thank you, Printer, for your experience and advice. DS has been taking Miralax daily for about 5 years now and he's had several different xrays and scans. His latest was a transit study where everything went through at a good rate, but the xray still showed a "heavy fecal burden". No one could tell me exactly what that meant, but said it wasn't anything to be concerned about. Unfortunately, when I asked his CF doctor about this pancreatic test he handed it over to the non-CF, GI doctor, so my plan of keeping it all in the CF clinic backfired on me. Sadly, because DS has a non-traditional presentation of CF, it doesn't feel like we get treated with much concern.

 

[Printer]

Google, Steven D. Freedman, MD, PhD, he is at the Beth Israel Deaconess Medical Center in Boston. I would ask your GI Doctor to call him. (617) 667-5576. He is one of the very few GI/CF Doctors in the world. A simple telephone consult could change your child's life.

5T with a high number (like 12) along with Delta F508 will act as a second mutation.

 

[Ratatosk]

With the correlation between lung function and maintaining a good BMI, I would hope your CF doctor would take this seriously. Are they at least concerned with his growth? I hated it when people compared my child's size to others his age because we struggled so much when he was younger with trying to maintain a 50th percentile; however, my son is 12 and is about 20 pounds heavier and couple inches taller.

 

[ByGrace]

Thank you, Printer, I'll do that

Ratatosk, I do believe they take note of his growth, but all they've done for it is give him enzymes. He is in the 21st percentile for BMI, but they don't see that part as a concern since it's considered a healthy weight. His CF doctor doesn't feel that any respiratory treatments are necessary with him and when we go for visits they don't even put on a mask. Fortunately he hasn't had any respiratory problems yet and hasn't cultured anything dangerous. From what I understand, DS' particular mutations don't usually present with lung issues, but I know that doesn't mean it can't happen. I'm guessing that since his lungs are healthy right now they aren't worried about his BMI being below 50%.

 

[lauryn.tubes]

Ratatosk - ByGrace's son is diagnosed with CRMS not CF. That makes it difficult for the doctors and dietitians to know where to stress things. My daughter has CRMS as well and we just had this conversation with her dietician. My daughter is in the 16% for weight to height even tho she eats like a maniac and shes PS. So...do you stress the 50% BMI or not if you're not sure if the child will ever have any issues...it's a hard boat to be on.

I feel very lucky that our clinic listens to the parents. I finally asked them "what's the harm in trying to get her to gain weight?". They really took that to heart and we're on a trial of enzymes to see if it helps her. ByGrace - i think you have to do what makes you feel most comfortable. If you want to work towards a 50% BMI...ask your team what they do for their CF patients to get there.

 

[Mistyme]

Hi Bygrace,

I have the same mutations (and slightly lower sweat chloride) as your son and I am in my 40's now. My body goes back and forth between loose, normal and constipated. I need a few hundred extra calories a day too despite being PS-even more when I am very active or have a long day. I almost never take medication for my GI issues- instead I try to eat plenty of whole grains because I think fiber helps prevent the constipation. Fiber supplements don't seem to work so I never use them. Brown rice, whole grain bread or cereal work well for me.
To maintain a healthy body weight I eat frequently. Right now I'm doing my morning treatment and have a cup of peanut butter hot chocolate to sip. I also supplement my caloric needs by taking slightly larger portions and adding things like peanut butter, nuts and seeds, olive or coconut oil, high cal dressing, etc. Avocados are great too. There are many great cooking tips and recipes for PI CF patients that can be modified/reduced to fit the needs of PS CF/CRMS patients.
I hope this helps.

 

[ByGrace]

Thank you for the advice, Mistyme. Since you have the same mutations, can I ask if you have any respiratory symptoms, or do you only have GI problems?

 

[Mistyme]

I have respiratory symptoms now but didn't as a child other than some bad coughs, congested nose and reactive airways. As a young adult I started getting frequent respiratory infections- that slowly turned into getting bronchitis every time I get sick (bad colds, flu, etc). I wasn't diagnosed until my mid 30's - I cultured staph aureus and HI. I am now in my mid-40's and cultured PA and 2 other new bugs. Late onset.
I am treated as a CF patient for infection control at my CF center and have checkups every 3 months that include PFT's and sputum cultures. My doctor wants my BMI to be good and the goal is to maintain my PFT's as high as possible. I do respiratory treatments twice a day and if sick 3x. I have been physically active for most of my life, I think that helps quite a bit and so does my doctor.