4 year old - G551D - Pancreatitis

[websterhome]

Hello, I haven't been on here in quite a while, but the phone call I received today has my mind thinking in doubletime and I thought this forum may be a good place to seek help. Our 4 year old daughter has overall had a very healthy course so far. She is pancreatic insufficient, has been on Creon since she was born, her current dose is Creon 6000, she takes 4 with meals & 3 with snacks. She has dealt on & off with constipation and does best with 1/2 cap of Miralax daily. About 2 weeks ago she had a cold with a cough, we did her vest & nebs a lot more as we'd always do, then she had a few days of vomiting & a fever, but pulled through without much intervention - just antibiotics & treatments at home. At her quarterly CF clinic appt she saw the GI doc and he wanted to act on the cautious side and send out some tests to try & find out why she's been having chronic tummy aches, they are't constant and they haven't seemed severe, but they have seemed noteworthy to my husband and me. He send bloodwork looking for Celiac Disease, pancreatitis and thyroid problems but then also said if it all came back okay but the tummy aches persisted then he would look at doing a gallbladder workup for gallstones. Well, I assumed it would all be fine and was shocked when he called today and said her amylase and lipase levels were so high that she has pancreatitis! I am a RN although I work in a Neonatal ICU and haven't cared for a patient with pancreatitis before, I just always assumed the symptoms would be much more severe than what she's had. Earlier today she was running around like crazy finishing up Christmas shopping with our family, she had a great appetite and hasn't complained of tummy aches in days. So.... help me understand this... what causes pancreatitis in CF patients? Could her Creon be the cause of the high amylase and lipase levels? -- afterall, that's two thirds of what's in creon... He wanted to admit her today to boost her hydration with an IV and monitor her. I convinced him to allow us to do clear liquid hydration orally here at home and have her blood levels (amylase and lipase) rechecked tomorrow... praying there is an improvement because he said if the levels are the same or worse then she'll be admitted, the only way home is if there's improvement. Praying that she responds well to the 1.3 liters of gatorade/ juice/ etc that I was able to get her to drink tonight! Any help understanding this would be greatly appreciated as would be prayers!
Many thanks,
Christy

 

[Julie7]

My 8 year old had acute pancreatitis which resolved in about 3 weeks with bowel rest, hydration and pain management. It is really painful so stsy on top of pain levels. My son also had a partial small bowel obstruction and we are not sure which came first. Wish a speedy recovery!!

 

[AUG19DW]

My son started when he was 12 and we did not know he had Cystic Fibrosis till the age of 15 when he had a really bad pancreatitis attack and had test after test ran and found out he had CF and had my daughter tested and she also has CF. Now my son was a very rare case in which he became worse and worse. Also he really became Chronic after the GI removed his gall bladder to see if it would help. There was nothing wrong with his gallbladder so I highly recommend never letting a GI remove it unless there are gallstones or blockages. Because my son went from 1 attack a month to 24/7 pain for years. He finally had to go to Minnesota and get the Total Pancretectomy/Islet Cell Transplant. He finally got his life back but of course still dealing with the CF issues. But at least the pancreatitis is gone. Dont want to scare you with all of this at all. Alot of kids or adults even only have acute attacks and some are not bad. I would not recommend going into the hospital for a mild attack. If she can handle the pain then she is fine. When the pain is bad yes take her to the hospital. What they usually do is make sure they are hydrated, and take them off of foods all together to let the pancreas rest. But if she is not in pain I dont see any point in doing that to her. Most of the time the attacks could last a few hours or a few days and then that is it. My sons would last a few days of alot of pain and then he would be sore for a few days after. That was the beginning for him. Now to let you know one GI told me there are very few people out there that may have pancreatitis but no pain at all. Very very rare but it does happen. My son unfortunately wasnt that lucky. He was in excrutiating pain for years and on alot of narcotics. Oh another thing dont let them give her narcotics unless it is absolutely necessary. If her pain is a 5 or above I would say yes to it but if it is something she can handle then dont. Tylenol is good. Dont do Ibuprofen or aspirin as they are not good for it and no Morphine with pancreatitis either. Tramadol or Toradol is good.

 

[Printer]

Have her admitted ASAP. Pancreatitis is nothing to fool with, even for an RN. Each attack will cause more irreparable damage. She needs to be NPO and on IVs.

Bill

 

[carrascolinn]

I am going through a similar case with my daughter she is 14, was diagnosed at 9months. She has always had great lung functions but has always had stomach issues, she just yesterday had testing done for oily stools she has been taking creons since she was 9 months old and just recently they changed her enzymes to a new brand because she has been having stomach pains. I have never heard of pancreatitis , and we are struggling to figure out what is causing her pain.

 

[websterhome]

Thank you everyone for your help with this. I guess we received good news, but it was still a bit unclear. Bear with me because it's a little confusing... Our daughter's amylase & lipase levels came down a little bit after being on just clear liquids and no food for a day, the levels were still amylase in the 200's and lipase in the 500's, so they're definitely still elevated but thankfully trending downward. The confusing detail comes because from what I've read pancreatitis only occurs in CF'ers who are pancreatic sufficient (PS), it doesn't occur in those like our daughter who take enzymes for pancreatic insufficiency (PI). Apparently, there has been discussion recently about newborns who are diagnosed PI needing to be rechecked around the 1 year to 3 year mark as it is possible for them to "outgrow" the PI and become PS. My suspicion is that our daughter may now be PS, which would overall be great news, but we'll have to wait for the stool elastase levels to come back from the out of state lab the week after Christmas. We will be following up with an abdominal ultrasound soon as well as a repeat amylase & lipase in 2 weeks. In the meantime, the GI doctor recommended she not eat high fat foods and that we decrease her enzymes by one pill to 3 w/ meals & 2 w/ snacks. What a change to go from always encouraging high fat foods to now lower fat foods until this gets all sorted out & the pancreatitis settles down. Thank you again for listening & responding! Wishing you all well ~

 

[Aboveallislove]

I jus was going to ask her mutations...I see in heading 551. What is her other. Given 551 I would think pancreatic sufficient. Did they actually test ever...ours didn't but he is 508. Also is she on kalydeco yet? I'd push for that immediately if not and also ask whether you can try some meals with even lower enzymes to see if ps, especially if they never tested.

 

[websterhome]

Sorry for the incredibly slow response! December was a real bugger for us & I'm trying to stay afloat... Long story. Anyway, with our daugter's pancreatitis, she was diagnosed at 6 days old with G551D & N1303K and on that day had a stool sample sent for PI, it came back extreme/severe PI with a level <50. We re-tested her the week after Christmas and just received the re-test, it was 141, much better but still mild to moderate PI. At this point, they still have her on Creon, they haven't changed the dose, they CF doctor says he changes enzyme dosage based upon symptoms and stool consistency and that even though she has improved PI/PS numbers, she still needs the dose she's on. There's a part of me wondering if she would do any better on Zenpep than creon? Just a thought... I don't know too much about the difference. Her amylase and lipase levels are currently Amylase 156 and Lipase 309... Still too high, we are going in for an MRCP, (a version of an MRI that focuses on the pancreas and the ducts that lead to & from it). Fingers are crossed that our 4 year old can be still enough to get through the procedure without sedation! I guess it can't be done completely with sedation because the patient needs to follow directions about breathing and holding their breath.

 

[JENNYC]

I'm sorry have to echo Aboveallislove...have you and your team considered Kalydeco?