Pancreatitis

[Tysmom]

My son is 6 yrs old. He was diagnosed with CF at 3 weeks old. After having two episodes of pancreatitis last year, they re diagnosed him with CFTR related disorder. One of his mutations is R117H 7t and the other mutation is G551D. The R117H is associated with pancreatitis. Since March 18 of this year he has had 2 episodes of pancreatitis and now last night it started again. Has anyone dealt with this? Please help me to understand why this is happening. Thank you for your help.

Kelly

 

[dc9381]

Hello! My name is Danielle and my fiance's name is Mike,
Mike was diagnosed with cf at age 13, and unfortunately I do not have his genes in front of me, but he does suffer from pancreatitis. This is what he said:

Sadly, this in unavoidable. Attacks are bound to happen. Even specialized doctors are very frustrating, giving little to no guidance other than "it happens with this type of CF" or "just keep coming into the ER when an bad attack happens." But as Mike got older, they did get less frequent. We have been together almost two years and he only had one attack just a couple weeks ago.

What helped him the most was watching how often he ate something. Mostly was spurs on an attack for him is too much breaded- high fatty foods. He stresses as soon as he felt better he would eat stuff like pizza and it would cause another attack within days. Now, this doesn't mean to shy away from the foods people with CF ultimately need to eat, but regulate the amount of food throughout the day. For example, if mike has pasta for lunch, I make something to affect of chicken, veggies, and one side of mashed potatoes for dinner. We like to think of it as not over working the pancreas with an overload of one type of food.

Also, check the dosage of his enzyme pills, for the first couple of years, they gave Mike the wrong dosage and they gave him a stronger one effectively changing his need of 15 pills a meal to 6 or 7 depending on the amount of calories. After that, the attacks did go down, but it wasn't for a couple years that they simply started fading away.

When an attack does happen, Mike only finds the fetal position comforting. It is an immense pain, and there is very little to help. Which is hard. In the end, what is best is monitor it, just make a list of when and how long the attacks happen, and compare it with a food diary. Because, sadly, with Mike it was a bunch of hit and misses even with a specialized doctor. On top of that, what is even more important is to be loving. In the end that is what he'll will remember most aside from the pain. When Mike talks of his attacks, it goes synonymously with a story about his grandma doing a funny dance; his mom stroking his back; his brothers watching a funny movie with him; all these other stories that helped get him through the rough times, and brought his spirits up.

If you have any other questions, or if we weren't very helpful, don't hesitate to ask again. Mike would love to help. Frankly, he's never met another CF'er with pancreatitis like himself.

 

[AUG19DW]

My son is 23 now and he started with Pancreatiitis at the age of 12. We didnt know he had CF until he had gotten worse with the episodes at age of 14 and went to several GI's and then one found out he had CF. He has the mutation 2789+5g>a and F508. Also my daughter was diagnosed after this also and has the same mutations. He is a very rare case is what we have been told so I dont want to scare you. But please follow the meal plans as dc9381 had explained and keep a chart it is very important.
But my son had gotten so bad that he had to go on Narcotics. He then at age 15 had to drop our of school because he could not function since he was having pain 24/7. I think what made them get this bad is the fact they removed his gallbladder to see if it would help. Of course it got worse. Before that surgery he was having 1 attack a month. 2 months after it was 24/7. He lived on narcotics and at the hospitals with no food or e.r. trips at least once a week. It was alot. Now like I said this is very rare. So dont think this will happen to your son. But we had to switch GI's about 6 or 7 times because everyone gave up on him and didnt know what else to do. I finally pursued the Total Pancreatectomy/Islet Cell Transplant the Dr. Sutherland at the Univ. Of Minn. had been doing and he had the surgery at age 17 where they removed his Pancreas and took the Islet Cells out and transplanted them into his main portal going to the liver, in which they make their way to the liver and start producing insulin. He was only diabetic for 1 yr and then off all of the insulin. It took a yr to wean off all the narcotics and then finally at 19 he had his life back. He still has some issues but nothing compared to the yrs of torture from Pancreatitis. He will have to have his colon rerouted soon but putting it off as long as he can for now.
The best way to keep these attacks away if watch what he eats and make him stay away from whatever food is causing it. Alot of fatty foods is the cause and caffeine. But everyone is different.
My son was very very different. He had gotten to where everything he ate and stress too caused it. It was a living hell! And you hate to see your child suffer cause you just want to take their pain away but just be caring and nurturing as you can. My son used to say his stomach was hurting and I just pushed it off as he didnt want to go to school. So did the GI's saying it was constipation. Not!! It was a total battle. I dont wish this on anyone ever.

 

[Julie7]

my son has poly 5t/7t and m470v x 2 copies. He had acute pancreatitis at age 9, it was horrible. he is not dx with CF, just atypical CF or symptomatic CF carrier. What CF center are you at? Agree with previous post about looking at enzyme dosage as well as food triggers. For my son, high fat foods, foods with a lot of dairy...seemed to really make things worse. We called it "shaky poops" because my son literally shoke when having these bad abdominal pains and diarrhea.
I sure hope your child finds relief.

 

[LittleLab4CF]

Pancreatitis, as CFers experience, is generally pancreatic auto-digestion. Water thin mucus loaded with digestive enzymes and sodium bicarbonate is normally produced in response to food and is squirted into the duodenum along with bile moved by a spasm of the gallbladder at the right time to meet food. If the digestive cocktail is made viscous, which CF does, it remains in the pancreas, eventually it will attack the living pancreas, digesting itself. Pancreatic auto-digestion in CF can range from total atrophy shortly after birth to a chronic condition of a semi-functioning and outrageously painful condition.

The worst pain there is, is the worst pain you've had. This is my explanation when someone tries to quantify pain or suffering. Certain types of pain are often used as a gross measure like a migraine headache, giving birth, passing a kidney stone, for a majority, pains like these are severe and common.

Long before anything was suspected of my pancreas and gallbladder I somehow found myself like a fly on the exam table while three doctors talked about pain. The bottom line was that pancreatic pain is in a category shared with bone cancer and advanced gout. My best estimate would be at least double the usual 10/10 pain measures.

When I asked my pain management specialist about what I overhead she reluctantly confirmed that it was generally true and I probably had serious chronic pancreatitis. It may sound ludicrous to consider giving a child narcotics, but to me it's tantamount to torture, if it is withheld without solid medical reasons.

I don't think there is much that can fix a pancreas that insists on having pancreatitis. I would add maximizing your hydration. Someone often suggests a gallon of water daily. In fact it is close to adult RDA for water meaning most people are dehydrated. I modify it a little by including all liquid water as long as it isn't coffee or other diuretic. With good hydration the digestive cocktail will be thinner than poor hydration. This is fact, its quality in helping depends on the individual.

The pancreas is structurally similar to a cluster of grapes except everything is produced in the "grapes" and flows to larger and larger stems. The grape like structures are hollow with a thick wall of specialized enzyme producing cells. My point is the pancreas is like a strange shaped sponge. With infants and kids, some rough play after a meal will indirectly move enzymes through our small, sticky ducts with body motion and pressure on and around the abdomen. This isn't an original thought, when an infant produces white-ish stools, implying that the bile isn't moving, rough play is often the solution because it is a mechanical problem.

The head of the pancreas is behind the bellybutton and it more or less wraps around the navel ending a little below and left of the navel. Enzymes are made starting with a meal but a meal or snack churns around in the stomach for an hour on average. Some food almost immediately passes through and when I self massage my pancreas, it seems best about an hour after eating. It isn't super critical to determine the best time for mashing a pancreas around just not so soon anybody would get sick or two hours later.

You have my sympathy, you want to keep an intact and fully functional pancreas for as long as possible. Either unmanageable pain or pain that involves so much "management" it's effectively unmanageable can become a factor in choices down the line. For most the pancreas stops hurting about the same time it stops working. In exchange for insane pain we become brittle diabetics unless an islet cell transplant is performed and we'd better get used to enzymes by mouth. The CF GUT is a common misery we quietly endure. I wish there was something to be done. I suggest strong advocacy for treating the pain of pancreatitis. Acute pancreatitis usually requires IV antibiotics and narcotics. For most this means some time in the hospital.

Hope things improve,

LL

 

[Julie7]

Littlelab, you always bring great insight. Thank you.
I agree with your statements around pain control. My son spent 3 weeks in the hospital. Nubain was barely taking the edge off his pain. Of course there are concerns over slowing the gut with narcotics but to see a 9 year old in that kind of pain is unbearable.
Once released I obtained his medical records and the hospital psychiatrist had actually written in his chart..."concerning narcotic seeking behavior. "
Really...I ended up transferring him 10 days into the admission to another hospital in a different state because of the gross negligence and incompetence.

 

[cam]

Littlelab, you always bring great insight. Thank you.
I agree with your statements around pain control. My son spent 3 weeks in the hospital. Nubain was barely taking the edge off his pain. Of course there are concerns over slowing the gut with narcotics but to see a 9 year old in that kind of pain is unbearable.
Once released I obtained his medical records and the hospital psychiatrist had actually written in his chart..."concerning narcotic seeking behavior. "
Really...I ended up transferring him 10 days into the admission to another hospital in a different state because of the gross negligence and incompetence.

I would have been so mad them sayin that bout pain meds people who really need them can't get help or its like pulling teeth for them to give anything that will help makes it so hard to get relieve. just like Getting cold medicine from behind the counter now people take it to far and doctors should know that is priority to get the patients comfortable i wish there was something we could do i hope everyone gets better and stays better and pain free

 

[stephen]

Tysmsm;

Is you son on Kalydeco? If not, why isn' he?

I see that one of his mutations is G551D. Kalydeco can do wonders (and is FDA approved) for people with that mutation. It can also do wonders for CF'ers with other Clalss 3 and Class 4 mutations.

 

[Tysmom]

Thank you to all of you that replied. I truly appreciate your help.
My son just turned six 2 weeks ago. Kalydeco was only approved for 6 and older. We have a clinic visit coming up. I will be asking if he can start to take Kalydeco in hopes that it will help with his pancreatitis. He has no lung issues and his CT scan of his lungs showed no disease. He has been sweat tested twice and his numbers are always borderline. They re diagnosed him CFTR related disorder so he may not qualify for Kalydeco. It's all confusing because he has 2 mutations, but the R117H 7t is so mild that is why they changed his diagnosis from CF to CFTR related disorder.
I just want him to stop having pancreatitis attacks. The GI doctor said there is no way to prevent them from happening.
Thanks again everyone. This site is awesome!

 

[Aboveallislove]

Tysmom, some of those on Kalydeco have been able to stop enzymes (others haven't). And Kalydeco works on the entire system. From what I understand, the pancreas is burning itself out because of CF (or the CF mutations he has). If it were me, I would insist they try Kalydeco to see if it helps the pain AND possibly prevents further destruction of the pancreas. According to cftr2.org r117h and 7t is unlikely to be disease-causing, but it would seem from the fact that his pancreas is affected that it is disease-causing to him and if he needs to be diagnosised with CF to get Kalydeco, then I'd push for that too, given that it IS disease-causing to him. Also, Kalydeco has been shown to work in virto for R117H and studies of older patients have shown great improvement in lung function. While he doesn't have those problems, I think that bodes well for Kalydeco working very well for your son given he has g551D and R117h because the med is then working on both mutations and that could really impact the pancreas. Also, fyi: Many have gotten off label under 6, so if your doctor is concerned re he is "just" 6, know that, as well as that the 2 - 6 study recently completed, so it has been studied now on younger so the safety of it is known for younger too. Good luck mom!

 

[Traveler620]

Hello,

My LO has these two mutations. He does not have any symptoms so far and has normal sweat tests 14/15 and 15/16. However, the last one was when he was 4 months. He is 19 months old now.

Does your LO take Kalydeco now? It has been approved for R117H and for 2-5 years old since this post. I am curious to see if your LO takes it and whether or not it helps with pancreatitis.

 

[Tysmom]

Hi Traveler620,
That is so interesting that you have a child with the same mutations as mine. He is doing really well and hasn't had an episode of pancreatitis in almost a year. He started Kalydeco 7 months ago. I think it is helping him. He told me he can breathe better even though all his tests showed that his lungs were healthy, he noticed a difference.

He has had three sweat tests since birth. The first sweat test at 3 weeks old was borderline with 24 and 30. He had another sweat test at age 4 and it was borderline with a 40. He had another sweat test at age 6 and it was a 51. They did another sweat test after starting the Kalydeco and his number was normal at 14. His diagnosis is CFTR related disorder. I take him to a CF clinic once a year and they will continue to follow him until something changes. Hopefully, he will continue to be healthy and the Kalydeco continues to help his pancreas.