7 month old son has moderate pancreatic insufficiency. Confused.

[Cricket's mom]

CF or not, if your child has health issues, you need to keep pushing for answers. One thing DH reminded me of when we had issues with doctors not wanting to perform tests or telling me that DS' cough (bronchitis) as an infant was normal, is that we are the customers and we are PAYING for these appointments. We were also advised that when we request a specific test and the doctor says no, to indicate that you want that put in writing and added to your child's file. "Parents requested a scan to determine if there was an obstruction or culture to determine possible infection. Clinic refused because...."

Thank you so much Ratatosk. Your husband's insight is such an important thing to remember. It's hard sometimes because Drs can make you feel like your crazy when you don't exactly see eye to eye with them. I will try to keep your husband's words in mind if i start to feel as if they are being bullies. So far, on the dr front, so good. I feel very fortunate that we have such good people looking into Chris' health. We'll figure out what is happening.

I also will, from now on, even where it concerns my own health, ask them to document their hesitation if they ever refuse to investigate something that is weighing heavily on me. I suspect that request would turn around an attitude very quickly.

Thank you again for the concern and advice you have given me. I thought of you all while she was examining the baby.

 

[Cricket's mom]

My advice is to keep fighting for what your gut is telling you. My story is very similar to Justinsmama. My boy was positive on the heal prick test and then negative on a sweat test (12). For the first 12 months we had so many Upper airway infections, sinus issues, ear infections, and your picture just brings back memories. my boy was seen by the CF team twice and on each occasion gave a negative sweat test. He was tested for everything else and nothing found. Finally he had to be put on crean as he started to get odemas in his feet from protein defficiency. We had a different child from that time on. No more tummy pains, direah or swollen feet. He is being treated by the CF team and will soon be going under to see what exactly is going on with his pancreas as they still don't feel it is CF. He will be having low swabs of his lungs as well for his cough. He has had his cough since birth and they cant accept it is all linked all because of the "gold standard" sweat test. I initiated a full DNA sequencing which came back with D508 and 1716G/A. The later mutation is in the CFTR gene but not known to be disease causing. It is linked to pancreatic issues. They also found nine sequence varients. The genmeticists told me that possibly with all linked together combined with his DNA structure could cause "mild" CF symptoms. The CF team are confused as "mild" is usually PS where my son (2) is PI, hence he is not text book and no diagnosis as yet. I am still fighting my battle, all the best for yours, the sooner he gets treatment the better and healthier he will be. goodluck!

I hope you also get some definitive answers. Best wishes to you.

 

[Printer]

Ratatosk:

Your hubby is wrong, you are not paying for appointments and tests, your health insurance company is. Be careful in demanding tests that your Doctor says are not indicated. If the insurance company refuses to pay, (based upon your Doctor's notes) it will be your sole responsibility to pay 100% of all costs.

Bill

 

[Ratatosk]

Bill, I was talking more in terms of the way one is treated in the clinic environment. We tend to get this more often than not because we live in a rural area where one is taught not to question the almighty wisdom of the medical professionals, who tend to have more of a generalized training in medicine rather than the specialization we get in the bigger cities. The idea that we're supposed to just take a doctor's word and condescending attitude, when we and insurance are paying for that "abuse" is ridiculous. Especially when they have obvious symptoms showing something isn't quite right. More often I'd cool my heels in a waiting room because the doctor was running late, have him just look at ds for 30 seconds, say "its a virus" and walk out the door.. When DS was 3 months old he had a horrible productive cough and the local CF doctor just looked at me, shrugged his shoulders and said "they cough" it's what they do and walked out the door. The kid had bronchitis and a simple throat culture as we requested would've shown he was culturing a bug common to NICUs. His cf doctor in the City pretty much yelled at us -- didn't we KNOW ds could've ended right back in the ER!

Had a similar issue with DS' most recent bowel obstruction a few years ago. Finally convinced them to do an ultrasound because they were sure it was just a stomach bug. Trip to the City, surgery and a 3 week stay in the hospital later....

 

[Printer]

Ratatosk:

I understand what you are saying. The PCP that I had at the time of my Dx would not accept that I had a Real Cystic Fibrosis, he would always say that I had Cystic Fibrosis Syndrome. I changed to a new PCP right after that.

My comment was BE CAREFUL, for the reasons stated.

Bill

 

[CFParent2]

Newborn screening

I was surprised that your child did not have a genetic screening test. I guess this varies with each state. Getting a genetic test is not only important for diagnosis, but also for treatment approach. Not all CF genetic mutations are currently treated the same way, as they were a few years ago.

Your experience emphasizes the need for newborn screening. The most painful part as a parent is not knowing enough.

 

[Cricket's mom]

I was surprised that your child did not have a genetic screening test. I guess this varies with each state. Getting a genetic test is not only important for diagnosis, but also for treatment approach. Not all CF genetic mutations are currently treated the same way, as they were a few years ago.

Your experience emphasizes the need for newborn screening. The most painful part as a parent is not knowing enough.

Hi CFParent2

We didn't have the newborn screen because our son was born at home with midwife assistance. They recently took blood for a basic genetic screen. We haven't gotten the results back yet



Small update: We just got the results back for the Chymotrypsin test and he had a normal number of 6.3 u/g. Today was the first day of the collection for the 72 hour fecal fat test. We met with the nutritionist yesterday and she gave us some cans of Pregestimil to replace our regular formula once the fat test collection is finished. It's been a long day. Tomorrow i plan to call and see if the swab results are back yet. Today was also my 6 yr old's conferences so i had an opportunity to let his teacher know all of the stuff that's been going on and all the running around we've been doing because we've noticed our 6 yr old has been acting a bit more stressed lately. I will be so glad when we have some solid answers and can settle into our new routine.

I hope you are all well

 

[Ratatosk]

DS was diagnosed due to digestive issues prior to newborn screening being available in our state.

I may have missed this post, but have they prescribed digestive enzymes -- creon, zenpep, pancreaze.... DS hated pregestimil, so we ended up using regular formula with a higher powder to water ratio (higher calories) based on a recipe from the CF clinic's dietician. DH and I tasted it after ds threw a total fit in the nicu and refused to eat. It tasted like vomit and the way road-kill smells. VILE!!!! We also started regular foods at 6 months or doctored up baby food, as baby food is very low in fat and salt. Hopefully you'll get some answers soon and be able to start a treatment plan for whatever issues your child has.

 

[Cricket's mom]

DS was diagnosed due to digestive issues prior to newborn screening being available in our state.

I may have missed this post, but have they prescribed digestive enzymes -- creon, zenpep, pancreaze.... DS hated pregestimil, so we ended up using regular formula with a higher powder to water ratio (higher calories) based on a recipe from the CF clinic's dietician. DH and I tasted it after ds threw a total fit in the nicu and refused to eat. It tasted like vomit and the way road-kill smells. VILE!!!! We also started regular foods at 6 months or doctored up baby food, as baby food is very low in fat and salt. Hopefully you'll get some answers soon and be able to start a treatment plan for whatever issues your child has.

No Ratatosk. No enzymes yet. I think they want to finish all the testing first since the enzymes would throw them off. Right now, we're on day three of the 72 hour collection for the fecal fat test. Someone needs to come up with a better collection method than saran wrap in a diaper. Seriously.

We can't start the pregestimil until we get the 72 hour collection finished. And she warned us to transition him VERY SLOWLY. I feel so bad because nursing and bottles are one of his very great joys and it makes me sad that it will become a battle instead. She said she wants to do the higher ration WITH the progestimil. I think that is just until we get his weight rebounding a bit. I've been trying, for some time to give him higher calorie foods with simple carbs because i know those are digestible. But my real worry is his little brain and the fat needs there. It's hard to say at this point, because he's been weighed on so many different scales, but he might be increasing his weight at a slightly higher tic than he was before. I think maybe the increased fruit is putting some weight on.

I asked the nutritionist if we should start giving him more foods with higher fat like avocado and she said not until we get him on enzymes. He is beginning to show some bloat and she thinks it's because of the fats he cannot absorb.

 

[Ratatosk]

I'd speak with Rebjane here on this site. She BF her daughter. And breast milk I believe is more easily digestible than regular formula... You may want to start another thread about breast feeding. I never intended to BF ds; however, it was very much encouraged in the NICU when ds was there.

 

[Cricket's mom]

Another update

So Cricket has another respiratory infection. "Wheezy bronchitis" His ped put him on an albuterol inhaler. The dr. suspects this to be a new virus and not related to the one he had last month.

The results of his 72 fecal fat came back normal, which is "very confusing" (using the ped's wording) given the low results on the two elastase tests. He's drinking the progestimil with no fussing but has been vomiting it up, every few feedings, since he began taking it. This morning, my poor boy laying in a puddle of formula vomit. I have a call in to the nutritionist to see what to do about the formula and to get back in to GI, i called the cf center to let them know of this current virus so they can add it to his record there and we scheduled a follow up with ped in 2 weeks. We also had to schedule a ped neuro visit because there is some concern over his skull, a ridge in his forehead and his soft spot being closed.

I want to tell you all i am grateful for this site and the encouragement and the advice. Being able to come here has really helped.

 

[Ratatosk]

Pregistimil is pre-digested formula -- more easily digestible, so I wonder if it could be affecting the fecal fat test results. DS HATED is, so we used regular formula, albeit a higher calorie one. As for the respiratory infection, besides the albuterol, have they had you do any chest physiotherapy (CPT) or Bronchial Drainage (BDs) to get the stuff moved up and out of him. Amazing what just a few sessions of patting them on the back, sides and chest do to clear out those lungs and get rid of that cough will do. Did they RUN a culture to see if he's growing anything -- h. flu, pseudomonas, strep, staph, mrsa, enterbactor choclae, RSV?

 

[Aboveallislove]

Yes, I second what ratatouille said. I'd try to get into cf center for throat culture and respiratory training for percussions. Hang in there momma!

 

[Cricket's mom]

Pregistimil is pre-digested formula -- more easily digestible, so I wonder if it could be affecting the fecal fat test results. DS HATED is, so we used regular formula, albeit a higher calorie one. As for the respiratory infection, besides the albuterol, have they had you do any chest physiotherapy (CPT) or Bronchial Drainage (BDs) to get the stuff moved up and out of him. Amazing what just a few sessions of patting them on the back, sides and chest do to clear out those lungs and get rid of that cough will do. Did they RUN a culture to see if he's growing anything -- h. flu, pseudomonas, strep, staph, mrsa, enterbactor choclae, RSV?

He had RSV last month and the ER ran a culture. The ped didn't run anything like that yesterday. I don't know much about the patting.

We started him on the pregestimil after the test for that reason. The collection for the fat test was very difficult to do. The samples needed to be collected on saran wrap and it just is nearly impossible to get that to stay put unless you hold it in place with the leg bands. When you do that, place it so that it is held by the bands, the saran wrap causes the diaper to leak. I spent three days trying to scrape diaper lining or scraping poop off of his legs, and surrounding environment. When left inside his diaper the saran wrap would simply bunch up and leak everything into the lining of his diaper. It was just a very difficult thing to get done. I am hoping those results were correct and not faulty as a result of the trouble i had collecting.

After your last comment about the taste of the Pregestimil, my husband did taste it and he agreed with you. He said it was the worst tasting stuff. Fortunately, the baby isn't complaining and will usually drink a whole bottle with little complaint. He does vomit a lot of it up though and it is like a mucous when it shows back up. Awful.

How is your little one doing Ratatosk?

 

[Ratatosk]

Yep, tastes like vomit and the way roadkill smells. Wonder if he isn't swallowing a bunch of mucus and that's upsetting his stomach, which in that case, it might be a good idea to contact the CF clinic and ask about cpt. IMO, in any event call the CF clinic and ask to be seen based on the vomiting and virus. Most general practitioners aren't quite as well versed in these sorts of things. Most of the time when DS got a cough, I'd call the cf clinic because if I took him to the local pediatrician he'd tell me it was just a virus or that "they" (people wcf) cough, it's what they do; however, the CF doctor in the City we saw preferred a baseline of NO cough.

Our son had a very rough start and spent his first 6-7 weeks in the NICU -- meconium ileus, bronchitis... But during that time we were able to learn from the RTs about cpt and nebulizers. Got the enzymes figured out for the most part. First year was a little scary with all the unknowns, but he actually thrived. Had a couple ear and sinus infections and we were forever trying to figure out his enzymes based on diaper output. But once we began treatment, saw some decent doctors and got into a routine... Things were fine. Today he goes to elementary school. We do vest treatments, he takes enzymes. He's had a few colds and stomach bugs over the years, but other than an obstruction a few years ago, we've been quite fortunate.

 

[ymikhale]

In response to the elastase number question, numbers above 200 are considered normal and usually do not require replacement enzymes. Since yours are below, it is likely he is pancreatic insufficient. Not to confuse you or anything, but there are cases when babies with CF (especially those whose mutations are caracterised by pancreatic sufficiency) start off as pancreatic unsifficient, are given enzymes,the enzymes "jump start" their pancréas and they become pancreatic sufficient. My dd was in that case: she had originally elastase of around 150, was put on Creon, and a year later her elastase was above 400 (and remained there since then).

 

[Nikole]

Yep, tastes like vomit and the way roadkill smells. Wonder if he isn't swallowing a bunch of mucus and that's upsetting his stomach, which in that case, it might be a good idea to contact the CF clinic and ask about cpt. IMO, in any event call the CF clinic and ask to be seen based on the vomiting and virus. Most general practitioners aren't quite as well versed in these sorts of things. Most of the time when DS got a cough, I'd call the cf clinic because if I took him to the local pediatrician he'd tell me it was just a virus or that "they" (people wcf) cough, it's what they do; however, the CF doctor in the City we saw preferred a baseline of NO cough.

Our son had a very rough start and spent his first 6-7 weeks in the NICU -- meconium ileus, bronchitis... But during that time we were able to learn from the RTs about cpt and nebulizers. Got the enzymes figured out for the most part. First year was a little scary with all the unknowns, but he actually thrived. Had a couple ear and sinus infections and we were forever trying to figure out his enzymes based on diaper output. But once we began treatment, saw some decent doctors and got into a routine... Things were fine. Today he goes to elementary school. We do vest treatments, he takes enzymes. He's had a few colds and stomach bugs over the years, but other than an obstruction a few years ago, we've been quite fortunate.

.


My son had to have sugar in his Pregestimil or he wouldn't eat it. He still had some vomiting on it as well, and had a huge tummy. He started Elecare....super expensive, but SAVED HIS LIFE. He only had to stay on it three months. Moved to Pediasure, and doing well. Is your kiddo on reflux medicine? It does help with the excessive vomiting of mucus. Both of my CFers went through that but grew out of it. They are on Omeprazole.