A time for change. 

A little over a year ago, we switched Corrigan’s protein source to something 100% plant-based and organic. Highly recommended and carefully reviewed by his team, I was so thrilled to change him over from the canned “nutrition” that had very little “food” and way too many corn syrups and corn solids. Over the last twelve months, his labs have been okay but not perfect, however he was meeting growth expectations so we stayed the course. 

Last March, Corrigan caught a Scarlet-fever-like virus that landed him in the ER. His ammonia wasn’t “awful” at 105 but rescue protocol was implemented just the same. However, our local hospital was full, as was John’s Hopkins, and after spending a miserable 21 hours in the ER, we were sent home. (His ammonia was under 20 after just a short bolus of rescue meds, so no concerns there) 

Several weeks after the virus left, all of the skin began peeling on his hands and feet. Not just a little bit either, but whole sheets would peel and then peel again days later. It went on for well over a week. 

At the end of March, he had a Hopkins appt and his team was pleased that despite his illness, and the energy required by the body to rapidly grow all that skin, he had grown almost two times the expected amount. Our boy was making magic happen on a very limited protein diet. 

The reality of the situation appeared after his labs returned a few days later. They were bottomed out. Low low low. His branch chains were lower than I’ve ever seen them. BCAA’s are isoleucine, leucine and valine and are three of the nine amino acids that the human body does not make on its own and needs to receive from foods. While most amino acids are broken down in your liver, BCAAs are broken down primarily in your muscle. As such, they’re believed to help improve exercise performance as well as reduce the breakdown of muscle.

For Corrigan, and his disorder, we carefully watch his branch chains. We worry about catabolism when they’re low or below the low end of “normal” and move quickly to affect those numbers when we see them depleting. 

Catabolism is the breakdown of muscle. When his BCAA’s are “comfortable” we breathe easier knowing that if and when his body decides to grow, his system is ready for the request. If his body is tasked with growing, he has to be stable. The body will take what it needs, whether it’s the perfect time or not, and if he’s not in a good place and his body takes it from his muscles instead, that breakdown can cause massive instability and lead to a metabolic crisis. 

So Corrigan’s labs were awful due to all of the growing he was doing while also growing skin. We added a gram of protein more to his diet, at his team’s order of course, and retested a week later. They barely moved. Increased some more. Tested a week later. Barely moved. This went on for eight weeks and I was getting more and more nervous with each increase. 

I attended a UCD workshop in SC in April and was shocked to hear the protein amounts of other UCD kids, Corrigan was on SO much more than others, even more than an adult patient with the same disorder.  He had now had more protein increased in two months then he last two YEARS combined. 

During this same time period, Corrigan’s behavior at school was becoming more than alarming. While he always has a short-fuse, the reports coming home placed the issue in the “crisis” category. After one particularly awful report of self-violence and destruction of property, leading to children being removed from class until he calmed, I took him straight to the ER for an ammonia check. 

It was lower than usual. Not elevated at ALL. 

We crossed off ammonia as a contributor to his issues and I started making calls to behavior therapists and reaching out to friends for recommendations. 

We repeated some labs a few days later and were shocked that his BCAA’s were now trending DOWN with no indications of growth spurt (we can tell by other labs too) and suddenly things blew up. Review of the last few months startled the doctors, he was on SO much protein, actually more than the federal guidelines for a healthy 9-13 year old male, let alone a metabolic one, they could NOT, in good conscience, increase another bit. 

But why was this happening and what do we DO? If we don’t get those levels up, we are setting him up for a dangerous crisis if he grew or used too much energy swimming or got sick with a virus. 

Some super smarties put their heads together and reviewed the last year. They suspected his protein source. I was devastated that we might have been giving him something not “complete” enough for his situation and also heartbroken to possibly have to cease using something that is so full of amazing, natural ingredients.  

The team decided to do a five day experiment.  We would IMMEDIATELY stop using his protein source (remember he is tube-fed) and use another source that they had full confidence in and a complete FDA verified amino-acid profile on (Lactaid) AND we dropped his protein total for the day 30%!  We were to do this for five days and if labs showed his BCAA’s were still trending lower, then we had an issue on our hands not related to his source. If the BCAA’s went UP, on LESS protein, then that would show what we were using before wasn’t “nutritionally complete” no matter what the packaging said. 

A week later we had our answer. His BCAA’s not only increased but one value increased 40%!!! On LESS protein. I keep saying that, 30% less, but that’s crazy talk. 

OH, did I mention that after ONE day of the experiment, the real Corrigan returned? Instead of angry and belligerent and cussing and upset, our sweet, silly, unique little guy was back. ONE DAY. This means that while his ammonia was stable (praise Ravicti forever and ever, amen!) the metabolites of the cycle process were possibly an issue due to the amount of protein his poor body was trying to fight through. Some doctors believe that the metabolites may cause agitation to the surface of the brain, causing all sorts of cognitive and behavioral concerns. Others do not believe that the metabolites cause these issues at all. Anecdotally, we can tell you that he had a miserable few weeks and was nearly instantly relieved when his protein load was reduced.  This issue of metabolites has divided many in our community and those that believe that even with controlled ammonia, metabolites cause cumulative brain issues, will  therefore choose transplant since these metabolites do not have treatment to control.  (Research is on-going. )

Repeat labs again last week show that on the Lactaid, his aminos are still moving in the right direction at a lovely pace. 

He can’t live on Lactaid forever though, and I will fight hard to prevent a return to Pediasure as a source, but our options are limited. We will decide in a few weeks after we get, and keep, him stable on his curent recipe. 

This goes to show how careful we have to be with every bit of nutrition that goes into his body, and while I’d love to try my own blenderized diet, it would likely mean bi-weekly pokes for blood labs to make sure I’m getting optimal nutrition into him. And honestly, these last eight weeks have caused Corrigan a tremendous amount of stress at the repeated trips to LabCorp. I’m going to have to use what Hopkins KNOWS is perfect. There’s just no room for “winging it” in his care, and I can’t put him through the stress. 

Right now, he’s wonderful. He’s swimming daily with full energy. He’s sleeping great, His attitude is back to his baseline and he’s enjoying his summer break. I’m thankful for a Team that experiments to find answers but I’m also so thankful for a team that said YES to trying that protein-source in the first place. Yes, our experiment failed, but for a year he took in the most amazing food with this product and I’m thankful that they didn’t shut me down instantly, as I see so many folks complaining about in the belnderized diet FB groups. Our team said, sure…he’s stable, let’s do it. And we did until we couldn’t any longer. I consider it “worth it.”

***I didn’t mention the protein-source in this post. I want you to know I still whole-heartedly believe in this tube-Feeding product. It’s likely totally safe for 90% of the people that use it, my boy and his disorder just require a level of precision that this cannot provide him. His body appears to work best on animal proteins, not plant-based so we do what his body’s shows us is best.   It’s a tightrope. 

4 thoughts on “A time for change. 

  1. Thank you so much for posting this!! We have been having a rough time trying to get our daughter’s diet right for months. For awhile we thought we were doing something wrong, but it’s nice to know that it’s a trial an error thing to get the right balance for each of these special complicated kids!! You give me hope that it can be done!! And what it’s a sick day feed like for your son? We’ve had many of those lately and our daughter is chunking up nicely!!


    • It can be done! With close monitoring, you’ll get there! Sick day is always ceasing protein and then solcarb for massive calories along with his metabolic powder and usually pedialyte. It’s can def cause some addition to weight but our kids need all of those calories when unwell to remain stable! Thanks so much for commenting here. I haven’t written in awhile, I’m glad I’m not just talking to the void 😜


  2. I wrote a bunch stuff in reply but it all got erased. But, the short version is I really enjoyed reading about this. I have seen some behavior and learning regression and poor growth since weaning Abbey from an elemental formula to probably the same organic vegan formula. I gave it a year and now I am adding animal protein and fats back and I am finally seeing growth again. Different metabolic diseases but similar findings. Thanks for posting about it! It’s a confirmation to me that I am not crazy about my theory.


    • I wonder if it’s the same product? If so, I do believe we owe it to the tube feeding community to let hem know that what they’re saying is “nutritionally complete” perhaps isn’t? Especially since we are talking about two different (but similar) disorders, you and I. Our dietitian said “never again” will she use this product with her patient’s and I think they should know why?


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