On August 11, 2008 we gave birth to a beautiful baby boy, Christopher Harry! Christopher was one of the first babies in GA to be picked up on the newborn screening with a rare inherited Fatty Oxidation Disorder called LCHADD (Long Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency). As Ryan and I (Stephanie) gained more understanding about our son's condition and began to watch him grow and thrive, we had a desire to share our story and raise money for research. We hope that by sharing our story we can raise awareness/encourage education about LCHADD and provide hope to other families whose child(ren) have this condition.


This blog shares our journey, hopes, and fears. We also want this blog to contain practical information! Entries will include: yummy recipes, how we manage his LCHADD, conversations about medical issues, educational tools, and useful links on the side of the blog! If you are ever curious about something I have shared feel free to contact me personally!

What is an FOD?

WHAT IS AN FOD?


FOD stands for Fatty Oxidation Disorder. For children and adults with FODs their bodies have difficulty breaking down (or oxidizing) fat to use it for energy. This occurs when an enzyme is missing or not working properly. There are several different kinds of FODs. Some examples are SCAD, MCAD, VLCAD and LCHAD deficiency. Fatty Oxidation Disorders are genetic, which means both parents must have the recessive gene in order for their child to end up with the disorder. It also means that children with FODs will not grow out of their condition. Their condition is just as much a part of who they are as the color of their eyes or hair.

What is LCHADD?

WHAT IS LCHADD?


LCHADD stands for Long Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency. Essentially children with this condition cannot utilize long-chain fats for energy. Did you know that the food that we eat has different size fat molecules in it? I never did until Christopher was born! There are short-chain fats, medium-chain fats, long-chain fats and very-long chain fats. I picture them as different size caterpillars (and this is how I explain it to Christopher) running around in our food. The short, medium, and very-long chain fats children with LCHAD can process. Unfortunately, most of our food and oils are primarily long-chain fats (the ones Christopher can’t process). If Christopher eats too much fat (right now he can only have 8-9grms of fat a day through food) then the fat will gather in his liver, kidneys and around his heart.


The other issue with Christopher not being able to breakdown long-chain fat is that it is a great energy source. For most of us, when we exercise or get sick and we burn through all of our glucose stores we start using fat for energy. Christopher’s body cannot do this, so his body starts to break down muscle and use it for energy instead. To say that this is “not good” is to speak lightly. When your body starts breaking down muscle you run the risk of having problems with lots of different systems in your body. Doctors worry the most about your kidneys because kidney failure can happen when your body is trying to process all of the broken down muscle (or myoglobin which is the by-product of the broken-down muscle).


In general, children with LCHADD are put on a very low-fat diet, drink a special medical formula, and most often use MCT oil (a unique oil comprised of medium chain fats) to give them a source of sustaining energy. Christopher's special medical formula, Lipistart, helps to ensure that he gets enough essential fat for brain and eye development without getting too much fat that his body can’t process. Lipistart also helps to provide a consistent form of energy for him throughout the day.



Wednesday, March 21, 2012

"Why can't I eat that?!"

So far this is not a question that has come up too often. As Christopher grows and he is exposed to more children, parties, and events where there are lots of food that he cannot eat...we are preparing ourselves for this question. Right now Christopher more often asks, "Is that Christopher-Friendly?" And seems satisfied with the answer, "No...because it has too much fat." I think it has also been really helpful for him to see that different kids and adults are at times limited in what they can eat. His cousin and another close friend are both severely allergic to peanuts...and mommy can't eat strawberries...and someone else can't have gluten. The other day we were eating a cracker and he asked me, "Mommy is this Kristina-friendly?" To which I said, "No sweetie." "But why?" "Her body does not like gluten very much." In that moment, I found myself grateful that all of our bodies are a little different...so he doesn't have to feel like his is s-o-o different.

Although it would be nice if these small conversations would be enough, I realize it will not always be this easy. And truthfully there is definitely part of me that has had to grieve the foods I wish he could just try (flavorful cheeses...rich chocolate brownies...or just plan old chocolate!) or the fact that he will never be able to go into a restaurant and get just "whatever" he wants off the menu. I worry about how we will encourage him through peer pressure and help him process some of his differences, and yet I know we will face these challenges head on as they come. Currently, two main tips have challenged me as a parent.

First Tip: "Don't feel sorry for him." I can feel sad that he can not try some yummy brownies, but the fact of the matter is, there are a lot of other yummy foods he can have! A dear friend reminded me that I need to sort through my own grief about his food so he can better develop his own perspective. She said, "Honestly, he may not be the type to care. And if he isn't and you teach him to feel 'sad' about his condition or his 'limitations' you will make it harder on him. Let him be sad or frustrated if at some point he feels that way...but be okay if this is not the emotion that he puts with diet." And so I find that I try to be careful when we mention that he cannot have certain foods. I try to make sure my tone is nonchalant instead of pity-filled. I think it also helps that we try to keep mainly Christopher-Friendly foods around the house so there are not lots of food in his daily reach that he can't have...home becomes a safe food place for him.

Second Tip: "When he gets to school try to make his lunches look like the other kid's lunches as much as possible...even if they are not." It hadn't crossed my mind, but makes so much sense. I mean there will be some things we just can't adapt. Then there will be the apple, raisins, box drink , pretzels that are in many children's lunches. Do you know how excited I was when I found fat free cheese and a special peanut butter called PB2? Yes, I realize that grilled cheese sandwiches, pizza and peanut butter and jelly are not the most nutritious choices...but to know that we can make an equivalent made me so excited and hopeful for some of his future social interactions around food. (PB2 has been wonderful! Who knew that they made powdered peanut butter where they extract the fat out...so it has only a tiny amount of fat in it! And the company has been amazing in trying to understand Christopher's disorder too!) I just feel like as he grows he will have to spend quite a bit of time explaining his diet and disorder. I hope that days with his special PB2 sandwhich, fruit and yogurt will not have to be one of those days!
"Why can't I eat that?!" And so we move forward and answer his question by looking on the back of packages when they are available. We show him the number next to the word fat and explain that there are too many of those fat "catipillars" running around in this particular food item. He responds, "Oh..." and takes a nice big bite out of the juicy strawberry he has in his hand.
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