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.



Tuesday, January 23, 2018

Will He Get Tired of Cooking?

      “Will he get tired of cooking…tired of eating…as he gets older?  How will this affect him?  Will this lead to him being off diet?”   This thought, or worry, goes through my head on occasion.  Typically it is after a long day when I myself am exhausted and the last thing I want to do is cook.  On those days I think about Christopher.  What will it be like for him when he is 20 and comes back from a long day of classes and doesn’t feel like getting something to eat?  How will we teach him to push through in those moments and do the right thing?  How do you prevent burnout from cooking?
            I have brought Christopher into the kitchen with me from a very young age, trying to encourage a love of food and “the process” that is cooking.  He used to love being in the kitchen flipping pancakes with me, but on an average night, after a long day of school he is much more interested in playing; which I totally understand!  Yet, I still try and create these moments, where I can teach him skills.  And for the now, this is enough.
            I am fully aware though, of how much time food prep takes for my little guy.  I easily spend three hours a day cooking and preparing food…And that doesn’t include dishes (thanks Ryan!).  So how will he carve out that time into his own schedule as he gets older?  How do I support him as a young adult?
Trying to make food fun for football game!
I am hoping to glean tips along the way from older parents, and the FOD Conferences.  One mom shared that she makes a couple of meals that her son can freeze and take with him to college.  Another mom and her daughter worked hard with the college cafeteria to make sure there was always food available for her.  I tuck these ideas in the back of my brain.  But what else?

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