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

Did you know fat chains have different sizes? Who knew?!


There were a lot of things I didn't know or understand about the body before I had a child with LCHADD. In college I took environmental science classes and so even basic anatomy was a little out of my league when I first started trying to understand what was really going on inside of Christopher's body. One of the many fascinating things that I learned was that fat exists in various sizes. Then when you consume fat your body begins to break it down into smaller and smaller forms of fat, to use for energy. I quickly learned that there are very long chain fats, long chain fats, medium chain fats and small chain fats. The length depends on the number of carbons that are connected together. And how your body processes these fat chains depends on the length.

As I was studying about fat and fat metabolism, I began contemplating how I would explain this all to Christopher. All of the drawings that I saw of fat molecules looked way too complicated ...and so the more I read about fat, the more I began visualizing them as caterpillars. The above picture is an excerpt from the children's book I wrote about LCHAD, VLCAD and TFP. I wanted to share it with you so you could visualize more clearly that fats have different sizes.Most of the foods we eat are primarily comprised of long-chain fats (the ones that Christopher has a hard time breaking down) but there are short and medium thrown into some of your fruits and vegetables too! Coconut is a little different. Over 90% of coconut's fat is medium chain. This is actually what Christopher's special MCT oil comes from! Although MCT oil has a low smoking point so we don't sautee with it; we can sprinkle it over food, make special butter with it (we made sugar cookies with this butter last Christmas!), and even make a "Christopher-Friendly Pesto" for pasta. This oil is really a life source for Christopher because it gives him a longer form of energy than just glucose and protein to use. We give him this oil and his special medical formula throughout the day to try to give him constant energy. If we know he is going to the playground we give him the oil in food right before we go and follow up with coconut water or Gatorade (which is another great energy source for LCHADDers)
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