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, November 28, 2017

It's Waffle Time!

  There are these moments, as your kids get older, that feel so surreal.  I appreciate these moments because I am able to stop, quiet my mind, truly enjoy the moment, and bask in what that moment “is”.  I find myself overcome with gratitude  when I watch Christopher take initiative with practicing his piano, when he still begs for a story time, or when he asks to do something new…and it clicks, “Wow, he is old enough to try this!”



  A couple of weeks ago, Christopher asked to make waffles by himself.  This may not seem like a big deal, but all store bought waffles have too much fat for him.  So when we make waffles, we do it from scratch.  I hesitated, then realize that we were really here.  We were really at a place where he could explore doing this and I could sit and read a book close-by in case he had questions, or I noticed something unsafe (like the waffle iron popping out of our old 1970’s waffle maker).
  He did such an amazing job!  And I will never forget this moment.  Here is the recipe that we use to make waffles.  They aren’t quite as crunchy as I’d like them, so sometimes I will put them in the toaster after we take them out of the waffle iron.  I was told that homemade waffles just aren’t crunchy the first go around…but I suppose it could be my iron too.


 
Buttermilk Waffles

Ingredients:
·      1 cup unbleached all purpose flour
·      1 cup whole-wheat flour
·      1 tbls brown sugar
·      1/2 tsp salt
·      1tsp baking soda
·      1/2 tsp ground cinnamon
·      1/4 tsp salt
·      2 cups non-buttermilk
·      2 eggs, separated (One egg yolk you will keep, another you will discard.)
·      2 tsp MCT oil
·      2 tbls Banana
·      2 tbls non-fat greek or plain yogurt
·      1/4 tsp vanilla extract
·      1/4 tsp almond extract
·      Non-stick spray for grill

Directions:
1.     Turn waffle iron on to pre-heat.
2.    Make non-fat buttermilk by juicing 1 lemon into a 2 cup Pyrex measuring cup.  Add enough non-fat milk to make 2 cups.  Set aside for 5minutes and your milk will turn into non-fat buttermilk.
3.    In a large mixing bowl mix flour, brown sugar, baking soda, cinnamon, and salt.
4.    In preparation for the eggs get a small bowl and medium size bowl and place on counter.  Crack first egg and put egg white in medium bowl and egg yolk in small bowl.  Crack second egg and put egg white in medium bowl and throw the remaining yolk in the trashcan.
5.    In small bowl with the egg yolk mix MCT oil, banana, yogurt, and extract until fully combined.  Set aside.
6.    With electric mixer beat egg whites until stiff peeks form. Set aside.
7.    Mix non-fat buttermilk into dry ingredients with a wooden spoon, quickly, until almost blended
8.    Add small bowl of wet ingredients, until combined.
9.    Gently fold in egg whites.
10.  Spray top and bottom of waffle iron with non-stick spray.  Then spoon out batter in the middle of the iron until it ALMOST covers the edges. (If you go all of the way to the edges the batter will spill over the waffle iron and it will be a mess!)
11.   Close waffle iron and wait approximately 2-3 minutes for waffle to cook before you remove


*7 grams of fat for the entire recipe.  Makes approximately 17 waffles.  Each waffle contains about 0.3 grams of fat.