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.



Saturday, March 20, 2021

Pizza Night!!!

      Friday night "Pizza Night" started before the pandemic and has created a positive "comfort" amidst all that has changed over the last year.  It's funny because on our new YouTube channel (https://www.youtube.com/channel/UCaF1wRA8FXIF-3O1CE5RfyQ/videos) Christopher says, "We have been doing movie night since I was 3 or 4," and although we haven't been going quite that long, it shows how much of a tradition this has become for our family!  Below is our pizza recipe!  Feel free to print this off and enjoy!  If you want to make your pizza with Christopher and I, click the above link!  I also provide helpful tips to making the dough, flours to use, how to get non-fat mozzarella cheese melting, and fat considerations if making pizza is a new adventure for your family!  Use all your favorite pizza toppings on this dough! 


What I enjoy is that we can each adjust our pizzas to our own liking, and most importantly we can keep Christopher's pizza extra low-fat using Non-Fat Mozzarella Cheese.  When Christopher was younger his buddies loved making pizza at our house!  Hope this will provide your family as much fun as it has for ours!

Pizza Dough

 

Ingredients:

·      3/4 cup warm water

·      7grams sachet dried yeast

·      1/2 teaspoon salt

·      1/2 teaspoon sugar

·      1 1/4 cups white flour (1.5 LCT)

·      1 1/4 cups whole wheat flour (3.75 LCT)

 

Directions:

1.     Warm water to approximately 100°F- 110°F.

2.    Add yeast, salt, and sugar to water.  Stir and cover with plastic.  Set mixture in a warm place until foamy (approximately 5-10mins)

3.    Sift flour into a large mixing bowl.

4.    Stir in yeast mixture and mix to form a dough ball. (Depending on the moisture in the air you may need a little more warm water if the dough feels too dry or a smidge more flour if the dough feels too wet.)

5.    Knead on a lightly floured surface for 10 minutes, or until dough is smooth and elastic.

6.    Separate into four balls for personal size pizzas or two balls for large pizzas. Let sit 10 minutes lightly covered.

7.    Roll each ball out with a rolling pin to an 8inch diameter circle, adding flour to rolling pin and pizza dough when it begins to stick.  Flip dough often, as you are rolling.

8.    Place the rolled out pizza dough on a stone pan or a cookie sheet lined with parchment paper.

9.    Roll and pinch outer edges of dough to make crust.

10.  To make pizza just add pizza sauce and desired toppings and bake at 400°F for approximately 10-15 minutes on stone pan or 375°F on parchment paper on a regular cookie sheet for 15 minutes. 

 

Tips:

*You do not cook the pizza dough before you add toppings! 

*Look for fat free pizza sauce (or we use Trader Joe’s fat-free spaghetti sauce) and non-fat cheese for toppings. Hormel carries a very low-fat Ham we enjoy putting on top!

*Pampered chef stones are fun to use and create crunchier crusts without burning!

*I also really enjoy substituting the above flours with bread flour! It makes dough more substantial!

*For a nice variation, use the pizza dough recipe to create a Stromboli!   Roll out the dough as you normally would for pizza.  Then put fat-free ricotta, broccoli, ham and fat-free mozzarella cheese all on half of the dough.  Lift up the dough side that is still empty and cover the dough with all of the toppings.  Press down the edges of the dough with your fingers and with a fork to create a seal.  Poke holes at the top of the Stromboli to allow steam out when it is cooked.   Place in oven at 400°F for 10 minutes. Put spaghetti sauce in small bowl for dipping.

*If you use this recipe to make two large pizzas each crust has 2.6g LCT total.

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