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, September 23, 2014

Non- Fat Pumpkin Muffins


        We are getting close to that time of year again when pumpkins take over our world and our dishes!  Pumpkin is a beautiful thing!  I think many of us have some fond memory of pumpkin, perhaps related to Thanksgiving, but when you are trying to cook foods very low in fat pumpkin becomes a beautiful staple that adds moisture and flavor!
        A  couple quick tips that I have learned cooking low fat.  First, it is best, whenever you can, to use fresh herbs and spices.  This gives your dish a burst of flavor that you won't get from certain dried and pre-ground stuff.  In this particular recipe if you have a couple extra minutes and a mortar and pestle, grind your own dried cloves!  You will be amazed at the difference and burst of flavor.  Second, don't be afraid to reduce and layer sugar! By layering sugar I mean try to use sugar in the raw, brown sugar, white sugar and a dash of honey- or some combination.  Yes, it takes a couple extra minutes, BUT I think when you are working with less fat layering sugar adds complexity back to the taste of your dish! 

       Last comment:  I have the muffins listed as non-fat, but as you know from earlier posts fat is in everything and nothing can be completely fat free.  So based on labels and the website: nutritiondata.self.com I estimate that there is a total of 3.5 grams of fat in the entire recipe or less than .3 grams of fat a muffin.  (Can you believe that a cup of pumpkin has 1 gram of fat?  Who knew?)  Below is one of the first recipes I created for Christopher to enjoy...but I will be honest...I end up eating half the muffins myself!  Enjoy!
 
Non-Fat Pumpkin Muffins 

Ingredients:
·      2 c. flour (1 cup white; 1 cup whole wheat pastry flour)
·      1/2 c. sugar (1/4 cup sugar in the raw; 1/4 cup brown and white mixed)
·      3 tsp. baking powder
·      1 tsp. baking soda
·      1 tsp. salt
·      1 tsp. cinnamon
·      1/2 tsp. ground cloves
·      1 c. canned pumpkin
·      1/2 c. skim milk
·      2 egg whites
·      1/2 c. mashed bananas
·      1/2 c. raisins

Directions:
1.     Preheat oven to 350°F
2.    Sift dry ingredients into large bowl.
3.     In a medium bowl stir pumpkin, milk, egg whites and mashed bananas until well blended.
4.    Add wet ingredients all at once.  Stir slowly, until dry ingredients are dampened. Do not over stir.
5.    Stir raisins in with only a few strokes.
6.    Prepare muffin tin with cooking spray.  If using muffins cups, spray these with non-stick spray as well, as these muffins can stick to the cups.
7.    Fill cups 2/3 full. Bake 20-25 minutes. Makes 12-14

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