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, May 21, 2014

Pancake Day!!!!

Are you getting hungry yet?
 Twice a month Christopher and I do what we call, “Pancake Day.”  I can hear his screams of excitement even now as I write this post!  Since before Christopher turned two he has loved pancakes; by age four I could genuinely call it an obsession.  Pancakes were an easy thing to add to his diet when he was small because we could make them virtually fat free using tapioca and white flour.  This was a necessity when he could only consume 3 grams of fat a day!  Now that he can have between 8-9grams of fat a day, we are able to use more complex grains (i.e. whole wheat flour, brown rice flour, fava bean flour, coconut flour).
 Pancake day started as a way for me to encourage Christopher’s excitement around cooking and a way to save time.   Basically we use a large flat electric griddle, make a big batch of pancakes, and then consume half of the batch of pancakes the first week and freeze the rest for the following week.  Like any routine this has metamorphosed, and I hope that we are able to continue this tradition for another couple years.  Not only do we make large amounts of pancakes now, I have been able to throw in math, writing…and even a little geography.
            Math:  When Christopher was really small I had him simply count the pancakes with me.  Then we started doing simple addition by adding the pancakes on the plate with the pancakes on the griddle.  Recently, after he learned how to read “total fat” on labels he started to tally fat grams from the blueberries and various grains that we use to cook with.  Pancakes are also a great way to introduce measuring and sifting because, lets be honest, pancakes are one of the most forgiving recipes.  Nothing has to be perfect and they still turn out yummy!!!!
            Geography:  Christopher went through a phase where he was really into maps and states.  One day he asked me to make “states” pancakes.  He would chose a state from his United States Map and either say it or ask me what it was.  Then I would do my best to make a blob that mimicked the state…and he would use his vivid imagination and say, “Great job mommy that looks just like Florida!”
            We always finish our pancake adventure with a movie or a Scooby doo episode!  Perhaps these days he is enjoying the grand finale of television the most, but I still enjoy the process and enjoy all the things he gets to learn along the way.
            I am including two pancake recipes that we use off and on.  One made with wheat flour and one that is gluten free (He has a good friend who can’t have gluten so this has been great for the both of them!)  Honestly we love getting creative with the flours and often do some kind of combination…and include coconut flour too for fun (DON’T USE COCONUT FLOUR IF YOUR CHILD HAS MCAD!).   We have tried fava bean flour and garbanzo bean flour to increase the protein, but beware that these have a little more fat and taste a little more harsh. 
            Enjoy the pancake recipes below!  And don’t forget to make something silly with your pancakes!  That’s for your sake as much as your kiddo!  We gotta keep smiling!

Extra- Protein Yum-Yum Pancakes

Ingredients:
·      1/2 cup 1% fat whipped cottage cheese (adds 1grm fat)
·      1 large banana
·      1/2 cup blueberries or diced strawberries
·      1/4 cup non-fat milk
·      2 egg whites
·      1/2 cup whole wheat flour
·      1/4 cup whole wheat pastry flour
·      1/2 cup white flour
·      1 tbl baking powder
·      1/2 cup- 3/4 cup non-fat milk depending on desired consistency

Directions:
1.     In small food processor or blender blend cottage cheese, banana, 1/4 cup non-fat milk and two egg whites.  (If a processor or blender is unavailable, mash the banana and then whisk these ingredients together.)
2.    Sift flour and baking powder in a large bowl or Pyrex cup.
3.    Add liquid from the food processor and stir.
4.    Gradually add the second amount of milk (1/2-3/4 cup) until batter is desired consistency for pancakes.
5.    Add blueberries or strawberries if desired and stir gently
6.    Pour pancakes onto non-stick pan with or without non-stick spray.
7.    Flip and Viola!

Tips:
* My brother got me an electric griddle for Christmas one year!  It is more than worth the investment!  I never have to spray the griddle when making pancakes, flipping is so easy and the amount of time I save by cooking 6-8 pancakes at once is nice!  Especially since my little guy LOVES pancakes!

*You can substitute cottage cheese for non-fat greek yogurt and this will decrease fat of overall recipe.

makes 11 Large pancakes; Batch has approx 51grms protein and approx 3grms fat.  1 pancake has approx= 4.6 grms protein and .27grm fat


Gluten-Free Low-fat Pancakes

Ingredients
·      1/2 cup tapioca flour
·      1 cup rice flour
·      2 tsp baking powder
·      1 tsp baking soda
·      1/2 cup mashed banana (approx. 1 banana)
·      1/2 cup blueberries
·      1 cup non-fat milk
·      2 egg whites

Directions:
1.     In small food processor or blender blend banana.  Add 1/2 cup non-fat milk and two egg whites and blend until slightly frothy.  (If a processor or blender is unavailable, mash the banana and then whisk these ingredients together.)
2.    Sift tapioca flour, rice flour, baking powder and baking soda in a medium bowl or large Pyrex cup.
3.    Add liquid from the food processor and stir. (Do not over-stir!)
4.    Gradually add the rest of the milk until batter is desired consistency for pancakes. (You may not use all of the milk depending on your climate’s humidity so go slowly!)
5.    Add blueberries and stir gently with spoon
6.    Pour pancakes onto non-stick pan.
7.    Flip and Viola!


*The total recipe has approximately 4 grams of fat primarily coming from the brown rice flour.