The Harry Family Blog: October 2015

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.

Thursday, October 15, 2015

Tapioca Enters Our Life

I think Christopher was about two years old when it hit me that he had endured oatmeal before bed every night for at least six months. We went in for his bi-yearly metabolic appointment, where I asked the dietitian for other complex carb ideas for night-time snacks. She suggested potatoes, rice and "of course" tapioca.
Tapioca? At the age of thirty I had heard of tapioca but never tried it. All of my knowledge of this substance came from the movie "Benny and Joon." Joon and Sam go to their local diner for a late night snack and Joon orders this white creamy pudding. Before eating it she takes out the "shriveled grapes" and has a long conversation about why raisins are disturbing...the thought still makes me giggle! I wrote down all of the dietitian's carb ideas and went home to see if I could find a low-fat/fat-free recipe for tapioca.

   Okay, so for any of you that are totally lost right now and don't understand why in the world I am carb-crazy at night with Christopher, let me explain. Many dietitians encourage their LCHADD patients to have a complex carbohydrate before bed when they are starting to go longer than 3-4 hours at night without food. Christopher started this around age one and a half. By age three we were encouraged to give Christopher a high carbohydrate snack and his Lipistart with raw cornstarch before he went to bed. The idea is that the extra carbs will store in the liver and slowly release throughout the night to provide him energy, thus preventing fatty acid oxidation. At age 7 we have switched things up a bit, with the same basic principle. We give him a high complex carbohydrate snack before bed and then 3 hours into his sleep give him his Lipistart with cornstarch. This is so that when he is healthy he is not going longer than 8 hours without food. Back to my story...
    So I went on-line in search of a recipe...and to my surprise tapioca is typically made with yolks, and often consists of half and half or whole milk! It is one thing to substitute an egg white here or there to reduce fat in recipes, but when I started thinking about how to make tapioca "Christopher Friendly" I felt like it would be equivalent to turning chocolate fudge or butter into something fat- free!
     Then I read somewhere about someone using coconut milk instead of real milk in their tapioca. I knew that using full fat coconut milk was not an option, nor using only coconut milk because the Tapioca would still be too high in fat, but I thought some substitution might bring back the creaminess and the flavor. All of this went into producing the recipe below!
   A couple clarifications: First, if you have MCADD, DO NOT USE THIS RECIPE! Coconut milk is comprised of 60-80% medium chain fats which your body cannot process well. For the LCHADD, VLCADD or TFP child this recipe should be perfect! Check with your dietitian, about how they would like you to calculate fat grams for this recipe. Our dietitian felt that there was such a little amount of long chain fats in a serving that we did not need to count it toward his daily allotted amount of long-chain fats. That being said, if you use Trader Joe's light coconut milk with this reciepe at 8grms of fat per 2/3 cup, you are at about 3.2 grms of fat for the entire recipe or 0.6 grams per 3/4 cup of Tapioca. Again, talk with your dietitian about how they would like you to calculate fat. And don't shy away from having a bowl yourself! I love this stuff, even with the "shriveled grapes"!!!

Tapioca

Ingredients:

2 1/3 cups Fat Free milk
2/3 cup low fat coconut milk (or “Light” coconut milk)
1/2 cup pearled tapioca (not quick cooking)
1/4 cup sugar in the raw
1/4 teaspoon sea salt
3 egg whites
1 teaspoon vanilla extract or 1/2 tsp almond extract
1/4 cup raisins

Directions:

Soak ½ cup tapioca in 2 cups of water overnight or for at least 5 hours. Drain off excess water and continue with recipe.
Stir together milk, coconut milk, tapioca, sugar, and salt in a medium saucepan. Bring the mixture to a boil over medium heat, stirring constantly. Reduce heat to low. Cook and stir 5 minutes longer.
Whisk 1 cup of the hot milk mixture into the beaten egg whites, 2 tablespoons at a time until incorporated. Stir the egg mixture back into the tapioca until well mixed. Bring the pudding to a gentle simmer over medium-low heat; cook and stir 2 minutes longer until the pudding becomes thick enough to evenly coat the back of a metal spoon.
Remove from heat and stir in vanilla/almond extract and raisins. Pudding can be served hot or chilled.

Makes Approximately five 3/4 cup servings.

* For a twist and to cut down on the sugar you can use :1/8 cup sugar in raw, 67g cup banana puree and 67g mango puree