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.



Showing posts with label Recipes. Show all posts
Showing posts with label Recipes. Show all posts

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.

Sunday, December 4, 2016

Christmas Cookie Reciepe!

     I do love Christmas time.  I love the music, the lights and staring at our Christmas tree...but I also love the smells!  The cinnamon, the cloves pushed into the orange, and the cookies!  Last year I promised Kristy that I would get this recipe posted, and I am so sorry that I didn't!  But alas, here it is, our Sugar Cookie Recipe.  I can't take credit though, it was my grandma's recipe that I exchanged the butter out for MCT butter...and it just so happened to work perfectly!  The MCT Butter recipe came from a dear LCHADD parent in Canada, so it was a group effort!
     I will start with the MCT Butter recipe because you will need to make this first and follow up with the sugar cookie recipe.  Enjoy!


MCT Butter

Ingredients:
·      1 tablespoon corn starch
·      3/4 cup skim milk powder
·      1 tablespoon lemon juice
·      3/4 cup water
·      1 cup MCT oil

Directions:
1.     Mix lemon juice, water and oil into a pyrex cup.
2.    Sift dry ingredients into a small sauce pan.
3.    Turn stove on medium heat.
4.    Slowly pour in liquid while mixing with a whisk.  (Alternate with a spoon if dry ingredients get caught in crevices of pan.)
5.    Continue to mix over medium heat until butter thickens and is smooth.
6.    Keeps in fridge approximately 2 weeks.

Tips:
* This recipe makes 2 cups of butter, so 2 cups of butter has 1 cup of MCT oil.
* DO NOT USE THIS RECIPE IF YOUR CHILD HAS MCADD or cannot process medium chain fats!
 

Grandma’s MCT Sugar Cookies

 Ingredients:
·      1/2 cup MCT Butter
·      1 tsp. salt
·      1 tsp. vanilla
·      3/4 cup sugar
·      3 egg whites
·      2 1/2 cups all purpose flour

Directions:
1.     Make MCT Butter using the attached MCT butter recipe.
2.    Combine MCT Butter, salt, vanilla, and sugar in a large mixing bowl.
3.    Beat three egg whites in a small bowl, by hand, until slightly frothy.
4.    Pour egg whites into large mixing bowl and stir to combine.
5.     Sift flour and mix well.
6.    Chill thoroughly in the refrigerator.  (This will take at least 2 hours!)
7.    After dough is chilled, pre-heat oven to 350°F.
8.    Line two cookie sheets with parchment paper.
9.    Flour counter top and rolling pin.
   10.  Take approximately 1/3 dough, lightly flour both sides and roll out flat with rolling pin until 1/4 or 1/8 inch thick.  Use cookie cutters to cut desired shapes.  Place cookies onto parchment lined cookie sheets. Re-shape dough, flour and roll until all dough is used. 
11.   Decorate cookies with sprinkles or crushed peppermint.  
12.  Bake at 350°F for 8-10 minutes.

Tips:
*When buying sprinkles be aware that different brands have different amounts of fat!  Some sprinkles are just colored sugar and have no fat!  Yet, the traditional “sprinkles” greatly vary on brand, so look close to see what best fits your child’s needs.
*This recipe is meant for any child who needs to supplement their diet with MCT oil, but should be safe for family members without FODs.  My husband and I enjoy a cookie with our son without a tummy ache from MCT oil!  DO NOT USE THIS RECIEPE IF YOUR CHILD HAS MCADD or cannot process medium chain fats!

*If dough is sticking to your rolling pin or counter top then you need to add more flour!  Sprinkle a little flour on the countertop so it is almost invisible, but still feels rough to the touch.  Sprinkle some flour on the dough and on the pin.  As you roll out your dough, flip the dough over every other roll, flouring the top of the dough each time so it doesn’t stick to the countertop when you flip.


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:
  1. Soak ½ cup tapioca in 2 cups of water overnight or for at least 5 hours.  Drain off excess water and continue with recipe.
  2. 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.
  3. 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.
  4. 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

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

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.