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, September 9, 2020

Plastic and MCT Don't Mix!!

Christopher and his two amazing teachers who came to support him!

        I'll never forget the beginning of the pandemic.  I suppose we all won't, for different reasons, but ours was centered around the GA State Science Fair.  It was March, Christopher had worked super hard on his Science Fair Project in the fall and had won first place in the district fair. (He got "best overall" in elementary school for all of Metro Atlanta!) He was super excited to head to the state fair!  At the time, there were only six cases of COVID-19 documented in Atlanta, and his doctors felt confident that it would be safe to attend the science fair, as long as we washed our hands really good.  So we headed down to south GA, enjoyed our hotel room, and the next day he presented his project.  He had as much fun presenting his project as he did connecting with other students!  I couldn't have been prouder!  As we headed to our car after the fair was over, it was then that we learned Atlanta was shutting down!  I thought, "Oh my gosh, out of all the times I could have taken him to be around 200 kids and their families...I chose to do this amidst a public health crisis!"

       We survived that event, without implication!  Unfortunately, amidst on-line learning and obsessing with trying to understand how to approach COVID-19 and LCHADD.  I lost sight of the promise I made to Christopher that I would share about his science project and results on my blog for families to see!  Sorry Christopher!! If your child uses MCT oil, I strongly encourage you to read this entry in its entirety!  Not only am I proud of Christopher for the amount of time and hard work he put into this project, but his results are really important for FOD families to share with each other.  It has changed some of the approaches we have taken as a family when we use MCT oil!

Chris chose a variety of materials to test MCT oil's reaction!

       It was early October when I got a new shipment of Brand Now MCT Oil (this was the type of MCT oil we used for Christopher since he was born).  I was surprised when I noticed that the bottle was no longer in glass but plastic!  I vaguely remembered someone sharing with me along the way that plastic and MCT Oil don't mix.  I contacted the company concerned, but to no avail.  I contacted Nestle, who is in charge of medically grade MCT oil, and they confirmed that MCT does effect plastic which is why they put their MCT oil in glass bottles.  It was around this time that Christopher was trying to figure out what to do for his science fair project.  Ryan said, "It would be great if he could do something related to LCHADD."  Christopher then asked, "Mom, what exactly happens to the plastic when it comes in contact with MCT oil?"  I didn't know...but Christopher wanted to find out!

       Christopher spent the beginning part of his project just trying to better understand what MCT oil was, how it worked in his body, who used it, and how it was made.  Then when he was ready to test "the plastic" he quickly realized that not all plastics were the same.  Plastics have different numbers depending on what they are made of, and what they are used for.  He looked around our house to see what plastic numbers were on our storage containers, water bottles, and measuring utensils.  Then he decided he wanted to test plastic numbers 1,2, 4, 5, and 6, along with aluminum foil (because we use this when packing food for trips) and glass because he was certain that this would be the least reactive.

       Christopher poured MCT oil into various containers about the same size and measured the results over the course of two weeks.  Here were his results!  He created two different visuals to show his results.  Notice on the left side of the chart all of the possible changes that occurred in his experiment!



       I think we were both surprised at the changes we were able to see with our naked eye!  Honestly, when he started his project I was a little worried that he might not observe many changes because we didn't have a microscope...and I wondered if two weeks would be long enough to see change.  I kept my thoughts to myself though because this is science, and he needed to try and observe.  So you can imagine how surprised I was when he went to make observations of his materials the first week and found that MCT oil had completely leaked through the styrofoam and visibly eaten away at the material. Then he also noticed it had leaked through plastic #1 and #5!!  How many times have I put MCT oil or Lipistart in Styrofoam cups when he was in the hospital? Or used plastic straws in a smoothie with MCT oil?!
Notice the squiggles around the "Chick-fil-A" sign!  They were not visable before!
       I also encouraged him to get curious about why plastic #2 and #4 decreased in measurement, but there was no visible leakage on the paper.  It didn't evaporate...all the containers had lids! By the second week he noticed more changes when he emptied the MCT oil from the containers to take a closer look! Look at the squiggles that were formed on Plastic #1!!

                         Also the styrofoam's  plasticity changed!

       Needless to say Christopher's project spurred new questions along with answers!  I also started paying attention to when we were using plastic measuring tools for reciepes, plastic spoons to stir things on the go, and plastic plates!  We were at my mom's house over Christmas and he had put his eggs on a blue plastic plate and without thinking we put MCT oil on his eggs.  Christopher came in with his eggs gone but said, "Hey mom, the MCT oil took the blue off my plate!  I had blue eggs for breakfast!"  Horrified, I recounted green eggs and ham, and laughed because he had just finished his experiment but apparently neither of us had transferred what we had learned to this portion of our life!  Here were some of his take aways:

       What I loved about this project were all of the "moments" of growth I saw!  First, it warmed my heart when he presented his material to his metabolic doctors and dieticians, and they really thoughtfully were considering his data.  Then there were the moments where we made connections between what he was doing and what "real" scientists do every day.  He got a feel for the scientific process, contacting "professionals", and gathering data!  He also got to see how meaningful research can really impact communities and how they do things! (We won't be using plastic spoons or plates with MCT oil anytime soon...lol) But more than anything, I was so proud of him for learning about his metabolic condtion and taking the time to explain his need for MCT oil with his peers and judges.  My boy is growing up!







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