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, August 28, 2018

"It's Cool to Have LCHADD..."


            This summer the Harry Crew took a road trip from Atlanta, GA to Minneapolis Minnesota and then on-ward to Lake Superior!  It was our first big road trip together that wasn’t to visit family and we stopped at several state parks along the way, and saw beautiful landscapes, boulders, and water.  After all of our adventures, what does Christopher talk about the most?  Where does he keep bugging me to go back?  “Mom, when can we go back to the Embassy Suites Hotel?”
            Yeah, sure, there was a pool, he got to watch soccer on a big screen, and he even got to eat something more than fruit at the continental breakfast (which is rarely the case).  But the zinger was that the FOD Family Support Group conference was there and for the first time, that he can remember, Christopher was able to connect with kids his age and older with FODs.

Learning how to make fat molecules and how they work in the body!

   This year at the conference, while the adults were learning about the latest research, and seeking to support each other through stories, their children were given the opportunity to do the same thing…in their own way.  We had two sessions for youth.

Learning about fat, protein and carbohydrates!

The first day we learned about our body: how it functions, how it metabolizes the food we eat, and how cells break down fat for energy in the mitochondria.  Our second day we did a science experiment discussing rhabdomyolysis (muscle breakdown) and then nine kids partnered up to role-play asking questions of doctors; confidently!
What touched my heart more than anything was watching Christopher connect for the first time with Luke, an older youth with LCHADD.  I’m not sure that they had super deep conversations about living with LCHADD (although perhaps I shouldn’t put anything past my 10 year old son), but Christopher enjoyed getting to spend time with him so much!  A space was created…a kind of space he hasn’t had before…where LCHADD existed outside of himself and his experience…and perhaps for a moment having LCHADD, counting fat, taking MCT oil, monitoring exercise; was a norm.  Christopher came back after playing cards with Luke Saturday afternoon and said, “It’s cool to have LCHADD!”  The door was open for him not to feel alone in this journey, and I still tear up thinking about it.
Luke took time to hang with Christopher during one of the adult sessions!
The conference plays an important role for every family who has a child with an FOD.  It creates space for clinicians to disperse new information, while inviting families to ask questions and seek understanding.  There is a tremendous feeling of support as you share your stories and listen to others.  These stories are also helpful for the clinicians to hear because when you deal with rare inherited conditions sometimes overlap of symptoms are noticed for the first time in environments like these. 
Thank you to Deb Gould and all of the volunteers that help organize this conference! And thank you to the clinicians and families that take the time to come!  Conferences like these are extremely expensive to put on!  Ryan and I are running a Half Marathon on November 10th at Chickamauga Battlefield in North GA to raise awareness about FODs and money for our next conference.  Please consider donating!!!  There are two ways to give.  You can either donate on-line: https://fodsupport.networkforgood.com/projects/44599-fod-fundraising-2018 (select amount, then “Chickamauga Battlefield”) or you can donate via check: Payable to: “FOD” and put in memo “Race donation/General Trust.  All checks should be mailed to: Deb Gould, PO Box 54, Okemos, MI 48805-0054 .  All donations are tax deductible!



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