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, December 9, 2015

Tradition...Tradition!!!

     The fall and winter are loaded with holidays!  I suppose I was never really awed by this until recently, when I began to mentally list them: Halloween, Fall Festivals, Thanksgiving, and shortly thereafter Christmas and New Year's!  Along with these holidays comes "the way" they are celebrated.  This is the essence of tradition.  
     One of my favorite Christmas traditions growing up was making fried apple pies with my mom on Christmas morning.  Every Christmas morning since I was a senior in High School we would wake up, put on our aprons, flour the counter top and roll out biscuits to make apple pies that we would fry in a pan of oil.  If you have never made fried apple pies before, it is truly a southern art!  If the oil is too hot the pie will burn, if it is not hot enough the pie absorbs the oil...gross...but, if you get the temperature of the oil just right the pie transforms into a crispy scrumptious treat.  This was an important tradition that I carried into my marriage and hoped to continue with my child one day.
One of our favorite fall traditions is apple picking!

     Fried Apple Pies!  Are you trying to calculate how much fat is in one fried apple pie? Don't try, your brain will explode. Our first Christmas together with Christopher I was rolling out dough to make pies when it hit me that this would be a tradition I couldn't share with him, at least not in the way I had experienced it.  I remember saying to my mom, "How could we make 'Christopher-Friendly' fried apple pies?"  What seemed like an oxymoron, led to a great conversation, and starting a new tradition! This was just the first of several traditions that needed to adapt or be left behind.  
     It's hard as a parent to give up the ways we have always done things, and it can challenge our creative prowess to attempt to adapt our traditions to new situations.  I just want to encourage every reader that it can be done!  There are lots of reasons as to why our traditions may need to adapt.  I think the biggest key to creating change is having parents who are on board and are able to get those around them excited about the new way of doing things!  This is not always easy, but it is possible...and perhaps the process can keep us young with new ideas.  If you already have kids and it is an existing tradition that you are trying to change, include your children in the problem solving effort.  If you are needing to change a tradition and it's your first child, like us, including our extended family and friends in the problem solving process has helped us feel loved and supported along the way. Below are ways some of our traditions have metamorphosed:   
     Pumpkin Pie-  Yes, you can make filling for pumpkin pie virtually fat free pretty easily.  You just substitute eggs for egg whites, and evaporated milk for fat-free evaporated milk.  When Christopher was small we just used custard dishes with no crust...and it was still yummy!  As he has gotten older, and can have more fat, we have created a graham cracker crust using "Erewhon" graham crackers (the lowest in fat I have found) and non-fat yogurt. 
Can't beat cookie making!
Sugar Cookies- Soon I will include a post with an amazing sugar cookie recipe made with MCT butter. Once a dear friend from Canada shared with me her recipe for MCT butter, it made the Christmas tradition of sugar cookies possible. Christopher LOVES making sugar cookies with his friends, and everyone can enjoy a cookie or two without worrying about upset tummies from the MCT oil.  
     Pizza-  What kid doesn't look forward to a movie night with pizza and friends?  And how many birthday parties do we go to a year where pizza is the main medium of food!  Pizza is a fun treat, but when you go out or order pizza there is just way too much fat for a kid who can only have 9 grams of fat for the entire day!  This tradition has adapted significantly in our family.  Pizza nights are no longer the "laid back" nights where I get a break from cooking.  Instead, they have become a creative adventure we experience with friends, neighbors and family.  We make our own dough at home, with no oil.  We roll out our own pizzas and then we all get to decorate however we would like!  I have found that this has not only become a fun family tradition but a lot of Christopher's friends enjoy 
Another successful pizza making adventure!
coming over and participating in this process.  Many kids in this day and age have never made their own pizza, so there is a huge since of accomplishment and surprise when they roll out the dough, decorate, and partake!  Christopher loves loading his pizza with veggies and uses non-fat cheese on top...and we can take extra slices of his pizza for him when there are celebrations where this kind of food is present.    
     Halloween-  Halloween and trick or treating is something we only did until I was in third grade, and then we did fall festivals.  I loved dressing up as a kid, it allowed my inner actress to come out and be thoroughly represented.  Christopher did not fall far from the tree!  This year he dressed up as Chewbacca from Star Wars.  He practiced those noises for hours!  (Perhaps we should have encouraged a different character...)  In years past we have not encouraged trick or treating.  Ryan is a teacher at the local school and a lot of his students come by our house, so Christopher was very content to dress up and pass out candy.  This seemed perfect because then we didn't have to go through the process of sorting out candy, and belabor what he can and can't have.  This year was different. Christopher really wanted to go trick or treating.  We talked about the fact that there would be a lot of things he couldn't consume, but he still wanted to go, so we thought we would let him try.  He and his best friend from school, Ireon, dressed up and we all went out together.  This whole experience was heart touching, and believe me I never thought Halloween would feel sentimental.  I had three neighborhood families contact me to make sure they had treats that Christopher could have, and then the evening of Halloween when I took Christopher 
Look at all of their Halloween booty!
and Ireon out, they had a ball!  After the first house Christopher was so excited, "Mom!  I can have everything that they gave me!"  Then at the second house he traded with Ireon something he couldn't have.  Then at the third house when a neighbor tried to give him chocolate, his friend Ireon spoke up for him and said, "He can't have chocolate, he is allergic."  The neighbor just smiled and asked Christopher, "What can you have sweetie?"  To watch neighbors and his peers love him in this way meant so much! 
     Christmas Day-  Do you remember as a kid waking up Christmas morning and dragging your parents out of bed, going to the Christmas tree and opening presents first thing?!  We wanted Christopher to have this experience too, but he has to fuel up!  At first I was really frustrated at the fact that his LCHADD had to take priority in his life this day too...why can't one day out of the year he just not have to think about "fueling up"?  I think we all have those moments where we just feel like it is not "fair" and wish we could do things the way we have always done them .  Yet, I am continually reminded that the more I am able to embrace our new routines and traditions the easier it is for Christopher...and for him these routines are nothing other than "normal".  On Christmas morning our routine is to give Christopher Lipistart right when he wakes up so he has some immediate
Dad, Christopher and Uncle Joey enjoying breakfast!
fuel.  Then we let him open a couple of presents from Santa, make a family breakfast of apple pies and eggs, all eat breakfast together and then open presents together as a family.  I love it, and have really appreciated this adaption.  It allows our Christmas morning to go at a slower pace, and really enjoy each others presence.
     Fried Apples Pies-  Did you notice I said apple pies in the above paragraph?  Yes! Upon feeling defeated about our Fried Apple Pie tradition, my mom suggest that we try baking some apple pies.  Not only did it work, but I have found the pies just as tasty! Christopher loves the baked apple pies and now can participate in the tradition!  Truth be told my mom and I still fry a couple for ourselves for old times sake, but honestly I love the baked pies!
     Traditions are so important, not only to kids but their parents too!  I find so much hope in knowing that adaptions can allow us to participate in the traditions that are most important to us.  G.K. Chesterton put all of this into perspective for me in his selection, "Enjoying Floods and Other Disasters."  In this writing he shares a story about a man who every day struggled to open a drawer in his dresser.  Every day the drawer was jammed and every day he would become more and more frustrated.  Chesterton suggested to his friend that how one views the situation can vitally change one's outlook.  If everyday you get up and you view yourself going to battle against this drawer, of course you will end up angry and frustrated, but if you can wake up and imagine, as you are pulling on the drawer, that you are "tugging a life-boat out of the sea" or "roping a fellow-creature out of an Alpine crevass" then the problem becomes an adventure.  He goes on to say, "An adventure is only an inconvenience rightly considered. An inconvenience is only an adventure wrongly considered." (G.K.Chesterton, "Enjoying Floods and Other Disasters", Spiritual Classics, 303) Chesterton pushed me to view my greatest moments of defeat, frustration and uncertainties as a way to access my ability to be creative.  And when changing a tradition  feels utterly overwhelming, I think about that drawer-and how our kids' situations give us the opportunity to become more creative and adventurous than we ever thought possible.
      




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