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 14, 2012

Adventures in Traveling

Traveling with a 3 ½ year old…ahh…the mystery, the wonder the…discovery!  The first plane ride that Christopher remembers was to visit his uncle in Colorado several months ago.  He bounced around the airport in amazement, and as the plane took off from the busy hub-bub of Atlanta he let us know, “It’s going to do it! It’s going to do it!”  Then when we got off the ground there was a huge handclap and shout for joy, “Hooray!” (I suppose I could have been a little embarrassed by the commotion, but who am I to squelcher a child’s joy?)  Our recent trip to Seattle, to visit friends and attend the National FOD conference in Portland, proved just as exciting.  To most of us connection
 
flights might seem like a drag (just something to elongate the process of getting to our final destination) but to Christopher these connection flights meant another adventure and more opportunity to see these massive creatures (the airplanes) take off.  He decided quickly after the flight to Chicago (which led to our connection in Seattle) that he wanted to be a pilot.  And so he carried his small airplane around and practiced “landing” his aircraft throughout the day! (with as much vigor and determination as an actual pilot, I would add)  So you can only imagine the amazement and excitement when a wonderful pilot let Christopher sit in the cockpit after our second flight!

The journey of traveling with a child who has LCHADD has a lot of similarities to anyone who decides to travel with children. There are the similarities like the story above, but also there are the missed naps, tantrums, songs sung to pass the time, beauty of new places, the joy of raw education, and the smell of hotel rooms.  Amidst all the similarities, differences exist as well…and these differences can lead to adventures in and of themselves!

Lets start with exhibit A (the picture to the right of the screen).  Now if you are an FOD/LCHAD parent reading this, the sight of the suitcase on the side of the screen probably brings a chuckle or a knowing smile…but if you are with the security at the airport the look on your face might be quite different.  The largest responsibility I have found in packing for our trips with Christopher is making sure that we have all of his medical formula, medical supplies (glucose monitor ect.) and his precious MCT oil.  I have to make sure that we not only have enough for the trip but enough in case we get caught somewhere because of bad whether, car breaks down or he has a crisis and has to stay in the hospital.  Then there are those every day experiences you have to prepare for…like spilt milk (i.e. medical formula)…literally!  So every trip we go on I count the cans and do the math at least 3 or 4 times to make sure that we have enough.  Then there is the MCT oil, this precious oil that gives him vital energy throughout the day.  Have I wrapped it enough with towels, so it won’t break?  Is it easily accessible?  If we are visiting larger cities there is the comfort that we might be able to find it in a natural health food store, but in the small towns finding this important medicine would be more than challenging.  Carrying this load of formula with us in our car is one adventure, but carrying it on the plane is another.  We have been really lucky going through TSA so far.  We carry a special letter from Emory so we can carry all of Christopher’s medical supplies, including his oil and formula, in a carry-on bag (you can’t risk putting it on checked baggage).  They scan and test everything for explosives, then let us through.  I am so glad that Southwest Airlines does not charge for your first two bags…because it would be nearly impossible not to check bags when you have to carry this much medical formula with you!

Now lets talk food.  By plane we carry a mini-cooler with a large assortment of snacks and meals, and by car we pack a very large cooler.  I must admit meal planning was something that I had to get used to when going on trips.  Before Christopher was born when Ryan and I would go on vacation, meals were a lot more relaxed.  Sure we would take snacks with us on road trips andcook at our destination, but during vacation time we eat out a lot more and were concerned less about meal planning.  Now it is so different because we know that eating well is so much a part of keeping Christopher healthy, we can’t take the “break” that we used to on vacation in regards to eating strategically (and I suppose we eat better because of it).  Before a big trip we always talk with friends or family to make sure that we will have certain staples when we get there (i.e. yogurt, potatoes, salad, peas).  Then we have to sit down and discuss the meals for the week: What meals will be “Christopher-Friendly”?  What meals will not be?  I try to make muffins ahead of time to always have on tap and fresh fruit and veggies are an easy go-to.  It is wonderful when friends and family offer to make a “Christopher-Friendly” meal when we travel because it frees my brain up for one or to nights! 

I find the most challenging thing about traveling via car is making sure that we have enough food for Christopher for several days.  Occasionally we are lucky enough to find a place to stop and get sushi or I can peel part of a chicken breast away at Chick-fila and add our own sides, but we have to plan as if these options do not exist, just in case.  Christopher at this stage is really accepting of what he can and can’t eat while we are traveling.  He is way more concerned with having a chance to play and having a cup with a straw in it when we stop to eat, than whether he can actually eat something off the menu.

The last two things that linger on my mind that wise older FOD parents have warned me about:  “Always make sure that where ever you travel to has a kitchen.” and “Remember that excitement uses more energy/calories.”  We have been really lucky because so far all of our trips have provided easy access to a kitchen…but I know that as we plan out future family trips this is something that will remain on the fore-front of my mind.  The whole excitement piece…I feel so grateful for the warning!  So far Christopher has done really well on trips, not exhausting himself from excitement, but I feel like I have to give credit to the families that have gone before us and shared that excitement can lead to extra calorie use and thus lead to metabolic crisis.  This warning has made us super vigilant on trips to feed him often even though it seems as though he is sitting more and possibly not using quite as much energy.

On a completely different note, I did want to let everyone know that we met our fundraising goal of $10,000 and that the clinic was very excited and grateful!  I apologize for my delayed response in letting you know (as many of you know I have been having some of my own health challenges the last couple of months).  If you meant to give but didn’t get a chance you can still give using the link to the side of our blog or by mailing in a check to the address below.  Please e-mail me if you gave and didn’t receive a tax credit letter, or the letter was mis-printed in any way!
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