"Why can't I eat that?!"

So far this is not a question that has come up too often. As Christopher grows and he is exposed to more children, parties, and events where there are lots of food that he cannot eat...we are preparing ourselves for this question. Right now Christopher more often asks, "Is that Christopher-Friendly?" And seems satisfied with the answer, "No...because it has too much fat." I think it has also been really helpful for him to see that different kids and adults are at times limited in what they can eat. His cousin and another close friend are both severely allergic to peanuts...and mommy can't eat strawberries...and someone else can't have gluten. The other day we were eating a cracker and he asked me, "Mommy is this Kristina-friendly?" To which I said, "No sweetie." "But why?" "Her body does not like gluten very much." In that moment, I found myself grateful that all of our bodies are a little different...so he doesn't have to feel like his is s-o-o different.

Although it would be nice if these small conversations would be enough, I realize it will not always be this easy. And truthfully there is definitely part of me that has had to grieve the foods I wish he could just try (flavorful cheeses...rich chocolate brownies...or just plan old chocolate!) or the fact that he will never be able to go into a restaurant and get just "whatever" he wants off the menu. I worry about how we will encourage him through peer pressure and help him process some of his differences, and yet I know we will face these challenges head on as they come. Currently, two main tips have challenged me as a parent.

First Tip: "Don't feel sorry for him." I can feel sad that he can not try some yummy brownies, but the fact of the matter is, there are a lot of other yummy foods he can have! A dear friend reminded me that I need to sort through my own grief about his food so he can better develop his own perspective. She said, "Honestly, he may not be the type to care. And if he isn't and you teach him to feel 'sad' about his condition or his 'limitations' you will make it harder on him. Let him be sad or frustrated if at some point he feels that way...but be okay if this is not the emotion that he puts with diet." And so I find that I try to be careful when we mention that he cannot have certain foods. I try to make sure my tone is nonchalant instead of pity-filled. I think it also helps that we try to keep mainly Christopher-Friendly foods around the house so there are not lots of food in his daily reach that he can't have...home becomes a safe food place for him.

Second Tip: "When he gets to school try to make his lunches look like the other kid's lunches as much as possible...even if they are not." It hadn't crossed my mind, but makes so much sense. I mean there will be some things we just can't adapt. Then there will be the apple, raisins, box drink , pretzels that are in many children's lunches. Do you know how excited I was when I found fat free cheese and a special peanut butter called PB2? Yes, I realize that grilled cheese sandwiches, pizza and peanut butter and jelly are not the most nutritious choices...but to know that we can make an equivalent made me so excited and hopeful for some of his future social interactions around food. (PB2 has been wonderful! Who knew that they made powdered peanut butter where they extract the fat out...so it has only a tiny amount of fat in it! And the company has been amazing in trying to understand Christopher's disorder too!) I just feel like as he grows he will have to spend quite a bit of time explaining his diet and disorder. I hope that days with his special PB2 sandwhich, fruit and yogurt will not have to be one of those days!
"Why can't I eat that?!" And so we move forward and answer his question by looking on the back of packages when they are available. We show him the number next to the word fat and explain that there are too many of those fat "catipillars" running around in this particular food item. He responds, "Oh..." and takes a nice big bite out of the juicy strawberry he has in his hand.

Did you know fat chains have different sizes? Who knew?!

There were a lot of things I didn't know or understand about the body before I had a child with LCHADD. In college I took environmental science classes and so even basic anatomy was a little out of my league when I first started trying to understand what was really going on inside of Christopher's body. One of the many fascinating things that I learned was that fat exists in various sizes. Then when you consume fat your body begins to break it down into smaller and smaller forms of fat, to use for energy. I quickly learned that there are very long chain fats, long chain fats, medium chain fats and small chain fats. The length depends on the number of carbons that are connected together. And how your body processes these fat chains depends on the length.

As I was studying about fat and fat metabolism, I began contemplating how I would explain this all to Christopher. All of the drawings that I saw of fat molecules looked way too complicated ...and so the more I read about fat, the more I began visualizing them as caterpillars. The above picture is an excerpt from the children's book I wrote about LCHAD, VLCAD and TFP. I wanted to share it with you so you could visualize more clearly that fats have different sizes.Most of the foods we eat are primarily comprised of long-chain fats (the ones that Christopher has a hard time breaking down) but there are short and medium thrown into some of your fruits and vegetables too! Coconut is a little different. Over 90% of coconut's fat is medium chain. This is actually what Christopher's special MCT oil comes from! Although MCT oil has a low smoking point so we don't sautee with it; we can sprinkle it over food, make special butter with it (we made sugar cookies with this butter last Christmas!), and even make a "Christopher-Friendly Pesto" for pasta. This oil is really a life source for Christopher because it gives him a longer form of energy than just glucose and protein to use. We give him this oil and his special medical formula throughout the day to try to give him constant energy. If we know he is going to the playground we give him the oil in food right before we go and follow up with coconut water or Gatorade (which is another great energy source for LCHADDers)

Race and Fundraising Update!

Okay, so pictures from last Easter were probably not what you expected to see under this title. But lets be honest, is there anything more glorious than a child running after Easter Eggs (especially when he is still at the age that it is more about the hunt of the egg rather than what is inside...)! And while we are being honest, looking back on pictures like these remind me not only how lucky we have been with Christopher's health...but encourages me to continue training for our Sprint Triathlon, even when it is difficult.

Difficult? Yes! I am not going to pretend that I have gained some super athletic power sense being in the hospital. Even Ryan is feeling quite challenged by the swim! But we are training hard. We take Christopher on bike rides with us once or twice a week. And he can still squeeze into the jogging stroller so we try to do a couple mile jog together once a week. Then we go to our local "Y" for lifting , running and swimming. Two weekends ago we did our first 16 mile bike ride without Christopher and tried a two mile run afterward. The bike ride went great. Ryan felt great...I was a little exhausted. Then we started our run. 5oo yards into our run I tripped and fell. I remember (as I was flying through the air-literally) that I needed to land in such a way that I didn't hurt myself too bad...as I came down on my hands and knees I mostly succeeded. My knees were busted open, and my hands scratched up...but no broken bones, chipped teeth...I did great! Well, that was until fall number two. (Can you believe I fell twice during one run!) So maybe it was the uneven sidewalk...or maybe it was just being tired...but that second fall I sprained my ankle...which left me healing for about a week before I tried to run again. I knew that training would be painful, but honestly I haven't scrapped my knees since high school and those boo-boos hurt when you are old! Who knew!

This week we are training strong...and hope to do another 16mile bike and complete a two mile run (and maybe our swim) in a couple weeks. We will see! In the meantime pray that I keep my balance and lift my feet off the ground when I run...lol!

On a fund raising note so far we have raised $1, 475 for the clinic. Emory keeps track of donations that are coming in with the various codes that are posted on them. We are so appreciative for all that you have contributed so far! As we see support come in, we are reminded of the vast community that is around us! It is our hope that we can raise $10,000 for the clinic, so if you have not donated yet, please consider this possibility! I keep thinking if we can just find 400 people to donate $25 we will meet our goal!

Thanks for all your love, letters, thoughts and prayers!

What is a child’s prognosis with LCHADD?

It’s a hard but important question! LCHADD, along with several other metabolic disorders were added to the newborn screening almost five years ago in GA. (Many of you might know this test as the “heel prick.”) This test is not only saving children's lives (it is thought that many children with LCHADD died of SIDs before the test was routine), it is also helping to better the health of children with these disorders. We were very lucky that Christopher was picked up on the new-born screening with LCHADD. He has been on his special diet since birth, and has not had a metabolic crisis until recently! Doctors are hopeful for children with LCHADD if they are able to stay on their specialized diet and are able to forgo metabolic crisis as often as possible. But, the fact still remains that there is a lot they don’t know or understand. Children with LCHADD have a high propensity of pigmentary retinopathy, which leads to blindness and they are unsure why. There is also still a lot unknown about the mitochondria and how it works (which is where Christopher’s deficiency occurs). Lastly, because FODs are rare inherited diseases many clinicians have not heard of LCHADD or have not had a patient with it. This can make LCHADD hard to treat when children are in metabolic crisis. We hope so much for Christopher, but there is a lot clinicians would like to learn in order to give him the best possibility to live a strong healthy life.

Christopher's First Hospitalization

On the evening of January 15^th Christopher appeared to have a cold…by January 17^th he wasn’t eating well and was unable to keep all of his medical formula down because his cough was triggering his gag reflex…so we headed to the emergency room. At the time of his admittance his CKs (or level of muscle breakdown) were 11,000 (normal range is 120-220). During his hospital stay his CK levels peaked at 70,000. There are several things that doctors worry about when children or adults have high CKs but the main concern is kidney failure. High “CKs” or creatine kinase in your blood means that you are having muscle breakdown. This can happen for various reasons, but for children with LCHAD elevated CKs typically occur when they have utilized all of their energy stored in the form of sugar. Their body turns to fat as an energy source but can’t break it down so it starts to breakdown the muscle instead. CKs are the by-product of muscle in the blood stream that doctors can measure. Myoglobin is another by-product whose levels coincide with the amount of CK in the bloodstream. It is the myoglobin that is hard on the kidneys and can create kidney failure and put a patient on dialysis. The large reason we have to take Christopher into the hospital when his body starts breaking down muscle is to get more glucose in his system so his body stops trying to use fat as an energy source and to keep him very hydrated, so as to protect his kidneys from renal failure.

It probably seems quite simple to say that having Christopher in the hospital was hard, but for all that we experienced it seems that this might be the best adjective after all. I must first say that we are really blessed to have such a wonderful metabolic team who worked extra hard at coordinating care with the pediatric docs in the hospital. Christopher’s clinic and head dietician stayed in touch with me throughout the whole process; updating me and answering questions along the way. Also we had amazing family and friends who called, talked, sent food and prayed. But having a child with a rare inherited disease like LCHAD means that the clinicians who worked so closely with him at the hospital had most likely not worked with a child with his condition. It meant that we as parents needed to encourage communication with his metabolic clinic and educate staff as much as possible. As with any communication among groups sometimes we did better than others. I found myself, worried, stressed, tired…and probably a little on edge with the docs at times. Then as he started mending in the hospital, there came the challenge of trying to find foods low enough in fat to feed him.

He was in the hospital for almost a week, when he was discharged. His doc was so nice to talk with him and give him a stethoscope so he could check his stuffed animal’s vitals at home. When we left the hospital his CKs were still not normal, and we were told that he was to do very low-key activities and not be around other kids for two weeks. Two weeks turned into almost a month, as his CK levels took three and half weeks after his hospitalization to get back down to normal. During that time we did a lot of drawing, painting, playing with animals, game…movies…but I must admit by week three we both were beyond stir crazy! Christopher has maintained a positive attitude through it all…and even when he had to go back for several blood draws after his hospitalization, although he didn’t great the blood draws with a smile, he was able to find one for his Dieticians and phlebotomist!

About a month and a half after his hospitalization

Christopher still mentions “Mr. Tubby” (his NG tube that he had to have put in while at the hospital) and checks Ni-Ni’s (his favorite stuffed animal) vitals and blood work quite regularly. He seems to be gradually processing the experience, but nighttime is still hard for him.

Five grams of fat a day! What does he eat?!

For kids like Christopher food is their medicine. We learned this early on! Emory’s metabolic team spent hours explaining to us what they knew about LCHADD and how to do everything in our power to prevent a “metabolic crisis.” The key they told us was to make sure that his body had enough glucose that it did not need fat as an energy source, because oxidation of fat would lead to muscle breakdown. To ensure that he had a steady amount of glucose in his system, we fed him every three hours, around the clock as an infant. As he got older we were able to go a little longer during the evening hours while he is sleeping. Currently, at age three and a half, he eats about every 2 ½ hours during the day (or more often if we are doing an activity that requires a lot of energy like hiking or playing on the playground). He also awakes in the middle of the night (most nights) to grab a sip of his medical formula and something starchy to keep his sugar up while he sleeps.

So what does he eat all throughout the day? Well, a lot of fresh fruits, vegetables, beans, and rice. He can eat almost any vegetable but the key is how it is prepared. You cannot sauté his foods in olive oil or add just any kind of dressing to his salad. There is no butter on his potatoes. (Did you know sweet potatoes are actually really delicious without the butter? Who knew? Ry and I find them marvelous and it is Christopher’s favorite food!) We have to be intentional about what kinds of crackers and breads that he eats, as these can be loaded in fat. Beans are great, but again, how were they cooked? And certain beans have more fat in them than others. (Did you know that 1oz. of chick peas has the same amount of fat as 1oz of chicken breast?) What is his favorite food besides potatoes, broccoli and pasta? Sushi! The main challenge is monitoring his intake of foods and making sure that he doesn’t have too much of the little amounts of fat…and they add up quicker than you could possibly imagine. We have also learned several ways to cook oatmeal and a yummy recipe for Tapioca, as he needs to have these starchy meals right before bed so he can go several more hours without food. Complex carbs store glycogen in his liver and gives him a steady energy source through the night!

He still drinks his medical formula, Lipistart every day. We call it his “Power Moo!” The other addition to his diet that is so important is cornstarch added to Gatorade or coconut water before strenuous activity…and of course MCT oil throughout the day. MCT oil is comprised of medium chain fats extracted from coconut oil. He can use this fat for energy, so it is an important part of his diet. We mix it into his foods and his drinks. One of his first phrases (outside of “Michael Jordan shoot ba-ball game.”) was, “MCT oil make body feel g-o-o-d!”

What does daily life look like?

In a lot of ways, from the outside looking in, our daily life with Christopher looks very similar to other families who have a three-year-old child. Christopher loves playing basketball, making art projects, reading books and is just curious about life. He also loves to test boundaries and must endure the occasional “time-out”. Christopher is a bundle of energy and we are always so excited to watch what he will do or say. (You know kids are the funniest creatures around!) Recently, when asked if he was excited about going to pre-school next year he responded, “Will there be girls there?” When I smiled and said, “Yes.” He continued, “Will they tickle me?” It took all I had not to burst out laughing! Then there was the trip to the grocery store where he just looked up at me and said, “I love my mommy!” How could your heart not melt! We are very thankful for all that feels “normal” and we are so grateful to our metabolic team, who has worked so diligently with us to make sure that he is as healthy as possible.

The challenging things for us are the unseen things. There is the constant counting of fat. Fat is in everything, even fruits and vegetables. He can only have 5 grms of fat a day right now, so you can imagine how quickly that adds up quick! Then there is monitoring to make sure that he eats enough to sustain his daily adventures. Typically this means he eats about every two hours or more. Lastly, we try to monitor, as best we can, his exposure to illness. The reason for this is that if he catches a cold or a stomach bug where he cannot keep food down, his body will start to metabolize muscle and he will have to be hospitalized.

But honestly, to look at him all you can see is the amazing kid he is and precious young man he is becoming.