What we know about VLCADD

The Basics

VLCADD is short for “very long chain acyl-CoA dehydrogenase deficiency.”

VLCADD occurs when an enzyme, called “very long chain acyl-CoA dehydrogenase” (VLCAD) is either missing or not working properly. This enzyme’s job is to break down certain fats from the food we eat into energy. It also breaks down fat already stored in the body. Energy from fat keeps us going whenever our bodies run low of their main source of energy, a type of sugar called glucose. Our bodies rely on fat when we don’t eat for a stretch of time – like when we miss a meal or when we sleep. When the VLCADD enzyme is missing or not working, the body cannot break down fat for energy and must rely solely on glucose. Although glucose is a good source of energy, there is a limited amount available. Once the glucose has been used up, the body tries to use fat without success. This leads to low blood sugar, called hypoglycemia, and to the build up of harmful substances in the blood. ( website)

It is a genetic disorder, so the mother and father must both be a carrier or one of them has the disorder. This also means that every child we have has the possibility of having VLCADD.

Diet and Lifestyle
As a baby, Benaiah’s diet is pretty much like everyone else. Up until he was 6 months old, he was exclusively breastfed. He had to eat every 4 hours so that meant even at night we had to wake him up to feed him.

Now at 9 and a half months, he is trying table foods and still has breast milk 5 times a day. He cant go longer than 5 hours without eating and he is limited to only 5 fat grams from food per day.

When Benaiah turns 1, he can go 6 hours between feedings and his fat intake will depend on his weight. He won’t be able to have whole milk, it will have to be 2% or maybe 1%, because of the fat content. His body can break down medium chain fats which typically aren’t in foods. So because of that, we will have to give him MCT oil (probably in his milk) and that’s where about 60% of his daily fat will come from.

(Have I confused you yet? Trust me, it’s a lot of information and I’m STILL trying to figure it all out!)

As Benaiah grows, his fat intake will increase as well as time between feedings. However, he will never be able to fast or go a long period of time without food. If he decides to do sports or other physical activities, we will have to take even more precautions than usual. (Thankfully we will have at least a few years until we get to that point! πŸ™‚ )

Benaiah’s Case
Benaiah has a unique case of VLCADD. Doctors have done multiple tests and they have all come back without a definite diagnosis. However, our geneticist believes that Benaiah is either a carrier OR has a mild case of VLCADD. Because of this, his diet restrictions aren’t as extreme as kids or adults that have a severe case. For that, we are extremely thankful (and always blessed)!!

Benaiah also has an emergency protocol if he were to become sick, start vomiting or refuse to eat. Basically we would have to take him straight to the ER so they can monitor everything and make sure he has enough fluids.

With our child having VLCADD, it’s hard to not think about the scary things that could happen. However, I am constantly reminded that the Lord is sovereign and He is in control of everything, for which I am so grateful for! God has blessed us with the most precious boy in the entire world and we know he has VLCADD for a reason. I’m hopeful that doctors will one day have another test we can take to determine either “yes he does had VLCADD” or “no he doesn’t”. Until then, I’m going to continue to share our story!

Our Story…

November 2012 was probably the most life changing month of my life (so far anyways).

My husband and I were expecting our first baby boy who was due to be born on November 19th. Starting towards the end of October, the labor pains began to set in and I was having contractions on and off every day.
Needless to say we had one false alarm where we went to the hospital, but they sent us home because I wasn’t far enough along yet. I went to the doctor the next day and he scheduled me to be induced the following Tuesday if I didn’t go into labor before then.

So Tuesday, November 13th, we woke up very early, had a 40 minute drive to the hospital, and were ready to meet our little boy! After being in labor for 18 hours, at 12:21 am on November 14th, Benaiah Cade was born! He was the most precious and beautiful little boy I had ever seen.

He was ours. He was created in God’s image and he was OUR son.

He was perfect. No one could tell me differently.

A week passed.

Then we got a phone call. It was from our pediatrician whom we saw just two days earlier. Grant, my husband, talked to him for what seemed like an hour, but was probably only 20 minutes. I kept hearing parts of what Grant was saying and I began to be very scared. Something was wrong. A million questions began to run through my head. My heart started pounding and I began to pace. Finally he got off the phone and told me what was going on.

Our doctor informed him that Benaiah’s newborn screen had come back (they take blood while they are in the hospital and run about 30 or 40 different tests) and the results showed that he could possibly have VLCADD. VLCADD is a metabolic deficiency in which the body has a hard time converting certain fats (very long chain) into energy. In order to determine this, we needed to take Benaiah to the local children’s hospital and have some more tests run.

My heart sank. Tomorrow was our first Thanksgiving with him and we had to take our 8 day old baby to the hospital to have blood drawn?

I completely broke down. How could something possibly be wrong? I just didn’t understand. Did I do something wrong when I was pregnant? Surely it was somehow my fault..

This was how our VLCADD story starts.

To make a VERY long story short, Benaiah had to go to the hospital 3 different times to get three different tests ran. All of them were done to determine whether he definitively had VLCADD or not. Well, the results came back and no definitive answer. 9 months later, we still don’t know if he has it or not. It may be years before more research is done and another test is available for him to take. Until then, we are treating him like he does have it.

What does that mean? Well for the first 9 months, we have had to make sure he eats every so often, meaning we have to wake him up every night. No full nights sleep for us, but Benaiah is such a good sleeper! He eats and then goes right back to sleep.

Now that he is 9 and a half months old, he can go 5 hours between feedings but we also have to limit his fat intake. He’s only allowed to have 5 grams of fat from food per day. When he turns one, he will be able to sleep up to 6 hours at a time, and his fat intake will increase based on his weight.

I decided to start this blog for several reasons. First, I want friends and family to know and understand VLCADD and ask questions. This is all new to us too but we are more than happy to talk about it. Benaiah’s diet will become our diet, as we will transition our food to be lower in fat.

Secondly, when we found out, we googled it and tried researching it and became overwhelmed. It was so confusing that we really just didnt understand it all. So I want to try to simplify what we know about it as well as be a support for anyone else who has to go through this.

Also, with our family transitioning to a new diet, I’m going to post recipes that we try and enjoy! I hope to make some substitutions with recipes we already love and post what we think of them.

In the mean time, please feel free to post questions and we will do our best to answer them! I will write another post soon that explains more about VLCADD for those who are interested πŸ™‚