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! 🙂 )
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!