Fat Dreams

As you may know we have to count every trace amount of fat so that Dominic's diet does not go past 1 gram of fat a day. Seems easy enough right, just pick baby foods that have 0 grams of fat. Well, it's not that easy EVERYTHING has fat in it, very small amounts but it's there. Fruit, veggies, all of it. So I go to the USDA website and plug in what I've fed Dominic and then it gives the amount like .12g and so on. Then I have to add it up to see what he can have for the rest of the day. Well if you don't know me, I suck at math, I'm horrible and it doesn't help that my memory is shot after having kids. Jeff thankfully is great at math and made me an excel program to help keep my math headaches minimal. With this daily system of figuring out the fat I'm now having bad dreams about food that I checked and had small amounts to where they were safe for Dominic. In my dreams I'm finding out that what I thought was safe really had tons of fat, these are actually night mares for me since my baby's health depends pretty much only on me since I'm the one taking care of him all day while Jeff is working. I'm hoping these dreams will stop soon, I know that I'm doing everything I can to keep the fat under a gram a day I guess my mind is subconsciously insecure.

I'm a little sad

Got a call from the Dietitian from the last set of labs where they were a little elevated. I have to cut back another breast feed and he can only have 1 gram of fat from food now. So I can only breast feed 3 times a day. I know it's best for him but it's a little sad for me.

Aidan is clear! & Dominic's Lab work update

Yay! Aidan does not have VLCAD - THANK GOD! That would have been a huge crisis to change a very picky boys eating habits among other things that would have had to be done. We were so relieved to hear it.

Dominic's labs came back and this time they were more elevated than the last 2 labs. His fat levels were a bit higher and his CK levels were higher than the last. They are okay enough though, we just have to keep getting them checked of coarse. The Doctor said he'll probably have muscle pain & weakness through out life. I've known that most VLCAD people do have this but not all so I was hoping maybe we'd escape it. It's always such a reality check to get the lab results. He has been doing so well so sometimes it seems like he's perfectly fine, though we know he has this life long condition that has to be managed closely. But he's made it 6 months with no problems like illnesses that would send him into the Hospital. Every day I thank God that they had the newborn screening available, it blows my mind that only 2 years ago they did not have it for this condition and we would have and were on our way to find out the hard way, had we not known so quickly. I'm an active reader on the support group boards and any new information I can find on VLCAD. I've read many many heart breaking stories, many children were not as fortunate because they did not know until a major crisis. But I've also read some good stories of healthy children with VLCAD that are thriving and going to school and have it all under control. There's lots of advice on how to manage on the boards, so thankful for that as well.

Formula

It's amazing how Dominic's personality changes when he does not get the MCT formula that he needs. I ordered last week but someone messed up at Vitaflo and so I called frantic yesterday that the formula is still not here. So last night was his last formula bottle. For the night feeds I breast fed only, then breastfed this morning again. By 9:30am he just wanted to sleep and has been so fussy. In 8 hours not having the MCT formula = a fussy over tired baby. We received the overnight shipment today and I gave him a bottle, he's back to his normal happy self. I'm shocked at how the lack of Medium Chain Fats affect him. It makes sense though because he cannot burn the Long Chain Fats for energy, with out the Medium fat he can burn he had no energy.
I hope to not run out of the formula or MCT oil when we start that. I'm gonna have to figure a way to have a good stock pile of it.

Things are looking up??

We had Domino's metabolic genetics appointment today. He's doing very well, growing like a weed. The last lab results on fat were very good, in the normal range so that means his diet is working. He had slightly elevated CK levels (muscle/heart damage levels) that I was worried about but the Doctor said they were nothing to worry about - woo hoo! He will get another Eco cardiogram at 12 months of age to be sure his heart is not enlarging.
We also got a Glucose meter to check his blood sugar when he doesn't eat very well or starts to get sick. We have to take him in if it's under 60 but we are not to rely solely on it, we are supposed to really rely on the way he is acting when he's not feeling well. So irritability, lethargy like when he was a newborn and of coarse if he's not keeping anything down we'd go in. We got through his first cold and he did great, kept on eating!
We found out today though while he is still on formula & breast milk until he's a little over a year he is only allowed 2 grams of fat from food, this is such a small amount! One baby food jar of chicken & rice dinner has 3 grams of fat! So we will mainly feed him only fruits, veggies & baby cereal until he's over 1, anything we give him for table food at first will have to be fat free. We didn't expect it to be that low of a number. Eventually he will be able to have more fat. So that was the only downfall to the visit, overall he is doing much better than we thought he would.

Aidan had his DNA testing done so that we can be sure that he is not VLCAD, it will be a major crisis if he is, trying to get him to eat fruits & veggies and no fat would be impossible! We highly doubt that he would have it, if he did it would only affect him mildly obviously, he's always been a healthy child.

He Poops!

Finally, he is pooping regularly. This has been so difficult for us. Dominic has had constipation and pooping problems since a few days after he first started the Lipistart special formula he's been on starting at 1 week old. Before the formula he was a regular pooper, 4-5 times a day. You'd think we wouldn't complain about not having to change poopy diapers but it's really hard to watch your baby struggle trying to get one out. Since we could not change formulas, we've had to force it or it doesn't happen, we forced it by by suppositories, kyro syrup and even prescription laxative almost daily. Since he started solids this week I've gotten a poo every day without doing anything!! Woohoo! Big milestone for us!

Captured the Smile!

Dominic has been smiling for a long time but he never lets us get it on camera, the second we bring out the camera he stops. So I finally got one!
He's been rolling around all over, giggling at all of us, he's one happy baby!

He seems healthy, is he?

I've recently had the question "he seems perfectly healthy, is there really something wrong with him?" asked about Dominic. So I want to answer this for anyone wondering. He is a healthy baby, doing very well. He is however missing/does not work the enzyme that breaks down fat. He will live a normal healthy life as long as his very low fat diet, his supplemented medium chain fat, and his more frequent feedings that are needed are followed. We do have to always keep an eye on him making sure that he is acting "normal" but again we normally would only have an issue that would require immediate hospitalization if one of the following happens:

-He gets the flu - *it is very important that we are not around people that are sick/have just been sick*
-Illness/infections
-He has diarrhea
-He has been out in the heat too long, overly heated
-Excessive exercise
-He is dehydrated
-He is not eating for some reason
-Going too long without food; currently it's feeds 3 hours during the day and 4 hours at night since you use less calories while you sleep
-Low blood sugar (we get a glucose checker soon)

Any of these would require hospital care and a continuous IV until he is better.

This would be called a metabolic crisis - here are some symptoms of one:

* extreme sleepiness
* behavior changes
* irritable mood
* poor appetite
* fever
* nausea
* diarrhea
* vomiting
* hypoglycemia

We have so far not had any problems, he has never been sick and he eats like a champ. We are just extra cautious when we are outside for long, we feed him on a strict schedule. He is doing much better than Jeff and I thought it would be. He had at birth abnormally high risk fat levels, they are now in the normal range with his diet and get checked regularly.

Sadly for me, Aidan will not be able to go to Preshool this year, out doctors think it would be best if he is not around so many germs that he could bring home to Dominic. As long as Dominic is doing well next year Aidan can then start Preschool for 1 year before Kindergarten. Aidan is actually very happy about not going to Preschool, he thinks school is some bad place for some reason. I'll be working with him at home.

So to anyone wondering, I hope this helps explain!

What a big boy!

We had Dominic's 4 month check up appointment today, he is so big! He's in the 80th percentile for height at 26 inches & in the 50th percentile for weight at 14 lbs 15oz~ this is so big for us as Collin & Aidan were only in the 5-10th percentile for weight and 50th for height. He's doing great developmentally as well. This formula with Medium Chain Fat is making him a super baby, he's so strong, which is perfect for what he has, we want him to be as strong as possible!

The initial shock

I had written this shortly after we found out about Dominic

We had Dominic on April 3rd 2008. He seemed like a normal newborn for the first 2 days of his life. When we came home I noticed he did not eat very well, he also did not have the fast sucking motion that Aidan and Collin had. He also seemed extra sleepy, barely waking up to eat even.
On April 9th, we received a call from out pediatrician’s office – they said that a result from the newborn screening had come back abnormal, they did not tell me much about it other than we needed to contact Children’s Hospital immediately. Of coarse I was already crying and very concerned over this news. I then contacted Children’s Genetics Department and was put in contact with the Metabolic Geneticist there. The words they were using did not make sense to me, they said he tested abnormally high for VLCAD –


I was told we needed to come in first thing in the morning for additional testing. Also they told me I needed to feed Dominic every 2 hours. They asked how he was doing, I had let them know my concerns for the lethargy/very sleepy and not eating well. With that information they wanted to see us as soon as possible so we needed to get the the emergency room for monitoring and tests. We watched Dominic getting a catheter, getting poked all over because his veins were too tiny to get blood from for all the testing they needed to do. We had a long hard night with no sleep watching our newborn that we thought was healthy and we still even at the hospital had no answers. Finally in the morning the Specialist visited us and explained what he has. We also met with the Dietitian and were told that Dominic would have to eat every 2 hours through out the night for at least the first year of his life. I could no longer fully breast feed and had to now supplement with a special formula every other feeding.


For the first few weeks after we found out our new baby had this disorder we were both so upset and having a hard time dealing with it. We couldn’t believe that here we had had 2 healthy boys together and the whole time we had a 1 in 4 chance of having a child with this disorder. I think I cried almost every time I looked at Dominic for the first few weeks of his life. Jeff & I would talk about how bad we felt for him and that it really makes you look at life when something difficult comes your way. Granted this could have been much worse, this is something that with proper care is manageable and Dominic had a good chance of a healthy long life. I’m so thankful to God that he is okay and doing well.

Latest on Domino

We recently had a visit with Domino's metobolic dr., he's doing very well. They have now allowed me to go 4 hours at night for feeds so really he only needs to eat 1 time during the night since I do not go to bed until 12:30am. We start solids in 2.5 months, that will be the harder part as we will have to caculate every calorie and gram of fat. We'll have to keep a log of everything he eats, kind of hard to do with the chaoticness of life right now, is that a word?? As long as his fat levels are good he'll be allowed around 15% of calories from fat, this can change if I stop breast feeding but I don't want to quit until he's at least a year old. We're pretty happy about this precentage, it originally was going to be alot less than that. He's been great so far, he's a strong boy!

My Story

My name is Dominic Doering, I was born on 4/3/2008. In the first few days of my life like most newborns I was very sleepy. My Mommy & Daddy started noticing that I was barely waking for feedings and were going to let my pediatrician know for my first appointment the very next week. On the 6th day of my life they received a call from the Peditricians office, my newborn screening came back with an abnormal result for a rare genetic disorder Very long-chain acyl-CoA dehydrogenase deficiency or VLCAD and the Doctor's office said they need to contact Children's Hospital right away. They called and explained how I had been so sleepy and not eating well and they said we needed to go to the ER as soon as possible. My parents were very worried. I spent the next 24 hours being a very brave boy getting Iv's, lots of blood drawn for testing. Once I was on the IV I was a different baby, alert bright eyed, Mommy & Daddy had never seen me awake for more than a few minutes from birth. The next morning with Mommy & Daddy up all night trying to sleep on 2 chairs put together, they finally met the special Metobolic Genetisist Doctor that I will now see for a very long time. They confirmed that I do have VLCAD and that I will need to be on a special diet for the rest of my life combined with a MCT Medium chain triglycerides suppliment so that I can have the fat that my body can use since it cannot use Long Chain fat. I am missing the enzyme that breaks down fat using it as energy. So it is very important that I do not go with out food for very long and that I stay well hydrated. It's also important that if I get sick or cannot eat that I go to the hospital for an IV and to be watched until I am all better. I will have to eat low fat food and watch what I eat but Mommy will come up with some good recipes for me so that I can eat yummy things still. Thank you for visiting my page!

More on VLCAD http://ghr.nlm.nih.gov/condition=verylongchainacylcoenzymeadehydrogenasedeficiency