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!