VLCAD Update

Today was spent in and out of doctors offices.

We had an appointment first with our Geneticist, Dr. Morgan at Vanderbilt Children's.  This appointment was fairly routine.  They checked her weight (15.8 lbs) and height was at 25 inches.  She's in the 98 and 94th percentile for those.  They commented 'she must get held a lot because she has a perfect head shape.'  Yes, indeed she does.

We had a few questions to go over with the geneticist. He confirmed that, for now, it was fine to continue exclusively breastfeeding, pending any unforeseen problems with cardiology.  I had read that MCT oil was good for VLCAD patients but he said her body is handling my breast milk fine and that MCT was not needed at this time.  I've also read a lot about the benefits of coconut oil and that it does make its way through the breast milk. Coconut oil is a medium chain fat (MCT) so if I have it in my diet she gets it in my milk.  He agreed that I should continue with the coconut oil and a healthy well balanced diet for her.

We talked about letting her cry at nap time.  He said it's ok for her to cry.  That babies were ok to cry. We'll see if I can let her cry or not.  I doubt it, but sister has got to learn to sleep in her bed.  She is a good napping partner and she does nap in the sling, but I'd like to have some time for her in her bed.

We talked about skin biopsy for VLCAD.  I had read about some patients having that but he said this wasn't necessary because her diagnosis was already confirmed.  You would have a skin biopsy if the diagnosis was uncertain.

He ordered a CPK test.  This tests the enzyme levels in muscles.  With VLCAD, this can be elevated when the body is under stress.  If her CPK levels were elevated we would discontinue breastfeeding as she would not need the high fat of my milk, and would need the medium chain fats in a special formula.  She has had this test in the past and levels were normal.

We then headed to the Cardiologist, whom we met with for the first time.  Dr. Exil.  He was fabulous.  Heavy on the accent so you had to pay close attention.  First we had her vitals checked, blood pressure, oxygen, and a EKG.  All came back normal and healthy.  Praise the Lord.  After meeting with Dr. Exil, he reassured us that his hope for Emerson was that she lead a long healthy life.  The important thing is feeding her often and keeping her healthy.

He explained VLCAD in a more understandable way.  Folks like you and I, our body stores fat.  Emerson's body does not.  So, daily, she pulls from my breast milk, what she needs.  And this is fine for her for short periods of time.  However, in the event she would get sick and not eat, miss a feeding, etc, her body doesn't have stored up resources to use while she's not taking in any nutrients.  Her body uses glucose, stored in the liver as glycogen for energy.  If there is nothing going in, there is nothing for her to use.  Therefore her sugar would drop.  Which would be bad news for her.  So, if she were to get sick, and not eat, miss 1 feeding, we would go straight to the ER.  A normal cold isn't just a normal cold for Emerson.  Thus the germ freak amplified.

We were given an emergency protocol letter on this visit.  Something to give to the ER should we not be close to our Children's Hospital.  Peace of mind on paper I suppose.  Being that most doctors and nurses have no idea what this is, it list specific instructions what to do for Emerson immediately.

He complimented me on how well she was doing.  Complimented me on what a fantastic job I was doing with nursing.  Reiterated to Lance exactly how hard it is to be a nursing mom and encouraged him to take the night time feedings so I could rest.  I told him I'd bake him cookies every Friday!  We'll see if this happens as it will take planning on my end, to pump in the mornings so he'll have milk in the middle of the night.  It sounds good though!  I haven't had more than 3 hours of sleep at a time since November 23rd.

Once we were finished meeting with Dr. Exil we headed back to Genetics to get the blood work done.  After a failed attempt and a very sad Emerson, we left with an unsuccessful CPK test.  Lance called it quits when Emerson and I were both in tears and the nurser was no where near close to getting her blood.  We'll try it again next time, but I'm NOT looking forward to that.

I feel good about this visit.  Again, we are just so very thankful for newborn screening and the fact that we KNOW that she has VLCAD.  It's not knowing when things can get dangerous.  As Emerson gets older, we will keep her on a low fat diet and give her MCT oil so her body can store those medium chain fats.  We will be hooked up with a dietician within the next few months.  We are still learning, and have so much left to learn.  But I feel like we are on the right path.

We will return to Genetics & Cardiology in 3 months, where will we have another Echocardiogram and discuss introducing foods.

For today, and the days to come we will continue to enjoy this little girl and the smiles and joy that she brings, and try our hardest to not worry about the 'what if's' of this disease.

Thank you all for your continued prayers and support!  Each prayer is felt!