Emerson Sloan:: 10 Months

Mercy Sakes.  10 Months.

These months are sure flying by.

This month, 

We walk.

We dance.

We shake our head "no".

We clap.

We 'talk' and laugh and scream!

WE HAVE FUN!

Emerson Sloan, you win our hearts over a little more each day.

Such a joy.  Such a little love.

We had our 9 month VLCAD Check Up last week.

Her CPK (Muscle Enzymes) were 219.  Normal is 29-169.

Her Liver Enzymes were:

ALT: 30- normal is 5-33

AST- 67- normal is 20-67

All good news from Genetics and Nutrition.

We continue to feed MCT ProCal in pureed foods.  Typically I do this at breakfast and dinner.

She gets 1 packet a day.  Roughly 6 teaspoons.

She loves to eat.  She prefers finger feeding to pureed but she'll eat puree if she's hungry enough.

And if you're quick enough.

We are still nursing.  Praise the Lord.

8am-12pm-4pm-8pm and once in the middle of the night anywhere between 3-5am.

Dare I say it, sleep has finally become routine.  

She's an early riser just like her brother, but I can take that with only on 'get up' a night.

Naps? well...  They happen everywhere but her bed.

But it allows for flexibility so we just roll with it.

She adores her brother.

She lights up when Daddy comes home. 

I can't believe in 2 months, she will be 1.

I guess the birthday party planning shall begin.

I'm thinking something soft and sweet.  Simple.  Peach & gold to match her nursery.

We'll see how it all plays out.

Happy 10 Months Emerson Sloan!

WE LOVE YOU!

Emerson Sloan:: 9 months

Emerson, You are a busy little thing.

Currently at my feet playing with a tiny plastic jar from Easton's cooking drawer.

Standing up. Taking up to 8 steps. 1 tooth. 

Lots of changes in your development this month.

Full of personality.

Funny little thing.

Quite the temper.

You're going to be the little girl with dirt all over her and her hair a disheveled mess at all times.

Sleeping better at night.  Going to bed by 8.  Eating around 3 or 4.  Sleeping till 6:30 or 7. 

Far better than what it had been.

Last night was a doozy but I'll chalk that one up to the Dr. Pepper I had at lunch and one too many Chocolate Oatmeal No Bake Cookies throughout the day yesterday.

You love to eat.  You prefer finger foods over puree.

But we have to do puree food to put MCT Procal in.

We started that about 2 weeks ago and you didn't even notice.

That is 3 tsp, 3 times a day in with your food.

This is for your VLCAD, which Praise the Lord hasn't been an issue thus far.

You are still nursing.  It's become more of a wrestling match than a peaceful 15 minute time to sit down for momma.  I'm finding that if I stretch your nursings from 4 hours to closer to 4 1/2 this helps.

You still don't nap in your bed.  I'm not sure you ever will.

I attempt it at least once a week, but you fight it and eventually win.

You love Easton.  Y'all play so well together.  He loves you so very much!

You're a strong little girl Emerson.

Physically and mentally.

A true joy to us all!

 

9 Months Emmy Boo!

We love you Sister!

6 Month VLCADD Check Up

We went for Emerson's 6 month VLCADD check up recently.

First we met with the nutritionist, then geneticist, then cardiologist, followed by blood work.

Nutrition and Genetics were pretty routine.  The real information comes from Cardiology and Blood work.

We had an EKG and an EchoCardiogram and both showed a healthy heart.  Praise the Lord.

The blood work came back and showed some elevation.

They measure 2 levels each time we go.  Muscle Enzymes (CPK) and Liver Enzymes (ALT).

Her CPK levels were 193 this time.  Normal is 29-168.  However a 'sick' child can get as high as 2000-5000.  So her levels are not alarming but they are up.  Her first check in November was 269, and down to 87 in January at her next check.  So, the fact that they've doubled in 4 months is concerning, but they are still not super high.

Her ALT levels were 36.  Normal is 5-30.  A sick child's levels would be between 360-500.  Her last check in January was 28.  So they were in the normal range then, but are now up a bit as well.

The cardiologist did say that they could be elevated because of her recent ear infection but it's hard to tell.  Her glucose was perfect and her heart was perfect, so he was pleased with that.

The results were then sent to our geneticist, who then relayed some concoction suggestion to nutrition.  She called me with the recomendation.

The geneticist didn't leave me with a good feeling about the future of our breast feedings, but after speaking with the nutritionist, I do feel a bit more comfortable in their suggestions.

There are a few 'options' of getting MCT oil into the body for VLCADDers.

There is MCT oil, that apparently you put IN foods.  There's MCT Procal- which I believe is a powdered formula, but is for children over a year old.  Then there's Liquigen, which is a liquid product to add into food/drink.  And lastly, Lipistart, which is a formula, that they drink.  I believe this is the most common way most children get the medium chain fats needed.

The suggestion was to give Lipistart before I nurse her each time.  Just an ounce or two before each nursing, would add in a lot of MCT to her diet and offer her a lot of protection.  However, with the addition of foods, she will gradually nurse less, thus taking in less of the long chain fats that are in my milk.  Our Cardiologist was fine with adding in MCT of some form into her baby food and continue nursing.  However, genetics wanted to add the formula, making her nurse less.

After speaking with her nutritionist I expressed my desire to try the MCT in her food first.  Either way they will recheck her levels in 6 weeks to see if they are going down.  In 6 weeks if they are not down, I will agree to the Lipistart.  But I don't want to decrease my milk supply if its not absolutely necessary.

This is such a frustrating process.  I don't feel like our 3 specialist are on the same page, or even communicating.  I really like the Cardiologist (who is leaving Vandy) and I like the nutritionist.  I don't have an opinion just yet about Genetics.  He's very 'to the point'.  Which is fine, but I also need a little compassion and explanation.  Our original Geneticist left Vandy so we've started over with a new one.  He's probably perfectly fine, but I was used to our other doctor.

I felt a little defeated after this conversation.  We had originally left the appointments feeling like we could continue on the same course as we were on, with the addition of foods to her diet, but then to have it changed, to adding in formula- nursing less.  It was what I didn't want.

Perhaps it's because for 6 months she's really been no different than a 'normal' child, other than making sure she eats every 3-4 hours for the first 6 months, and now 4-5 hours.

Now, with the suggestion of adding MCT into her diet, and possibly putting her on Lipistart, I feel like she becomes one of them.  I feel like it makes her have VLCADD a little more.  I don't know.  I have such mixed emotions.  Of course, I want to do whats absolutely best for her, but I also feel like no one really knows what would happen if I just continued to nurse her, for the first year and added the MCT in her food.  She's not going to nurse more now than she did when she was a newborn, so there will be a decrease in the very long chain fats that she gets.  That has to work in her favor.  In our favor.

This disease is so rare, and no two children are the same.  And there are adults with VLCADD who didn't know they had it until later on in life, and survived without any MCT, or fasting.  No, I don't want to buck the system, but there are just so many grey areas with this disease to me.  Perhaps it's because I don't know enough about it, perhaps it's really because it is grey.  Either way, I'm still learning.

I, by no means, know all there is to know about this disease, but it is my desire as her mother, to be her advocate.  To ask all there is to ask.  To understand the why behind the suggestions.  To decide, ultimately, if we will follow their leading.  I pray we will always make the right decisions when it comes to her health.  Selfishly, I do wish to nurse her at least the first year, but if that's not what's best for her then I pray I can gracefully bow out if need be.  I'm trying not to let my mind go there yet, and praying that we can continue nursing.

We should hear back from the Nutritionist in a week or so to determine which route Genetics wants to take.  Either way, I'm thankful for a healthy, happy, thriving, milestone reaching, strong willed little girl.

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!

Emerson:: 3 Months

Emmy Boo:: Emmy Loo:: Apple Dumplin'

3 Months Old

Diapers: Target's Size 2

Clothes: most are 3-6 months

Nurses: around the clock

7-10-1-4-7-10{{ish}} (2-3 times at night as well)

Mommys Diet Limitations: No milk, yogurt, etc. 

Attempted cheese on some sandwiches and it caused spit up & fussiness.

Not worth it.

Very limited caffeine.

Sleeps: in our room, beside me, in a bassinet

Thinking about trying the pack-in-play soon.

Naps: on the go, in her bed (minimal), in her swing

Smiling, talking, cooing, laughing.

Found her hands.

Sits in the Bumbo seat and can hold her head pretty well.

Still not a fan of tummy time for too long.

We're working on that.

We have a Cardiologist & Geneticist appointment this week.

Anxious to see what they have to add, if anything.

We've found a Facebook group for parents of children with VLCAD and it's very helpful.

Also, made a new friend on IG, with a son with VLCAD!

What a blessing!!!

Life with 2 is busy, but good.

Hope you all have had a wonderful Sunday!

VLCAD:: abnormal newborn screening

Now that we have some peace of mind and clarity, I'll update on what we've been going through. 

Emerson had an abnormal newborn screening.

They called us from the pediatricians office the Tuesday after she was born and sent us straight to Vanderbilt.

After a series of blood draws and heel pricks, it was confirmed that Emerson has VLCAD.

VLCAD was fatal prior to newborn screenings but as the geneticist has reminded us, 'we know that she has this and we know how to treat it.'

VLCAD means for Emerson that she cannot use the fats that normal people use.  Her body cannot process very long chain fats.  Where our bodies turn fat into energy, hers cannot use the very long chain fats.  There are a few different levels of this disease, hers being the mild form.  Praise Jesus.

We are still learning about this very rare disease.  The first thing they want to make sure of, is that her heart it healthy.  We had an EKG a few weeks back that was normal and an Echo cardiogram on Friday and the results came back from that today.  Results from that were all normal as well.  Praise the Lord!!!! 

With VLCAD, if her body gets too many very long chain fats, her heart has to work extra hard to try to process them.  Thus enlarging her heart.  If in this echo, or any future echo's, would have shown any sort of enlargement of her heart we will supplement breast milk with formula- a formula containing the medium chain fats only.  Breast milk is very high in fat.  After speaking with the geneticist Friday, and understanding more about what that 'supplementing' would look like, we have decided to continue breastfeeding until the echocardiogram tells us to do differently.  I don't feel it's best at this time to stop breast feeding 'just in case'.  It's a bond I treasure and wish to continue as long as possible.  We will closely monitor her heart for the first 6 months of her life to ensure there is no enlargement.  After that we will begin her on a heart healthy diet with an addition of MCT oil to provide her those medium chain fats.  MCTs are a fat source for patients who cannot tolerate other types of fats.

The second thing they routinely monitor is her CPK enzymes.  This would show any muscle break down.  That blood test was performed on Friday as well and CPK enzymes are all normal.

The danger with VLCAD comes in times of fasting or times of sickness.  If Emerson were to get the flu or a stomach virus or something else that causes her to not eat we would go directly to the hospital.  Her geneticist would then request an iv of sugar water to keep her blood sugar up until she got over the virus.  To me, it sounds like it all starts with her blood sugar.  If we keep that in check in times of sickness, we are on the safe side.  We are all now on high alert for germs and sickness, even more so than before. I don't think God could have given her to a more perfect family as far as germ awareness goes.  If you know me at all, you know that I am a major freak when it comes to germs and sickness.  So, if I ask you if you are healthy if you're coming for a visit or to wash your hands

For now, times of fasting, for Emerson means anything over 4 hours.  So she MUST eat every 3-4 hours around the clock for at least the next year.  At that point we will evaluate how her body responds to no food for certain periods of time and go from there.  So, don't ask me if my child is sleeping through the night yet, cause, that's a no. 

We now have a geneticist, a cardiologist and soon we will have a nutritionist to help with her diet (and ours).  If she's going to be on restriction, we all are.  We could all stand to be on a heart-healthy diet.  I never could have imagined having a team of doctors looking over my child's health but I am thankful for the amazing children's hospital that we have close by and for this group that knows about this disease. 

This is all I know for now.  At least all I feel somewhat comfortable having an educated discussion about.  There are many things we don't yet know.  What her life will look like, only time will tell. Her doctor did say that he expects her quality of life to be excellent and as a mother, you can't ask for more.

Yes, this diagnosis is heartbreaking.  Yes, this could cause some 'disruption to our lives' in the future.  But I am so very thankful for newborn screening and that we KNOW this about Emerson and we know how to care for her the very best we can.

The doctor told us best case scenario would be that we caught this early enough and that hopefully all we will have to do is monitor her through her life but she'd never show any symptoms or complications. 

We are believing in best case scenario.  We ask that you pray for that for her as well. 

 

God has really been working on me lately.  My faith.  Sitting still.  Believing.  But that's for another post.