Final Makeover

Above two pictures: Living room, before and after

 

 

 

 

 Apparently I don't have any pictures of the dining room before, so here it is after.

 Above: Our bedroom, before and after. I love this color. It's called "Scandinavian Sky" and it's Olympic from Lowe's.

 Above: The toy room, before and after. I love this color too. It's called "Lettuce Alone" and it's Olympic from Lowe's. And no, my toy room doesn't usually look like this!

 The kitchen, before and after. Love the sink!

 The bathroom, before and after. Better? Absolutely. I'll show you the bathroom closet next time. It's a-maz-ing!

We love this house.

Best. Birth. Ever.

You have been patient. And now you are going to be rewarded for your patience. Here is how it all went down. Let's start on Tuesday, April 23rd. Matt and I are furiously trying to make our house look bee-yoo-tiful because our realtor is coming the next morning. We work and work and work. I am 39 wks, 3 days and in excruciating hip and nerve pain, but I keep pushing myself.

April 24th. Our realtor comes and we sign papers. She leaves, Matt goes to sleep and I notice some back pain. It's not necessarily pain, but tension, which I attribute to the amount of physical labor I put in the day before. I notice the back pain most of the afternoon, but only when I'm standing too much or in a weird position. After dinner, Matt leaves for work and the girls and I go back to Lowe's because I had left my wallet there the day before. When we get in the car, I begin to realize that the occasional back pain feels a lot more like back cramps and is coming every 20 minutes or so. It's uncomfortable enough that I dread the cramps a little, but that's it.

I continue to have these cramps all evening and at some point I think I realized they were contractions. After the girls go to asleep I clean up the house. I have enough foresight to think that if these contractions go somewhere, the house needs to be super clean so I put everything away and the house has never been so tidy. I also get on my hands and knees so scrub the floor (so if we show the house, the floors look nice). While I'm washing the floor, the contractions stop. After scrubbing the floor I decide to take a bath before bed. And again, the contractions stop while I'm lying in the tub. I have a few whenever I stand up, but when I'm not vertical, no contractions. I go to bed without contractions and decide it was false labor because I had been working so hard. That was at midnight Wednesday night.

Sometime while I'm sleeping the contractions start up again and I start waking up during the peaks. I am never coherent enough to realize they are contractions and always go right back to sleep. Until 3:45 am. In my dream I have to pull over the car for a contraction and then I woke up, right at the peak. I realize it's a contraction (still only in my back) and that I can't sleep through them.

I try lying quietly in my bed through them, but that's not comfortable. So I lean over a bunch of pillows, turn on my Hypnobirthing track and start timing. I started timing around 4am. I time for about 25 minutes. Contractions are at irregular intervals, for irregular lengths and easy to relax through. I go to the bathroom during this and there isno show or water breaking, so I still think I am a few hours from having a baby. At 4:30am I decide to call Matt. It takes a few minutes to get a hold of him, but he says he'll clock out and come home. Contractions are getting more intense, but not very painful and I can still talk through them. Between 4:30 and 4:45 (when Matt got home) I put away the laundry, call the babysitter and start making Emily's lunch. I go to the bathroom again and notice a little show.

Matt gets home around 4:45am and I start transition (I know this because I felt nauseous during contractions and could no longer talk through them, though I was still fairly relaxed). My hospital bag is ready and Matt starts grabbing a few things for himself. At 4:50am, I am standing by the couch. Since I am uncomfortable but still not in a lot of pain, I think we still have 20-30 minutes. Matt comes in and says that everything is in the van. I tell him to move the bag to the Jetta so the babysitter can use the van. He goes out to do that and comes back in. Tells me to get in the car. Our friend isn't here yet so I hesitate and while I'm trying to decide if we should just leave, knowing she is super close, another contraction starts. 4:50 am.

And then I feel Lydia descend. And I know that we're not going to make it.

I tell Matt (fairly calmly I might add) that she is descending and to  get things ready for a home birth. He says, "No, get in the car." I reiterate that she will be born at home and Matt says (also calmly), "Okay. What do we need?" I tell him to grab a towel, he runs to the laundry room (but I put it all away!), so then he runs upstairs and gets a towel. When he comes back down I tell him to go back upstairs because I am going to get in the bathtub.

I get upstairs, I strip down and get in the tub. Matt starts the shower, takes down the shower curtain and gets some more towels. Right as contraction 2 hits, I instinctively get on my hands and knees.

Contraction 2 hits and my water breaks. It is seriously stained and I notice it and worry about it. Matt asks if I pooped and I laugh, telling him my water broke. (Yes, I chuckled during a contraction. It STILL wasn't very painful and I was fully aware of my surroundings.) I feel her descending and start crowning. I think I grunt and reach down to support my perineum.

Contraction 3 hits. My body pushes once, she crowns and her head emerges. Matt comments that she's not breathing, but we know she's okay for a little longer as long as she's still attached to the cord. Matt asks me to move my hand as he cradles Lydia's head.

Contraction 4 hits. I push, hard, and Matt gently pulls her body out.

She is absolutely covered in meconium. She was born anterior (my first anterior baby!) and we think that's why she came so fast. Despite the speed of her labor, it barely hurt at all.

No. I am serious. The only thing that hurt was when she crowned. The contractions were never unmanageable. I barely felt any pain during contractions, just tension. I almost had a painless childbirth, something I didn't believe was possible. Because it was so painless I barely made any noise besides talking to Matt. I was also able to stay completely calm and rational. Very different from Charlotte's birth where I was pretty unaware of my surroundings. Lydia's birth was very quiet and calm.

Matt wraps her in a towel and hands her to me. I sit back and we admire our baby while she screams her head off!

The prettiest thing

Gosh, we just love this girl!

What do you know about fatty acid oxidization? (a really long post about Lydia)

Are you ready for a brief, but applicable, biology lesson? Great!

Lydia has a metabolic disorder called MCADD. It stands for Medium Chain acyl-CoA Dehydrogenase Deficiency, so everyone just calls it MCADD. Hold on to your seats and put on your 10th grade Bio caps while I explain what this means.

The biology part:

When you eat food, your body breaks it down into different parts for use. For energy use, one of the things your body breaks food down into is fatty-acid chains (f-a chains for our convenience). There are very long f-a chains, long f-a chains, medium f-a chains and short f-a chains. Then your body begins to break down these f-a chains, two f-a at a time in the mitochondria of each cell. This process of breaking it down two at a time is called beta oxidization. As your body progresses from very long to long to medium to short, different enzymes come into play. It's a very specialized process and each length has a different enzyme which is used to break the chains down. Each fatty acid chain will be broken down completely and used for energy, from complex carbs to proteins to simple sugars.

There is another compound (made of two amino acids) called carnitine that helps in this process. If the fatty acid chains are the semi-trailer, the carnitine is the semi-truck. The carnitine shuttles the f-a chains to the mitochondria and the appropriate enzymes. Carnitine is found in many natural foods we eat; your body also makes carnitine.

In Lydia's body, the enzyme which is able to break down medium length fatty acid chains is missing. It is a genetic mutation (I'll spare you that lesson) and every single cell is missing this. This means that after the body has broken down the very long and long fatty acid chains, the carnitine brings the now-medium length (8 to 12 fatty acids) to the mitochondria and there is no enzyme. The cell cannot break down these medium length. Her body can still break down short chains (or simple sugars) but cannot get short chains from medium chains. The carnitine cannot drop the medium chains until they have been oxidized and so as the medium chains aren't oxidized, the carnitine is still attached and can't transport simple sugars to and from cells.

Also remember one important thing that will later come into play: the liver is where simple sugars are stored.

What does this mean for Lydia?

 It's fairly simple. Since Lydia cannot break down medium chain fatty acids, she has limited sources of energy. First and foremost, because she can process long and very long chains, she has a slow-release source of energy. For example, she will be able to eat pasta and get energy from it, 8-10 hours later. She can also process short chains, so she will be able to eat a popsicle and get energy from it 30 minutes later. She can eat medium-chain food, but because she's missing that enzyme, she won't be getting energy from it 5-6 hours later. And for her, after her body processes the long chains, all she has to use for energy are short chains or whatever simple sugars here liver has stored. As an infant, her liver is small and can only store very small amounts of sugar.

Here's the applicable part:

As an infant this means that we have to feed no more than every 4 hours. Even if she starts sleeping through the night at 2 months, we still have to wake her up to eat. As she gets older and her liver matures (and can store more simple sugars in it) she will be able to go longer.

As she grows she will be able to tolerate longer times without food, with the max being about 12-15 hours for an adult. So no 24-hour fasting, crash diets, or protein-only diets. She doesn't have a restricted diet, but she shouldn't eat a lot of fatty or deep-fried foods since her body, quite literally, can't process it.

Another important part of this disorder:

Anytime Lydia's body gets stressed (such as an illness), she needs more energy. If she doesn't get that energy from long or very long chains, her body relies on the short chains. If she doesn't get the food energy she needs, her body will use up all of its simple sugar stores and then essentially go into a diabetic coma. Untreated cases lead to death. If she gets an illness, even something small like a fever or ear infection, we have to force her to eat-- even just things like popsicles. If she cannot maintain an adequate calorie intake for whatever reason, she has to go to the hospital and get IV glucose until she can maintain an adequate calorie intake. The IV glucose will provide her with enough short chains to get through the extra high needs of something like an illness.

Obviously as an infant, she is more sensitive to this and as an adult, she will be able to have a 12-hour bug without a problem. Mature livers can store a lot of sugar, but her liver is small and immature.

As a parent and as a family:

Right now our girls don't know anything is wrong with Lydia except that we've had to go to the hospital a few times for "shots" (blood draws to confirm the disorder) and that we had to go to Nationwide in Columbus and meet with a "special doctor" (geneticist) about Lydia. As they (and Lydia) get older, we will be talking with them about why she needs snacks before bed, why we're so worried when she gets sick and why we have a special letter for the hospital for her (emergency protocol letter). We'll also explain what they need to watch for and how important it is to tell us if Lydia exhibits certain symptoms.

As a parent, I'm grateful and scared. Scared of course, because of illnesses and not catching the symptoms in time. For example, last night Lydia has some diarrhea and threw up (definitely NOT spitting up, it was downright vomiting) once. Matt and I watched her and over the next hour she became more lethargic, hard to wake, uninterested in nursing. We didn't have any glucogel packets on hand (little packets of glucose gel you can buy at any pharmacy, usually diabetics use them), so Matt mixed up a highly concentrated sugar syrup and we fed it to her through a dropper until she started perking up and then I nursed her a little. Since she didn't throw up or have diarrhea again, we didn't take her to the ER. But I woke up every 2 hours to feed her and give her "sugar shots" as we call them and since she's still under the weather today, we are continuing that procedure until she returns back to normal eating patterns. I'm also nervous because we're moving and that means finding another pediatrician, getting referrals to another geneticist. And one of my biggest worries for her is that we will protect her while she is growing up, but that she will choose to not take care of herself when she's on her own someday.

Grateful, because newborn screening has dramatically cut down on MCADD crises and MCADD deaths. I'm grateful that our geneticist at Nationwide was on top of it and got us an appointment the next day after receiving her newborn screening results. I'm grateful that Matt's a nurse and that I understand science easily so that we both can fully understand what is going on with her. Our pediatrician, who was amazing before, has been extra amazing during this. I'm also grateful that there are children's hospitals within hours both of where we live now and where we are going to live in Georgia, and that the children's hospital in Atlanta actually has a Metabolic Clinic so we have that as a resource too.

I'm also especially grateful that this disorder is so easy to manage-- simply feed her regularly and watch her when she's sick. Of all the genetic disorders she could have gotten, this one really isn't bad. And I'm grateful for blessings because I know the Lord will help Matt and I as we navigate this. If there's anything that's been impressed to me since Charlotte's accident, it's that the Lord is in control and He is protecting our family.

Q and A

What is Matt going to grad school for and where?

Matt is going to become a nurse-practitioner. He got accepted into a school in Georgia. It should be quite the adventure for us, and I vacillate between excitement and knowing how much I'm going to miss Ohio. I'm a Midwest girl at heart, but I know there will be fun things to experience there too.

Are you going to share Lydia's birth story?

Yes. I am working on it, little by little. Right now I'm writing the long version for my journal/family history, but when that is done I will post a shorter version. Don't worry-- you'll like it!

Has anyone looked at your house/do you have any offers?

Yes and no. Matt and I were hoping for a miracle, but to be realistic, it will probably take awhile. Yes, this is difficult because I have to keep my house spotless...and I just had a baby. I know.

How are the other girls adjusting to Lydia?

As with any major change, they are all showing different responses to Lydia's arrival. The biggest response is that they all adore her. She is coveted and it can be difficult to keep them off her. Emily has been a little more defiant than usual. Nathalie has been more distracted than usual. Charlotte wakes up at least once a night crying for "Mama".

What is Lydia like as a baby?

Like all my other newborns! Right now she's still sleeping a lot, nursing almost as much as she sleeps (she can multitask and sleep and nurse at the same time). She's a good nurser, but kind of lazy with her latch. She loves to cuddle and be held. She loves to be swaddled. She does not like diaper changes or her carseat (at all-- we can't even drive to the store without screaming). Her cry is almost like an animal cry. It usually starts out as a mewling cry and she goes from 0 to 60 in about 2 seconds.

How do you do it with Matt's work schedule?

I've been incredibly lucky that Matt has only had to work one night since she was born. This weekend will be the first time that I will be on my own for more than 24 hours. And I'm nervous.