Day 35/36

Our first day at home was pretty crazy. Jeremy and I kept waking up worrying about her all night. I kept checking the monitor, wondering if she was breathing, thinking i wouldn’t wake up, afraid she was cold and scared that she would get unswaddled and cover up her face. Needless to say, not much sleep.

We had an appointment with the genetics clinic at Vandy yesterday. What I thought would be an hour turned into 3.5 hours. They needed blood for a lab that had to get done for the transplant list. After 4 different nurses, 7 different stick locations, a rubber band around her head to get the veins to pop and multiple sticks they finally got it. She screamed so much and it was so hard to watch but I knew we had to get the lab work done. She was so tired the rest of the day. She ate really well and slept great that night. Poor nugget.
Ammonia was in the 40’s, great news.
Nikki and Janie are still not sure what to think about her. They keep smelling of her head and trying to give her kisses. Apparently the metabolic formula smells pretty tasty to dogs and horrible to us.

We are trying to take in these moments and cherish them because we know she will grow so fast. Home is definitely different them the hospital and ill take a crying baby multiple times a night here over a sleeping one in the hospital.

I also lost a cousin today in an accident. He was life flighted to Vanderbilt and had severe head and skull Injuries. They did emergency surgery but he was pronounced brain dead and they ask the family to come say goodbyes. He was in his 20’s and its hard not to ask God why things like this happen. Prayers for my family please.

I just read my devotional after writing that last paragraph… Pretty amazing for what I’m feeling right now.

“The Peace that I give you transcends your intellect. When most of your mental energy goes into efforts to figure things out, you are unable to receive this glorious gift. I look into your mind and see thoughts spinning round and round: going nowhere, accomplishing nothing. All the while, My Peace hovers over you, searching for a place to land.

Be still in My Presence, inviting Me to control your thoughts. Let My Light soak into your mind and heart, until you are aglow with My very Being. This is the most effective way to receive My Peace.”

Ryman pics galore:

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Day 34: The 24 hour Blog

Part 1.
We will use this as a running 24 hour blog so keep checking in to see how our adventure unfolds. It has been a few days since we updated, we were busy changing rooms, getting the Broviac out, mixing formula, maintaining low ammonia, getting some help from Jeremy’s mom who flew up and our 5th wedding anniversary. Today is the big day, we are going home! Thank you God for answered prayers. Prayers for safe travels.

Ryman in her hospital gown.
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Daddy’s girl.

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Thanks to Jeremy’s mom we got to do something for our 5th Anniversary while she stayed with Ryman.

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The Little family, 5th wedding anniversary.

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Part 2.
We are officially out of the hospital! On our way to Georgetown to meet with the transplant team. First dress and First CAR ride!!

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Part 3.
Just left Georgetown! Parking was seriously $20 for 2 hours, hopefully we get a transplant parking pass. We got to tour the PICU and the rooms we will be in post transplant so we know what to expect. We totally changed Ryman’s diaper on their conference table, hope they are not eating lunch in there today.

Part 4.
So far Ryman has been a great traveler. Must get that from her dad. Slept for 4 hours. Stopped to feed and change her and now she is right back to sleep. She seemed very confused by her surroundings but she is enjoying the car.

A little over 400 miles to go.

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Diaper changing fun in the car.

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Where am I and where is my limo?

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Part 5.
Mini update to thank Mr. Matt Singleton for getting our tags and registration done before we left. That, along with Ryman, just saved us a ticket.

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Part 6.
Tennessee!!!! Wait, we are still 300 miles from home. Boo. What is this Texas?
Home State.

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Dad feeding Ryman at the stop. She had us a really nice diaper.

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Part 7.
About 100 miles from home!! I’m going to nap before we get there so I’ll have energy to love on some puppies! We are so excited to see them. We also can’t wait to carry our baby girl into the house and to her nursery. That room was pretty hard to walk into without her being there.
So close!!!
The girls!

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Part8, The end and the beginning.
We are home!!! Jeremy’s words: all I can say is 680 miles.
I can not even explain how great Ryman did today. She made the ride so much easier than it could have been. Plus having Patti to help us out at each stop was so amazing! We arrived home and our amazing friends/framily had set up balloons, flowers, a banner and stocked us up with some stuff. It was such a sweet surprise. The girls got to meet Ryman and were very curious. We are so so so so so… (times infinity) glad to be home, even if it is just for a short time. We love you all so much.

Thank you lord for getting us here.
We head to the genetics clinic tomorrow morning to meet with Dr. Morgan and get her ammonia checked. It was 27 this morning!

Home!

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1 Month

Happy 1 Month Ryman, we can’t believe you have already been here for one month.
We are so blessed to have this beautiful baby girl. She has definitely given us an exciting 30 days.

photo 2Happy 1 Month!

We finally got the results from Baylor yesterday that Ryman does in fact have CPS1 deficiency.
What does this mean? Well, the same thing that we have been planning for, a liver transplant is the only option. CPS1 is one of the most difficult urea cycle disorders to manage long-term due to the instability of ammonia levels. In order for Ryman to have CPS1 she had to have gotten one bad gene from me and one bad gene from Jeremy, or it could in an extremely unlikely scenario have been a freak mutation. They sat Jeremy and I down today to try to explain what mutations each of Ryman’s genes have. One of the genes has a mutation that has only ever been documented in one other person and the second gene has a mutation which has never been documented before. Yet another ‘Of Course’ moment. She is such a special girl :). Jeremy and I will have blood work done once we are back in Nashville to determine which mutation came from me and which mutation came from Jeremy. They also talked about what Ryman’s future would like as far as having children goes. No matter what she will pass along a bad (mutated) gene to her children. Therefore, if she were to have a child with someone else who has 1 bad gene then there would be a 50% chance that their child would have a urea cycle disorder. If she were to have children with someone who also had a urea cycle disorder, the doctors said they have seen this before – WHAT?), any children they had would definitely have a urea cycle disorder.

Ammonia level was in the 50’s today so the plan is to check it again tomorrow morning, give her Sunday off and then check it again on Monday morning. If all goes well we will take her to Georgetown on Tuesday to meet with the transplant team and we will head home after that appointment. We are meeting with them so Ryman can be listed on the transplant list on Thursday. They wanted to go ahead and list her so we can get the best possible liver for her, this gives them the ability to be really picky and find the perfect match.

Once we are back in Nashville we will be outpatients at the genetics clinic at Vanderbilt. Dr. Morgan spoke with Jeremy yesterday and made us feel much better about Ryman being back in Nashville. We are not sure how often we will start out testing her ammonia levels but we are confident that Dr. Morgan and the genetics team at Vandy are going to take great care of our little girl. They got her stable before we left for DC and have been in communication with Children’s National since we got here.

Ryman passed her car seat test and she even got to take a nap in it today. We are hoping she likes it as much as it seemed because our drive home Tuesday will be 11+ hours. Jeremy’s knuckles are going to be very sore!

We thank God tonight for our Ryman Ali. We are so proud to be her parents!

Lots of love, Brandy

Nap time.
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29 days

Can’t believe it’s almost been a month. To say life has been moving at a fast pace is an understatement. Can’t stress how much the support of family and friends has helped. Every day I’m honestly surprised and blow away by the generosity of y’all. Needs being met before we have to even ask have made us able to focus on Ryman. I don’t know if we could handle this if we had the added stress that a house, money, dogs, logging, etc bring.

Ryman is amazing. It’s really fun to get to experience all the things that being a (normal) parent bring. Yeah our first month isn’t normal and the next few won’t but to watch a personally form is pretty amazing. Yeah she cries and poops a lot but she also is starting to smile and make funny faces. I try to remind myself to soak up these moments. It’s going to be very easy to look back at this and hospitals, doctors, and traveling be our first memories.

The next week. Brandy mentioned yesterday that there is a chance of getting to travel back to Nashville soon. Basically we have come to realize that spikes in Ryman’s ammonia are just gonna be part of this process. The team at CNMC and Vandy have been in communication with each other and us. We know that when we get home even if it’s just for a few weeks that she’ll be okay. Please remind us of this if we act crazy. Cause we will… At least Brandy will. It’s looking like Tuesday the 28th is the day. Funny cause the 27th is our 5 year wedding anniversary. Wouldn’t that be an amazing anniversary gift. Getting to go home.

-Jeremy

Day 28

Ryman isn’t much of a fan of sleeping lots during the day. She was awake and looking around so much that one of the nurses got a mobile for her bed. She seemed to really like it. She is also not a fan of waiting for her bottle to warm up, neither am I. She has some strong little lungs. One of the nurses said that Ryman makes her presence known and she is queen of the hallway. Her ammonia was 50 at 4am, they haven’t tested again since then but she seemed good today. They were able to lower it by changing the amount of protein and the dosage of Buphenyl. This is really good because we wouldn’t need an Ammonul or an IV to bring the level down. Ryman had lots of attitude and ate so much today. She had around 115 cc’s at two feedings today. Trying to hurry up and gain that weight to get her new liver!

I pick Jeremy up from the airport at 10:30am, I can not wait. We miss him so much but we are glad he is still getting to work. It will be really nice when we can go back to Nashville with him. There are still some talks of it but I’m almost afraid to mention it because every time we do her ammonia level spikes. Taking her home is still a very scary thought but as each day goes by I start to have a little more faith that we will know if something is wrong.

Prayers for Jeremy and I, that we would be able to trust ourselves and our instincts to know if her levels are up.

We love you all!

“Jesus Calling”-
Remember that I am sovereign over your circumstances, and humble yourself under My mighty hand. Rejoice in what I am doing in your life, even though it is beyond your understanding.

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Day 27

My heart is heavy today for the families who have lost loved ones and children in the tornadoes. It is so sad to think of all the loss that happened in one storm.
I am praying for those who do not get to see their children grow. I know the scary feeling of not knowing whether your child will be okay and can’t imagine the feeling of actually knowing they are no longer with us in this life.

Jeremy flew out this morning and I am convinced that Ryman knows. Her ammonia was in the 60’s this morning and is now in the 160’s again. Same thing happened last time Jeremy left. She just really doesn’t want him to go :). Instead of putting her back in Ammonul immediately this time they are going to try something else. They are giving her an extra dose of Buphenyl and just feeding her the Pro-Phree (protein free formula) tonight to see if they can get it lower without putting her on the IV meds. They think the spike could be from the blood they had to give her yesterday. She was anemic and they tried to wait it out but they ended up having to give her blood. Her color looks way better but her ammonia does not. Blood has protein in it so she got more protein yesterday and they think that could have caused the spike. If the extra meds and protein free feedings do not lower the ammonia they will out her back in Ammonul tonight.

Ryman was wide awake all day yesterday and has some major personality. We enjoyed getting to hang out with her and have a few calm days. Jeremy will be back on Thursday and I hope those numbers are way lower by then.

We love you all!

Thanks Marcia for the devotional, it always says exactly what I need to hear.

Today’s “Jesus Calling” devotional:

I, the Creator of the universe, am with you and for you. What more could you need? When you feel some lack, it is because you are not connecting with Me at a deep level. I offer abundant Life; your part is to trust Me, refusing to worry about anything.

It is not so much adverse events that make you anxious as it is your thoughts about those events. Your mind engages in efforts to take control of a situation, to bring about the result you desire. Your thoughts close in on the problem like ravenous wolves. Determined to make things go your way, you forget that I am in charge of your life. The only remedy is to switch your focus from the problem to My Presence. Stop all your striving, and watch to see what I will do. I am the Lord!

What, then, shall we say in response to this? If God is for us, who can be against us? He who did not spare his own Son, but gave him up for us all—how will he not also, along with him, graciously give us all things?
—Romans 8:31–32

But as for me, I watch in hope for the Lord, I wait for God my Savior; my God will hear me.
—Micah 7:7

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Day 24

Nothing new today which isn’t a bad thing. God is granting us some days of quiet and calm. Jeremy is back and Ryman is trying to show off. She ate 80 cc’s at her 12pm feeding and 90 cc’s at her 3pm feeding. She is a hungry girl. They weighed her last night and she is up to 6 lbs 10oz. Over a pound more than her birth weight. A little over half way to the transplant goal weight. She and dad took a nap together today too. Couldn’t resist taking a picture of my two favorite people sleeping so peacefully.

It’s been 1 year today that my aunt Rhonda passed. So hard to think it’s been that long. I miss her so much but I know she is watching over Ryman tonight. Love you Aunt Rhonda.

John 16:33 I have said these things to you, that in me you may have peace. In the world you will have tribulation. But take heart; I have overcome the world.”

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