What a difference VEPTR makes

Before and After

Here are Ava's X-rays. Before surgery, a lot of things were scrunched in a small space and are now more opened up. It also really helped the scoliosis and her ribs aren't so close together. When she gets her second Veptr on the other side it should help even more. She is doing well at home, but it is a little hard to get used to the guidelines they gave us for her holding and moving her. We aren't supposed to let her crawl or put any pressure on her arms for another month. That will be tough since she started doing those things before surgery. Tomorrow Morgan and I are going to take off her bandages for good and give her a bath, so I'm a bit nervous about that. 

Home at last

After being gone for 3 weeks, we are finally home! I am so tired so will post more tomorrow. We are all doing well and made it home safe and sound. Time to get our lives back in order.

New Baby

Day 12

Ava has been a little energizer bunny. For some reason she decided she doesn't need to sleep anymore. Morgan and I are loosing our minds a little bit. I think it's the combination of being stuck at the hospital, attached to machines, etc. But, they are talking about letting Ava go home tomorrow! They are pretty sure she won't need BiPAP for home. They say it's not uncommon to need a little help with BiPAP after the VEPTR because there can be some areas of collapse in the lungs, causing the lungs to not efficiently expel the carbon dioxide. Her levels have gone back to normal which is so great. I can't tell you how cute Ava has been in the last few days, but she is like a new baby in some ways. For one thing, she is taller because her spine is more straight, but she is also more smiley and alive in general. She also doesn't seem to be struggling to breathe as much. When she cries and gets really upset, she doesn't turn dusky. I can't believe it. It's amazing.

Go to Sleep!

Day 11

Ava woke up at 5:30 this morning and  didn't go to sleep until 10:30 p.m. (17 hours) .We tried everything all day and she was just wired. They say it is withdrawal. She didn't have BiPAP all day as a test run to see how she would do without it. She did great. She was so cute all day, but had to be held the whole time, because she would throw a fit. They told us to keep her pretty calm for her test run, so crying it out wasn't really an option. That was challenging. We will see what the Docs say tomorrow. She may be going home pretty soon! Goodnight.

Even Better

Day 10

Today was the first day Ava smiled and laughed. She was much more herself today. She got to have 3 breaks from the BiPAP  and loved every minute of it. She still has to have it, but her carbon dioxide levels have gone down to a very good level. We are so grateful for that. We will have to see if she can keep her levels down without the mask, if not than she will have to have it at night. She was a lot more active today(compared to laying around in bed) She sat up quite a bit, and even stood up when leaning against me. We have to be careful not to push her to hard because she is still very weak and shaky. Her pain seems to be pretty much under control now. She is still on tylenol and one other medication, but she hasn't had to have morphine since thursday. She might be having a little withdrawal from it, but nothing too extreme. We are planning on posting her x-rays soon, to show how much the VEPTR has changed her. It will take around 6 weeks for her to get back to normal, but in the mean time she will have to get her cleft palate repaired. Poor girl. So many surgeries in such a short time! One day she will catch up, but she is doing so well developmentally for all of the setbacks. She was talking a lot more tonight and we were all having a grand time. I'm so glad things are looking up.

Better

Day 9

It's been a better day. Ava is still on BiPAP, but has resigned herself to it. She is not happy about it, but not fighting it either. The carbon dioxide levels continue to come down and they gave her a break (called sprinting) for 1 hour today. She really seemed to love the break and her oxygen was fine without it, but they put it back on because she needs it to lower her carbon dioxide levels. My parents came back up and brought our guitar, so we have been enjoying playing for her. It really humanizes the hospital room.  She was very cuddly when she got her mask off for that hour, and it was great to hold her. The BiPAP is a good thing because it puts pressure into her lungs and helps expand them, but we hope she won't need it permanently. If she did, she would need it only at night. Morgan and I are doing fine. I think we are keeping our sanity fairly intact.

Our little Scuba Diver

Day 8

Okay,  so she is not happy in this picture, but the good news is that she can sit up by herself! That is a big milestone after surgery. Unfortunately she has to wear this bulky mask for who knows how long. She has been doing better today and her carbon dioxide levels have gone down, but they are still not where they should be. Ava isn't quite herself today. For a large part of the day she was awake but in a daze. There was one point where I was drawing next to her and I showed her the picture and she grabbed my colored pencil and hit it against the paper making a lot of marks. It was very cute and I was proud. Ava doesn't get the best sleep because people are always coming and going in the PICU, but we try to make her comfortable. Morgan and I were saying today what a stupor our minds get into when we hang out in this hospital all day. The days all run together and we always seem busy but there really aren't that many different things we do. Mainly standing by Ava's bedside helping her to calm, or taking care of business. We found out from Doctor Campbell that we need to come back in 3-4 months for the second VEPTR, so that's a little sooner than we thought, and right in the middle of the semester. Thinking about real life is hard right now. School is very important, but not as important as this. It's a great balancing act indeed.

Not crazy about BiPAP

Day 7

All day long I felt like crying. In fact I did. I am tired of all this! Can I say that? Well I am. I have had it. No more, thank you very much. Ava has had a rough day, the nurses were very unfeeling and unhelpful, and more problems keep popping up. I will spare the details, but will just say that Ava is having an even harder time getting oxygen than before her surgery. They say it might be because of pain and post surgery, but it could be longer term. They have put her on bipap because her carbon dioxide levels are high, but she really doesn't like it. We are doing what is best for her, but I still can't help feel like we are torturing her in some way. Irrational as that may be. I dream of the day when our blogs won't be so heavy. I know I need to be more optimistic. I know it's just not helpful to focus on the negative. Where your focus is, determines your reality. It determines your whole experience. There is just so much pain pulling me back to the negative, so it's very hard. Let me try to be positive for a moment and see what it does: I have a wonderful husband who helps me with everything (even washing my pumps), I have a beautiful daughter who is so pure and loving, we were able to get this surgery done from the man who invented it, we have good insurance, we have so many friends and family who care and have been very supportive, the Ronald Mcdonald house lets us stay here for practically free (and it is in a beautiful old mansion), Morgan and I have our health( we never realized what that meant until now), we have everything we need and more, and we know what the purpose of life is. As hard as that is to accept. I have really struggled feeling close to Heavenly Father for a long time, and with all of this pain we have experienced with Ava, I sometimes feel like I don't know what anything really is. Like everything is nothing, if that makes any sense, But, in my more true moments, the moments I really come to myself, I know there is something much bigger than me and even bigger than all pain. I would be ungrateful to not say that. Heavenly Father is doing more for our family than we know. But, it is intensely difficult to move forward and we will keep going. We don't have another option. I just let my thoughts go. It felt good. Goodnight.

It's been a long day

Day 6

Today was a mixture of good and bad. Ava is doing very well, but has her moments; boy does she have her moments. She gave us quite a scare today. At some point after we tried to sit her up and put her back down, a lot of loose congestion from intubation came up and she started gagging. She started turning dusky and quickly turned blue and her oxygen rapidly went down from 100 to 9. Her little body turned limp and she almost passed out. It was the worst I have ever seen her. Our nurse was there, but was a little frozen about the whole thing, so I ran into the halls yelling HELP! A lot of nurses came and they got some good oxygen going and suctioned her mouth. She recovered but was very traumatized. We were all traumatized. They also changed Ava's dressings on her back for the first time and so we got to see what her back looks like since surgery. It's not pretty, but the wounds are healing very well and have no signs of infection. Her kyphosis (hunch back) looks so much better! It's not completely where it needs to be, but is very improved from this single surgery. We are so glad about that. She still has this bizarre rash all over her body from the antibiotic they gave her after surgery and her liver levels are still high, but they have gone down since yesterday. The main concern with this reaction is that she may not tolerate many antibiotics in the future. She has had them before, but they say this could be the first sign of her body building a hypersensitivity to them. On a good note, we got to hold her today! She seemed very happy to be held and was being so darn cute. She has started making ma-ma sounds. I love that of course. I think she will have a better day tomorrow because she had some really good moments today.

Big Baby! She really has gotten taller from the surgery. It's not uncommon for her to be playing with the oxygen.

Papa and Ava. He loves his little doll.

Moving Forward

Day 5

We go through this stuff way too fast

Today was pretty much the same as yesterday, except that we were informed this morning that Ava had developed Hepatitis. It's always something new. However, it is not as bad as it sounds. Apparently, hepatitis simply means inflammation of the liver, it's the cause of the inflammation that makes it serious. In Ava's case, she was just reacting badly to some medication or rash (no one knows for sure), but she is not contagious and it should go away in the next few days with the help of a steroid and Vitamin K. She was up a little more today, and her personality continues to come out to the delight of all around. Today she waved at the nurse and started making cute little vocal sounds. She also was playing very roughly with her air tube. We were able to go out with Lindsay's parents to see Eclipse and to eat at Bobby's Burger Palace (The famous Bobby Flay of Food Network) and it was awesome! Being a burger connoisseur myself I can honestly say it was the best burger I have ever had, and the milkshakes were very impressive.

Another day

Day 4

I had a picture of Ava to post but thought better of it. In the photo she looked in pretty sad shape; a swollen/puffy body covered in a weird rash, squinty eyes, and a big foam pad taped to her back. Not to mention about 20 wires and tubes flowing out from her chaotically. All in all she is doing better but it is hard to see because she is sleeping most of the day still (heavily medicated of course). One cute thing was earlier she woke up for about 15 minutes and interacted with us. Her eyes are almost swollen shut but she was waiving at us and clapping, even though her little fingers were swollen like sausages. It's kind of weird to have Ava so zonked out. We miss her and her delightful personality, but I am all for the sleeping as opposed to her feeling the pain. She has been having a hard time keeping her food down, so they are giving her very minimal amounts for now. Tomorrow is another day. Here are some pictures of the hospital, I think it is beautiful.

The main atrium 

Looking out of our window

Sleep

Day 3

For the most part, Ava has been sleeping the whole day. She had a blood transfusion today and that seemed to help her. She was so pale and has a little coloring back. The nurses are pretty impressed with Ava. They say she is a superstar because there is such a strain on her body right now and she is compensating for most of the deficiencies completely on her own without any extra support. She breathes harder and faster to do this, but she does it and the nurses are very amazed. We are glad she is sleeping, it is better for her not to be awake during so much pain. A little earlier she woke up and her mouth was so dry, so we gave her water soaked on little sponge lollipops and she loved it.

Rough Day

Day 2

Sleeping after a Hard Morning

Well, last night went alright. Ava was waking up for short lengths of time throughout the night, but going back to sleep rather quickly after some minor fussing. However, this morning things got a lot worse. She started moaning and crying out in pain and it was obvious that she was suffering a lot. She was breathing very hard and fast and was not able to fall asleep because of the pain. They upped her dosages of morphine a couple of times and started another pain killer. They also gave her a nasal cannula with high flow oxygen to try and assist her breathing so she wouldn't have to work so hard. Both solutions seemed to help and she fell asleep for the rest of the day. The first couple of days after surgery are always the worst. Overall she seems to be recovering alright.

After the Storm

Day 1

Everything went extremely well today. We feel so grateful. We have no doubt that there was divine intervention, and that all of the prayers and fasting and love from everyone has helped this miracle to be. They only placed one of the VEPTRs instead of two, because the time that she was under anesthesia was getting to be too long for her little body. She also didn't have a lot of muscle and skin on the one side of her ribs to work with, so Dr. Campbell wants to put it in later when she has a little more meat on her. He didn't seem too concerned about this for now and said that we definitely have the hardest part out of the way. He actually was able to expand the rib cage today, which allows for more lung expansion, but said that we have to wait for the lungs to grow and the muscles to strengthen before we will notice a lot of change. So many things can go wrong with this procedure, but none of them did, so we are very fortunate. She has been in a lot of pain and discomfort in the moments when she wakes up, but the medication is helping somewhat. The hardest part is that we can't pick her up because of the soreness in her back, so it's overwhelming to watch her suffer and not be able to comfort her in the way she is used to. The first few days are rough, but hopefully she will be feeling better soon. She may even be ready to go home in a week, but we will see. We can't believe how supportive everyone has been. Thank you. We love you all.

Waiting Room

Right now we are in the waiting room. We got here at 7:15 this morning and she is in the OR, but they are still trying to get a central line in her. When they get that taken care of, they will start the surgery which will take about 5-6 hours. So we will be here for quite a while. We walked to the hospital this morning from the Ronald Mcdonald house and the weather was beautiful. That is our favorite way to get to the hospital because we walk through the University of Pennsylvania campus pretty much the whole way here. The campus has very old buildings and walkways covered with trees. Ava was very happy. She was making a lot of cute sounds and pleasantly looking around at everything. Last night Grandma and Grandpa Smith drove here and Grandma Linda has been here since Monday, so Ava was more excited than we have ever seen her. She had 5 adults standing around her clapping and cheering her on as she performed her little tricks and sounds and baby things. It was pretty much a We love you Ava party. I have never seen her so happy. She was squealing so loud and laughing and smiling from ear to ear. It was a great night and I felt a lot of peace about today.

Ava's New Friend

Ava waiving at Ronald McDonald (HD)

And so begins VEPTR

Looking to the future

Ava in her Pack n Play at Ronald McDonald House in Philly

July 8th. That's the day Ava gets VEPTR. It will probably be the most intense surgery out of a series of surgeries over the next 14 years of her life. Dr. Campbell called last week to let us know that a day opened up that he could do this surgery. It was a miracle because they have been booked for quite some time and there was no assurance that we could get it any time soon. He said he was concerned about her based on the last time he saw her and wanted to move more quickly. They are going to attach vertical titanium ribs to both sides of her rib cage in hopes that it will straighten out the spine and give her more lung capacity. These ribs will be expanded every 4-6 months until her spine is done growing, so we are committed. Let me just say, Ava is a real trooper. She goes along with everything and still continues to smile. We on the other hand, are the ones that can barely stand it. We had no idea at the beginning that what we thought was just "scoliosis", could be so life threatening. It is wonderful to us that there is something that can be done. We are going to update our blog regularly during her stay at CHOP, so stay tuned until then.

We wanted to show pictures of her back pre-surgery

Cute Pixie

A more extreme view of her back and chest