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

       

Always an adventure at CHOP

I don't know if I mentioned that last time we came to Children's hospital of Philadelphia (CHOP), we were only there to see Dr. Campbell in Orthopedics, but Ava got Pneumonia and had to be admitted for several days. Well, yesterday she came to CHOP for an MRI and it was going to be a fairly simple matter, but things got crazy really fast. They had planned to give her a mask of Oxygen while she was under anesthesia but her breathing was worrying the anesthesiologist and he intubated her (breathing tube in the airway). She has been intubated before, but somehow this time it was worse and as soon as she woke up she started gagging, coughing up blood and turning blue. They were concerned about this so they admitted her to the pulmonology department for overnight observation. Ava has been having alot of these dusky/gagging episodes lately especially when in the carseat, but the intubation really enhanced it. The doctors are all concerned but think that the spine/rib surgery will help her. It was a good thing for the doctors to witness theses episodes because it made them take things more seriously and hopefully move things along faster. The issue is just when and what are they going to do. They think alot of it is that she struggles to breathe anyway because of a limited lung capacity, so when anything makes her have to work harder (crying hard or throwing up), she gets dusky and looses oxygen. It's quite a predicament. Supposedly all the doctors got together and talked about Ava on Friday, so we are anxiously waiting for their call on Monday to hear what the plan is. CHOP is a great hospital and we are glad to be near enough to go there.

A cute little video of Ava when she is feeling much better!

What we are up to

So much has happened since the last post about Ava. In December she went through the whole jaw distraction process which took about 2 months from start to finish but she was only in the hospital for 5 days. They taught us how to extend her jaw by turning these little screws for 11 days, then she had to have consolidation bars put in and we waited for about 7 weeks for her jaw to set. In february they removed the bars and cut her tongue free from her lip where they had previously sewn it. It was difficult to say the least, but now that part of our lives is over. Thank goodness! Unfortunately in the middle of all the craziness of the jaw surgery, we found out that Ava had scoliosis. They couldn't tell us the extent of it at first because of everything that was going on with her other issues, so we had to wait. That was hard. Eventually we met with Shriners (which is an orthopedic specialty hospital) and they said she also had kyphosis which is a forward hunch, and a concave rib cage on her right side. They said with her complexity of airway/breathing issues, they didn't think that they could help her but that a doctor at Primary Children's might be able to. We had to wait again for an appointment with him, and he told us about a specialized procedure called Veptr, where they attach titanium ribs to her ribs to straighten out the spine. He said that she was too young to know exactly what was needed and that we should come back in 3 months. So we waited again...but in the mean time, Ava stopped eating half of what she needed to gain weight. For the next 3 months our focus shifted to her eating, which was one of the worst experiences to date. She began loosing weight and would just not eat much. We tried everything! So many feeding specialists, different GI doctors, a barium swallow test where they had to force barium down her to see it through an x-ray, we tried different bottles, I stopped eating dairy, we tried 5 different reflux medications, feeding her all day long, and we even tried a nasal feeding tube. We didn't have alot of success and Ava stayed around 12 lbs for 4 months. In April Ava ended up getting the G-tube surgery (tube in her belly) so we could supplement her feedings that way. We were scared at first, but it has been a huge relief and she is gaining weight now. We ended up going back to the orthopedic doctor at Primary children's and he said her spine didn't look good. He said the kyphosis is pretty severe and that her rib cage is also too small for her body. He said that there are things that can be done but that it is complicated especially with her being as young as she is. He thought it would be a good idea for us to get an second opinion at Childrens Hospital of Philadelphia with the doctor who pioneered VEPTR, so we scheduled an appointment with him and waited agian. We felt good about coming to Maryland for the summer, so that is where we have been since the beginning of May (Morgan got an internship in D.C. which we will tell you about later). We went to the appointment in Philadelphia and found out that we needed to come back for further testing to determine the best course of action for her surgery. So that is where we are now! We had to wait a whole month for these tests, but now we have done genetic testing, met with a pulmonologist, and are planning to do a Dynamic Lung MRI on Tuesday. That test is going to be very extensive and give the doctors the best idea of exactly what is going on with Ava and what she really needs. She has to go under anesthesia for that which is always a little scary, but we are excited to get the results so we can move on to surgery. We also have to coordinate a cleft palate repair this summer at Johns Hopkins which is tricky because we really don't know when they will be able to do the spine surgery. Anyway, it's been a busy time for us, but we are doing alright considering it all.