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.