A Medical Update from CHOP

Aubree is still inpatient at CHOP. We are here over a week longer than we expected, but are just trying to keep good attitudes as we roll with the punches! We are here so much longer than expected because the interface (mask or nasal cannula) that delivers the cpap (pressurized air with additional oxygen as needed) Aubree has been using during nightime sleeping is a newer system, and only two home medical supply companies support and supply this for home use. One is in PA, and one is in CA. For Aubree to be discharged with cpap and an interface, a somewhat local home medical supply company has to supply and support it. Since PA and CA are not very close to AR, we had to go back to the drawing board for finding an interface that Aubree tolerated well and was widely supported for home use. The initial interface that we cannot find a home medical supply company for is called a ram cannula and is just a little bulkier than the nasal cannula that she already wears during the day. Not really a big change for her and she handled it like a champ. However, the other interfaces that are readily carried by home medical supply companies are much for invading and involve a nasal mask, large tubing, and headgear. We were worried about her tolerating this while sleeping because she had tried two of these types of interfaces while at AR Children's Hospital a couple of months ago. She did as well as could be expected for a baby to sleep in them, but they constantly shifted when she would roll over or move because these interfaces are just not intended for infants and are very difficult to fit. This meant mom and dad were getting up constantly to help situate the mask back on her nose and face correctly and soothing a baby back to sleep who was suddenly awakened by pressurized air blowing in her face from the mask. However, the last two nights Aubree has tried a pixi mask again and it fits much better now since she has grown. We are still having to get up a few times a night to readjust the mask, but not nearly like we did a couple months ago. The hope is to have a practice night here in the hospital on the cpap machine we will be taking home (required before they let you go home with it) and then be discharged tomorrow.  I have put a picture of Aubree wearing the pixi mask at the end of this post.  Please pray for me as I can get kind of frustrated with the seemingly extremely slow process of discharge. Others just don't seem to move with the sense of urgency I think they should sometimes :)

One benefit of still being inpatient is that Aubree was able to have an infant pulmonary function test. Someone had to cancel, and Aubree was able to have the open spot. This test involved Aubree being sedated, which was kind of scary for us since she has never been sedated before, but she handled the sedation just fine. The main goal of this test was to give us information as to Aubree's current pulmonary function as a baseline prior to surgery. She will be having additional pulmonary function tests annually to see how the VEPTR surgeries and expansions have changed her pulmonary function. As far as results from the pulmonary function testing, there were not suprises. Aubree has a substantially lower lung volume than would be expected for her age. They expect her lung volume to improve with surgery. Additionally, she cannot exhale the volume that would be expected for her age either. This is because her chest is very rigid, where a typical chest is flexible and can help push air out of the lungs. This will not improve with surgery. Nothing can really be done to change the flexibility of the chest wall, but the surgery should help improve her lung volume. I have put a couple of pictures of Aubree during the testing at the bottom of the post.

Concerning surgery, Aubree is still scheduled to have surgery on January 2nd to have the VEPTR device implanted on the right side of her chest. At this time, Aubree will also have a gtube placed. While Aubree is in a good place nutritionally right now, after surgery we know that she will feel absolutely rotten and not feel like eating the amount necessary to help her heal properly. She also has to continue to have good soft tissue coverage to cover her expanding chest and to cover the devices that will be implanted on the left side in about 3-4 months. This decision was made with lots of research and consultation with the medical team about what would be best for Aubree. Aubree will still be able to eat anything and everything she wants, but we can use the gtube to give Aubree supplemental nutrition. This would be like if she didn't finish a bottle or needed an extra feeding while asleep.  Please pray for this aspect of the surgery to go well and without complication. 

Also, Aubree did great while under and coming out of anesthia last week for her MRI and ct.  Praise the Lord!  Thanks so much for praying about this matter.  The images from the MRI and ct revealed that Aubree's spinal cord is not in danger of being damaged by her cervical spine.  Another praise!  Concerning her lungs, we found out that Aubree's right lung has about 2/3 of her total lung capacity and her left lung has about 1/3 of her total lung capicity.  In a typical person, the right lung has 55% of total lung capacity and the left lung has 45% of total lung capacity.  Her total lung is also substantially less than a typical child.  We could also see from the dynamic lung MRI that Aubree's chest cannot rise and fall like it would on a typical person.  Instead, her lungs expand mostly in her abdomen.  This is not ideal because her other major organs are there, and there is major competition for space.  However, Aubree's physiology is going to change so much with surgery, and we look forward to seeing her breathe easier!  Please pray for Aubree to get the most benefit possible from surgery and need less support.  Also, please begin praying for the medical team that will be caring for and operating on her.  Please pray for Derrick and I as well because it will be so very difficult to see Aubree in such a fragile and painful state after surgery.  As much as I have thought about it and asked questions to try and prepare myself, I know that I am not prepared to see my baby in such pain. I know that this is necessary, and we are confident in our decision for her to have this life-altering and potentially life-saving surgery for Aubree.  It is just a lot to process and deal with.  Below is a picture of an xray of another child who had VEPTR surgery for Jeunes syndrome.  This is very similar to what Aubree's chest will look like after both sides will be done. 

Thank you to everyone for all of the support and prayers.  They really mean such much to us!  Also, sorry for the delay in updating.  I had a post written 2 nights ago, and it got deleted right as I went to publish it.  I have just now gotten time again to update.  My next post will about fun things and updates about Aubree not dealing with medicine!