Sunday, December 16, 2012

Still at CHOP

(I actually wrote this post last night. Think accordingly as you read when I say terms like yesterday and such.)

We are still at CHOP. Aubree did great coming out of anesthesia. Praise the Lord! Thank you all so much for praying for this matter specifically.

From the images of her lungs and thoracic cavity on the ct and MRI, we got to visually see her lungs and how they are functioning on the inside. Because her ribcage is so small and rigid, it does not have much rise and fall with the lungs expanding like a typical person's would. Instead, almost all of the movement her lungs do is downward into her abdomen because that is the only place where there is room for them to go. This is obviously not ideal because there is major competition for space with the other major organs of her body there.

Additionally, we could see how the sizes of her lungs are not in correct proportion. Of a person's total lung volume, typical lung volume is 55% in the right lung and 45% in the left lung. Aubree has about 2/3 of her lung volume in the right lung and about 1/3 in the left lung, with both of them obviously with less lung capacity than a typical baby her age.

While these images show that her lungs are not able to fully expand, these images also showed that her lungs look healthy with no signs of aspirations or any other issues. Praise the Lord! Hopefully with surgery, these healthy lungs will expand once her ribcage is expanded and give Aubree more lung capacity.

These images also confirmed that her spinal cord is not in danger of being damaged from her cervical spine, which can be another complication with Jeunes syndrome. An answer to prayer!

The general surgeon also came by for a consult regarding a gtube for extra nutrition. He said that he did not think that would be appropriate for her because she is at a good weight with adequate amounts of soft tissue. He said if anything, she might need an ng tube (tube feeding through the nose that goes into the stomach) post-operatively if she struggles with eating enough because of being in pain or uncomfortable. This would be temporary and not surgical like the gtube. However, the team will come together and make a conclusive recommendation.

Upon advisement of the pulmonologist, we have also tried Aubree on a Cpap machine at night. This is a constant flow of air, in Aubree's case with supplemental oxygen added, delivered at a certain pressure through a nasal cannula. This pressure forces Aubree's lungs to expand somewhat more than they are able to do on their own. This allows her to take deeper breaths and blow off more co2 (Aubree co2 levels are sightly more elevated than normal). The hope is that by doing this, more lung tissue that is currently unable to be utilized will be "recruited" for use and continue to improve her co2 levels. Last night, we had a bit of a scare with Aubree spitting up/vomiting a large amount in the middle of the night. We believe that this was probably caused by some of the pressurized air getting into to Aubree's tummy. Please pray for this to not happen again because she could easily choke on this since she is asleep when this happens and has air from the cpap machine potentially forcing it down into her lungs. Also, our biggest obstacle in getting to go home right now is finding a medical supply company in Arkansas who will support and supply the particular nasal cannula Aubree has been using and doing well with while on Cpap. Please pray for this to hopefully get worked out so that we can take our baby home soon!

We are still looking at a January 2nd surgery date for the right side of Aubree's chest. The recovery time is usually 10 days to 2 weeks in the hospital with complete healing internally taking up to 3 months. The most common complication with this type of surgery is infection, which can be very serious. Please start praying now for Aubree to be infection-free post-op so that her body can focus on healing from such a major surgery and not also have to fight infection at the same time. We had the doctors show us where the incisions would be on Aubree and where the VEPTR devices would be implanted on her ribcage. Very large j-shaped incisions starting on her back between her spine and the edge of her ribcage, then going down several inches before curving around the ribcage. The device will also protrude from under her skin, making it's presence visible to the naked eye.

Of course the mama in me worries about her one day being self-conscious about the evidence of her surgeries or someone making fun of her or hurting her feelings about this. However, dad and I know we set the tone about how she deals with all of this. She will always know that this is something that helps her to be able to breathe better, just like glasses help some people see better and hearing aids help some people hear better. The teacher in me thinks I might even write a children's book about it someday!

Along with all the medical stuff going on, there have also been some highlights during our time at CHOP that do not revolve around medicine! We actually found out through another family who has a son with Jeunes syndrome about CHOP doing VEPTR surgery much earlier, allowing for more lung growth, than the other hospital where we had Aubree evaluated. Madden is just a few weeks younger than Aubree, and he and his mom Mandy have been at CHOP either inpatient or outpatient since mid-October as they await his upcoming VEPTR surgery date on December 28th. We actually got to meet them, another family on a similar path as us! It was so great to finally meet in person after much messaging back and forth over the last couple of months. Please pray for this family and baby Madden as mom and son are here at CHOP and the rest of the family is back in south Florida, visiting when they are able. What strength this family must have! Also, please pray for his surgery to be free of complications.

Our sweet girl has stayed so happy through all of this and has been doing new things. Today, she gave kisses for the first time! She gave mommy 3 open mouth kisses on the cheek. So precious! I am not surprised she picked up on this because she gets kisses all day long! She has also tasted one goldfish cracker and tried saltine crackers a couple more times, as well as apple slices. We mostly just let her suck on them, which she thinks is fabulous! She is really enjoying the crackers and tries to actually shove the whole thing in her mouth and starts chewing. I have had to swipe cracker out of her mouth a few times because I am terrified of her choking on it, but have given her a few minuscule pieces of cracker mush that she seems to enjoy, even though she would much rather have the whole cracker. Aubree's favorite baby food is definitely sweet potatoes and she cannot slurp them down fast enough! We tried turkey and broth stage one baby food, but she acted like I gave her poison. Needless to say, she was not a fan. We tried beef and broth baby food today, and she liked the flavor of it, but gagged once she tried to swallow the beef bits, not just the broth. She realized quickly she couldn't just suck it down like sweet potatoes!

Sorry for such a long post, I have just now gather time to give an update and there was lots to share. Please continue to pray for us to make the best decisions possible with the recommendations that the doctors have for us. Also pray for our endurance as we do not get much sleep in the hospital and for us to remain positive as we are here longer than originally told or anticipated with no firm discharge date as of yet. I equate discharge from the hospital to the last day for seniors in high school, very much anticipated. However, imagine if that date kept getting pushed back further and further with no idea when the last day would be. Frustrating to feel like we are spinning our wheels at times, but know that all of this will be worth it for our little girl.


  1. Such extraordinary news. God is so good. Praying for Aubree to be infection free and healthy. She seems like the sweetest thing. Can't wait to meet her. Love to all of you
    Scott and Leah Wright

  2. Thank you for the update on that sweet baby girl! Praying for her discharge and upcoming surgery and for all of you! I am happy you met the other Jeune's baby too. I will pray for him too! Hugs to all of you. Donna Frederick