Friday, December 28, 2012

Christmas & Other Updates

We had a wonderful Christmas at Ronald McDonald House (RMH).  It was definately different than any Christmas we had before, but Christmas was going to be different no matter what because we have Aubree with us this year.  We enjoyed video chatting with our families and opening presents together.  Aubree really did great ripping the paper on the presents and pulling items out of her stocking.  She really loved the old timey telephone with the pull along string, a baby kitchen for sitters, and the baby snacks in her stocking.  She especially like the baby cookies and ate them like a pro!  She has also had many Ritz crackers since then and gets excited when she sees them now.  We also took shifts (so that someone is always with Aubree upstairs since she can't come down) eating a very nice catered Christmas breakfast and dinner here at RMH.  It was a very special treat to have the normal Christmas fixings being so far from home.  Also, we made a nativity scene out of play doh to have a representation of the real reason we celebrate Christmas, and Deb and I made a couple Christmas treats to enjoy on Christmas day. 

While Christmas was a fun time of making new memories, we have especially enjoyed just soaking up all the time we can with our sweet Aubree.  We have been playing with her new Christmas toys and some old favorites, as well as reading lots of books, and helping her jump on the bed.  Lots of fun, simple times together!  We are trying to get as much play and quality time in as possible since we know she will not feel like playing (and definitely not as hard as she does now) for who knows how long after surgery. 

Aubree will be readmitted to CHOP on December 31st.  New Year's Eve in Philly!  All pre-op things will be taken care of this day and on January 1st before she is to have surgery on January 2nd. We can hardly wrap our minds around what it will be like for our precious baby to have to undergo such a major surgery.  While it is hard to comprehend, we are so thankful that no matter what happens during and after surgery, that Aubree is in God's hands.  When Aubree was born, she was gray as a ghost, not crying (and did not cry the entire time we were in the delivery room while she was receiving assistance), and struggling immensely from a respiratory standpoint.  God spared our sweet girl then, when He didn't have to, and for that we are so thankful.  God has allowed more joy in our lives in the last almost eight months with Aubree than we could have ever anticipated.  I have told people that we were happy before Aubree; we just didn't know how much happier we could be!  We praise God for our precious gift and trust in Him regarding Aubree.  He loves her so much more than we can (which is hard to imagine because we love this little girl to pieces!) and wants the best for Aubree, whatever that might be.  Of course, our hope and prayer is for this surgery to be successful for providing Aubree with the best quality of life possible with increased lung capacity to meet her respiratory needs long term.  We want our daughter with us and healthy as long as possible!  Thank you so much for the all of the prayers lifted on behalf of us and Aubree and her medical team.  Please continue to cover her and us in prayer in the days and weeks to come.  The peace we have throughout this process is a testament to all of the prayers of so many and the grace of God! 

Will you also please continue to pray for Madden, the other baby here at CHOP with Jeunes syndrome?  He had his first VEPTR surgery this morning.  He is out of surgery and in the PICU where he will stay for the next few days until he can be moved to the pulmonary floor.  Please pray for fast and full recovery for him!   His progress can be followed under Team Madman on facebook.  Also, please pray for another Jeunes baby who was born the day before Aubree.  His name is Will, and he and his family will be traveling to CHOP in mid-January for a consulation with the Thoracic Insufficiency Team.  These appointments are so helpful and informative, but also come with a lot of information to process and sometimes decisions to make.  Will's mom Mary has a blog that she updates about Will and their family here

Thank you to those of you who have sent us cards and packages of encouragement while we are here.  They really brighten our day!  Please enjoy the many pictures capturing some of our moments here in Philadelphia. 

Monday, December 24, 2012

An Update from RMH & Aubree in the News

We are settling into our home away from home.  It is honestly so much better than being at the hospital.  I am genuinely so much happier outside of those teal colored walls, and I think Aubree and dad are, too.  While we are so thankful for Aubree to be seen and treated at CHOP by such a phenomenal group of medical professionals, we are cherishing the time we have to spend together as a family before Aubree has to be back in the hospital for quite a while for surgery.  Please continue to pray for peace for us in the days before surgery and after.  Also, please pray for Aubree to stay as well as possible throughout this process and for her to have a hedge of protection around her at all times.  We just want our sweet girl to be okay! 

Also, Aubree and her story have been in the news recently.  Last week, Larry Stroud from Derrick's hometown newspaper, the Batesville Daily Guard, interviewed us to write an article about Aubree.  He did a wonderful job relaying her journey and our hopes for Aubree.  There is not a free link to see the article (you must be a paid subscriber to see the article).  However, highlights from the article are attached to Aubree's picture on the Batesville Daily Gaurd facebook page.  Here is what it says:

Seven-month-old Aubree Elise Shaw will have her first Christmas far, far from home and facing a serious operation that hopefully will allow her to celebrate man...y more Christmas Days.

Aubree is the daughter of Derrick and Audra Shaw of Springdale and has an extremely rare genetic condition called Juenes Syndrome which affects the way a child's cartilage and bones develop.

“With this disorder, the main issue is that the rest of the body will grow at a relatively normal pace, but the rib cage grows very minimally if at all,” Audra said.

Simply put, the baby’s rib cage is much too small to allow her lungs to expand properly.

“She’s already requiring oxygen,” having been on oxygen since October, and also uses a continuous air pressure (C-pap) machine to force air into her lungs while sleeping, her mother said.

Aubree’s lungs function normally — they just do not have space to grow. Doctors say most babies with Juenes do not live past two years.

But things are looking up for Aubree since she arrived at Children’s Hospital of Philadelphia, known as CHOP for short, and the Shaws met the surgeon who invented a device called VEPTR — a vertical expandable prosthetic titanium rib.

“They’ll attach the VEPTR to the topmost rib and the bottommost rib. They’ll essentially break all the ribs in between,” said Audra. That will allow that side of the rib cage to expand to give Aubree more lung capacity.

An Aubree Shaw account has been set up at First Community Bank locations in the Batesville area in case any individuals, churchs or organizations wish to donate to help with the family’s expenses.

When she can, Audra puts new posts on to try to keep blog watchers informed.

And she has a request for readers: “Please pray for Aubree and our family.”
Larry Stroud also submitted this article to the Associated Press, which formed another brief of the original article.  Once it was picked up by the Associated Press, other news organizations picked up the story.  KATV in Little Rock aired the story twice, and Today's THV in Little Rock also aired the story.  While I appreciate Today's THV wanting to be considerate of our family, we would have had no problem with them sharing her name.  I can't seem to get the videos to upload here on the blog.  All I get is an error message.  Maybe I can figure something out on that later!

 Here is the link to the article on KATV's website.  The article is the same on the different news sites that have picked it up.  After googling Aubree Elise Shaw, we have found that many other news stations have also picked up the story.  While it is neat to see Aubree's adorable picture in a news article, we are more excited about her story being shared and recruiting the prayers of as many people as possible.  Also, with these articles comes awareness about Jeunes syndrome and all that it entails.  And, from my experience in teaching, I know that with awareness comes acceptance.  Our desire is for Aubree to be accepted for who she is, and part of who she is her having Jeunes syndrome.  Do not get me wrong; Aubree is sooo much more than Jeunes syndrome.  It is just something that we deal with that is more invasive and involved that other challenges some families might face. 

Again, we are overwhelmed by all of the love, prayers, and generousity that has been bestowed upon us during this journey.  Thank you all for loving us and our sweet girl and praying for us all along the way.  We love you all and cannot tell you how much your support means to us. 

Below are pictures of the article as it appeared in the newspaper, Aubree in her pink fluffy body suit, and Aubree tasting corn on the cob.  She loved sucking the juice out of the corn on the cob! 

Friday, December 21, 2012

Breaking Out of the Hospital & an Address for Ronald McDonald House

Aubree got discharged from the hospital
mid-day today, and we are so happy to be out of the hospital for a few days. We are settling in at the Ronald McDonald House. Derrick's parents had everything extra clean in preparation for Aubree and even had a Christmas tree set up. It is starting to feel a lot like Christmas since the temperature has dropped here in Philly. It is also quite windy!

Before we were discharged, Aubree got pictures with some of the great people who have helped us at CHOP. The picture with the ladies are "the play ladies," or child life specialist and play coordinator who were so sweet to have fun activities for Aubree. The younger doctor is Dr. Shah (pronounced like our last name) who was Aubree's resident doctor the while time she was inpatient. He was always so patient and great to answer all of our questions. The other picture is Aubree with Dr. Mayer, the pulmonologist. We have genuinely been so impressed with his level of expertise and care for Aubree. He came by to see her everyday to check on her and answer any questions we had, even though he was not the hospitalist the last two weeks.

Also, several people have asked for an address for us while we are here. Here is our address:
Ronald McDonald House
Attn.: Derrick Shaw
Room 16
3925 Chestnut Street,
Philadelphia, PA 19104

Thursday, December 20, 2012

Staying in Philly and Fun Times at CHOP

Because of Aubree's extended inpatient stay at CHOP, we will be unable to come home before she has her surgery on January 2nd.  Just the driving time from here to home is 20+ hours and that is without stops for food, sleeping, and generally baby needs.  It took us about 2 1/2 days to get here and it would take about the same to get home.  If we were to go home, we would hardly get to spend a day or two there before it was time for us to turn and come back to CHOP for Aubree to be admitted for pre-op on December 31st.  Also, if there was any kind of severe weather like snow or ice, it could potentially keep us from making it back in time for Aubree's surgery.  We cannot miss this surgery date!  While we would love to be home for Aubree's first Christmas and have her around the tree opening presents, we would much rather stay here for her to have surgery so that she will be around for many more Christmases to come!  We will be staying at the Ronald McDonald House here in Philadelphia until Aubree has to be admitted again.  Derrick's parents have been staying there during this time and say that the volunteers there are very nice and make it as homey and fun as possible.  Please pray for us as it is difficult being away from home for so long.  Anticipated recovery period in the hospital after surgery is 10 days to 2 weeks barring any complications, making it mid- to late Janauary by the time we are able to go home. 

While staying in the hospital is not ideal, there have been some fun experiences.  The pulmonary floor in the hospital has 2 wonderful child life specialists who try to make the hospital experience more fun for patients.  They brought in new toys and books for Aubree to play with.  They have also brought in activities for Aubree to do, such as finger (and feet!)painting.  She was covered from head to toe in paint and had so much fun.  It literally just dissolved off of her in the bath, so cleanup was a breeze!  They also brought in a sugar cookie in the shape of a  gingerbread man for her to decorate.  She tried to eat the cookie like a big girl and did have a few crumbs of cookie and some icing!  She loved it! Aubree also liked playing with the sprinkles!  The child life specialist also brought Aubree the cutest Build-A-Bear that was donated through Bears for Smiles which donates Build-A-Bears to kids at CHOP during Christmas time.  So sweet!

Another highlight of the trip was meeting Madden, who is Aubree's age and has Jeunes syndrome, and his mom Mandy.  Like I mentioned before, it is great to talk with a family on a very similar road since ours can be so different.  We also got the two babies together for a photo.  We normally do not expose Aubree to any outside people except medical professionals and close family, but made an exception because Madden is also on strict germ prevention for the same reasons as Aubree.  Neither one of them is sick and haven't been exposed to anything.  Also, they can both look back at these pictures and know that they are not the only ones going through surgeries or that have such a rare disorder.  And they are both so stinkin' cute, we had to get their picture together :)  

Again, we appreciate all of the love from all of our family and friends during this time.  It is comforting knowing we have such a great support system back home and in our hometowns.  Please continue to pray for us and especially Aubree.  She is truly our joy and still smiles and laughs through all of her time in the hospital.  This keeps us smiling and keeps us going! 

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! 

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.

Wednesday, December 12, 2012

At CHOP and Staying Busy

Aubree got admitted yesterday and so much has happened since then. Yesterday, they took some additional X-rays of her spine and chest. They also tried her with no oxygen at two different times while sleeping, but she did not maintain her oxygen saturation and her work of breathing increased, so we put it back on pretty quickly (after 30 seconds the first time and after 1 minute the second time). Her co2 was also monitored throughout the night and stayed within her normal level. We also met with the pulmonologist that works with the thoracic insufficiency team here.

Today, the drew blood for some labs, all which looked great from what we understand. We also met with the orthopedic surgeon and inventor of the VEPTR, Dr. Campbell, and discussed the VEPTR surgery. He said that Aubree had enough soft tissue coverage on her back to cover the VEPTR devices. This is a huge praise because we have worked and continue to work VERY hard to make sure that Aubree is eating enough and gaining weight appropriately. This is very hard for children with Jeunes syndrome because they are burning extra calories with the increased respiratory work they are doing. We were actually told she was the first Jeunes baby they had seen that is on the growth chart for weight!

While we are thrilled that he has enough soft tissue coverage for surgery, Dr. Campbell would still like to see even more on her for her would be growing chest cavity and for her to be in the best shape possible for recovery. We talked with him, a nutritionist, and a resident surgeon about the possibility of her having a gtube put in for supplemental feedings at night. Please pray for us and the doctors to make the best decisions regarding this. While this is not nearly as major of a surgery as the VEPTR surgery, it still has daily implications and potential risks in and of itself. We just want to make the very best decisions for our little girl!

Dr. Campbell also ordered another ct to see what her lung volume looks like now, a spine ct, cervical spine X-rays, and a dynamic lung MRI. The X-rays will be either today or tomorrow and the MRI and ct scans will be Friday. Please pray for Aubree concerning the ct and MRI because she will have to be under anesthesia for these since she is a baby and can't hold still for that long. There is added risk for Aubree because of her restrictive lung disease with her being put under and ventilated.

We met with the pulmonologist again today and are going to try Aubree on Cpap (air going in with pressure) tonight to see if it is more beneficial for her than just the low flow o2 she is on. We shall see!

Everyone has been so nice! Everyone has been so patient with any questions we have had. The child life specialist has also been so sweet to bring brand new toys for Aubree to play with to help her from getting bored in the hospital.

Lady and Papa have also been great to bring us food, play with Aubree, and wash bottles. We are so thankful they are here with us. And, a room opened up yesterday at the Ronald McDonald house for the grandparents to stay at and they even have a place to safely park the rv! Not an easy feat in such a big city with little parking, especially for a vehicle that size!

We have been very busy with the many consults and labs we have had the last two days. This picture is the only one I have had a chance to snap. She was tired from so much traveling and a big day yesterday! Enjoy our sleeping beauty and please keep us, especially Aubree, in your prayers!

Tuesday, December 11, 2012

Getting Close to Philadelphia

Thank you so much for your prayers for traveling graces yesterday! We hit some pretty heavy rain storms for a few hours and some very thick fog while on the mountains in West Virginia and Pennsylvania. Since we started yesterday in Ohio, we traveled through three states in one day!

We are now just a couple hours from CHOP and will check Aubree in today. Please pray for a smooth admission process and for a hedge of protection from Aubree catching anything while in the hospital. Also, please pray for an opening at Ronald McDonald House.

Below are so pictures of our sweet girl enjoying some sweet snuggles and playtime in the bed with Mommy and Daddy before going down for the night. She really has been a trooper! She also had her first taste of saltine cracker yesterday and loved it!

Monday, December 10, 2012

Still Going

Our first day of traveling went well. Aubree was a trooper, and Papa and Daddy did a good job driving. We hit some pretty intense rain but it didn't last too long. We slept east of Indianapolis last night and have already made it to Ohio this morning. We're making progress!

The temperature has dropped significantly since leaving home, but we are keeping Aubree warm! This sweet girl keeps is smiling! Thanks so much for all of your prayers and well wishes! Please continue to pray for us!

Sunday, December 9, 2012

On the Road

We have left for CHOP! It is about a 20 hour trip just for driving with no stops. So, we are breaking the trip into 3 legs with longer legs on Sunday and Monday, and a shorter leg on Tuesday when we are to arrive at CHOP.

We are so thankful to Lady and Papa (Derrick's parents) for taking us in their RV. Aubree can actually have room to get out and stretch during stops! They love Aubree so much, and it will be great to have them with us at CHOP as well. Some have asked why we aren't flying, and we aren't because we do not know what Aubree's oxygen needs would be at the high altitude. Additionally, putting her on a commercial flight with that much exposure to germs is way too big of a risk for Aubree as well.

A big thanks to Aunt Amber for coming and staying over the weekend to help get things ready for the trip and watch Aubree while we were working on packing! They both loved getting some good quality time together before the big trip!

Thanks again for the sweet words of encouragement and prayers! They mean so much! Please pray for safe travels and for everyone on the trip, particularly Aubree, to stay in good health. We will try to continue updating as we can!

The first picture is of Aubree in the RV on the way to CHOP. The others are Aubree opening her first Christmas gift, an early one from Aunt Ginger, Uncle Danny, and cousin Canaan. She loved the opening and the gift!

Friday, December 7, 2012

Going to CHOP

We just got the final call yesterday about taking Aubree to Children's Hospital of Philadelphia (CHOP).  We will be arriving at CHOP on Tuesday for Aubree to be inpatient for evaluation under pulmonary service to have all of her scans, films, and bloodwork done, as well as her consults with the four different doctors that will be on Aubree's service. They are thinking she will be there about a week, be discharged, and be back in a week for pre-op things.  Of course, all of this is just tentative and could be susceptible to change.  All of this is done with a tentative VEPTR surgery date of January 2nd.  Lots of things happening fast!  Things to pray specifically for would be for traveling graces, the doctors to make the best evaluations and decisions possible for Aubree, for us to make the best decisions regarding Aubree's care, and for the Great Physician to keep our girl healthy and strong throughout this whole process.  We will try to keep everyone updated as we know more!  Thank you all for the prayers and sweet words!

Monday, December 3, 2012

A Big Update on Aubree

We have so much to be thankful for! We are especially thankful for our sweet miracle, Aubree. When Aubree was born, she had respiratory complications that kept her in the NICU for 4 weeks. During her time there, the super attentive and skilled NICU doctors and staff noticed Aubree had a ribcage that was smaller than normal and long bones that were shorter than normal. After a few months of consults with a geneticist, skeletal X-ray evaluations, and genetic testing, we found out Aubree was born with an EXTREMELY rare genetic disorder called Jeune's syndrome, also called asphyxiating thoracic dystrophy. With this disorder, the main issue is that the rest of the body will grow at a relatively normal pace, but the ribcage grows very minimally if at all.  This is such a huge issue because, while her lungs are normal, they cannot grow beyond the container they are in (her ribcage).  So as she grows and her oxygen needs increase as they would with any child, her lungs are not growing to meet that need and are having to work much harder to meet her oxygen requirements.  There are other potential complications.  More can be read about Jeunes syndrome here

At Aubree's first geneticist appointment after finding out her diagnosis, we were told she had already reached 2 great milestones: she was born alive and we got to take her home. As far as we know, she is the only child living in Arkansas with this genetic disorder. The occurrence of this syndrome is about 1 in 130,000 with a mortality rate is 60-70% in infancy and childhood for children affected with this syndrome. Our sweet girl is truly a miracle from God! Because of her smaller than normal ribcage, Aubree's lungs cannot fully expand. This mean she doesn't have the lung reserve of a typical person, which is able to keep you still breathing normally even when you are sick with some type of respiratory infection. Because of this, any type of cold or flu is potentially lethal for Aubree, and we have to keep her isolated from germs as much as possible. Only home, the doctor, or outdoors for our little sweetie right now!

One of the only treatments available for helping expand the ribcage and lung volume for children with Jeunes syndrome is a major surgery of the implantation of a VEPTR (vertical expandable prosthetic titanium rib).  Basically, an expandable curtain rod type device is attached to an upper rib and a lower rib and the ribs in between those two are broken.  One side of the ribcage is done and then the other side is done a few months later.  The rods are expanded about twice a year in less invasive surgeries.  As the rods are continually expanded, the goal is for the broken areas to continue to grow new bone matter to gradually expand the ribcage over time, allowing the lungs to grow as the ribcage grows. These expansion surgeries will continue until the child reaches physical maturity and stops growing. 

Currently, Aubree is on a low flow of oxygen through a nasal cannula to help reduce her work of breathing (lower her elevated respiratory rate or breaths per minute, which in turn reduces her elevated heart rate).  Aubree has been on this oxygen for a little over a month and it has been so beneficial for her!  Her work of breathing has greatly decreased to closer to normal levels, and it has even helped her severe reflux practically disappear!  It doesn't inhibit her in any way, and she goes about her normal activities as happy as usual!  Concerning surgery for a VEPTR device implantation, we took Aubree for a consultation at Christus Santa Rosa Children's Hospital in San Antonio which sees children with this rare disease.  Since this is such major surgery, we are seeking a second opinion from Children's Hospital of Philadelphia where the inventor of the VEPTR system is currently practicing (he was previously at Christus Santa Rosa Children's Hospital where he invented and implanted the VEPTR system for the first time). 

Sorry for just now letting everyone know what is going on.  This was quite a shock to us as we had a normal pregnancy with no indication of Aubree having any genetic issues.  It took us awhile to understand what we were dealing with and to process all the things we were facing.  We know so many of you have been praying for us, and we appreciate it greatly!  Please continue to keep us and especially our sweet Aubree in your prayers! Pray specifically for Aubree to continue to stay healthy, continue to thrive and gain weight, for the doctors she sees to make the best evaluations possible, and for us to make the best possible decisions regarding Aubree's care and potential future interventions. 

We were completely unprepared for how much JOY Aubree has bought us!  She is the absolute light of our lives!  Seriously, she is amazing, and we praise God for blessing us with Aubree!  Please enjoy some pictures of our sweet daughter!