Monday, May 16, 2016

5 Years with Angel Lungs

Wow, it has definitely been a while since I updated, which for the most part is a good thing. It means we Elizabeth has been happy and healthy.  And I don't have alot of  "free" time to blog with two busy little girls :)  This post is pretty much a recap of the last year and a report from her recent 5 year post lung transplant check-up. 5 YEARS - for those of you who have been on this blog from the beginning know that 5 years was a super scary number based on the statistics we were given at transplant. The past 5 years have been filled with fears, anxiety, loss of friends we have meet along this transplant journey and the constant question of what does the future hold. But it is also been 5 years filled with memories, love, laughter, and overwhelming pride in seeing our little girl do things we never thought she would be able to do.

           Some days it doesn't feel like its been 5 years, but at the same time the routine of daily medications, checking oxygen levels, making food for the feeding pump, ordering supplies, making dr appts, dealing with insurance is just "another day" here. I worried we would be overwhelmed with the care that Elizabeth needed but you just learn to adapt and it becomes part of our daily life.


 Elizabeth has had a pretty good year and has enjoyed being a full time student :) I can't believe her first year of Kindergarten is almost over! I am SO thankful for the teachers and principal at her school, they made this scary transition so much easier. Elizabeth has really taken to one of her teachers, Ms. Tracie, and I am so thankful for her and how much she cares for our little bug. It helps me to know that she has people who care about her so much, and understand our concerns.

Elizabeth overall had a pretty decent school year with attendance and illnesses. She did have a pretty big break in school right before Christmas until the beginning of March. She had a stomach virus right before Christmas, added her already horrible GI system she was was out of school unable to keep any food and losing alot of weight until the beginning of March. We finally have a plan for giving a medication 10 days a month to help with her motility. And she will be having testing done in July at Cincinnati to see if we can figure out exactly what the issue is and a better way to deal with it. I am very thankful for the new GI doctor that she is being seen by at Cinncinati, he understands the connection between all her belly issues and the Filamin A mutation. Even before transplant she has always had these issues, and it just seemed like the different doctors wanted to just treat the symptoms and not look for the underlying issue. I hate that its taken this long to finally have a "plan" but so far she is doing well on the medications and we will hope for an even better plan after the testing in July.

 In April we went to Cincinnati for her 5 year check up. During her check up she had a chest CT, xray, bloodwork, DEXA scan, cardiac MRI and bronchoscopy done. Overall her lungs looked good, and the team is happy with where she is. The main concern that came out of her trip was the issue with the aortic root dilation that we have been monitoring since after transplant. Elizabeth has been on medication since she was 2 to try and slow the progression of the dilation, but it is now at a point where the cardiologist feels that intervention needs to be considered. So we will be planning for Elizabeth to have surgery in June in Cincinnati to replace part of her aortic root. This is a very scary decision for us to make, to put her through a major cardiac surgery. Without this intervention there is a possibility that her aorta could dissect which would most likely be fatal. By intervening now and replacing the part of the aorta, we will hopefully prevent any future issues. As with any cardiac surgery there are major risks, and add in being a immune suppressed kid with a rare genetic condition and the list gets a bit longer. Matt and I both feel that the timing is right for this surgery, and I just pray that everything goes well and she is back to her happy self quickly. We were told to expect 7-10 day hospital stay after surgery. She will be sedated and on the ventilator for the first 24 hours or so to rest and then should be able to be extubated but will most likely be on some pain medications.

   When we first started this journey, we were told its like a roller coaster ride, and that has definitely been the most descriptive way to to explain everything about the past 5 years. As we head for another loop in this ride, I remind myself of all that Elizabeth has endured over the past 5 years. It breaks my heart that I can't fix this or take it away. But I also know that she is so strong, and has fought so hard in the past there is no doubt in my mind that she will continue to do so through this and I have faith that she will wake up asking for her ipad!
    Thank you for your continued prayers and support, most of you who will read this listen to me vent and stress all the time and I know I don't say it often enough, but I appreciate your support, love and understanding. And most of all I appreciate your love and prayers for Elizabeth!