We meet with transplant and everything looks good with her lungs at this point. They did not do a biopsy this time, so again we still have no definite idea on rejection other than she is showing no symptoms, and her lungs look healthy. So unless she shows a decline, they don't plan to biopsy.
The transplant team is happy with her current growth and development. We know she is still small for a 3 1/2 year only weighing about 28 lbs. But as long as she is growing and making progress, everyone is happy. Developmentally she is doing really well, talking alot more, wanting to be more mobile and walking when you hold her hands or she holds on to furniture to get around. We are hoping to start working with a walker soon so that she can become more independent.
We did meet a new Cardiologist during this trip, one who has experience and conducts research in aortic root dilation patients. So we now have a better understanding of Elizabeth's current heart issues. We have known since her birth that she has aortic insufficiency, her aortic root is dilated, and she has a bicuspid valve. Once she was diagnosed with FLNA mutation, we learned that all of these issues, along with her lung problems, low muscle tone, and the brain changes are part of this condition. The cardiologist explained things more in depth and we have a better understanding of what the "plan" is for her future. Basically with the aortic root dilation Elizabeth's is currently measuring at 2.5 ( 2 1/2 times larger than what it should be) when/if it gets to 5 she will need to have surgery to have her aortic root replaced. They increased her heart medication to hopefully help slow the dilation down. She will have a cardiac MRI during our next trip in April, to compare to her last one in December 2012 to get a rate of dilation so we have a better idea of how fast this is progressing. We are hopefully that it is a slow progression and she has time to grow and get stronger before she needs this type of surgery, or be lucky enough to not need it. So overall, not the best news ever to have to think about. But we are SO thankful to have a new cardiologist who realizes the importance of monitoring her closely and ensuring that we are doing all we can for the best outcome with her cardiac issues. I have been so concerned about the lack of follow up and concern with our last cardiologist, I left this appt feeling so much better about her care now.
Her airway issues are still a bit concerning but we have decided, and transplant agrees with us that as long as she is showing no problems we will hold out on any surgical interventions for now. The risks just seem to great for a surgery that may not fix her airway. So for now, she will continue to use supplemental oxygen during sleep and will most likely always have a harder time during illness due to her airway issues.
|Sick on Halloween - No trick or treating but she still dressed up :)|
|She is ADDICTED to the Ipad now|
We want her to have normalcy and be able to go to school, play with other kids and enjoy this gift of life she has been given. But there is a very thin line that we have to walk with her. Every illness she has could damage her lungs, and lead to rejection. Some people don't realize how serious any illness is for her, and we try to educate everyone around us about her. So please, know that if I question you, or tend to tell you about it alot, its only to protect Elizabeth. We explained all of this again to her teachers at school and they have been doing a good job with making sure her hands are cleaned and we are notified if any of the other kids are sick. And our family/friends are always so great with staying away or letting us know if they have been sick. We appreciate everyone for all you do to help us make sure that Elizabeth stays as healthy as possible!
We hope all of you are staying healthy and can enjoy the upcoming holidays with your families. We hope to enjoy the holidays at home celebrating our Miracle Girl and soon the arrival of her little sister :)