Saturday, August 13, 2016

If Only...

If only it were just the heart defect...If only. I find myself thinking that a lot these days. I know it's not a healthy train of thought but it's impossible to prevent it from seeping into my head when I least expect it.
There are a lot of "if only's" in the last 8 months, but none of them matter now. Because it's not just Avelyn;s heart that is sick, it's her kidneys too (and lungs to some degree). Recently we have been confronting the high probability that we will soon have to sacrifice Avelyn's kidneys in order to try to make her heart as healthy as possible and in turn, the best kidney transplant candidate as possible.
It's all very complex but basically, she needs heart cathertizations to balloon or stent her distal pulmonary arteries. Proximally her arteries look a lot better. Both have grown significantly (which was unlikely) without interventions but out closer to the lung fields, she is still believed to have narrowings. These narrowings cause the right side of her heart to need to pump harder to get blood through the small vessels into her lungs to be oxygenated.
It is believed that since growth is so rapid in the first couple years of life, that now is the best time to intervene with these narrowings. If they can be dilated now, the arteries will have a better chance of growing with her and not occluding over time.
So how does ballooning her pa's affect her kidneys?
Well to do the heart cath's the doctors will have to inject her with dye to be able to see her vasculature and find the places where intervention is needed. This dye is nephrotoxic, meaning it is damaging to the kidneys. Normal kidneys can filter out the dye, but Avelyn won't be able to with her decreased renal function. Her kidneys will worsen, possibly considerably, with each cardiac cath. She will likely need to be completely sustained by peritoneal dialysis, something we were hoping she could come off of.
While none of this is new information, it's being provided and insisted upon from a different perspective by Avelyn's providers. The providers seem to believe that if left alone her heart, while ok for now, will not be able to sustain optimal function needed to be a kidney transplant candidate as time passes. They also believe that if left alone, we'll miss the optimal window for cardiac intervention and she may not be able to achieve as good of results later, and will still nearly certainly need a kidney transplant at some point prior to high school. So the school of thought is to get her as healthy as possible from a cardiac perspective to be able to best handle the transplanted kidney when the time comes.
So why not go ahead with a transplant asap?
Well the above mentioned heart cath's for one. She'll need multiple of these in the first few years of life and the dye could jeopardize the transplanted kidney's function too. Also, she'll need another open heart surgery in a few years to upsize her artificial pulmonary conduit. The one she has won't get her into puberty. The conduit replacement will require a pump run (heart lung machine) which can also damage the kidneys (actually, this is how Avelyn's kidneys were damaged in the first place). So in order to give her the best shot, she'll likely need to have already undergone the conduit replacement prior to transplant.
So where does this leave us?
Taking all of this into account leaves Avelyn in a very precarious position. She'll need to be fully sustained on dialysis until the optimal time for transplant. That could be 2-3 years. That's more terrifying than the transplant process itself. While sustained by dialysis, her growth, blood pressure, bone development, hormones, electrolyte balances and a lot of other things will be affected.
Also finding a match for her transplant could prove impossible. Sean and I aren't matches, though there are organ swapping programs out there that may be an option. While we have loving and absolutely amazingly generous family members who have offered, a lot goes into being a kidney donor and a match, so even those willing to donate may not be able to.
Lastly the transplant road is not an easy one. She will suffer from a compromised immune system her entire life. She will be in constant danger of rejection. She will need very likely need multiple transplants over her life time and cadaver kidneys have the longest wait time, often exceeding 5 years. Realistically, Avelyn won't make it 5 years, so a living donor is likely her only option and it's not guaranteed.
I guess that's been Avelyn's life story up until this point. Nothing has ever been guaranteed and yet she fights on. As long as there's still hope, we'll continue to fight along side her. 



  1. Thank you for sharing Somer. Earnest Hemmingway once said a few things that I think apply to Avie, "His talent was as natural as the pattern that was made by the dust on a butterfly's wings. At one time he understood it no more than the butterfly did and he did not know when it was brushed or marred" & "Courage is grace under pressure." Most folks called E.H. Papa. Well said Papa.
    Love her. Love you. Love Sean,
    Avie's Papa

  2. Thank you baby girl, for going into such graphic detail, for those like me, who just need to see it in writing to understand it. My heart's desire is to see Avie functioning as a "normal" baby-as normal as her normal will be suits me fine. I love her! I love you and Sean! I want a lifetime of happy family memories to be shared with our Avie. If she needs one of my kidneys, there's no hesitation in saying she's got it. I don't care about the hoops I have to jump through to prepare my body to sustain a part for hers. Just please let me help. The physical pain could never be as bad as the emotional pain of watching you 3 from the sideline. I NEED to be able to help her. This may be the miracle I've been begging God for...none of us knows what tomorrow brings, but Dear Father please guide us down this road. Give us eyes to See YOU, ears to Hear YOU, and a heart to FOLLOW YOU LORD. Thank you for the Blessing of our Avie ❤️��

  3. You all are the bravest!! I am believing and seeing her as a well baby girl and young lady. I too am MORE than willing to be tested as a live donor. I love you all so very much and so grateful you have been so willing to share these very intimate details of your lives. Mucho kisses and hugs <3 <3 <3

    1. Thanks Aunt Wilma, that's very kind ❤️❤️ Love you!