I cannot calculate the times in Annie’s life when a doctor has sat kindly but stiffly across from me to tell me bad news. Starting with the day of her diagnosis while she grew wrongly in my womb to her most recent cardiology checkup last week, the news about Annie’s broken heart, delicate body, and uncertain future feels mostly like a haunting echo on repeat.
“She’s sick. This isn’t good. We don’t have a crystal ball but the days ahead are numbered.” While I hear the haunting echo, I watch a happy little girl growing up. Begging for a baby to love. Laughing with her sister. Asking questions about chapter books. Sending cheerful letters, obnoxiously splattered in Valentine’s stickers and smiley faces, to distant friends. She’s old enough now to begin to understand but she still doesn’t know it.
There’s a beauty and a pain in that. She has no idea.
In March 2020, Annie was honored in the Philly Spin In. She got treated like a real-life celebrity by Children’s Hospital of Philadelphia. She was recognized for her courage, for her outcomes, for her life that’s still good! She even got her own photo shoot, complete with posters of her around the city. Very well deserved for such a brave girl.
I had the chance to share some of Annie’s story at the event.
Even though the refrain of hard news echoes behind all those closed cardiac clinic doors there’s one place, one team continues to never give up on Annie. That’s CHOP. I was able to tell how even though Annie’s diagnosis of half of a heart is terrifying and her prognosis is tough, there’s a center who continues to fight for Annie’s life with us.
No easy guarantees
No center can guarantee outcomes. No that’s impossible. But we have found a center who continues to guarantee that they’ll try. That they’ll listen to the next set of research. That their cath doctors will continue coming up with new ideas about decompressing the thoracic duct in the middle of a sleepless night.
A center that will say yes to trying a medicine that I found. I’m not doctor. But I am Annie’s mom.
There was a tiny amount of very convincing data. When her albumin levels dropped again in November 2019-February 2020, I pleaded with her team to let us try. We refused other typical treatments that haven’t been proven to work. I needed a different answer.
So in February 2020, Annie was the first pediatric patient to start Midodrine as treatment for PLE.
We’ve been thrilled that it works! For 19 months the midodrine stabilized Annie’s alubumin levels.
Until one day recently when it didn’t. There aren’t any other good answers. So we talked with her doctor, agreed to double her dose of midodrine, and keep hoping.
It’s still such a trial. So far the double dose brought her levels back up. But at her December cardiology appointment her doctor frankly reminded us what we all were afraid to say out loud.
“I think we’re seeing this probably won’t last forever.” We nodded.
But we’re still trying
“We’ll trust it as long as we can. We all hope it lasts for years and years ahead. But at whatever point it doesn’t we’ll need to make a quick pivot to plan B. Which would be a transplant evaluation.”
I don’t want to be here: exhausting our last options. I want there to be more answers. More crazy research studies I discover in the middle of my sleepless nights. I want there to be more time for Annie to grow up with this heart.
Praying for more
So I’m going to keep praying that the medicine continues to work. Will you join me?
In the meantime, I’m so thankful that there’s still somewhere that will help us help Annie. A place that will continue to advocate for what is best for her and hope alongside us that we can figure out what her body needs.
For today, we’ll sign her up for cheer clinic, watch her December dance recital on repeat, and beg God for continued stability on her March 2022 upcoming labs.