Ever since Sienna’s diagnosis with FOP in May 2012, I’ve thought quite a bit about genes. Sienna has great genes. Amazing genes. 2 totally healthy (though over-worked) parents. 4 healthy grandparents. And until today, a 98 year-old great-grandmother who enjoyed a long healthy life.
Today we remember Betty Kelber, my Nana, who lived 9/21/15-12/7/13.
My Nana was an amazing woman. She raised my mom all by herself and saw her share of hard times. But to know her, you would have never known. She was filled with love and joy. She had an infectious laugh and a great sense of humor. And the twinkle in her eye let you know that she was a little mischievous. I was her co-conspirator in sneaking extra cookies many times – from when I was little and she brought them to me, to when she was older
and I brought them to her.
Growing up, we were lucky in that my Nana moved to be close to my family and followed us each time we moved. She helped shuttle me from school to many swim practices and would always stop at my favorite frozen yogurt store on the way. She would often have a hot dinner waiting for me in the car when she picked me up. She knew that coming out of practice I would be so hungry I couldn’t wait. It would be a healthy microwave meal or takeout, but it would be delicious. (And that is exactly the type of thing I find myself serving to Sienna. If someone else can make it deliciously and easily, why waste time with homemade? We are practical women in this family 🙂 ) And she always had a sense of humor and went with the flow rather than getting caught up if things didn’t go right. A perfect example is when she got an email address in her 80’s and signed her first email to me “Love, Nanag I don’t know how to erase the g.”
Sienna was given her middle name, Elizabeth, in honor of my Nana. I see so many of her great qualities shining through in Sienna: her love of animals, her great sense of humor, her sweet tooth, her resourcefulness, her energy and her determination to do whatever she sets her mind to. That determination worries me sometimes when Sienna wants to run around and I want to keep her safe from falls. But I know Sienna will be ok. She comes from a long line of strong and determined women and I know she will find her own path.
FOP is a spontaneous genetic disorder (meaning it is genetic, but not inherited from either side), a kink in the ACVR1-ALK2 gene. Just one tiny little gene sending the wrong signal to her body to keep making bone when it shouldn’t. But Sienna has a lot more genes than that pesky one. With genes from a woman like Nana, I will not be surprised if Sienna decides to become a scientist and just goes ahead and cures herself. That will be the kind of legacy my Nana will smile down on, with a twinkle in her eye. In fact, she’ll probably be the one guiding Sienna to make it happen.
We love you and miss you much, Nana!