Conversations with Jessica Hoefler

Today we’d like to introduce you to Jessica Hoefler.  

Hi Jessica, we’re thrilled to have a chance to learn your story today. So, before we get into specifics, maybe you can briefly walk us through how you got to where you are today.
Piper Grace came into this world on Thursday, April 23, 2020, at 8:29 pm. Her story, however, began weeks before that in utero. What appeared to be an uneventful pregnancy turned out, in retrospect, to be quite different. At 30-31 weeks, I noticed that her movements changed. She was still rolling, but her kicks and punches had almost stopped. My OB was not concerned because I am not a big person, and her heart rate always remained strong. 

The birth itself was uneventful but quick (and natural). She came out gasping and struggling to breathe. They whisked her away to try and clear her lungs of fluid, at which point they became aware of her neuromuscular problems. She was immediately intubated and then transferred to Akron Children’s Hospital NICU with my husband, Ryan. 

After days of testing and consults with a team of doctors ranging from Geneticists, Neuromuscular Specialists, and Neonatologists, we received the crushing diagnosis of Spinal Muscular Atrophy (SMA) Type 0, a very rare form of an already very rare genetic disorder. Here is where our journey takes a painful turn. Now we were faced with what choices, if any, there were for a baby diagnosed with a disorder few were familiar with. We were told that nothing would reverse her condition, and any hope of her breathing, eating, or even moving (fine or gross movements) was impossible. In the end, she did not qualify for the gene therapy, so our choices took on a different focus. We continued to consult with many specialists, including a leading specialist and researcher in SMA at Nationwide Children’s Hospital in Columbus, Ohio. One of the terms that she used in describing Piper’s condition was that she was “locked in” her body. This resonated with us as we had put a “Piper lock” on the train bridge near our house that our Quinn loved to visit to watch the trains go by. We felt that this was a sign and realized that our only real gift we could give to Piper was to let her use her wings to fly to heaven. 

On Wednesday, May 20, 2020, at 4:32 pm the NICU staff at Akron Children’s orchestrated our final goodbye. I was able to carry her outside with a ventilator rolling behind me and our incredible NICU and Palliative Care family next to us. Outside in a beautiful courtyard surrounded by blooming hydrangea bushes, we sat with Piper still hooked to her ventilator feeling little raindrops on our cheeks. We felt the moment to remove her ventilator was upon us, and just as the clouds parted, her tube was removed, and Piper looked calmly into my eyes, free from medical equipment for the first time in her life. As the sun came out and beamed down on her, our sweet Angel took her last breath. 

Piper’s life began with a hectic gasp but ended with a peaceful, soft breath. Piper Grace will forever be missed, but we take comfort in knowing she is unlocked from pain and freed by love. 

Though she left the physical world, her presence and feisty spirit is ever so present though Piper’s Key. Piper’s Key is a 501(c)(3) nonprofit charity that gifts inclusive books to children with disabilities that represent their unique selves in a positive light. Had Piper survived her rare disease, she would be living with multiple disabilities and would need opportunities to grow a positive self-concept. It was through Jessica’s grief that propelled her to ensure that Piper’s siblings were able to say their sister’s name without the heavy sadness constantly associated with her passing. 

Since Piper’s Key was launched on her 1st birthday, 4/23/21, Piper’s Key has gifted over 6,000 books and counting to children with disabilities throughout the US and Canada. Each book gifted is matched to each child’s unique syndrome, characteristics, and age. Our mission is to ensure every child is able to see themselves positively in a book, as the book is the first media device a child experiences. 

Can you talk to us a bit about the challenges and lessons you’ve learned along the way? Looking back, would you say it’s been easy or smooth in retrospect?
Surprisingly, the biggest challenge we have faced is the unexpected demand! I didn’t realize how underserved the disability community is when it comes to children having the opportunity to see themselves accurately and positively represented. Because of this, I found myself struggling to keep up at first, primarily because I was doing most of the work myself with the help of family and friends. Now, we have an absolutely incredible and diverse board of directors. Each member brings a love for Piper, her mission, and resources to our cause! 

Thanks for sharing that. So, maybe next you can tell us a bit more about your work?
I’ve been lucky to be able to bridge my professional career with Piper’s mission. I am an educational audiologist at the Summit Educational Serve Center, serving children with various hearing and listening needs throughout school districts Northeast Ohio. My primary role is to ensure each student has access to instruction within the classroom; however, my team at focuses on developing a successful self-advocate as well. Because of this, I have been able to gift books to the students I work with daily and watch their reactions! With every smile, I feel Piper is smiling right next to them! 

Contact Info:

• Website: Piperskey.com
• Instagram: @pipers_key
• Facebook: Piper’s Key

Image Credits
Jessica
Quinnto