Ornella Liesenfeld slides her tiny 6-year-old body down the slipcovered couch to show me the purple monster she drew on her white T-shirt.
"It kind of looks like Grover," I say. She looks puzzled and shakes her head.
"Purple," she points out.
"And what color is Grover?" I ask.
"Blue," she said. Of course.
Upon closer inspection, she's right. Her monster looks nothing like Grover.
A sprightly and opinionated kindergartner, Ornella loves to draw, play with the family's goldendoodle, Spark, and listen to stories about Rapunzel.
Doctors have warned her parents, Aicha and Micah Liesenfeld of St. Louis, that this is the year when things may start to change for Ornella.
She was 14 months old when she developed a fever that wouldn't break. She ended up in the ICU at St. Louis Children's Hospital, where doctors initially thought she had cancer because her immune system was shot. After months of testing, her parents got a diagnosis they had never heard of.
Ornella has a very rare genetic neurodegenerative disease: ataxia telangiectasia, also called AT. The disease causes a progressive degeneration of a part of the brain, gradually leading to a general lack of muscle control. Patients lose their ability to write, and speech becomes slowed and slurred. Even reading is eventually impossible as eye movements become difficult to control. On top of that, a patient's immune system is severely compromised, making certain types of cancer 1,000 times more likely.
Aicha asked about her daughter's prognosis. Ornella's life expectancy would only be about 20 years, the doctors said then. She asked if there was a cure.
No, they said. In fact, there's no great treatment to stop the decline once it starts.
"It was devastating," Micah said. No one in their families had a genetic disorder. Aicha is African American and originally from Burkina Faso, and Micah is white and from St. Louis. What are the odds a couple from opposite ends of the world would both carry such a rare disorder?
"It was just mind-boggling to us," Micah said.
That first year was filled with fear and anxiety. A simple cold or infection could become life-threatening for a child with AT, so they are careful to limit her exposure to germs. Ornella gets a weekly IV infusion to boost her immune system. Other than a slight unsteadiness on her feet and some slurred speech, there's no outward hint of the gravity of her illness. It's hard to imagine that she will likely be in a wheelchair in a few years.
Aicha started researching, and found an online community of people who have a family member with AT. The support group became both a lifesaver and a warning of what was to come. She also discovered the AT Children's Project, a nonprofit that funds research for treatments and a cure for AT. The organization is connected with about 400 families who have a child or young adult with AT. Most of the group's $2 million annual budget is raised through grassroots efforts by its members.
Aicha had started making specialty soaps and lotions when their older child, Julian, now 9, had eczema. The gentle formulations healed his skin, and she also made them as gifts for family and friends.
In 2018, one of their family friends asked if they could order some soap that included cards about AT to raise awareness.
That's when it clicked.
Aicha decided to turn her hobby into a way to raise money for research. She set up an Etsy site (etsy.com/shop/soap4cure) to sell her products, wrapped in packaging designed by her husband. Before the pandemic, she would also sell her soaps and lotions on weekends at local markets.
The first year she raised $1,000. This year, she expects to donate at least $2,000. Researchers are investigating gene therapies and ways to better manage the symptoms of AT.
The research supported by ATCP gives Aicha hope for Ornella. Making beautiful soaps in her kitchen after her children go to bed gives her a way to be a part of that hope. When she sets up shop at farmer's markets and craft fairs, people ask about why she started the side business. She has met parents whose children have overcome significant illnesses.
"All those stories I have been collecting throughout the years," she said, help keep her positive.
"Obviously, as a mom, I still go through episodes of sadness, uncertainty and fear," she said. "But I have a little more hope now than back then, for sure."