Pete Henkel’s siblings want you to know that he was funny, smart and kind, that he loved attending Radnor Middle School, playing Philadelphia Flyers PowerPlay wheelchair hockey and watching the Eagles. Most of all, Ian, Lucy and Chris Henkel want you to know that Pete was loved for every moment of the 13 years he was alive.
That’s what Pete’s siblings spoke about when they eulogized him in January 2018 to a crowd of more than 2,000 mourners at St. Thomas of Villanova Church. Chris and Lucy stood on either side of Ian as he read what they’d written. “We wanted to pay tribute to my brother’s life by telling stories about him,” says Ian, the oldest sibling. “We told stories that reminded people to first laugh, and then that he was a kid with a disease.”
That disease is spinal muscular atrophy, a congenital chromosomal disorder. SMA disables nerves that control muscle movement. There are five types of SMA, with Type Zero being the most severe. Pete was diagnosed with Type 2 SMA when he was an infant. He didn’t have use of his leg muscles. Throughout his life, he used a wheelchair. Pete had nine spinal surgeries. Respiratory issues required many trips to emergency rooms.
None of that stopped Pete from raising awareness about SMA. He joined Cure SMA, a national advocacy and support group, and so did his parents, Tim and Allyson. Although SMA is not well known, one in 40 people carry its genetic mutation. One in every 11,000 babies is born with SMA, making it the primary genetic cause of infant death in the United States. But SMA was not part of the newborn screening test in Pennsylvania. The Henkels decided to change that. Their efforts were linked to the 2016 FDA approval of Spinraza, the only drug proven to slow SMA’s progress. “Unless there’s treatment available, diseases aren’t typically added to the screening panel,” explains Dr. Oscar Mayer, Pete’s pulmonologist at Children’s Hospital of Philadelphia. “When Spinraza became available, it was a seismic shift for SMA.”
Dr. Richard Finkel participated in clinical trials for Spinraza. He was also Pete’s neurologist at Children’s Hospital of Philadelphia and is now chief of neurology at Nemours Children’s Hospital in Orlando, Fla. “Clinical studies show improvement when Spinraza is given to babies under 7 months,” says Finkel. “SMA is no longer a death sentence for infants. This treatment is by no means a cure, but it’s more than we’ve ever been able to offer these families.”
The availability of Spinraza encouraged the Henkels to advocate harder for SMA. In December 2017, Pete and his mother went to Harrisburg to ask legislators to add SMA to the newborn screening panel. They looked on as House representatives discussed SMA on the floor of the state capitol. Pete had prepared remarks about living with SMA, and he was scheduled to speak to the board that oversees the newborn screening panel. But that meeting adjourned early, before the Henkels arrived.
Six months later, after Pete’s death, Lucy gave that speech for him. She’s not just Pete’s sister—she’s his twin. In May 2018, she stood in front of state officials in Harrisburg and spoke Pete’s words. “I got through the whole thing, but afterwards I was pretty emotional,” Lucy recalls. “So were others in the room. After the meeting, people came up to me and said I did a good job for my brother.”
She must have. In September 2018, those state officials unanimously agreed to include SMA on the state’s newborn screening panel. The addition is awaiting approval by the Governor’s Budget Office.
Meanwhile, SMA kids are being treated with Spinraza. The medication is administered through a spinal injection. Seven doses are needed in the first year. After that, one injection is needed every four months. “If patients have scoliosis or have had many spinal surgeries, that makes administering it more challenging,” Mayer says. “But many people believe Spinraza is a way to stop SMA’s progression, and it seems to be effective without remarkable side effects.”
Pete’s parents continue their work with Cure SMA, and the family still grieves his death. “Life has gone back to a normal schedule, but we’ll never be normal,” says Chris, who still carries one of Pete’s socks in his pocket.
Lucy wears one of Pete’s religious necklaces, and Ian has his own way of mourning. “I write letters to Pete,” Ian says. “There’s no way to send them, but I write them.”