Tackling a disease: SU offensive lineman keeps rare disorder in check with infusions at Upstate
BY JIM HOWE
He’s 6 foot 4, weighs nearly 300 pounds and plays football for Syracuse University.
And, about every three weeks, Sam Heckel settles in for 12 to 16 hours of infusion treatment at Upstate to deal with a rare blood-clotting disorder. He might start by getting the very slow plasma drip at the Upstate Cancer Center, then finish up at the Upstate Golisano Children’s Hospital, or vice versa.
Although he towers over the other pediatric patients and turns 22 this year, he is cared for by pediatricians until graduating from college, as are most young people in long-term treatment.
Heckel’s disorder is known by its initials, TTP (see “About TTP,” below).
“He is my first and currently our only patient with congenital TTP,” says Andrea Dvorak, MD. She is the Upstate pediatric hematologist/oncologist who specializes in children’s blood disorders and cancer and who oversees his treatment. A few other patients have had acquired TTP, the more common type of the disorder caused by an inherited genetic mutation.
“With the acquired form, there are treatments that can make it go away, but with Sam we don’t have that ability. Research is being done, but unless some new treatment comes up, this is his routine from now on,” Dvorak says.
Heckel hopes to contribute to research. He’s president of the SU chapter of Uplifting Athletes, a nonprofit group of college athletes that aims to create awareness of rare diseases and raise money for research.
Heckel learned he had TTP as a child growing up in Waukesha, Wis. “I was about 2 years old, with my mom and dad at a family get-together, and we were all swimming. When my dad went to grab me and set me down, he left handprints, like bruises, where he held me,” Heckel recalls.
It was the first hint of the disorder.
Young Sam was diagnosed soon after, but his family didn’t find suitable treatment until he was in second grade. Then he began what has become his treatment routine at the Children’s Hospital of Wisconsin, in Milwaukee, about a half-hour from his family’s home.
“It normally takes about 14 hours,” he says of his infusions. Sometimes he is done by 10 p.m. Sunday, sometimes by 2 or 3 a.m. Monday.
“I usually just hang and watch movies, and if I absolutely have to do homework, I’ll do it, but usually it’s just a day to relax,” he says.
(Heckel talks about his disease and football in this video interview.)
“It’s the next day that I feel it. With all of the medications, it does not make life enjoyable the next two days.” The plasma and medications he must take can leave him feeling bloated and out of sorts for a few days.
Having TTP means Heckel has to stay “super hydrated” to avoid straining his kidneys and be careful about catching illnesses, since he is vulnerable to infections, “but I don’t have to make a lot of adjustments in day-to-day life,” he says.
He does have to guard against excessive bruising — which might seem difficult, given that he plays on the SU offensive line.
Some doctors may have advised against contact sports for a child with TTP, but Heckel’s pediatrician agreed to monitor him closely. “It’s worked so far,” Heckel says.
He has played football regularly since second grade. The lineman job was pretty much chosen for him by his massive size, he says. He played on the defensive line in high school, and he also played tight end a little last season. He is a redshirt junior this fall and will be able to play one more season at SU after that. Heckel enjoys earth science and says he would like to teach after college.
When choosing a college, Heckel made sure a hospital that could treat him was nearby. Upstate is next door to SU and less than five minutes from where he lives. His doctor in
Milwaukee knew Upstate pediatrics professor emeritus Thomas Welch, MD, who put him in touch with the pediatric hematology program. That’s how Heckel connected with Dvorak, his doctor at Upstate.
(Click here for Heckel’s SU football profile and here for an SU football overview.)
Dvorak closely monitors Heckel. “We check his lab reports (blood tests) weekly during football season, and we have to watch his platelet count and kidney function and have to be sure he is staying healthy,” she says.
She also keeps in touch with his athletic trainers. “Before he started, I spoke with the team doctor, and his trainer gets a copy of his labs every week. They know what he needs in order to play,” she says.
In the off-season he does not need to be monitored as closely but still needs his infusion.
“He’s a good kid. He’s motivated. The fact is that both in school and in football … he hasn’t let this stop him. This disease should not interfere with his having a long life,” Dvorak says.
Heckel echoed his doctor’s assessment when asked what he would tell a youngster diagnosed with TTP: “You can’t let that disease define you. You can’t let it limit you. You can do whatever you want.”
Thrombotic thrombocytopenic purpura is a rare disorder in which people lack an enzyme called ADAMTS13.
This makes their blood “sticky” and prone to form clots, according to the TTP Foundation.
These clots are made of platelets, a blood element that normally promotes clotting at injury sites to prevent bleeding. In TTP, unnecessary clotting can restrict blood flow to the body’s organs, placing them at risk for damage. Also, the platelets being used in unnecessary clots are not available to prevent bleeding elsewhere.
Treatment includes slow infusions of normal plasma, the liquid part of the blood, which contains the missing enzyme.
About three or four new TTP cases per million people are diagnosed each year in the United States.
This article appears in the fall 2019 issue of Upstate Health magazine. Click here for the full online version of the magazine.