Syracuse redshirt sophomore offensive lineman Sam Heckel shared his rare blood disorder story with the world to inspire others with hope. The 6-4, 280-pound Heckel, a native of Wisconsin, is the Syracuse Football Uplifting Athletes Chapter President. The rare disease cause is personal to Sam and he chose to use his platform to shine a spotlight on the Rare Disease Community. This story is being re-posted courtesy of Cuse.com with permission.
SYRACUSE, N.Y. – The process lasts anywhere from 14 to 20 hours.
That’s how long it takes redshirt sophomore offensive lineman Sam Heckel to pump 1,800 units of plasma – the liquid component of blood – into his body.
Heckel doesn’t need the plasma. Just the ADAMTS13 enzyme it contains.
Diagnosed with congenital thrombotic thrombocytopenic purpura (TTP), a rare blood disorder, when he was a toddler, Heckel will likely undergo plasmatherapy to receive the enzyme for the rest of his life.
It’s the primary reason Heckel felt compelled to become president of the Syracuse chapter of Uplifting Athletes. He understands the organization’s mission to raise awareness and research dollars for the treatment of rare diseases better than anybody. Because he lives with one.
The symptoms started when Heckel was an infant. He was frequently sick and wasn’t getting better. He was in and out of hospitals, but doctors couldn’t identify what was wrong.
Then in 1999, when Heckel was two years-old, he was playing in a kiddie pool at a family party. When his father, Steve, lifted him out of the water and put him down there was a dark, hand-shaped bruise on Sam’s body from where his father had picked him up. Knowing immediately that something wasn’t right, the family again sought medical attention.
Shortly thereafter it was discovered Heckel had congenital TTP, which is caused by a lack of ADAMTS13 in the blood. Without the ADAMTS13 enzyme to breakdown large proteins, clots can form in the body’s small blood vessels. Signs of the disorder include not enough red blood cells to carry oxygen and not enough platelets to efficiently pump blood through the body. If not treated, congenital TTP can lead to organ failure and problems in the nervous system such as headaches, confusion and seizures.
The congenital form of TTP is extremely rare. In 2015, there were only approximately 100 reported cases of congenital TTP worldwide and the chances of being afflicted with it were less than one in 1 million. There was a time when Heckel’s case was believed to be the last reported incidence of congenital TTP in the United States.
Without treatment, the mortality rate for congenital TTP is greater than 90 percent, but with the introduction of therapeutic plasma infusion that number has dropped to about 15 percent. Plasmatherapy also prevents long-term organ complications due to chronic relapses.
But in 1999, so little was known about the disease that there were no reliable treatment options. Heckel suffered for years with what his mother and father termed “episodes.” He was frequently ill and missed school. He was constantly fatigued, pale and had difficulty urinating.
When Heckel was in second grade things got so bad he was admitted to the ICU at the Children’s Hospital of Wisconsin near the family’s home in Waukesha. Heckel’s platelet count had dropped to approximately 6,000. It should have been 200,000. His kidneys began to fail.
“I didn’t understand it at the time, but my doctors didn’t think I was going to make it,” Heckel said.
Heckel was hospitalized for two and a half weeks. He eventually recovered, but unsure of what would come next or how much time their son had left, the Heckels contacted the Make-A-Wish Foundation.
Started in 1980, the Make-A-Wish Foundation arranges experiences – known as wishes – for children with life-threatening diseases. Often times it’s a child’s last opportunity to experience joy without the constant presence of doctors or painful medical procedures.
For Sam, the choice came down to turning the family’s basement into a Dave and Buster’s-like game room or a trip to Hawaii. The Heckels decided they would visit Hawaii, and in 2007, when Sam was 10 years-old, his wish was granted. Sam and his family spent two weeks swimming with dolphins, hiking Diamond Head, attending luaus, surfing and relaxing on Waikiki Beach.
About six months after their Hawaiian vacation, the Heckels received good news. Plasmatherapy had progressed to the point where Sam’s doctors thought it could be an effective form of treatment. He started going every three weeks for infusions.
During the treatments, Heckel sat and did homework, read books and watched TV. When it was over he rested until he was well enough to resume his normal activities.
While Heckel felt run down and he was more susceptible to infection than most children his age, his relapses decreased. He was allowed to continue playing sports, including his three favorites – baseball, football and basketball.
As Heckel grew older and entered high school, his ability on the gridiron became more and more evident. He started attracting attention from college coaches and developed into one of the top linemen in the state. As a senior, Heckel earned Associated Press All-State First Team honors and was ranked the No. 2 guard in Wisconsin by Scout.com. In addition, he helped his high school – Waukesha West – to consecutive WIAA Division 2 state championship game appearances.
During the recruiting process, Heckel was very up front about his disease and the constant treatments it requires – even during the football season.
“There were some schools that didn’t want to bring in an athlete with my condition,” Heckel said. “But mostly it was about finding the right fit of football and a place where I could get treatments.”
Head coach Dino Babers and the Orange staff made it clear they would welcome Heckel and work with him to ensure he received the care he needed. That assurance, Syracuse’s status as a Power-Five football program in the ACC, and the University’s academic reputation appealed to Heckel and his family. Syracuse also had something else going for it – Golisano Children’s Hospital is right across the street from campus.
One of Heckel’s childhood doctors in Wisconsin had a connection to a member of the Golisano staff and made arrangements for Heckel to receive his treatments there. That sealed the deal.
The Mondays after plasmatherapy are difficult for Heckel. The process starts at 8 a.m. every third Sunday with the insertion of an IV. It ends late Sunday night or early Monday morning depending on his platelet count and how many units of plasma he needs. The injections are slow and Heckel often feels weak afterward. He has a pump that allows him to control the flow of plasma into his body. He injects a little bit at a time to minimize the risk of an allergic reaction.
During the treatments, Heckel’s in frequent communication with the strength & conditioning staff and his coaches about how he’s feeling. If necessary, they make arrangements to adjust his workout schedule and meeting times, so he can recuperate. Heckel is also closely monitored by team physician Dr. James Tucker and the athletic training staff for signs of distress related to the disease. Once the season starts, Heckel’s blood is drawn weekly to ensure his platelet count stays within the normal range.
So far, the disease and treatment schedule haven’t affected Heckel’s performance. After redshirting his first season, Heckel started all 12 games at left guard in 2017. He led the Orange with 41 knockdown blocks and played a total of 1,032 snaps for a Syracuse offense that ranked third in the ACC in total offense and second in passing.
Off the field, Heckel developed a strong bond with former Syracuse quarterback and Uplifting Athletes president Zack Mahoney. After hearing his story, Mahoney took Heckel under his wing and made him vice president of the Syracuse chapter for the 2017-18 academic year. Heckel observed first-hand how Mahoney organized the group’s signature event – the “Lift for Life.”
“Zack did so much behind the scenes. He was such a great leader in terms of football and Uplifting Athletes,” Heckel said. “He’s just such a great guy. You won’t find another Mahoney.”
Now Heckel holds the chief leadership role and is looking forward to executing his first “Lift for Life” as president this Friday.
“This year has been a little bit slow because this is my first year and I’m still learning,” Heckel said. “But I believe we can do more. We have to do more. That second-grade episode was supposed to be it. My wish was granted. I owe it to others, now.”
There is no cure for congenital TTP and likely never will be, but Heckel hopes more efficient methods of transferring ADAMTS13 are coming.
“There aren’t enough people with this disease to justify spending the amount of money it would take to find a cure,” Heckel said. “But they are exploring ways to concentrate the enzyme, extract it from plasma, and deliver it.”
Balancing the rigors of being a Division I athlete, a full academic course load, and his treatments has been difficult for Heckel. So has been opening up about his disease, something he’s never done before.
“My teammates know I go in for treatments. I’m not sure they understand fully what it involves, but it’s not about me,” Heckel said. “It’s about creating awareness for this disease and if my story helps do that then this has all been worth it.”