Community to support boy’s battle with rare disorder with benefit in Hamilton Feb. 7
by James McEvoy, Managing Editor
ROBBINSVILLE — In May 2012 Alec Perusich, then 7, went to bed with a headache and awoke the next morning with a bit of redeye and a small rash under his eyes.
Initially, pediatricians thought Alec may have pink eye, but when his symptoms did not abate his parents Robyn and Scott decided to take him to the emergency room at Children’s Hospital of Philadelphia (CHOP).
They were blindsided when informed at the ER that their son, now 9, had a rare condition called Steven Johnson Syndrome (SJS).
The disorder, which causes severe blistering of the skin is often triggered by a reaction to medication.
However, Robyn said Alec wasn’t on any medication at the time and that doctor’s next best guess was that it was triggered by walking pneumonia.
Robyn said their son was treated for six weeks at CHOP, including a four-week stay in CHOP’s intensive care unit and a week and a half of rehabilitation.
During Alec’s initial six-week stay at CHOP, Alec was put in a medically-induced coma and put on a ventilator for ten days due to concerns that his esophagus would start swelling and to help control the pain endured during four-hour daily dressing changes over his body.
Robyn told the Messenger Press the pain compares to that of burn victims, and that the bandage changes were required due to Alec losing nearly 70 percent of his skin as a result of the syndrome.
”It truly was not even just a day-to-day situation with Alec,” added Robyn’s cousin Meg McCarthy, of Allentown. “It was truly an hour-to-hour, minute-to-minute situation.”
”I’ve never seen a child in pain like I did that first day Alec was in the hospital,” Ms. McCarthy continued. “It truly was beyond anything I’ve ever seen. He has been extremely brave over the last 18 months.”
”I had never heard of Steven Johnson Syndrome,” Robyn said. “So when they told us in the ER what it was I thought, ‘OK. They know what it is. They’ll treat it and we’ll be home.’”
However, there is no “cure” for the SJS, Robyn said, noting doctors informed them it simply had to work its way through Alec’s body, with physicians having to treat the symptoms as each arose.
”It happened really quick,” she said. “It was just a matter of days.”
Doctors began to work to suppress the disease and help Alec’s body fight it off. They were also quick to tell Robyn and Scott SJS often impacts the eyes, potentially causing blindness.
Alec began experiencing photophobia — intolerance of light — and dry eyes. However, he was able to return to school as his parents began looking into PROSE lens, a special type of contact lens that would help reduce his symptoms and allow him a higher quality of life.
Alec’s parents thought the worst was over.
Then a secondary autoimmune disorder, Ocular Cicatricial Pemphigoid (OCP), which doctors believe is linked to his SJS, began attacking his eyes, further damaging his vision.
”Prior to this latest autoimmune disease that was diagnosed in the summer he was doing OK,” Robyn said.
Alec underwent numerous eye surgeries in 2012 and 2013 — all membrane graphs to cover epithelial defects.
Specifically, Alec’s vision has been compromised mostly due to a limbal stem cell deficiency.
The family traveled to Bascom Palmer Institute in Miami to see a specialist, as doctors are currently researching the best way to fix the stem cell problems, but a lot of the procedures are still awaiting FDA approval.
In addition, Alec is unable to make his own tears, but again doctors at Bascom are working with doctors in other countries who are researching this as well.
He is on four different eye drops to help keep his eyes lubricated as they feel like sandpaper when he is not lubricating them.
In addition, to help suppress the OCP, Alec is undergoing apheresis, IV autoimmune suppressants and IVIG treatments.
Though his eyes have been the only impact of SJS so far, Robyn acknowledged they’re not out of the woods yet, noting other cases when other organs were impacted.
”You just don’t know. That’s the scary part,” she said. “We’re just holding tight that it has just effected what we know and nothing more.”
Despite the challenges, the most recent of which involved an infection stemming from a port — which was implanted in Alec to better provide medications — his mother said he has exhibited courage and a positive outlook.
”He has a very good attitude and he’s extremely hopeful that he’s going to get 100 percent better so that’s what’s keep him going. He’s really looking forward to playing Minecraft again so in his head he’s mapping out Minecraft maps,” she said, referring to a popular video game.
While bravely learning Braille, Alec has also began listening to books on CDs and using his imagination while playing with Star Wars action figures.
”He’ll complain about Braille, but he knows he has to do it and he does it willingly,” she said. “I think he likes to learn because his one sense is being taken away so I think it’s just natural for his other senses to take over so I don’t think he minds it as much. It’s challenging, but he’s doing pretty well with it.”
She acknowledged that, as a parent, his strength gives her strength.
”When he’s in pain, I’m in pain. It’s awful,” she said. “But when he’s happy and positive it makes all the difference in the world.”
Despite the strength and courage he has already exhibited, Robyn said the support of family, friends and the community has been wonderful.
”It’s wonderful. We feel extremely lucky and supported by everybody,” she said. “There are no words really.”
Alec, who is in the fourth grade, is currently being homebound taught by staff from Pond Road Middle School.
”They’ve been wonderful. All our friends, neighbors, the school, the Commission for the Blind and, of course, our CHOP doctors,” she said.
Community support for Alec will be on full display at a benefit on Friday, Feb. 7 from 6 to 10 p.m. at the Nottingham Firehouse in Hamilton.
Tickets for the benefit are $50 a piece, and include dinner, desert, drinks – beer, wine, soda, coffee and tea, and $15 worth of raffle tickets. The benefit will also feature a 50/50, tricky tray raffles, door prizes and silent auction items.
Ms. McCarthy, of Allentown, along with her husband Tom, Scott’s sister Lori Perusich and Robyn’s sister Tracy Cole, is organizing the benefit.
The Knights of Columbus Council 7333, of Allentown, is sponsoring the benefit.
Tom, the TV play-by-play announcer for the Philadelphia Phillies, will be the emcee for the evening. Sports memorabilia will be offered through silent auctions at the event.
In addition to helping raise funds to offset costs of treatment, Robyn is simply hoping to get the word out about the SJS.
”They say it’s rare, but I hear of more and more people with it,” she said, adding she wants people to “learn from what we’ve gone through.”
She noted while doctors don’t believe Alec’s case was caused by a reaction to medicines, that people should still be aware of what they’re putting in their body.
Robyn also acknowledged the disease works differently in patients, with SJS working slower in others and causing a variety of different symptoms.
For more information about SJS and how you can help, visit www.sjsupport.org.