Pirates fascinate Ethan Pennel. Pumpkins, too.
And like most 5-year-old boys, Ethan likes a good game of Angry Birds.
But if he gets too excited – and when he starts to cough – the little boy with those wide eyes could wind up in the pediatric intensive care unit at Women & Children’s Hospital.
Just like he’s done 33 times before.
“They call him the mayor of the PICU because we’re there so much,” said Nichole Pennel, Ethan’s mother. “He knows that he’s sick, and he’ll say: ‘I don’t feel good. I always don’t feel good. I’m sick all the time.’ He thinks it’s normal.”
Since the day he was born, Ethan has fought through severe respiratory issues that restrict his lung capacity and compromise his ability to breathe. Time spent outdoors is limited to five-minute spurts that exhaust him.
“His lungs just give out and he has to rest,” said Brad Pennel, Ethan’s father. “He can’t handle physical activity.”
The Pennels, who live in Salamanca, have not received an official diagnosis, but doctors that are involved with their son’s care and treatment know that what he has is rare. Ethan is currently taking a new medication, which could determine if he has cryopyrin associated periodic syndrome, a genetic autoinflammatory disease that is so rare it affects one or two in every 1 million Americans.
Yet, according to his father: “He’s happy, very happy all the time. The only time he’s not happy is when he is in the hospital and getting an IV or something.”
Halloween makes Ethan smile. He would have every day be Halloween if he could, said his mother.
“He’s a 365-day-a-year Halloween kid,” said Nichole. “His favorite character is Jack the Pumpkin King from ‘Nightmare Before Christmas.’ ”
In October, the Pennel family – including big sisters Molly, 19, and Lauren, 17 – will visit Disney World to meet Jack the Pumpkin King and attend the “not-so-scary” Halloween party.
The trip is on Ethan’s mind as he endures visit after visit to the Cleveland Clinic. The family makes the trip once every two months for re-evaluation. It was there that the Pennels learned Ethan’s treatments would be changed from injection to infusion to help block the white blood cells that are attacking his lungs.
“We were in the doctors’ offices for four hours yesterday sitting there talking about him and his health,” Nichole said. “He’s sitting there coloring and playing on his iPad. He knows we’re there because of him.
“When we pulled into the clinic, he announced: ‘We’re in doctor’s world.’ ”
Nichole, who is 39, operates an online retail business that specializes in children’s clothing accessories. Brad, 42, has worked for Cutco Cutlery for 14 years.
Ethan was a surprise, they said.
“We had tried to have another child, but it never happened, so we basically gave up,” Nichole said. “Six years later I was pregnant with Ethan.”
The girls, who were 11 and 14 at the time, have grown up caring for their brother.
Molly, who went away to college for a semester, missed her brother so much she moved back home, said Nichole. Now Molly attends Jamestown Community College for nursing so she can stay home with Ethan.
Ethan attended school for six weeks as a pre-kindergartener, but he was hospitalized four times, so the family pulled him out. He has not been back. His contact with other children is limited – except for Stone’s Buddies.
Stone’s Buddies is a program for chronically ill children at Women & Children’s. Created in memory of former patient Stone Filipovich, the program provides a network of resources, support, friendship and fun for the children who are regular patients.
“The way it looks, he will probably not go to school at any point,” said Nichole, who home-schools Ethan. Currently they are working on letters, numbers and planting pumpkin seeds.
“We call him our little farmer because he loves to plant and grow,” Nichole said. “He’s got a fascination with growing pumpkins. We started seeds last May, and he wound up getting sick. He was on life support with lung bypass for two weeks. They told us twice that he wouldn’t make it through the night. He fought through.”
The 6-inch incision surgeons put in his neck during the lung bypass procedure gave Ethan some credence in the pirate world, according to his mother.
“He called it his pirate scar,” Nichole said. “He was in a pirate fight, and that’s how he got the scar.”
The Pennel family has learned a lot from watching Ethan weather the bouts of illness that fill his life.
“We’ve learned to slow down and to appreciate things a lot more than we used to,” Nichole said. “With Ethan, we just don’t know. We try not to look too far into the future, because that’s when I get upset and I get scared.”