'Life is good': Manton co-captain Helsel overcomes rare liver disease

Aug. 20—MANTON — "Life is good," Jack Helsel says.

The Manton 15-year-old sophomore couldn't always quite say that, but he's leaving that in the past, much as he aims to leave his crosscountry opponents in the rearview this season.

Helsel overcame a rare liver disease, one so rare it doesn't even have a name.

Although the family struggled to find out what was happening to their only son as a toddler, eventually doctors found the cause. And it made sense that they didn't look for it, since less than 100 cases have been documented since its discovery.

"It's extra baggage he carries, but you'd never know it," said Jack's mother, Nicole Helsel. "He's always able to put things in perspective."

Progressive familial intrahepatic cholestasis (PFIC) is a disorder that causes progressive liver disease, which typically leads to liver failure. In people with PFIC, liver cells are less able to secrete bile, and the buildup of bile in liver cells causes liver disease. There are three known types of PFIC: PFIC1, PFIC2 and PFIC3. His version of PFIC is due to a mutation in a gene called MYO5b, a discovery recently made in 2017.

Jack's form is a fourth type, so rare it's referred to as an "orphan disease" because not enough people have it for it to even get a name.

DIAGNOSE

As a baby, Jack kept itching his skin raw. His parents put socks on his hands to prevent him from tearing open flesh to feed the itch. They tried all sorts of home remedies at first, but soon sought professional help.

Doctors weren't quite sure what to make of it, originally speculating it was severe eczema.

When medications for eczema weren't helping, despite increasing doses, one pharmacist refused to fill the prescription, saying it was too high of a dose for a child.

The Helsels asked him what to do. "Get a new doctor," he suggested.

A new doctor's office gave Jack some blood tests and sent the Helsels home. The tests quickly revealed something much more than eczema.

"We were still in the parking lot and they called and said his liver count is off the charts," said Nicole Helsel, an eighth-grade science teacher at Mackinaw Trail Middle School in Cadillac who previously taught at Manton and is the school's volleyball coach.

More testing revealed the diagnosis of PFIC. Jack wasn't even a year-and-a-half old.

The Helsels said doctors told them there were only five cases documented worldwide at the time, mostly in France. The cause of the itch was bile from the liver getting into his bloodstream.

So at 18 months old, Jack had his first surgery called an external biliary diversion — one to cut a hole in his abdomen for an exterior ostomy bag that'd allow the bile and acids to drain that way instead of getting into his blood.

Dr. Neal D. Uitvlugt, a pediatric surgeon in Grand Rapids, did the procedure to implant the ostomy bag port for free 13 years ago because the Helsels' health insurance company wasn't going to pay for it.

MANAGE

In kindergarten, for show and tell, Jack brought himself. Indeed, nobody else probably brought something so rare there's only five in existence.

He pulled up his shirt, showed the entire class the bag attached to his abdomen and explained he needed it to live.

That cut off other kids from teasing him before it could start. Manton cross country coach and teacher Jeff Harding said the entire 2026 class knows about it, and much of the rest of the high school.

He'd keep extra clothes at school for times when the bag would leak. It also prevented him from playing any sports. He was told by doctors not to play any contact sports anyway, but the bag discouraged others as well.

"I always wanted to be a swimmer, but the bag would always come off," Jack said.

He couldn't get it wet, either, so they at one time tried wrapping his abdomen in cling wrap to prevent it from getting water on it. That didn't work so well.

Jack was put in a long-term study at 18 months old. He returns to Chicago every year for further study, hoping the results will help others afflicted by PFIC, now and in the future.

He has a checkup every six months in Chicago, staying at the Ronald McDonald House. It was staying there the Helsels saw what other families were experiencing and realized their plight wasn't that bad.

"Learning about what symptoms patients with the type of mutation Jack has helps doctors to understand the disease better and be able to advise future patients on what types of symptoms they may have," said Dr. Alyssa Kriegermeier, a specialist the Helsels saw at Robert H. Lurie Children's Hospital in Chicago. "Additionally, learning that these types of surgeries can provide significant benefit to patients like Jack's helps us know the options for future patients that might be successful — just like they were for Jack."

RUNNING LATE

Jack took up running as a freshman, at the behest of Harding.

It was when Jack was helping his mother with the volleyball team that Harding spotted him and started recruiting him as a runner.

"He's an A student, he's thin," Harding said. "That's a cross country runner if I've ever seen one."

Harding's sophomore daughter Kelsey plays volleyball on Nicole Helsel's team, so it kind of seemed natural that the switching of kids and sports would be complete with Jack running for Harding's team.

"When he came to us last fall, he had never done cross country or any sport at all," Harding said. "He improved a lot through the season. He broke into the 19s as a freshman, which is impressive for anybody."

Last year's Rangers XC team was a senior-laden one, with five seniors and two freshmen. Nothing in between.

This year's team is almost the opposite, two sophomores and five freshmen. Jack Helsel and one of his best friends, Robert Dykhouse, are the two sophomores. The team is so young, not a single member of the squad can drive yet.

With only one year of varsity running under their belts, Helsel and Dykhouse find themselves as co-captains and in positions of leadership.

"It's a good thing," Dykhouse said. "Last year, those seniors really taught us how to run it. Now we get to use that knowledge."

They run together around Manton frequently, sometimes going to Cadillac to hit trails.

One of Jack's goals is to be the Rangers' No. 1 runner, although that means passing Dykehouse in the pecking order. Jack and Robert both said a goal is to make the cross country all-time leader board in the gym.

"It's exciting to see what he's going to do this season," Harding said. "He's going to go far beyond Manton cross country."

OPERATE AGAIN

Still, running with the bag had its disadvantages. He'd tuck it into his waistband, but it still would be in the way on the course.

That's when the family looked into other possibilities.

Jack researched an experimental surgery called an ileal bypass to get rid of the bag and internalize that function, re-routing the excess bile flow to his intestines instead of an exterior receptacle.

Ultimately, they decided the side effects of the surgery were much more treatable than the looming possibility of liver failure.

"We were going back and forth on it," said Jack's father Russ Helsel, a first-grade teacher at Franklin Elementary in Cadillac.

"I picked the right thing," Jack said.

The surgery at Lurie's Children's Hospital last November left Jack with a four-inch vertical scar on his stomach, which he refers to as his "six-pack," and another to the right where the ostomy bag connection previously resided.

"I cannot overstate how positive and upbeat Jack and his whole family have been throughout his whole disease, and especially during his recovery after surgery," Dr. Kriegermeier said. "Getting to take care of him is a real privilege and seeing his constant positive attitude in the clinic lifts everyone on the team's spirits. Jack was previously cared for by another (liver doctor) hat moved to another institution, and despite this, she still asks how he is doing and wants to check in on him and make sure he's OK. This just tells you how much of a special type of patient he is to all who take care of him."

He's currently the youngest person in world with internal bag.

"He was very worried," Dykhouse said. "After the surgery, there were some setbacks, but he put his mind to it."

He also lost a lot of weight, slimming down to 98 pounds.

Then he started going to school early, with Coach Harding opening up the weight room for him. Now he's back up to 130 pounds.

SPEEDY RECOVERY

Doctors initially thought Jack might not live past age 3, or that he'd be very short or developmentally challenged.

Instead, he's the oldest runner on Manton's cross country team, stands 5-foot-10 and is at the top of his class academically.

Most students at Manton know he has a condition, but not the details of it. Not even some of his close friends. It's just the reality, and he deals with it rather than talking about it.

"I'm pretty sure they know there was a condition, but they don't know what it is," Dykhouse said. "I don't even know what it is."

The danger isn't completely gone, but the spring results show how dramatically Jack improved after the surgery.

He's feeling more confident, feels better and his number of medications is down from an amount almost hard to keep track of to just two.

"I've been able to walk around with my shirt off," Jack said. "I've never been able to do that before."

He's taken a scientific approach to running, studying film of top track and cross athletes to pick up technique tips.

"I can't say I really enjoy running," Jack said. "I really enjoy the team and being together."

But the results speak for themselves. He placed in the top five in 1,600-meter races six times, and put up top-10 finishes in the 3,200 in 10 of 12 meets. In both events, he posted a personal-best in the Highland Conference championship meet to take fifth place.

"To have something like that and then have a hard work ethic, it's insane," Dykhouse said. "Fast forward to track season, and he's better than ever."

Jack takes vitamins and supplements to complement his diet, as certain foods — such as red meat — upset his stomach as a side effect of bile irregularities.

"He has the cleanest diet of any kid I know," said Harding, who added he's never seen Jack drink pop or eat Doritos. "It has to be. His diet is his therapy."

After his journey in and out of hospitals and seeing "too many" doctors, Jack said he wants to go into medicine to be an anesthesiologist.

"I really enjoy my hospital trips," Jack said. "I used to be afraid of needles. Now I like talking to my doctors."

After all, it was those doctors who found the problem's cause and fixed it.

While the light at the end of the tunnel is getting bigger and bigger, PFIC does present a risk of cancer. There's also the possibility of needing a full liver transplant somewhere down the road.

That's just another possibility to deal with for a teenager who's handled it all well so far.

"I just love the school, love the sport," Jack said. "Life is good."

Good, indeed.