Baby's rare disease rocks Shelbyville family

May 30--DECATUR -- Leo Cohan looks like a healthy 19-month-old, with a full head of blonde hair, quick-moving legs and light blue eyes. He cracks a big smile when he runs from his mom and makes her chase him.

But inside, Leo is suffering from the effects of a rare disease called Congenital Lobar Emphysema (CLE). Much of Leo's left lung had to be removed and his right lung has over-expanded, pushing his heart to the right of where it's supposed to be.

Samantha said doctors are currently in wait-and-see mode, hoping his left lung can recover to both help him breathe better and push his heart back into place.

CLE, which causes enlarged air spaces in the lungs, is extremely rare, and even more rare in both lungs. Because of the disease, Leo has to be careful not to expend too much energy, and he has several side effects, including a food aversion that has kept him from gaining weight.

Because CLE is so rare, the Cohans have struggled to find treatment. And while Medicaid covers most of Leo's medical bills, there are out-of-pocket expenses. Bills have piled up, and a benefit held last month didn't produce as much as the Cohans had hoped.

Because of Leo's condition, he needs constant care, and nearly all of it falls on Samantha's shoulders. She had to quit her job as a Certified Nurses Assistant and put nursing school on hold. And with no income coming from Samantha, her husband Jim Cohan was the only source of money, and his job in Shelbyville wasn't cutting it. He left for Freeport, Texas, to find work and just landed a job. But at this point, future plans to reunite the family are in limbo.

"People feel bad for you when you have a child with a disease like this, but I don't think people know what it's like," Samantha said. "I don't think they really think about what the kid is going through and the struggle the parents are going through. It's hard on a relationship. It's hard on a family. It's really hard."

'Really rough'

Leo, short for Leonard, was the Cohan's second child -- Leo has a 5-year-old brother named Sawyer. Samantha's pregnancy with Leo was normal, and though the delivery was complicated -- both of Samanta's children had shoulder dystocia -- Leo was nine pounds, one ounce and appeared to be healthy on delivery.

The Cohans held their newborn baby for awhile and Sawyer was allowed to come up to the room and meet Leo as well. A little less than two hours after Leo was born, at about 6:15 p.m., Samantha said a nurse came in the Cohan's room at Sarah Bush Lincoln Health Center in Mattoon to take vitals and noted that his respiration rate was too high. They placed him in the nursery area, then came back two hours later and said they were going to keep him in the nursery and on oxygen for the night.

At midnight, Leo's pediatrician came in the Cohan's hospital room and said Leo was being taken to Carle Foundation Hospital in Champaign, where Samantha said Leo was diagnosed with transient tachypnea -- short-lived rapid breathing sometimes seen in premature or bigger newborns.

But the breathing problem didn't diminish. On Oct. 31, at four days old, Leo was diagnosed with CLE. His over-inflated left lung was pushing his heart out of position and collapsing his right lung. A day later surgery was performed to remove three-quarters of his left, upper lung -- the area in which CLE had caused the lobes in his lung to over-expand.

"He was in surgery for four-and-a-half hours, and when Jim and I finally got to see him, he was on a ventilator ... that was really rough," Samantha said. "Jim had been holding it together pretty well through all of it, but when he saw Leo on the ventilator, he broke down. I just threw myself on the ground."

Leo was in the hospital for more than a month, and while his breathing hadn't slowed to a normal rate, it improved and he was allowed to go home.

'He just wouldn't eat'

Leo's respiration levels were out of the danger zone, but other complications began to emerge -- mainly, he wouldn't eat, and he threw up what he did eat. At two months old, he weighed less than he did when he was born.

"It was frustrating -- he would go back and forth between eating, then not wanting to eat," Samantha said. "It was like he just didn't have enough stamina to do it. He'd get worn out."

By December, at two months old, Leo would go as many as 12 to 14 hours without eating. Samantha said her pediatrician told her not to force him to eat because that would only make it worse, but by January he was barely eating at all. He went through a couple of trips to different hospitals, where he would eat at first, then revert back to refusing food.

"He just wouldn't eat, and we couldn't figure out why. Nobody could," Samantha said. "They couldn't find anything."

For awhile, Leo had a nasogastric intubation (NG tube) placed -- a plastic tube is inserted through the nose, past the throat, and down into the stomach. Eventually, though, he quit tolerating it.

Finally, Leo had surgery to get a gastronomy tube (commonly called a G button) -- a tube inserted through the abdomen that delivers nutrition directly to the stomach. While Samantha is still trying to get Leo to take food orally, she said 90 percent of his food intake is still through the G button.

Samantha said neither she nor Leo's doctors can say whether or not the food aversion is related to CLE.

"I've tried to figure that out. They have no idea," Samantha said. "There are so many unknowns. They just don't know everything that comes along with it."

Recently Samantha said they've found some flavors he likes -- sour cream, ranch and even habanero.

"We take him to food therapy and they said he's a flavor seeker," Samantha said. "Here lately he's been taking a little more interest in food. We're hoping that's a good sign."

'In tune'

Because so little is known about CLE, finding doctors within driving distance who have experience treating it is difficult. There is a hospital in Houston, near where Jim is working, that has doctors who specialize in treating it, but for now Samantha is hesitant to pull him out of the care he's receiving at OSF St. Francis in Peoria.

"I love his team there -- he has a cardiologist, pulmonologist, GI doctor and a nutritionist, and he also goes twice a month for feeding therapies trying to get him to eat," Samantha said. "The main thing is we got some answers in Peoria. Their doctors have been willing to reach out to doctors who have more experience dealing with it. I feel like they're really in tune with everything going on with him."

It was at St. Francis that Samantha said CLE was found in the upper lobe of Leo's right lung, which had expanded across his chest and pushed his heart over and outward. It's the same thing that had happened when he was born to his left lung, but this time his doctors are putting off surgery.

"For right now it's pumping blood normally and all the levels look good, so they're leaving it alone," Samantha said. "They want to keep him away from a surgeon as long as possible. He just doesn't have enough lung to keep chopping bits of him out."

Samantha said the plan is to do a sleep study this summer to determine whether or not the lung is working or not, and then go from there on what to do next. Surgery may be the only option, and at some point a transplant could be a possibility.

"The doctor wants to give it a chance to grow," Samantha said. "It may decide at age 5 it's going to work, or it may never work. But as long as it's living, they're going to leave it alone."

For now, Leo is on medication and breathing treatments, and is put on oxygen anytime he sleeps or becomes over-exerted.

"We have it ready for him whenever he needs it," Samantha said.

'It's a mess'

Leo has appointments in Peoria at least twice a month, and often more. If he falls asleep on the trips -- and he usually does -- he needs to be on oxygen, but the Cohans' insurance doesn't pay for travel oxygen tanks.

"It's $1,000 for six tanks," Samantha said. "That adds up fast."

It became too much for the Cohans to afford, especially with Samantha unable to work.

"Trust me, I'd love to work. I want to go back to work," Samantha said. "But I was told from the beginning, no daycares, no malls, no Walmarts, nothing. He can't risk getting an infection. I was homebound. I never left. I never went anywhere.

"And then you add in his G button. I've love to have someone come watch him, but you have to have someone who has the training on how to feed him using the G button, and that's not easy to find."

With Samantha at home, it was up to Jim to support the family financially, forcing him out of the area to find work. He left on Mother's Day to find work, and recently landed a job.

"It makes it really hard because he wants to be here, but he can't, and there are days I need a break and can't get one," Samantha said. "Right now it's just up in the air. We're just trying to figure out what to do and the best way to do it. He's getting good treatment at Peoria, but there's one of the best hospitals in the nation in Houston. It's hard to know what we should do."

Sawyer has had his own issues with his brother's illness. From the day Leo was brought home, Sawyer had to take a shower and change his clothes as soon as he walked in the door from the outside world. And as his parents had to spend more time away at the hospital with Leo, Sawyer began to develop separation anxiety.

"He'd never really been away from us. He'd never even really stayed at anyone's house," Samantha said. "But once we started to have to spend more nights in the hospital, Sawyer had a terrible time adjusting to it. He was supposed to go to kindergarten this year but he just couldn't get it together to be away from us.

"And now, with Jim gone, he refuses to talk to Jim on the phone. It seems like he's angrier. It's a mess."

Mary Thompson, Samantha's mom, does what she can for her daughter and grandchildren, but often feels helpless.

"It's pretty tough -- it takes away from Sawyer's time because Leo needs constant care," Thompson said. "And for them to be separated right now -- I hate it. But there's only so much I can do. It's hard to watch your child struggle while she takes care of her child. You can see she's trying her hardest, but she's by herself. It's hard. He's such a beautiful baby. He's an amazing child. I just wish they could find all the answers for him."

jconn@herald-review.com|(217) 421-7971

___

(c)2017 the Herald & Review (Decatur, Ill.)

Visit the Herald & Review (Decatur, Ill.) at www.herald-review.com

Distributed by Tribune Content Agency, LLC.