“He’s a tough kid” | Corewell Health

Back pain.

It’s hard for Breanna Kessler to believe this is how a heartbreaking health journey began in January 2021 for her 13-year-old son, Izen Tsitsos.

At the time, Breanna gave Izen a painkiller to make him more comfortable.

A few days passed with her back still sore, but not enough to keep Izen from her daily activities. Breanna dropped him off at school on a Wednesday morning and went to work at a local restaurant near their home in Sturgis, Michigan.

Two hours later, Izen called, asking her to pick him up from school. Her back was in excruciating pain and her legs were now hurting.

By the time Breanna picked him up, he was sweating in pain and could barely carry his backpack to the car. Now he also had pain in other parts of his body.

“It was the weirdest thing,” Breanna said. “I knew it was more than just back pain. I thought, ‘This is not good.’

Unfortunately, she was right.

An uncommon diagnosis

Little did they know it then, but Izen would eventually be diagnosed with a rare autoimmune disease called juvenile dermatomyositis, commonly known as JDM for short. It is an inflammatory disease of the muscles, skin and blood vessels that usually causes muscle weakness. It affects about three in a million people.

That Wednesday afternoon, Breanna and Izen immediately visited her local pediatrician and at 4:30 p.m. the doctor called.

Izen’s blood work revealed that his creatine kinase levels were alarmingly high. He emailed the results to Breanna and told her to take him to the ER immediately and show them the results.

“I was flabbergasted,” she said.

The local hospital they visited that day admitted Izen – and from there he would be in two hospitals and a rehabilitation center for nearly four more months.

Over the days and weeks, Izen’s creatine kinase levels fluctuated, but increased. Meanwhile, Breanna watched Izen grow weaker and weaker.

“He got to the point where he couldn’t move,” Breanna said. “He couldn’t even turn over in bed. His muscles were breaking for no reason. I didn’t know what to expect. I did not know what to do.

Eventually, she said that Izen got to the point where he had trouble swallowing.

Doctors at the local hospital where Izen was a patient consulted by phone with specialists from Helen DeVos Children’s Hospital of Corewell Health. Exactly four weeks after admission, Izen was transferred to Children’s Hospital.

There he came under the care of a pediatric rheumatologist Elizabeth Kessler, MD. (Although they have the same surname, they are unrelated).

She suspected juvenile dermatomyositis and treated him as if he had it, but his case was a bit confusing to her and other experts.

“His case was unusual, in that the majority of children who have muscle inflammation have classic biopsy findings, as well as other test findings. He didn’t have that,” Dr. Kessler.

“He was a rare presentation of a rare disease,” she said.

The day Dr. Kessler met Izen in person at the hospital, he was “very weak”.

“He wasn’t able to sit up on his own or walk,” she said.

While the high-dose steroid treatment he had been undergoing started to work, it was a new treatment, called intravenous immunoglobulin, or IVIG, that really helped, Breanna said. IVIG is a therapeutic treatment prepared from a pool of immunoglobulins (antibodies) from donors, helping to prevent the body from attacking itself and decreasing inflammation.

“It was kind of like a whole new ball game,” Breanna said. “He could actually sit down a bit…I was finally starting to see some light at the end of the tunnel, and I thought maybe there was hope and we could figure it out.”

In March, Izen was discharged to Mary Free Bed Rehabilitation Hospital, where he continued rehabilitation therapy initiated at the hospital, including walking short distances with a walker.

“He was still very, very tired and was still very weak,” Breanna said.

Four weeks later, in April, Izen returned home. He received outpatient physical therapy four days a week and visited the local hospital once a week for IVIG and steroid treatment. He was also taking several medications at home.

Breanna will never forget what happened on a Saturday in May. Izen, who had been a very active and athletic kid, asked to go see his traveling baseball team play. On the way, Breanna’s phone rang. It was Dr. Kessler.

She had contacted Lisa Rider, MD, a pediatric rheumatologist and head of the environmental autoimmunity group at the National Institutes of Health, in Bethesda, Maryland. She is a leading expert on juvenile myositis.

“Dr. Rider called her back instantly on a Saturday, and then Dr. Kessler called me,” Breanna said.

It was confirmation of the diagnosis they were both looking for: juvenile dermatomyositis.

“I felt a little relieved because we finally had a diagnosis,” Breanna said. “Now we can deal with the problem and work from there.”

Invested in his own care

Dr. Kessler recommended eight rounds of chemotherapy for Izen at Helen DeVos Children’s Hospital.

“An overactive immune system was the cause of the severe inflammation in his muscles, and chemotherapy was combined with additional drugs to target his immune system,” Dr. Kessler said.

Before that, however, Breanna had another difficult conversation with her son: chemotherapy could end his chances of biologically having children. So the doctors offered her the opportunity to participate in a fertility preservation research study in Chicago. He decided to do it.

Chemotherapy came with its own challenges: hospitalizations for an infection and later a blood clot in her leg, Breanna said.

But the treatment returned him to the active boy he had been, playing baseball and basketball again.

“Where he was up to now, it’s like a whole new ball game,” Breanna said.

He is now on steroids and IVIG every six weeks, and Dr. Kessler monitors his blood work every two months. He is also on two other immunosuppressive treatments, Dr. Kessler said.

She is happy with her progress.

“I think the unique thing about Izen is that he was really invested from the start,” Dr. Kessler said. “He knows his medications. He follows his laboratories. He asks questions. His understanding is more than I would expect for someone his age. The fact that he has a good understanding of everything and then seeing it improve along the way is very gratifying.

She credits the hospital’s coordinated care and multidisciplinary approach to her care, including neurology, hematology, infectious diseases, and physiotherapy.

And of course, his beloved nurses, including Jaclyn Kukulies, RN.

“Izen and her mother are amazing people,” Jaclyn said. “They have been so on top of his care from the start. I have never met a child like Izen, who is so involved in his care and eager to learn. He defends himself, which is exceptional for a child of his age.

She said Izen and Breanna exude positivity.

“They were treated a lot and they handled it all with such grace,” Jaclyn said.

Breanna said she tries to keep life as normal as possible through everything.

“I never really put our lives on hold,” Breanna said. “We just kept going. I had no choice.”

It meant maintaining a busy schedule for her 17-year-old daughter, Bella, who plays travel volleyball and recently accepted a scholarship to play at university.

“You just have to focus and keep your mind on what you want to go on,” Breanna said.

Izen got back into sports and even went to summer camp with the help of her friends, she said.

“I just want to make sure he has the best life possible,” she said. “I let him tell me what he can and can’t do. He’s a tough boy.

Meanwhile, they continue to partner with Dr. Kessler as she figures out what to do next with her treatment. Although no one knows the cause and a relapse is possible, they do everything possible to avoid this.

“We love it,” Breanna said of Dr. Kessler. “This whole team is amazing. For her to reach out and seek other doctors to help her… She was trying to do her best for Izen.

So they fight.

“I don’t want this to destroy our lives,” Breanna said. “I want us to be able to destroy it.”