Ja'ir Breathes Easier, Thanks to Versatile Team at Cincinnati Children's
Diagnosed with a rare disease, young Ja'ir was running out of options until a team of doctors, respiratory therapists and interventional radiologists came up with a plan.
Frequent nose bleeds. Shortness of breath. Weight loss. Janay Gray knew something was wrong with her son, Ja'ir.
It was the fall of 2021 when the seventh grader developed a persistent respiratory illness. Doctors in their hometown of Indianapolis treated Ja’ir for sinus and ear infections, as well as pneumonia.
But when it didn't go away and symptoms worsened, Janay took her son to a nearby emergency department where doctors discovered that Ja’ir’s kidneys were beginning to fail.
“Ja’ir was a typical kid and seemed healthy to me until one day he got an ear infection that wouldn't go away,” Janay said.
Diagnosis of Rare Disease, ‘Scary time’ for Family
Blood samples indicated possible abnormalities and a follow-up X-ray and CT (computed tomography) scan revealed granulomas (small areas of inflammation) in his lungs. Doctors diagnosed Ja’ir with a rare autoimmune disease called granulomatosis with polyangiitis (GPA), which most commonly affects the sinuses, lungs and kidneys.
“It was a scary time for our family,” said Janay.
Formerly known as Wegener’s granulomatosis, GPA is a type of vasculitis. This group of disorders causes inflammation of small blood vessels. As the blood vessels narrow, tissues and organs in the body don't get enough blood-rich oxygen.
In Indianapolis, Ja'ir received treatments that included aggressive immunosuppressive drugs and corticosteroids. He seemed to have a positive response to these therapies. After a month-long stay at the hospital, he returned home where he continued with school homebound instruction due to his weakened immune system.
Referral to Cincinnati Children’s for Multidisciplinary Procedure
By February 2022, Ja’ir’s shortness of breath had gotten worse. He was now winded walking from one end of his house to the other.
Janay and Ja’ir returned to the hospital, and doctors discovered that he had progressive, diffuse bronchial stenosis. This widespread narrowing of the lung’s tiny airway passages does not respond well to medications.
Ideally, Ja'ir would undergo a bronchoscopy, a procedure using a small, flexible telescope that is inserted through the nose and extends down the back of the throat and through the windpipe to provide doctors with a view of his lungs and airway passages.
Unfortunately, endoscopic tools are built with adult patients in mind. The bronchoscope, which is about the size of a cell phone charging cord, was too large for Ja'ir.
The Indianapolis team referred Ja’ir to Cincinnati Children’s and pediatric pulmonologist Erik Hysinger, MD. Here, he would have access to safe, precise care in the hybrid operating room (OR). The space contains advanced radiology technology such as X-ray, fluoroscopy (live X-ray), ultrasound and CT. In this environment, surgeons from various disciplines work alongside interventional radiologists, who specialize in using these technologies. This combination of multidisciplinary skills and expertise benefits patient care.
“The hybrid OR allows this multidisciplinary team approach to managing complicated situations,” Hysinger said. “At Cincinnati Children's, we’re the pioneers in doing interventional pulmonology work and trying to help kids with complex airway disease to have better outcomes.”
An X-ray image of Ja'ir's chest.
While working in the Hybrid OR, experts from Pulmonary Medicine and Interventional Radiology collaborate during surgery. Pictured: John Racadio, MD, in foreground with Erik Hysinger, MD, performing the bronchoscopy.
Scaling Down an Adult-Sized Procedure
By the time Ja’ir arrived at Cincinnati Children’s, his kidneys and inflammation were improving. But pulmonary function testing showed that his lung function had dropped from the expected 97% to 37%. A bronchoscopy showed the narrowing of his airways wasn’t improving.
A lung transplant was the next—and last—option. But that wasn’t possible because the underlying issue of the autoimmune disease wasn’t resolved. Because of GPA, the bronchial stenosis would eventually return, even with new lungs.
Without effective breathing support, doctors worried that Ja’ir wouldn’t survive. Hysinger worked with otolaryngologists (ear, nose and throat doctors), respiratory therapists and interventional radiologists to find a creative solution.
“We needed to open these very narrow airways. There’s no tool designed to do it for children, and adult tools are too large for kids,” Hysinger said. “We had to find a way to circumvent that. To do it, we collaborated with interventional radiologists to use some of the really small tools they use in blood vessels to apply to the airways.”
Multidisciplinary Solution to Ja’ir’s Care
Hysinger led a multidisciplinary team to perform a balloon dilation to open Ja’ir’s bronchial airways. He enlisted the support of others within Cincinnati Children’s, including: John Racadio, MD, director of interventional radiology, research and innovation; Nicole Hilvert, program manager of the hybrid operating room; and Carolyn Wallace, lead respiratory therapist for bronchoscopy. Together they developed a novel technique for treating pediatric bronchial stenosis.
The first step in the process was to identify which airways could be restored for improved lung function. To do so, they used imaging to track a special type of inhaled gas through Ja’ir’s lungs and find areas to open.
Some sections of Ja’ir’s airway were completely blocked by scar tissue. To break through the obstruction, the team used X-ray imaging to guide a transbronchial biopsy needle to these areas. To open the airway, they maneuvered an angiography balloon to these areas and inflated it. Finally, they injected steroids and performed cryotherapy to keep these areas open.
“We used the same techniques that we use all the time in the vascular system and applied it to the lungs,” Racadio said. “With our tools and help of X-ray, we could guide our catheter further into the lungs than it could go with the bronchoscope or with a traditional balloon.”
“We used the same techniques that we use all the time in the vascular system and applied it to the lungs,” Racadio said. “With our tools and help of X-ray, we could guide our catheter further into the lungs than they could go with the bronchoscope or with a traditional balloon.”
Since February, Ja’ir has undergone 14 procedures to re-open the many areas of narrowed and closed airways. His lung function has nearly doubled. Hysinger and his team will continue to monitor Ja’ir every few months.
“The success in this case relies so much on the collaboration and multidisciplinary care,” Hysinger said. “Without that, we would not have been successful.”
Living Life to the Fullest
After receiving homebound instruction for most of seventh grade, Ja’ir, now 13 years old, is glad to be back in school with his friends. Janay describes him as a typical teenager. He loves playing video games, socializing with friends and going on dates to the movies. He’s even playing pickup games of basketball again.
Throughout his ordeal, Ja’ir has stayed brave, happy and funny. He often cracked jokes to keep his medical team and his loved ones laughing.
“I was unfazed that I was diagnosed with a life-threatening disease,” Ja’ir says. “I knew there was a possibility I could die, but I’m thankful that my mom and dad noticed I wasn’t feeling like myself. I’m happy they noticed before it was too late. Besides the doctors at Cincinnati Children’s, my parents and family are my real heroes.”
Ja’ir is already setting career goals: Recently he told his mother that he wants to become a life coach. He wants to help others get through difficult times in life, as he has done.
“He’s been through a lot within this last year, and he is not letting the disease define him,” Janay said. “He still has goals in mind and talks about the future. This disease is not a death sentence for him.
“I’m so grateful for the entire medical team at Cincinnati Children’s. They are amazing. I'm just happy that they were able to develop a procedure that could help my son. Otherwise, he likely would not be here with us. I’m forever indebted to them."
Ja'ir with his mom, Janay.