For five years, Nikki Adamy and Adam Buchanan had never heard their son, Mason, laugh.
Not that Mason wasn’t a happy little boy. He was always smiling. He played with his big brother, Aidan. He had fun at preschool. He loved his toys and going on vacation. But the tracheostomy tube that enabled him to breathe stopped him from laughing out loud.
‘Floppy airway’ limited breathing
He was born with Down syndrome, and one complication is low muscle tone. In Mason’s case, his trachea had low tone, called a “floppy airway,” which prevented him from breathing normally. Doctors near his York, PA, home determined he needed a tracheostomy — an opening in his neck kept open with a tube — to breathe, to survive.
“At first, I didn’t understand,” Nikki says. “I knew it would be life changing. We talked to another doctor, and he said it plainly: Mason might be OK for a while, but the minute he got sick, it could be life-threatening. Of course, we went through with the surgery.”
Mason was 2 months old at the time of the tracheostomy. His doctors initially thought he would only need the trach tube for a year, that he would grow out of his floppy airway and the tube could be removed, a process called decannulating. When he still needed it at 4 years old, his family sought a second opinion at the Center for Pediatric Airway Disorders at Children’s Hospital of Philadelphia (CHOP).
The Airway Center offers a procedure called laryngotracheal reconstruction that can strengthen and widen a patient’s windpipe. The Buchanans were hopeful Mason was a candidate for the surgery.
Triple scope shows promise
The Airway team of otolaryngologist Ian Jacobs, MD; pulmonologist Pelton Phinizy, MD; pulmonologist Joseph Piccione, DO, MS; and gastroenterologist Matthew Ryan, MD, evaluated Mason in CHOP’s Airway Clinic.
“At first it was a little overwhelming, all those doctors,” Nikki says. “Who’s who? But they make it easy on you. You stay in an exam room, and they come in one at a time. They’re very friendly and informative.”
The team recommended a “triple scope.” This procedure, done under anesthesia in the operating room, combines a microlaryngoscopy and bronchoscopy (so Dr. Jacob could examine the vocal cords and trachea), a flexible bronchoscopy with bronchoalveolar lavage to collect cultures (so Dr. Phinizy could examine the lower trachea) and an upper GI endoscopy (so Dr. Ryan could examine the upper GI tract). The benefits are the child undergoes anesthesia just once and all three specialists gather information needed to collectively determine the best treatment.
“I’ll never forget sitting with Adam in the parent waiting room when Dr. Jacobs came in, shook our hands and said, ‘I look forward to decannulating Mason,’” Nikki says. “I cried I was so happy.”
Step by step to decannulation
There were several necessary steps before Mason could have reconstruction surgery and be free of his trach tube. First, he needed his tonsils and adenoids removed. “That made so much sense since he’s had strep throat many times,” Nikki says. They came to CHOP for the surgery.
Next, Mason needed a sleep study to determine if he had sleep apnea, a common condition for children with Down syndrome. Indeed, the study showed severe obstructive sleep apnea, meaning he momentarily stopped breathing frequently while asleep — 138 times in an hour!
Sleep Center doctors prescribed CPAP (continuous positive airway pressure) treatment. Helen Ku, MSN, CRNP, a nurse in the Sleep Center, explained everything, showed Mason the mask and the CPAP machine. “We made a big deal out of it, calling it his super hero mask,” Nikki says. “He did really well; it didn’t bother him. Helen says a lot of children cry and scream, but Mason, while he didn’t love it, left it on.”
The Airway team scheduled another triple scope to determine exactly what was needed to widen Mason’s airway. This time, Dr. Ryan also introduced what’s called an impedance probe, a device that measures any reflux, or stomach acid that bubbles up. “He explained that reflux will mess up the graft before it has a chance to heal,” Nikki says. “Mason did have some reflux and was started on medicine to control it.”
Single-stage laryngotracheal reconstruction
With his tonsils and adenoids out and his sleep apnea and reflux managed, the team was ready to reconstruct Mason’s airway.
First, Dr. Jacob removed a piece of Mason’s rib cartilage. “He explained to us that rib cartilage is the same material as his trachea,” Nikki says. “They form it in a circle so when they put it in his airway and suture it in place, it props his airway open.”
Doctors removed the trach tube and closed his tracheostomy, then put in place a new breathing (intubation) tube from below the surgical site up through his nose to keep the reconstructed area open while it healed.
“Nurses told us that many kids are so uncomfortable being intubated they have to be sedated or have their hands restrained,” Nikki says. “Mason didn’t try to pull it out once. Nurses called him the rock star of the ICU.”
A step forward, then back, then forward
Four days after the surgery, doctors removed Mason’s breathing tube. He played games with his brother and cuddled with his grandmother. “I heard a funny sound, and I realized Mason was laughing!” Nikki says. “It was amazing.”
But later that day, Mason was in sudden pain and struggled to breathe. Nurses thought he might be having an allergic reaction. “He was panicking and nurses called a code,” Nikki says. “Everyone rushed in. It was very scary, just like in a movie.”
They intubated Mason again through his mouth, and rushed him back to the operating room. It ended up that the area where his rib cartilage has been removed had poked a hole in his lung when Mason, who had become constipated due to the pain medication, tried to push out a bowel movement. One lung had collapsed.
Dr. Jacobs inflated his lung, and Mason was on the mend. X-rays showed the graft in his airway was healing well. He began working with a speech therapist to relearn how to eat and swallow again. He went home three weeks after the surgery.
Life without a trach
After another three weeks of recovery at home, Mason started kindergarten. He has speech therapy to help him catch up with his speaking, both word formation and volume. Overall, life is easier for him and his entire family.
“I don’t have to worry about little kids going up to him and grabbing his trach tube,” Nikki says. “When we leave the house, I don’t have to have the bag of medical supplies and the suction machine. He can even taste things better now. He is enjoying the independence.”
And now Mason is able to have fun like other kids. He can play in a ball pit and climbing tubes since he’s less susceptible to picking up germs. On their Myrtle Beach vacation, he could go under water for the first time. He’s able to wrestle with Aidan. He laughs — a lot.
“Hearing Mason laugh is the most wonderful sound ever,” Nikki says. “And it’s all because of the care he received at CHOP.
“We had been so discouraged all those years when we had been told he’d have the trach tube for life — and he would have if he wasn’t a candidate for airway reconstruction. Dr. Phinizy told us the Airway Center specializes in kids like Mason, kids who thought they were facing a lifetime with a trach. It’s wonderful that CHOP is a place that truly changes children’s lives. Mason is proof.”