Picture living in Hawaii, on the beach. The ocean is steps outside your door, but jumping in the waves is off limits. Until Maxwell came to the Center for Pediatric Airway Disorders at Children’s Hospital of Philadelphia for care, that is what he faced.

Maxwell was born with paralyzed vocal cords (also called bilateral vocal cord paralysis). His vocal cords flopped above the wind pipe in his throat (trachea) when he inhaled, closing off his airway. When he was 2 weeks old, doctors needed to place a tracheostomy tube in his neck, through an opening called a stoma, so he could breathe.

He didn’t need oxygen, but the trach tube opening had to be exposed so air could flow in. That meant time on the beach was stressful. Wading was OK, but if Maxwell had fallen into the ocean, water would have gone right into his lungs. “And then there was dealing with the sand, trying to keep it out of his stoma,” his mother, Laura, says.

What treatments will help?

No cause of Maxwell’s bilateral vocal cord paralysis was ever found. “It’s a one-in-a-million diagnosis,” says Jake, his dad. “We thought he won’t ever have use of his voice and would have to have a trach his whole life.”

Laura is an emergency room nurse and Jake is an ER physician, so they didn’t have trouble managing the care that comes with a trach — suctioning, keeping the stoma clean, dealing with the heightened risk of infections. But they also were determined to find ways to improve his quality of life. They dug into medical journals to learn what treatments might help Maxwell.

“Dr. Zur’s name kept popping up in articles,” says Laura. Karen Zur, MD, is Chief of the Division Chief of the Division of Otolaryngology at CHOP, the Director of the Pediatric Voice Program and Associate Director of the Center for Pediatric Airway Disorders. When Laura checked with other otolaryngologists, they “raved about her.”

Their local otolaryngologist, Susan Tan, MD, reached out to CHOP to arrange for a consultation. Dr. Zur worked with Dr. Tan to have some preliminary diagnostic tests done in Hawaii. After reviewing them, Dr. Zur felt she could help Maxwell.

CHOP, here we come!

Maxwell was 18 months old when the family first came to Philadelphia in July 2018 to see Dr. Zur in person.

After examining Maxwell, the Airway team had him undergo a “triple scope” to determine treatment options. This procedure, done under anesthesia in the operating room, combines a microlaryngoscopy and bronchoscopy (to examine his vocal cords and trachea), a flexible bronchoscopy with bronchoalveolar lavage (to collect cultures) and an upper gastrointestinal (GI) endoscopy (to examine the upper GI tract and rule out reflux). Dr. Zur saw he also had a granuloma (a buildup of extra tissue) in his trachea and removed it.

While he was still under anesthesia — it’s always a goal to have as few anesthesia episodes as possible — he also had a laryngeal electromyography (EMG), a test where a very fine needle is placed in the vocal cords to measure the electrical activity produced by voice box muscles, which is then analyzed to determine nerve function. It confirmed Maxwell’s bilateral vocal cord paralysis.

About vocal cord paralysis

Time for surgery

The best option for him was a double stage airway reconstruction. The first stage is the surgery; the second is trach removal. It would be a delicate procedure. A piece of Maxwell’s rib cartilage would be placed at the back part of his larynx (voice box) to act as a splint, pushing the vocal cords apart enough to let air through. The family returned to CHOP nine months after his diagnosis was confirmed for the reconstruction.

“We asked a lot of questions, and Dr. Zur was so patient, answering every one of them,” Laura says. “The tricky part was to give him room to breathe and not to interfere with his voice.”

After his earlier Philadelphia visit, Maxwell had been using a speaking valve on his trach opening and, with the help of a speech therapist, was just getting the hang of using his voice, although it was very soft.

Maxwell’s airway reconstruction was on April 10, 2019. “It was perfect. There was enough space between his vocal cords that he could make sounds and he could still breathe through his nose and mouth,” Laura says.

Maxwell spent a couple days in the hospital before they headed to Laura’s Aunt Ellen, who conveniently lives in Bucks County, about an hour away from the hospital, where they stayed between appointments and while Maxwell healed. And on May 18, his trach was removed. He could breathe safely through his nose and mouth. Laura and Maxwell spent a total of two months in the Philadelphia area, with Jake flying in for the decannulation.

Unfortunately, the stoma did not close on its own, as often happens when a child has had a trach for a long time. In the summer of 2020, with the COVID-19 pandemic in full swing, Dr. Zur checked his stoma during a telehealth visit while the family stayed in Hawaii. Jointly, they decided a surgical closing was necessary. The family came back to CHOP in September 2020 for the surgery, which also included removing scar tissue.

“Throughout everything, the whole Airway team was so supportive and responsive,” Laura says. “They have a combination of competency and confidence that is really reassuring.”

Bright future includes swimming, shouting

One of the first things Maxwell did when he got home was to dunk his head in the ocean. “His whole life he’s been told he had to keep his head out of the water,” Laura says “Now he can swim with his friends, and he loves it.”

These days, his voice is strong, “normal for a 4-year-old,” Jake says. “Since his vocal cords don’t vibrate, he won’t be able to control is tone enough to sing, but we never thought he’d be where he is today.”

“CHOP has changed his life, changed our whole family’s life," Laura says.

In his first years, Maxwell also needed to avoid games and exercise that could have dislodged his trach. Now, he’s always riding his bike, scooter or skateboard when he’s not playing with his Transformers.

“When he exercises strenuously, you can hear stridor (a whistling sound) since his vocal cords don’t open,” Jake says. “I noticed it a few days ago when he was on the trampoline. But he was jumping on the trampoline! And that’s wonderful.”