When a child is born, there is joy, hope, and excitement for the parents. But when they hear a cry that is not normal, note the look of concern flashing across the physician’s face, or see their child struggling to breathe, what should be their happiest days turn into scary, worrisome days.
They strive and then find a team of specialists to provide answers and solutions to regain that joy, hope, and excitement. But what happens when that occurs to you twice?
This is the story of one family’s trials and triumphs with airway disorders and the experts who utilized innovative care to help them. This is a story of strength, perseverance, hope, and a little bit of magic.
A diagnosis of BVFP
JC was born at 37 weeks at an outside hospital. At birth, he had increased work of breathing, stridor, and respiratory distress that required immediate intubation. He was transitioned to his local NICU where he was eventually extubated to CPAP.
Unfortunately, JC continued to have distress and increased oxygen requirements. The outside hospital ENT evaluation noted bilateral vocal fold paralysis (BVFP). Despite all attempts at noninvasive ventilation, JC received a tracheostomy at 5 weeks old. His parents received trach training but felt uncomfortable with tracheostomy changes since he would cry during them, so they went to their local ENT to have it performed.
The family was part of an online community for children with a tracheostomy and received the recommendation to seek out Ian Jacobs, MD, Director of the Center for Pediatric Airway Disorders at CHOP. Upon researching the center — a CHOP Frontier Program — the family learned that it has a multidisciplinary team comprised of ENT physicians, nurse practitioners and nurses, pulmonologists, GI, speech/feeding specialists, and social workers, all of whom are leading experts in neonatal and pediatric airway disorders.
A key component of the center is the Neonatal Airway Program. Together, these two programs have specialized in airway issues for 20 years, including subglottic stenosis, tracheal stenosis, laryngeal clefts, vocal fold paralysis, and chronic tracheostomies. In fact, over the last 13 years, 400 tracheostomies have been performed at CHOP in infants under 1 year of age.
At their office visit, Dr. Jacobs performed a flexible laryngoscopy, confirmed BVFP, and presented the options to the family: A) letting JC grow and see if function returns to the vocal folds, or B) letting JC grow and the possibility of performing a posterior costal cricoid graft vs. lateralization.
With either option, JC would still have to rely on his tracheostomy tube until he grew older. Along with BVFP, JC’s medical history included subglottic web, subglottic cysts, gastroesophageal reflux, and eosinophilic esophagitis (EoE). Through repeat aerodigestive endoscopies (meaning ENT, pulmonary, and GI performed their endoscopies under 1 anesthesia) and airway evaluations, JC grew and thrived.
As JC’s mother later shared, “We were supported by our family, but we were also supported by the team.” Eventually, he started having some vocal fold function return, creating a larger airway aperture. JC was decannulated by 3 years of age, avoiding an airway reconstruction.
A repeat diagnosis
About a year after JC had his tracheostomy removed, the family welcomed a full-term, normal pregnancy second son, BC, who was stridulous at birth. He was transferred to his local NICU where the local ENT physician noted BVFP. MRI of the neck, EKG, and ECHO were all normal.
The chief neonatologist of the local NICU had concerns, and the recommendation was a tracheostomy. However, the parents wanted to give BC a chance since he was PO feeding, slowly gaining weight, and had no cyanotic or desaturation episodes. He was discharged home with close follow-up with his pediatrician.
As BC grew, his stridor got louder, his weight gain slowed, he vomited frequently despite Zantac®, and had intermittent mild retractions. The family made an appointment with Dr. Jacobs. Despite close monitoring, BC was admitted for nutrition and a complete workup. He eventually ended up a patient of Janet Lioy, MD, Medical Director of the Neonatal Airway Program, in CHOP’s Newborn/Infant Intensive Care Unit.
“It was a mixed blessing that they knew most of the airway team members,” says Lioy. “But the family was overjoyed to be at CHOP.”
The next step was a trip to the OR for a microlaryngoscopy and bronchoscopy (MLB) with another CHOP airway specialist, Luv Javia, MD. Unfortunately, the child was diagnosed with BVFP.
His mother recalls, “I crashed at that moment. I didn’t want to believe it. I remember the airway nurse practitioner started having tears in her eyes, too. And we all started crying. I thought he would need the trach at that point.”
The airway and NICU teams — as well as the family — remembered it was almost a year to the date that BC’s brother, JC, was decannulated.
A new treatment approach
A meeting was held with Dr. Jacobs, Dr. Lioy, the airway team, and the family. It was decided that BC would have a second evaluation in the OR. The plan was if the vocal cords were fixed, then a tracheostomy would be placed. If the vocal cords easily moved apart when palpated with a microinstrument and there were no signs of reflux, then they would consider a fairly new procedure that could avoid a tracheostomy: endoscopic anterior-posterior cricoid split with balloon dilation.
The family was shocked. “Our eyes sparkled when we heard there was one surgery that could avoid a trach,” recalls the mother.
On the day of surgery, the family was nervous. “We had done our research on the procedure,” recalls the mother. “The NICU staff prepared us for what he would look like after the surgery, with swelling and everything. We were familiar with the CHOP surgery and bronchoscopy process. We were a little nervous if it did not work out. However, we kept thinking that JC was a very normal kid, even with a trach, and it went well, so if we have to go through everything again, OK.”
Dr. Jacobs was able to perform the endoscopic anterior-posterior cricoid split with balloon dilation. Postoperatively, BC was kept intubated, under heavy sedation, and in a medically-induced state of paralysis for more than a week. Repeat MLB was performed and within 1 week, the child was extubated and transitioned to PO feeding. “It was so amazing,” says his mother. “It was really like magic to us. It was magical.”
Where they are today
Remarkably, BC and JC are thriving at home without any artificial airway or feeding equipment. JC had his patent tracheocutaneous fistula surgically closed. BC still has mild stridor, but no respiratory distress or retractions, and he is gaining weight.
Although these two boys had the same diagnosis, they had two different journeys, fortunately with the same result. It was the family’s pursuit of experts that led them to the Center for Pediatric Airway Disorders and Neonatal Airway Program. Through the expertise of this collaborative, multidisciplinary team, the family had access to pioneering and innovative care. The family is grateful for these specialized programs.