When doctors transferred him to The Children’s Hospital of Philadelphia, they weren’t even sure he’d survive the trip. But they knew CHOP’s Newborn and Infant Chronic Lung Disease Program was Evan’s best chance for survival.
Today, the bubbly 14-month-old is home with his family in Linwood, NJ.
“The doctors on the chronic lung team figured out what Evan needed and when he needed it,” says his mom DonnaLee Stickle. “His condition was like a complicated puzzle and they had the key.”
Beginning the journey
Things weren’t always so complicated for the Stickle family. DonnaLee’s pregnancy with twins Evan and his sister Avery was uneventful until the amniotic sac around Evan ruptured. Doctors anticipated the babies would be born prematurely, and gave DonnaLee steroid shots to help the twins’ lungs develop more quickly.
On Dec. 1, 2013, the twins were born at Pennsylvania Hospital in Philadelphia. The babies were only 30 weeks gestation and were small, but they appeared fine — at least at first.
It was actually Avery who first showed signs of distress. Doctors discovered she had a cardiac condition called aortic valve stenosis and transferred her to CHOP for surgery.
Then Evan began struggling to breathe. He was intubated, put on a ventilator, and began receiving low-levels of oxygen therapy.
“At the time, they thought he just needed a break. His lungs were very immature,” DonnaLee says.
But Evan’s condition got worse. The breathing support measures and oxygen levels regularly used to treat premature babies just weren’t enough to keep his lungs fully functioning.
His care team helped him the best they could — switching to a high-frequency oscillating ventilator to increase pressure in his airways, increasing his oxygen levels until he was breathing 100 percent oxygen, and adding nitric oxide therapy — yet still he struggled.
“The doctors at Penn told us there was nothing more they could do,” DonnaLee says. “They said Evan’s best chance was at CHOP — that CHOP’s chronic lung disease experts would give him a fighting chance.”
Transfer to CHOP
At CHOP’s Newborn/Infant Intensive Care Unit, a team of skilled doctors — including David Munson, MD, Huayan Zhang, MD, and Kevin Dysart, MD — was waiting to meet him. They completed an initial assessment and discussed immediate goals for his care. And because of CHOP’s partnership with Pennsylvania Hospital, the team knew everything about Evan’s care to-date.
“Evan’s lung disease was very severe,” says Dr. Zhang, medical director of the Newborn and Infant Chronic Lung Disease Program. “He has underdeveloped air sacs and vessels in his lungs and also has soft airways (tracheobronchomalacia). He needed constant pressure to keep his lungs and airway open.”
Despite a bleak diagnosis, DonnaLee said she and her husband Matthew grew optimistic once Evan was at CHOP. “We felt that these doctors would find a way to help Evan,” she says. “We had hope again.”
After stabilizing Evan, the chronic lung team — made up of neonatologists, nurses, respiratory therapists, dieticians, pharmacists, social workers, physical therapists, occupational therapists and speech therapists, all of whom have specialized experience in caring for children with CLD — began the complex process of figuring out the best way to treat him.
“The CLD team at CHOP focuses on the child’s overall well-being, not just the chronic lung disease,” Dr. Zhang says. “Our challenge with Evan was to figure out the best support we could give him so he could continue to grow and develop.”
As with many complex conditions, there are risks involved with the different courses of treatment. Oxygen therapy, for example, can be a lifesaving treatment, Dr. Zhang says. But it can also have negative side effects. It’s up to the chronic lung team to weigh the risks and benefits of each therapy for each individual patient.
“Care has to be coordinated yet individualized,” Dr. Zhang says. “One size does not fit all.”
CHOP’s CLD team meets weekly at each patient’s bedside. Depending on the child’s condition, the team may ask other specialists — such as pulmonologists, cardiologists or otolaryngologists (ENT) — to join the discussion. Together, the multidisciplinary team reviews each child’s case and creates a customized care plan for that child.
Rising to each new challenge
The team’s initial goals for Evan included weaning him from nitric oxide, decreasing oxygen therapy and extubating him.
“It took a couple months to wean him off the nitric oxide,” DonnaLee says. “The doctors had to move very slowly so his body had time to adjust.”
Evan continued to take small steps forward. As he grew and gained strength, oxygen therapy was slowly decreased and he was extubated.
Physical, occupational and speech therapists next began to focus on building Evan’s motor skills. Months of lung and respiratory issues had taken their toll; though Evan was 5 months old, developmentally he was still very behind.
Unfortunately, non-invasive respiratory support without the breathing tube — even at a high level — wasn’t enough for Evan. After a few weeks of therapy, Evan continued to struggle to breathe and wasn’t making as much progress as the CLD team had hoped. They began discussing ways to increase his respiratory support to keep his lungs and airways open.
One option was tracheostomy surgery, in which doctors would insert a tube through Evan’s neck into his airway (trachea), giving him a more stable and flexible airway that would allow him to breathe more easily with ventilator support. DonnaLee and her family were initially hesitant; they had hoped Evan could avoid surgery.
“We’d been talking about a trach for a while, but it was Dr. Fox (William W.] Fox, MD) who finally convinced me it was the next step for Evan — that it would help him improve developmentally,” DonnaLee says. “That’s when we realized this journey was going to take a while. Evan wasn’t going to get better overnight.”
On June 6, 2014, Evan underwent surgery to implant the flexible tracheostomy tube. The tube was connected to a ventilator that provided Evan with airway pressure to keep his airways and lungs open.
Over the next few months, Evan grew bigger and stronger. By the end of the summer, he was transferred from the N/IICU onto the Progressive Care Unit at CHOP, and later to Weisman Children’s Rehabilitation in New Jersey. The CLD team helped coordinate the transition of his care to CHOP’s extensive network of specialists to help him with other health issues as they developed.
Home at last
On Jan. 13, 2015, Evan finally went home.
Today, he is quick to smile at his parents and loves spending time with his sisters. He’s fascinated by the talkative Maddelee, 3 — who insists on kissing Evan every morning — and his twin sister Avery, who tries to play with him by tugging on his respiratory and feeding tubes.
Evan still has extensive medical needs. He receives continuous positive airway pressure through his trach to keep his lungs open and, for now, needs 24-hour nursing care.
“It’s been a long road and our journey isn’t over yet,” says DonnaLee. “But right now, being home and surrounded by family is the best medicine Evan could have.”