It takes a team effort to limit the effects of bronchopulmonary dysplasia (BPD), a serious lung condition resulting from a baby’s lungs not developing properly in the womb or after premature birth.

This is where the Johns Hopkins Children’s Center Bronchopulmonary Dysplasia Center team comes in.

The BPD Center provides specialized inpatient respiratory care for infants born prematurely with breathing issues, and outpatient support for children and adolescents already diagnosed with BPD. The team also offers bedside consultation at partner locations Mount Washington Pediatric Hospital and Kennedy Kreiger Institute, both located in Baltimore.

Each year, an estimated 50,000 babies in the United States develop BPD, also known as chronic lung disease of prematurity. It’s the most common complication of premature birth.

Risk factors show before birth, but a diagnosis of BPD is not made unless the child still needs breathing support at 36 weeks gestation. Given the urgency, it’s complicated timing that requires planning ahead of the diagnosis.

“There is a lag time before the formal diagnosis is made,” says pediatric pulmonologist J. Michael Collaco. “Our goal is to intervene in the window that we have before that point. Then, once the disease is established, we try to minimize the severity of it and manage the long-term consequences."

Providing Rare, Specialized Collaborative Care

Coordination and Care During Hospital Stays

For inpatient care, the team meets babies at high risk of BPD in the NICU within the first month of life to understand the baby’s condition.

“We focus on optimizing lung health and nutrition early on, even before a formal diagnosis of BPD,” says pediatric pulmonologist Samar Atteih. Atteih adds that the team supports the babies, families and primary teams in decisions on respiratory and ventilation strategies, nutritional support and medication needs.

“Meeting the families that early is important,” says neonatologist Kartikeya Makker. “So we can say, ‘Your baby has a high risk of developing BPD. This is what it means now, and this is what it means years down the line.’ That level of preparing families for the long-term is very meaningful to them.”

To maintain continuity of the child’s care, the pulmonology team follows these children from the NICU to the pediatric unit. The team also helps develop a care plan for when the baby goes home, as babies with BPD often continue to have breathing problems at home, and can require respiratory medications or home oxygen equipment.

This collaboration of NICU and pulmonology, and the seamless handoff between specialties, is not commonly found among other similar programs. “There’s no other place in Maryland where you can get your child’s care transitioned to pediatric pulmonology out of the NICU with that transition being managed in house,” says Collaco.

“A lot of programs do have BPD teams for NICU with pulmonology consultation, but not many are as integrated as we are in partnering on prevention and management of BPD rather than providing just consultation services,” adds Makker. “That really adds to the value, both from patient care and patient satisfaction.”

Continued Care After Hospital Stays

The outpatient clinic sees children with confirmed BPD and those who were born prematurely with respiratory issues during infancy. Children can be referred for evaluation from local NICUs, subacute facilities and pediatric practices.

Children born prematurely often have other medical needs, so the team collaborates with Johns Hopkins pediatric services including otolaryngology, cardiology, gastroenterology and home care nursing, as well as the child’s primary pediatrician. Having the child’s care coordinated this way allows for more effective management across conditions.

Transition into Adulthood

For many children with BPD, their respiratory symptoms improve with age and growth. But some have chronic lung disease that requires ongoing treatment with antibiotics and steroids, emergency department evaluations and admissions to the hospital. The outlook for long-term lung function can be lower in children and adults with a history of BPD.

Because of this, the team may continue to follow patients for care up to age 22, and work with adult clinics at Johns Hopkins in transitioning them to adult care. This convenience of in-house transition from pediatric to adult care is rare for most BPD programs in the country.

“I think we provide really nice continuity for these kids who are very vulnerable,” says pediatric pulmonologist Brianna Aoyama. “Families have a lot going on as they are transitioning to life at home, and there’s a nice sense of ‘This is a person who I’m going to get to know and who is going take care of my child.’”

Learning More About BPD Through Research

The team is also active in the latest research on BPD. But learning about the long-term effects has been challenging, as preterm children discharged from the NICU remain an understudied group. To answer some of their questions, the researchers turned to their patients and families.

In 2008, the team began using in-clinic questionaries to collect information from patients about what made their child’s breathing better or worse. They compiled this information in an anonymous registry, which has in turn helped provide families with more accurate estimates of treatment risks and benefits.

The team also combines information with other leading medical centers around the world, an initiative they co-founded called the BPD Collaborative. “It allows us to harness ideas from physicians and scientists at other medical centers, as well as pull larger sets of anonymous data together to answer more difficult questions,” says Collaco.

The team’s current research has looked deeper into topics like the increased risk of complications in children with BPD, barriers to subspecialty follow-up, and the links between physical growth and lung growth during early childhood.

“We hope our ongoing work will provide novel insight into some of the early life issues that these children and their families face,” says Aoyama. “And that it improves the care that they receive to hopefully improve lung health for a lifetime.”

Medically reviewed by: J. Michael Collaco, MD, PhD, Brianna Aoyama, MD, MHS, Samar Atteih, MBBChBAO, Kartikeya Makker, MBBS