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Billy's Bronchopulmonary Sequestration Survivor Story 

Billy BPS survivor

Nearly every set of expectant parents looks forward to their first ultrasound, and the chance to see that little baby that causes so much excitement. Julie and Ben were no different when they went in to see their baby boy, Billy, for the first time. Unfortunately, their ultrasound showed more than just a growing baby boy; their baby had a mass on his lung.

Doctors initially thought their Billy had a CCAM (Congenital Cystic Adenomatoid Malformation), a cystic mass which forms in the lung tissue. Over the next few weeks the mass continued to grow, and Julie was referred to the St. Louis Fetal Care Institute at SSM Health Cardinal Glennon Children’s Hospital.

“The mass kept getting bigger and bigger, and the lungs and the heart were being pushed to the side.  We started to wonder if there was going to be a baby,” remembers Julie. The Fetal Care Institute team met with Julie and determined that the lung mass was actually a rare lung mass known as a bronchopulmonary sequestration (BPS).

A bronchopulmonary sequestration (also known as pulmonary sequestration or BPS) is a piece of lung tissue that develops without being connected to the airways, sometimes inside the lung and sometimes outside of it.

The blood vessels going to the BPS also form abnormally. The BPS does not get blood flow like the rest of the lung. Rather, it steals blood flow away from the body via a separate artery.

The doctors at the SSM Health St. Louis Fetal Care Institute continued to monitor the BPS. They quickly found that the mass was causing Billy to have a large pleural effusion, a condition where fluid fills an area of the baby’s chest, preventing the lungs from properly developing. This worsened the situation by causing Billy’s one healthy lung to be compressed as well, the team knew they needed to intervene to reduce the amount of fluid in his chest.

Julie was scheduled for a fetal intervention where a needle was inserted into Billy’s chest to drain the fluid and give the lungs more room to develop. The first surgery went exactly as planned. After that, all they could do was wait.

The physicians monitored the baby through ultrasounds, and much to everyone’s disappointment the mass continued to grow, and the fluid returned to the chest. “We prepared ourselves for the worst, and didn’t think there was anything that could be done for our baby,” said Julie.

But, the Fetal Care Institute found something that could help. The team was aware of a procedure called laser ablation. During this minimally invasive fetal intervention, a small needle is inserted into the BPS Laser AblationBPS, and a laser fiber is targeted at the abnormal blood vessel going to the BPS. The goal of the operation is to use laser energy to stop the blood flow to the BPS, causing it to stop growing. After the surgery, the BPS steals less blood flow from the fetus, and the heart and lungs start growing more normally as the BPS shrinks in size and the pleural effusion goes away.

This was the only way that Julie and Ben’s little boy would have a chance at survival. The only challenge? The procedure had never been done in the United States.

“We saw the ultrasounds, we saw the size of the mass and how it was impacting Billy. We knew the good and the bad, and weighed our options,” explained Ben. “We decided to take a leap of faith, knowing that if this didn’t work for Billy it may help another family down the road.”

Based on their experience and fetal surgery skills, the team knew they could perform the procedure successfully. But, because this was a new procedure, the case was presented to the St. Louis Fetal Care Institute Scientific and Ethical Review Board. The committee evaluated Billy’s case and felt that the possible benefit of performing the new laser procedure outweighed the risk, given the life-threatening situation.  

“We were on pins and needles waiting for a response from them. At 5 p.m. the phone rang and we were told to be at the hospital at 7 a.m. for the surgery,” explained Julie.

“I barely slept that night I just couldn’t stop thinking about what was coming next. Was I preparing for a baby or a funeral,” she remembers.

The surgery was successfully performed at SSM Health St. Mary’s Hospital - St. Louis. Doctors worked together to guide the needle right next to the feeding vessel to the BPS. The fetal surgeons inserted the laser fiber, and after several firings, the blood flow to the BPS was stopped.

Julie woke up, and after this high risk, new procedure, the only evidence that she had surgery was a small band-aid where the needle had been placed. “The surgery was a breeze, but we were so nervous for the upcoming ultrasounds.”

A few weeks after the surgery Julie and Ben finally received good news at an ultrasound. “The moment when we were able to let them know that the mass was shrinking and that Billy’s lungs were growing was awe inspiring,” explains St. Louis Fetal Care Institute Nurse Coordinator Katie Francis. “The sense of excitement and relief that spread through the room was unforgettable.”

The rest of Julie’s pregnancy was as normal as could be expected. Billy continued to grow and develop. At 39 weeks along, Julie was induced for a vaginal delivery. Since both of the fetal interventions had been needle based, and not open fetal surgery she was able to have a normal delivery.

“We were nervous, they said Billy may need to go straight to the NICU (neonatal intensive care unit) depending on how his lung were after birth,” explained Julie. “Our doctor was there to make sure everything was okay, and along with us was thrilled when Billy started crying when he was born.”

Billy did so well following the delivery he didn’t need any time in the NICU and was able to go home two days after delivery, just like any other healthy baby.

When he was 6 months old Billy had surgery to removeBilly BPS Survivor what was left of the BPS. The team found that Billy’s lungs were entirely normal, and his BPS was very small and scarred, unlike a normal BPS. It is believed that this is because the blood supply to the BPS was cut off during the pregnancy. The team has every expectation that Billy will lead a full, healthy, normal life.

When looking back at the journey Julie said, “We wouldn’t give him up for anything, we fought so hard to get him here and the St. Louis Fetal Care Institute team fought so hard to get him here, he’s just a miracle.”

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