Lucas' birth was sudden and terrifying for his parents, Betsy and Nehemia. During a routine 36-week prenatal checkup, Betsy learned Lucas' blood pressure had plummeted. He needed to be born — immediately. Doctors ordered an emergency C-section.
“I heard him cry for three seconds, and then I didn’t hear anything,” says Betsy. She wouldn't see her son again for 13 hours.
Only later did she learn that doctors had to resuscitate and intubate Lucas as soon as he was born. Doctors at her hospital in New York hospital diagnosed the baby with chylothorax, a rare lymphatic condition, pulmonary hypertension, kidney reflux and a grade-one brain hemorrhage. Lucas' health was fragile; his future, uncertain.
Sick with worry, Betsy began to frantically research Lucas' conditions and ways to potentially treat his issues. During her search, she found the Jill and Mark Fishman Center for Lymphatic Disorders at Children’s Hospital of Philadelphia (CHOP). The Center provides highly specialized care for children and adults with lymphatic leaks and lymphatic flow disorders.
Betsy reached out to CHOP and heard back the same day. In consultation with Lucas' New York doctors, Lucas' family and CHOP doctors decided to transfer the baby to Philadelphia as soon as he was stable enough to travel.
A ray of hope
“When the CHOP transport team came, I can honestly say my heart burst. I saw hope," Betsy says. "It’s not that I didn’t have any before, but when I saw the team, I felt a peace come over me. I cried tears of happiness because we were closer to a breakthrough for him.”
Lucas’ parents and family live in New York and were unsure where they would stay in Philadelphia while Lucas was being treated. CHOP social workers stepped in to help.
“They helped us find a place to stay at the Ronald McDonald house, and helped me be closer to Lucas during the scary nights and the happiest days," Betsy says. "I needed to let him know his mom was close by.”
Another unexpected source of support for Betsy and her family came from CHOP’s child life specialists, who helped Lucas’ six-year-old sister, Kristen, understand what was happening with her little brother and to not be afraid.
Stabilizing Lucas’ condition
Lucas’ lymphatic system was leaking lymphatic fluid in and around his lungs, leading to worsening pulmonary hypertension (high blood pressure in the arteries of the lungs) and difficulty breathing. The first order of business for Lucas' care team was to stabilize his condition and prepare him for a procedure in CHOP’s Cardiac Catheterization Laboratory to seal the abnormal lymphatic channels in and around his lungs that were causing the leakage.
Yoav Dori, MD, PhD, Director of the Jill and Mark Fishman Center for Lymphatic Disorders, performed an MRI with contrast to identify where the leaks were located, then performed an lymphatic embolization on Lucas. Using a cardiac catheterization technique, Dr. Dori injected a poppy seed oil, which seals the abnormal lymphatic channels causing lymphatic fluid to leaking in and around the lungs.
When the procedure was completed, Lucas was sent to the Harriet and Ronald Lassin Newborn/Infant Intensive Care Unit, where he could be continually monitored by skilled neonatologists, nurses and others trained to help critically ill children.
“It was scary in the N/IICU. Lucas had so many tubes going in and out of him, we just felt helpless," Betsy says. But after watching Lucas for days, she determined he was strong-willed and stubborn — two qualities that could help him survive.
“I just felt in my heart that he was going to make it.”
Because Lucas was intubated soon after he was born and had spent nearly the first three months of his life breathing with assistance, his lungs were weak and prone to collapse.
Lucas also developed an aversion to taking any food or drink by mouth. Because of his intubation experience, he had a strong gag reflex. Feeding tubes into his stomach and intestines were used to provide nutrition during those critical first few months.
But as Lucas’ condition began to stabilize in the late spring of 2018, the long, slow process of weaning him off respiratory support and feeding tubes began.
Dr. Dori, Nurse Practitioner Erin M. Pinto, MSN, RN, and Pulmonologist Stamatia Alexiou, MD, were instrumental in Lucas’ care and recovery, and helping the family understand the day-to-day details of his condition.
“One of the things I liked about CHOP is that the medical team — from the doctors, nurses, social worker, dietitian and everyone — didn’t mind sitting there and explaining things to me," Betsy says. "They would sit and explain what was happening in detail. It helped me understand what was going on and feel part of the decisions being made about my son."
Over the next three months, Lucas was slowly weaned off a breathing assistance machine. Five days before he was discharged from the hospital, Dr. Alexiou said his breathing had gotten so strong that she felt comfortable sending him home without respiratory support. On August 25, 2018 — when Lucas was nearly 6 months old — he was finally ready to go home.
Support from a lymphatic dietitian
When the family returned to New York, the CHOP team continued to provide support for Lucas and his family. A lymphatic dietitian, Melanie Savoca, MS, RN, CNSC, LDN, provided nutrition and feeding advice and continued support to Betsy — in person, over the phone and by email — as she coaxed Lucas to eat food by mouth at home and wean his tube feeds. CHOP clinicians also trained Betsy how to recognize signs of possible respiratory distress so she could act quickly to help Lucas if needed.
“I took everything they gave me and followed it," she says. "I was persistent.”
Lucas began to drink milk from a bottle in January 2019. By March, he was eating oatmeal, using a spoon to feed himself and growing stronger. He loves the coconut pigeon peas his mom makes. “He is crushing it now. I can’t get him to stop eating,” says Betsy.
On March 1, Lucas celebrated his first birthday. Now 14 months old, Lucas continues to receive Early Intervention services of physical and occupational therapy to help him developmentally catch up to his peers. And intermittently, he returns to CHOP for a check-up with his lymphatics care team.
His mom is waiting to wean him off a gastric tube, which is still in place, though used less frequently since Lucas began eating on his own.
“We are beyond the scary stage and feel confident, especially with the care team we have at CHOP," Betsy says.
“We know he'll grow, run, swim and do all the things he should be doing because of this team. Hope lives at CHOP.”