Naelani was born on Christmas Eve, in San Antonio, Texas. She was a small baby, just 4 pounds, 4 ounces. Naelani was born with a condition known as truncus arteriosus, in which the heart has just one large artery carrying blood from it, rather than the normal two. Her parents, Alexis and Terrance, had learned of the heart problem in a routine ultrasound, weeks earlier.
As a result of her condition, Naelani’s blood did not have as much oxygen as it should have as it circulated through her body. When she started feeding, she became symptomatic. She couldn’t nurse and breathe at the same time. Naelani was put on oxygen to support her breathing and was scheduled for surgery.
Ongoing medical complications
The procedure to reconstruct Naelani’s heart was performed when she was a month old. But a new problem was discovered soon after. Abnormal blood tests led to the diagnosis of DiGeorge syndrome, a genetic condition also known as chromosome 22q11 deletion for the affected gene. In Naelani’s case, the syndrome had left her with a greatly weakened immune system.
Naelani’s next challenge was nutrition. She wasn’t feeding normally and remained in the hospital on a nasogastric (NG) feeding tube for several weeks. Her medical team suggested placement of a gastrointestinal tube (G-tube). They expected the change would make feeding easier and allow her parents to take her home.
Abdominal surgery triggers lymphatic leaks
Naelani had the G-tube placed in March. At the same time, the surgeon performed a procedure called Nissen fundoplication to reduce reflux. After the surgery, Alexis noticed pink fluid leaking from the site where the tube entered her abdomen. A week later, as plans were being made for her release, she noticed that Naelani’s belly was swollen.
“She was so swollen she could barely breathe,” Alexis remembers.
Naelani’s medical team tapped her abdomen to drain the fluid, which they determined to be a leakage of chyle, the fluid of the lymphatic system. Two days later, they drained more. Since the fluid buildup was continuing, a drain was placed in her abdomen. Naelani was found to be leaking close to half a liter of lymphatic fluid every day.
An ultrasound was done to look for the source of the leak, but was inconclusive. Naelani’s formula was modified to remove fat, which can decrease the volume of lymphatic fluid, and she was given medication to decrease the fluid. Neither had much of an effect.
“Her doctors said, ‘We have an option,’” Alexis recalls. “'We can try to get you to CHOP.'’ I asked them what CHOP was, and they said:
“It’s the Children’s Hospital of Philadelphia, and it has the only program in the country to repair this kind of leak.”
Innovative treatment at CHOP
The Center for Lymphatic Imaging and Interventions, a joint program of Children’s Hospital of Philadelphia (CHOP) and the Hospital of the University of Pennsylvania (HUP), has unique expertise in treating lymphatic leaks. The center’s team has treated hundreds of children and adults with lymphatic leaks and lymphatic flow disorders, making it the largest and most active treatment program of its kind in the world. The center is led by Yoav Dori, MD, PhD, a pediatric cardiologist at CHOP, and Maxim Itkin, MD, an interventional radiologist at HUP.
After Naelani’s case was reviewed by the Lymphatics team, arrangements were made to transfer her to CHOP. The transfer date had to be moved back when Naelani contracted cytomegalovirus (CMV), a common congenital viral infection. With her weak immune system, Naelani became very sick.
After recovering from her infection in May, Naelani was transported by air ambulance to Philadelphia. Naelani’s leak was significant, making it challenging to pinpoint the source of the leak.
Her medical team performed a surgery called a laparotomy, so that they could identify the source of the leak. In preparation, they changed Naelani’s formula to increase, rather than decrease, the lymphatic flow in order to make the leak more visible. They found the leak near the site of the Nissen procedure, and they were able to seal the leak with a special glue. The procedure worked to stop the leak, and the flow of milky fluid stopped.
A return to good health
When Naelani was well enough, she was transported back to San Antonio, where she spent an additional month in the hospital to stabilize her breathing and feeding.
Naelani was 7 months old when she finally came home from the hospital. She had a G-tube for feeding and required supplemental oxygen at night.
“As soon as she got home she started changing,” says Alexis. “She could do more. She started sitting up by herself.” Now a year old, “she’s the happiest, craziest little baby. She talks a lot. She loves to dance. She does a little shimmy and claps her hands to music.”
Alexis and Terrance are grateful for the breakthrough in Naelani’s care they found at CHOP.
“They have some of the best doctors in the world, and we have a special place in our heart for Erin Pinto, MSN, RN, CCRN, FNP-BC," says Alexis. “My child got the best of the best there.”