Davis’ lymph system — a major part of the immune system — was leaking fluid into his chest and abdomen at an alarming rate. A prolonged loss of lymph fluid can result in devastating malnutrition and immunological disorders.
“His doctors were doing everything possible,” says Davis’ mother, Baiba Janus. Doctors had drained the fluid and tried to close the leaks, but despite their best efforts, Davis was getting sicker and sicker. He was iron deficient, he wasn’t getting enough protein or vitamin D, and he was developing a metabolic disorder.
“The complexity of Davis’ condition exceeded the capability of the medical technologies in Latvia, and there were no options left for us there,” says Baiba.
Searching for an overseas option
Davis’ physician began looking for doctors outside of Latvia who could help. During an Internet search, he came across a paper about a specialized treatment for lymphatic leaks, called thoracic duct embolization. The article had been written by interventional radiologist Maxim Itkin, MD, director of the Center for Lymphatic Imaging and Interventions at Children’s Hospital of Philadelphia and the Hospital of the University of Pennsylvania (HUP). Davis’ doctor immediately contacted Dr. Itkin for advice.
Dr. Itkin and his fellow Lymphatics Center director, pediatric cardiologist Yoav Dori, MD, PhD, discussed Davis’ case and offered to help. They planned to use an advanced imaging technology they developed, called dynamic contrast MR lymphangiography (DCMRL) to map the anatomy and flow of Davis’ lymph system and pinpoint the leaking vessels. This enhanced imaging technique provides a clearer image of the lymphatic anatomy and lymphatic flow than conventional lymphangiography.
After the analysis of these images on screen, the team can also simultaneously perform a selective lymphatic embolization procedure to seal the leaking ducts.
“With this technique, we can see exactly what we’re doing,” says Dr. Dori. “That means we can precisely target the ducts that are causing the leaks, while sparing the rest of the lymph system.”
“when davis' doctor told us about the options at chop, we had hope again,” says baiba.
The journey to CHOP
The Janus family knew Davis needed to be in Philadelphia, at CHOP. Their first step was to get in touch with the Hospital’s Global Patient Services (GPS) team. The Janus family’s main contact was Jaime Powers, BSN, RN, CCRN, a clinical nurse coordinator for GPS.
“When international patients are referred to CHOP, GPS coordinates their care. We review medical records to connect families with our specialists, assist with financial clearance and provide families with resources for travel and housing in Philadelphia,” explains Powers.
“We were very excited, but also nervous, before the trip to Philadelphia,” says Baiba. “We had many questions and concerns about our stay there, especially because my English is limited. But I used the translation services, which really helped with the understanding between me and Davis’ doctors.”
“Even if an international patient comes to CHOP with a family member who speaks English, our policy is to provide them with a medically trained interpreter,” says Powers. “The medical information the doctors are giving to families is often very complicated and we want to ensure it is interpreted precisely.”
Coordinated, family-centered care for a complex case
The Janus family arrived at CHOP on Dec. 16, 2014. Dr. Dori and Dr. Itkin began the MR lymphangiography contrast procedure they typically use in patients with lymphatic leaks, but didn’t find much to indicate where Davis’ leaks were coming from.
“In Davis’ MRI, the central lymphatic system appeared not to be involved in the disease process, which was surprising to us,” says Dr. Itkin.
Because tests had revealed Davis had an enlarged liver, the pair decided to perform imaging of his liver lymphatic vessels. Using a technique called liver lymphangiogram, they successfully found the leaking lymphatic vessels.
With the source of the leaks identified, Davis’ team successfully sealed off the leaking vessels during a lymphatic duct embolization. Because of the location of the leaks, physicians in the Division of Gastroenterology, Hepatology and Nutrition, including pediatric gastroenterologist Elizabeth Rand, MD, were also called in to consult on Davis’ case.
Within one week of the embolization procedure, Davis’ leaks had stopped. Within two weeks, he was back to eating a normal diet and beginning to gain weight. He was also regaining enough strength to enjoy his stay at CHOP.
“Davis really liked the playroom, the food, and music therapy, where he got to play the guitar,” says Baiba.
By Davis’ third week at CHOP, he was told he could head home to Latvia.
Heading home, staying close
Since returning to Latvia on Jan. 7, 2015, Davis has been doing well — he recently went swimming and looks forward to getting back on his bike. The Janus family also remains in close contact with the team at CHOP, as his doctors are still unsure why the lymphatic leaks occurred in the first place — or whether they’re likely to return.
“Even when our patients return to their home countries, they’re still part of our CHOP community of patients,” says Powers, who has remained the Janus family’s link to Davis’ healthcare team in Philadelphia. “Families often have questions weeks or months later and we can help them get back in touch with their physicians.”
“Davis already has nice memories of his time at CHOP. Dr. Itkin and Dr. Dori are the best in their field and Global Patient Services at CHOP helped us with everything,” says Baiba.
“davis already dreams about going to philadelphia again sometime in the future — as a healthy kid next time, of course!”