Adrian Santos couldn’t stop catching colds. At one year old, the Grafton toddler had a new ear infection every three weeks.
His mother wondered if the chronic cough indicated asthma. His pediatrician suspected pneumonia. A pediatric cardiologist detected fluid around his heart.
When a “water pill” to decrease the fluid didn’t work, hospitalists drew a sample of it. Lab tests showed that it contained chyle, fluid leaked from Adrian’s lymphatic system — a sign of an extremely rare condition called lymphangiomatosis.
“The initial shock was hard, but after reading what little there is online about this disease I was terrified,” says Adrian’s mom, Diana Westren. “I learned that life expectancy was about six years from date of diagnosis.”
Adrian was in the hospital for nearly two months while pediatric specialists in Akron, Boston and Cincinnati tried to confirm the diagnosis. Lymphangiomatosis is difficult to identify because it is so unusual and its symptoms are vague.
The condition is caused by an abnormally developed lymphatic system. Lymphatic vessels throughout the body expand and interconnect, invading other tissues and organs. Depending on where and how much the vessels take over, lymphangiomatosis can cause coughing and breathing difficulties, bone pain and fractures, digestive disorders, and kidney or liver problems. The condition is often misdiagnosed.
After Adrian’s long hospital stay, he was still having a hard time breathing. Diana wanted a second opinion, so she made an appointment at Cleveland Clinic Children’s. X-rays showed a large amount of fluid around Adrian’s left lung. He was hospitalized right away.
While in Cleveland Clinic Children’s, a team of pediatric hematology/oncology specialists confirmed that Adrian had kaposiform lymphangiomatosis — distinguished by the type of lymphatic cells involved.
“Adrian had chest tubes to drain the fluid and daily chest X-rays to determine whether his disease was progressing,” says Diana.
Because lymphangiomatosis can be debilitating and may affect different areas of the body, it requires a team of specialists to provide the best care. In addition to the hematology/oncology specialists, Adrian’s team at Cleveland Clinic Children’s included a pulmonologist, a nutritionist, interventional radiologists, and physical and occupational therapists.
“It’s not only the medical support that has been so wonderful, I feel that Adrian’s doctors really care for him. The nursing staff is especially excellent."
“Standard treatment involves chemotherapy, immunosuppressants and steroids to inhibit the growth of extra lymphatic pathways,” says pediatric hematologist/oncologist Grace Onimoe, MD. “That’s what Adrian is receiving now. We also work to improve his nutrition and provide blood transfusions, as needed.”
Adrian’s treatments will be lifelong, but his medical team is cautiously optimistic.
“All necessary care is on board,” says Dr. Onimoe. “We hope Adrian will continue to respond to the medications and that his lesions regress. We also are investigating new interventional radiology approaches to close off his abnormal lymphatic channels.”
Now three years old, Adrian is back at home and doing well. He is fascinated by animals and loves trips to the zoo and aquarium. He also is an avid movie-watcher and enjoys repeating lines and re-enacting scenes with his toys.
“We are just trying to maintain, and allow him time to grow and be as normal as he can be,” says Diana.
Adrian has a catheter on each side of his chest from which Diana drains fluid from his lungs twice a day. She monitors the amount of fluid drained as well as Adrian’s blood pressure. Every few weeks, Adrian returns to Cleveland Clinic Children’s for chemotherapy. He also has regular X-rays to check the status of his condition.
Diana also praises the Lymphangiomatosis & Gorham’s Disease Alliance (LGDA) for support and education. In July 2017, Adrian’s Birthday Bash & Fundraiser for LGDA raised approximately $4,000 for lymphangiomatosis research. The family plans to continue to host the event in July 2018 and beyond.
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