by Julia Fisher
Luka was born on March 4, 2014, the only day of the year that tells you to do something (march forth!) — and coincidentally Mardi Gras.
Yet, soon after his birth, we knew something wasn’t quite right (an early ultrasound had given us a hint, too.) After battling failure to thrive and a growing skin infection despite antibiotic ointment his first two weeks of life, a simple blood test at a general pediatricians office indicated Luka’s absolute neutrophil count was 0. The doctors even repeated the test since “they had never seen a 0 before.”
After the same result repeated, we were sent to UNC Children’s Hospital and met Dr. Philip Roehrs who prescribed G-CSF to stimulate Luka’s bone marrow. Just 10 years ago, this treatment wouldn’t have even been possible. At the time, it was suspected that Luka’s condition may be worse than not having neutrophils, or “neutropenia” given how early he presented and other factors.
Little did we know at this time how extensive his unique condition was, enduring what seemed like endless diagnostic, genetic, lab, and whole exome sequencing testing in his first 12 months of life. From that testing, we found that there were other components of Luka’s immune system that were missing, yet he didn’t fit into the classic SCID (Severe Combined Immunodeficiency) diagnosis. He had four gene mutations one of which was novel and typically associated with another rare disease, HLH (Hemophagocytic Lymphohistiocytosis), and hadn’t been seen before.
And, after several mysterious bacterial and fungal blood infections, low IgG, low NK cell function, and an inability to thrive without IV antibiotics, it was determined that Luka’s bone marrow wasn’t compatible with life. Luka underwent chemo preparation and received a bone marrow transplant from an anonymous donor. Yet, complications post transplant from several viruses and an auto-inflammatory response was too much for his body. He passed away peacefully at the UNC Children’s Pediatric Intensive Care Unit (PICU) on #GivingTuesday, December 1, 2015, surrounded by his Pride in a circle of love.
The Luka The Lion Foundation honors Luka who fought bravely like a lion his entire life. He endured a lifetime of medical treatment to earn a total of 48 feel of Beads of Courage. Luka did this all while teaching us to embody the characteristics of a lion of beauty, bravery, strength, and resilience — and to always remember to roar. Despite his passing away, Luka continues to guide us as we give support kids like him who brave rare disease and their care-giving families so nobody ever feels alone.