They mark the time with beads

Baby Maya’s family tracks her journey toward health with a tactile memory tool, By John Church Ogdensburg — Maya Gordon marks every medical experience with a Bead of Courage. Every day in Morristown Hospital, every blood transfusion, every fever, every dressing change, every round of chemotherapy, every day in Children’s Hospital of Philadelphia and every time she comes home again is marked by a specific color bead or charm. Maya doesn’t keep a journal, she is only 11 months old, but her chain of beads is long and growing longer every day. Maya has familial hemophagocytic lymphohistiocytosis. FHL occurs when defective genes are inherited from both the father and the mother. The onset of FHL is usually early in life and can be triggered by an infection. “Maya had a persistent fever and we took her to Morristown Hospital Dec. 17,” said her mother Shana Gordon. “The doctors ran some tests and were fairly certain Maya had FHL. We went to Children’s Hospital of Philadelphia where the diagnosis was confirmed.” The fever was her body’s reaction to an infection but the FHL was allowing her immune response to run out of control. Symptoms include pallor, jaundice, liver and spleen enlargement. “The doctors are continually amazed at how strong Maya is,” said Gordon. “Typically the swelling liver and other organs make breathing difficult and patients are typically put on a respirator. With treatment the swelling dropped quickly and she continued to breathe easily on her own.” Current treatment of FHL is a combination of immunosuppressive drugs and chemotherapy, and that will only achieve temporary remission. To eliminate the condition Maya must have a bone marrow transplant. Maya’s parents, Shana and Kris, are not good candidates as they already carry genetic combinations that combined to cause the disease. A donor outside of the family genetic history is needed. “Doctors have found a basic match and are calling for more refined tests,” said Gordon. Meanwhile Maya has chemotherapy every other week. She still has most of her hair, her color is good and she is very physically active for an 11-month-old. When a compatible donor is available Maya will undergo three days of intensive chemotherapy to kill off her bone marrow in preparation for the transplant via infusion. “The doctors tell us she will be very sick after three days of chemo,” said Gordon. “After the transplant she will be in the hospital for at least a month.” As the waiting goes on Maya’s Beads of Courage keeps getting longer.

There is a lesson to be learned in all of this. I don’t know what it is yet.” Shana Gordon