A Hereditary Rare Disease: How My Father Died of “Benign” Tumors
October 16, 2013
When I turned 50, I was diagnosed with a tumor in my stomach. The surgeon said it was a “benign” GIST. My father and his sister died of benign tumors. I knew tumors would come back. Through reading NIH publications, I discovered if I had a SDHB mutation it would link my Pediatric-Like Gist tumor to my father’s paragangliomas. After two years of clinical trials for DNA mutations, I learned I had inherited dad’s cancer.
My father, George Sherman, was Head of Publications at Walt Disney Studios and wrote comic books in the 1960s. His first tumor was in his abdomen at age 30, the year I was born. Numerous tumors, paragangliomas, returned when he was 40 and riddled his body. First tumors were in his ribs, next his hip, then spine and last–in his brain, killing him in 1974 when I was 16 years old. When my father died he was the tenth known case of his cancer and my Aunt Ann, the eleventh, in 1987. I knew I had to stay pro-active to stay alive . . . these were not benign tumors.
An SDHB germline mutation can create four different types of cancer tumors: paragangliomas/pheos, Pediatric-Like Gist (Gastral-Stromal Sarcomas), Pulmonary Chondromas and Renal Carcinomas. I found a GIST specialist, Dr. Heinrich, at OHSU in my home state and started getting six month CT scans. In 2011, mets were found in my liver and 2/3rds of my liver was cut out. The liver grew back in four months.
I then applied to see some of the top research doctors in our country. I was flown to NIH/NCI in Bethesda, our nation’s medical research center. Since I had an extremely rare cancer, I wanted to give NIH my blood and tissue for future research. I was there for a week of experimental PET scans, MRIs, blood tests and appointments. They didn’t find tumors up and down my spine, which had paralyzed my father from the waist down. They did find that tumors had returned to my stomach, liver and renal area. These were small. They’d have to be watched.
Every six month CT scans tell me whether I’m good for another six months or whether surgery is in order. There is no chemo, radiation or TKI to date that is known to work on these tumors. Patients with “regular” GIST have a series of meds starting with Gleevec. Unfortunately this line of medications doesn’t cross over to Pediatric-like Gist tumors. I’m on that leading edge of monitoring a rare disease yet . . . if tumors are found there is very little that can be done but wait and watch and hope tumors don’t travel to inoperable areas. No known treatment in 1974. No known treatment in 2013. But research doctors love the complications of this mutation. The cards I was dealt.
About the Author: Cathy of Ashland, Oregon
Cathy is author of the book, “A Disney Childhood: Comic Books to Sailing Ships”: a memoir about a Disney childhood being pulled out from under her by her dad’s rare cancer diagnosis and death and her coming to terms with this rare cancer 35 years later.
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