An update. Since my book came out February 2012 I have had time to continue my journey into a rare cancer and at times thought maybe I could find the pattern that links everything together and solves this mysterious disease. I’ve done a ;year of medical research, learning terms, looking up body systems and got a fairly good grasp on why tumors present themselves. I felt it was then time to take advantage of an offer I’d had made to me 2 years ago . . . to go to the now yearly Pediatric /Wildtype Gist Clinic in Bethesda Maryland. This Clinic is at the NIH/NCI. I’d heard great things about it but wasn’t expecting it to be such a great decision. There are 12 patients from around the world invited for 2 1/2 days of tests and seeing experts in their fields on this rare cancer that I have (that they are still having a problem coming up with a name but at this point I think the doctors are leaning towards SDH deficient Gist . I had two nicknames of my own I’ve named it, “Cancer of the Krebs Cycle” and “Cancer of the Nerve Endings” . . . representing the Wildtype Gists that start in the stomach and then the mets that can be anywhere in the torso to brain. I just returned from a week at NIH. I was in two clinics at the same time. The Pediatric Gist Clinic with Dr. Helman and a Paraganglioma Clinic run by Dr. Pacak. To those who haven’t been to NIH it is an amazing place. I’ll start with my diagnosis and then continue with my thoughts and feelings. The Paraganglioma Clinic was a five day intensive with 2 MRIs and 2 PET scans. The PET scans are to look for functioning paragangliomas/pheos. The doctors then analysis the cell activity a they look for tumors. On Friday I had a short meeting with Dr. Pacak who saw 4 new small tumors. Two back in my liver (which frankly could have been the section they didn’t operate on last year, at least that is my hope), one new tumor in my stomach (damn!) and one near the kidney. Dr. Pacak’s philosophy is to get out the tumors before they reach 3 cm which then turns them into very serious with the possibility of not being able to be surgically removed. There seems to be no current truly successful medication to date except for one participant, Becky apears to be having great success with the IGF-1 Lisitnib inhibitor that is currently closed to new patients but should open up again. There is another medication being tested for renal cancer that the doctors hope will also get approval to be used for Wildtype GIST/Paraganglioma patients. Dr. Pacak thinks my tumors are GISTs not paragangliomas. My understanding was the SDHB germline mutations can produce at least four different tumors. They are all neural based cell cancer: GISTs, paragangliomas, benign lung tumors, and renal tumors. The Pediatric Gist Clinic is special because of the dedicated people involved. The research coordinator, Lauren Long and Dr. Boikos, both went out of their way to help with my anxieties, my endless questions and are very dear caring people. I felt it a pleasure and honor to meet Dr. Stratakis whom I immediately trusted. He has his own SDHx research Clinic at NIH and I’ll enjoy future correspondence with his lab. It was his paper that linked the GIST and paraganglioma together. This was crucial information that started me looking for SDHx mutation tests (which proved I had indeed inherited my father’s cancer). All the doctors, staff and patients are NIH were open, caring, informative. Everyone on campus deals with very rare cancers it was easy to talk to all other patients creating a commoraderie of us in the boat trying to be proactive against the medical sea of current knowledge and future landings. I came away relieved I don’t have tumors up and down my spine (my biggest fear) even though four new small tumors were found. One of the “side effects” of being at NIH that is unique and special is almost anyone wearing a “NIH lanyard ID badge” was willing to talk about their cancer experiences struggles and thus as patients we could support one another. Most all the patients I met were “self referred” meaning they sought out more medical knowledge when often their doctor had just given them six months to live and go have fun! My husband, Brian was with me and learned a lot about the goodness of human nature as well as Wildtype GIST. Since there isn’t any insurance paperwork to do the feel of the hospital was everyone trying to learn and help solve rare cancers. Very encouraging. We just need to try and keep budget cuts from affecting this truly unique institution. I met many patients in “my boat.” The young women Triad patients from my Pediatric/Wildtype Gist website were amazing in stance and beauty. Remarkable to see how well they are doing despite their past and current diseases. We all missed Dr. Kim immensely but he was on a three year fellowship and that ended. Dr. Boikos is now on the three year fellowship and I feel is a deep caring man with lots of knowledge that sometimes others miss because of his accent (he was born in Greece) but is an experienced doctor and will continue to monitor and is dedicated to the health of all those who attended. I was put up at an accommodation on the NIH campus for adult cancer patients called the Safra Lodge. I feel the intent of this lodging has been realized. In the common kitchen patients and caregivers could talk and get and give support to others. There were two amazing women at the Lodge who’d left their family (with young children) to be by the side of someone they’d known sometimes less than a year. These women were my heroes in their own self-sacrifice to be with someone going through something extremely complicated. One had learned the day she left she herself had Stage 2 breast cancer. One woman’s daughter had wires attached to the back of her brain (skull cut open) hoping to catch a seizure thus they’d know where to try to zap the brain to stop them. We had a morning cry to her hoping her daughter had a seizure that day so they could proceed with treatment: “Seizure, Seizure” . . . not the “Good luck” expression those in the “normal” world chant. I found the tests all easy except the for one blood draw, 12 large vials, that left me really weak for a day. The NIH campus is beautiful. This June it was very hot outside (90 with 90% humidity). Hard for me to “visit” DC but we managed slowly coming back to NIH when I ran out of strength. The best part was meeting in person some of the names I’ve written to for years on my cancer list serve, Donna and Jon, Becky, Phyllis and Kara, Gary and his wife Jacqui from London, Patty and Stephanie (such a lovely strong young woman), a new family Taylor age 12 and her mom Kelly, Mark who wasn’t at the social events but is 25 years old from Toronto Canada where his 11 cm stomach gist did respond to Sutent and it is now 7 cm and they are thinking of surgery. Every one we met on campus or off campus was open with their disease, and their fears. I felt we all gave each other ideas, strength, compassion and support to be pro-active in getting the right information on our various rare cancers. I asked at the “head doctor meeting” if my layman’s terms for these Wildtype GIST and Paraganglioma could be called “Cancer of the Krebs Cycle” and “Cancer of the Nerve Endings”. They said that was appropriate. My own situation is I was able to dispel my greatest fear (tumors down my spine). And even though they found new tumors I can go back to Dr. Heinrich in Portland Oregon for scans and have NIH as a support system as well as a place where I can get future care including surgery “for free” and expert advice in the meantime. They have yet to grow a cell line from an SDHB mutated tumor and need fresh tumors to accomplish this. I remember Dr. Kim was willing to drive a 200 mile radius of NIH to collect any fresh tumors for analysis. There was one patient at the Lodge that was having basically what is close to a bone marrow transplant but the new procedure was to take out her own cells out, treat them for the cancer, then reinject them in the tumor sites. Before injecting them the patients would have chemo for 5 days with the intention of killing off the cancer cells along with the good cells. I heard one patient after this procedure was already up and walking around the next day. Very positive experience. 12 hour flights to get back to the West Coast. If all our medical facilities could run as warm and informative as NIH our country would have a great health system.
You wouldn’t know where we were staying was a cancer institute not a vacation lodge in Britain!
adisneychildhood 