It was more than 7 years ago the word cancer entered my life. It was not invited nor did I ask it to stay.
Through the use of the internet I’ve been able to ask hard questions of even the most versed researchers. The doctors have answered me honestly they don’t have a clue to why tumors start to grow or how to stop them except by surgery. This was the same case with my father. Unfortunately he didn’t have the imagining we have today to look for the little ones thus attack them before they get too big and unmanageable.
Every 3-4 months my husband and I drive to a GIST expert in Portland Oregon a five hour drive from our house. In one day I get scans, evaluation and next four month treatment plan. My last appointment was yesterday. I’m going to give a detailed account of that appointment here for those who are also following SDHB germline cancers.
February 10, 2016 Portland left 4:30 am for 10 am PET scan up the hill. This requires parking at the lower campus and taking a tram to radiology. I added 45 minutes to our travel time so I wouldn’t panic and there was some last minute rush hour traffic as well as Brian missing a turn and having to go down a one way street the wrong way in order to avoid getting back on the rush hour traffic freeway. I only thought twice on this trip that we could have had a major traffic accident thus I have acquired the habit of just closing my eyes. Good think I’m not driving.
First stop at OHSU is the bathrooms then tickets to the tram. One can “ride the tram” for free but one can also say they are visiting a patient or have an appointment up the hill and thus get a free tram ticket if desired for the Disneyland part of this adventure.
PET scans take 1 hour mainly because after they inject one with a radioactive isoprobe (in my case FDG) a patient then waits in a quiet room with a warm blanket for an hour for it to distribute throughout the body especially to the tumors greedy for sugar. Then a full cup of water because they like a full bladder for imaging. The actually PET is similiar to CT (maybe same machine, I’ll have to look that up) with 15 minutes of back and forth through the “doughnut machine” where I pretend I am at the beach or on the “Inner Space” ride at Disneyland.
Back down the tram hill for a blood draw. 3 vials. And then I can eat. I’ve found a diet that is currently allowing food again. Last year I’d just fast on this day until we got back to Ashland at 10 pm to avoid my overactive nervous system which triggered diarrhea. Something is working better in my system and I’m not sure if it is my added supplements or the medical marijuana that is currently very high in CBD (which works in epilepsy thus maybe also on my out of control nervous system).
After lunch we settle in for two hours until appointment in Dr. Heinrich’s waiting room where I now bring a pillow for a short nap. I met another Gist patient, Paul, a school teacher. He was full of questions for a “wildtype gist” especially “How can you live without knowing Gleevec can help?” (Gleevec a targeted chemo that has literally saved the lives of those with “regular” gist. It is just not effective on my SDHB mutation which is a broken Kreb’s Cycle, regular gist has an oncogene, c-kit, that can be targeted and thus this chemo works for those with that mutation.) Dr. Heinrich was running 30 minutes behind before I met with him. I’m sure he was running an hour behind after my appointment.
We looked at the PET scan. No new tumors and he couldn’t really tell size from the PET but they appeared to be only a little growth. We’ll order a PET and CT next time so we can measure tumor size. I’ll also scheduled with my surgeon, Dr. Billingsley, to verify these can be removed, and when, depending on tumor size, do we need to switch from “wait and watch” to “action”. The procedure being offered is ablation which burns them out and he’ll cut the ones not suitable for burning. The ablation has to be planned before they get too big but also making sure that any new growths are included in the procedure.
We then discussed my theories. This was the first time I was excited that Dr. Heinrich seemed on board with where my research has been leading. We still don’t have an SDHB gist cell line (that was what I was trying to accomplish last surgery with getting fresh tumor tissue to research labs). But there are some SDHB cell lines for renal cell that he is going to work with. He has an SDHB lab and said he consults maybe 20ish patients including their families (which more than I thought). I will start sending patients his way. I’d been hesitant before because “Gleevec” kept coming out as part of my game plan, a part I wasn’t interested in pursuing. He didn’t mention Gleevec at all except when he agreed with the high price of these medications (Gleevec sells at $100,000 a year and costs $160 a year to make). The pharmaceuticals say it costs $1 billion to bring a new medication to market. NIH is receiving a 1 billion research funding increase with Moonshot to cure all cancers . . . This budget increase won’t go far but that didn’t stop me from writing Joe Biden and Dr. Collins (Head of NIH) to put what monies they could back into the research labs that work with SDHx: Dr. Stratakis, Dr. Helman, Dr. Pacak, Dr. Linehan, and Dr. Metzler’s labs.
We then discussed the research done on my tumor tissue from Columbia University. He said he’d relook at the report and send it to me but felt all the solutions were chemo agents that hadn’t been tried on SDHB and he doubted any would work. Not having a mouse model limits the knowledge whether any of these chemos could be effective.
I brought in two theories I wanted to explore with him. The first was if a real hypoxia situation could trigger tumor growth. Something triggers the good part of the gene to go bad. Dr. Heinrich was skeptical but I gave him correspondence I recently had from Dr. Baysel who studied low oxygen levels were a trigger in the Andes. His research lead to the discovery of the SDHD mutation in 2000. I think Dr. Heinrich will think more about this as a possible real trigger.
My second concept involved bypassing the broken Kreb’s Cycle, Complex II, with supplements. He didn’t think any supplement could help. No matter how much air you surround a flat tire with, it is still going to be flat unless you can figure out how to get the air inside the tire. This is the key we can’t figure out.
Another theory was bypassing the mutated Kreb’s Cycle and using the glycolysis cycle to achieve more energy. I feel my Bud Light is part of this equation and showed him my charts explaining this theory of increased NADH – NAD+ ratio. I think again it was “outside his original box” of thought but didn’t advise me against it. The fact I gained 10 pounds and feel I’m functioning at 90% is enough evidence in itself to “don’t change what’s working”. I think there is probably a substitute for Bud Light that would also accomplish this glycolysis but I don’t know what it is yet. I never thought fresh vegetables (my favorite foods) could almost kill me last summer . . . after two months of chronic diarrhea I remembered people die of dysentery more than cancer. I haven’t touched a fresh fruit or vegetable in a year and have my digestive system is back under control. Very strange upside down thinking but that is what I need to keep doing.
Last was the science of gene mutations. There are two types of DNA mutations. The most common is that the cancer tumor itself was a “bad mitosis” (replication) that lead to damaged DNA and as it divides more and more until we have a tumor we can see on a CT scan. Inherited mutations (called germline) are rare. A germline/blood mutation means every cell in your body, not just the tumor cells, have a good gene from mom and a bad gene from dad. The good gene has to go bad for tumors to grow. At the moment they feel most cancers are not inherited. Maybe 20%. I think that percentage is low and as more gene mutations get recognized we will see a larger population of patients whose grandfather had a pituitary cancer and the granddaughter is growing paragangliomas and they are linked by the same gene mutation.
When my first tumor was removed and labeled “benign” I was lucky not to believe my local surgeon and that a paper had just been published by Dr. Stratakis linking gists with paraganglioma by means of a SDHB DNA. It did take me two years and two research studies to get this accomplished but my instinct proved correct. What I didn’t know that every cell in my body with this mutation is operating fine (at least at half speed) and it is only the tumors that have lost the good half of the gene leading to tumors. These types of tumors can’t grow if there is oxygen present. I then suggested how we get more oxygen into my blood to “ward off” potential tumor growth. My iron level is low and I feel iron supplements might increase blood oxygen. A theory I will continue with.
I asked if my asking for tumor tissue to get to other research institutions was a problem as a researcher. He said “No” but time and money is involved so it does need to have the proper paperwork with “Who is paying” included.
My “Rogue” tumor tissue was received at NIH Monday for a complete genome. Yes! My June surgery I had a panel done by Columbia University though Dr. Califano’s lab. Dr. Heinrich did not have that report/analysis with him and was going to look for it. He felt at the time it was a list of chemo agents that had just not been tried on SDHB and he was not going to be the one to start experimenting with these unknown agents on a patient. I appreciated that.
Another theory is hypermethylation of “good” DNA then leads to damaged DNA. This is what the researchers think drives the Carney Triad kids, a methylated SDHC gene. There are some “demethylation agents” being tested for leukemia which we will watch. He hopes NIH does a study on 5-aza so we’ll know its affect on SDHx patients. He wouldn’t want me on any initial trial because of the strength of this medicine destroys good cells along with the bad ones.
Conclusion: Susceptibility to tumors is what the gene mutation brings. Actually growth of tumors is a “trigger” yet to be discovered. Possibly hypoxia including “altitude”, as Dr. Baysel’s research suggests. This is the part of the equation I think the most about and I felt like Dr. Heinrich was also looking for this as well as what agents might slow or stop growth.
My theories were considered and I felt a successful visit on two levels, my tumors are stable and Dr. Heinrich is now on the same page as I am in trying figure out triggers. I
n June we’ll repeat yesterday with an added CT scan so we can measure tumor growth precisely. I’ll also met with my surgeon to get an estimate of the time frame before I’d need surgery (his off the cuff estimate was a year). I felt positive that an honest, respected and real discussion was accomplished at my appointment. I’ll reconsider the supplements I take in terms of cost verses outcome. If these have helped with the diarrhea then they’ll be no changes. I can guarantee that the L-Methylfolate at 20 mgs a day has helped more with depression than my Lexapro. I challenge others with mood swings to try this supplement for five days. I knew immediately that it helped me go from daily tears to barely cried since starting it. This positive reaction I feel is because I have a second gene mutation, MTHFR, the folic acid system and NADPH to NADP+.
Another fun discovery. On the way home, a long drive staring at 4:30 pm with Portland rush hour traffic and our wanting to be “home” hours before we would be I took one of my cannabis pills. Turns out I can get “high” and was quite amused for a bit on the three long mountain passes where dragons with blinking red eyes that lived on most mountain tops. Then there was a cartoon movie I made out of the characters driving the car in front of us. I truly LOL for many miles.
Plans for the immediate next four months is reserving time to be with daughter and granddaughter, continuing with my research and mentoring others, and finding an opportunity to visit my son and brothers in San Diego and making sure Brian, my faithful and diligent caregiver, is supported in his needs. Yesterday could not have been easy on him either. I’m doing something right although others would look at my lifestyle as totally “out there” . . . I’d much rather be outside the box than having chemo dripped in my veins. Feeling positive.