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| The tumor-suppressing protein healthy genes produce to prevent tumors from growing. |
Some people who know me might be thinking, “you get a lot of tumors, girl!” Yes I do.
This new tumor will probably mean yet another surgery. And this will be the fifth major surgery for me (if I count tonsils and wisdom teeth, it will technically be 7 surgeries). The primary reason for all these surgeries is a genetic defect that is known as Von Hippel Lindau Syndrome or VHL.
VHL occurs when someone is born with a faulty tumor-suppressing gene. I actually have a family-mutation-number-code-thingy on a genetic testing report: 596 delG VHL exon 3 L201X. Sounds impressive, doesn’t it?
Regular folk are born with a backed up hard drive. They have two of every gene. And as you age, eat pesticides, go through hormonal changes, get x-rays, use your cell phone, stand in airport body scanners, get hit by random cosmic rays etc. normal healthy genes occasionally experience mutations. But if one gene is messed up, there is a back up. People with VHL don’t have a back up of one particular gene. And just like a computer file, not having a back up kind of sucks when your original gets corrupted. When VHL folk get a mutation in our only good tumor-suppressing gene, we get tumors.
Depending on our family tree (the specific mutation) we get tumors in all kinds of places: adrenal glands, kidneys, pancreas, spleen, brain, spine, reproductive organs—even eyes and ears. I have had VHL related tumors in my adrenal glands and currently have them in my pancreas, brain, spine, and eyes. The ones in my brain, spine, and eyes have been monitored for a while and aren’t on the worry list. They don’t become cancerous, and only become an issue if they get too big and start rupturing or pressing on things.
These pancreatic tumors are troublesome little buggers. They can become cancerous. As I understand it, when the tumors are over 2cm the chances of cancer start to pick up, and once they hit 4cm it’s almost guaranteed. The first time I got a pancreatic tumor they didn’t find it until it was 4.2cm.
Which, considering how many darned scans I get, is totally bogus to me. I went through all the trouble to get in that cold narrow MRI tube once every couple of years, get IV injections of dye, listen to the kechunk-kechunk-kechunk of the machine and some disembodied voice instructing me to hold my breath, or not swallow for 4 minutes (which is darned hard; if someone tells you not to swallow, it’s like telling you not to think of pink elephants)—and after all that they totally missed my pancreatic tumor.
In fact if I had not moved from Canada to the US and accidentally gone for a scan before I was due, I would probably be dead right now. The fantastic radiologists at Johns Hopkins caught my tumor and looking back at the Canadian scans, from only months before, clearly saw the same tumor, the same size on scans that were called “clean” by my Canadian doctors. I don’t know how the Canadian radiologists at St. Paul’s Hospital missed that, but it certainly shook my faith in the Canadian Healthcare system!
To make things worse, the MRI showed it as a 2cm tumor, but when they did a CT scan it turned out to be 4.2cm. Which shook my faith in opting for low radiation scans!
I was rushed in for a Whipple surgery, which is about as major a surgery as you can get. The tumor was in the head of my pancreas. And you can’t take out the head of the pancreas without taking out 1/3 of my intestines, and the gall bladder. After all this dissection, they reconstructed my bile ducts and reconnected what was left of my pancreas to what was left of my intestine. As serious as that all sounds, I breezed through the surgery--it was the infection I got after that almost killed me.
During the surgery they found the tumor has spread to a neighboring lymph node. Which according to my doctor was “technically cancer”. I use quotes because I kind of feel like I got the cheaters version of cancer. I never needed chemo, I never even knew until after the surgery, I didn’t have any of the typical hardships you associate with cancer. I had it. It happened, and now it’s gone. No sweat.
Though, I did have a scare a few years later when they suddenly saw a new tumor on the tail of my pancreas and also several spots on my liver. My doctors started going into full panic mode. They Talked about taking the rest of my pancreas, leaving me diabetic, and taking “ice cream scoops” out of my liver. None of them would really talk about odds of survival, and they avoided questions about how long I might have to live if this was the cancer. So I suspected it was pretty bad. I looked online and the best I could find was 6 months. I have to say, that I was pretty fricking scared by that.
My surgeon’s office called to book a surgery date. I called them back and left a freaked out message, because I hadn’t even spoken to the surgeon yet. After my freaked out message, the surgeon called to tell me his office had jumped the gun and they wanted to send me for another scan before they booked me for surgery.
This was a pretty cool scan actually. Most scans shoot radiation at your body to detect stuff, but in this scan they inject you with a radioactive substance that is drawn to the tumors, and your body actually shoots radiation at the scanner. I felt like Spiderman!
After the scan showed nothing, the Tumor Alert was suddenly downgraded. They decided that the specks in my liver were “artifacts”, which is the doctor equivalent of a smudge on your screen. Whew!
I sometimes tell people that I like to live my life to the fullest, because I have “really almost died twice”. I wasn’t aware or scared the first time, when my tumor got bigger than 4cm right under the doctors noses, and probably would have metastasized enough to kill me if I hadn’t goofed and scheduled my scan early. Then I got that infection, which hospitalized me for 11 days and almost killed me due to several complications. I wasn’t scared of dying for that one—I wished I would just die at a few low points though. Then the one time I was scared of dying, it out to be a false alarm. So there really doesn’t seem to be a point to being scared. What’s gonna happen is gonna happen, you do your best to cover the bases and cross your fingers for the rest.
This new tumor is less than 2 cm, and was just as hard to spot as the first one. They only saw it because it is blocking my pancreatic duct now (once again, looking back on old CT scans they see it "in retrospect"). They might have to remove my whole pancreas this time, which would leave me an insulin dependent diabetic. Which would totally suck, because I love food and I have a huge sweet tooth.
To date, I have quite an impressive list of body parts removed: adrenal glands, head of the pancreas, gall bladder, duodenum (intestines), fallopian tube, tonsils, adenoids, and wisdom teeth. If I were playing a game of Operation I would be winning!
But even with another surgery looming, I am pretty chill. I usually am. People think I should be scared. And because I am not, they call me brave. But to me it’s not bravery—I am just well informed and confident.
VHL has been known to be in my family for three generations. My grandfather had it; my aunt, uncle and mother had it; my sister and I have it, and now my nephew has it. I have been tested for VHL since I was a baby. When I was in diapers my mom would have to do 24-hour urine collection tests on me—every drop for 24 hours had to be collected. Kudos to her for figuring out how to do that one! I am tested so much, I actually think I have better odds at beating cancers than “normal” folks, because I get scanned from head to toe every year or so, I get to catch it early.
I think of it like I am a pro-athlete always training for the Olympics. Now it’s game time and I am ready to go. I am in my element—familiar turf. I put my toe on the starting line and aim for the finish line. Life would be less complicated if I didn’t have to have surgery, but I am prepared. I know what’s going on, and I have been through it many times before. It’s same ol’ same ol’.
The scariest part is when they put you under. Technically a thousand little things can go wrong during even a routine surgery. As they put you out, you could be experiencing your last moments on Earth! You may never wake up! That freaks me out. But I kind of put off thinking about it until the last moment. Ask me then if I am brave, as I ask the nurse for Valium!
If I were to call anyone out for being brave it would be those who get news like this sprung on them. Those poor folk who are not combat-trained like me, and haven't had their whole lives to prepare. Who never expected to be in battle. And still they carry on. When you have no clue what is happening or why, are trying to play catch-up and learn a ton of medical information, and you can keep a brave face through it all. That is honest to goodness bravery. And of course all of those who have to stand by our bedside. The parents, siblings, and spouses who have to sit in that waiting room while we sleep through the hard parts. The ones we leave behind when things don't go our way. Those are the bravest souls of all.
But for me, it isn’t bravery that keeps me going. It’s just a matter of what I am used to. Just another day with VHL.
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