Can I just Get a Zipper? - #Wattys2015

Bravery?

So I have another tumor in my pancreas. It’s a neuroendocrine tumor. What’s that? Well I like to say it’s the Steve Jobs kind of pancreatic tumor, not the Patrick Swayze kind. Of course they both died, so that probably doesn’t mean as much to people as I think it does. Just know that the Steve Jobs has a more hopeful outcome, especially if you get it caught early, and don’t delay treatment.

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 2 cm the chances of cancer start to pick up, and once they hit 4 cm it’s almost guaranteed. The first time I got a pancreatic tumor they didn’t find it until it was 4.2 cm.

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 2 cm tumor, but when they did a CT scan it turned out to be 4.2 cm. 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.