But It's Not Cancer Right???

Abbie had an end of the year school picnic at the park today.  She's been in the two day preschool class at the same preschool where Carolyn attended 3 day preschool last year.  For that reason there were quite a few Moms there that I had gotten to know from Carolyn's class last year.  Being the socially inept little duck that I am I ended up just mainly chatting with a friend of mine from church (who I'm really enjoying getting to know, by the way), but seeing these people made me think of how much has happened since last year.  When I finally did speak with one of the Moms that I used to chat with last year she was surprised to find out that I'd had a baby.  As I was driving home I thought to myself, "Girl!  You don't know the half of what kind of crazy has happened since I last chatted with you!"

In situations like that I don't usually offer up a whole lot of information about Tomas.  Or, I guess...  I play it by ear depending on the person.  Part of the reason is that it's all just so hard to explain.  And part of that is because of the 'c' word factor. 

The 'C' word factor has kind of followed me around like a rain cloud during this whole experience.  And I realized today that I've been reluctant to give myself much grace partly because of the 'C' word factor.

As I've mentioned before paragangliomas are tricky.  With many masses you take them out and look them on pathology slides and you know 'cancer' or 'not cancer.'  With paras and pheos you don't know unless there is a metastasis somewhere in the body where they don't normally originate.  That can happen years or even decades down the line.  Most of the information out there broadly categorizes them as 'benign neoplasms.'  Most of the time they AREN'T considered cancerous, but basically even with that 'probably not' you still have that whisper in the back of your mind of, "but it could be..."  And then you have the different genetic mutations that come into play.  Past that, from what I've read and from the stories I've heard it seems like when a hard and fast rule is made about these tumors a case comes along to bust it.  So while  the cancer issue is considered to be a factor usually only with larger tumors or with certain genetic mutations there are still cases of people with sporadic disease and/or tiny tumors who have metastasis pop up and end up with malignant disease.  Because of all of that, some people--even some medical professionals--DO consider them cancer from the get go, but in the form of a 'low grade malignancy,' AND there are a lot of varying opinions.  To a point that makes sense to me. 

On top of that, even when they aren't cancerous they still need to come out.  They still represent a malady in the body.  It's still a tumor.  They can still compromise systems in the body.  They can still wreak havoc.  They are still scary. 

That totally confuses people:  I had a tumor.  But the tumor wasn't cancer?  What?!

And in that confusion I find a frustrating feeling:  Sometimes I almost feel like having a paraganglioma is something akin to having the red headed step child of diseases.  Because as a patient you don't fit into anybody's easy little boxes.  You don't even fit into doctors' easy boxes  How many times have been trying to explain Tomas to someone and their eyes glaze over and they say, "But it's not cancer.... right?" 

And in this "right?" I hear a lot of things that aren't necessarily said but may or may not be implied, "So it's not a big deal.  So we'll mentally classify you in the 'worrisome but fixable' category of the prayer chain right next to Uncle Stewy's trick knee and cousin Sophia's infected toenail surgery.  So you really shouldn't be scared.  So you're  really not fighting a hard battle.  So your feelings are dismissed."

I'm the daughter of a woman who died of lung cancer.  Believe me when I tell you I get what a scary deal cancer is.  And I'm glad that what I had is probably not cancer. 

I still had a tumor.  A rare one.  Rare enough that I chose to travel to the other side of the country for it's removal.  I still lived with something inside of me that shouldn't have been for months.  I still agonized about whether or not catecholamines would be secreted when I delivered Alaine.  I'm still wondering what will come of the 'nodule' on the other side.  I'm still wondering if this is it or will there be more.  And I'm still not sure that the 'c' word isn't going to rear it's ugly head. 

Some people with pheos or paras that are secretory have to deal with hypertensive crises, cardiovascular "events," high blood pressure, panic attack sympotms and emotional variability due to the release of catecholamines and metanephrines  from their tumors.  That's not the 'c' word, but you better believe it's pretty darned serious.  People with a genetic mutation and a predisposition to growing these tumors have to be vigilant in watching for symptoms like that even when their original tumors weren't secretory.  And then there's the scariness of having something growing inside your body without presenting with obvious symptoms.  Tomas was one of the non-secretory tumors with no symptoms other than a bump.
In short, it might not be cancer, but it's still scary.

I think about American Cancer Society events and t-shirts that say 'survivor' and I wonder if on the other side of those 'But it's not cancer right?' questions is the implication that I haven't survived something major. I'm sure no one intends for that benign (see how afraid of the 'c' word we are with the language that we use) little question to spur so many wonderances in me.  No one intends to make me feel like my tumor wasn't something to be concerned with.  And really the issue here is me and my own brain and feelings about the issue.  Which leads me to the next paragraph...

Worst of all is the 'C' word factor and myself....  Because I taunt myself with it.  "Yeah it was scary, but it wasn't cancer, so stop it.  Yeah, you got through it, but it wasn't cancer so it's not like it's that big of a deal."  "It's not like you needed chemo or radiation..." quit stewing about it.  I *feel* less deserving of the survivor t-shirt because of the 'c' word factor.  Because if it had been the 'C' word....  THAT would have been something to have survived...  but it's no big deal to survive a 'benign neoplasm.'  And 'Paraganglioma Survivor" is kind of a mouthful. 

So I beat myself up and act like I haven't really been through anything.  I mentally get frustrated with being classified in the 'needed surgery because of an ingrown toe nail category' by others, but I put myself there constantly.  I don't give myself the grace or the space I need to process because always a little part of my brain is saying in a sing-song voice, "But it wasn't cancer."  And I expect myself to not have feelings about it all three months after surgery.

This is a weird disease.  Everyone who has it feels rare and alone as soon as that first doctor looks at them and says, "I've never seen this before....".  When it does swing to the cancer side of things that's absolutely terrifying and even more rare.  When it doesn't, at least from my experience...  We feel almost guilty for that.   It's a twisted kind of survivor guilt, I guess.

But it occurred to me today, I AM a survivor.  I am a survivor of a rare disease.  I survived a year of fear and anxiety and frustration.  I survived a surgery on the other side of the country.  I survived the struggle of  finding a doctor who knew what I was talking about when I said the word 'paraganglioma.'  I survived having a baby in the midst of the what-ifs that Tomas caused.  I survived having something so rare that when you encounter someone else who has had it you have a little mental party because even though you don't want anyone to have to go through something like this, it feels so good to NOT be alone in it.

So in answer to the "Right???" implications and feelings I can say this:  No, so far it doesn't appear that Tomas was cancer.  Just the same, what I went through WAS scary....  It was terrifying.  And the implications of having Tomas and the what ifs that are still out there continue to be frightening.  More than that though I *am* a survivor.  I survived Tomas.  I am surviving the wait for my genetic mutation results.  And God willing I'll keep surviving what ever else Tomas and his minions may throw at me.

(P.S.  No, Still no news on the genetic testing...)

Waiting... Wondering.... Choking on Chicken

We’ve been in waiting mode since getting back from the NIH.  Everything has felt quite up in the air.  First of all, we were waiting on orders.  Where are we going to live?  We’re headed back to Sea Duty.  What will managing Tomasities look like on Sea Duty? Secondly, we’ve been waiting for the genetic testing results.

I was told that I’d be tested for the SDHD/SDHB mutations only as part of the NIH’s research protocol.  I was told that it would take at least two months to get them back. 

It’s been two months and three weeks.  Not that I’m counting or anything.  And it’s not like I wake up every day and wonder if today is the day that I’ll get the call.  It’s not like I hold my breath every single time the phone rings and feel both let down and relieved when the caller I.D. doesn’t say, “Health and Human Svcs.”  It’s not like I’m sending an email to Karen every week saying, “Are the results in yet?” leading her to put me on her top ten list of pain in the tuckus patients.  It’s not like I’m feeling a little nutty about it all.

Really.  It’s not. 

You don’t believe me, do you? 

Last week we got the news that we’d most likely be staying here.  Today we got the hard copy orders.  So that’s settled.  We're gearing up for Sea Duty and with that comes plenty of it's own wonderings and ambiguity, but at least we're not planning a move on top of it all and we know where Andrew will be employed.

So now I just need to know if I have a mutation/which one it is. 

What’s the big deal about the mutation you ask?  People kind of look at me funny when I say that I'm anxious about it all.  Talking about it in terms of letters and mutations seems to remove people from thinking about it as tumors and disease.  What's the big deals if a few of my genes are 'misspelled?'

I’m not sure if everyone in Pheo world would completely agree, but from where I’m sitting here it basically feels like I’m waiting to find out which disease I’m being diagnosed with.  Or if I have a disease since a small part of me (and it might be the denial part of me) is still holding out hope that this could all just be sporadic.

If I find out that I have the SDHD mutation it will mean that I’m predisposed to paragangliomas and potentially pheochromocytomas.  People with the SDHD mutation most often get them in their head and neck, but also sometimes in other parts of the body.  I may have an adrenal pheo at some point.  There isn’t a straight line progression.  It doesn’t always happen the same way.  And some people with SDHD, despite the fact that it’s considered low risk for malignant potential (around 10%), do indeed end up having malignant disease.  The ‘genetic imprinting’ of SDHD would mean that my girls “most likely” would not develop tumors themselves, but could pass them on to their offspring if they were carriers for the gene.  If I have the SDHD gene I will continue to be scanned twice a year and can back that on down the line other tumors will probably grow, though I suppose it could be argued that they might not.

If I find out that I have the SDHB mutation it would still mean that I’m predisposed to paragangliomas/pheos.  It will mean that there’s a higher chance of them being functional and/or in the abdomen.  It will mean that there is a much higher chance for malignancy (38-83% in carriers of the disease).  Again there's no predictor for how or if it would all progress, but we'd know more about what to look for.  The genetic crapshoot of SDHB would mean that I could have directly passed on the mutation to the girls.  Again, I'd continue to be scanned twice a year and we'll deal with what we find from those scans.

I’ve never in my life hoped more fervently to get a ‘D’ instead of a ‘B’ on a test.  I really don't want to have either of them.  But the truth is, whichever one I’ve got, we’ll fight it and we’ve got the heavy duty folks on the team already.  They’ve got my back and I trust that the procedures they follow will help us to root out these tumors so we can deal with them early and decrease the risk of complications.

I just want to know so I can wrap my mind around it, and so I can stop feeling crazy every time my phone rings.

In the meantime, since I just can’t let life be too boring I went ahead and choked on some chicken and had an emergency endoscopy under general anesthetic Monday evening.  That was a typically Roseberrian crazy song and dance complete with an EMT body checking an agitated patient who had gotten in between my husband and our kids, and my being grilled by the surgeon doing the Endo. About the Tomas Surgery for a good 20 minutes.  At least I got to chill out with some Valium for a few minutes.

We’re through that though, and things are fine and despite what I’ve written above, I’m not sitting and babysitting the phone and I’m not paralyzed with fear.  I’m just anxious to know.  Anxious to process it. Anxious to start to settle into what we're dealing with. Do you think anxiousness could make you choke on chicken?


Could be....