Monday, April 30, 2012

But with your history...

A while back, I discovered a lump in my arm. It was right next to a mosquito bite that was driving me crazy. I decided to keep an eye on it, but I knew it wasn't going to go away. I showed my neighbor who promptly said- yeah you need to get that looked at. I showed my husband who immediately said- it's not a tooma. But then he promptly decides he must do a thorough exam of all potential parts to make sure. I have several lumps and bumps. Some look like cysts, some scars, no one really knows. All I know is that at some point, with the p53 mutation- little things can become big threats.  It became the elephant in the room, the thing I'd get to if life ever calmed down. Right after I got around to losing weight and cleaning the house, managing the man-child's diabetes and scheduling Lily's next round of scans, all the while trying to get a nonprofit to run efficiently. A week turned into months and I realized- it was never going to get easier. It sure as hell wasn't going to get easier without an arm- so I needed to get in and get that bad boy out. One night Phil was taking inventory of the lumps and bumps and one on a less than sunshiny place somewhere on my backside seems to have taken a turn for the worse. Not ugly yet- just growing. One of the first bad signs. When he started looking at me that way- you know like you look at people who have worse luck than you- I scheduled my yearly exam and showed my PCM. Yeah, we should get that looked at by a surgeon. It's probably a lipoma, but with your history....

I schedule an appointment with the surgeon.  He's your standard run of the mill retired army surgeon. He's seen the glory days and now is still trying to do what he loves until it's no longer an option. He looks at the lumps and says. It's probably a lipoma. So I tell him the story of Lily's leg. I took her to the pediatrician- it's probably a lipoma. I took her to the surgeon- it's probably a lipoma. Please just take it out and I will feel better. You were right Mrs. Mallory, it's a plexiform fibrohistocytic tumor- a really rare slow growing(thank God) sarcoma, we have to go back in and get wider margins.

So at the conclusion of my story I can feel the surgeon's non impressed-ness. I stop and wait until he looks up from whatever he was thinking about. I look him in the eye and tell him- I know a lot of people with Li Fraumeni Syndrome. They all have lumps and bumps, they are always told- it's probably a lipoma. Sometimes they are. Usually they are rare sarcomas. I'm betting this is one of those. I want it out, I want clean, wide margins. He calls the nurse in with the schedule book. It's not urgent- so it will be a few weeks. I expected no less. He tells me it will be either him or the other surgeon, depending on what day of the week I choose.  I ask him which day would be better for a lipoma that was probably a rare sarcoma. He didn't answer. Yeah- I don't think there's a better day for that either.

The first available day is the day before Bella's birthday. It's not ideal, but then again- what is? Bella spends months planning a party, a cake- all of which I am to play an instrumental- hands on role in. I manage to whiddle it down to cupcakes for the class(I can buy) and going to see a movie. She is fine with that. The day of the surgery rolls around. I know it's minor- but it's like staging for the big event. The nurse finally gets me and takes me back into the corner of the clinic. We go into what seems to be a storage room for abandoned equipment and gurneys which also connects to 3 offices. One of those offices happens to be that of my genetic counselor. I poke my head in and say hi. She accompanies me into the next "office" which is actually a treatment room. Awesome. Fortunately it looks better than the ante room- but that's what you get with budget cuts.

We discuss problems with arranging future scans for screening me. I am introduced to the nursing student and the med student who will be "observing". The last time I had a student , he helped "observe" a huge scar on the back of my shoulder. One of the many reasons I was less than enthused to go through this whole process. The surgeon comes in and I won the luck of the more experienced, other surgeon. I'm pretty sure he's not a day less than 75. I reassure myself by thinking at least he's seen a few world wars and probably has some mad slicing skills. He also doesn't have the current filter for conversation that most surgeons develop and he proceeds to tell me all about his family and their polycystic kidneys and kidney transplants. Got it doc- my "probably lipoma" is not an organ transplant.  So I tell HIM the story of Lily's adrenal tumor and leg sarcoma. I tell him about the p53 mutation and what it means for sarcomas and that I know a lot of LFS folks. I look him in the eye and ask him to take wide margins, don't nick it and get all of it.

He then launches into a discussion about numbing and earning patient trust for the students. As he is doing this- he is numbing my arm. I don't feel a thing- not a poke, not a burn. Trust earned. The procedure begins. I don't have a clear view, as I am on my back and two grown men are hovering over my extended arm but they are dissecting away. Still dissecting. Still dissecting. He pauses to let me know that if I start to feel anything- let him know right away. I feel the tugging and pulling, but no pain. Until he gets the electric lightening machine(electric cauter something or other) and sticks it in my arm. It felt like someone stuck a sparkler in there so I calmly point out it's burning. He adds a bunch more numbness and proceeds. I know it's deep. They finally get the little bugger out. It looks like a tumor. It's fatty looking, about the size of a grape tomato. I am slightly unimpressed and comment that it should at least have teeth or hairs or something.  He plops it down on the tray and cuts it open. I knew was probably not a lipoma. Surgeons(mass generalization- but probably deserved) have slight God complexes. If it was just a lipoma, he wouldn't have needed to see what was on the inside- it would be lipoma-y. They close my arm up and go about removing my other bump. Yeah- the one on the rear. I've had four kids. But having 3 men gathered about your backside is a bit intimidating. At least they were armed(no pun intended) with more magic numbness. That took a couple minutes, so I knew- it was probably nothing.

 I then get to be on my merry way with surprising little direction. My GC grabs me and gets Phil so we can discuss more fun topics like mris, prophylactic mastectomy, doing the best we can to keep me around as long as possible. At the prophylactic mastectomy talk, Phil flinches. He love the ta-tas. I haven't discussed this with him, as a self preservation technique. If he thought his moments with them were numbered- I'd never get a moment of peace. Although I'd have to say this whole experience did the same thing. He is a bit unnerved. Boys.  We spend the car ride home discussing how it's not really necessary, right? I joke that at least they are replaceable and I could even get an upgrade-or at least put them where they were before I nursed 4 kids....He did not appreciate that.

I wait out the week for my post op appointment. No waiting this time, the surgeon waltzes right in plops the pathology report on the counter and says- well it's a sarcoma. We sent it off, they couldn't identify it here. I wanted to say no shit sherlock- at least you could have told me that I was right. But instead I said- Ok, so what now? Can you come back in tomorrow to meet the surgical oncologist? Well I guess I COULD, but I am here now- is he available today?  Well he's very busy, I can see if he's available- there might be a bit of a wait. I wanted to say- More or less wait than the 2 hours round trip- headache of parking and the checking in process?  But instead, I said- I can wait. While I waited I got 2 sets of stitches removed, read 100 pages, talked with 3 nurses and my genetic counselor and finally scored a meeting with the surgical oncologist and his nurse. We start to plan the billion scans that need to be accomplished for the next surgery.

Again I repeated my history, again I illuminated them to the finer aspects of limiting radiation exposure. And reminded them 2 more times after CT's and xrays were mentioned that- unless there was an urgent need- could we find a non radiological way? At one point the nurse stopped and said- Wow, you know a lot about this syndrome. It is so great that you can advocate for yourself. I know the translation of that is that I am a difficult patient. I get that. But despite the fact that it may be quicker and more convenient to blast everyone with radiation- I believe that the effects are not seen until much later. I believe that we mutants are more sensitive than most and our much laters are much sooner than the average bear's. I know I can't avoid radiation- but I limit what I can. I also try to limit the amount of time at the hospital, for any reason. Plus- there is a fine line between just being curious and being able to do something about it. Many of those scans have the potential to find things there is nothing you can do anything about- that is why there is no set protocol. To each their own.That is why I wanted to be involved in Li Fraumeni Syndrome Association- to help others who may not know to advocate for the care they need and deserve with this syndrome. It sucks. It sucks now that I have to spend my time dealing with this BS instead of helping others. And then the other part of me says- at least it's me and not one of the kids. And then I get really mad because it shouldn't be anyone. But then again with our history....

Tuesday, April 24, 2012

What to Expect when you are Expecting Cancer

About 1.6 million people will be diagnosed with a new cancer this year. According to the American Cancer Society, that number doesn't even count basal and squamous cell skin cancers. 577,000 people are expected to die of cancer. That is one person a minute. Many of those cancers can be prevented by not overusing alcohol or tobacco. For some people, those who have family histories of cancer or mutations that are known to result in cancers- the preventative measures are bleak. What is abundant are the questions, the worries, the risks, and the unknowns.

Will I get cancer?
When will I get Cancer?
Is there anything I can do?

For those with known mutations, like in the p53 tumor suppressor gene-also called Li Fraumeni Syndrome, these questions can often lead to an overwhelming sense of futility. I've heard of ladies being told they have merely months to live- so they cash out their life insurance policy, run up all available credit cards and live it up for months- travelling, partying, living. Yet when those months turn into years, suddenly they are faced with a new prospect- Living on borrowed time, and a bunch of debt. Also what many non mutants call, life.

Everyone at some point faces a life altering, spirit moving moment. A moment that forever changes the path they were on due to a change in the expectations for the future. That moment came for me when my daughter was diagnosed with adrenal cancer. I spent most of my life knowing that cancer followed me like an unwanted shadow- sometimes leaping in front of me- a reminder of the darkness that envelops when you are consumed by it. I never pretended that it wouldn't touch me- I just chose to live the moments I could until it did and not waste those moments worrying that it would.

But then there were these big green eyes asking me- why? And her brothers and sisters wanted to know why too. As often as I told them it wasn't there fault- the little voice inside my head screamed- of course it's not- it's yours. The gene after all came from me. A gene that had a 50/50 chance of hitting them. I would never point a gun at my head or theirs with those odds- why was I cavalier about these?  Because there are no certainties. I was in my 20's and perfectly healthy. I had seen many incredible places and experienced many of life's wonderful treasures- one of which was falling completely and totally in love with my best friend and becoming a mother. Like the hopeless romantic- I believe that love conquers all and that I would definitely choose to love and have lost than not love at all. That is the same choice each person makes for themselves. There is no right answer, just truth. I look at my children as opportunities to love, not endless opportunities for loss. When your child is severely hurt or diagnosed with a major illness- your mind goes to the possibility of the endless abyss that is losing a child. You are then faced with a choice- to live life with the shadow of loss- or live life with the potential for greatness.

Facing a future with cancer is no different. There are days when the shadow of the abyss creeps closer- the days that are filled with scans and preventative screens. Those days are when the rattle and hum of magnetic imaging machines are silenced by the internal whispers of worry. Days where lumps or bumps or fevers pop up that are nagging darkness threatening a storm. You could ignore these steps, but everything and everyone tells you the storm is coming- your best bet is to catch it early and wait it out. Sometimes it is nothing. There is celebration. Sometimes it is definitely not nothing. It is impossible to not feel the weight of the finality of cancer. It is impossible to deny the pain, emotional and physical and the scars are permanent. But so are the lessons. So is the reaffirmation of life. We get warning bells when others are silently taken. The path is not easy- but it is what we make it. If we make it what we expect- that may be cancer- if we are open to receiving the gifts that are unexpected- we may get much more. And sometimes you have to be careful how hard you look for something- because you may find something you didn't want to. If you are expecting cancer- sooner or later- you might find you have cancer. If you expect to live- sooner or later you might find forever is shorter than you thought and sometimes when you expect to die you might just discover an amazing will to live. That is what you can expect, when you are expecting cancer.

Lily Kay Monkey

Lily Kay Monkey
November 2008 Photographed by Shelley Detton (7 Layer Studio)