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 then...it 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....

How We Found the Rock.

Lily was born pretty darn hairless. She had blonde little goofle-feathers as my mom would say. At a year, we wondered if she was going to get hair. And then she did. Great big furry, spirally curls. She calls it her BIG hair. When she looks at her baby pictures, she scrunches up her nose and asks Where's my big hair? When she turned 3, she started getting furry in other places. Places it's not polite to talk about. It started as a rogue hair or two, which prompted a trip to the pediatrician in May. He ran some blood tests, but after a little research wasn't too concerned- it was probably precocious puberty. That would explain the slight acne, but my dad and brother died from brain tumors- there could be something more to this. He assured me that we start at hormone levels and go from there. The hormones came back "normal". Really there was nothing to indicate further tests unless there was continued growth or other secondary characteristics.

I also pointed out a lump on her calf that I noticed the morning before Phillip bruised his pancreas(April). The same advice was given- if it gets bigger, red, more swollen...yada yada, bring her in and we'll run more tests. In September we had what was to be our last visit with Dr. E, the pediatric surgeon who followed Phillip through his case of traumatic hemorragic pancreatitis. Unfortunately his ultrasound showed no decrease in the dilation of his pancreatic duct and she wanted him followed by the pediatric endocrinologist to make sure his enzymes and blood sugars were all normal. They pretty much are. His blood sugar runs a little high- but he lost about 50% of function in his pancreas...so we just keep an eye on him. Anyhoo- I asked Dr. E about Furry Lily, since I trusted her opinion. She immediately referred us to Dr. P who is the same pediatric endocrinologist seeing Phillip. She looked up the blood tests on Lily- they were not normal for a 3 year old little girl, the testosterone was too high. We needed to run more tests. She suspected a genetic condition called Congenital Adrenal Hyperplasia indicating a malfunctioning adrenal gland. Three weeks later we found out the blood tests did not support this, so it was time to look at the organ in question. We traipsed down to the radiology department for the ultrasound. Phillip had tagged along so Dr. P could see how he was doing. The U/S technician looked at our name, then at Phillip. "Hey- is he Pancreas Boy?" Yes, flying into hospitals everywhere is the action figure known as 'Pancreas Boy.' He immediately turns some insane shade of red and busies himself throwing paper airplanes around the room. Lily makes me lay down next to her, her "fro" effectively blocking any view of the screen. Although I am not a radiologist, I sometimes play one on TV- so therefore I like to look at my children's innards in cases like this. Sure it looks like random blobs- but you pick up on clues like- why so much time on that blob? You ask key questions like- is that the adrenal gland/kidney/whatever organ might be shaped like that particular blob. The key is in the response. If they respond- No that's the such and such- you're good- if they pause and say something like- No, that's NEAR the liver- well pretty much plan on being there for awhile. And when the honest to goodness radiologist pops in to introduce themselves- realize you will probably be getting to know the staff at the institution intimately. So we finally finish and are on our way back to talk to Dr. P- who now has a posse of 3 with her. Yep. This is not good. I supply Phillip and Lily with toys and snacks and hope to God that the day-to-day obliviousness and selective deafness they like to employ are on full force. Dr. P begins,

Lily has approximately an 8 cm tumor on her adrenal gland. There are two possibilities. It could be an adrenal cortical adenoma which is benign, or an adrenal cortical carcinoma........

I immediately grab my folder and start looking for a pen. One of the residents grabs a pen and says he can write it down for me. Oh really- you were gonna write down "G*& D&^% Mother F#@! Son of B&%$! because that was kinda what I was thinking. Pardon my language. Weird things pop into your head when they tell you your child has a tumor. Fortunately they were explaining what benign and malignant meant so I had some mental time to swear some more. Lily and Phillip were happily tossing the doctor's toys into the air to see who could actually get admitted to the ER first....Oh they are looking at me. Hmm I think someone asked a question and darnit I've been nodding this whole time swearing in my head. Sh#@. Fortunately they just wanted to know if they could do a physical exam. SO for another half hour or so it was words words words. I spent 4 years in college getting a degree in genetics- I knew all this- so I'll let them ramble on about Li-Fraumeni syndrome like Li and his bud Fraumeni hadn't been over to dinner once or twice in my not so distant past. OK not literally- but they discovered the link between the p53 and hereditary cancers. Their work and my family history was one of the reasons I wanted to become a genetic counselor back in the day- instead I decided that procreating was a more pressing occupation and here I am. Ugh. Words words words, appointments, surgery, yada yada, someone will call us to schedule, please call at any time. Great. We can leave. The pediatric Oncologist and her posse split to start the research, and Dr. P looks at me. Don't make eye contact- you are successfully holding yourself together......So I guess Mr. Mallory is gonna get quite the call at work huh? I laughed- allright we'll keep you- you have a sense of humor. We banter about something- could not for the life of me tell you what and I managed to get the kids to the car. I can do this- I can drive home. I'll leave Phil a message at work to call me- he will call when he can. I take a deep breath- the airman at the front desk answers and a voice that sounds nothing like me shakily asks for Buddha. I'm not religious or anything - that's what they call him. He's debriefing- she says- I'll go and get him... NO, I cut her off, Just have him call as soon as he's done. I spend a few minutes trying to collect myself, and we start home. Phil calls and asks if everything is Ok. I blurt out LILY HAS A TUMOR. Where are You? On the way home I tell him. I'm on my way. I hang up. Phillip says "Is something really wrong with Lily?" So I tell him with the air of- really it's a little thing in her belly and it has to come out so she doesn't get sick. The look in his eyes conveys the terror- I reach back and squeeze his hand. It's gonna be OK buddy, she's gonna be fine.

It's not a tooma, it's a rock.

So the battle has begun again. Many do not know the sordid past of my tumor history or this particular part of my sense of humor. I personally have not had a tumor. And I don't really find them particularly funny. But sometimes you have to step outside and laugh a little and so tumor humor was born. I did not invent tumor humor- but I was raised by it. You have to deal with the hand you are given- even if you have to trade a few cards in along the way- you are still stuck with your basic hand. My basic hand was riddled with tumors. My dad and all of his siblings have or had cancer at least once- and only 2 of them are around to tell their stories. My brother and cousin both died from cancer in childhood. As my uncle recently informed me- you are allowed certain leeways when you are the oldest surviving member in the "dead zone". Tumor humor is one of those things. You make jokes because you have to, because the days are long enough- you need to laugh to get through. If you aren't ready for that- I suggest perusing something a little more benign(puns count...) like a blog about the weather. I'm sure if my grandmother had better sight and any knowledge of what computers can do- she would have a kickin blog about the weather- she can tell you down to the degree the weather anywhere she has ever lived or where anyone she has ever known currently lives or has lived. But this blog is gonna be about Lily, and her tumor which as she says" it's not a tooma, it's a rock in my belly."