End of Summer Clean up

The follow ups this year are a shadow in Bella's arm and a lump that appeared on Phillip's ribcage. Hoping for nothing, but we don't know until we know. I think of the swollen lymphnodes a few months back- were they trying to fight this lump off? who knows.

Phillip was of course referred out to a dermatology consult. Which was fine with me, I've been to Tripler derm a few times and the scars to prove it. Lily was referred out years ago and I was impressed.

With LFS there is a lot of self advocating. I'm trying to give the kids the tools they need to navigate the crazy healthcare world with a rare condition.  I tell them to pay attention to biology, anatomy, in school and in person. Know their bodies. If anything weird pops up and lingers( we have a 2 week rule) we get it checked out.

Phillip is on the cusp of adulthood-he's no stranger to medical stuff as he's been independently managing his diabetes for awhile. I appreciate that he tells me about concerns. It's a hard concept to understand- taking care of things early could mean the difference of life and death for us. I prepare him for my gut reaction- to remove it. He has some time to think on it. I will let it be his call.

The nurse calls us into the room, she recognizes Phillip. I ask if she shops at Safeway, we discuss their Friday deals and great flowers. I share with her a bit of our family history and Li Fraumeni syndrome and why we are here for this little lump.  She looks at it, visibly taken back by our history.

When she leaves I explain to Phillip why I go over LFS and our history. We are lucky to have a team at Tripler that knows us and our history and automatically takes into account our mutated p53. little things that don't concern normal people- could be bad for us. They could be nothing too. I tell him most of our time is advocating for ourselves. Most doctors have never heard of LFS and it's on us to make sure they understand. I show him the living LFS pamphlet I brought as well as the latest journal article on LFS and screening that I printed out. I'll leave both with the doctor.

As we sit- Phillip paces- the view overlooks Aiea and we can see the Arizona Memorial. He asks a few questions, I answer. The doctor finally comes in. Phillip takes off his shirt, the doc draws around the lump. Its about a 1 cm oval, palpable, not fixed. He's not concerned and says considering history, lets remove it. I agree.

He asks me several questions about Li Fralini - it takes about 5 tries before we got the name down_ I tell him we call it LFS for short. He asks me if I'm a nurse. Nope. not officially. He asks me specific questions with medical terms- he's feeling me out and my knowledge- I take the bait and answer equally as medically. He is not familiar with sarcomas, I ask him to consider wider margins than he was planning so we don't have to go back- I hold up my shark bite. I explain about Lily's sarcoma and 15 year old LFS friend was just diagnosed with an extraosseous osteosarcoma- our lumps tend toward weird.  I pray it's nothing- I've had several of those too, we just have to be careful. And oh would he mind doing a once over look at his other skin to make sure everything is ok.

He smiles, almost condescending, but he has kind eyes so I let it go. He says he realizes I've been traumatized but not everything is cancer. I would rather have a non cancer removed then for it to become something that cannot be treated. I explain again that both my brother and father died of brain tumors, phillip's sister had adrenal cancer in addition to sarcoma( which was just a nothing...) He interrupts- medullary tumor? No- It was adrenocortical carcinoma. That got his attention. In addition to her plexiform fibrohistiocytic tumor, mine was also fibrohistiocytic but I think they are calling them pleomorphic something or other's now- I can't keep track. That was the same time as my breast cancer which metastasized to my lungs, bones and brain- so yes there has been LOTS of trauma and lots of cancer flags.

He pulls up Phillip's shirt and makes a wider margin around the existing one. He turns to me- you should really consider starting a patient advocacy group. I pick up the living LFS pamphlet and say - I have. This is our group.

Living LFS? What does that stand for?

Oh holy fuck- again with the Li Fraumeni Syndrome pronunciation guide. I tell him a bit about some of the cancers and rarities our families face, but it is the most phenomenal group of people. He asks to keep the literature- of course- please.

We schedule the surgery- it'll be minor, but we have to work around his college schedule now. And so now we wait.

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....

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."