I can't think of anything worse than losing a child, except perhaps seeing them suffer. So today was one of the longest days for many reasons. The longer it was, the longer I could avoid calling my own mother to let her know the news.

I have breast cancer. And a sarcoma- but that last bit seems pretty minor now.

Equally difficult was sharing the news with my children.  The last thing I ever want is for anyone to worry about me- least of all these little people in my charge. So for them I find strength and courage because there is no other option.

I find strength in knowing there is a 7 year old upstairs who is stronger than I will ever be. I find courage to be strong for the 11 year old whose worst nightmare is to be unpretty or different- if for nothing more than to emphasize the truth of beauty and strength for her to know and find within herself. I find a smile from a 6 year old who knows something really bad is going on and forges ahead reading a book to me despite 5 other people vying for my attention because she needs me to see her read.  And for the 12 year old- who will be strong, yet terrified and rise to the occasion because I know he will, after all he is his mama's boy.

There will be long days ahead and we have not had time to prepare. Prepare for the physical or emotional loss.  But I know preparation is a luxury this disease will not be afforded, time is of the essence and the eviction notice has been given.  I will face it with my best friend, by my side as he has been from the beginning. In sickness and in health- he has been my rock, my foundation and together we can get through anything. I have been luckier than most and the universe tries to find balance- it will again tip in our favor. Until then, please offer a thought of strength, a prayer, any positive energy you can spare for my monkeys and their dad tomorrow- as I will be temporarily unavailable.  Hugs.

Saving the Ta-Tas....

Do you really want to save something that's trying to kill you?  My husband and I have been wrestling with this decision for a few weeks. Well mostly I have been wrestling emotionally and well- you know how boys are with toys- especially when you threaten to take them away. They go out of their way to play with them to show you how valued they are. Yet that fact was never ever in doubt. If sheer love and affection could stave away cancer- my ta-tas would not only be safe- they would probably emit a cancer fighting aura. Yet that is not the reality.

The reality has been palpable for months. Mostly by me- obsessively with the express desire and hope the little lump that liked to become sensitive once a month would just fade away with the PMS. But it did not. When I could no longer ignore the lump in my arm, it was time to take action. Tomorrow we will know for sure. There are definite lumps, but tomorrow the pathology will let us know how evil those little lumps are.

It is a battle. I know damned well that early detection is key to survival. There are pink ribbons all over the world declaring that continuously. I get that. I feel also that breast cancer treatments , though lightyears better than even a decade ago- are.still. barbaric. Most cancer treatments are. You can give them the advanced feel with super advanced photon something no one can pronounce name- but my gut still is telling me- they aren't the answer. The problem is they are the only solutions right now. Or are they?

And I can once again attest that you honestly don't understand something until you experience it. So far this experience has pretty much sucked the big one and I haven't even gotten to the really ugly parts yet. I don't know how many times I have to say it- I don't really have to know everything- I'm ok with that.  It makes me feel no better whatsoever to know that hundreds of thousands of women have been through this and many more will join the journey before it gets easier or somehow goes away. 

Perhaps it's the enlightenment of my husband declaring- Holy cow! as he witnesses the extreme compression of my mammogram that drives home how there has got to be a better way. Or perhaps the extreme burning of the core biopsies in areas that apparently didn't take up the lidocaine . Or the repeated assault of the mammogram POST core biopsies that didn't take the lidocaine that make me doubt this whole process. Seeing the bright little lumps light up on the big fancy screen were some how simultaneously comforting(Thank God the whole darn thing isn't lighting up) and horrifying(Oh that's gonna be a big chunk of ta-ta that has to come out). As the biopsy , partially medicated proves- I am a wimp when it comes to pain. Coming home and looking at post lumpectomy pictures- probably not a good plan either. Then I go back to trying to be positive- they can be replaced- I cannot be. And my grandmother's words keep lurking in the back of my mind- it could be worse.  Part of the problem is knowing that the worst is no where near from over. And as my uncle told me in his infinite wisdom and many years spent past the dead zone- when it gets really bad- just think that somewhere right now someone is to the point of tears because they have a flat tire, or a zit or their eggs were over medium instead of over hard and it is what we make of it. But then again, he pointed out it also reminds us that most people don't have a clue. Some days it seems it would be a little easier not having one.

Just Keep Swimming...

A couple months ago, a new rec center opened here on the island. The KROC Center- is a state of the art worship, pray, play, exercise, fun kind of place. The fees are affordable, especially for a family and I don't have to spend hours afterwards cleaning sand out of everywhere. It is a great opportunity to watch people, while the kids can splash around with Lifeguards at every turn.

I just can't believe some people. They go to the pool and drop their kids off and go work out or don't even pay attention. I can't tell you how many times I see the lifeguard grab a kid and take them over to their parents because they repeatedly don't follow the rules. 

We started going to the Kroc center every Wednesday(short day of school) and most weekends. The kids have come to rely on it as part of our schedule- but it is also a great incentive to get them to clean their rooms and do their homework quickly. This Wednesday was even shorter due to the May Day Program and I had the girls' suits packed and ready to go in the car.

So this woman isn't even through the door,  her kids are off down the hall and she doesn't even go into the locker room to supervise. She is on her darn cell phone. 

It poured after the program, I was so grateful the clouds held off like they did. I was waiting for my rainbow- I needed one that day. I didn't see one, but I packed the girls- already slightly damp into the car-they were going to get wet anyhow. I just hope the rain moves past by the time we get there. It looks like it will. I have to keep positive. I hear my phone buzzing away in my bag. I have put a new ringer on for all calls from the hospital, it's called sci-fi. It's like spooky whistling you hear on all the shows when they talk about alien abductions. It makes me laugh because I know most calls from the hospital are not good.

The kids come running out of the locker room and just throw their clothes on a chair. The mom shoves them into a bag- the whole time on her phone. The lifeguards aren't babysitters. 

I check the messages while the girls are in the locker room changing. I don't know if the signal is in there or not. We head to the chairs and I find one that has a decent view of all areas of the pools so I can try to keep tabs on the girls. I have a call I have to make for Li Fraumeni Syndrome Association, we have our Board Meeting coming up and I need to make sure some details are taken care of.   But that will have to wait- the message is from my Genetic counselor. She has been trying to fix the recent comedy of errors that is what the hospital refers to as "business".  Last night I got a call from the surgical oncology nurse. They moved my surgery date to the 18th of May to take into account the potential need to deal with another tumor that popped up on the MRI.  After reasonable pleading and making a case for common sense that it should not take 2 weeks to perform the necessary imaging and biopsying of said new tumor if it was that urgent and that my plans to travel on the 23rd were not flexible and 5 days was not enough time to heal from surgery when traveling with 4 kids. But Jennifer- this may be cancer and it could be very serious.   As am I when I say- that is not enough time and if it IS serious- this all needs to move faster anyhow. It did not matter. An entire weeks worth of appointments were wiped out and rescheduled without my approval. Tests were ordered that I had already refused based on radiation exposure.  I was a mess. My husband was again gone - the first bad omen. The other being the Mallory family attempting any sort of trip. We used to laugh at the show LOST and how the island didn't want them to leave, yet seemed to be trying to kill them at the same time. I was beginning to relate.

Look at her, doesn't even put her cell phone down for 2 seconds. Probably chatting to friends while her kids are right there in front of her. The littlest one can't even swim. Engage.

I walk over to the side of the pool. Bella and I had been working on her swimming technique before I had my 1st surgery. I should be able to be back in the water by now, except the minor wound- the one that isn't cancer and doesn't have to be completely cut out anyhow- dehisced. A fancy word for came wide open and now has to heal from the inside out. Which means, no pool, no baths, no long luxurious hot showers until the little eye on my backside completely heals. So now I am relegated to the sidelines, miming the various swim skills I would like her to practice. Kiera, like always is happy to help show her some moves.  I am grateful for that as the phone is ringing yet again. It's my genetic counselor, she has fixed the schedule and it makes for a really busy week- again being at Tripler almost every day- but I need to make this happen. I walk back to the table where my latest notebook is to write down whatever we discuss. I learned long ago- if I don't write it down- I won't remember.  

Thought for a second she would engage, nope back to whatever is more important than her kids. I wish people would realize they are missing out on what's right in front of them. 

I debate whether or not I want to ask the hard hitting question. After all, I am in a public place with my kids and now is not the time to hear things I can't unhear. Which means it's out of my mouth before I can stop it- I want to know exactly what the radiologist said, I want to know exactly what everyone is thinking. I don't want decisions made for me, this is my call. I am a part of the team and I need the information to decide what we are going to do.    Ok, the report is Bi-rads 5. There are two lesions, one is nodular, one looks like it is in the duct.  Most radiologists read that as highly suspicious, about a 50% chance of malignancy. Our radiologist reads it as probably malignant.  We need to do a biopsy to know for sure. We can do a fine needle aspiration, but that won't give us as much information as a core biopsy with nodes. Why can't we do an old fashioned surgical biopsy when we are doing surgery on Friday? You know wide margins- unlike the arm thing. It will take weeks for the path to come back anyways. It probably is better to know IF we are dealing with a malignancy before we go into surgery.  That is a good point, although we can probably guess  it is. Well we can know how big of margins to take, you never know it could be a cyst. The surgeon has already been briefed and is ready to do the lumpectomy.  Ok, let's do the core. I'll schedule it, probably Tuesday or Wednesday next week, I will send you a message with all of the appointment times. Thank you for fighting for me. No, you have a pretty good handle on this. I'll be in touch. I look down at the screen. I don't feel like I have a good handle on this. I don't want to handle this at all. I want to jump into the pool and swim with my girls like nothing is wrong and there isn't a gigantic timer that has just been placed above my head. I know everyone has one, but I don't want it to be so BIG right now and so LOUD. I call my mother in law. She knows the pathologist and chief of surgery at the hospital. It's time to hedge my bets. I need all the help I can get at this point. I wipe away the tears. There is not time for feeling. Yet the fear, the anger all of it keeps trying to seep out of my eyes. Big old betrayers.

Oh god, is she crying? What the hell? Is a day at the pool too taxing for her? Seriously- you don't get any easier than this. 

The clouds are moving back in. The girls are cold and hungry. My phone rings again. Go get changed and we'll go to lunch- I answer the phone and move everything under the umbrella. It's a friend with LFS, I sent her a message that I needed her expertise on saving the tatas. She is up to her eyeballs in trying to find the best treatment for her son and a new rare condition, but I trust her and I know she will call if she can. She is an information hound and she is real. She has been through 2 breast cancers and treatments with LFS so she knows what there is to know. Is this your 1st cancer? Yeah- I didn't want to tell the LFSers because the first one was just a sarcoma.  Wait, you have a sarcoma too? Where? Is it one of those fibrohistio things? You are holding it together much too well for your first cancer. I was a complete wreck. I don't feel like I am holding together. I literally feel the seams snapping string by string. I do know that this feels much more manageable than when they told me Lily had cancer.  I feel 1000% better talking to her. She says she agrees with getting the lumps out- but taking the trip is what I need, do it. She also points out how long it has taken her to progress with her son- we know the routine. No one wants to listen to you when you say something is wrong- then when they decide it's urgent you still have to hurry up and wait until you are on their schedule. The kids are changed and just waiting, we gather up our stuff as we move to the door, the phone rings again. All three sag and moan- not again. Although I need to return that call, I also need to spend time with my girls. This is the balance- a new balance that I have managed before and will get through again. Managing cancer with living. Certain things will have to give and certain things I will have to let go. I have to let go of how I feel other people see me, after all they don't know where I am coming from and things aren't always what they seem.  But I do know- sometimes you have got to just keep swimming, well at least when the incisions heal you can!

I'm with the M...R....I

I've never been a big fan of horror movies. I guess there are enough things in day to day life that scare me, I don't need the extra horror. Today's big challenge is to get through the first MRI without a panic attack. I'll deal with the next two MRIs as they come- which no one really knows because of the way "the system" is set up.

Up today is the breast MRI. It has not been easy to get here. Months ago, during my annual exam- my doc put in the order for breast mri to avoid irradiating my already radiosensitive tatas. It was promptly denied. I guess that they are only performing these for active duty women on site and Tricare doesn't want to pay for me to go off site. The denial actually said they wouldn't pay for it unless I could show that I am high risk breast cancer. That I can do- with the genetic test for a cancer predisposition syndrome that they paid to have done. A syndrome that makes one sensitive to radiation.

Tricare- Oh, Ok, let me look in my file- Oh it says here it's already been approved.
So why did I just get a letter of denial?
 It must have been sent out before .
Before what? Before anyone read the request? Or my file? That seems inefficient.

So I call to schedule the mri.
We can't do that here.
Oh here we go again.

I call my genetic counselor.
We are working on that. We don't want to send you to town because things, well, you know.
Get lost? Aren't done in house? Don't transfer? Cost more? Yeah I know.

I don't know why patients are non compliant- this seems like a perfectly reasonable way to spend my time.

Fast forward 2 months. Still no Mris ordered/approved.
I am sitting with the surgical oncologist who would like to see what else is brewing in my arm, and lungs- since that's where these little buggers like to go. He orders 2 more Mris. He throws another breast Mri in there for good measure. Wait 24 hours and call- they receive the order, it has to be approved by the radiologist then you can schedule. I know the drill- we do this every 3 months with Lily monkey. They are actually much more efficient with the kids- of which I am glad for. The Genetic counselor and oncology nurse go to physically speak with the radiologist to let him know about Li Fraumeni syndrome and why these tests are being ordered.

I wait a day and call. The order says arm mri, the lung mri was cancelled as was the breast. Seriously?

I call the Genetic Counselor.
Well that was not very nice. She says.
I was thinking- GDMFCSSOB.

It's hard enough because I don't want to do these scans. I am grateful to be able to be proactive with my care and try to "catch" cancer at the earliest and most treatable stages. But at some point you are just beating your head against the wall. This is not in line with my quality of life goals. Spending time fighting over scans I don't want to do because the mere thought of causes waves of nauseous anxiety.

Days pass. While I am in the waiting room, waiting to be seen by my PCM- who needs to look at my 2nd-indelicate incision which has decided to come undone and after 8 calls to 3 different numbers- surgery clinic has no one available to tend to it's inconvenient dehiscience on a Friday afternoon. I learned new words that week. Dehiscience usually is a concern for abdominal wounds because of the risk for evisceration. Not pretty. Although I wouldn't mind part of my arse falling out- it would then be lopsided and that's just no good. But I digress- I get a call from radiology. They want to schedule my breast and arm mris and chest CT.

I am not doing a chest CT.
It says here that a lung mri was ordered, but the radiologist wants a chest CT.
I am sensitive to radiation is why the lung Mri was ordered. I am not doing the chest CT.
Well another guy will have to schedule that, I can only schedule the mris. How about Tuesday and Friday?
I can't do Friday- It's my kids' May Day Program. Why 2 days?
That's how they ordered it? How about Thursday?
Who?
The radiologist.
It seems to make more sense to do it all at once- especially since I am clausterphobic and have to take meds for the procedure.
Oh you are clausterphobic? That's why they ordered it for two days. Make sure you have someone drive you- the meds they give you make you pretty woozy.
It would be less daunting for me just to get them over with.
That would be a long time to be in the machine, especially if you are clausterphobic. 
Thursdays Fine just schedule them. My husband is out of town and I have 4 kids- I'm sure finding a driver won't be an issue- for 2 days.
 Oh you need to do an xray too on your arm.
Really.
Yeah- if you could do that as soon as possible, that would be great.
How about if I do it after my MRI on Tuesday.
Oh that makes sense.
That's why I get paid the big bucks.
What do you do?
I schedule mris- it's a full time job.
silence.

So I get a call yesterday. The lung MRI has finally be approved. Same scheduler.
SO I would like to schedule your lung mri.
Ok, what days you got?
I can do Tuesday.
(The irony not lost on me...) OK let's do tuesday.
Oh wait you have an MRI on Tuesday. You can't do Friday because of your kids' May Day program Right?
Right(creepy- you remember I have May Day, but not an MRI that day.)
How about Wednesday?
So I would need a driver, for 3 days- of which I would be taking an addictive sedative- for 3 days and that is more convenient that just doing it all at once?
Let me ask. Pause. I can't get a hold of anyone I'll have to call you back.

Oh I'll be waiting.  My plan is just to be surprised this morning when I go in- the magical multiplying MRI game- maybe we can turn 1 into many.

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....

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.

I cannot take Lily anywhere without someone reaching out to comment or touch her amazing curls. As she also likes to dance her way through every situation- be it walmart or doctor's visits- she has been likened to Shirley Temple on more than one occasion. I love her curls. LOVE them. I hate trying to tame them, wash them, and brushing them is just a pain, for everyone involved, but they are a part of her. So today when she insisted on having them shorn off, I was taken back. Back to three years ago, fighting through tears as I shaved her tiny 4 year old head so we no longer had to watch the beautiful curls fall out. She rocked bald then- it never bothered her not to have a head full of bouncy curls- she was still Lily. Just as I realized then- it occurred to me today- the thought of cutting her hair was my issue- not hers. I needed to trust what she wanted and help her in this journey- remembering a time, many many years ago when I demanded a Dorothy Hamill do and spent years unable to set foot inside any hair cuttery without a panic attack.  Just like 3 years ago- she was comfortable in her choice, even if I was not. My heart literally hurt as I watched the dark blonde ringlets tumble down  to the floor to be stepped on without care. I faked 2 phone calls and took another to walk outside to catch my breath.  I kept reminding myself it was hair, it was only hair. I reminded myself that 3 years ago, I hoped she would just make it to 5 and just yesterday she turned 7. This was only hair.

Every year, right around now- St. Baldrick's events roll around. I never understood them. I've supported many, been to one, but when asked if I am shaving my head- respond- nope- I'm sure I'll get my chance- such is life when you have a hereditary cancer syndrome.  It mystified me that people want to do this, that they have this choice. It bothered me immensely the year Lily was bald. I hated that everywhere we went- her little noggin elicited such pitiful looks, some would avert their eyes - others would give you that knowing nod and every bit of it hurt. It hurt to be on that end, it hurt to be pitied- it hurt to know there wasn't a damn thing I could do about it. Yet Lily never noticed, the looks, the pain- that was adult business- kids would just ask- why don't you have hair? It bothered me that an entire group of people would shave their heads when they were perfectly healthy and did not have to. But then I looked into where the donations went- to really good programs and research efforts. And people really have to step outside of their comfort zone to do this- and it costs next to nothing to do. So many organizations put so much money into overhead costs, printing, branding and whatnot- all of which is not going into the pockets of researchers looking for a cure or on the head of a little munchkin fighting for their life. It changed my whole outlook on these fundraisers. I really respect St. Baldrick's and those who are brave enough to shave their craniums to stand beside our littlest fighters- rocking bald.

As Lily's curls fell around her today, I again fought back tears. Those curls were more than just hair- they are 3 years of healing, 3 years of growth, they represented everything we fought to get back from 3 years ago. We cut her hair then so we didn't have to continue to watch them fall out bit by bit. Today she chose to cut them, because she wanted to. It was when she sat there grinning ear to ear that I knew this is exactly where we are supposed to be.  Once again it took a brave little monkey to show me something I had overlooked. When she turned to me I saw nothing but her big green eyes and a huge dimple, both which were usually obscured by gigantic curls. For at least 2 of the past years I fought with her daily to pull her hair back so I can see her beautiful face- today she won that fight- but then again so did I.

LIVING with Li Fraumeni Syndrome

Li Fraumeni Syndrome is something I have lived with since I was a child. It didn't have a tough to pronounce name back then- we just called it cancer. Then we called it p53. It was our family joke- no one knew what it meant, but it was our bond. It changed our dynamic, reduced our numbers, left scars and broken hearts in it's wake, so we turned it into a big irish wake. It also gave many of us an immense strength, unsurpassed bravery, and a compassion that is unparalleled.  It is part of my history, something to look for in the future and really tough to LIVE with. Some has asthma- and face many episodes of not being able to breathe- living with LFS is not like that. It can be- it knocks the wind out of you and threatens to kill everything you hold dear in record time- but there are times when you can breathe just fine. LFS is like being one of Dr. X's mutants- you spend a lot of time waiting to see what your super ability might be as a trade off for the mutation. So far the only increased power is the ability to field 10x the normal sense of urgency. Yet when you meet someone else with LFS- you get a feel for what the trade off is- you are in a select group of people with a strength and sense of humor like no other and I am grateful I get to know them.

Most days you feel like you are in the middle of a lightning storm, feet firmly on the ground- you look perfectly rational except you seem to be holding a kite with a metal key tied to it. This could be a sign of absolute genius or mental instability and you won't know for sure until the lightning strikes.  You could be brilliant, but you might be a crispy critter- it all depends on if radiation treatment is called for or not. Every headache threatens brain tumor, the flu hints at stage IV stomach cancer- but could also be a brain tumor. Is this a bug bite or sarcoma?

So you feel like an evil scientist after reading more than your average bear on every cancer known to man, being ready for potential symptoms and signs- you start the experimentation process. YOU are the experiment. There are no set protocols to screen for the plethora of cancers that could befall your meagre human form and doing the individual scans and tests to cover every body part that might be affected takes 2 months- I've done it. I found out a few things along the way- like I am clausterphobic. VERY clausterphobic- which is not good unless you consider that mri machines look like gigantic powdered donuts and the sight of both now gives me cold sweats-it might be a good diet plan along side the go lytely(or swiftly and furiously) and the fasting for bloodwork and scans- but somehow, through great dedication and perseverance  I still manage to keep my weight up. I'll blame it on the other positive side of the gene pool that gave me this mutation- the stress eating gene.

 If you think about it- what doctor in his or her right mind would stand behind so many screenings and tests in a person who has an 85% chance of getting cancer in their lifetime- machines are bound to be run by humans- if the machine doesn't miss it- the human might. Good doctors will.  If you support the screening and the cancer isn't detected- who's neck is on the line? The only person who really loses here is the LFS person. If you recommend that LFS folks dedicated a huge chunk of their already limited time on this planet to lying in cold sterile rooms that make awful noises while enduring sometimes undignified and uncomfortable procedures- with the hope they might catch a cancer early enough to treat it, provided that it is even treatable- does it make a difference in the overall survival? Sometimes hope is the biggest key to survival and I've found that depends on the person. Some people want to know and spend a lot of time taking control of the situation and regularly get every scan imaginable. Some watch the signs closely and go into a provider at the sign of trouble. Some just want it to go away and wait to the very last second and often it's too late. Only one group of these people will a set protocol help to find their comfort and control in a bad situation. But the point is it will help people, so why not?  Because they don't know if it works. Technically ALL cancer treatments are still experimental and few are PROVEN to work. Patients are presented with information and given the choice to undergo treatment. It's a game of odds. I know LFS is rare , it will be next to impossible to get the sheer numbers for numbers sake to "show" that screening works. But the odds are in favor of survival the earlier cancer is detected- this seems like a no brainer to me.There are so many negatives to this situation- let's harness the positive. Let's encourage our best odds.

It's tough enough to have a family history of cancer. It's tougher being told you are probably going to get cancer and not only once but many times- but that's only if it's not one of the bad cancers because those will kill you quickly.  I see why no one wants to say anything- it's a no win situation- so take it back old school. Forget the bells, whistles and fancy names. You have a family history of cancer. Ok, let's watch you closely for known cancers in your family- and take into account other possible cancers. Then you have to trust your gut.  You have to know your body and what is normal and pay attention to things that are not. When Lily had a lump on her leg, my gut told me over and over again that it was not nothing. I've talked with enough LFS survivors who have said the same thing- some lumps didn't bother them and others they just wanted out and those ones usually end up being some rare fibrous or atypical something or other. So when it comes down to it- the best screening method is you. And then you have to fight to survive which in the case of LFS is sometimes a fight to get screened and then a fight to get that paid for. But you would be hard pressed to find many people who won't fight to survive once they have been diagnosed with cancer, I'm saying kick it back a notch and fight to survive before you have cancer. It's not easy, no one ever promised it would be- but it will be worth it.  It is possible to LIVE with Li Fraumeni Syndrome.

Li Fraumeni Syndrome Support Group

Li Fraumeni Syndrome is caused by a mutation to a tumor suppressor gene. In even the best case scenario- a mutation here is not a good thing.  p53 has been called the "guardian of the genome" and plays a HUGE role in making sure cancer doesn't go haywire. It makes sure DNA damage gets noticed and repaired,it makes sure the cell isn't reproducing like a bunny while damaged, and if it can't get a cell repaired- it makes sure the cell dies. It's a pretty cool thing. Except when you don't have it. Then you have p53 envy, you find you have a huge risk of getting cancer- and not just one cancer- many cancers over your lifetime. Kids who have the p53 mutation get more cancers than those who don't and I'm not the first to say- nor will I be the last to say- Kids should never, ever get cancer. It's just not right. That fact, especially when the tumors are brain or adrenal,  is actually one of the BIG flags to doctors that a family might have a mutation like the p53.

I learned about Li Fraumeni Syndrome in the late 80's, early 90's. My brother had just been diagnosed with a brain tumor, months after my cousin had been diagnosed with a brain tumor. An aunt and an uncle were also diagnosed with cancer during these months. Our first thought was- stop drinking the flipping water- but then an amazing article hit the newsstands about these doctors in Boston who were studying family cancers- especially brain tumors. My grandfather died of a brain tumor- so my mom grabbed the phone and started making calls. She talked with a young doctor named Frederick Li who sympathized with our situation, but my brother passed too quickly to be helpful to their studies- there was no biological material. It was mentioned that if my dad were to get a brain tumor- that would be an interesting case study and please let them know. 3 years later, my mother had to make that call. My father was tested, he had the mutation. There were many sleepless nights spent discussing the implications for me and being tested. They let me have the choice. In true 17 year old fashion- I said- um No, thanks, can I go out with my friends? I didn't want to know. I wanted to live, I didn't want to be shackled with the burden of knowing that I was going to die of some rare painful cancer. From what I saw with my dad and brother- knowing vs. not knowing didn't change the outcome one darn bit. That harsh reality came crashing down around me in 2008 when my 3 year old was diagnosed with adrenal cancer. I thought for sure she had a brain tumor. I had gotten a degree in molcular biology, I studied genetics- we talked about Li Fraumeni Syndrome- I never processed that adrenocortical cancers were one of the main syndrome cancers. I had done my research. Before it was Li Fraumeni Syndrome, it was called SBLA- but not in any circles that I was in. It was a wikipedia article that I stumbled on when I was looking up adrenal hyperplasia that brought that to light. It happened to be the day before my son's surgeon called with a referral to an endocrinologist for his pancreas injury-I asked her about my daughter's symptoms and told her our family cancer history and she gave Lily a referral to endocrinology too. This after 2 pediatricians told me not to worry about precocious puberty in a 3 year old- it could happen.

So I called the doctors that studied my family in the 90's and I asked what to do for my other kids, I wanted to know the benefits of testing them. They were supportive and helpful and I also learned about an upcoming conference the following year. It had been decades, but my assumption was this was so rare- there weren't very many other families out there. I wanted to be there. I wanted to meet the others. I wanted to see if they learned to laugh through the tears like my family does- because sometimes laughter is the only medicine.  I wanted to see what progress had been made and where things stood. I wanted hope that my children wouldn't have to watch me die from cancer and I sure as heck don't want to see any of them go through it over and over. At this conference I met others who wanted, like me- to help support each other and work together to find better treatments, screening and ultimately a cure. We are tired of watching our families suffer. We want to help bring together all the different facets of knowledge, research, and experience with this syndrome. We want to be the link the connects the disease to the cure. We are that link. It will take time, and sometimes more time than those with LFS are given. I was so saddened to hear that a lady I met at the conference recently passed. It was agonizing to lose a friend in December who I met because of this disease. But these were two amazing, strong women. 2 women who brought so much love and light to those around them and who both had so much more living to do, but couldn't because of LFS. Since we started in 2009, we have hit lots of bumps and several new cancers in our group. That is the nature of the beast. But through people with Li Fraumeni Syndrome is a better understanding of cancer and through this understanding will be a cure!  If you are interested in donating time to a great cause- check out our support groups at Facebook- we welcome help with open arms. There is one for those with LFS and one for Friends and Family.  But most of all I have to thank my monkeys and my husband who have dealt with a pretty messy house and a scattered mom- but do so gracefully so that I can try to help create something that will do so much good.

https://www.facebook.com/groups/90503981891/ 

Adrenal Insufficiency Initiative

There is an initiative to get the life saving medication Solu-cortef ready available by EMS to treat people like Lily who have an adrenal crisis and are found unresponsive or in an emergency situation. Please support us by signing the petition. This initiative could save Lily's life. The condition may be rare, but it is easy to treat with a medication called Hydrocortisone which can also be used for other conditions. Thank you!

http://www.change.org/petitions/adrenal-insufficiency-united-create-protocols-for-adrenal-insufficiency#