Sunday, August 31, 2014

Living LFS

Have you hugged a MUTANT today?

I have. 5 years ago I learned that not only did I pass a dreadful genetic mutation on to one of my children, all four of them had it. Just like me.  I think of how I never got a chance to meet my grandfather. How my children never got to meet my dad. I want to meet my grandchildren.

Our bodies don't fight cancer like "normal" people's bodies do.
Where we only have one cancer fighting p53 gene, Most people have two.

So cancers grow and grow and grow.

Sometimes two or three at a time. Sometimes in itty bitty children. Sometime more than one family member at the same time.

Families should stick together. They should NOT get cancer together.
Children should NOT get cancer.

Almost 80% of pediatric adrenocortical tumors have mutations in the tp53 gene.  Not only did Lily's tumor have mutated p53- all of her cells do. She is a mini-mutant. It means she is at much higher risk for developing cancer again. So we screen her and hope we will be lucky. That she will be lucky.

The other kids have the mutation. We have been lucky so far. We screen them and hope we will be lucky. That they will be lucky. Those that know us know we just finished this routine. It's stressful. But we do it because it's our best odds. It's a part of living with a hereditary cancer syndrome.
Lily's drawing of us walking. It's in our genes. 

50% of people with Li Fraumeni Syndrome, mutants- as we affectionately call ourselves for the mutation in our genes, will get one cancer before they are 30 years old. I was lucky. I beat the odds. I was 36 when I got my first cancer.  My brother wasn't so lucky. He was 15.

In 2009, I tried to find others with Li Fraumeni Syndrome online. Facebook connected us, this rare group whose sense of humor was as twisted and warped as their DNA. Yet I am convinced, these mutants exemplify the theory that adversity only makes us stronger. They are truly the most resilient, positive group of people I have ever met. I heard stories just like mine. I heard stories more heart breaking than mine. They researched for me, with me. I researched for them.  They cried for me, with me. I cried for them.  Then we laughed together. We grew stronger together. We talked about a need in our LFS community, a need for community. They all were Living LFS with me. We were Living LFS together.

We finally got to cash in years worth of virtual hugs at a LFS Conference in Boston.  Real Hugs. Real Mutants. People who understood what it was like to have Li Fraumeni Syndrome. And they were living with it. Even though LFS is rare, our stories are not. In the LFS community the stories are far too much like mine, generations of cancer, far too many kids with cancer, way too many cancers.
I am proud to be a part of a new organization that will focus on bringing mutants together to share those stories.  This organization is LivingLFS.  I am passionate about helping mutants find the information and support they need to make living with Li Fraumeni Syndrome easier. I sincerely hope that someday we can truly make an impact and support research that will change the fate of LFS. We are already planning our first mutant meeting in Portland this October! Just to keep life interesting between raising 4 mini mutants in a military family in between chemo treatments!

In order to do this, we needed funds for a meeting space and since the Team Mallory Shirt was such a huge success- we decided to give t-shirts a whirl for LivingLFS through a CustomInk Booster. And it worked! It's working! We already have enough support to cover the meeting room and even some snacks during the meeting. I am so proud of the community and support for mutants, especially since it is such a rare syndrome. If you would like to show your support for LFS and our mission of supporting those with LFS and raising awareness for this disease, please check out the link below or just tell someone if the subject presents itself that you know someone Living with LFS- because that could really make a huge difference to someone with LFS! Have you hugged a mutant today?

Friday, August 22, 2014

The EVERYday challenge of LIVING with debilitating diseases

It's AUGUST and I am already dreading October. I'm dreading the pink washing of "awareness" that comes with Breast Cancer Awareness Month.  Millions upon millions of dollars have been raised for breast cancer research, awareness campaigns and survivor support. Treatments are better. Awareness is Greater. The disease is being caught earlier and being treated earlier. But the cure is elusive. I know these things take time- but as the world readies to cloak itself in PINK in October, the clock ticks for me and many others who are fighting the battle with the disease. We know a little more about who has a better chance at winning the war- but there is the undeniable element of luck.

When I first heard of the ALS Ice Bucket Challenge I was mostly annoyed. I tend to get annoyed easily and especially in the heart of chemo weeks on the tail of a summer that never really developed.  I was annoyed again the people just don't GET it.  If you are dumping ice on your head and not donating to charity- how does that help anything? It seems only to raise awareness that buckets of ice are cold and people will do crazy shit instead of donate to charity. Yet somehow in the past few weeks- over 55 MILLION dollars has been raised for a great cause. SO as it turns out people like to do crazy shit and can be compelled to donate too. For ALS is a horrible disease to have to face, to know that you have a family history of something and you just have to wait for the inevitable, secretly hoping you might be the lucky one. Watching those around you suffer,  wishing you could ease their pain all the while feeling guilty for wishing you could escape the fate they are not so lucky to.

The ALS Ice Bucket Challenge showcases the power of social media. It shows the power that awareness can bring. It HAS raised awareness of a rare disease and much needed funds to help support a community that is too busy fighting to LIVE. It highlights the importance of genetics and need of research for rare diseases. I know ALS families must cringe and dread the ever present cancer awareness and fundraising campaigns- while they watch family members deteriorate with few options to assist them. Most people not even knowing what Amyotrophic Lateral Sclerosis is. I hope sincerely that this campaign changes so much for these families. Just as the March of Dimes changed the face of Polio and has gone on to care for a greater need, I hope this changes the face of ALS. Living with debilitating disease is traumatic enough but to live it with no one around you truly understanding what you are facing and how it impacts your day to day life- forever- unless they have lived with it.

Living with Li Fraumeni Syndrome in all senses can be a debilitating disease. I have seen it destroy families and relationships and dreams. It can rob people of their physical and intellectual presence- essentially robbing them of who they are- much like ALS.  It changes relationships. It takes away birthdays and anniversaries, college dreams, hopes of having a family or a normal day. It robs people of savings or the ability to save. Time is spent on 24 hour care and unplanned surgeries and doctor's visits, at a minimum preventative care.  I consider myself one of the lucky ones- I didn't face cancer until I was 36. I had 36 years of living, I hope luck holds out for more. Many of those years were dedicated to others fighting LFS cancers, both family and friends. But many of them were care and cancer free too. Each one leaves an indelible mark on you. Each one is a little harder. But through those battles I see strength and I choose to see the positive. It isn't easy. It is damn hard when you watch a 3 year old battle multiple cancers- yet 5 years later when she is defying the odds- you retain hope. It is infuriating when friends battle the same cancers over and over- their lives being put on hold, hoping to be able to live. It's humbling to see others who have lost so much, give so much back to each other- because they know how hard it is.

There are hundreds of rare diseases and special causes out there. Each one DESERVES millions of dollars in funding. Most don't get it. I don't resent that, I appreciate it. Those that get funding nationally are those that post the greatest risk to the most people. Rare diseases don't fit the bill. Until you have a rare disease- then it fits every bill.  ALS, LFS,  Adrenal Insufficiency, Type 1 Diabetes,  childhood cancer, and so many more. The people most affected by these diseases are the least likely to have the resources to start fundraising campaigns and devote time to awareness. It is the outstanding people that we touch that are moved by our stories that can really help make a true difference in our lives. A lot of times it is just the luck of timing that determines the success of a campaign.  I know I feel exceptionally lucky to know so many generous caring people that have helped us and many other causes.  I think when we judge less and compartmentalize less and come together more as a community, we can make a huge difference- one rare disease and one challenge at a time.

Saturday, August 16, 2014

Don't Drive Angry Phil

Every time Phil pulls into a parking spot I cringe. He's never hit another car while parking but he seems to be a little more aggressive at squeezing into tight Hawaiian parking spaces than me.  I'm constantly yelping as he swings the van around and he laughs every single time.

It is one of the many ways chemo has affected me. I'm jumpier, more off balance. I don't mean to question his skills, but I do find myself critiquing his driving, a lot. I feel bad because his commute is pretty brutal- at least an hour each way- bumpah ta bumpah, sun in face bra. I often quote one of his favorite movies, Groundhog Day-

Don't Drive Angry Phil!

Yet he doesn't really drive angry, he just has much better skills than me- especially chemo sabe me. So one night we are sitting to dinner- recounting a fun story of how our 14 year old boy child decided it was ok to take the van for a spin around the block. I was not amused at the time- but in our house- as it was in my house growing up- public ridicule is a really good deterrent. The discomfort of facing the people you affect is an important life lesson. So we were laughing at his joyride and Phil was nice enough to share an anecdote from our youth. 

Now mind you- my brother turned 16 shortly before he was diagnosed with a brain tumor. I do believe he may have actually taken the official driving test post op with a baseball cap on to hide the scar. I'm pretty sure issuing driver's licenses to brain tumor patients has always been frowned upon- but as my mom always says- you do what you gotta do. I - in my 14 year old wisdom, refused to get into the car after a few episodes of crazy driving. Of course I had witnessed mild seizures and some of his memory deficit at home, but all in all it was standard 16 year old driving and standard sibling rivalry in a non standard situation. Yet I fell on my proverbial sword of traffic safety and concern for pedestrian welfare. 

The irony lies in how angry of a driver I was for the next several years. As I recovered from the loss of my brother, thrown into the brain tumor drill again with my dad- I was an angry teen driver at it's worst. And Phil bore witness to a lot of it and specifically one instance he decided to out me in to our children. The long and short of it involved me tailgating someone with much better brakes than my 82 Honda  and a swerve , mounting a curb and almost taking out a fence. Yet no cars, Jens or Phils were harmed by the conclusion of this tale.  I will leave the real story for Phil to tell- as it really is his- but will say the kids were in tears with us laughing at our past mistakes. I was also showing the 14 year old that we could relate, that we were human and that at the end of it- I learned to scale back the road rage and limit my law breaking to speed violations.  

The thing about cancer, or injustice, or pain, is there is a certain element of loss to all of it. A loss that is out of our control. It is very tough to relinquish control. To learn to accept the feelings that come with loss of control- the grief, the anger, the acceptance.  Some people never learn to regain control and hold out for others to give them direction. Some people take this loss and use control over others to fill it. Some people realize the loss is part of them, learn from it and use that to move forward. 

In my teens I liked to drive fast. It was a risk I thought I could control. My dad taught me to drive, mom's nerves couldn't handle it. After bearing 4 children who continually test their mortality- I'm with my mom on this. BUT back then I was a different girl, we were a different family. Dad would take me to the new neighborhood way down Smoky Hill Road. It was the skeleton of a neighborhood- just roads, no houses. He would let me take turns and try to stop and start and he would yell out situations- A car is coming at you and a dog just ran in front of you! I said I would swerve. He said I just failed my driver's test. If I swerved into the car- I might kill myself and everyone in the car. If I slammed on the breaks, I might fishtail to the same result. He gave me scenarios and possible results. Rain, snow, black ice, falling trees, other cars. I learned about defensive driving and planning ahead. I learned to predict problems to get a lead turn on reactions. 

I would get in the car and we would start with 12 points. When I lost all my points, the lesson was over. Even when I wasn't driving, we were always tallying points until it became a running joke. I'll give you two points for the kid on the bike- no way- he's in the crosswalk that's 5.  It that moment  we were tallying the value of life in relation to the rules of the road. All the while, he was teaching me to be on my guard- he would say there's a kid there on a bike to your right- if a car were to turn in front of you right now- where's your out? And we would talk about it. I learned to think about actions and reactions and to be prepared. 

Yet I wasn't prepared for my first ticket. It was spring break the year after dad died, Phil and I had gone to visit friends in Arizona. I was hauling mail.  Between the two of us- we could expertly spot cops hiding over and under over passes. Say that three times fast. Well we missed one and I was tagged going well over 90 in a 55. I was pretty worried that this was going to be the end of my driving career. The cop knocked it down to 80 and gave me the option of Driver's School. I would have to pay for the course, the speeding fine, but it would be no points on my record. Well glory be if I hadn't learned that come hell or high water I better keep points off my license. So the next day I called and enrolled in the class. 

The location was inconvenient, the time was inconvenient and I could tell the second I sat down in a dilapidated inner city classroom that everyone there felt the same enthusiasm towards being there. When this soulful woman walked in and asked who was excited about being here all of us looked around to see who exactly might take the bait. Not a hand went up. She smiled- me neither she told us- she had better things to do- and she knew we all did as well but something put us together in this room and she was going to be darned if she didn't do something with it. I think at that point everyone was as nervous as I was about the next 6 hours we were going to be spending there. 

She had us get up and arrange the desks in a circle so we could see each other. Then we went around the loop and told why we were there. For each person, she would ask a question- a really simple question like- to the guy who ran into another car in a school zone- what would you have done differently- he said- probably not eat that second cheeseburger. As it turns out- he was unwrapping a cheeseburger- and a glop of ketchup fell into his lap and while he looked down-slam! So she talked about distracted driving and asked everyone who was distracted when they got their ticket to raise their hand. Everyone did. We talked about life and anger and the big picture. We talked about zooming to get there 1 minute sooner- how important was that minute - because 1 minute could become 30 minutes late if we were pulled over- or never if we were in an accident. She reminded me of dad. She was no  nonsense- this is how it is- don't lie to me kinda lady.  Everyone in that room had a story and their story impacted why they were there at that moment and she had us talk about it. I half wondered if I had walked into a candid camera sponsored support group by mistake. 

When we had sufficiently been counseled as to our impact on the driving world and the larger world as a whole, she handed us an exam booklet. When we were done, we were free to go. It had been barely 2 hours. But in those 2 hours what I learned was more than defensive driving. I learned about actions and reactions, I learned that my actions have consequences but seemingly bad consequences aren't always bad. I learned that complete strangers can come together to help each other. I learned that there are people out there who are amazing and in their little corner are making a world of difference one bad driver at a time. This is where I learned that it's not so much what you do, but HOW you do it that makes a difference. If you are passionate and work to help other people make themselves better, not only will they be better, you will feel better. It is one of the many experiences that taught me people are angry for a lot of reasons and if you take the time to find out why, it makes a difference. It also taught me not to drive angry!

Sunday, August 10, 2014

Keeping Calm through Craziness and Cyclones

Everything happens for a reason.  That doesn't mean it's good or fun or that we don't have a say or that we do have a say- but if we can find the purpose, sometimes it makes the roughage a little more palatable.

After week one of chemo-lite- I was feeling pretty good. Running here and there getting the kids ready for school.

spending time with Bella, who I had promised just mommy and Bella-time.

Then the icky hit. I had overdone it. I knew at dinner- twice having to leave the table of leftovers and certain smells were making me nauseous.  When I feel ok, I feel the clock ticking the loudest. My lists are full, the checkmarks are few.  So the days where I feel ok- I take deep breaths and do what I can. Later that night the unrelenting nausea set in. I wasn't expecting it- after all I had only done the "lite" chemos. Maybe it was something I ate. It hits furiously- Phil comes in- not sure how to possibly help- there is no hair left to hold back- I guess that's a perk. I'm tired of finding the bright side.

As I get all four kids off to school, the news hits- not one but 2 hurricanes are on the way. GDMFCSSOB. Phil says they won't leave. He is reassuring himself, not me. He is kidding himself, not me. I am.  I know what this means. It means he will pack up and leave. He and the rest of the available pilots will escort millions of dollars of precious flight power over the ocean to dry land while those left behind prepare and protect our priceless treasures. Ultimately ironic as one of the primary missions of the unit is to protect the island, just not from this kinda threat. I know this drill- it's been many years since I've done it in earnest, yet it doesn't really differ from the living with cancer drill. You wait, you hope, you prepare the best you can- but you just don't know if or when it will hit and how bad it will be until it does.

I go about business when the realization hits. Lily's scans. The hurricane is supposed to hit the night of Lily's scans. OH mother of GDMFCSSOB. The familiar PTSD scanxiety sets in. He still thinks they aren't going. I ask my sister in law to be on call to take the kids to school the morning of scans. I know he will be gone. He packs a bag, because superstition dictates if you are prepared- it won't be as bad. That's bullshit. Sometimes it just doesn't matter how prepared you are- it still is bad. But I plug along with mental checklists- start doing laundry. Get out the huge lemonade jug for water, grab a few supplies from walmart and safeway. There is no water or bread to be found in the stores. People are expecting the worst.  The last two hurricanes that hit hard were Iwa and Iniki. We are due. It doesn't help that the beyatch's name is Iselle.  I secretly hope she is graceful as her name and not a force to be reckoned with like my Belle.

I look through my supplies. Somehow my emergency radio and can opener are missing. I hit amazon and overnight some supplies. I know they won't be found in stores. The thing about hurricanes is you have time to prepare- it's not like a tornado that just pops up or an earthquake that sneaks up and shakes you. Phil comes home with three cases of water and a metal suitcase. The one that fits in the jet. He rallies the kids to help him put all the patio furniture in the garage.  I feel lost. I am grateful that I didn't have abraxane.  We barely speak.  I know he has to go. Part of me finds the bright side. For months he's gone out of his way to cook for me, make my load easier. Part of me worried that he thinks I can no longer do it. Part of me wonders if I can. He wouldn't have left if he thought I couldn't handle it. Not exactly the way I wanted to rally that vote of confidence.

I am at the sink washing dishes when a thought hits me.  If she were still alive, my grandmother would have been calling asking how we were. She'd be praying up a storm and keep the family updated. A calm settles after the tears. What will be, will be. I know my angel of weather will look out for us. Phil shaves my head and I take a cold shower- because in the flurry of events- no one noticed that there is no hot water. He resets the water heater and we cross our fingers, we go to bed.  Phil and I get up at 4am. I see him off and get ready for a long day. I wake Lily and we begin our adventure to scan land. It is just another morning in Hawaii- sunny, breezy. No hint of the impending danger on any front. I hear a wimper from the back seat and my heart breaks. It's going to be ok monkey- I tell her.

Did you grab the movies?  She sobs.  Oh no. 2.5 hours in the tube and she forgot her movies. I'm wondering what exactly is in the 35 pound backpack she is lugging around.  I take a quick detour to walmart. This could be an epic bad decision. epic. News stations had been feeding images of crazy lines outside stores, people waiting for water. The parking lot is nearly empty. I sigh. We jog in- grab the latest Barbie flick and are out of there in a flash.

We are the first car at the valet and the first to check in at MRI.  They allow me to come back with Lily as she gets ready. She hands off the movie and the tech scans the length- Barbie movies aren't particularly long. Ideally titanic or dances with wolves would be better suited for today's scan- but she wants to watch Barbie- it's her hospital visual comfort food. She is prepared that they have to stick her.  She gets settled, her movie goggles on and I make my way to the waiting room. It's hurricane coverage. This is not helping my calm. I have to move. I let them know I'm going to get some tea- they let me know it's going to be awhile.

I stop to visit one of my favorite nurses on the way. We chat for an hour. I decide I better check back. I grab my tea and as I get settled in the waiting room- Lily bursts through the door wearing a ridiculous hair net and strikes a grand pose. She declares that she has gone to the bathroom and wanted to check on me. I sneak her a big bite of purple flower cookie that I grabbed for her. She jumps up and down, then heads back in to finish her MRI.

They call me back. My heart lurches and sputters, settling somewhere in my ears- a pounding drum.  She just wanted to say hi- they were taking a quick stretch break.  She wanted to make sure I was still there. I'm still here. Her leg hurts. Try and stretch it.  They only have 15 minutes left- you can do it Lil.

She finishes up and practically drags me out of there- lest there be a chance they call her back for more. I know that feeling. Overall it was less than 3 hours. That's a good sign. The tech seemed fairly chipper as we left and told me how great she did- I'm hoping that's code for - nothing to see here- just nice pretty boring organs.  We won't find out for days, the official report. I hope with the hurricane- they don't get lost in the chaos.

We stop to get gas and ice on the way home. Drop the ice in the cooler and grab celebratory Panda on the way to get Bella from school.  The post scan migraine I always get is starting to creep up behind my eyes- but I just don't have time for it today.  We detour to the beach by school since we are 5 minutes early. Lily collects shells, I collect thoughts- tally lists in my head. Phil texts that he made it to Vegas.  One more worry to check off the list.

We head home and finish preparations. Phillip calls to tell me wrestling is cancelled because of the storm and can he go work out with his friend? I give him an hour and a half. I lay down for an hour then head downstairs to wait it out. Then I remember we were supposed to go to the Ballet. Well I remember because the girls are asking incessantly. As of yet, it's not cancelled but there is no way. There is just no way. The girls are in tears. Phillip flies through the door at 4:59. A whole minute to spare before Hurricane mom knocked out all his electronics. I shoo the girls to the park- as of now it's still partly cloudy- they need to enjoy the weather while they can.

Phillip is staggering around the kitchen. At first I don't pay attention- he usually comes right home and forages for food- he's 14- its what they do. I have no discernible dinner plan, so I allow it. I realize he is drenched in sweat and pacing. GDMFCSSOB- his blood sugar is dropping and fast.  What's your blood sugar?  He looks right past me. I yell at him to sit the hell down.  He moves towards the chair and mumbles 32. THIRTY TWO?!? Its supposed to be above 70. Usually people lose consciousness around 20-something. He is fumbling with an applesauce packet- that won't do- straight sugar is needed. I grab a juice box- empty it in a cup and dump in two spoons of sugar and hand it to him.   I go to his room and get his glucometer.  It's still dropping he says. I start rifling through the pantry- where is our emergency dr. pepper?  I finally found it. He takes his blood sugar again- its 36. shit.shit.shit.damn. I hand him the pop and he sucks it down.  We are dangerously close to needing the glucagon shot and that means possibly riding out the storm in the hospital. GDMFCSSOB. We wait 10 minutes and he takes his blood sugar again- my sister in law texts to see if he's coming over for dinner. shit- she was going to feed him for me while the girls and I were at the ballet.  He takes his blood sugar again. 56. We are at least on the rise. I hand him another juice box. I text an apology and that we are dealing with a tanked blood sugar.  I think for a second- what if we had left for the ballet? Would he have been able to get through this alone. I decide not to think about it. Everything happens for a reason.  She texts asking if we want pizza- oh dear this woman is a god send. Absolutely.  Pizza.

We sit around watching the news, eating pizza. My adrenaline stores depleted, energy gone. I bleach the bathtubs so I can fill them with water. I plug both the kids bathtubs and run a hot bath for me. The girls' tub is draining and the faucet is leaking. Hmmm. I invert a bowl and put it over the drain- it suctions down. I think I have fixed the problem, so I go to turn my faucet off and it spins to off- yet it is definitely not off. It is the opposite of off. I spin the knob back and forth- it finally grabs and I get it turned off before I have premature natural disaster flooding. The par on this course sucks. I refuse to say it could be worse because according to the news- worse is still barreling towards us.  All I can do is try to relax in the bath and then crawl into bed, expecting to wake up to the roar of wind and the crash of trees.

I wake up to quiet. It's cloudy but the sun is poking through. I turn on the news- still waiting but its not looking too bad. I check the tubs- both drained. Add that to the honey-do list.  I make breakfast and take a walk with the girls before the storm officially blows over us.  We go to the beach to check out the waves. A few surfers are out tempting fate- but it doesn't look too stormy. We go visit with the cousins for awhile and head home for lunch. A big sigh of relief. The world has now heard of the storm and I get a couple texts from afar. I assure everyone we are fine. Although fine seems to have taken on the meaning -perpetual state of crisis with a background of paradise.

I hear the kids grumbling how we did all that work and getting ready for nothing. I explain to them that it's not for nothing- that is exactly WHY we do this. I know it's hard for them to understand. We feel a sigh of relief because we are lucky, the storm did not hit us. But there are many on the Big Island who weren't so lucky. It could have easily been us.  It is amazing to me how scanxiety parallels waiting for a hurricane that's on track for you. It's a lesson in being prepared and faith and control. And sometimes you just have to take it an hour at a time. You don't know what could be around the corner- it might just be sunshine.

Lily Kay Monkey

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