Kortne’s Story about Learning to Live with LFS

“If you were me what would you do?”

Working as a nurse, I have been asked this question so many times. It wasn’t until I was the one asking this same question, that I could truly identify with the countless number of emotions hidden behind it. Behind this question is so much more,

it is an unsure where to start feeling,

a feeling of being scared beyond belief,

it is anxiety,

it is needing assurance,

it is searching for empowerment.

This question is the first step of processing a cancer diagnosis that is so unfair and so unwanted.

Dealing with cancer and receiving an unwanted diagnoses is a foreign world.

Understanding the disease process

awaiting pending pathology reports and diagnostic testing

preparing for ultimately bad news.

For me, this foreign world has become too familiar. Cancer chose me not once …not twice…not even three times. FOUR. Four times cancer chose me. I never chose it or anything that comes along with it! I hate cancer. But Guess what? I’m still living, I’m still enjoying my life, I’m still fighting.  As much as I hate to admit, cancer taught me so much about myself and about life. It’s easy to forget how precious being alive really is!

My first battle with cancer was at age 9. I had a rare type of sarcoma called a liposarcoma. After many appointments, tests, and scans the team of doctors recommended surgical resection. 2 surgeries later, I was deemed cancer free. Going through this experience at such a young age made me grow up faster than others my age and process thoughts that children should not have to worry about. That was my first cancer; it was also my first cancer life lesson. One that had a lasting impact on my life.

My first cancer was the “ah-ha” moment that inspired me to want to become a nurse. I like to think that this was cancer’s way to open my eyes, my heart, and my mind to see that I needed to help others through medicine. If it wasn’t for cancer would I still have dreamed of becoming a nurse to help others like I was helped? No one knows.

15 years later I received my 2nd cancer diagnosis, this time melanoma. I was guilty of using the tanning bed and laying out in the sun as any teenager does. Did I think it would cause a minor sun burn at times? Yes. Did I think it wound cause melanoma, the deadliest type of skin cancer there is? Absolutely not. I wish I knew then what I know now!

The plan of attack this time was surgery with a wide local excision to remove the cancer and check my lymph nodes with a sentinel lymph node biopsy.  Then they had to cover the gouged out area (think a putt-putt golf course hole) with a skin graph. I felt fortunate that the cancer had not spread to my lymph nodes and no further treatment was necessary. My prayers were answered and I was blessed. My life resumed, I got married, my career advanced, yet there was always a small cloud of fear hanging over me that this cancer would return or metastasize to other areas of my body.

I just could not have predicted the hard hitting craziness 3 years later in 2017. I got my flu shot the second week in October.  1 week later I developed sudden numbness and tingling in my right arm and hand, the same arm as my flu shot.  Of course I downplayed it and tried to convince myself it was a reaction to the flu shot. Nurses have a bad habit of self persuasion. It continued the next day and on the 3rd day, while I was driving to work I went to press the brake and my right leg wouldn’t move. I knew I could no longer downplay this and convince myself it was from the flu shot, it was in my best interest to see my doctor immediately.  Deep down in my heart I knew it was more.

I was scheduled for a same day MRI. As a nurse, I knew all of the symptoms I had suddenly developed over the course of 3 days pointed toward some kind of neurological diagnosis. I refused to accept that possibility. As the MRI techs locked the “bird cage” over my head, the  fear of what the MRI might show hit me all at once. I do not remember a time in my life that I had ever been so scared. The tears flowed as I was slid into the machine. I just did not want to know, but needed to know so bad all at the same time.  I was walking out of the imaging center, my phone rang and my heart dropped.

“I am so sorry I have to tell you this but the MRI showed that you have a brain tumor.”

I had so many emotions,

so many questions,

so many thoughts,

yet I was numb and spaced out.

Every bit of my medical knowledge went away and I was left with nothing not say. I needed time to absorb and time to think.

That same night as my husband and I laid there attempting to sleep, I must have had a strange feeling because I said to him in my serious patient education nurse voice “do you know what to do just in case I were to have a seizure? Not that I’m going to or anything but just in case I were to ever down the road – just roll me onto my left side and call 911.” 3ish hours later he did just that. Things moved so fast from there.

Less than 2 weeks later November 9, 2017 . I was anxiously rolling into my awake craniotomy. I knew it would forever change my life. The outcome was uncertain. I was as still and calm as possible because I knew that on the other side of the sterile drape was the man my husband, parents and I had trusted to cut into my brain. My brain!!! So crazy to think about! As the hours went by, I was constantly communicating with the physician’s assistant who was under the drape with me, evaluating my neurological status the entire time. He never left me and I was not fearful because I wasn’t alone under there.

Flash card after flash card and a series of zings and zaps to test for feeling in my limbs and it was finally time for the intra-op MRI. This showed a small amount of residual tumor and my surgeon elected to go back in after it. At this point, he said I would be put to sleep for the last part of the surgery and would wake up in recovery. Call me crazy, but I asked to be allowed to stay awake for the remainder of surgery. My thoughts were- if I was asleep I would not be able to tell the surgical team if I had any “weird feelings or sensations.” I was the only one who knew what I was feeling in my own body and I wanted to make sure I did everything in my power to get the best end result! It was no surprise that I was the first patient to ever make this request. Maybe it is my interest in the medical world or maybe I’m just crazy.

Living with a Brain Cancer diagnosis turns your world upside down. There is no cure. The pathology of my tumor came back, Astrocytoma Grade II. With this diagnosis and it being my 3rd primary cancer, I was referred for genetic testing. Genetic Test results came back showing that I have a condition called Li-Fraumeni Syndrome (LFS) which is linked to a mutation in the TP53 tumor suppressor gene. People with this mutation have a much higher risk of developing cancer or multiple cancers in their lifetime. Mission accomplished, I guess.

I previously thought that I had to be the unluckiest human on the planet. The diagnosis helped make sense of the cancer, finally an answer. LFS is not only emotionally overwhelming but comes with an overwhelming amount of MRIs and other screenings. This caused a new condition- extreme “scanxiety” being fearful of what may be found and what possibilities I could face next. I  completed the recommended imaging and all that was left on the to-do list was an EGD and a colonoscopy.  I was not particularly worried about either since the MRI of my chest, abdomen, and pelvis showed no signs of malignancy. I figured I had nothing to worry about, right?

After waking up from the procedure I was told the doctor would come talk to me in a small room outside of the recovery suite. “EGD looked great, but there is an area in your descending colon that I am very concerned is cancer.” A phone call a few hours later confirmed that I did indeed have colon cancer. The news itself was terrible and the fact that I had been diagnosed with brain cancer 4 months earlier made it almost impossible to process. I was reminded again how much I hate cancer.

In April 2018, I had a a left colectomy and lymph nodes removed. The pathology returned as stage 3b with lymph node involvement. My medical team’s treatment plan for me is 12 cycles of FOLFOX chemotherapy.  My personal life plan remains the same as always, to be tougher than cancer, any and all cancers. I want to be the light to others and share my story to show that even though sometimes cancer tries to suck the life out of us, we can not let it.

Life can still be wonderful in countless ways! We are blessed in so many ways.
It’s hard to figure out how exactly to plan like you will live forever while living like you are dying at the same time. I do not know what my future holds and the unknown is always the scariest part! Being only 28, this is an “adulting” situation I still have to figure out. The one thing I do know is that LFS and cancer has changed my life and the lives of my family and those who love me. I have faith in God’s plan for my life and that good will come from all of the bad.

I stopped thinking I have to have a 5 year plan or a 10 year plan and learned to just live.

To make the most of every day.

To do the things that make you happy.

To spend your time with those that mean the most.

To find the goodness in tragedy.

To go on the trips.

To take lots of pictures.

To cherish making memories.

To love all the sloppy dog kisses.

To eat macaroni and cheese for breakfast.

To try new things. To learn new things.

To be kind to everyone you meet.

To right all wrongs.

To be best person you can be. And to share my story to help someone else .


International Women’s Day- LFS in WOMEN

 

Women with Li-Fraumeni Syndrome have close to 100% chance of developing cancer in their lifetime. About half of all these cancers develop before age 30 and about 50% of the risk is due to breast cancer. The risk of breast cancer by age 60 for women is 85%.  So, what’s a lady mutant to do?

Understand YOUR Risk.

Not everyone’s risk is the same. Meeting with a practitioner, ideally a Genetic Counselor familiar with LFS is a good way to get help assess your risk of cancer based on your mutation, family history and other factors. Understanding your risk can help you make screening and treatment decisions. Find a Genetic counselor at www.nsgc.org

Women with LFS tend to get breast cancer earlier than the normal population. The cancer risk in general is higher for certain cancers at certain age ranges.

  • 0-10 years- higher risk of Soft Tissue Sarcomas, Brain Tumors, and Adrenocortical Carcinoma
  • 11-20 years- higher risk for Bone Sarcomas
  • >20 years- higher risk for Breast and Brain Tumors

Know YOUR Body.

Just as one size jeans don’t fit everyone, neither do our genes- even within the same family. We do look at family history for trends, but we are all unique and rare and often our cancers are too. It’s important to not only do monthly self breast exams, but self exams. Know your body, what works and makes you feel your best and what doesn’t. Have any changes checked out by a professional. We usually follow a 2 week rule for lumps, bumps, and weirdisms- if it hasn’t resolved or gotten better in 2 weeks, it’s time to have a doctor take a look and go from there. Ignoring it doesn’t make it go away. We grow weird, not all of it is cancer. Just as you know what jeans feel the best, you know your body. If things are off or don’t feel right, it’s time to call and get an opinion of a clinician.

LIVE Despite YOUR LFS.

LFS is rare. You are even more rare. You get to make the calls on how you live YOUR best life. You get to make the calls about screening, about treatment, about your health. There are so many tough decisions in life, even tougher ones in dealing with Li-Fraumeni Syndrome. What to eat? How to avoid radiation? Should I have children? Should I have my children tested? What is that lump?

Learning Li-Fraumeni Syndrome

Learn as much as you can. Learn from doctors. Learn from reading. Learn from others. Learn about LFS. Learn about nutrition and radiation. Learn about cancer. Then tuck that knowledge away and hope you won’t need it and LIVE. Learn by living.  For You. There are many things we do not have any control over, but we can control us. We can’t control our feelings, we can control how we react to them. We can’t control IF we get cancer, but we CAN control how we navigate our risk. If the recommended screening is too much and adds too much stress to your life, finances, and sanity- talk with your provider. There are options. There is support.

Find your Tribe. Sometimes you can feel like you are on an island called LFS. You take trips to normal, but often are yanked back by screening, scanxiety, cancer. You are not alone. You are strong. Look at finding support as prophylactic action to prevent insanity. Your Tribe does not have to be LFS related(if you are looking for LFS support- please check out our support groups on Facebook. They are simply amazing). Find Support Here. Find what and who works for you.

The majority of our support groups are women. This represents not only the cancer risk associated with LFS in women, but how people deal differently. Although there are different risks associated with gender, coping is not age, gender, or cancer specific. Neither is living LFS.

More Reading on LFS:

LFS Gene Review:  https://www.ncbi.nlm.nih.gov/books/NBK1311/

Breast Cancer and TP53:  https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5790840/