Please Tell Me More About the BRCA1 and BRAC2 Mutation. 
Or...the story of how MightyMightyMunson found out she's an XMAN...er...BRCA-WOMAN?



Blog Updated: March 31, 2017 at 9:35 PM MDT 


Are you wondering what in the weird, wonky world I'm muttering about when I mention the BRCA1 mutation that I inherited? This is a very good question, so here's a super-quick crash course into my genetics, or as I like to joke, the gift that keeps on giving. SUPER IMPORTANT NOTE: If you have this mutation, have been diagnosed with cancer (HUGS!) have family members with this mutation or a family history of breast or ovarian cancers, please get additional information so you can be empowered and educated on how to reduce your cancer risk (I have some helpful hyperlinks below!) 

So, when everything is working correctly (which, thankfully, is most of the time for people) BRCA1 and BRCA2 are human genes that are responsible for suppressing tumor growth by producing special proteins that repair mutated or damaged DNA. When something happens to either of these genes (in my case, an inherited mutation of the BRCA1 gene) then damaged DNA may not be repaired properly. 

Over time, due to the lack of repairs, additional mutations or damage may occur and this increases one's risks of cancer. Most people who have heard about BRCA1 or BRCA2 automatically associate these mutations with breast and ovarian cancers, but people with these mutations have statistically higher risk for several other types of cancers, including pancreatic and prostate cancer. (Yes, men can inherit this gene too and absolutely should be tested if the mutation runs in the family!)

OK - SO HOW DO I KNOW IF I HAVE THIS MUTATION?
Great Question! To know for sure if you have inherited any mutations in these genes, you will need to work closely with your medial care professional, discuss your personal and family cancer history and, if you appear to be at risk, you will have blood drawn which will then be sent to a special laboratory for testing. If the lab determines that you have atypical mutations that put you at increased cancer risk, your health care team will be notified and together you will come up with a plan to help protect you and those you love. 

WHAT'S MY STORY ? (SO FAR...)
In my case, I have a STRONG (okay, this can't be overstated enough) family history of both breast and ovarian cancer on both sides of my family. My mother and two of her stupendous sisters are breast cancer and/or ovarian cancer survivors (but do not carry either the BRCA1 or BRCA2 mutation). While they don't have the mutation, having a first degree relative (my mom) and several second degree relatives (my aunts) puts me at higher cancer risk, so I was already religious about getting yearly mammograms and doing monthly self breasts exams. 

On my dad's side of the family, however, it's a different story. (Think the genes that time forgot horror type of story) My grandmother had breast cancer at a young age and had a radical mastectomy that kept her alive for many years, but left her suffering with horrendous scarring and limited mobility. She died from cancer when I was in high school. I also lost my beautiful aunt to ovarian cancer. When one of my younger cousins was diagnosed with cancer while she was still in her 30's we found out as a family that the BRAC1 mutation was present. My cousin is an amazing mom and wife, a marathon runner and has fought like a lioness to beat this blasted disease and I love her and pray for her every day. 

When another cousin who I absolutely adore got tested, she too found out that she had inherited the mutation. This gorgeous southern belle cousin of mine is also whip snap smart and has her master's degree in nursing administration and being very much in the medical know, immediately went about reducing her cancer risk AND became a passionate advocate for early detection and education about the risks associated with hereditary cancers. Since my father has already passed away, she encouraged me to get tested to see if I had inherited the mutation and as I love, respect and have looked up to her my whole life, I marched into my doctor's and said, "Let's get me tested!"

So when I got the results back that I was BRCA1 positive and also had Lynch Syndrome (I'll talk about that later), it wasn't a surprise, but was deeply sobering. I'm 42 which is two years younger than the average age that a woman with the BRAC1 mutation develops cancer, so my first priority was to get a base line and ensure that nothing was a brewing in my breasts or reproductive organs. 

ALL-RIGHTY THEN - HOW DO I DEVELOP A HEALTHY BASE LINE? 
Before I go into baselines, I need to give a serious, serious shout out to my health care professional. She's SO smart and has been a wonderful partner and friend in this process. For now, unless she indicates otherwise, I'll keep her name private, but I know that I am extremely fortunate to have such a brilliant and compassionate advocate on my team. I also want to thank my husband, 2 loving teenage boys and my angel-mother, who has walked this journey and is holding my hand and as I too, go through this experience. I also have to note that my base line process is still very much a work in process, which is why I wanted to start this blog and document what a person goes through. 

REDUCING REPRODUCTIVE ORGAN RISK
(AKA - LET THOSE OVARIES GO! They're bad eggs!)
The first thing we have done to establish a baseline, was to have a CA125 blood test (which looks for uterine cancer markers in the blood) and a trans-vaginal ultrasound of my ovaries, fallopian tubes and uterus. In addition to the BRAC1 mutation, I also have poly-ovarian cystic disease (POCD) which means that my ovaries get cysts on them that can cause bleeding and pain. There are also other snazzy side-effects (difficulty losing weight and the not so great ability to grow a mean mustache) that I have tried to counter with medicine like metformin and a Mirena IUD.

The ultrasound revealed that my ovaries had cysts all over them (no surprise there!) and that I had fibroid tumors (these are non-cancerous) growing in my uterus. This WAS a surprise. One tumor, who I have named Sir Piss-A-Lot (Yes, I name weird things) is a big ol' fellow (think small grapefruit sized) and is pressing directly on my bladder, which explains FAR too many mis-adventures in female urinary incontinence. 😠 Seriously folks, I have to plan a sneeze several steps in advance or I am in trouble, if you know what I mean!

DOES A TRANS-VAGINAL ULTRASOUND HURT? 
  • No, but it CAN be uncomfortable. The ultra-sound tech takes a wand (not the fun vibrating kind, mind you) and inserts it into your vagina. In my case, with the fibroids, cysts and an ill-tempered left ovary that was not keen on staying put, the tech had to move the wand around to find everything and a couple of times, it was mildly painful. This however, is the exception to the rule. Bear in mind that often a trans-vaginal ultrasound is not needed and a regular abdominal ultrasound can work perfectly well, so don't let this stand in your way of reducing cancer risk. 
WHAT ABOUT THE BLOOD TEST? 
  • As of 3/31/2017 I am still waiting on results and I'll post them as soon as I get them. (Crosses fingers, toes and other cross-able appendages)
SO, WHAT HAPPENS NEXT? 
  • I have a full hysterectomy scheduled for mid-April with a fabulous surgeon. This will be a laparoscopic surgery that will remove my ovaries, cervix, fallopian tubes and uterus. Once removed, my organs will be sent to a pathologist to make sure that my dearly departed lady parts were, in fact, cancer free. If I get the green light, then I am in the clear, metaphorically speaking. If not, then we move to a plan to deal with whatever type of malignancy is found. Believe me, if that happens, you'll know about it!
WAIT? WHAT ABOUT YOUR POOR BREASTS? HAVE YOU FORGOTTEN ALL ABOUT THEM?
No, my girls (the right one is named MOLLY and the left one is named FRIEDA - as mentioned previously, I like to name strange things), are also going to seek greener pastures than my broad chest. The risks for keeping these old DD's is too darn high. Based on my genetic profile, I have an 87% chance of contracting breast cancer in my lifetime (YIKES!) and about a 50% chance of contracting breast cancer before I turn 50 (DOUBLE YIKES!). I don't have to be a gambling woman to know those are pretty crappy odds. 

Before the mastectomy, however, I am also having a breast MRI to double check and make sure the girls are cancer free, just to err on the side of caution. This procedure is next week and I will update this page with results. While self-exams and mammograms are absolutely essential weapons in your arsenal, if you are younger, have dense breast tissue or are high risk, a MRI is the gold standard of setting a solid baseline of how you boobies are doing. It can detect cancers too small to feel or see via mammogram which is why I am getting one before surgery. 

Regardless of the result, I am going to move forward with the double mastectomy and have a consultation with the same surgeon that operated on my mother at the beginning of May (one adventure at a time, right?) to get everything set up. As with my other doctors, she is amazing and her last name has the words "TIT"  and "Sore" in it, which I love!  I have chosen NOT to have any sort of breast reconstruction. Yes, that's right. After years of back pain, this Utah harbor chick is going as flat as a board. (Oh, that my tummy were so easy to flatten. Le Sigh.) IF and when I want to look "perky", I'll wear prosthetic's. (I have a secret diabolical plan to weird people out by putting an "A CUP" on one side and a "D CUP" on the other side or point the false boobs in the wrong direction, etc. This is going to be serious fun!)
ARE YOU SURE? 
Do you see how happy and confident Olive Oyl is? Yes, I am 100% sure. Every woman has to make this choice and my heart, mind, spine and sense of angular adventure are pointing firmly towards being delightfully flat chested. I am a low maintenance woman and having implants, while they honestly look amazing, are no low maintenance items, which is another big factor in my choice to go without reconstruction. For those fearing I will miss Molly and Frieda, please know that I am planning on replacing them with very pretty and slightly irreverent tattoos once everything has healed up. 

For some people, this seems like a really big step to take. Yes, some women do yearly MRI's and others hope for the best, but for me, I don't see any other option that makes statistical sense and while I am a deeply spiritual woman, I am also very big on math, science and the numbers are so seriously not in my favor, that I am left with no doubts, whatsoever, that this is the right path for me. 

So, this is where this page ends for the evening....EXCEPT FOR SOME AMAZING and OH, SO EDUCATIONAL HYPERLINKS! Read up, my darling dears! 





As always, if you have any questions, want more information or need to chat, leave a comment below and I will do my utmost to answer. If you want to be a troll, go find another bridge to snivel under. I've got my life to live. 

Namu Amida Butsu! 

MMMunson 

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