Thursday, October 30, 2014

I made up my mind when I was a young girl; I've been given this one world, I won't worry it away...

Oh, hello there. Were you searching your local grocery store for my face on a milk carton, or frantically looking on one of those "Have You Seen Me?" ads? Humor me and say you were.

I'm sorry, but this never gets old for me. 

A lot of you reached out wondering if I've had my surgery or if I was having a bad lupus flare, and thankfully neither of those things has happened; I needed to unplug for awhile and clear my head. I wasn't overwhelmed with having a mastectomy until I made it "public" and blogged about it, then all of a sudden I was all HOLYSHITTHISISHAPPENING.

A few weeks ago I was going through my planner, and it dawned on me it's been one year since I first saw McDicky. You can read all about that first encounter here. The day McDicky and I met, he said to give him one year to make me feel better, and two to get me in remission.

As I sat there reminiscing him saying he didn't care if I thought he was a dick (hence, McDicky), I realized I feel better. This is the best I've looked and felt in three years. That damn McDicky was right!

So instead of moping in bed watching bad TV as I had been the few previous days, I decided to get off my ass and enjoy this time. Because, who really knows how long it will last?

And that's what I've been doing- enjoying the beautiful fall weather, dancing on top of bars, Friend's Dinner, tailgating for an Eagles game, and spending as much time out of the house as I can. Cause Lord knows once I have the mastectomy, it's going to be a looooooooong, painful winter, on the couch watching bad TV.

Moping with Princess Matzo Ball

Wings and pumpkin beer with my hunky guy

Friend's Dinner

The Girls 


Dressed up for a family wedding at the place Steve & I got married!
The guy in the top right is Sean, the bartender at our wedding almost 7 years ago!

My best friend's husband was diagnosed with ALS earlier this year. It's been nothing short of devastating, as Genesis and Jason have two beautiful children and more loving family and friends than any two people I know. I can't explain how helpless I feel as the life of my best friend and the man she loves crumbles around them. There are no words. But over these few months, I've watched Genesis take on these burdens with the power of a super hero. She holds it together better than anyone I've ever seen, for the sake of her family, for her children, for Jason. Because that's what you do when life deals you one hell of a shitty hand. 
Last Friday we went to the Beef and Beer of all Beef and Beers. The event put together was incredible, and I'm convinced Kisha and Tiffany need to start an event planning business. Hundreds of people, so many that have never even met Genesis and Jay, but came to support their beautiful family. It was amazing to look around and see so many there for two of the most important people in my world. The reality is their journey with ALS is not going to get easier, unfortunately, quite the opposite. But in those few hours, I hope both Genesis and Jason know that they will never be going through this alone.
I love you both <3

And that's where I've been, making the best of the worst. Because what else is there to do? More to come.

Love you all <3

linking up with my gal Joey

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Wednesday, October 1, 2014

My lovely lady lumps...

Our recent SteveFlix (Netflix only better!) binge has been Breaking Bad. Damn, what a fantastic show. It speaks to my inner chemistry nerd (don't forget, I'm the girl who does stoichiometry for fun), and it's just damn good. Bryan Cranston- love him!

In season 2, Walt has a PET/CT scan to determine if the treatments are working, or if the lung cancer metastasized. He gets a glimpse of his scan and sees a giant mass in his right lung. Convinced he has weeks, at most, to live, he spends every waking moment cooking enough meth so his family will be financially stable once he's gone.

Fast-forward to he and his family getting the results at the oncologist, they are told that not only is the treatment working, but also he's in remission. The mass he saw was inflammation, an effect from the radiation. His family is overjoyed, and Walt sits there in disbelief, questioning the doctor. Next you see him in the rest room, he's irate and starts beating the hell out of the paper towel holder.

When I received the call from the oncologist (hi Danielle!) that the mass in my breast was not malignant, I was at Olive Garden with Steve, Brie, Pete and PJ. The mass was atypical lobular hyperplasia (ALH), pre-cancer, and I would still need surgery. I yelled out the good news, Brie cheered and cried, Steve and Pete were thrilled, and I think PJ threw his crayons. I smiled and laughed with my family, but the truth is, I was in disbelief and seething inside.

I felt like Walt; I didn't see my films, but the radiology technician told me immediately after my diagnostic mammogram the radiologist categorized it BI-RADS 5, highly suggestive of cancer (I Googled BI-RADS the minute I got in the car after the biopsy and read a score of 5 means a 95% chance of breast cancer. Enter panic attack). Then she left me alone in the waiting room to sit and cry before I was called back for the biopsy. So when it wasn't cancer, part of me was angry, surely they were reading someone else's results. I refrained from heading into the bathroom and going batshit on the paper towel dispenser, but my smiles and cheers were fake. I don't know how to articulate this without sounding like a loony tune, and I certainly hope anyone who's received a breast cancer diagnosis doesn't take offense to this.

It sent a shock through my entire being. I spent those 4 and a half days on an emotional roller coaster waiting for the biopsy results, CONVINCED of the worst. I KNEW that lump was cancer; lumps in the breast are ALWAYS cancer in my family. I researched statistics, options, physicians, I even wrote out lists of the drugs I would receive in chemotherapy. And when I got the news it wasn't, it made my brain explode.

Steve came to Olive Garden straight from work, so we drove separately. That drive home was not full of joyful, happy, beautiful tears, they were angry, banging the steering wheel, feeling so confused, livid and overwhelmed, burning hot OMIGODWHATTHEFUCKISWRONGWITHME tears.

Breast cancer has come after almost every woman in two entire generations on my mother's side. For us, it's a matter of when, not if, a black cancer cloud always looming over our heads. When an oncologist raises her eyebrows when I go over my family history, I take the hint that it's not good.

There are very few things we have control over in life. I chose to marry Steve (dude, what were you thinking?), but I can't control my punching him in the face if he watches another episode of Dr. Who when I'm home (kidding! But that show, ugh, awful!). I had no control over getting sick with lupus, and how it affected our weeks-old marriage. I can't control if I'm going to wake up one morning in kidney failure or unable to walk from a stroke caused by lupus, and I have no power over when my pulmonary valve will start failing from my congenital heart defect and I'll need open-heart surgery.

But, there is one thing I can take control of, and that is a way to GREATLY reduce my risk of ever developing breast cancer.

I've decided to have a preventative double mastectomy with breast implant reconstruction as opposed to having the lumpectomy to remove the ALH in my left breast. Despite my jokes, this is NOT a decision I have taken lightly. I've spent months researching, reading articles, seeking out opinions from highly regarded cancer institutions, undergoing genetic testing, talking with a friend who both she and her sister underwent the same surgery, going on online discussion boards, speaking with friends, family, my husband, etc... I could go on and on.

Being diagnosed with ALH increases my chances of developing an invasive breast cancer in the future, about four times higher than someone without the diagnosis. If it is found before the age of 45, the risk is even higher. Lobular masses usually go undetected by screening mammogram, and the one I had in August 2013 was clear. Had it not been for the breast MRI (the one I almost blew off) ordered by the oncologist so we had a baseline to refer to, who knows when I would have felt the lump?

If I were a healthy 33-year-old woman who had the same diagnosis with the same strong family history, I wouldn't have come to this decision. I would've had the lumpectomy and continued with extensive monitoring. But the thing is, I'm not a healthy 33-year-old woman. I've had 4 open-heart surgeries and now live with severe pulmonary regurgitation. I have lupus and a number of overlapping autoimmune diseases. Besides damage to my heart, lungs, brain, and baby maker, there's no true way to determine the havoc my lupus treatments are causing, and if and how much they increase my risks for developing specific cancers (many of the medications I take come with an increased risk for Lymphoma).

Despite all of the above, I'm actually in the best place health wise that I've been in in almost 3 years. I can thank McDicky for that. My reasoning for going ahead with this surgery now is exactly that; that I am in this stable place at the moment where all my specialists feel the surgery would be well-tolerated. Why wait until my heart and other organs affected by lupus have suffered more damage? Why roll the dice and see how breast cancer treatments are after I've endured another 3-year-flare?

Tamoxifen is an anti-steroidal, anti-estrogen drug that was given to women after they received breast cancer treatments (chemotherapy, surgery and radiation) to prevent the disease from coming back. April of 1992 began the Breast Cancer Prevention Trial (BCPT), a study to determine if Tamoxifen could prevent breast cancer in women who were considered high risk, but had not yet developed the disease. My mother not only took part, but also was the Philadelphia spokesperson during her years in the trial (she's a MUCH better public speaker than me!). By 1997, of the 13,000 enrolled in the study, data showed treatment with Tamoxifen to have a 49% reduction of the development of invasive breast cancers across all age groups. Extremely significant. The final reports were released in 2005, and it was concluded that the overall benefits outweigh the overall risks.

I know what you're thinking; why don't I just take this miracle pill instead of lobbing off my breasts? What's another pill to add to my collection? If only it were as simple as the oncologist writing a prescription. Is it ever that simple? Due to my lupus and specific antibodies that come along with it, I'm at an extremely high risk for developing blood clots and having a stroke, and because of this I can't take birth control, any type of hormone therapy, or anti-estrogen medication such as Tamoxifen.

In the past, having SLE was an immediate contraindication for receiving radiation as cancer treatment because of complications that may arise. Those with lupus and other connective tissue diseases, specifically scleroderma, are at an increased risk of developing severe scaring and a late complication of subcutaneous tissue fibrosis in the exposed area. There is also evidence that the radiation can cause elevated SLE activity and lead to severe flares.

Today, there are conflicting reports if radiation therapy causes more harm than good in lupus patients. As with any disease that isn't one size fits all, some studies indicate no issues in those with lupus after receiving radiation for a cancer diagnosis, while others experienced severe flares despite being treated with a reduced dose of radiation therapy. Each patient and his or her medical team need to weigh the risks vs. benefits.

When I initially thought about undergoing a mastectomy, one of the first things I did was sit down with McDicky. I wanted to voice my thoughts and concerns, discuss my family history of breast cancer, and I needed his opinion. In my research, I found a number of articles (reputable? who knows) stating a cause-effect relationship between silicone breast implants and lupus. I figured why waste the breast surgeon's time if McDicky was going to say absolutely not?

I won't go into specifics, but McDicky had choice words regarding the supposed negative correlation between breast implants and lupus, assured me it was absolutely safe for lupus patients to get implants, and there is no scientific evidence of this so-called cause-effect relationship. He felt in my particular case, he wouldn't want me to undergo radiation therapy because of the risk of inducing a significant, organ-damaging flare. *Note- for those with mild SLE or who haven't had major organ involvement, the benefit of radiation therapy as cancer treatment could outweigh the risks. Again, everyone is different.*

He wrote a letter to the breast surgeon clearing me for surgery with specific instructions regarding my immunosuppressive medications and stress doses of steroids he expected me to receive in the operating room and 24 hours post-operatively.

Next up was seeing my cardiologist, and I wasn't sure how this would go. A mastectomy with breast implant reconstruction is typically a 6-7 hour surgery, which is a long time to be under general anesthesia for anyone, let alone someone with a heart defect.

Once again I laid it all on the table, the diagnosis (ALH), my family history, that I'm unable to take Tamoxifen or other anti-estrogen preventative medications, and the recent revelation that McDicky wouldn't want me to undergo radiation therapy in the event of a true cancer diagnosis.

I voiced my concerns from a cardiovascular standpoint; many chemotherapy drugs can cause cardio toxicity, and being that I already have cardiac issues (tetralogy of Fallot from birth and aortic regurgitation caused by lupus) if that made me more prone to developing cardio toxicities. Of course, there is no relevant data of CHDers who have gone on to develop cancer in their adult life, so she planned on speaking with the cardiac oncology specialist and would get back to me.

We both asked each other questions, and gave answers to the best of our ability. I guess my point was to go ahead and do this NOW, while my heart is in good shape to handle a 6 hour surgery, and not have to endure any treatments that could be potentially detrimental to my cardiac health. Before she gave me a straight answer, she wanted to speak with the cardiac oncologist, and I had to get an exercise stress test.

After speaking with the oncologist, the cardiologist called and informed me again, there is no true data out there because it hasn't been researched. The oncologist said that having CHD and cardiac issues from lupus doesn't make the risk of cardio toxicity any higher, but if I were to go on and develop it, it would hit me a lot harder and cause much more damage. Just how much harder, no ones truly knows. I had an A-OK stress test, and a detailed letter clearing me for surgery arrived from my cardiac team.

And finally, I had one more appointment with the doctor who played the most integral role, the oncology breast surgeon who I saw two weeks ago. I decided before I went in that if she didn't agree that going ahead with this was more beneficial than risky, I would have the lumpectomy and revisit things if any new masses popped up in the next few years. I trust her opinion, and bouncing around between hospitals trying to find a surgeon who would do the surgery seemed rather daunting.

I was a nervous wreck, wondering if she was going to tell me I was absolutely crazy for even considering this. Hey, it wouldn't be the first time someone told me I was absolutely crazy. And when she came in, I went into my usual I'm-nervous-so-I'm-going-to-spew-out-a-ton-of-verbal-diarrhea.

32 and I already have pre-cancer... shouldn't get radiation... can't take Tamoxifen... my heart... what would chemo do to my heart... not trying to be Angelina Jolie... genetic testing… strong family history... lupus... steroids... methotrexate... implants are ok... go big or go home... sorry, I'm nervous, ignore me.

When I finally looked up, I met her eyes and she said she agreed I was making the right decision.

Come again?

It is exceptionally validating when someone you trust implicitly agrees with you. In the past two years, I lost my hair, I lost my looks, and less than a year ago I lost the ability to carry a pregnancy. It took me months to come to this decision, to being ok with letting go of another part of my femininity in order to have some semblance of control over my health.

I know nothing is ever 100%, and some people reading may not agree with my decision. There's obviously no way to predict if I would ever develop breast cancer, and if the treatments would cause more harm than good. But, why take the risk if I can do something about it, and I have the blessing from the people whose opinions actually matter to me?

In my lifetime I've already heard the words "you have a complex congenital heart defect," "you have lupus," "you have cervical cancer," "you have bechet's disease," and quite a few other diagnoses along the way. I've been through more medically in 33 years than most 90-year-olds. I'm tired of hearing I have all these unwanted things. When am I going to hear that Charlie Hunnam stumbled on my blog and now wants my hand in marriage?

I can get this one thing off my chest (ok, TWO things as the case may be!), and I know it's risky, drastic, aggressive, scary, life-changing, painful, and will be at least a 4 month process (it's actually 2 separate surgeries), but it me gives the security in knowing there is at least a 90% chance of never having to hear those words, "Marla, you have breast cancer." Those are the words that have plagued my family since my grandmother got her diagnosis at age 45 and succumbed to the disease at 46, leaving my mom at age 9 and Aunt Anna at age 14 to grow up without their mother. Unfortunately, it didn't stop with my grandmother, and appears to not be slowing down in my generation.

This has been a long, drawn out, wordy post, and I tried to keep it on a serious note, rather than my usual f-bombing, douche lording self. I don't need to explain anything to anyone, but I guess I want to reiterate that this was NOT an easy decision; I assure you I didn't wake up one morning, "I think I'm gonna cut off my tits!" I did my research, I went through all the appropriate channels, I spoke to my therapist, I sought out many opinions, hell, I'm still trying to find as much relevant data as I can.

I remember the chart with pink plastic rope with instructions and pictures of how to do a breast self-exam hanging from our shower head growing up. My mom never hid how the disease has terrorized our family, and I've been giving myself breast exams before I had anything to put in a training bra. Even though I started having mammograms at age 30, I never thought this would be a decision I'd ever be making at age 33.

No, I haven't been bit by the Angelina Jolie bug and I'm not suffering from The Angelina Effect (Although, does that make Steve Brad Pitt? Princess Matzo Ball must be Zaharra and Linky Cat little Knox! Ahem…)  I'm not sure going forward with this makes me a hero, or brave, but no doubt a move to allow me to hopefully stick around in this world for a bit longer. To cause a ruckus. To MAKE A DIFFERENCE.

Sorry, I can't help myself.

I have big plans, you know.

This is the decision I've made and, insurance approval pending, the surgery will be happening in the next several weeks. Whether you agree with me or not, please respect my decision and support me along the way.

Love you all <3 <3

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Linking up with Kathy, Shanna

Wednesday, September 24, 2014

This opportunity comes once in a lifetime...

I know all of you are doing that hand bobbing waving thing Eminem does is his video. If only Mekhi Phifer were here.

Sorry for the seizure inducing pic

I don't think I shared this on my blog, but a few weeks ago I was contacted by someone who asked if I was interested in interviewing Sandra Raymond, President and CEO of the Lupus Foundation of America (LFA), and lupus expert Dr. Diane Kamen, a physician and professor at the Medical University of South Carolina.

Um. Hell yes.

The day of the interview coincided with the release of the results from the UNVEIL survey, a study conducted by the LFA and Eli Lily & Company. The UNVEIL study involved over 1,000 people with lupus and lupus caregivers, highlighting the "devastating impact lupus has on all aspects of life including family, work, finances, treatment experiences, and  overall quality of life."

Throughout the course of their disease, people with lupus experience significant symptoms such as pain, fatigue, depression, cognitive issues, and physical impact... Despite these devastating complications, lupus can be hard for others to see because there are no visible symptoms. The UNVEIL study provides the public an opportunity to see how life can be interrupted by lupus and why we need everyone's help to fight this cruel disease."     
                       -Sandra Raymond, President & CEO, Lupus Foundation of America
There are so many amazing lupus blogs on the interwebs, some of which the authors have written books, so the self-deprecating gal that I am was wondering how the hell I was chosen to interview such influential women in the lupus community.

I used the time to have both Sandra and Dr. Kamen better explain lupus and that the disease is not only life-threatening, but life-altering. Yes, those are things I often write about, but I think hearing it straight from two leading women in the industry makes what I write even more credible.

So instead of rambling on for another 12 paragraphs like I usually do, I present to you the audio to the interview. I'm guessing Preston & Steve (Philly AM radio show hosts who are totally office!) won't be knocking down my door to join their team, but it was a great experience and another awesome opportunity that I am extremely grateful for.

Thank you to Valerie for finding my blog on Google and reaching out to me, and a HUGE thank you to both Sandra Raymond and Dr. Diane Kamen for taking the time to speak with me.

For results from the UNVEIL Survey, please click here.

If you listened to the interview, you heard where I asked about the disgusting remarks Judge Judy made  regarding Lupus on TV around this time last year. Here was my blog post about her remarks. It's a good read :-)

Linking up with Kathy and Shanna

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Monday, September 22, 2014

Payin' anything to roll the dice, just one more time...

Happy Fall, my loves! This is my favorite time of the year, having the windows open and snuggling under the covers at night (Linky Cat and Matzo Ball snuggle more when it gets chilly), tank tops get traded in for hoodies and sweaters, flip flops become Uggs, and pumpkin spice EVERYTHING. Yep, I'm that girl.

This weekend was mostly spent getting my lazy on. Saturday was my first post-op venture (not counting a doctor's appointment last Wednesday), out of the house, and we went to a wine festival. We've had these tickets since the end of February, obviously months before I knew I'd be having surgery the week before. I wouldn't have gone had we not had the tickets, but I've been looking forward to this for over 6 months. In February we waited in line one cold Saturday morning to score tickets for one of the VIP tents, which were limited. Having the tent was the only way I could have gone, as it was the perfect way for me to enjoy the day, and still be lupus safe in the shade. I had a great time with my friends, loved being out of the house, and got to try pumpkin spice wine. Yep.

I've been home for two weeks already, but I'm just now getting around to writing about my Chicago trip. I went out there for the Adult Congenital Heart Association's 7th annual conference, being able to be there on both a professional and personal level. I met a number of ACHD physicians and nurses, as well as over 100+ CHD survivors. Since all this lupus nonsense started, sometimes I almost forget I'm part of this elite group. It's scary that my heart defect that required 4 heart surgeries is on the back burner compared to all the other medical issues I deal with these days.

Since I'm not currently working, I feel like my nursing skills and knowledge keep slipping further and further away from me. When I attend conferences, I try to soak up ALL THE KNOWLEDGE like a sponge. What I love about medicine is that there is ALWAYS something new to learn every single day, and I'm grateful I was able to take away a lot from this conference.

I always find it disheartening (no pun intended), the broadness of the knowledge base ACHDers have of their defect and long-term issues that may arise. My parents made certain that even at a young age I knew I had a defect and its' name, that I didn't have pulse or blood pressure in my right arm (true story), my limits in gym class/playing sports, etc... All of my cardiologists believed education was imperitive, and I remember the 1st time I met my cardiologist as an adolescent, he showed me a picture of a Tet heart and told me I needed to know my defect. They were all especially cognizant in reminding both me and my parents, that even though my heart was good for the time being, I would eventually need open-heart surgery for a pulmonary valve replacement, and that other issues could come up, such as my developing cardiac arrhythmias.

I guess I always took for granted that my parents and all my doctors made sure I had the proper education (thank you Dr. F., Dr. W., and Dr. D-squared!), and maybe I was na├»ve in thinking all pediatric cardiologists focused on education for each patient at some point (and for those CHDers with cognitive deficits, then educating the parents). While sitting in a patient breakout session, I almost fell out of the chair hearing over and over how many knew nothing about their defect, how their parents never really asked questions and sent them off to the OR just wanting the surgeon to "fix" them, and thinking they never needed lifelong cardiac monitoring.

I was shocked that a lot of them hated saying their defect was "fixed," because to them that meant cured. I always thought of my heart like a car; if something on you car breaks, you take it and get it fixed. But eventually, that part will more than likely break and need to be fixed again. The same with my heart- it was broken, and Dr. Norwood took me to the shop (err.. operating room) and fixed it. But, with wear and tear and over time, I'll need it to be fixed again. While of course the thought of knowing I'll eventually need another heart surgery makes me anxious at times, I can't imagine what it must have been like of those who truly thought they were "cured," and learned they needed a valve replacement. Scary indeed.

Most of my time was spent listening to lectures for the medical professionals, getting the ins on what's up and coming in the ACHD surgical/interventional field. Amazing stuff, I tell you. It's pretty cool knowing that me and all my "Zipper Sisters" (and brothers!) are at the forefront, and basically the guinea pigs of ACHD care and interventions. Some may not like to see it that way, but I know that how we are surgically and medically managed will play a huge role for even greater long-term survival in the future.

What other way to end the event with a bang, then by holding a Gala?! Doesn't that sound so fancy? I had never been to a Gala before, and was pretty damn excited.

 Roslyn, Christy and I are CHD survivors and cardiac ICU nurses! Talk about coming around full circle! #zippersisters

Pink is more our color, wouldn't you say?

After cocktail hour and dinner was served, all of the CHD survivors were called up to the dance floor for a group picture. I looked around at all of us and became pretty emotional (it doesn't take much, I'm a softie). Somehow, we all managed to beat some pretty terrible odds, and that a mixture of desperation from our parents, dedication from some of the most talented doctors and surgeons in the world, and a general bad ass-ness and fight to survive that's been instilled in all of us is why we were able to be there on that dance floor. 

Front and center, the only one looking at my camera. Typical.
#ZipperSisters #survivors #CHD 

And then the DJ blasted the intro to Don't Stop Believing, and we all squealed, and clapped our hands and danced together. There are a few nights that will always stand out in my adult life, and this will forever be one of them. We all had different journeys (ha ha! Journey!), some harder than others, but in those few moments, none of it mattered. I've never been so proud to be a part of something so big.

For more information on the Adult Congenital Heart Association visit