Chronic Illness  
 May 17, 2016

Luck Fupus

If you were a child of the 80’s then most you likely remember Smurfette from The Smurfs. She was blonde, sweet, cute as a button, and had trademark Smurf blue skin. I was a real-life Smurfette; blonde, sweet, cute as a button and I had blue skin.

I was born with tetralogy of Fallot, a complex congenital heart defect comprised of 4 physical abnormalities that occur together, leading to low blood oxygen saturations causing cyanosis (appearance of blue skin). Without open-heart surgery, I wouldn’t make it to my 2nd birthday.

I had my first heart surgery when I was 6 months old, and went on to have three more open-heart surgeries by the time I was 6. Life at times was pretty touch and go, but somehow I managed to get through each obstacle stronger than the last.

It blows me away when I think about what my parents went through; practically going bankrupt to pay for everything, the surgeries, sleepless nights, endless medications, cardiac arrests, carrying an oxygen tank everywhere they went with me, holding blood drives, congestive heart failure, “tet” spells, pneumonia, cardiac catheterizations. It’s enough to make anyone’s head spin.

On top of obviously being physically traumatic, it all took a toll on me emotionally. Growing up I was always much smaller than my peers, I developed much slower both physically and cognitively, I had a huge scar going down the middle of my chest, and I wasn’t allowed to participate in any type of contact sports. I desperately wanted to play soccer, and begged my mom every fall to let me play (sorry for the guilt trips, Mom). I never quite felt like I belonged.

When I hit my teens, my trips to the cardiologist were only once a year. I still had physical limitations, but I was your normal, awkward adolescent with your usual teenage problems. I graduated high school, went away to college and got to experience everything newfound freedom had to offer. There were frat parties, boyfriends, pulling all-nighters to study, and even a few bad decisions here and there. Even with my limitations, those college years were the first time I felt like I fit in.

I graduated from college with a degree in nursing, and I quickly started working at the only place I ever wanted to work- The Children’s Hospital of Philadelphia (CHOP), the hospital where I had my heart surgeries and was still a patient. It was my way of giving back to the place that saved my life too many times to count. After getting a few years of nursing experience under my belt, I began working in the Cardiac ICU at CHOP, taking care of patients born with congenital heart defects. Do what you know, right? I felt like I could relate to them on such a different level because I had once been there. I was able to connect with patients, and instill hope in parents. I came full circle.

I’d love to say that my story ends there, but unfortunately, it doesn’t. Three weeks after we returned from our St. Lucia honeymoon in March 2008, my brand-new husband rolled over one morning, opened his eyes to see his bride looking like Shrek.

No, I wasn’t green with horns, but my face was round, huge, itchy and my eyes were practically swollen shut. From that moment became a months-long game of Mystery Diagnosis where I had blood drawn, was x-rayed, scanned, and scoped in every part of my body.

In August 2008, I was finally given a diagnosis- Systemic Lupus Erythematous (SLE), lupus, an autoimmune disease that can attack ANY organ system in the body. Because UV light can increase disease activity, my doctor believed that being out in the St. Lucia sun on my honeymoon triggered this initial flare.

It was like my body was literally rejecting my marriage.

Since my official diagnosis, I’ve received diagnoses of Idiopathic Thrombocytopenia Purpura, Behçet’s disease, Sjögren’s Syndrome, Acquired Angioedema, and Raynaud’s Phenomenon, which are all considered overlapping autoimmune diseases.

The diseases and treatments have wrecked havoc on my body; I’m currently on a regimen of two chemotherapeutic agents, a biologic infusion, steroids, and a number of other medications. Lupus has damaged my heart (lupus carditis), my lungs (interstitial lung disease), and my brain. Lupus has taken my ability to have children of my own. I had to give up my beloved nursing career. Lupus has affected every single aspect of my life, has its’ own agenda and I have little control over it. Not having any control to what lupus did to my body has been the hardest part.

Due to a strong family history I had my first mammogram at age 32, and even though it came back clean, the breast oncologist suggested I start getting annual breast MRI’s. I put it off for months because I knew it would be fine, and I was already dealing with a million other labs and tests. You can imagine my surprise (and fear) when I learned a mass was detected in my left breast. After a diagnostic mammogram and ultrasound guided biopsy, I was diagnosed with a pre-cancerous lump, atypical lobular hyperplasia.

It was something else to pile onto my already full plate, and it sent me into a tailspin. I went to two highly regarded cancer institutions, did a great deal of research, spoke to doctors, friends, family, and anyone who was willing to give me insight.

I couldn’t control my getting sick with lupus, how it affected my weeks-old marriage, if I’m going to wake up one morning in kidney failure or unable to walk due to a stroke. I have no power when my pulmonary valve will start to fail due to my congenital heart defect, and I’ll need open-heart surgery. The one thing I could take control of was a way to greatly reduce my chances of ever developing breast cancer.

In December 2014, I underwent a preventative double mastectomy with reconstruction. The months that followed were hell, and I had a few set-backs due to infections. But now over a year later I can look back and have no regrets in my decision. It was empowering to have some control, to know I most likely will never hear the words “Marla you have breast cancer,” like so many women in my family have.

When I first left my job, I was angry and bitter and displaced those feelings on my family and friends (OK, mostly my husband). After some convincing from my sister, I started a blog, Luck Fupus. What started as an outlet to vent my frustrations, has turned into a forum to educate and empower patients. I didn’t think anyone besides my mom and sister read it, so when I started getting emails from people with lupus thanking me for putting myself out there, it hit me that in some small way I was making a difference. In 2014 I won the WEGO Health Hilarious Health Activist Blogger Award (I’m funny, who knew?) and that has been a platform for me to start going to health conferences across the country to share my story, empower patients and do what I can to improve the patient experience for everyone.

I will always despise lupus and all it has taken from me, but without it, I wouldn’t have the opportunities I’ve been lucky enough to have come my way. In my 34 years I’ve been dealt some pretty heavy hands, and it would be too easy to curl up in a ball and hide from the world. But knowing my blog may help just one person makes it all worth it, so I continue to fight every single day and will share my story as long there is at least one person willing to listen.

My favorite quote. I have it tattooed on my upper right arm and describes me perfectly
“My mission in life is not merely to survive, but to thrive; and to do so with some passion, some compassion, some humor and some style.”
-Maya Angelou

MarlaJan DeFusco
Twitter- @Marlajan
Periscope- @Marlajan
Instagram- @Marlajan
Pinterest- Marlajan81