My entire life, I had been relatively healthy. Like anyone, I had had health troubles here and there, like bouts of unexplained fatigue, or Reynaud’s syndrome (where my fingers get cold and blue if I’m chilly) – but nothing to write home about. I was a 30-year-old avid runner, had just had a very healthy pregnancy and was in good physical shape. This diagnosis was a shock.
What exactly is PAPVR and ASD?
Let’s start by looking at a normally functioning heart. In a normal heart, the right side of your heart receives oxygen-poor blood (blue) from your body: it arrives in your right atrium, goes to your right ventricle and is pumped into your lungs through the pulmonary artery. In your lungs, your blood receives oxygen and oxygen-rich blood (red) returns through your 4 pulmonary veins to your left atrium, travels to your left ventricle and is pumped through the aorta to your body.
With PAPVR or PAPVC (Partial Anomalous Pulmonary Venous Return or Connection), one or more of your pulmonary veins are bringing oxygen-rich blood from the lungs to the right side of the heart (the right atrium) instead of the left side (the left atrium). This means that the right side of your heart has to work overtime to re-pump blood that it already sent off to the lungs. Yikes! On top of that, the majority of people who are born with PAPVR also have what’s known as an ASD or Atrial Septal Defect. This is a hole in the wall between the right and left atrium. This means that oxygen-rich blood from your left atrium flows back into your right atrium through that hole, giving the right side of your heart even more blood to re-pump back to your lungs. That’s a lot of work for the right side of your heart: 2.8 times more work in my case, to be exact! That explained why my heart was getting larger and larger over time. It wasn’t a question of whether it would fail but when.
1. Give thanks to your heart and to those who helped diagnose you
Working in the field of permaculture, I wondered what lessons I could draw from permaculture to apply them to my situation. Then the obvious hit me: Beauty is in the eye of the beholder. This proverb accompanies the very first principle of permaculture. How I decide to view this moment in my life is completely up to me.
I could be angry. Angry that cardiologists had missed this when I was hospitalized at 5-years-old and got an echocardiogram, angry that cardiologists had again missed this when I developed a heart murmur at age 17 and checked it out with an echocardiogram, and missed it yet again when I went in for another echocardiogram check-up at age 23. I could be angry that I had to deal with all of this now that I had a brand new baby girl who needed her mom more than ever.
But I wasn’t. I was grateful. I was so grateful that my nurse from the Women’s Birth and Wellness Center had noticed that my heart murmur sounded louder and longer post-partum. I was so grateful that my cardiologist this time knew how to read my echocardiogram and noticed that the right side of my heart was very enlarged. I was so grateful that I just so happened to be living in the United States at the time, where the absolute best care was available for me to treat this rare condition. I was so grateful that this happened right after Dakota’s graduation, right when I’d started to wean my daughter off breast milk, and right before our move to Switzerland.
I realized that this wasn’t a matter of “crap, I might die in the OR”, but “this diagnosis just saved my life”!
It took me about a week for the news to settle in, for major freak-outs to occur, and for me to eventually have this mental shift.
After calling my nurse and cardiologist to thank them for helping diagnose me, I knew my heart needed some loving too. It bothered me to hear friends and family say that my heart was “defective” or that we were going to “kick PAPVR’s butt!!”. I didn’t quite see things in the same light. I wasn’t trying to fight my heart and show her who’s boss. I wasn’t angry at her for being so unique. If anything, I felt bad that I’d gone so long without healing her and lightening the load for her. After all, she’d given me 30 spectacular healthy years on this planet and a perfectly healthy baby without a hiccup! So I did let her know, in my own way, how grateful I was for the gift of a healthy life she’d given me so far.
2. Figure out how your situation can help others
When I first found out my condition, my cardiologist didn’t know too much about it, because while he was smart enough to diagnose me, it wasn’t his specialty. And remember, I had freshly landed in Michigan, with no go-to cardiologist! When you find out you need open-heart surgery, but have virtually no details about the success rate, the procedure, where to do it, etc.: you turn to Google. I didn’t come across much information at all about adults being diagnosed with PAPVR and ASD. Turns out, kids get diagnosed and treated young. I was the odd ball out.
But then I stumbled upon Jersey Girl Talk’s blog post: My Husband’s Journey: Open-Heart Surgery for Adult PAPVR and ASD. John, the same exact age as me, had had the same exact surgery I was going to need earlier that year. I couldn’t believe my luck that I’d found this person online! I sent his wife Adrienne from the blog a message and within less than 24 hours I had John on the phone, talking to me about PAPVR and what I could expect.
Needless to say, speaking to him was the single most comforting thing I could have possibly hoped for. I hadn’t had a chance to speak with a specialist yet, so I was terrified. He reassured me that my surgery, while rare, had a very high success rate and that I’d be back on my feet in no time. He even gave me tips as to hospitals to look into that specialize in adult congenital heart defects.
The relief I felt when I hung up the phone was overwhelming. I knew right then and there that my case was too rare to keep to myself. I needed to share my story to help anyone else who might be going through this. So I started filming PAPVR videos to document my story. It helped me process the unreal and difficult news, and I knew the information I was learning would be helpful to some other person or family going through this.*
(*If you are an adult preparing to have PAPVR surgery, ask your surgeon and anesthesiologist about alternatives to tranexamic acid. Other antifibrinolytic drugs are less likely to cause post-operative seizures in adults like they did in my case. Note that seizures are still rare with this drug and it’s my understanding that they occur in 1% of adult cases.)
3. Ask for help and accept help
When I got my diagnosis, I had just arrived in Michigan, where I knew no one except for Dakota’s family. While they were of course very supportive from the start, I did feel isolated not having close friends to confide in. I knew right away that I wouldn’t be able to go through this alone. While I normally keep to myself about health issues or trying times, I let everyone around me know right away exactly what was going on.
What did it change? Everything. Suddenly, it didn’t feel like I was alone carrying the weight. I felt so loved and supported. It really did feel like everyone’s love was holding me up.
It made a world of difference to ask for help, but also to accept it. My dear friend Meg from the Eco-Institute asked me whether she could organize a community vigil event the day of the surgery. How beautiful and thoughtful, yes! My mother, father and sister wanted to come be by my side during the surgery. I want you there more than ever, yes! Several friends asked whether they could send me energy medicine. Yes! Can I give you a phone call? Yes! Can we pray for you? Yes! Can I organize a special spa day for you? Yes, yes, yes! What a difference it makes to accept help. It’s sometimes seen as weak to ask for help from others, but I felt that asking for what I needed and accepting help is exactly what made me strong.
The last days before surgery, I wondered how in the world it was possible that I was so calm, collected, and able to enjoy moments of beauty and laughter that were given to me. I realized it was because I had a community of friends far and wide who were rooting for me every step of the way.
4. Do your due diligence, but know when it’s time to let go and trust
PAPVR is a rare congenital heart condition, so it’s important to find an excellent surgical team that is specialized in congenital heart defects. I spent a lot of time researching children’s hospitals, interviewing surgeons, and sending my files left and right. After a while, the numbers and success rates and stats all become a big blur.
I ended up choosing Mott Children’s Hospital because not only were they the closest to us, but they were ranked #3 in the nation for pediatric cardiology and heart surgery. (Check out U.S. News Health Rankings for detailed ranking information for hospitals you are considering.) I met the head of the pediatric cardiology department and felt confident with him. I went with my gut feeling and booked the surgery date.
Dakota and I were packing up the car to leave to the hospital for pre-op day when I received a phone call. My surgeon had to go on an unexpected leave of absence and wouldn’t be available in the foreseeable future. Surgery could go on as planned, but it wouldn’t be with him. My heart sunk. I had put all my trust in this man. First of all, was he ok? He was such a brilliant man; I hoped nothing bad had happened to him. Secondly, would I be ok? How could I choose another surgeon just days before my surgery? It took me about 5 minutes for the news to sink in. And then I made a decision. If there was anything the past 2 months had taught me, it was that nothing was really in my control. I had come this far. Weeks of mental preparation. Days away from surgery. I wasn’t about to back down. All I could do was trust.
I remembered the list of intentions I had written to my daughter Luna prior to her birth: “My Intentions as Your Mommy”. The last one was that I intended to find hope, light, serenity, and trust, no matter the situation. So that’s exactly what I decided to do, and I went ahead and booked surgery with the talented Dr. Romano.
5. Don’t spend too long entertaining dark thoughts: distraction, distraction, distraction!
The first week after my diagnosis was the toughest. I didn’t know everything about the details of the surgery, but I did know doctors would stop my heart and put me on bypass while they repaired it. Dark thoughts flooded my mind. I pictured the worst. It’s unfathomable to go through such a surgery, so of course, it’s a free-for-all for your brain.
I ran – oh yes I did! I was still cleared to run, so baby I ran! I beat Dakota at ping-pong matches. I got a total makeover and captured timeless photos of my pre-surgery self. I bought a secondhand pineapple shirt from Plato’s Closet and invented a pineapple shirt song and jig that drove Dakota absolutely bananas. I learned the lyrics to the Irish song Ho’ Ro’ the rattlin’ bog and drove Dakota even crazier and laughed until it hurt. I even made a live video for my vigil group minutes before going in for surgery joking that I was about to bring them live to the operating room! Humor and distraction got me to the very last moment when I finally had to hug my family and baby goodbye.
Thank you to each and every person who helped me through this journey. You helped me more than you know. If you or your loved one has been diagnosed with PAPVR, please comment below or reach out to me if I can be of any help at all.
Much love to all of you from a healed heart,
Want support for your heart diagnosis?
Join the PAPVR & ASD SUPPORT GROUP on Facebook.
Permaculture Principles applied*:
*Bear with me on this one, I got creative here! 🙂
Beauty is in the eye of the beholder. The lens with which you decide to look at your situation is completely up to you. Is the glass half empty or half full?
Supportive friends are there for you, ready to jump in when you’re in a tough spot. Accept their positive energy, their thoughts, their hope, their prayers, and bring that beautiful energy with you to the hospital. It made a world of difference for me to feel their love lifting me up on surgery day.
The negative feedback that my heart was giving me from working 3x as hard for 30 years was slow to emerge. But I accepted her feedback and planned accordingly for the future to ensure my heart would continue to function well.
The heart repair itself is brilliantly designed from patterns to details. Instead of closing the ASD hole and then cutting and moving the anomalous veins to the left atrium, Dr. Romano did what is called a baffle. She simply rerouted the anomalous veins so that they drained into the left atrium through the ASD. She fed two birds with one scone. Genius.