Faces of CTEPH
Jessica Armstrong … the Journey Continues …
I was 29 years old the first time I was told that I could never have children.
It was Halloween of 2011. Four months after the discovery of five pulmonary emboli (blood clots in my lungs) had led to my medical evacuation from Afghanistan, I continued to have worsening symptoms: I was constantly short of breath, I had chest pain, climbing stairs was so difficult that it took me 45 minutes to get to my front door most days, and I routinely showered on my knees to avoid passing out.
Despite all this, I was given a clean bill of health at the military hospital where I was being treated. When I received orders to return to Afghanistan, I decided to seek a second opinion, although I believed what I’d been told: that my symptoms were normal for someone with blood clots in their lungs. I expected to hear that I was on the mend and that I’d be completely fine in another month or two. And so, I drove to Kansas City, MO for a battery of tests, including a ventilation perfusion (V/Q) scan, a right heart catheterization (RHC), and a pulmonary angiogram. As I lay in the recovery room after my RHC, a nurse came rushing in to announce that I was being admitted immediately. There was a problem with my test results.
Shortly after, I lay in a hospital bed as a team of physicians explained something I had never even heard of: pulmonary hypertension. They showed me images of my chest and explained that, while my lungs were inflating, very little oxygen was reaching my bloodstream. My clots had increased in number from five to nine. All of them had hardened into chronic, permanent blockages and were now preventing the flow of blood and oxygen within my pulmonary artery. I had effectively lost use of 75% of one lung and 25% of the other. My heart was working overtime to try and push through the blockages, and could go at any moment. I would not be returning to Afghanistan. I was a ticking time bomb. At best, my condition was only sustainable for another five months. I would never get married. I would never have children. I was going to die.
Two days later I was released from the hospital. Quite simply, I was told, there was nothing they could do. I remember being incredibly angry that this had happened to me. I’d see young families out together and struggle to swallow my bitterness at seeing what I knew I could never have. Ironically, the news that I could never have children made my desire to have them more intense than it had ever been.
Some of you may be familiar with my story and with what happened next: a young pulmonologist on my medical team, unable to cope with giving such a poor prognosis to such a young patient, did some research. It turned out I had chronic thromboembolic pulmonary hypertension (CTEPH), which meant I had a surgical option. She’d learned of the pulmonary thromboendarterectomy (PTE) program at the University of California, San Diego and referred me for surgery. On January 20, 2012, that procedure saved my life.
After my surgery, I moved to Arizona where I met the wonderful man who would become my husband. As our relationship became more serious, I sat him down for the toughest conversation of my life: I had to tell the man I knew I wanted to marry that I could never have children. He handled the news amazingly, telling me that we’d adopt ten children if that was what I wanted. We were married a little over a year later.
But only fairy tales end with a wedding and so, four years later, there’s a lot more to tell. We eventually moved to Maryland to be closer to my family. We both took jobs at the University of Maryland, and it was at a campus event that I first learned of the PHA and this incredible community. In January of 2014, I joined PHA to manage the Early Diagnosis Campaign, inspired by the loss of my 29 year old cousin, Christina, just two months earlier from undiagnosed CTEPH.
My involvement with the PHA community (and the prodding of PHA co-founder, Judy Simpson) led me to seek a PH-treating physician here in Maryland. Over time, the outcomes from my surgery have proven to be exceptional. My resting PA pressures have returned to normal, and I do not currently require any PH-specific medications. In October of 2015, I successfully finished the Philadelphia Rock-n-Roll Half Marathon, beating my goal time by 11 minutes. Even so, I was shocked when my PH-treating physician shared some surprising news: post-PTE CTEPH patients whose PA pressures have normalized can sometimes, under a doctor’s close supervision, safely become pregnant.
Armed with this information, I elected to go through a full PH workup to see if I was one of these patients. This meant two months of testing: a RHC, a V/Q scan, pulmonary function tests, a pulmonary angiogram, repeated six minute walk tests, and multiple echocardiograms. Images and test results were duplicated and shipped across the country and to various points in Maryland. After all of that was done my medical team, including my Maryland PH-treating physician, my surgical team at UCSD, a high risk obstetrical team, and my hematologist, conferred for several weeks. Their final conclusion was yes, I could safely carry a pregnancy to term.
This past holiday season, I learned that I was pregnant. I was elated and terrified. Because of my medical history I’ve required extra-close monitoring, and I routinely see eight different doctors. Each time I see the images of our little girl on the sonography screen, I marvel about how far I’ve come and how truly blessed I am. There are so many ways this could have ended differently: I could have been mis-treated for PAH (my original diagnosis) and not made it to surgery in time (or at all). I could have had clots that were surgically inaccessible, or had another complication that made me a poor candidate for surgery. Last but not least, if it weren’t for the PHA and this community, I likely would have missed out on getting appropriate follow-up care, which would have meant that I never had this chance.
I’ve decided to share this next chapter of my CTEPH journey for a number of reasons. First, over the past several years this community has become incredibly important to me, and many of you are like family. I wanted to share my happiness with you. Second, like any good family, I knew that if I didn’t tell you that you’d learn I was pregnant and worry; I wanted to reassure you that I’m making medically-appropriate decisions about my heath. Third, my circumstances are unique. My recovery post-PTE represents what can only be described as an unusually favorable outcome to the standard course of treatment for CTEPH. I want to make sure that everyone understands that, as well as the fact that I’m doing all of this under the closest medical supervision imaginable. Because of the risks, I would never advocate for any patient in this community to become pregnant without first getting approval from their PH-treating physician.
My deepest hope is that as this field continues to advance, one day, more members of our community will be able to safely conceive and carry children, should they so choose, and ultimately receive the cure we’re all so desperate to see. Until then, I’ll continue to fight for early, accurate diagnosis of all PH patients and to advocate for more research into a cure. And I’ll take joy in knowing that when my daughter arrives this summer she’ll be incredibly blessed, because she’ll be born into a PHamily of some of the most incredible people I have ever known.