The Difference an Early Diagnosis Makes
It was dusk and the men at the Afghan National Police headquarters were turning on the generator that powered the electric lights in the station. We sat in a haphazard circle that stretched across the furnitureless room, our bellies full of kabob and naan bread. My interpreter and I had spent the week working with the Afghan men who formed the police force in this village, and had earned ourselves an invitation to join them as they unwound from a day’s work. Now relaxed, we sipped green tea and played a traditional board game, similar to the air hockey I had grown up playing with my brother in Virginia. Late into the evening, the pleasant sound of overlapping conversations mixed with laughter and the thrum of Afghan pop music… it was a perfect evening.
Two days later I lay in a hospital bed on Bagram Airfield. I had collapsed the morning after returning from two weeks in the field, falling in and out of consciousness for more than four hours before I was able to belly crawl to a phone and call for help. I was physically unable to stand. The doctors were initially confused as to why a seemingly fit and healthy young woman would present with a blood oxygen level of only 58; frankly, I was confused, too.
At 29 years of age, I was in the best shape of my life. I had been in Afghanistan for seven months, participating in all day patrols — while carrying an excess of 90 pounds of gear — at least six days a week. On my days “off” I hit the gym hard, working to develop the balancing muscles needed to support my heavy kit and running six miles a day. Prior to my deployment I’d played rugby, competing at the national level, and was an avid runner. Then, in April, I was walking to the chow hall and noticed I was breathing hard. I chalked it up to the poor air quality on the base, and made a mental note to start wearing a dust mask during my runs. Two weeks later I began feeling strange while on patrol. With my heavy breathing drawing looks and teasing remarks from my buddies, I volunteered to go back to the vehicle, where I blacked out. I woke up shortly before we returned to post where a visit to the “med shed” yielded an asthma inhaler. A month later I was patrolling another village on top of a mountain. At 17,000 feet, in the most sensitive area I’d worked in, I suddenly found myself unable to breathe. To the distress of my patrol sergeant, I took off my protective body armor, shameful with the knowledge that I was putting the entire patrol at risk, but no matter how much air I took into my lungs it felt like I wasn’t breathing. We aborted the mission and I was taken back to the post hospital. This time I was given a bag of fluid and two asthma inhalers.
This last collapse occurred on June 17th, two months from the start of my symptoms. I was scared, but it now seemed that the CT scan had identified the problem: I had developed five pulmonary emboli (PE). The prognosis was not good. Within 48 hours I was in a second hospital, this time in Germany. The doctors in Germany stabilized me, releasing me after a week into the hands of a young Staff Sergeant who had been assigned to see that I returned safely to the US. Unbeknownst to me, the doctors in Germany had expressed to my superiors their concern that I might not survive the trip.
In October, just four months after my evacuation, I was cleared to resume my exercise regime. Returning to my daily runs, I found that I struggled to complete a single mile. I also noticed chest pain from the exertion, but decided that these were things I needed to work through, and that exercise could only improve my condition. After just three weeks of exercising regularly I became convinced that my body had beaten the PE, and decided to see some non-military doctors for a second opinion.
Thinking back, Dr. Svetlecic must have thought I was a crazy person. Here I was, less than four months after a major PE event, hopping up on her table as I announced that the purpose of my visit was to have her confirm my clean bill of health, and sign the paperwork clearing me to return to Afghanistan. Instead she hospitalized me. And there I sat, two days before Halloween, looking sullen as she explained something I had never even heard of: pulmonary hypertension. She brought in a cardiologist and a hematologist. The three of them showed me images of my chest and explained that, while my lungs were inflating, very little of the oxygen was reaching my bloodstream. 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 I was at immediate risk for heart failure. My parents were called and flew in for the conversation that no parent ever wants to have: I would not be returning to Afghanistan. I was a ticking time bomb. My heart could go at any moment. At best, my condition was only sustainable for another five months. I was going to die. And then, I saw my father cry.
Two days later I was released from the hospital. Quite simply, we were told, there was nothing else they could do. My parents and I returned to my apartment in a fog. That afternoon, as we sat in my kitchen not knowing what to say to one another, Dr. Svetlecic called. She had done some research, and had learned of a center at the University of California, San Diego. UCSD had pioneered and perfected a procedure called a pulmonary thromboendartectomy (PTE). As an otherwise healthy CTEPH patient I was a good candidate, but it was a long shot. Their waiting lists were long. I gave her permission to refer my case.
On January 20, 2012, I underwent a pulmonary thromboendartectomy at the Sulpizio Medical Center at UCSD, once again embarking on a long, hard road. It was painful; more painful than I ever could have imagined. Dealing with the pain from the fresh surgical site and the loss of my former life simultaneously, launched me into a period of deep depression. Bit by bit over those first three months, my outlook improved. I joined a church in my neighborhood, I made some friends, and then, an absolutely wonderful thing happened: I met someone special. Really special. Someone who could accept me, scars and all, and who, after I opened up to him about my illness, asked only one question: “if something ever happens, what symptoms do I look for, and what can I do to take the best care of you?”
Last September we were married, and I began my married life with the profound sense of being extremely blessed. Despite stretching across three countries and five U.S. states, my diagnosis had come relatively quickly, within six months, as opposed to the average of 2.8 years that most pulmonary hypertension patients go without diagnosis. I was fortunate to have been in the care of a pulmonologist who was unwilling to give up on me, even when her hospital’s administrators said there was nothing to be done. I was also fortunate to be connected to UCSD’s PTE program, which only began in 1990 — 22 years before it saved my life, and to have access to the Sulpizio Cardiovascular Center, which opened less than one year before my surgery. I was blessed by all the researchers and all the patients who had come before me, who had done the hard work that led to my happy ending. I had a second chance at life, and at a good life, and I was grateful to have it.
Then, on November 6, 2013, we got the news that my 29-year-old cousin, Christina, had died in her sleep of right heart failure. A member of my own family had died from undiagnosed CTEPH! My reaction was a myriad of emotions: I felt profound guilt for not knowing that Christina had been sick and for not making sure that everyone that I knew was aware of the symptoms. I felt overwhelmed by the extreme disparities in our outcomes. I felt grateful to be alive, and then instantly ashamed of that emotion.
Christina’s death changed my life, but not in the way you might imagine. In my grappling to understand how this could have happened, I finally began to study the disease in earnest. I learned that not only do most pulmonary hypertension patients go an average of 2.8 years before being successfully diagnosed, but that the average survivability without treatment is also 2.8 years. I learned that, in addition to a curative surgery for CTEPH, there were nine, now 12 approved therapies for pulmonary hypertension. That week I made a decision to dedicate my life to changing the patient experience for patients like Christina, and for patients with PAH and IPAH, and PH with associated conditions. Earlier this year I honored that pledge by joining the Pulmonary Hypertension Association’s Early Diagnosis Campaign — www.SometimesItsPH.org — a dedicated effort to reduce the time between symptom onset and accurate PH diagnosis. Every day I work to reduce the knowledge gaps and the barriers contributing to the high rate of missed and delayed diagnoses. I do it for Christina, I do it for myself, and each day, I am humbled by the incredible people who stand beside me.