Raele Robison was a student in a demanding PhD program when she was diagnosed with scleroderma and pulmonary hypertension. Now armed with answers about what was causing her mysterious symptoms, she is living her best life with PH.
By Raele Robison
I remember the way that the paper on the examination table kept making a crunching sound every time it rubbed against my scrubs. It was the loudest sound in the room, but the distraction was welcome. The setting and the circumstances were familiar enough, of course. I had found myself in this same spot less than a year ago — on this table, in this room, starring at this doctor, waiting for answers. However, that visit was six months ago. The doctor ordered every test he could think of and had me sent off to the lab to have 15 vials of blood drawn. The phlebotomist looked at my orders, at the tray running over with vials and then, concerningly, at me. She asked if I had eaten a snack prior to today’s visit, thinking that I could use the extra boost of energy. I assured her that I had. This marked the first time that I had lied about being more prepared for what was to come than I actually was.
The doctor called me a week later to say that,despite the effort, they hadn’t found anything. He was convinced that something was wrong, but whatever was attacking my body simply wasn’t detected in my blood tests. He told me to do my best not to worry and to follow up in one year. I hung up the phone feeling confused, but I had a life to keep living. I did my best to take his advice. I tried not to worry when I started noticing changes to my body. I became better at rationalizing pain. Those swollen feet I had? I must have been wearing the wrong shoes. The muscle cramps and aches? Must have been those new exercise classes I was trying. Running out of breath when I reached the top of a flight of stairs? Must have been a combination of being out of shape and tired. The exhaustion was the simplest one to explain away. I could sleep weekends away and still feel exhausted, but I justified it as a byproduct of my life. I was a student in a demanding PhD program after all. A little bit of sleepiness was to be expected.
There were two glaring symptoms I could not ignore, though. Invasive and seemingly always present, they were not as easily dismissed as “something else”. First, there was my skin. Growing pale in some places, scaly in others. It was also tighter. It was almost as if a large amount of my skin had disappeared and what was left was being stretched to its limit to cover my shrinking frame. The tight skin was devastatingly constricting. It made even the most basic tasks seem like daunting conquests. I felt tightness in my lungs, too. Each breath seemed to come out more labored than the last. Eventually I could barely walk 10 feet without feeling like all my oxygen had escaped me. This tightness led me to call my doctor, not a full year later, but six months earlier than expected. This is what brought me back to that examination table, staring at the doctor, waiting for answers I knew this time he’d have.
After minutes of pinching my skin, measuring my mouth and watching me struggle to lift my arms above my head- he had a diagnosis – scleroderma. This rare disease causes skin tightening and hardening. It was attacking my body. And it was finally apparent in my blood tests. I would never be the same person again after this diagnosis. Oddly enough, I didn’t cry when he told me. It just didn’t feel like it was the right thing to do in that room. Instead I listened as the doctor prepared me for what was to come. He finished off his list by again reminding me not to worry and I almost laughed at the enormity of his statement, except laughing didn’t feel like the right thing to do in that room either.
The next few weeks were a blur. A CT scan indicated that the left side of my heart was swollen because the right side of my heart was failing. More blood had to be drawn and one of those vials confirmed heart failure. My doctor, a rheumatologist, wrote me a message saying that yes, the tests had shown a failing heart and that he had put in a referral for the pulmonary hypertension clinic to see me. At the time, I naively believed that a failing heart would warrant a speedy appointment. Turned out, though, they were booked for the next three weeks. I panicked and felt desperation to stay alive like never before. I contacted the rheumatologist to plead my case and even made an emergency trip to the ER thinking this might help expedite things. The response at both ends was the same – yes, your heart is failing… no, you’re not in any immediate danger… try your best not to worry.
I knowingly walked around with this knowledge weighing on me for 25 days before my appointment at the clinic arrived. The doctor listened as I explained my story and then told me about pulmonary hypertension and how I likely developed it as a consequence of the scleroderma. He’d need to run more tests. A VQ scan and a right heart catherization were scheduled. He told me that,unfortunately, there was no cure for PH, but advancements in treatment were helping to drastically improve quality of life. He told me not to worry and, in that moment, he became the first person that made me believe things might actually be okay.
The V/Q scan was done on a Tuesday and the right heart cath was completed the next day. Truthfully, I expected the cath to be the end of the whole ordeal. It would measure the pressures in my heart and lungs, someone would write a prescription for a pill whose name I couldn’t pronounce and this whole process would be a memory I could tuck away, too distant for me to ever reach again. Except after the cath was complete, the doctor came to visit me and explained that my pressures were more severe than he had thought and I needed to be admitted to the medical ICU to begin an aggressive form of treatment. I was expecting a pill and instead I was about to be on continuous IV therapy. As he explained this new treatment, I started to cry, not caring whether it was the right thing to do in that room or not.
I spent a week in the MICU watching Law and Order: SVU on repeat, adjusting to the IV drug and two other pills recommended to treat PH and entertaining a steady stream of visitors who filled my room with love and support. On the second day of my stay, a doctor on the floor came in with medical students and asked if they could observe me. He commented to them that I looked bad in that moment, but rest assured in a week’s time I would be “running laps” with the help of IV therapy. I can report that it has been several months since I was discharged from the hospital and I have yet to run any laps, but I understood his point. I would not be the same person leaving the MICU as I had been coming in, I would be able to breathe again.
Throughout my entire diagnosis and treatment journey, the irony of my life and my situation was not lost on me. I became a patient during the same time of my life that I was in a rehabilitation sciences PhD program learning about diseases. I was trying to understand what turns someone into a patient and what can be done to help them recover once they become one. My personal research interest has always been in understanding how people compensate when their body’s resources become completely used up. I wanted to understand how people keep going when the odds are stacked against them and, more importantly, where they find the motivation to keep going. In a way, I am thankful to scleroderma and PH for helping to give me insight into these questions. I have learned there is no clear-cut answer or recipe for overcoming these types of obstacles.
In all honesty, after I was diagnosed, I almost walked away from my PhD program and from research because I figured staying in bed would protect me and would be the easiest thing to do above all else. Then I realized how selfish of me that would be. After all, my life and the lives of so many others have been spared due to continuous research into these rare diseases fueling new therapies. I felt it was my duty to get back into the lab and learn as much as I could so that I could repay the favor to patients just like me some day.
How do I live my best life with PH? I have realized that I live my best life by simply choosing each day to get out of bed and continue to live out my dream. Some days it is harder to get out of bed than others, but the days that I manage to make it out are my best days. Because on those days I realize my infinite potential. I can walk and breathe and talk and learn and adventure and experience. I suppose I had been worried that scleroderma and PH would mark the end and in some ways they have, but in the best ways. My diagnosis marked the end of me not experiencing every step and breath that this life has to offer and the end of me not knowing what my purpose in life is. But most importantly, my diagnosis marked the end of my days of worry. It turns out that the doctor’s orders were right. Life is too short and precious to be worried about what these diseases may or may not bring. Instead I lean into each day trusting in the unknown, believing that if I get out of bed to try my best, certainty that I am exactly who I am meant to be in this moment will find me.
