PH Professional Network: From Patient to Provider: How My Diagnosis Makes Me a Better Nurse

As a nurse on an advanced lung disease and pulmonary transplant floor, I always brace myself when I have a patient with pulmonary hypertension (PH). Although they are my favorite patients, it always hits a little too close to home. One day, I was assigned a patient that had severe PH and was supposed to go home on hospice. As I attempted to get my tasks completed, we started talking about how she felt. Morbid jokes were sprinkled throughout our conversation, almost as an acceptance of our disease without a definitive cure. When I shared my diagnosis of idiopathic pulmonary arterial hypertension (IPAH), she looks at me and chuckles as she says “Well, I hope your outcome is better than mine.” While our conversation continued, my PH specialist (who happened to be on PH service that week) walked into her room with a plan that did not necessarily include going home on hospice. After he left, she said, “I can’t believe I found my two,” as she heard one or two in every million are diagnosed with IPAH. Throughout that day, our bond grew immensely. At the end of my shift, she asked, “Where were you all of this time since my diagnosis?” Although our paths were very different, sharing my story gave her a bit of hope.

I was a healthy teenager prior to my diagnosis. My focus was school, horseback riding, and violin. However, slowly, I was losing the ability to ride my horse and walk to class in my small high school. Something in my body just told me to stop and take a break; I later I realized this was severe dyspnea on exertion and presyncope. One day, my biology teacher said people with heart murmurs have difficulty walking to their cars. This statement made me realize that something other than being lazy and subjectively overweight may be going on. That night, I told my dad we should schedule an appointment with my pediatrician to help figure out why I was so tired all the time.

The appointment was uneventful. With a shrug of the shoulders, my pediatrician recommended that I get more sleep and exercise. However, my CBC showed polycythemia, which led to a CT scan of my abdomen to check my kidneys. A radiologist found cardiomegaly, and I was sent to a cardiologist’s office the next day. One of the first things he said to me was, “I think I know what you have, and if I am right, you will need to go to UCSF [University of California, San Francisco] immediately.” After an echocardiogram with many concerned looks from both the echo tech and my cardiologist, I heard the words “pulmonary hypertension” for the first time. I had the choice of going via ambulance or driving with my parents. The stubborn student in me demanded that I go to my English presentation first, to which my cardiologist said ,“Well, considering you walked in here, I can’t see why you wouldn’t be able to go.”

After getting through the rough afternoon traffic from Sacramento to San Francisco, I walked into the unit that would become my home for the next 9 days. I was taken for my first right heart catheterization, where I gained the title of “the worst pressures” these experienced PH providers had ever seen. My pressures were suprasystemic, and I had poor right ventricular function, which I later learned stratified me to a very poor prognosis. I was started on triple therapy, including subcutaneous Remodulin, all of which I am still on today. A few days later, my PH specialist, Jeff Fineman, came in and said, “there’s a PH 5K in a month, and you are going to walk it.” I, along with everyone else in the room, looked at him very confused. How could I walk a 5K when I could barely walk 238 meters on my 6-minute walk test? Jeff had a lot of hope during a time where it was difficult for me to comprehend what my future may entail. I was able to walk that 5K, with my PH and Cardiac Intensive Care Unit team behind me.

After my diagnosis, I had one main goal – to live as normal of a life as possible. I begged my parents to let me move away from home for college. I had learned how to manage my medications and doctors’ appointments and wanted to experience a true freshman year. I attended Cal Poly SLO, and I truly thrived. During my first year, I started working as a medical assistant for a family practice and achieved the President’s List every quarter. That summer, I started shadowing my PH team’s nurse practitioners, who inspired me to pursue nursing. I transferred to UCLA’s School of Nursing, where I began my journey to be a pediatric Cardiac Intensive Care Unit nurse. It was a journey full of ups and downs. I had people tell me they were not sure that I was cut out to be a nurse because of their preconceived notions of people with PAH. However, I proved them all wrong as I graduated at the top of my class in 2020.

I moved to Pittsburgh, Pennsylvania, to pursue a job as a nurse in an advanced lung disease and pulmonary transplant floor. I had to go through a bit of an adjustment, learning how to take care of myself while taking care of very ill patients. This included taking diuretics and dealing with subcutaneous Remodulin site changes while working a physically demanding job. I quickly learned that I must take care of myself first in order to provide the best care for my patients. Working with this population has been incredibly rewarding, but also very difficult to see patients severely ill after their life-saving lung transplants, as I know it is likely a part of my future.

My patients with PH always have a special place in my heart. Despite the large age differences, we have a shared experience that brings us closer together. Although I have shed many tears for these patients, I am truly thankful for the days that I get to spend with them. When I care for adult PH patients, I have a difficult time not seeing myself in their shoes one day, struggling for every breath and having to make some of the most difficult decisions of their life. A few months after our initial meeting, I saw my would be hospice patient was a patient on my unit again. When I went to see her, the first thing she said was, “Aren’t you surprised I’m not dead yet?” She passed away a few nights later, but our relationship taught me how to find the humor in every situation. Although these relationships with patients have been incredibly difficult because of the often harsh reality of PAH, it makes each grueling day at work worth it knowing that I made a difference.

As I transition to my new job in the pediatric Cardiac Intensive Care Unit, I hope that I will show children and their families some hope in their diagnosis. I want them to know that they can pursue their dreams despite many of the challenges that their diagnosis may throw at them. Without PAH, I would not be the nurse I am today, and for that, I am forever thankful for my diagnosis and for being a part of this amazing PH community.