Here to Bring you Hope: Nikki Richard
My story starts like most patients living with pulmonary arterial hypertension (PAH). One day I became short of breath without any real cause. I remember thinking I must have been coming down with the flu because I became very fatigued after taking my laundry out of the dryer and carrying it to the next room. I had to sit down and catch my breath—I was gasping for air. I wasn’t sure if this issue was being caused by my scleroderma, so I made an appointment to see my rheumatologist the next day, and was prescribed steroids. I felt fine for a while, but eventually, I tapered down from the steroids. The shortness of breath was back, along with the fatigue and a new symptom; chest pain.
Eventually, I was referred to a pulmonologist. After being told I had asthma, I was immediately prescribed an inhaler to try—that didn’t help. After a year of trying different inhalers and asthma treatments, my symptoms became worse, and I finally decided to get a second opinion. The new pulmonologist set up some tests, including an echocardiogram (ECHO) and a pulmonary function test (PFT). By this time, my symptoms were worse, and the second pulmonologist said I had to see a more specialized doctor in Boston by the name of Harrison “Hap” Farber. Within the same week, I found myself going into the city for a right heart catheterization to meet this “Top Doctor.”
When I awoke from the procedure, I was told that I would not be going home that night. My numbers from the heart cath were so severe that I immediately had to be put on veletri (the newest form of epoprostenol), a continuous IV infusion to treat pulmonary arterial hypertension. I couldn’t believe what I was hearing—I was in shock. I had so many questions. I remember being overwhelmed with strong feelings of fear and sadness. The emotions took over me, and it felt like my whole life had just been turned upside down. Suddenly I would be living the rest of my life with a medical pump (Cadd Legacy) attached to me requiring a central line to be placed and maintained for the duration of treatment.
As if it couldn’t get worse, not only do you have to mix the medication vials yourself with diluent, but you also have to keep it refrigerated and change the cassette every 24 hours. VELETRI, along with other supplies, has to be ordered through a specialty pharmacy each week, and this makes the whole process very draining as a patient.
Here I am, at age 33, and I should be in the prime of my life, not having my dreams and aspirations shattered. You work so hard to reach your goals, and another serious illness like pulmonary arterial hypertension comes into the picture and changes everything. I’m not going to lie, the first year after the diagnosis was incredibly hard. I hate to use the word “depressed” because I’ve always been a very positive and upbeat person, but to some degree, I probably was. At the time, I was working full-time, so my short-term goal that I had made for myself was to get back to work as quickly as possible. I was looking for that sense of normalcy again, or what I thought was my “normal.” I’m also lucky to have such a loving, supportive family that helped me with everything that living with the disease entails.
Seven years later, and now I’m 40. Along the way, I hit a point where I realized I was going to make the best of the situation and try to live my best life. My goals have also changed—I’m not going to be afraid to talk about my illness anymore. I will do everything I can to raise awareness and help make a difference for patients living with the disease. Now there are more treatment options available, and more innovative solutions are on the way. Due to amazing organizations like Team PHenomenal Hope, who fund medical research for improved treatments, there may one day be a cure for those who suffer. Until then, I’ll continue to be P-H-FABULOUS (PHabulous)!
I am thankful for veletri, as it allows me to live, but now I must face my next journey with PAH. My lung transplant journey. My name is Nikki, and I am proud to be a member of the PH community, and I’m here to bring you hope.
Nikki is a recipient of the Team PHenomenal Hope Unmet Needs Patient Impact Fund. Learn more about how this fund supports PH patients at teamph.org/unmetneeds.