5 minute read
New Handout Addresses Contraception and Pregnancy
Family ‘Bands’ Together
To Fight Heritable PH
RHONDA CRAMER was 15 when she lost her mother to pulmonary hypertension (PH). Fifteen years later, Rhonda was diagnosed with heritable PH. After sharing her PH journey at the PHA 2014 International PH Conference and Scientific Sessions, Rhonda was inspired to raise money for the Pulmonary Hypertension Association. Now 51, Rhonda, of Saylorsburg, Pennsylvania, organizes an annual PHA fundraiser and says she is living her best life.
BY RHONDA CRAMER
At left: Rhonda Cramer holds a photo of her mom, Janet Cramer, who died in 1986 of PH. Opposite (left to right): Rhonda’s son Brandon, daughter Kara (holding granddaughter Cora), Rhonda and son Brett. My mom was very sick from the time I was born, but I didn’t understand the severity of her illness while I was growing up. Throughout my childhood, she was always short of breath with blue lips, fatigued, and often sat near a bucket because she was nauseous. Although she frequently was away to see doctors, our family never really talked about it.
I started my sophomore year of high school in 1986. Within the first month of school, my mom was rushed to the hospital and passed away. She had the flu, and her heart failed.
I learned then what her illness really was: PH. She was 44. I was told that doctors couldn’t do much for her because it was such a rare disease. There wasn’t enough research about PH at that time.
A year later, her sister also passed away from PH, although I didn’t know that until years later. My family never really discussed PH after that, and I never researched it further. I just assumed it was a rare disease and there was nothing more I could do.
SYMPTOMS DEVELOP Although it was difficult after my mom’s passing, I finished high school and went on to receive an associate degree in accounting and finance. Shortly after graduating from college, I got married and decided to start a family. I had my daughter at 26, and twin boys two years later.
My second pregnancy was very difficult. Early on I noticed I would become short of breath while drying my hair or making my bed. When I asked my doctors about it, they associated it with my pregnancy. I never suspected it could be PH.
In May 2001, we were hosting a summer picnic at our home. After my family had left, I noticed that my ankles were extremely swollen. I had been short of breath for years, but it wasn’t until I saw my swollen ankles that something clicked in my mind. I remembered my mom’s swollen ankles vividly as I sat there staring at my own. That’s when I realized that I had PH. I knew without a doubt that I’d had it all along.
SUSPICIONS CONFIRMED The next day, I made an appointment with my primary care physician who knew my mom’s history. I underwent an echocardiogram, and my cardiologist confirmed what I already knew: I had PH, just like my mom.
Although I knew deep down what the outcome of the test would be, I was still in shock. I was only 30. The next few weeks were a blur of tests. In June 2001, I met with Robyn Barst, M.D. at Columbia Presbyterian Hospital. Dr. Barst sat my family down and told us how serious PH was.
Toward the end of the conversation, she said I had about 10 years to live. That’s a lot to swallow when you are a young mom. My children were 5 and 2½ years old.
In 10 years, my daughter would be 15 — the same age as I was when I lost my mom — and my sons would be 12½. All I could think about was that my children would have to go through exactly what I went through. How would I explain that to them? How would I prepare them for that?
We set up a treatment plan, and over the next few weeks, I kept asking, “Why me? How am I going to do this?”
DETERMINED TO FIGHT I saw how hard this disease was on my mom, and I didn’t know if I was strong enough to handle it. After beginning my medication and starting to feel somewhat better, my mindset changed. I told myself there was no way I would live for only 10 more years and leave my kids.
I was determined to fight.
During my treatment, I took part in gene testing to see if my PH could be genetic. That’s when I found out I had the mutated gene BMPR2, which led to my diagnosis of heritable PH. That’s also when I found out that my aunt as well as my mom suffered from PH. In all three of our cases, our symptoms didn’t surface until our second pregnancies.
After four years of treatment at Columbia, I was transferred to the University of Pennsylvania. At that point, I could barely walk from the car to my house without getting out of breath.
When I met my new care team, Paul Forfia, M.D. and Fran Rogers, M.S.N, CRNP, I received a new treatment plan. They told me if I didn’t start a new medication soon, I would die. After I started the medication, my symptoms began to improve. Just like that, I realized I was walking up two flights of stairs in my apartment without stopping to catch my breath.
FINDING PURPOSE In 2014, Fran asked me to speak at the PHA International PH Conference and Scientific Sessions in Indianapolis to talk about my PH journey. I had never done public speaking, but without hesitation I said yes. It was the best decision I ever made.
Through the years of dealing with my disease, I always felt like it was my burden to carry. I didn’t talk about my illness because I didn’t want anyone to
