Charitable Giving – Pulmonary Hypertension Association

Beth’s PAH Story

I was diagnosed with Pulmonary Arterial Hypertension (PAH) shortly after having my son, Jensen, in late 2012.  At the time, we lived in Salt Lake City, UT.  My husband, Chris, and I were very surprised, to say the least.  I was 30 at the time and had never had any real health concerns. Growing up, I was diagnosed with asthma; I never used the inhalers because they did not do anything.

I would always tell my doctors that, but there was never any further action. I was able to do normal activities, however, I was always the kid in gym class that did not run the mile, but rather jogged and walked it.

I was diagnosed with Pulmonary Arterial Hypertension (PAH) shortly after having my son, Jensen, in late 2012.  At the time, we lived in Salt Lake City, UT.  My husband, Chris, and I were very surprised, to say the least.  I was 30 at the time and had never had any real health concerns. Growing up, I was diagnosed with asthma; I never used the inhalers because they did not do anything. I would always tell my doctors that, but there was never any further action. I was able to do normal activities, however, I was always the kid in gym class that did not run the mile, but rather jogged and walked it.

During my pregnancy, things got much worse. Prior to pregnancy, I worked out regularly and I had planned to continue that as long as possible. By week 6 of my pregnancy I was no longer comfortable exercising even at a low level, so I stopped. By week 16, I started taking the elevator at work to go up one floor to my desk. I told my OB about the shortness of breath at each appointment and she said it was normal for a pregnant woman to experience it.

I made it through to deliver my son at full term. He ended up being much smaller than expected. He was 6lb 12 oz; we were expecting an 8 or 9 lb baby based on our sizes and family history. We found out later that he was small because of my low oxygen. So far there are no other negative consequences of my illness on him.

While I was in the hospital for his birth, my oxygen levels were low and I was still very short of breath. The nurses were concerned, but I convinced them to let me leave. A couple of days later I ended up in the ER after a short walk up a hill nearly made me faint. As soon as I got to the ER, the doctors checked my oxygen saturation and found that it was in the low 70s after only minimal exertion to walk to the room; they were very concerned.

They ran some tests and were not able to determine the problem. They wanted to admit me for more testing, but I did not want to stay. I had a 5 day old baby that I had to go home with. They were able to convince me to stay by explaining that I might not live very long if I did not stay. I was in the hospital for 5 days and was the subject of a lot of tests including a right heart cath. It showed that I had severe PAH (I was functional class IV), but they did not know the cause. They let me go home on oxygen 24/7 with plans to see the local specialists soon.

The local cardiologist and pulmonologist saw me several more times over the following 6 six weeks.  At first, the cardiologist thought that the PAH was a side effect of my pregnancy and that the pressures would go down shortly after my pregnancy.  He did another right heart cath just a couple of weeks after the first one to see if the pressures had gone down.  To his dismay, the pressures had not gotten any lower.  Next, he thought that the high pressures could be the result of a hole in my heart known as an ASD, Atrial Septal Defect.  To be sure, he asked me to undergo a TEE, transesophageal echo, which would let him see my heart from the back side.  This procedure was done in December of 2012.  It did reveal an ASD that the cardiologist thought could be closed via a device inserted during a cath procedure.  He went on to tell me that this closure would fix my PAH.  We quickly decided to move forward with the closure and we had to do yet another right heart cath just 1 week after the last one.  During the procedure, he inflated a balloon to test the fix before inserting the device.  When he did that, the result was not as expected, and he aborted the closure.  At that point, he decided my case was beyond his expertise and he referred me to the specialists in Salt Lake City.

I was referred to the Intermountain Medical Center Pulmonary Arterial Hypertension clinic with Dr. Elliott and Dr. Brown. They ran their own set of tests and determined that my PAH was caused by a more severe congenital heart defect. I have a leaky Mitral valve and atrioventricular septal defect (AVSD) which is several holes in my heart that allow blood to flow from the high pressure left side to the low pressure right side. Over time, the extra blood has increased the pressure on the right side of my heart and cause the PAH.  Dr. Elliott and his team wanted to do open heart surgery to correct the defects, but my pressures were too high to safely do the surgery.

In March 2013, I was put on Remodulin in an attempt to hit the problem hard on the first try so that surgery could happen sooner. At a checkup in November 2013, the Remodulin had not lowered my pressures, so the doctors added Adcirca. The next heart cath a year or so later showed that my pressures did not move significantly.  My doctors increased my Remodulin again and added Opsumit to try to get improvement.  The medicines helped me feel much better.  I was able to work full time and take care of my little boy. He is full of energy and I had to keep up.

In March 2013, I was put on Remodulin in an attempt to hit the problem hard on the first try so that surgery could happen sooner. At a checkup in November 2013, the Remodulin had not lowered my pressures, so the doctors added Adcirca. The next heart cath a year or so later showed that my pressures did not move significantly.  My doctors increased my Remodulin again and added Opsumit to try to get improvement.  The medicines helped me feel much better.  I was able to work full time and take care of my little boy. He is full of energy and I had to keep up.

In August 2013, Chris and I decided to move to Portland, OR.  We had owned land in the area for a couple of years that we were planting with grape vines.  Chris had always dreamed of owning a vineyard and we were working to make that a reality.  The move was also very good for me because of the elevation difference – Portland is basically sea level whereas Salt Lake City is close to 5,000 ft.  PAH patients do not do well with higher elevations.  We were so glad to finally be able to relocate.

Over the next 3 years I continued to take the 3 medications for PAH.  My functional status improved; however, my elevated pressures did not significantly change.  I was able to work full time and watch my baby grow into an intelligent, happy little boy. 

Chris and I have also moved forward toward his dream by expanding the vineyard to nearly 10 acres and building a winery on the property.  In 2015, we produced our first vintage under the name Chris James Cellars.  We bottled about 200 cases the first year with plans to increase that in the years to come.

In May 2016 I was finally approved for heart surgery to repair my heart defects.  I underwent open heart surgery at Oregon Health and Science University.  The surgery went well and I was released about 10 days later.  I had a minor setback when I was affected by Post Cardiac Surgery Syndrome (aka Dresslers Syndrome).  I have since recovered and am being careful so that I do not relapse.  The surgery was a success which is best illustrated by my improved oxygen saturation with exertion.  In spite of the corrections, I will always have PAH and be on the medications.

My son, Jensen, is 11  years old now and I am 41.  Even though I have PAH, I have plans to stick around for a long time.  Prior to surgery, I was not sure I would even be around to see Jensen start school; now I expect to attend his graduation (still a few years off!).  I want to help plan his wedding.  I want to be here to meet my grandchildren.  Fortunately, with the current medications and your help to fund research, my future plans are more and more likely to happen.