Hi, my name is Jody and I am 31 years old and was diagnosed with Pulmonary Hypertension on August 17, 2010. I also have a son who is 10 years old and is my whole life. I am a single mother. My life has changed in ways that I never thought possible. I can remember everything that I have been though since that dreadful day. Like many people I had no idea what PH was or how severe it could be.
It was a normal day in August and I worked the midnight shift, I was dropping my son off at my Mom’s when I just didn't feel right. All of a sudden I felt like I couldn't breathe and was disoriented. My Step Dad took me to the hospital right away. Upon getting there, they did the normal check-in things, when they took my blood pressure it was 170/115. That was a red flag right there. Well, I of course was admitted and the next day my PCP came into see me and explained that they were going to run a bunch of tests including an echo. The echo came back showing the right side of my heart was enlarged. Next step on to see the cardiologist, I was still in the hospital at this time. After he came and talked with me he then referred me to a man at Allegheny General Hospital known as Dr. Benza. After my week stay at Sewickly, I was discharged with some new medicine and was told to see Dr. Benza as soon as possible.
Well, within that week I was at his office, which is where the real journey had just begun. After doing extensive tests including the famous right heart Cath, the verdict was in fact Pulmonary Hypertension. So, Dr. Benz and his staff went over everything with me and started me on my first medicine Revatio, along with other medicine for swelling and things. I remember going home that day thinking well, it's going to be fine, I'm going to be fine. I'll just take the medicine and I'll start to feel better. Of course it didn't go that way. As months passed I didn't really notice any difference on the Revatio, I was still swelling and short of breath, I was still working at this time.
Well, January 2011 is here and I'm still only on the one medicine and making it through day by day, until......well one day one of our cats get out the front door, who is an indoor cat and has never been out. Well my instinct was to run after her, so I did, well thankfully my son was home and our maintenance man was in our hallway because I ended up passing out. An ambulance came and took me to AGH, and the passing out was attributed to the running. So Dr. Benza said "no more sprints down the hall" lol lol. I then had another heart Cath and started another medicine called Letaris in Feb of 2011. I also had to quit my job and go on disability due to swelling and other issues. I made it through most of the summer without any real issues, I had a few hospital stays due to fluid overload but, I was taking it day by day.
Now to just recently, in December of 2011 I had another heart Cath and my numbers were sliding on the hill again. Dr. Benza said that it was time to get aggressive with it before I slide over the hill and said I was going to start a medicine called IV Remodulin. On December 28th I was admitted to AGH and began the IV Remodulin. It was the worst week of my life! I had every side effect imaginable from this. I had terrible headaches, vomiting, jaw pain, leg pain, constant bowel movements. I wanted to quit by day 2, but they wouldn't let me lol lol.
I make it through that week and came home with a pick line in my right arm and my IV Remodulin! It was hard at first, trying to shower, sleep, get dressed and just getting used to having that line. Mixing the medicine was another challenge but, I had wonderful support from a nurse named Lynn! Now the time had come to put in the Hickman so it was scheduled for January. What a day! I went in at 7:30 am that day to special procedures and they were prepping me when they took my temperature in the ear it was reading 102. I said I felt fine, I didn't feel sick or warm etc..... I asked for it to be taken orally and they said that they didn't have those down there; well needless to say they removed my pick line put another one in my left arm and admitted me. Upon arrival to the 5th floor, they check my vitals and my temperature orally was 97.7. So, I was apologized to and sent home, there was no infection and they had made a mistake.
The following week the Hickman is rescheduled and it is inserted on the right side of my chest. I go home and 6 days later I'm sick as ever. I have chills, and a fever. I go straight to the ER and I'm admitted, I have an infection. So I spend another week in the hospital and they put a pick line back in and take the Hickman out. I go home on IV antibiotics for an additional two weeks. I just received the second Hickman on the left side on Tuesday Feb 28th. I am also on oxygen now, since I had the infection. I am on 4 liters 24 hours a day.
So, that is my story in a nutshell. I am also on medication to help with the days that I just want to cry. I know that once I reach the goal for the Remodulin the payout will be all worth it. My road hasn't been an easy road but, I keep fighting and praying every day. I have the best doctors and a great support system and faith!! I never would have imagined my life the way it is now but, you carry on, you fight, and you never lose hope that someday there will be a cure. Thank you for letting me tell my story and God Bless.

Jody, PH Fighter - March 3, 2012

MY LIFE BEFORE AND AFTER PAH DIAGNOSIS - Deanna Modzelewski

For as long as I can remember, probably since high school, I have always hated stairs and walking up hills. I was always out of breath by the time I got to the top; I thought I was just out of shape. There were other signs too. I played basketball in high school but always felt like I could never really keep up with the team. I went on to college. Walking across campus I would get out of breath especially walking up the small hills to the dining hall, etc.; again I thought I was out of shape. My friends never seemed out of breath or tired. After college, I had an apartment on the second floor. By the time I walked from my car up the stairs to my apartment, I was always so short of breath. But again, didn’t think anything of it. I kept telling myself I needed to exercise (but never did). That was in the late 80’s and early 90’s. Was this a sign of things to come? Did I have PH back then? I don’t know and probably never will, but it sure makes me wonder.
Fast forward to 1997. I had returned to school to become a physical therapist assistant. I needed a physical to start my clinical rotation. My blood work came back abnormal which then prompted months of doctor’s appts and test. They were not sure what was wrong with me. Finally I was sent to see a liver specialist at the University of Pittsburgh Medical Center. They determined I had a blood clot in the Portal Vein causing portal hypertension. I also had liver disease (nodular regenerative hyperplasia). They determined the blood clot had been there a very long time. Possibly from birth; possibly from birth control pills. And there was nothing they could do except monitor me and list me for transplant in the future. Fortunately over the next several years, I remained stable.
Life was good. I was working as a physical therapist assistant, a job that I really enjoyed but was very physically demanding. I worked with many patients teaching them how to walk again. And working with respiratory patients teaching them how to conserve energy and breathe with activity. Something that I would find myself doing a couple years later…how ironic. While I was working, I found myself getting short of breath a lot but kept telling myself, it was a very physically demanding job and I was just out of shape, or so I thought.
Then in March 2003 things started to go downhill. I had traveled to Atlanta, GA to see my brother and his family. After that my ankles started swelling up, my blood pressure was skyrocketing, and I was exhausted all the time. But I kept working. After several dr appts locally, I was again referred to Pittsburgh. My pulmonary valve was leaking and I may need surgery to fix it. Finally on May 1, 2003, I had my first Right Heart Cath. The doc looked at me and said you have pulmonary arterial hypertension. The cause was my portal hypertension and liver disease. I was told: You won’t be having surgery, you will be fine without fixing the valve and you will no longer qualify for a liver transplant either. But I did need treated for the pulmonary hypertension and they would start with a drug called Remodulin. They never explained the severity of my disease. I found that out on my own months later. And now I am kind of glad they didn’t tell me. However, I would not be able to continue with my current job. So all I was worried about was how I would work and pay my bills. I never realized the mortality risk involved until I read the letter my doc had written for me to give to my employer. It said the disease carried a poor prognosis. To this day, I still don’t really believe it. At least for me.
Shortly thereafter, I started on Subcutaneous Remodulin. It was not at all what I had pictured in my mind. I thought it would be like an injection like a diabetic would take insulin. WRONG! Oh well, I decided if this was what I had to do to feel better, then that was what I was going to do. I had heard about the site pain and fortunately for me, I had very little to no pain at all. One of the few and very fortunate. That did not last forever though. Over time I eventually developed site pain. Some days it was so bad I felt like quitting and just ripping the catheter out of my body. But my determination prevailed and I learned how to manage the pain by using it on my lower back and upper buttocks for sites. I am back to being pain free most of the time and if I do happen to have pain, it is tolerable.
Once I stopped working and started on the Remodulin, I started to see an improvement although it took a few months. And I had to learn my “new normal.” I lived by myself so I had to learn how to take care of myself and manage my disease and do all of my household chores etc. I learned what I could do easily and what was more difficult. I learned not to push myself and not try to get everything done at once. I learned how to pace myself and take breaks so I would not use up all my energy doing one task.
A few months went by and I started feeling a lot better. I was approved for disability but I wanted to do more. I still wanted to work so I decided to return to school. I took a few classes at a local college and got a certificate in medical coding. I took classes part-time and was able to manage schoolwork along with maintaining my apt. I was happy about that. The biggest challenge was climbing the stairs and lugging books around the campus. But I would just get there early and take my time walking to class. And I was still able to manage my disease and I was very thankful for that. Then in September of 2005 I was fortunate enough to be hired on at a local hospital as a medical coder. I loved it. Unfortunately, despite it being a sedentary job, full-time was a bit much for me. After about a year, I dropped back to part time and was offered the opportunity to work from home. I continue to work in that capacity to this day and I am very thankful for that. It allows me the opportunity to work and feel like a productive member of society, help maintain a household, AND manage my health at the same time.
Throughout my entire life including my diagnosis and the start of my treatment for PH, I had remained a single woman, living by myself. My family was local and they were very supportive but I was still in search for the love of my life. I wondered what would happen now since I had PH and this little pump attached to my body. But in June 2005, I met my now husband Bill. Right away I told him everything about my diagnosis, treatment, not being able to have kids, etc. I wanted to know what he felt about it early on, because if he had a problem with any of it, I was not going to waste my time. To my pleasant surprise, he was fine with all of that!! We ended up getting engaged and then married in September of 2006. Since then we have enjoyed a wonderful life together. And as a caregiver, I couldn’t ask for a more supportive husband.
Something else I have done since being diagnosed with PH is travel. My honeymoon was a trip to Florida and a Caribbean cruise. We have taken numerous trips to Florida and just recently drove across the country to Arizona and the Grand Canyon. Traveling with PH is possible. You just need to be prepared.
While my PH and liver disease both remain stable, I am now faced with a very large pulmonary artery aneurysm. While there is a risk of rupture (and I would die within minutes if that were to happen), repairing this, in my case, would be even riskier based on the fact that I am doing so well functionally. Therefore, any type of surgery is on hold for now. At times I question this decision in my mind, but in my heart I know that it is the right one, at least for now.
So to all of you newbies just diagnosed (and oldies too) the moral of my story is this: Don’t let being diagnosed with PAH stop you from living your life and doing the things that you want to do with your life. Know what your individual limitations are and how to manage your disease. Be happy and thankful to still be living on this earth. Remember that none of us (with or without PH) know when we will be called home. You only get one chance at life, so make it count!!
Love and hugs to all of you!! 3-29-2011

I was asked to write this by Merle Reeseman and I put it off for several weeks. I finally sat down and wrote my thoughts and feelings and I am glad I did. Thanks Merle

Alan has not been as fortunate. He has been diagnosed with pulmonary arterial hypertension which is so much pressure with his lungs that it makes it hard for the blood to pass through properly. Alan has not been able to work for the past two years, needs oxygen 24/7 and in April, started to bleed from his lungs. Coils and plugs had to be put in to stop the arteries from bleeding further. We know this is temporary and doctors have confirmed Al needs a double lung transplant to survive. He is only 47 years old and looks great on the outside. However, I watch the man I love become weaker each day, and harder for him to walk without taking constant breaks. I listen to his labored breathing while he sleeps, knowing all I can do is pray and thank the Lord for just one more day.

The costs for a double lung transplant is costly -- far beyond what my insurance will pay for and Alan will also need immunosuppressent drugs that he will need to take for the rest of his life to keep him from rejecting the new lungs. We don't know what we can do.

Alan is making candles to help pay for his lung transplant ~ part of the donation will go to PHA for Research and Awareness

Mary Anne Adams - October, 2008