It’s no big secret that I have Pulmonary Hypertension. I want to share with you my story. I also want to share some links to some sites. Some of the sites were created for myself to bring my situation out into the open. Some of the site pages were created and published by an amazing group called Pulmonary Hypertension Association. These people have done more for those of us suffering with this disease than anyone I know. In some cases, they’ve even done more than some of the doctors we’ve seen when first seeking a diagnosis. I have a strong feeling this blog is going to be a lot longer than I initially intended for it to be. If you’re willing to stick with it until the end though, I appreciate it! Do you have your snack and beverage of choice all ready? Ok, then! Here we go:
I was born with a hole in my heart. Some refer to this as a heart murmur, or thrill. The technical term is Patent Ductus Arteriosus or PDA. A thrill as best as I understand it is the sound a doctor hears through the stethoscope when the blood being pumped from the left side of the heart into the right side of the heart, backflows into the left side again. In other words (because I realize as I typed that it sounded a little confusing), when your heart beats it pumps blood from left to right, the same as you read and write. With someone that has a PDA though, you often get some of the blood flowing back into the left side of the heart which causes a definite sound or “thrill” that doctors can hear during any exam. Apparently my “thrill” is so loud that it’s rare. Imagine being 21, having a young not-at-all-ugly male intern walk into your hospital room, stethoscope in hand, saying he came to look for your thrill! This of course happened before anyone explained to me what a “thrill” technically was! No wonder he pulled back so fast. My face must’ve had quite the horrified look on it. Luckily I have a good sense of humor and once he explained to me what it was, I told him I hoped it was good as he expected it to be!! Nothing like a little light-hearted flirting to ease an awkward situation! Anyway, I digress a little… Every doctor I’ve ever seen knew about the PDA. For whatever reason, unbeknownst to me of course, none of them ever really looked into it beyond the general physical exams every child gets. No testing. No bloodwork. Nothing. Just listen to my heart and lungs, and send me on my way. My pediatrician would ask about it and I guess the people in charge of my care at the time didn’t realize what was really going on. I would get out of breath faster than other kids, I recovered very quickly though. I wasn’t an outdoorsy, running around kind of kid. I was happy with my crayons, Barbie dolls, and Leggos (which I got from a close friend). I didn’t run anywhere, ever. I didn’t ride bikes. That just wasn’t me. I figured because I barely ate enough to keep a bird alive, and wasn’t overly active that I was just out of shape. I knew I had a heart condition and my gym teacher(s) was always content to let me “sit this one out” for a minute or two when I needed to. No big deal. As it turns out it was a big deal…a very big deal. When I was 11 my pediatrician finally decided it was time to find out what the PDA was all about. He sent me to Children’s Hospital in Pittsburgh for a left/right heart catherization.
I was admitted to Childrens, and the test was to be early the next morning. I didn’t sleep all night. I was scared both of what was going to be done during the test (as I’d never had anything other than a booster shot before this), and what they may find during the test. To top it off they were wheeling me out of my hospital room to prepare me for the cath and none of my family were there yet. I was half way out of my hospital room when I saw the one person who meant everything in the world to me walking quickly down the hall! He had made it after all. He grabbed my hand, told me I’d be alright and he’d see me when I was all done. They wheeled me into the heart lab and I was relieved to find out they were going to let me keep my favorite stuffed toy (a grey Pound Puppy that I of course named Fred) with me during the cath. After starting an IV, and giving me 3 or 4 shots of local numbing stuff (not sure now exactly what it was), I felt the doctor making the two small incisions near my groin area where my thigh met the bottom of my hip. I still have the scars today! It didn’t hurt. I just felt pressure. I could see my insides on the monitor that the doc was using as he fed the tiny camera up through my vein. Talk about being more than slightly freaked out!! During the test they injected dye into the tube. Also during the test a couple different times, they put a clear plastic box over my head for a few minutes. They wanted to purposely make it difficult for me to breathe so they could see how it effected my heart and the bloodflow. It’s definitely the weirdest sensation I’ve ever felt to have a small tube being fed up through my vein all the way to my heart. Yes most of the time you’re supposed to be “knocked out” for this test or at least in that “twilight” state of not quite awake and not quite asleep. I wasn’t. They didn’t knock me out because they were afraid of complications from the anesthesia with not knowing yet exactly what was going on with the PDA. So, the doctor does the cath and finishes up and stitches the incisions closed. I then sat in my hospital room with my childhood Hero for a few hours. They had sandbags sitting on my leg to make sure bloodclots didn’t form and to make sure also that the bleeding didn’t start up again. During this time, they (I’m assuming the doctor performing the test and the lab techs that read it afterwards) talked to my GrandpaDad outside in the hallway with my hospital door closed. I never have known exactly what was said between them. I was later told that it was too risky to close the PDA (this was in 1986) and that it should eventually close on its own and I should be fine. Scary test, great outcome, over and done with. I was getting dressed to go home and I started to get sick to my stomach. That’s how I found out I was allergic to the contrast dye they used. They use the dye to see exactly where the blood is flowing as the heart pumps. I was sick to my stomach and back in the bed for another overnight stay. I got to finally go home the next morning and I had two small badges of honor to take with me as a memento. Unfortunately you couldn’t see them unless I was wearing a bathing suit, I didn’t care though!
I went through the next 9 years as any normal child/teenager does. I went to school, didn’t get the grades I could’ve, I did alright though. I graduated in 1992 at the age of 17. Immediately after graduating, I got my first job at a local amusement park. Everything remained the same. I started at Westmoreland County Community College in the fall of 1992. I was working towards an Associates Degree in Small Business Management. I was going to college full time, and I was working at a local clothing store full time as well. In 1995 I had about 8 college classes left to take before I earned my degree. I had changed jobs and was working at the local WalMart store in Greensburg. I started that job in September 1995. Little did I know that by early January of the following year, I’d never work anywhere for a long, long time and would have to quit school.
It was December 1995, just before Christmas Day and I woke up with a horrible cold. I couldn’t breathe and couldn’t stop coughing. I figured with being in school and working full time, I was exposed to any number of colds, sinus infections, etc. I didn’t think anything of it. I woke up one day I think it was December 20th and I couldn’t walk from the second floor of my house down to the main floor. I had just woken up and was going downstairs to do my usual morning stuff and get some breakfast. I was still really sick with whatever it was I had. I got to the bottom of a flight of 8 steps and I had to sit down on the bottom step. I was literally so out of breath that my lips and fingernails were blue. I was huffing and puffing like a freight train and couldn’t catch my breath. I was dizzy and even sitting still I couldn’t get everything around me to stop spinning. I managed to get through the morning and call my family doc for an appointment. I saw him and needless to say he wasn’t so quick to dismiss what was going on. He did some pulmonary function tests to check for asthma. They were negative. He, much like myself, figured it was the chest cold I had and he put me on antibiotics for it. I was on break from school since the spring semester didn’t start until March. I was trying to force my way through my work schedule, and ended up going home almost as soon as my shift started every single day. I finished my antibiotics. I still had the worst time breathing, although the rest of the cold symptoms were now gone. I went back to the doctors on January 5th, 1996. He gave me a note that put me off work and school until we could figure out what was going on and get it fixed. I had some bloodwork done for him, and he recommended that I go see a Cardiologist to have the PDA checked again.
I saw Dr. Chandra Polam in his office around January 18, 1996. He did an EKG in his office and clearly saw something he didn’t like. He also listened to my heart, obviously hearing the loud thrill that was there as well. On January 24th, 1996 he performed a left/right heart catherization at St. Francis Medical Center. This time I had just turned 21 a couple weeks prior to this. So much for going out and celebrating that milestone! There were several med students behind a screen watching the test as it was performed. I’m a great learning patient. Interns will see things with me that they may never see again in their entire medical career. Later that evening, as I was laying in bed talking to a very kind nurse, with sandbags on my leg wondering how I was going to go to the bathroom (because I couldn’t bend my leg and couldn’t walk on it so it was quite a conundrum), Dr. Polam came in and told my family member and I that the right side of my heart was enlarged, and that he thought I needed to have a heart transplant.
On January 26, 1996, I was transferred by ambulance to Presby Hospital in the Oakland section of Pittsburgh. Between the dates of January 26th and February 2nd, 1996 I was poked, prodded, questioned, examined, and had more blood drawn out of me than I even knew I had. At 8:14 AM on February 2, 1996 a woman walked into my hospital room to tell me I was finally going home. She said that she was a Transplant Coordinator (her official job title) and also told me that I needed to have a double-lung transplant. Apparently after having a meeting with several specialists, doctors, lab techs, and hospital officials, I was indeed put on the organ donor waiting list for just that. I was also set up with oxygen, which I am on 24/7/365 no matter what I’m doing. My world turned upside down in that moment and Groundhog Day would never ever mean the same thing to me. The official diagnoses were, the PDA (which we already knew about), Eisenmenger’s Syndrome, and Primary Pulmonary Hypertension or PPH. PPH is basically high blood pressure in the arteries of the lungs. There I was, recently turned 21. I couldn’t work. I couldn’t finish the 4 classes I had left to get my degree. I had student loans and no income. I couldn’t even get off the couch in the living room. I lived on that couch for a full year. I couldn’t bathe myself. I couldn’t walk 10 feet without having to stop and catch my breath. It was horribly scary. I didn’t know what was going to happen to me. If I did go out anywhere, it was in a wheelchair. I hated every single second of it.
In February 1997, I finally had enough of that life. I woke up one morning and decided that if I was on my to Heaven, I wasn’t going to die in a wheelchair or laying on that couch. I got up several times a day and forced myself to walk no matter how much it hurt (my lungs would burn I’d get so out of breath), and no matter how tired I got. I walked a little further every few days. In March 1997 I walked down the flight of steps to get to the garage. I got in the car all by myself. I drove to my good friend’s house (about 10 minutes away) all by myself. I walked into her house all by myself without even having my wheelchair with me. That was the end of my couch living. I’ve been unstoppable ever since.
- Do I still get out of breath? Absolutely.
- Are there still limitations to what I can do? You bet!
- Am I still in need of that double-lung transplant 19+ years later? Yes!
- Do I let this incurable disease control me? Not if I can help it.
- Did I ever finish college and get that Associates Degree? Yes. They offered online classes and I did wonderfully in them!
- Can I still do a lot of the things I enjoy doing? Yes, if there’s a will there’s a way. It’s all about adaptation.
- Can I run? No way. Do I want to run? No so it doesn’t matter!
- Can I ride roller coasters? No. I hate them so that doesn’t matter either!
- Can I plant flowers and vegetables? Technically no. I’ve done it though. I put on latex gloves, and a longer pair of gardening gloves over them, with a long-sleeved t-shirt and just worked slowly and within my limitations.
You see, it’s all about how you look at things. You make your life what it is. If it’s not what you want it to be, change it. If you can’t change it completely, adapt it to make it more than what it currently is. Don’t sit around with a “woe is me” attitude expecting everyone to feel sorry for you. They will for a short time, then they’ll get sick of hearing it. Who can blame them? I get sick of dealing with it myself, so I can only imagine what it would be like if I were to focus on all the negatives. If I was asked to offer two words of advice to any other PH patients? BE BRAVE!
There are several pages on the PHAssociation website that have great information about Pulmonary Hypertension. It’s an excellent learning source. They have started a campaign called PHAware! They are trying to get the word out about PH so they can raise money to do research and hopefully some day find a cure. Right now the PH patients’ only hope are medicinal therapies and transplantation. The average life expectancy is only 2.8 years without treatment. What is the life expectancy with treatment? Nobody can give a definitive answer about that one. That’s not nearly good enough! I’m honored to join forces with other PHighters and work on getting that life expectancy a lot higher! We’re worth having a chance at life just like any other person.
They also have a YouTube channel where you can watch videos and learn more about Pulmonary Hypertension.
You can also visit their Facebook page, and follow them on Twitter using the hashtag #PHAware.
That being said, the cost of the medicines, insurance copays, and medical supplies are astronomical. This is where I ask you kindly to visit my patient page on my fundraiser website. There is a nonprofit organization called helpHOPElive that has started a fund in my honor. All money donated is tax-deductible. I cannot use that money for anything that is not transplant related. They get the money dontated in my name and place it in my personal fund under their watchful eye. When I receive a medical bill that’s transplant-related I turn it into them and they pay it using the money in my fund.
No matter whether you donate to my fund or not, I appreciate your sticking with me through my PHight and hope that you’ll be willing to share information about this disease. I have a Facebook page dedicated to PH and my personal PHight. We need to raise awareness. Nobody knows about it and it’s so often misdiagnosed. It only costs a little bit of time to retweet, share on Facebook, or if you’re so inclined to share this blog. I greatly appreciate everyone who is now, was since the beginning, and will join me in the future. It’s all of you that help keep me going! Well you and some pretty awesome doctors!! Thank you, Dr. Dennis McNamara! You and Diane ROCK!!
I will still do general blogs filled with my “Nonsensical Meanderings”. I will now add to those blogs about my personal PHight with PPH, occasionally sharing facts and statistics with you so that you can better understand what I and all the other PHighters out there face! Again, thanks for being in my corner! I appreciate you all! Don’t forget…. Laugh A Lot, Live Well, and Breathe Easy!!