Patient story

James Grea

Solomon's Return - My Boogey Man: Pulmonary Hypertension 

James GreaMy name is James Grea. I write stories for children with a magical fountain pen named Solomon J. Inkwell. I spend a great deal of my time writing about Boogey Men and things that go bump in the night. I love spooky tales. They are my favorite. My monsters are harmless and even quite fun because they are imaginary. But there are times when monsters can be real, and sometimes they can even live inside of you.

I arrived at outpatient registration at 5:30 AM with my sister and my best friend. I was surprisingly calm. I swear to you, I don’t know why. Maybe I had convinced myself that I was going to receive good news. The mind has an odd way of masking the unimaginable, especially when it is standing right in front of you. We sat in the lobby waiting to be called. My mind began to wander as I scrutinized the room taking mental snapshots of the other people who were waiting with us. Some were tired; some were sad; many were anxious. My sister patted my leg and smiled at me, snapping me into reality. My uneasiness wasn’t apparent to those who didn’t know me, but she knew I was shivering on the inside. Soon they called my name and my chest tightened at the sound. I took a deep breath and followed the nurse who showed me to my curtain-walled preparation area. She handed me a gown and a bag where I would stash my belongings while my test was being administered. Then, she left me to myself.

Two older gentlemen were on either side of me hidden behind their own curtain walls. They were apparently having the same procedure as I that morning. I tried my best not to listen to the questions the nurses were asking them. “When did you have your first heart attack?” “Have you had any issues with your pacemaker?” “How many medications are you are taking?” “Do you have a living will?” I crawled into my bed, placed my hands over my ears, and began softly humming to myself. Listening to the historical accounting of pacemakers, bypasses, and coronary blockages was making my stomach turn. I wished the nurses would go ahead and give me the sedative I had been promised, something that would calm my nerves. I stared at the ceiling tiles trying to think of other things, things I would do after I received the good news, movies I would see, food I would eat.

Right heart catheterization—it is a fairly simple and relatively painless procedure where a measuring device is inserted into the right femoral artery of the leg. It travels to the heart where pressures are measured and dyes are injected. It’s a common test that can locate blockages in the arteries. It’s also the only exact method of diagnosing Pulmonary Arterial Hypertension (PAH). You’ve probably not heard of this disease—most haven’t. It isn’t like coronary artery disease, cancer, or emphysema, which is recognizable to the population. PAH is a rare disorder that causes uncontrolled high blood pressure in the pulmonary system. The pressure of the pulmonary arteries is normally much lower than what typical blood pressure tends to be. But in PAH, the pulmonary pressure becomes so great that it begins to damage the arteries inside the lungs, making them stiff and thick in a process known as fibrosis. This directly affects the right side of the heart that is responsible for pushing circulation to the lungs. The heart begins to compensate, and, as a result, the patient begins to experience right-side heart enlargement. This can lead to what is known as cor pulmonale, or failure of the right ventricle of the heart because it cannot keep up with the strain. There are some disorders that can cause elevated pulmonary pressure, of course. Valvular diseases/disorders, severe COPD, or pulmonary embolisms (blood clots of the lungs) can all cause elevated pressure. However, there are instances where the pressures increase for unknown reasons. This is known as Idiopathic Pulmonary Arterial Hypertension (IPAH). There are also instances where the disease is inherited. This genetic form is called Familial Pulmonary Arterial Hypertension (FPAH). And there is no cure.

I come from a fairly large family consisting of eight children in total, of which I am the youngest. By the time I was twenty-one years old, I had lost my oldest brother (diagnosed age twenty-four, passed age twenty-seven), my nephew, who was ten months younger than myself (diagnosed age eleven, passed age twelve), and my mother (diagnosed age sixty, passed age sixty-one)—all of them victims of PAH. You could say that the disease has stalked me and my family all of our lives. We all spend a great deal of time praying it doesn’t catch us. My new adventure had begun with a routine EKG that had shown an abnormality, which led to further testing. It was then they eventually found mild enlargement on the right side of my heart. I heard the word “right” and immediately knew what was happening to me. And it was those tests that had led me to my curtain-walled nightmare—completely terrified and staring at a stark-white ceiling. On the positive side, the heart cath would immediately provide results. In just a matter of minutes I would know the truth. And that terrified me, because a small piece of me already knew the truth. Suddenly, a small, elderly woman entered through the soft white walls of my area. Written on her badge was the name Sister Mary Elizabeth. She had a kind, sweet face and she spoke very softly to me. Placing a small hand on my shoulder, she said, “Hello, my name is Sister Mary Elizabeth. Are you having a heart cath today?” “Yes,” I said, smiling at her through my obvious fear. She laid her hand on mine and said, “Would you like me to pray for you?” “Yes, I would like that very much,” I said. I could feel the slight sting of tears in my tired, dry eyes. I can’t tell you what she prayed. As a matter of fact, I can’t tell you a word she uttered. I just kept my eyes closed, hoping that God was hearing her words among the countless prayers that were being said throughout the hospital at that morning. I thanked her, and with a smile she nodded and left. I tried my best to dry my eyes and compose myself. I continued to speak to God. I begged him to help me, or at least give me the strength to face whatever it was that I was about to face.

Soon after, the orderly arrived. He wheeled me into the catheterization laboratory and began to prep for the procedure. I welcomed the sedative and tried to focus on its feeling, allowing myself to be lulled by its effect. I began to think of my mother, my brother, and my nephew. I missed them, especially my mother. I began to speak to them in my mind. Then, there was a single sharp stick at my hip, and I felt nothing more. Through my wooziness, I could see the large monitor above me. On the screen was my beating heart and I watched as the thin cath line traveled into its chambers. I took a deep breath and tried to relax myself. Moments later it was over. The rather abrupt cardiologist I had been working with had no experience with PAH. So, he thought nothing when he rounded the table and said nonchalantly, “Well, it looks like Pulmonary Arterial Hypertension.” Stunned, I asked, “How high was the pressure?” “110 over 44,” he said. I sank into tears. From experience, I knew that number translated into a mean pulmonary artery pressure (mPAP) of 66 mmHg. The resting mPAP should never go above 25 mmHg. Yes, the Boogey Man under my bed had found me at last.

The doctor quickly disappeared once I became emotional. To me, hearing PAH was like hearing end-stage cancer. After all, all of my family members had died within two to three years after being diagnosed. They wheeled me into the hall where my sister and friend awaited me. The doctor was standing with them hurriedly trying to get the basics delivered and get away from the emotional scene as quickly as possible. I just looked at my sister and said, “Well, this is it, then.” They wheeled me back into the recovery room where I had to remain until my surgical site set. And I couldn’t stop sobbing. I enjoyed life so, and now I felt it was over—over at only thirty-eight years old—and right before I made it on Oprah, too. My sister, who was also in hysterics, called my family doctor and made an emergency appointment for later that afternoon to get something for my anxiety. I was referred to a PAH specialist in my area, which was fortunate because in my family’s history there had never been a specialist in our area. We always had to travel to Vanderbilt University Hospital in Nashville. The bad news was I wouldn't see this specialist for a month; a month of knowing what was happening to me, but not quite knowing. And I continued to cry—I continued to cry for three solid days.

To me, my life was over. I wondered how much time I had left. How long would it be before I couldn’t walk to the bathroom without passing out? How long would it be before I wouldn’t be able to talk in full sentences without becoming short of breath? What did I want my funeral to be like? How much life insurance did I have? Did I want my service held where my mother’s had been? I tried to calm myself, trying to be thankful that I wasn’t presently showing severe symptoms. I hadn’t been dizzy, faint, or extremely breathless. I had been getting winded when climbing flights of stairs, but I thought that maybe I was out of shape. I would never have thought I had PAH. It is such a rare disease that to believe that so many people from one family ended up with it was inconceivable. I felt I had completely lost my identity. I was no longer James Grea. I no longer wrote scary stories. I was now a zombie who wandered around my house doing nothing but sob. Finally, I decided to reach out to the Pulmonary Hypertension Association. I began reading everything I could on their site, trying to find any sign of hope, any sign of someone who had lived with this disease longer than two or three years. I obtained their Envelope of Hope, which contained a wealth of information. I went one step further and called their support line where I spoke with some great people who were living with PAH. Surprisingly, I learned that in today’s world I have great chances, far more chances than others in my family had.

In the past, there was nothing at all that could treat PAH. It was an undeniably fatal disease. But today I learned it is very different. Today there are nearly ten different medications and therapies that are used to treat symptoms and provide improved functionality to patients. Then, I began to do things I hadn’t done in a while. I began to talk more with God. I began to see the value in little things in my life that I had taken for granted, like walking up my driveway, being around the ones I love, laughing, going to work every day, and being able to simply live. I began to realize that not one of us is guaranteed to see tomorrow. At any moment we could receive that test result that could drastically alter our lives forever, or even get hit by a moose. I began to realize that at thirty-eight years old, I had nearly lived longer than my nephew and my brother combined. And I’ve never said, “Why me?” Why not me? What makes me better than anyone else? Absolutely nothing. So, I began to have hope. For the only other alternative is to lose hope, and that is unacceptable.

So, why am I telling you this? I’m really not sure. Maybe I want you to come along with me. Maybe I want us to be like old friends, sitting in a room, drinking coffee, and talking about the frailty of life. Maybe I just want you to listen. I’m not certain. I only know that I am moved to tell you these things because a writer must write. It could be possible that you, too, are afraid—for yourself, or someone you love—and maybe it will make you feel just a tad less lonely to know that somewhere out there I am here, being afraid along with you.

I got to see my specialist today, who is a wonderful doctor. I felt quite blessed to be placed with him. I will begin my treatment on Remodulin SC soon. I am going to be trained on how to use the pump that administers the medication next week. Who knows what tomorrow holds. Miracles happen every day, and my miracle could be right around the corner. Ironically, being diagnosed with this disease could very well prove to be my miracle. So, I will look toward today and value what I have in my present world, my present health, and my present life. For today, I am alive—we are alive. And what could be better than that? And yes, I am slowing finding my identity again… My name is James Grea. I write stories for children with a magical fountain pen named Solomon J. Inkwell. And I just happen to have Pulmonary Arterial Hypertension.

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