LIVING WITH PEDIATRIC PULMONARY HYPERTENSION
BY JESSA ANN SCOTT
From: “Ask the Expert” section, Columbia Medical Journal
How can I possibly explain to somebody; health care worker, doctor or anybody who isn’t sick and dying – what the actual daily fight for life and the feeling of my organs deteriorating inside of me is like. At 22, I wasn’t expecting this- regardless that it was mentioned as a possibility when I was first diagnosed at 16. Unless you are enduring or have survived a life threatening illness, it’s truly impossible to fathom the oppressive, pervasive burden, pain and fears associated with likely or imminent death. That being said, this is my best attempt.
There are two popular methods to attempt to explain what life is like for those suffering with a serious illness such as Pulmonary Hypertension. One is the comparative “spoon theory” in which spoons represent energy and a fixed number of spoons represent the total amount of energy available for any and all activities. For the chronically-ill PH sufferer, there is no “digging deeper”, once you are out of spoons the machinery just quits. For comparison, a healthy individual might need one spoon to breathe for the day, whereas I require five. A certain amount of spoons are consumed for each activity such as showering, dressing, and if it’s a good day, walking a few feet. It is an attempt to show how cautious we must be every day, carefully sacrificing and planning daily tasks. There is also the straw method in which a healthy individuals nose is pinched closed and forced to breathe through a straw. This depicts how little oxygenation PH patients really get, and how difficult it is just to preform the most basic and natural task of life.
However no method can adequately explain the heavy thoughts we are forced to always carry. The anxious, ever-present panic of pain and suffocation for carrying out any activity. The need to constantly question; “will there be stairs?”, “how far do I have to walk?”, “where can I stash my arsenal of back-up medication?”. There is no way to try to articulate the misery implicated to the body that we experience every day. Simply stating crippling back and chest pain, body aches, headaches, nausea, vomiting, and diarrhea is a truthful summery of symptoms, but it still doesn’t quite allow for a mirrored reflection of the life PH patients lead. In lieu of all of these trying struggles, for me the hardest component was the emotional strain. The loneliness from being plucked out of school and my social life and put in hospitals on a regular basis. The fear from feeling a rattling in my chest cavity and wondering how much blood will be coughed up this time. The embarrassment from being stared at constantly in public and knowing that I am no longer just a twenty two year old girl, but a wiry mess of oxygen and intravenous medication tubing. The terrifying realization that I am battling for my life- and regardless of my positivity and hope, that I might lose.
After enduring endless hospital visits and doctors appointments my doctor informed me that I had exhausted every treatment available (orals; Revatio, Letaris, Adcirca, inhaled Ventavis, subcutaneous Remodilin, the “gold standard” Flolan, and even coiling to try to stop my hemoptisys) and would eventually die treating symptoms. My only option left was an evasive operation of double lung transplants and open-heart surgery. Since I had secondary pulmonary hypertension due to an ASD, I needed to have my heart repaired in addition to transplants. This news was like a double edge sword; on one hand I have the opportunity to get away from all this pain and suffering yet I would have to face my scariest obstacle yet and there were no grantees on how well or long I would live afterwards. The work-up to get listed was not unusual for me, since I was well accustomed to frequenting hospitals and being poked and prodded. When I was listed, it only took a little over two months to receive my transplants and it was truly in the nick of time. I never got to experience getting “the call” because I was already in the hospital for my biggest case of hemoptysis yet- five cups of blood. Although I was unexpectedly placed on ECMO, which made the situation a little more challenging, my overall result was incredible. I had transformed from a dying patient to a perfectly healthy individual in a mere nine hours.
From the very first moments of consciousness, I felt different. I felt fifty percent amazing and the other half aching and hoping for more morphine since I was sore and bruised and still full of tubes. My skin (save my incision sites) was clear and beautiful, no longer marred by red splotchy irritation from Flolan and I no longer had my battery powered pump companion to fret over. The most amazing thing of all however, was that I could breathe. The past couple years before my operation, I constantly felt like I was running and gasping and if I could just take in a deep breathe I would be okay. Yet I was never running at all (in fact that word was practically erased from my vocabulary), and I never could obtain that deep breath. Gasping was my form of breathing- similar to a fish out of water, literally suffocating yet surrounded by air. Now, just a few months shy of my one year anniversary, I can run and jump and race up flights of stairs. For most, this just seems like normal and mundane yet I know it to be a miracle. Life is so precious and incredible to me now that I can have the ability to live it and not be in perpetual survival mode. I take heed to the advice to “stop and smell the flowers” and actually do so, and marvel in the small wonders we usually look past every day. One could never expect for someone else to truly understand the depth of the struggle and despair of a terminal illness. In fact, now living as a healthy and virtually symptom free individual, I am beginning to forget precisely how the pain felt, how much oxygen I could muster, and how I ever put a smile on and endured it- which may be for the best anyhow. One thing I am certain I will never forget though is that life is a miracle, and to be thankful and enjoy each and every second of it .