When I First Began to Process the Seriousness of My IPF Diagnosis

In November 2012, I began to experience shortness of breath. My throat and esophagus also felt somewhat cold, like I was breathing and exhaling cold air even when I was in the house. I didn’t think anything of it and didn’t attribute these symptoms to an illness.

On a trip to Cancún, Mexico, several months later, I began to have short coughing episodes and trouble with exertion while walking on the beach, snorkeling, and doing other activities. Upon our return home, I still felt I was having symptoms related to a bad cold, but I gathered up enough energy to go to the Cleveland Indians home opener. I had enough after the second inning and called my wife to pick me up and take me to the emergency room. All of my numbers were off, my chest X-ray looked bad, my blood counts were off, and my heart didn’t appear to be pumping properly.

I was admitted and subsequently diagnosed with lipoid pneumonia and interstitial lung disease (ILD). The ILD diagnosis was not specific, but doctors said that some inconsistencies required further observation and testing. During this time, a pulmonologist took over my care. One of the last procedures was a bronchoscopy. Mine was done caveman style: no anesthesia, just an Ativan (lorazepam) and lidocaine spray in my throat. My wife could hear me in the waiting room, making gasping sounds and struggling not to choke. The bronchoscopy came back inconclusive.

I spent the next 18 months in the assigned doctor’s care. I would see her every 3-4 months, and each visit consisted of a pulmonary function test and an X-ray. Although I was getting slightly worse, I felt I was going to be all right. When this disaster began, I was 44 and in relatively good shape. I was a little on the heavy side but could still work outside for 12 hours spreading dirt and mulch, and completing my other activities around the house. Steps, however, were not for me. I avoided them like the plague — at work, at home, and anywhere else my journey would take me. As long as I was walking on flat ground, I would usually not get short of breath.

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I was referred to the Cleveland Clinic’s Respiratory Institute and assigned Dr. Leslie Tolle. Dr. Tolle completed a thorough first appointment and began to eliminate all possibilities under the ILD umbrella. After all of the possibilities were eliminated, there was only one disease to confirm: idiopathic pulmonary fibrosis (IPF). A video-assisted thoracoscopic surgery, or VATS, procedure was needed to confirm this diagnosis, as the previous bronchoscopy was inconclusive.

On Dec. 19, 2014, I received the results from Dr. Tolle confirming the IPF diagnosis. He was very optimistic with his verbiage regarding my future. Upon hearing the diagnosis, I felt numb but also optimistic. My wife and I asked some questions, but mainly just listened and processed what he was saying.

Dr. Tolle also said it would be a good time to begin the process of transplant evaluation, as I was relatively healthy. We agreed, and he submitted the request to be evaluated by Cleveland Clinic’s Lung Transplant Center. I was initially prescribed 3 lpm of oxygen on a portable oxygen concentrator. Dr. Tolle began the paperwork for me to receive Ofev (nintedanib), which at the time was newly approved by the FDA as a medication that may stop the progression of IPF.

On Feb. 4, 2015, I had my first series of appointments with the Cleveland Clinic Lung Transplant Center. I was recovering from respiratory syncytial virus and an IPF exacerbation. My prognosis took a serious downturn since my last meeting with Dr. Tolle. My wife, Rebecca, and my sister, Krystyna, attended the appointment. It was during my first meeting with Dr. Wayne Tsuang that I realized my destiny. There were two choices: die or receive a transplant.

I asked Dr. Tsuang to describe to me people’s experiences at the end of life when passing from IPF. He explained to me in great detail. My usually stoic self began to feel the dynamics of what was going to happen to me. Hearing this news broke me down. It really hit me hard when I saw the anxious and worried looks on the faces of my wife and sister. I knew I did not want to leave this earth so soon and would commit myself to getting in the best possible shape for a transplant. I now had processed what this deadly disease could do!

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