Fibrosing Mediastinitis

My story

Glossary of terms used in this story

Over the Labor Day weekend 1999, I went to the ER to be evaluated for some chest pain and a cough I’d been having. Chest x-rays, blood gases, and blood work turned up nothing, but the doctor taking care of me that day, Dr. Michael Bain was insistent that my symptoms were not normal. He ordered a lung scan which showed complete occlusion of my left pulmonary artery; that is, I was getting no blood flow to my left lung. I thought I had a pulmonary embolism, but I was told that the cause of my obstruction was, most likely, a mass, but it would be impossible to biopsy due to the location. The mass seemed to have been pressing against the artery and had caused a blood clot to form inside the vessel. A needle biopsy would certainly result in severe bleeding. I was hospitalized and placed on blood thinners. Next, I was taken for a pulmonary angiogram to evaluate whether or not I had a blood clot. The next thing I had done was a bronchoscopy to evaluate whether or not I had cancer cells in my lungs. I was sedated, but I remember the scope going into my nose and straight down into my lungs which caused severe coughing and discomfort. I spent the next several days in the hospital, growing more short of breath and having more pain than I had previously. At one point I got into the shower and was unable to get back to bed without help. They told me that my life would be like this because I was living with one lung. I could not understand how I'd gotten so much worse in just a few days. The doctors told me they would re-evaluate me in a few months. I was sent home a week later with pain pills and blood thinners and no real diagnosis.

The night I got home I was awakened in the middle of the night with severe pain and shortness of breath. I called my brother who took me immediately back to the hospital where I ended up in ICU on blood thinners and morphine for the severe pain. I was in and out of awareness because of the drugs and a few times I thought my life was really over. My mom stayed with me most of the time. Several doctors consulted on my case. I was taken to the radiology department where they placed a PICC line in my left arm. The thoracic surgeon felt the best thing to do would be to take out my lung. I didn’t agree with this decision and refused to have it done. No one seemed to know what to do with me. There was discussion of sending me to either Vanderbilt in TN, Mayo Clinic in Minnesota or to UCSD in San Diego. Three days later I was taken on a plane to the Cleveland Clinic. My mom boarded the tiny plane with me. I don’t remember anything about the flight as they had sedated me with Ativan and Morphine. An ambulance transported me from the airport to the hospital where I was initially placed on a medical unit. The flight nurse said, “I will not leave this patient here. I took her out of ICU and I’ll take her right back where I brought her from.” I was in and out of coherence because of the morphine, but I remember thinking, My god, they’re going to put me back on the plane and take me home. Somehow I ended up in a monitored step-down unit. All of the special care I’d received at my home hospital was gone. I called my doctor back home and begged him to let me go back to Cincinnati. I was scared. He told me I was exactly where I needed to be. My begging was futile. I was stuck.

I had no idea what was going to happen to me. I had strange dreams. I woke up when I thought I was already awake. I would hear people in my room who weren’t there and on more than one occasion I felt someone tapping on the bottom of my feet. I thought it was my mom, but each time I opened my eyes to see her there was no one in the room. I was scared, but tried to keep my sense of humor. I thought about all the things I hadn’t done in my life. I had always wanted to go to Europe.

I was taken to the cardiac cath lab where I underwent a rather unpleasant catheterization through my jugular vein as opposed to the femoral vein I had previously had accessed. The radiologist was rather rough and was rather aggravated that the catheter would not go into the space he wanted. I was forced to keep my head turned to the left with a wooden box covering my face. I could feel my heart palpitating when the doctor attempted to place the catheter through my heart, causing dysrhythmias. The nurse counted off the abnormal beats and I tried to concentrate on what was going on, thinking to myself: As long as I can hear her voice, I’m alright. Finally, I was taken back to my room. The sheath was left in my neck and stuck out past my right ear. I could feel it stabbing every time I moved my head. The catheter tip was in pulmonary artery near the blockage and the clot. Figure 1 The idea was to instill a continuous infusion of tPA, a clot-busting enzymatic drug. The drug also caused severe bleeding because it inhibited natural clotting in the body. The infusion would last for several hours throughout the night. During the night the PICC site and the catheter in my neck bled continuously. My foley catheter was bloody. Blood ran down my neck to my chest and onto the bed. Blood spurted out from the PICC site every time I moved my arm. The nurse placed pads underneath my arm to catch the blood and took away heavily saturated pads all during the night. “Quit moving your arm,” she scolded me. She wanted me to hold my arm in an unnatural position to keep the blood from coming out. The whole thing was a nightmare. I was worried about losing so much blood, but no one seemed to be too concerned.

In the morning a resident I’d never seen came in and said, “Oh, you’re bleeding too much.” That’s what I’d been thinking. They stopped the tPA infusion. I was taken back to the cardiac cath lab where they checked the status of the occlusion. Nothing had changed. The radiologist said very little to me. He yanked the catheter from my neck and walked out, leaving me on the table. On his way out he said, “Your lymph nodes are all enlarged.” Figure 2 I had no idea what that meant and no one explained to us what was going on. I was taken back to my room.

The next procedure they wanted to do was a mediastinoscopy, a surgery where they would make an incision in my neck, put a scope into my chest and biopsy the mass. I waited around for several days while the tPA got out of my system, but I remained on a Heparin drip. The surgeon came in and explained the procedure and told me my Heparin drip would be turned off the night before my surgery. Around 2am on the night before my surgery I noticed my Heparin drip was still going. I called the nurse in and told her what the surgeon had said. “We don’t have an order to turn it off,” she informed me.
“Well, you better get an order because I’m not waiting around another day to have this surgery.” I was frustrated because I’d had to wait for 3 days as it was and I still did not know what was wrong with me. I had very little confidence in Cleveland Clinic at this point. A short time later, the heparin was turned off.

The next day I was taken to the huge OR where 2 doctors spoke to me in a French accent. One took my right hand and plunged a needle into my radial artery to start an arterial line. The next thing I remember is that I was waking up in the recovery unit, my throat very sore and hoarse. I now had a suture line across my throat. The next day I was up and about a little better. I still had the pain and was pretty weak.

My mom and I spent a lot of time sitting outside by the fountain and walking through the hospital. Another resident, a frumpy woman with whitish hair came in to see me. She said something about “my diagnosis.” My mom said, “What is her diagnosis?”
The resident looked at us. “You mean you don’t know?”
“No,” we said in unison. “No one’s told us anything.”
“You have fibrosing mediastinitis.”
I’d been a nurse for several years and my mom had worked in the medical field even longer and neither of us had ever heard these words before.
“What do we do about it?” my mom asked.
“There’s nothing to do about it. There’s no cure and it’s progressive. I’m sorry. It’s caused from a Histoplasmosis infection.”
We were stunned. I had a disease we’d never heard of and I was going to die from it.

Finally, after a few more days, they weaned me from the blood thinners and started me on Coumadin. I was taking Sporanox, an anti-fungal drug, and Prednisone. I was released from Cleveland Clinic. A family friend flew me home in his private plane. I returned to my apartment, but did not return to work for several weeks. I went to the hospital for a routine blood test about a week after I returned home to check if my blood was thin enough with the Coumadin. I ran into the thoracic surgeon that had taken care of me before I was sent to Cleveland. He asked me why my voice was hoarse. I could barely speak above a whisper, but I had attributed it to the endotracheal tube that had been placed during my surgery. He said, “Your voice should be normal now. I think you should have it checked out.” I made an appointment with an ENT specialist. He put a scope into my nose and down my throat to evaluate my vocal cords. He told me they were paralyzed. He didn’t know if my voice would come back so he gave me two options: I could wait to see if my voice would come back on its own or I could have him remove and repair my larynx. Having already had my neck probed and slashed within the past 3 weeks, I wasn’t too motivated to have more surgery. I opted to wait. I called the surgeon in Cleveland Clinic who had performed my mediastinoscopy and he said, “Yes, I probably knocked your laryngeal nerve around. Let me know if your voice isn’t back by spring.” This was October.

I returned to Cleveland Clinic to follow-up with the pulmonologist, Dr. Mehta. He told us that I was still having chest pain because I was “throwing clots” to my lung. He told me that they could not stent my pulmonary artery because it was so narrow they could hardly get a guide wire into the opening. There was no surgery that would help. His only advice was to “live my life” and he could not give me a prognosis; only telling me that people with my disease live until their early 40’s. He told me to seek help if I 1) had more pain 2) had more shortness of breath or 3) started coughing up blood.

Back home, I persuaded my supervisor to let me return to work even though I was still short of breath and had no voice. I also still had a pleuritic pain. She told me that I should consider going on disability, but I told her there may be a time when I really do have to go on disability and I wanted to wait. She allowed me to return to 4 hour shifts, then 8 hours and gradually back to 12 hours. I couldn’t speak very loudly which made it difficult to talk to hard-of-hearing patients. My co-workers gave me a whistle to use in case I needed help because I could not yell.

By mid-November my voice started to return on its own. I had always wanted to travel so I started looking into travel nurse jobs. Against the advice of my friends, I decided to take a job in California, in San Diego. My thought was that if I ever got sick again I’d be close to UCSD. I moved myself to San Diego and did pretty well for a while. Then I had episodes where my feet and hands would turn blue. I was often short of breath and my endurance level was very low. I had chest pain all the time. Chest pain had been fairly constant for me since the beginning and I was learning to live with it. For a couple of days I had more severe pain and a co-worker who worked part-time in the ER at UCSD took me to Thornton Hospital in La Jolla. I was admitted under the care of a pulmonologist named Dr. Kim Kerr. She gave me a dose of Solu-Medrol which dramatically reduced my pain. She reviewed my history and ordered an MRI which showed that I probably never had a blood clot, but that the fibrosis had completely encased my pulmonary artery. She said to me, “I have a friend who is a pediatric cardiologist. He has tiny instruments and I think he can do an angiogram on you.” I was taken by ambulance from Thornton Hospital to the main hospital in Hillcrest in downtown San Diego.

On May 9, 2000, I underwent another pulmonary angiogram with Dr. Abraham Rothman. This time I was asleep for the four hour long procedure. When I awakened the first thing I was told was that Dr. Rothman managed to open my pulmonary artery with 2 stents (Figure 3). I was taken to the ICU where I spent the night in case I went into flash pulmonary edema, a possible side effect of having a sudden rush of blood to my lung that had not had circulation for a long time. Fortunately, I suffered no ill effects. Dr. Kerr had another lung scan done the next day and the blood flow to my lung seemed to have disappeared. Once again, I went back to the cath lab to have the stents checked. The angiogram showed that everything was working fine. I was released from the hospital on coumadin and a few days later I was in my truck driving back to Ohio. The experience at UCSD was the antithesis of my grim hospitalization at Cleveland Clinic.

For a few years I did really well. In 2002, I met my partner, Monica and settled on the central California coast. The pulmonologist I went to told me outright, “I don’t want to be your doctor. I don’t know anything about your disease.” He encouraged me to go back to San Diego. I had episodes of chest pain that sometimes became more severe, but tests always showed that my blood flow was adequate. Usually, I would be placed on short-term steroid treatment and the pain would subside to its usual tolerable level. Living with this disease is still frightening and frustrating at times. In December of 2002 I returned to San Diego to see Dr. Kerr in hopes that she could shed light on why I was still having chest pain. She told me I could have another MRI, but, she warned, if the mass was changing there was nothing she could do about it. She did and echocardiogram to make sure I wasn’t developing pulmonary hypertension. It was normal. She told me I would have “flareups” and that they should be treated with steroids. I opted not to have the MRI because I only wanted to concentrate on things I could have fixed. Over the next few years I noticed my stamina decreasing and my flareups becoming more frequent. In I visited another pulmonologist who told me that I needed to have another angiogram to check my stents. I knew it was the prudent thing to do, but the truth was that no one in my network was willing or able to do a pulmonary angiogram, and if they did, they would not be able to do any interventions. In January 2005 I had to have a minor surgery to biopsy a lump I found on my breast. In the recovery room I had horrific chest pain. The pulmonologist was called in to see me and he surmised that my blood pressure dropped causing an ischemic chest pain because of the compromised pulmonary artery. Nearly a year went by until summer 2006 when I developed some of the worst pain I had ever had. I thought it was just another flareup, but something told me it was more serious. I was also experiencing more fatigue and having blackout spells when I’d lift or stretch. I tried to wait it out, but I ended up going to the ER where, once again, I was told I needed to have an angiogram to check the stents. I realized the best thing to do was to try to find Dr. Rothman and ask him what to do.

A few days later I found Dr. Rothman after searching for him on the Internet. He told me he wanted to do the angiogram himself and wanted me to make arrangements to fly to Las Vegas where he was now practicing. In September I went to Las Vegas with Monica and met up with my mom. Dr. Rothman examined me and was not surprised by any of my symptoms. The angiogram revealed that my stents had nearly closed off completely. He said I was as ill as I had been when he first met me in San Diego. Apparently, the tissue in my vessels grew through the stent and closed them off. “You’re a tissue grower, “ Dr. Rothman told me. Amazingly, he managed to open the stents up using balloon angioplasty (Figure 4) even more than he had initially six years before. The next day I could tell the difference in how I felt. The plan is for me to have angiograms every two years to check the stents and reopen them if need be.

In 2003, I finally took the trip to Europe I had always dreamed of.

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Fig. 2 Back to text

Fig. 3Stent
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Fig. 4 Balloon in arterial stent
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