My Story

I was born in New Jersey, but was raised in the Ohio Valley. My childhood was spent playing in the woods around our home. I had the usual run of childhood illnesses, but nothing very serious, except for a bout of scarlet fever when I was six.

I graduated from nursing school in 1986 and worked in intensive care and the emergency department. During the mid-1990's I had what seemed to be a never-ending respiratory infection. My family doctor would put me on antibiotics, but I never seemed to get over it. I persuaded him to let me go and see a pulmonologist.

As soon as the pulmonologist saw me he told me I had asthma. He put me on inhalers and I accepted my fate. I continued to have a frequent cough and shortness of breath, but I just accepted that this was normal.

One day, while I was at work, taking care of a patient in the recovery room, I completely lost my vision. Everything went dark. I had no other symptoms, but I was alarmed. One of my co-workers took me across the street to the eye center where I sat in the waiting room, blind, for nearly an hour. During that time, my vision gradually came back. The doctor finally examined me and declared that I had an "ocular migraine." The same thing happened during the summer of 1999 while I was cleaning my apartment. It lasted a bit longer, but I attributed to this "ocular migraine" and didn't worry about it as much.

Over the Labor Day weekend in 1999,  I began having terrible chest pain. Every breath felt like a knife going through my chest. I went to the ER to be evaluated. 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 didn't like the fact that my resting heart rate was over 110.

Because I had recently returned from a long road trip, he ordered a lung scan to see whether I had a pulmonary embolism. The lung scan 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. It would be impossible to biopsy the mass because it was lying against the artery. The radiologist believed a blood clot had formed inside the vessel. A needle biopsy would certainly result in severe bleeding. I was hospitalized and placed on blood thinners. The obstruction in my artery was also, most likely, the cause of my earlier temporary blindness.

Next, I was taken for a pulmonary angiogram to evaluate whether or not I had a blood clot. Then I had 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.

My normal right lung

My left lung, blocked by FM

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, but no one had explained what was happening to me.  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 with pain pills and blood thinners, but 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 the 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.

I knew most of the nurses who were taking care of me since I worked with them. They were extremely attentive and protective and I had visitors all the time. At one point, a close friend I knew from college showed up from Columbus. It was atypical of her to wander far from home so when I saw her standing in my doorway I felt a sense of dread. "Oh, geez, I must be dying," I told her. We still laugh about that.

 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, a treatment I was not too keen about. I 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 Tennessee, Mayo Clinic in Minnesota or to UCSD in San Diego.
 Three days later, I ended up on a plane bound for Cleveland Clinic. A parade of my friends and co-workers lined the hallway from the ICU through the ER and out to the awaiting ambulance. Later, I found out that one of the nurses told my close friend, "She's not coming back." I was never fully aware of how sick I was.

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 Cleveland Clinic 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."

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 that 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 was taken to the cardiac cath lab where I underwent a rather unpleasant catheterization. The doctor went through my jugular vein as opposed to the femoral I had previously had accessed. The radiologist was rough and was aggravated that the catheter would not go into the space he wanted. I was forced to keep my head turned to the left and they had placed this strange wooden box that covered my face.

As a critical care nurse, I had helped with several of these procedures. I knew that passing a catheter through the heart could cause irregular heartbeats or dysrhythmias. It was even possible to put a patient into a fatal arrhythmia.  I could feel my heart palpitating when the doctor attempted to place the catheter through my heart, causing these 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 my pulmonary artery near the blockage and the clot.

The plan 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. The urine in my catheter was crimson. 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 and 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.”  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 the 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 waited for three 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 surgical suite where two 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 and feeling a little better, but 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 pulmonary resident, a frumpy woman with whitish hair who I had never seen before, came in to see me. She said something about “your 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." Then she told us that it was caused from a Histoplasmosis infection. 

I never had asthma. We were stunned. I had a disease we’d never heard of and I was most likely 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 three 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. 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." 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 had more pain, more shortness of breath or 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 would 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. I was a little scared about leaving my job, but I was feeling fairly well and thought I should seize the opportunity. I also had an ulterior motive.
Against the advice of my friends, I decided to take a job in San Diego, California. My thought was that if I ever got sicker I’d be close to University of California San Diego (UCSD). I moved myself to San Diego and did pretty well for a while.

A couple of months later, I began to have episodes where my feet and hands would turn blue. I was often short of breath and my endurance level was very low. I had been having chest pain every day since the previous summer and I was learning to live with it, but in the spring of 2000, it had gotten worse. 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.

 I had developed a pericardial and pleural effusion that caused severe pain. Dr. Kerr would stand outside the door of my room where she could actually hear my lung squeaking as I breathed. "That hurts, doesn't it?" she asked.

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.” 

She knew I had been told that my artery could not be stented so she made no promises. 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. 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 on the central coast 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 an echocardiogram to make sure I wasn’t developing pulmonary hypertension. It was normal. She told me I would have “flare-ups” 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 flare-ups becoming more frequent.  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 insurance network was willing or able to do a pulmonary angiogram, and if they did, they would not be able to do any interventions.

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 flare-up, 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 2006, Monica and I flew to Las Vegas and met up with my mom who flew from Ohio. 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, using balloon angioplasty, he managed to open the stents up even more than he had initially six years before. The next day, I could tell the difference in how I felt.
I followed up with Dr. Rothman in March 2009 and had another angioplasty. This time my artery was not as closed and he felt it would be possible for me to wait longer before doing another procedure.

As far as I know, my FM has not spread. I still have chest pain periodically with shortness of breath. Two times I have received narcotic pain killers for unrelated conditions and developed the worst pain of my life. I am not entirely clear what that is all about, but I think it may be related to my FM and the low perfusion of blood to my chest. I don't tolerate high altitudes well, but I basically live a normal life. I no longer take my health for granted. Living with FM has opened my eyes to what is important: love, family, and experiencing as much as you can.