The Story of My ELST

I don’t remember ever receiving shots as a kid, so I was spared the trauma many children in Montenegro share. Treatment with injections is extremely common, and probably no child can avoid it at a certain point. Doctors prescribe them for this and that, but I was a healthy child. At eighteen, when I was drafted for the military service and thoroughly examined, I was perfectly healthy, aside from some mild pneumonia from swimming in the sea in early June and spending the fresh night out in only a tee-shirt. The military reports confirmed my perfect health, but now when I look back, I know that they missed some subtler indicators of what was to develop into a chronic disease with multiple complications.

What were those early symptoms of my lifelong illness? First of all, occasional dizziness. I remember not being able to do a front roll and a few other exercises in the PE class that other kids did for fun. I have always been slightly nerdy and preferred books to sports. When I attempted the dreaded front roll everything would go upside down in my head, I would get nauseous, and it always took me a while to regain my balance.

It is not easy to pinpoint the exact time when I started having problems with dizziness. However, I know for certain what aggravated it: cigarette smoke. Most of my friends in high school were smoking. Although I wasn’t drawn to tobacco, I wanted to fit in and practiced smoking on my own to get the hang of it. The obvious practice venue was the bathroom, next to an open window. When I first inhaled the smoke, I would get lightheaded, slightly nauseous, and at times seriously dizzy. I remember having to go to bed one time very early in the evening because of a cigarette. Still, I didn’t want to give up. After a while I got used to it, and gradually became a serious smoker. So, between seventeen and twenty-one I was a smoker like so many in Montenegro. Fortunately, I stopped in 1991 during military service, and never resumed the habit. One reason to quit was that cigarettes really messed up my balance, and I hoped that problem would stop. It didn’t.

Another strange symptom that occurred at around the same time was a ringing in my right ear as well as a gradual hearing loss. I don’t remember seeing any doctors about these symptoms while I was in high school. The problems were manageable, the dizzy spells infrequent, and even though I sensed something in my head was amiss, I wasn’t dwelling on it too much. I thought that whatever it was would pass.

I turned nineteen in the early summer of 1990 and spent it in Croatia where I was born and where I had a large extended family on the maternal side. It was great fun, but my diary entries also register dizziness problems which became more frequent and more serious as the summer drew to the end. On certain mornings it was difficult to get up. I would feel shaky on my feet and dizzy when I turned my head. Some days were better, some worse, but I was managing somehow.

Back home in Montenegro, only a few days before my departure for Serbia where I was to spend a year for my military service, I had a severe spell of dizziness. Lying on my bed, I saw the ceiling corners of the room whirling in circles before my eyes. There were even some days that I could not get out of bed. Still, I managed to see some friends before my departure, wrap up my life as much as I could, and leave for the city of Niš in southern Serbia.

What complicated the military life for me was the continuation of the episodes of lingering dizziness. I called them “head on the door” events, after a line from a song by The Cure, not really knowing what the phrase meant. One morning, as I was standing on the parade grounds getting ready for the reading of the routine orders, I was unexpectedly and instantly hit on the inside of my head so hard that I fell back on the concrete. I had never experienced anything like that before. It felt as if a big hammer struck the inside of my head from within and knocked me over. Somebody helped me up, and I was taken to the local infirmary in the barracks. The wait was endless, I gradually got a little better but still felt unstable on my feet. They didn’t find anything wrong, nor did they bother much. I was returned to my unit.

One month later I again found myself in the military hospital. It was the first time that I was hospitalized for my problems in a fairly good facility, and I hoped they would find a solution. There were many soldiers in the hospital with raccoon-like eyes and big, fat bandage masks over their noses, since many had plastic surgeries to correct nose deviations. I was offered one, too, as a potential solution for my problem. The sight of those eyes and long swabs of cotton stuffed into the nostrils probably dissuaded me from accepting the offer, but I also sensed that the root of my dizziness did not lie there, but elsewhere.

I spent one week in that hospital. The doctors prescribed some pills that never worked, and I was sent back to the barracks. Still, the ordeal in my head was not over. In all kinds of weather and circumstances, with or without drugs, it seemed relentless. I became desperate.

My next step was to stop smoking, which I hoped would set my balance straight. I had been smoking for a couple of years by then, and since joining the army, I began smoking more and more. What else was there to do on a short break but light a cigarette? After a while, I was smoking two packs a day. It had to stop. I tried many times, but I would start again soon after. Not really knowing why or how, I finally stopped on 1 May 1991, the date when serious problems emerged in former Yugoslavia (Croatia, Montenegro and Serbia, as well as Slovenia, Bosnia and Herzegovina, and Macedonia, comprised what was at the time known as Yugoslavia) as a dozen local policemen were killed in a conflict with the Serbs near the town of Vukovar. The event announced the beginning of the local wars that lasted over four years. The general mood in the barracks at that time became even gloomier. Many guys started smoking, out of anxiety. I stopped, probably also out of anxiety, and never smoked again in my life, save for maybe one or two cigarettes.

In September 1991, I was out of the army, heading back home to Montenegro. I entered University and studied at the English Department. My dizziness returned periodically, and I tried to discern a pattern of recurrences—perhaps the bouts were somehow worse in springtime, or perhaps they had to do with this or that. No conclusions were convincing. The “head on the door” did its own thing, unexpectedly and frustratingly. My hearing in the right ear became worse, the tinnitus was loud, and the ringing sound also started in my good, left ear.

I had one more attack similar to the one on the parade grounds in the barracks. I was in the living room of our apartment, and I fell backwards on the carpet. Like that first time, I recovered quite quickly, but felt unsteady on my feet for longer periods of time. Such attacks never again recurred, but I had no clue what caused them. I did not see doctors because my stay at the military hospital in Niš, which was a much better facility than the local ones available to me, had proved futile. An urge to help myself made me become prey to one of a multitude of medical frauds that were abundant in those years. I started ordering “bioenergy cards”—supposedly a panacea advertised in a magazine. The card was made of cardboard, the size of a credit card, and one was supposed to hold it next to the ailing part of the body for a while, every day, for a few weeks. The card would then “expire,” and a new one had to be ordered. I thought it was mildly helping me, so I ordered it several times, hoping it would cure my mysterious condition. Naturally, it didn’t. I stopped ordering the bio-cards and felt discouraged that I would ever find a way to deal with what I had.

I spent the next fifteen years experiencing occasional dizziness. The persistent symptoms continued into late summer of 2007. Just a few days before the beginning of the semester at the University of Montenegro where I was now teaching, my dizziness returned, accompanied with the inability to focus my vision. It felt as if my eyes were wobbling on oil. When I walked, I could not see the faces of passers-by properly, as they would bounce up and down before my eyes. In the mornings I felt reluctant to get up because I did not know if I would feel dizzy, so I preferred to stay in bed and hope that the day would not be too bad. The very thought of going to work tired me out. Luckily, the dizziness did not last for too long this time, and it was mostly gone by the time the semester began. But in spring it was back. The dizziness and eye problems returned in March, April, and May 2008.

In the summer of 2008, I flew to America to participate in a conference on Aldous Huxley in Pasadena, Los Angeles. I enjoyed it a lot, but the conference and fun were not the only purposes of this two-week trip. I also arranged to see a doctor about my persistent dizziness. It had wreaked havoc in my head for twenty years.

I need to go into a brief digression in order to cast more light on my search for a solution to the dizziness problems. Two years before my trip to Los Angeles, and by some unusual turn of events, I found out that I had a half-sister who was one year older than I and who lived in Germany. We met soon after we discovered each other, and concluded that we had a lot in common. One of the things that we shared were similar health issues. She did not have hearing or balance problems, but she had had a brain tumor, with a scar in the same spot (the back of the head), as well as kidney tumors and problems with eyesight. The difference was that in Germany she had been diagnosed with vHL, while I did not have a common diagnosis for my problems. But learning this about her got me thinking. So one year later, in the summer of 2007, I went to Germany to get tested for vHL. Expectedly, the tests came back positive. This offered me more insight into the symptoms and complications. On my own I concluded that the problems in my head were probably a result of an ELST—an endolymphatic sac tumor. As soon as I read the material on vHL and learned that ELST is one of the complications, I knew that it was the root of my symptoms. It is a small tumor in the inner ear, causing balance and hearing problems. It was now a matter of having it confirmed by a specialist.

On the Internet, I found Dr. Friedman at the House Ear Institute. Dr. Friedman is a specialist in the tumors of the brain base, so I booked an appointment. From him I finally got the long-awaited confirmation. Yes, he could see an ELST in my right ear. He said he would be willing to operate on me.

This news was liberating. A resolution of the two-decade tyranny of my inner ear problems was at hand. I read many case studies of people with vHL who had this tumor, which is in essence benign but causes many discomforts. In one case, a patient’s untreated ELST made a hole in his palate, and cerebrospinal fluid began dripping on to his tongue. This terrified me, but a solution was now finally possible. However, there was another major hitch: finances. The surgery in America cost a lot. Too much. I could have sold my apartment, and it would still not cover all the costs. Where would I live then? I had no other savings. Still, this confirmation meant so much to me, and I set off on a mission to find a way to get rid of the tumor in my inner ear.

In my search for the facility that would perform my surgery, I chanced upon vHL Alliance in Boston with Joyce Graff as its then head. I wrote to her directly and explained the problem. Very promptly and very kindly, she responded to my inquiry and suggested that I contact the National Institutes of Health (NIH) in Bethesda, near Washington DC, who do research on vHL. If I managed to get into their protocol, I might get the surgery for free. That idea sounded too uncomplicated to be true, particularly taking into account my readiness for a lengthy struggle to have it done somehow and somewhere. But I did contact the NIH and communicating with them turned out to be even easier than it had seemed after Joyce’s e-mail. Very quickly, very efficiently, and very kindly, without unnecessary documentation, they invited me to see them just after Christmas 2008. On that day a new chapter in my dealing with vHL began.

The fact that I visited Washington DC for the first time in my life on Christmas was very fortunate because I could afford to stay in a good hotel because the prices were much lower than normal. Everybody I spoke to was very kind, and the city differed greatly from any other American metropolis I had visited by then. There were Christmas trees in front of the White House representing every American state and territory. I walked the National Mall and listened to Grace Jones’ album Hurricane. I also needed to book a hotel for two weeks after my release from the hospital, but average prices because of Barack Obama’s inauguration went up as hotels raised their fares. I decided to spend that time at a friend’s place in New York, but a lot was to happen in the meantime.

I arrived at the NIH on the metro train on January 4, 2009. The enormity of the place amazed me, and everything was new to me. I did not know I had to go through a security check very much like those at the airports. When my bag was being scanned, they saw that I had a big bottle in my backpack. “Do you have some alcohol, sir?” I said yes, I had brought a bottle of local Montenegrin brandy as a gift to Dr. Lonser, who was going to operate on me. Bringing gifts to doctors is a standard procedure in the Balkans. Some even expect gifts, including money. I never offered money or gifts to them, except for small tokens of appreciation to some of the doctors who were professional and kind. But this time I thought a bottle of local alcohol from a small, exotic country might be a good idea, just to show that I was very thankful for being admitted. The security person at the scan said briefly: “Sir, no alcohol can be brought into the campus!” I understood and said: “Okay, no problem, you can keep it then, have a party.” He repeated: “Sir, no alcohol is allowed on campus. You can take it back to your car.” “But I haven’t come here with a car. What am I going to do with it now?” “You cannot take it in here!” So I went out of the visitors’ center with the bottle in my hands and chucked it into the first trash can. However frustrating it was on one hand, on the other I was delighted with the order, the observance of rules, and the strictness of the zero tolerance for alcohol and cigarettes. I thought then about Montenegro, where these rules are breached everywhere, even in hospitals, and I knew I had come to a different world.

I was admitted at the Clinical Centre and continued to be amazed at the level of professionalism, kindness, and comfort. The room was almost like a hotel. I got a menu from which I could order food over the phone, with no restrictions, and with great variety. Each bed had a computer connected to the Internet, which was unbelievable to me, but so helpful in those pre-wi-fi days. There was another patient in the room, a cool Hispanic guy who was receiving some test treatment for Alzheimer’s, I think. I was sent to extensive screenings. My life whirred around in my head during the long MRI sessions, and my emotions were floundering. But I had to keep it all down because I was now here where life had taken me. The schedule was full of different tests and interviews, which were also a novelty for me, and a good distraction. The approach of the staff was something I had not experienced before: they were open, willing to talk, explain everything, and chat about things unrelated to health They urged me to ask questions. It felt as if I was participating in one of the medical TV dramas, and I got accustomed to the culture very naturally. One of the nurses asked me where I was from, and I said from Croatia, which is easier to say than Montenegro because many more Americans have heard about Croatia than Montenegro. It is also true, since I have always considered myself to be as much from Croatia as from Montenegro. The nurse responded: “Oh, there is another nurse here who is from Croatia. She will be here on the night shift, her name is Jeanne.” I thought that Jeanne, or Žana as we say, was a very likely Croatian name, and I was happy to have a chat with someone from my own culture, in my own language. However, it turned out that Jeanne was a Russian from Kyrgyzstan! But she was very, very nice, and we had a laugh about the mix up. Croatia and Kyrgyzstan are as similar and close to each other as Uruguay is to Greenland.

All in all, my confidence that everything was going to be good was strong, and I was more or less prepared and calm for the resection of the ELST, my long-time companion who had given me a lot of trouble. The doctors listed the potential risks of the surgery, such as damaging the facial nerves or even death. The fact that it was me who was to make the final decision about whether I was having the surgery or not was new to me, and I can’t say I didn’t hesitate upon hearing the risks spelled out so clearly. However, the negative outcomes were unlikely to happen, and the ELST had given me a hard enough time, so I decided to go through with it, no matter what. The only expected downside was the complete loss of hearing in the right ear, but since my hearing on that side had not been very functional for a long time, I didn’t mind. The surgery was scheduled for January 7, 2009, which is Christmas in some Eastern Orthodox traditions including Montenegro, because they follow the Julian calendar and have not yet updated to the Gregorian one. This is the one-line entry in my diary from that morning while waiting to be taken to the operating theatre: “Ready for the operation. My blood pressure is perfect, even without medication. I am very calm.”

The surgery lasted around four hours. I spent one night in the ICU. The next day I was on the floor, in my room. I could not believe that things went so quickly, so smoothly, and with so little trauma. On January 8, I was already walking and inwardly praising this amazing hospital and its people. My hearing on the right side was gone for good, but everything else was fine.

The next two days were more difficult. My temperature would go up, I would have bad headaches. It affected my emotions as well. On January 9, I wrote: “All of this has somehow broken me down, but I force myself not to think about anything. I feel as if floating, I am sensing the things are not good, but I am not thinking about it.” On January 10, there was again an upswing. I was reading the book Baltasar and Bluminda by José Saramago, which at some point became a little difficult, so I switched to The Reader by Bernhardt Schlink, which I read in two days, and then returned to Saramago. I was able to go up to the seventh floor where the patient library is, and spend some time on the Internet, but I got tired quite quickly. I was also worried about how I was going to travel to New York in only eight days. I was not sure I’d be well enough in such a short time to make such a long trip on the train. But somehow I trusted things would turn out fine.

The rest of my hospital stay was marked by ups and downs. All my bodily functions were returning and stabilizing, but dizziness would bother me at times, which was only to be expected with all the fluids still unsettled in my head. There were moments when the pain was too bad, but the medication helped with that a little. I made a list of post-discharge questions to ask my doctor about, and with those answered, I finally left on January 14, one week after the surgery. I took a taxi from Bethesda to central Washington DC, the ride felt long and bumpy, and arrived at the Red Roof Inn in China Town with a heavy suitcase and backpack. I was not supposed to lift anything heavy for a few months after the surgery, but what was I to do? I had no choice but to carry the bags into the elevator myself and then up into my room. They were not light, and I prayed in my mind that this feat did not damage anything in my head. I was very glad when I finally did it, and closed the door behind myself remaining in the privacy of my own room. Leaving the ICU for the floor always feels liberating, leaving the hospital even more so.

The toll of carrying the heavy bags kicked in soon thereafter. Very quickly after I checked in, a most excruciating headache ensued. I have heard that the seventh day after an operation always ends up in terrible pain, or perhaps this time it was because had I lifted the suitcase. But despite my relatively high tolerance for pain and discomfort I thought I was going to die—that is how much it hurt. I immediately took a Percocet and, unable to lie down, started washing my socks over the toilette sink. While washing them, the medication started working, and the pain slowly subsided, but it left me shattered and sleepy. I was getting hungry; it was dinnertime. I decided to go to the closest restaurant across the street and eat something. On my head I still had the staples and the bandage, and I was wearing a woolen hat over it. I put my winter jacket on and very slowly walked out of the hotel to the nearest eating place. It turned out to be a Burmese restaurant. I had to climb a flight of stairs to reach the eating area. My hat was on, I was not taking it off, just as I was not taking off my jacket, not because I needed them—it was very warm in the restaurant—but because the movement of taking the coat and the hat off would involve head movements that would cause me to stumble. So, I sat and ordered a full meal, and asked for chopsticks. I think I remember having something with pumpkin as the main dish. While eating I felt too warm and too sleepy. A few times my head nodded off and almost bumped into the plate in front of me. I was thinking about how others saw me: a weird, hunched guy, with a hat and a coat on, eating with chopsticks, and falling asleep into his meal. In a city like Washington, homeless people and drug addicts are not a rare sight, but I think I still differed from them a little, and I don’t think anyone could have guessed what was really happening to me. But nobody asked me anything. I could not turn my head around to look at other people’s reactions. I finished my food, left a $20 note on the table, and slowly exited. I slept well that night, and the intense pain never returned after that.

On Friday 16 January, I noted in my journal that I was feeling 95% normal. I was in no pain, I walked normally, and had a good appetite. Friday was also the day that I returned to the NIH for the removal of staples on the incision, and that went quickly and smoothly. In the evening I took a walk across the National Mall. The evening was brisk, and the atmosphere busy. The preparations for Obama’s inauguration were taking place. Green portable toilets were lined along the Mall. New energy was about to come to the world, and the ELST from my head was gone. It would prove to be the end of my over two-decades-long spells of dizziness.

A few weeks later, after my stay in New York, I was seen again at the NIH. Dr. Lonser was happy with how the wound healed and how I was feeling. My general impression at the end of these five weeks was that everything passed rather quickly. Everything turned out well, and I was full of optimism.

This experience at the NIH was just the start of my long engagement with this amazing hospital. However, things are always unpredictable with vHL, so I could not have known that I would come back there for five more surgeries: one on the brain and four on the kidneys. I am afraid more surgeries will be needed in the future. We vHL people have to leave things open, live as normally as possible, and be prepared for whatever comes. That is, I think, a principle that applies to everyone, vHL or not, but in our case it is maybe a little more pronounced because the potential health risks take place more frequently than in the general population.

To live with vHL means to be prepared for disruption every day—whenever the illness poses a threat and whenever a surgery is needed. Relationships suffer due to these disruptions, as well as work. For example, I started writing an academic book titled God Behind the Screen: Literary Portraits of Personality Disorders and Religion in 2013. Before I completed it for publication, I had to abandon the research and writing four times for major surgeries in the USA, as well several more times for check-ups and other vHL-related distractions. Besides, frequent surgeries, which involve such derailments from the steadfastness of our lives, also mean serious emotional upheavals, in many cases depressive episodes, financial insecurity, and a lot more. Still, I feel lucky for managing to stay reasonably healthy, which means that I lead a more or less normal life, while many vHL patients, unfortunately, either suffer grave complications, such as blindness, loss of the ability to walk, partial paralysis, and the like, or lose the battle with this illness altogether. Raising awareness of this rare disease, and advancement in the research for finding a cure, would mean a lot for our community of approximately 200,000 people worldwide affected by vHL. Writing this essay is a modest contribution on my part to raise such awareness.