ON THE RECEIVING END Patient, Physician, and Researcher My Life’s Journey with Recurrent Spontaneous Pneumothorax Rickard Ljung Unit of Upper Gastrointestinal Research, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden; and Unit of Epidemiology, Institute of Environmental Medicine, Karolinska Institutet, Stockholm, Sweden

On January 13, 1987, at the age of 19 years, ¨ the author was admitted to Ostersund Hospital in Sweden with a life-threatening hemopneumothorax. This was the second of his six visits to a hospital emergency department for sudden onset of severe chest pain. The experience of recurrent spontaneous pneumothoraces influenced his choice of a career and has shaped his adult life in many ways. I experienced my first pneumothorax at the age of 17 years and my most recent episode at the age of 32 years. I am 46 years old now, and I wonder how soon, if ever, I will experience my next pneumothorax. My early episodes influenced my decision to become a physician. Subsequent episodes have taught me to avoid certain triggers and to seek medical help promptly when I develop chest pain. I believe that my experience of recurrent spontaneous pneumothoraces has made me a more empathetic physician. I am sharing my story with the hope that it will provide clinicians who treat pneumothoraces with insight into life with this condition. I was born in Sweden in 1967 and have lived in affluent suburbs of Stockholm for much of my life. I am an Associate Professor at the Karolinska Institutet and a public health researcher. I am married. I have two healthy children and no familial history of spontaneous pneumothorax, lung disease, or autoimmune disease. I never smoked cigarettes and was not passively exposed to cigarette smoke as a child. I play floorball

and jog regularly. I am HLA-B27 positive. I experience Raynaud phenomenon and symptoms suggestive of a spondyloarthropathy, which has not been further classified. I am tall (192 cm) and slender (body mass index, 16–20). At the age of 21, I underwent fiberoptic bronchoscopy and was found to have bilateral congenital bronchial anatomic abnormalities. I participated in a casecontrol study that found a strong association between bronchial abnormalities such as mine and spontaneous pneumothorax (1). I experienced my first pneumothorax at the age of 17 years while coughing during an episode of acute bronchitis. I was suddenly stricken with shoulder pain and shortness of breath. After learning more about spontaneous pneumothorax, my parents recalled other episodes during my adolescence when I complained of similar, though less severe, discomfort. They did not remember the triggering factors for those earlier episodes. Two years after my first pneumothorax, during my military service, I went on a recreational downhill skiing trip for a weekend. The weather was very cold at 2308 C. Again, as before, I was stricken with diffuse left shoulder pain. I took acetylsalicylic acid as a painkiller for a day. I felt tired and was uncomfortably short of breath. I stopped skiing, skipped dinner, and went early to bed. Despite my earlier pneumothorax experience, I did not initially connect the two episodes and instead cursed my bad luck as “a strained muscle” during my much-anticipated days

off for skiing. Later that evening, I awoke because of chest pain and then lost consciousness. A chest radiograph obtained in the emergency department of a nearby hospital showed total collapse of my left lung with an associated pleural effusion and lateral displacement of my mediastinum (Figure 1). I recall uncomfortable rapid shallow breathing that forced me to sit in a crouched position and being reluctant to take a deep breath due to pain. My doctor told me that I was pale and tachycardic. Breath sounds were audible by auscultation only over the right side of my chest. I do not remember being afraid at that time. A chest tube quickly drained 1,000 ml of bloody fluid from my chest. Due to the unexplained excessive bleeding, my doctors performed a thoracoscopy and drained an additional 700 ml of bloody fluid from my chest. The thoracoscopy showed a fibrous pleural surface, indicating that I had previous or chronic pleural inflammation. No bullae were seen. I first became afraid during the thoracoscopy when I noticed that the surgeon seemed unsure of what she saw. I remember asking, “Do I have cancer?” I was flown to Karolinska University Hospital for continued care. I was hospitalized for 3 weeks altogether. When discharged, I weighed only 55 kg (body mass index, 15). My recovery was slow. I was told never to smoke tobacco and to avoid scuba diving. However, I was able to complete my military service and was accepted, 10 years later, for reserve medical officer’s training, which I fulfilled. I came to realize how serious my

(Received in original form October 8, 2013; accepted in final form December 29, 2013 ) Supported in part by grants from the Swedish Research Council through the Swedish Initiative for Research on Microdata in the Social and Medical Sciences (SIMSAM) (2008:7496). Correspondence and requests for reprints should be addressed to Rickard Ljung, M.D., Ph.D., Associate Professor, Unit of Epidemiology, Institute of Environmental Medicine, Nobels Vag ¨ 13, Karolinska Institutet, SE-171 77 Stockholm, Sweden. E-mail: [email protected] Ann Am Thorac Soc Vol 11, No 3, pp 442–443, Mar 2014 Copyright © 2014 by the American Thoracic Society DOI: 10.1513/AnnalsATS.201310-348OT Internet address: www.atsjournals.org

442

AnnalsATS Volume 11 Number 3 | March 2014

ON THE RECEIVING END

Figure 1. Posterior-anterior and lateral chest radiographs obtained in the emergency department of a hospital documenting the author’s second spontaneous pneumothorax.

condition was and how easily I might have died had I experienced my major pneumothorax a century earlier. I endured my third pneumothorax 1 year after the second, when I was 20 years old. I was standing on a public bus holding a handle with my right hand when the bus made a quick stop. I held on, but felt a sudden pain behind my right shoulder. That time I recognized the symptoms immediately. After I reached the hospital, a chest radiograph verified a right-sided pneumothorax. I did not need a chest tube; however, I did have to cancel a ski trip to Italy. Six months passed and I was beginning to think my episodes of pneumothorax were behind me when a fourth pneumothorax spoiled the celebration of my 21st birthday. I was dancing at a club when I suddenly again felt my all-to-familiar right chest and shoulder pain. I did not want to go to the hospital in the middle of that night, so I waited. I was angry at my disease and refused to accept that it would continue to affect my life as it had for 3 years. Because of persistent sharp pain, I relented the next day and sought help at a nearby hospital, where I was found to have yet another right-sided pneumothorax. No chest tube was placed. My hopes for no more episodes were dashed later that same year. I was working

extra night shifts in a post office, where I was required to do heavy lifting. After a particularly heavy lift, I experienced a sudden characteristic pain beneath my shoulder. Once again, a chest radiograph confirmed a right-sided pneumothorax. I was again hospitalized, and this time a chest tube was placed. The senior consultant’s first comment when she saw me was, “So, you haven’t stopped smoking yet”? I had my wits about me, and I replied “Hey, I have never, ever smoked a single cigarette.” That was the third pneumothorax on the same side within a year and my fifth episode overall. Thoracoscopy revealed a fibrinous pleural area at the apex of my right lung and several nearby bullae. During a subsequent open thoracotomy, the bullae were oversewn and a partial pleurectomy was performed. The operation went well. A month later, I started medical school. Fortunately, I experienced no episodes of chest pain during my medical training. In fact, I did not suffer another pneumothorax for a decade. At the age of 32, during a floorball game in the hospital gymnasium, I felt a familiar and characteristic sudden chest pain and shortness of breath. This time, a chest radiograph did not confirm a pneumothorax. A computed tomography scan of my chest showed only pleural thickening on the right.

Today, as a physician patient, I recognize that I have experienced similar subclinical episodes throughout my life. Even though more than 14 years have elapsed since my last documented episode of severe chest pain, I remain very much aware of my condition. I am subconsciously cautious when lifting heavy objects or reaching for objects above my head. I am careful not to become fully exhausted when participating in sports. I think about it every time during take-off and landing when flying. I worry that my emphysema-like lung defects will recur or progress. I have several symptoms related to autoimmune or connective tissue disease and worry about associated anomalies or changes in other organs. Being also a father, I hope that my children will never experience my lung disease. A considerable number of articles have been published in the medical literature regarding treatment options for first and recurrent pneumothoraces. I am grateful for this attention, but am not consistently impressed with the quality of the research on my condition. To date, no other researchers have confirmed or further explored the striking association between bronchial anatomic anomalies and spontaneous pneumothorax reported by the investigators who included me as a study participant when I was young (1). As a patient and researcher, I would like to see greater emphasis on higher-quality studies to understand the pathogenesis and risk factors for spontaneous pneumothorax. I hope that large welldesigned trials will soon be conducted to develop scientifically sound strategies for preventing recurrent pneumothorax in susceptible individuals. n Author disclosures are available with the text of this article at www.atsjournals.org.

Acknowledgment: The author thanks Laszl ´ o´ Bense, M.D., Ph.D. for valuable input and support.

Reference 1 Bense L, Eklund G, Wiman LG. Bilateral bronchial anomaly: a pathogenetic factor in spontaneous pneumothorax. Am Rev Respir Dis 1992;146: 513–516.

On the Receiving End

443

Patient, physician, and researcher. My life's journey with recurrent spontaneous pneumothorax.

Patient, physician, and researcher. My life's journey with recurrent spontaneous pneumothorax. - PDF Download Free
466KB Sizes 0 Downloads 3 Views