The Disease of Chopin. A comprehensive study of a lifelong suffering - Victoria Wapf

The Disease of Chopin

A comprehensive study of a lifelong suffering

Victoria Wapf

Cover designer Julia Kravchenko

© Victoria Wapf, 2017

© Julia Kravchenko, cover design, 2017

Created with Ridero smart publishing system

Acknowledgement

This book wouldhave been impossible to complete without a great deal of help and support ofmany people from around the world. That is why I am glad to seize thisopportunity and express my gratitude to those who, in one way or another, helped me to reach the goal.

First of all I want to thank my family. They have not only granted me spare time to work on the manuscript, but also kept encouraging me along the way, nurturing my confidence and scientific curiosity.

Many thanks to Professor Tadeusz Dobosz, Head of the Molecular Techniques Unit of the Forensic Medicine Department at the Medical University of Wroclaw (Poland) for his precious comments and deep appreciation of my work.

Sincere gratitude  to Mr. Piotr Mysłakowski, publisher and researcher in the field of genealogy and biographical sciences, a renowned architect and designer for his valuable notes and corrections in regard of Chopins genealogy.

I am thankful to Dr. Lorenzo Käser from the Department of Graduate Education in Human Medicine, Zurich University  for providing an excellent learning and teaching environment and serving as a brilliant example of a physician, mentor and educator I always wanted to be.

A special thanks go to Prof. Wolfgang Jungraithmayr of the University Clinic of Thoracic Surgery in Zurich. A talented pianist and violinist, he infected mewith the idea to research the medical history of Frederic Chopin.

And, of course, I can not thank enough Julia and Maria, who assisted me with proofreading and graphicdesign, because without them all my efforts would be worthless. Both of themput every effort to create a book of a truly good quality.

I also thank my dear friends and colleagues Dr. Maria Bär, Dr. Iryna Cranny, Dr. Alexander Garmaev, Dr. Heiko Frühauf, Dr. Ursina Jansen, Dr. Yves Jaccard, Prof. Dmitry Kazakov, Ina Ladnar, Dr. Barbara Lütolf, Fahim Mozaffari, Nora H. Gräni, Dr. Elisabeth Hohenstein, Prof. S. Vavricka, Dr. Elisabeth Rushing, Bota Tynybekova, Philip Twaites, Dr. Matthias C. Walter, Cyril Wechsler, Pamela Wible, Priyatharsan Yoganathan und Nishanththan Yogarajan  who appreciate and support my eagerness to learn new things every day.

Abstract

Introduction

The life of the Polish-French pianist and composer Frédéric Chopin (18101849) was, to a great extent, influenced by his disease. Nevertheless, the diagnosis and differential diagnoses of his suffering remain a matter of debates in numerous biographical studies on the composers life. This study shall conduct a systematization and overview of Chopins medical history, in an effort to outline pathways to his most probable diagnosis.

Overview of methods

In this paper, the medical literature on Chopins disease was examined to weigh up the existing evidence in the light of todays medical knowledge and to define possible ways to extend such evidence with modern diagnostic methods. The literature search included three steps. First, a thorough search for available publications was conducted to identify and acquire all medical documents describing F. Chopins status and disease (s). The second step involved sifting of the reference lists in the articles retrieved from the first step. The third step was aimed at the retrieval of complementary documents of interest (for example, biographic, demographic or historical documents authored by specialists other than medical professionals) that may help to understand Chopins disease in its historical, cultural and social context.

Results

Based on the existing data, it was found that the diagnosis of tuberculosis still outweighs the cystic fibrosis version in Chopins case. However, various noxious factors during his life may have shaped the course and severity of the disease. Fully inadequate  by todays standards  and often potentially harmful treatment (including bloodletting, herbal remedies or other potentially toxic substances) as well as a prolonged exposure to tobacco smoke (passive smoking) could have played a crucial role in Chopins pathology, bringing the pianist and composer closer to his death.

Conclusions

The total body of evidence gathered from the current and historical literature allows for a strong presumption of tuberculosis as Chopins diagnosis. However, other primary diagnoses, comorbidities, as well as consequences of an iatrogenic exposure should still be regarded and may not be fully discounted.

An analysis of existing tissue specimens could yield additional valuable information and help to resolve the decades-long discussion about Chopins diagnosis.

List of abbreviations

AAT  alpha-1-antitrypsin

ABPA  allergic bronchopneumonic aspergillosis

CF  cystic fibrosis (mucoviscidosis)

CVID  common variable immunodeficiency

EGPA  eosinophilic granulomatosis with polyangiitis

GPS  anti-glomerular basement membrane disease (Goodpastures syndrome)

HHT  hereditary hemorrhagic telangiectasia

PAH  pulmonary arterial hypertension

PAVM  pulmonary arteriovenous malformations

PCD  primary ciliary dyskinesia

PH  pulmonary hemosiderosis

PID  primary immunodeficiency

SERPINA  serine proteinase inhibitor

GPA  granulomatous polyangiitis

Key words: Frederic Chopin; tuberculosis; cystic fibrosis; lung diseases; differential diagnosis.

Introduction

Both life and creative endeavors of Frédéric Chopin (18101849), a pianist and composer of French-Polish origin, were in many ways shaped by his disease. Chopins chronic condition still remains not clearly identified and evokes debates. Given the scarcity of medical-biographic data, Chopins symptoms provoked a number of thoughts and discussions both in the historical and medical community. However, despite the obvious interest in this topic, and a number of recent publications  especially around the recent bicentennial (2010) of the pianist/composer  there were no broad review of his possible and probable diagnosis in the recent time. The classic work, Chopins pathography by Edmond R. Long (1956)1 was focused on tuberculosis only. Later scholars explored further possible diagnoses, with cystic fibrosis (OShea, 1987)2 being the most known. Nevertheless, virtually all reports on the composers life and disease were centered on one or two possible pathologies only. In this comprehensive review, the medical and historical literature on Chopins disease and on the medicine of that era was examined to evaluate tuberculosis and cystic fibrosis, as well as other possible conditions for their significance in influencing the health of the innovative musician during his lifetime. It was found that though tuberculosis remains the usual suspect, several other hypotheses cannot be discounted either, especially if further exploratory methods may potentially become available. Chopins situation is unique, because in his case a tissue specimen could possibly shed further light on his disease, and, consequently, on our understanding of his life and works.

Moreover, the study of Frédéric Chopins medical history is an exciting opportunity to gain additional insights into the nineteenth century medicine, in the physician-patient relationships, and to see how the progress of medical science in the newer times has contributed to diagnostic and treatment of formerly fatal diseases. While working on this review, clear preference was given to the studies and interpretations of Chopins life and disease done by medical professionals  physicians, medical historians and pathologists. Other sources, such as personal communication of Chopin himself and his family and friends were used as well.

This study is divided into several chapters. It starts with a nearly forensic review of Chopins medical history, following a standard pattern of a patient examination. The primary purpose of this chapter is to provide as many clinically relevant data as possible in order to lay a foundation for diagnosis and differential diagnosis. The following two chapters contain information about Chopins physicians, their professional profiles, and modes of treatment. A profound understanding of Chopins disease history would only be possible with knowing on who treated him and which principles each treatment was based on. As it is often the case throughout the whole human and medical history, a treatment itself may become an aggravating factor or even the cause of the patients suffering. Chopin, as a patient with chronic disease (s), had an extensive list of medications, many of them with a potential for toxicity or interactions. That is why his treatment deserved a separate chapter here.

Following the examination of Chopins medical history is a discussion on the terminal period and post-mortem exploration of the composers disease. Chopins case is standing out because at least in theory, a further exploration with todays instrumental methods is technically possible thanks to the existence of the tissue specimen.

The next chapter is the most crucial one. It is focused on the diagnosis and differential diagnosis of Chopin. Beginning with an outline of two leading hypotheses for Chopins most suspected diagnoses, namely tuberculosis and cystic fibrosis, other, less known or less probable diagnoses and discuss possible comorbidities will be reviewed and described in detail. Finally, an attempt to lay out an examination plan with the twenty-first century methods of analysis will be undertaken in order to advance our understanding of Chopins disease.

1.1 Methods: detailed description

The literature search for this study involved three steps: the search criteria, the timeframe and what information to extract  all this was set prior to the start of the study. The first step of the literature search took place between October 16th and November 16th 2013 and had the following objectives:

 databases search and retrieval of relevant documents

 quality assessment of those documents

 weighing up the total volume of evidence for CF and TBS

 identification of knowledge gaps, i.e. subjects of search at the step III.

At this step, a search for available publications was conducted to identify all medical documents describing Chopins status and disease (s). The following search criteria were employed with strong preference for academic journal articles as sources:

 records by attending physicians  if any such exist;

 records by other medical and/or paramedical professionals (coroners, pathologists etc.)  if any;

 publications at the PubMed and other medical/scientific databases (Questia, Science Direct, MedlinePlus, Cochrane Library, CHBD (Circumpolar Health Bibliographic Database), the Directory of Open Access Journals, Web of Knowledge, Worldwide Science, and Index Copernicus);

 other relevant publications by medical professionals (such as biographic, demographic or historical documents);

The search languages were English, German, French, Polish, and Russian. Though valuable and interesting publications exist in such languages as Norwegian and Hebrew, they remain outside of the scope of this review. Since it would be obviously technically challenging to do a comprehensive search in a greater number of languages, this search was limited to those languages spoken/written by Chopin himself. Following words were used for the search in all databases: <Frederic Chopin disease>, <Fryderyk Chopin disease>, <Frederic Chopin Krankheit>, <Frédéric Chopin maladie>, <Fryderyk Chopin choroba>, <Фредерик Шопен болезнь>. Additionally, the PubMed database was searched for <Frederic Chopin> and for <Frédéric Chopin>. Following information was set to extract: symptom descriptions, treatments descriptions, dietary preferences, lifestyle details, quality assessment of studies. Inclusion criteria at the first step were: date of publication later than 1948 (the year when Chopins heart tissues were made available for research); a provision of justified (i.e. based on official archive documents or similar sources) hypotheses. The first steps exclusion criteria were lack of references, languages other than those ones listed above, wrong author (not a medical professional or a medical historian or a related official, for example a coroner). Hence, the standard set of reasons of rejection included wrong topic, wrong specialist, and wrong language.

The next (second) step involved sifting of the reference lists in the articles retrieved from the first step. The inclusion criterion at this step was a provision of justified (i.e. based on official archive documents or similar sources) hypotheses. At this step also publications by authors other than medical professionals were included (for example, biographic, demographic or historical documents authored by specialists or family members). The step II exclusion criteria were a lack of references and wrong languages (i.e. other than those listed above).

Finally, the last step of search was aimed at a retrieval of additional documents of interest that may not fit into the step I and II criteria, but may help to understand Chopins disease in its historical, cultural and social context. A hand search was attempted for the genealogical data and for original publications by Chopins attending physicians.

The first step of the literature search (between October 16th and November 16th 2013) was aimed at identifying of all medical documents describing Chopins status and disease (s). Six publications were found at PubMed3. Sixteen publications were found with additional key words at PubMed: Frederic Chopin4. With a related citations option: 152 publications were found in a PubMed search5. Both PubMed and Go PubMed searches yielded the same results as the PubMed search. A Cochrane library search brought no results, as well as the CHBD (Circumpolar Health Bibliographic Database, the Directory of Open Access Journals, Worldwide Science, Index Copernicus (this database includes over 700 journals from Poland), and the Medline Plus. A search in the Questia database yielded twenty-two books and two academic journal articles6. A search at the Web of Knowledge: twelve results7. Sixty-two more results were found at the Science Direct database.