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The Disease of Chopin. A comprehensive study of a lifelong suffering
The Disease of Chopin. A comprehensive study of a lifelong suffering

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The Disease of Chopin. A comprehensive study of a lifelong suffering

Язык: Английский
Год издания: 2016
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The November 1838 trip to Mallorca together with George Sand and her children became a disaster. This journey was described by Sand in a book “Un hiver à Majorque” (A Winter in Mallorca). According to her, Chopin had all signs of pneumonia and the island inhabitants grew suspicious and distant to the couple, as they believed that Chopin has had a contagious disease. The fact that Sand and Chopin were not married did not help their popularity among Mallorcans either. That is why it was all but impossible to obtain a better dwelling than the one where Chopin and Sand have stayed. Both two-story chambers they occupied at the monastery Sa Cartoixa de Jesús Natzarè in Valldemossa were barely heated and damp. Sand wrote that Chopin coughed up sputum “by the bowlful”. That winter Chopin was seen by at least three Mallorcan doctors. As George Sand recollected, the recommended treatment included application of plasters at various intervals during the day and consumption of milk. However, no milk was available there38 – likely due to the resentment of local inhabitants who did not want to have a potentially contagious patient to be around. In addition of all those hardships and his health continuing to deteriorate, Chopin’s piano has got stuck with the Spanish customs, so he did not compose much till the end of December 1838: “Meanwhile my manuscripts are sleeping whereas I cannot sleep”39

But already by mid-January 1839, Chopin sends the Preludes op. 28 to Fontana with an instruction to pass them to Mr. Pleyel, a music publisher40. A very productive phase followed and Chopin wrote, corrected or finalized during his stay on Mallorca following works, to name a few: Polonaise in C-minor, op. 40, Mazurka in E-minor, op. 41 no. 2, Scherzo in C-sharp minor, op. 39 (drafts), Nocturne in G-minor, op. 37, no., Tarantella op. 43 (draft), and Sonata in B flat minor, op. 3541. Most of those pieces were done by Chopin which has not yet fully recovered from the disease on “a poor Mallorcan piano…”42

Later in 1838 he experienced pulmonary problems again43. After three months on the island, Chopin and Sand with her children left Mallorca on the 13th of February 1839. On their way back to Paris, in Marcel, Chopin recovered, at least for a while. His strength was back, there were no more hemoptysis and he started to gain weight again44. Nevertheless the very same author, referring to G. Sand, says that for the next few years, Chopin continued to have cough bouts with dyspnea and intermittent fevers. Sand’s recollections show that he was then never absolutely well and experienced a slow progressive decline in his health.

Chopin was successfully treated with ‘oats and honey’ and often “courses of belladonna. which he continued to take through most of his life”45. Belladonna has centuries-long history of use as a medicine46, however it is not clear who, when, and for what purpose exactly prescribed or recommended it to the pianist. Kuzemko (1994) argues that a diet may have had a positive influence on the course of Chopin’s disease, citing the composer’s letter to his parents from Berlin, written on September, 27, 182847: “I am quite well… as long as I avoid meats, sauces, soups”48. However, this very same letter is quoted differently by another source (text in bald – VW):

I am quite well, and have seen all that [was] to be seen. I shall soon be with you again. In a week, from the day after tomorrow, we shall embrace […] I count among the great events of my visit here the second dinner with the scientists, which took place the day before the conclusion of the Congress, and was really very lively and entertaining. Several very fair convivial songs were sung, in which all the company joined more or less heartily. Zelter conducted, and a large golden cup, standing on a red pedestal, in front of him, as a sign of his exalted musical merits, appeared to give him much satisfaction. The dishes were better that day than usual, they say, “because the scientists have been principally occupied during their sittings with the improvement of meats, sauces, soups, etc…49

It is beyond of scope of this study to assess the biographic precision of both sources and to define which translation conveys a better sense of Chopin’s letters (they were originally written in French). Both sources do not specify whether that “quite well” description referred to Chopin’s digestive or respiratory issues. However, according to the authors in favor of the cystic fibrosis version, those gastrointestinal symptoms were not new for Chopin. Yet during his early adolescence he developed intolerance to ‘fatty foods’ which resulted in recurrent prolonged episodes of diarrhea and weight loss50.

The slow decline of Chopin’s health continued for the next four years until (in winter 1843/44) he become severely cachectic and so weak, that he could no longer go upstairs by himself and had to be carried up51. 22 June 1849 Chopin had two episodes of hemoptysis in one night52 (according to Chopin’s letter to his friend Grzymala, as cited by Ganche, p.78). At that point he had less than four months to live.

1.4 Family anamnesis

Some scholars, especially those in favor of the cystic fibrosis hypothesis find the genetic disposition of the Chopin’s family problematic53. Both his father and two of his sisters – Emilia and Ludwika – had reportedly had pulmonary symptoms. Other authors view the family health as quite robust. For example, Myslakowski (2010) underscores the fact that Chopin’s mother, father and one sister (Izabella) have lived well into their 70s, which is about 30 years more than the average life expectancy at that time54. This view of Chopin’s family is echoed by Neumayr (2007) who suggests that the Chopins enjoyed a good health and fully dismisses a possibility of a genetic disorder55, without addressing, for instance, an existing possibility of Chopin being a “mosaic” gene defect carrier with variable phenotypic expressions56. In fact, it is difficult now to draw a valid conclusion about the true health status of Chopin’s immediate family, and more so for the distant relatives. While an extensive, tedious research work was done for the paternal side of Chopin’s family shortly after the World War II, little, if anything, is known about his maternal ancestors57. Many documents that could have helped an evaluation of genetic patterns in Chopin’s family are either lost – such as birth and death records – or intentionally destroyed (for example, personal letters) or, most likely, never existed (detailed medical histories).


Also the life expectancy might not be that informative as an indicator of health, because by definition it is an arithmetic mean. An age-specific death rate or median life duration of the population could be more helpful for such evaluation. Indeed, according to the data of the National French Institute of Demographic Studies, the average life expectancy in France in 1810 – the year Chopin was born – was thirty-seven58. That figure was already a part of an increasing trend – the life expectancy at the time when Chopin’s parents were born, was even lower – twenty-five to thirty years. Based on those figures alone, one may arrive to a conclusion that even Chopin himself has lived quite a long life – that is, almost ten percent longer than an average French citizen! Of course, such conclusion would be certainly misleading. Two factors influenced the life expectancy in the early 1800s greatly: the neonatal mortality and Napoleonic wars. The wars took their toll, claiming many lives of younger men, thus decreasing the average life expectancy. At the same time, many children died at an early age, but as soon the child lived up to the age of ten; his or her chances to reach a very advanced age were improved greatly. Actually, as soon as a child survived to the age of twenty, his/her life expectancy was nearly equal to the average life expectancy in the twentieth century.


Hence the presumption that the Chopins were such a healthy kin, that their life duration was double of that of an average European of their time – may be wrong. While several members of the family lived well into their seventies, the others died at an early age (for example, one or more siblings of Chopin’s mother see Appendix). Those early childhood deaths might well be attributable to innate pathological processes, including genetic defects, but at least equally well – to a lack of basic hygiene or insufficient health care in the 19th century Europe.


Parents

Nicolas Chopin (Mikolay Chopyn, Nicolai Choppe) was Frederic Chopin’s father. French by origin, he was born on April 15, 1771 in Maraiville-sur-Madon. He died on the 3rd of May, 1844 in Warsaw. His parents were Francois Chopin and Marguerite Delfin59. A pipe smoker60, he was prone to develop infrequent respiratory tract infections and became very ill on at least two documented occasions61. Nicholas Chopin’s presumable cause of death was lung disease (allegedly tuberculosis) at the age of 7362. He was buried in the catacombs of the Powązki Cemetery on May 6, 1844. In 1948, after the catacombs’ destruction, Nicolas’ and his wife Justyna’s coffins was transferred to a new grave at the back of the church of St. Charles Borromeo, where they remain to this day. A thorough anthropological examination was undertaken on the occasion of exhumation, which may allow establishing Nicolas and Justyna’s appearance.


Tekla Justyna Krzyzanowska (Justyna de Krzyżanowskie, as in F. Chopin’s baptismal record), Frederic Chopin’s mother was of Polish origin. Her exact date of birth is unknown, but it must have occurred shortly before September 14, 1782, the date she was christened at the parish church of Izbica, receiving the Christian names of Tekla Justyna, She died on October 1, 186163. Throughout her whole life, Chopin’s mother reportedly remained in good health64. Tekla Justyna’s parents were Jakub Krzyżanowski (ca. 1729—1805) and Antonina Kołomińska; both most likely came from the noble class. Justyna was born at least ten years after her parents were married and had at least two elder siblings: brother Wincenty, born in 1775, who died in infancy, and his sister Marianna, born in 1780. Some unverified sources (amateur genealogy forums) mention other siblings of Tekla Justyna, who died as young children, but no documented evidence was found in this respect.


Jakub Krzyżanowski, the maternal grandfather of Chopin was most notably acting as a manager of the royal estate. As the archive records show, Jakub was repeatedly involved into quarrels and legal issues over property and money. The family has also frequently changed their place of residence, most likely due to “mixed relations to their employers”. Perhaps this could possibly be seen as a sign of an instable mental state, maybe even a disorder. It is difficult to draw a conclusion now, when no direct evidence may exist. Jakub died in Świętosławice on the 29th of October 1805 at the age of 76. The cause of death was “dropsy”, a major death factor in those times. In modern terms a dropsy (a hydropsy) is a generalized edema, most notably related to a right heart failure. Mercury was frequently prescribed to treat dropsy at the time of Jakub’s death – due to its diuretic effects. As a toxic substance, mercury may have widely contributed to lethality, too65.


Siblings

The older sister, Louise Chopin or Ludwika Marianna Jędrzejewiczowa (April 6, 1807 – October 29, 1855) suffered from recurrent chest infections and died from a respiratory illness at the age of 47 years66. She was outlived by her four children. Chopin’s second sister, Isabelle Chopin or Justyna Izabella [битая ссылка] Barcińska (born on the 9th of July, 1811, died on the 3rd of June, 1881) had reportedly good health, “led an uneventful life and died at 70 years”67. Her marriage to Anthony Barcinski remained childless68.


The youngest sister, Emily Chopin (aka Emilia Chopin) lived only fourteen and half years long (1812 – 1827). She was described as a frail child; from the early age her health was a subject of concern. She was underweight and suffered from periodic bouts of cough, breathlessness and ‘asthma’ (episodes of wheeziness). With regards to Emilia’s symptoms, which became especially severe when she was about eleven years old, the biographers opinions vary. Some researchers suggest that she started to have hematemesis and consequently died from a massive gastrointestinal hemorrhage, most likely from portal hypertension due to cirrhosis or severe gastric erosion69. Yet other biographers consider her symptoms mostly pulmonary, noting Emilia’s frequent respiratory infections and syncopes. They refer to Emilia’s blood spitting as hemoptysis, not hematemesis, and suggest that she had pneumonia in her terminal phase:

“From her early childhood Emilia’s health was a matter of growing concern. Early symptoms of an illness (probably tuberculosis) caused a general weakness of the organism… Despite medical attempts (whose efficiency has been questioned and even accused of having speeded up her death) Emilia’s illness quickly developed and she spent her last months coughing with blood and often losing her senses”.70

In a letter to a friend as of March 14th 1827, Chopin describes his sister’s sufferings that lasted already four weeks. He also describes Emilia’s anorexia and the treatment she received:

«…the bloodletting, which was done once, twice, innumerable leeches, vesicle-producing plasters, mustard plasters, and herbs, adventures over adventures. During this whole period of time, she did not eat and was so run down that one could hardly recognize her, and only slowly did she somewhat recuperate.”71,72

Emilia died less than a month later, on the 10th of April, 1827. There is no sufficient data to say with confidence whether Emilia’s death occurred on the grounds of a pathological process in her lungs with or without portal hypertension or due to a gastrointestinal disease. Depending on the initial pathology, it is possible that her death was caused by cachexia and anemia, both probably exacerbated by the wrong treatment Though chronic iron deficiency may rarely lead to death directly, a severe (or even moderate) anemia can cause sufficient hypoxia to aggravate underlying comorbidities (in Emilia’s case pulmonary and, probably gastrointestinal disorders)73 and become lethal this way.

Speaking of an exacerbating treatment, bloodletting (or bleeding) was widely practiced at that time and, according to Frederic’s accounts, Emilia Chopin underwent this treatment, too. It is important to note that the regular amount of blood extracted on each occasion was substantial: 600 – 1 000 ml. The treatment regime varied and Emilia might lose close to 2 800 ml of blood in three days, or 3 000 ml in 4 days. As much as 6 200 ml of blood could be let over a six day period74. In total, Emilia was losing blood – both as a result of her disease and her treatment – at least eight week long. Taking into consideration that patients at that time were regularly bled to syncope75 and both her nutritional status and food/liquids intake were absolutely inadequate, the cause of death could well be a posthemorrhagic anemia, and not an underlying pulmonary or gastrointestinal disease. With Emilia’s prolonged history of treatment with bleedings, an iatrogenic infection, such as hepatitis B virus (HBV), remains a possibility, too. Such infection may have affected the hepatocellular function, interfering with production of thrombopoetin. The resulting thrombocytopenia could additionally contribute to the Emilia’s hemorrhages, and, finally, to her death.

1.5 Social history

Living arrangements

Chopin’s living arrangements varied greatly. Reportedly, he had never owned a house himself and lived in rented accommodations, at times sharing them with friends. It is likely that many of his dwellings have helped to an exacerbation of Chopin’s pulmonary symptoms and progression of disease:

– Fireplaces and cooking stoves – are all known sources of irritants of the upper airways

– Cold damp dwellings (for example, the one on Mallorca).

– Mold and fungae are regularly present in damp settings and may have contributed to Chopin’s cough bouts, too (Szpilczynski) discussed Chopin’s allergic predisposition in 196176).


Marital status / Children

While known for having had numerous sexual relationships – some of them lasted for years – Chopin was never married and no biological children are known.


Drug use

Though the composer did not like wine, he occasionally got drunk, likely on social grounds or as an effort of self-medication for his bouts of melancholy77. Chopin detested tobacco smoke which made him cough. However, chronic passive smoking was an important factor influencing his lung disease. Throughout the most of his life he was surrounded by many cigar and pipe smokers such as George Sand, Liszt and his father Nicolas, to name a few78.. Fair to note, the adverse effects of tobacco smoking on health were not widely known at that time. A century after Chopin’s time, in the 1920s, a German internist F. Lickint has published the results of his scientific investigations of health issues related to alcohol and tobacco, describing lung cancer and stomach ulcer associated with prolonged smoking79..


As brief Chopin’s recreational drugs list is, as ample was his usage of medications. He frequently took opiates80. One such remedy, laudanum, is known to contain [битая ссылка] morphine, codeine, [битая ссылка] morphinan, thebaine, papaverine, and noscapine (narcotine). Laudanum is a [битая ссылка] tincture of [битая ссылка] opium containing approximately 10% powdered opium [битая ссылка] by weight (the equivalent of 1% [битая ссылка] morphine). In the nineteenth century laudanum was widely used “against many ailments”81. Opium was used not only as laudanum tincture and not only as a single medication. It was a part of numerous prescriptions and well as home remedies. It was mixed with virtually anything available: sugar, alcohol, mercury, hashish, cayenne pepper, ether, chloroform, belladonna and so on82. Chopin’s attending physicians have most likely recommended laudanum – and not another tincture – not only due to analgesic and antitussive effects of this medication, or to control Chopin’s frequent diarrhea, or to alleviate his sleep problems, but also because laudanum was a strong emetic. Emetics were popular, (as one can see on the example of Emilia’s treatment) since medieval times they were deemed important as “body cleansers”83. The emetic treatment is further discussed in Chapter. As Chopin had a pre-existing pulmonary condition, the use of opium tincture was especially dangerous due to the risk of a respiratory depression, even at therapeutic doses84. Opium is also known for releasing histamine from skin and muscle. Histamine, in turn, plays a primary role in the respiratory system as a bronchoconstrictor85.


The composer’s dependence on opium86 may explain its frequent use. A number of Chopin’s symptoms can be found on the vast list of opium side effects: drowsiness, headache, malaise, CNS depression, insomnia, mental depression, nausea, vomiting, anorexia, stomach cramps, neuromuscular and skeletal weakness.

Ganche (1935) lists other medications and remedies prescribed to Chopin: foxglove (digitalis), monkshood (aconite), creosote, protioduret , jelly lichen (collema), gum water (diluted acacia sap that was a popular prescription for phthisis87). Most of those remedies are known for their toxicity, but little is known on how Chopin’s treatment may have influenced his symptoms. Caruncho and Fernandez (2011) discussed opium toxicity as a possible reason for Chopin’s mental problems, only to dismiss it on the grounds that the symptoms (particularly hallucinations) were present before Chopin began receiving the treatment on a regular basis and that the hallucination pattern differed from that of toxicity. The authors point out that the exact treatment chronology remains unknown. Chances are, the symptoms’ onset might, in fact, have preceded or coincided with Chopin’s treatment. Certainly, a simultaneous occurrence does not equal to causality, but it is possible that at least some of Chopin’s pathology might be explained with toxic effects of the remedies he was treated with88.


Foreign travel

At various periods of his life Chopin has visited geographic areas that today are known as Germany, Poland, France, Austria, United Kingdom, and Spain89. Following diseases were present in those parts of Europe in the 19th century and/or caused outbreaks of a significant importance:

– typhus

– scarlatina

– measles

– smallpox

– cholera

– smallpox

– tuberculosis

– influenza

However, the course of Chopin’s disease does not match the spread and the magnitude of those epidemics. For instance, neither his gastrointestinal symptoms nor his pulmonary problems were aggravated or first present at the times of cholera or influenza outbreaks, respectively.


Exposure to environmental pathogens

With an exception of a regular exposure to biomass (wood) home heating and passive smoking (as many of Chopin’s friends and family were heavy smokers), no particular environmental exposures are known.

1.6 Review of systems

According to ample verbal and visual evidence Chopin had asthenic habitus. In his travel passport, used for the journey to England in 1837, his height is stated as 170 cm90, weight – 50 kg91 at some points – in 1835, and, probably, in 1838, too – dropping below 45 kg92. Both parameters substantially deviate from population averages (see Appendix). Chopin’s body mass index (BMI) varied between 16 and 17, which is considered underweight (normal range BMI ranges between 18.5 and 24.9). A number of sources depict Chopin’s poor exercise tolerance and failure to gain weight93.


Musculoskeletal system

What reliable evidence might help to assess Chopin’s appearance from a clinical viewpoint? Obviously, various verbal and graphic depictions are always to some extent subjective and may sometimes be rather a telltale of their creator, not so their object. Various portraits, inclusive photographs are consistent in portraying of asthenic, thin-faced man. The written sources convey an image of a man who is ‘whiskerless, beardless, fair of hair, and pale and thin of face … a prominent aquiline nose94. But it is barely possible to draw a clinically relevant conclusion based on something as trivial as a caricature95. For example, based on a sketch by P. Vairdot, Kuzemko (1994) suggests that Chopin has probably had emphysema, since he become apparently barrel-chested in his early thirties. However, that very sketch – as fairly pointed out by other researchers – shows Chopin with a disproportionally giant head, too.

The other authors describe Chopin as having “thin, long and barely muscular limbs, very slender, delicate hands96. Those extraordinary thin limbs might probably be interpreted as an early sign of emaciation97. Almost all observers noted the extreme thinness of his limbs. Here is one fact that let us think that Chopin may, indeed, have had a distorted musculoskeletal development that goes beyond a mere asthenic habitus. While travelling in horse-drawn carriages, Chopin feared he may fracture his frail limbs98. Both Erlinger (2010) and O’Shea (1987) hypothesize that this could be due to his pulmonary hypertrophic osteoarthropathy, that manifested itself by painful swelling of distal joints and soft tissue99,100. Quite evidently (see Appendix 10.7,“Postmortem hand cast”), Chopin did not have digital clubbing (finger clubbing). Though finger clubbing is most commonly seen in patients with bronchiectasis (as well as in those with cystic fibrosis and bronchial carcinoma) and not commonly seen in patients with pulmonary tuberculosis101, this sign is neither specific nor particularly sensitive for lung pathology102 and cannot be reliably used for a differential diagnosis in Chopin’s case.

Throughout most of his adult life, Chopin frequently suffered of pain in the ankles, feet and hands103. During the terminal phase of his illness, he also developed severe pain in his wrists and ankles, which was relieved partially by massage and sometimes worsened at cold and wet weather. The hot weather was also poorly tolerated: according to O’Shea (1987), Chopin had frequently experienced prostration and hyperhidrosis in summer104. As mentioned above, at least once in his life – namely in winter of 1826 – Chopin had nodal swelling that Kubba and Young, referring to Chopin’s letter dated February 12, 1826 to his friend, physician Jan Bialoblocki105, regarded as a cervical lymphadenopathy. Cervical lymphadenitis is a common (about 15%) manifestation of extrapulmonary tuberculosis, especially in patients with compromised immune system106. A nodal regression is possible indeed, but only under chemotherapy107. Other infections or neoplasia, and rarely, drug reactions may also cause a nodal enlargement that in some cases can resolve untreated108.

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