Читать книгу The Disease of Chopin. a comprehensive study of a lifelong suffering - Victoria Wapf - Страница 8

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 29^th 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 14^th 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 10^th 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.