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4. Haemophilia And Royal Families


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4. Haemophilia And Royal Families

  1. 1. Haemophilia and the royal families of Europe
  2. 2. What is haemophilia  It is an X-linked genetic disease, hence the genetic abnormality is found on the X chromosome.  There are two types heamophilia A and B.Haemophilia A has a deficit in factor viii and B in factor ix  It usually presents in infancy with recurrent bleeds, ,painful haemarthoses and haematomas and intracranial hemorrhages  Bleeding episodes are treated with Factor viii or ix replacement. DDAVP is also used
  3. 3. Inheritance  The Earliest written recognition of hemophilia was in 2nd century AD in Jewish writings  Females are usually carriers, Since they have a chromosomal make-up of XX, inheriting an X chromosome with disease mutations will generally not produce the disease condition as the other normal X chromosome can compensate.  Hemophilia affects males much more frequently (1 in 10,000) than females (1 in 100,000,000).  Spontaneous mutation can occur . In 33% of patients there is no family history of haemophilia
  4. 4. “The royal disease” haemophilia became known as the “Royal disease” because it spread to the royal families of Europe through Victoria’s descendants  Haemophilia appeared in Queen Victoria’s children, and through them spread into the royal families of Europe
  5. 5. Queen Victoria family tree
  6. 6.  Queen Victoria had 9 children. One hemophilic son and two daughters that were carriers  Queen Victoria had no ancestors with the condition but soon after the birth of her eighth child, Leopold, in 1853 it became evident that he had haemophilia (the son Leopold died at the age of 31, from a intra cerebral haemorrhage and was described as “very delicate”)  With the appearance of hemophilia in the royal family the Queen could only protest that the disease did not originate in her side of the family.and they often blamed the “curse of the Coburgs”.
  7. 7. How did the gene get introduced into the royal family  There were several suggestions how the haemophilic gene entered the royal family  1 .spontaneous mutation  2.and the most controversial : that queen Victoria wasn’t the Duke of Kent’s daughter.  The most accepted theory is that of spontaneous mutation ,as 30% of cases represents with new mutations, it is more likely that Victoria's carrier state was due to a new germ line mutation in her parents or grandparents  The haemophilic gene has now died out in these Royal families, Thus we do not know to this day if the condition was haemophilia A or B
  8. 8. Affecting European royalty  Queen Victoria was known as a successful dynasty as her children and grandchildren married widely among the powerful ruling families of Europe.  Hence the haemophilic gene spread through the royal houses of Europe as monarchs arranged marriages to consolidate political alliances . Hence the Russian , Spanish and German royal families were affected. ( as two of the daughters of Queen Victoria were carriers)
  9. 9. The Russian connection  Alix of Hesse was one of Victoria’s grand daughters and married Tsar Nicholas II of Russia, becoming Tsarina Alexandra.  she was a carrier and her fifth child and only son, Alexi, was born in 1904. He was a haemophiliac  Alexi’s hemophilia discovered shortly after birth because of excessive bleeding from his umbilicus
  10. 10. Rasputin  Rasputin got involved with the Romanovs when Alexei got a bruise after falling off a horse and the tsarina was looking everywhere for help. And Rasputin was suggested by her best friend as he was known to be a “charismatic peasant healer”.  He seemed to be the only one able to soothe Alexei  It is still unsure how Rasputin was able to soothe the child. It is believed that he hypnotised the boy.  Rasputin was a very unpopular and controversial figure, leading a scandalous personal life. His connection with the Romanovs stained the royal family  Rasputin was given much authority by Alexandra because of his “healing powers”, and he was thus able to influence politics and decision makingHe soon became her making confidant and personal advisor. He also convinced her to fill some government offices with his own handpicked candidates  This reliance enhanced Rasputin's political power, which seriously undermined the Romanov rule during the First World War.
  11. 11. The revolution  At this point Russia was in turmoil  Alexandra was unpopular in Russia during the first world war, as her connection with the German royalty and she was thought to be a spy.  The Russian Revolution began in 1914. Tsar Nicholas II was not a strong leader, and his son’s illness only added to his inability to rule his country by making the family more sheltered and out of touch with the people  Nicholas II abdicated in 1917  The family was executed on July 17, 1918.  Many historians attribute much of the Tsar’s fall to his son’s illness and his inability to take more effective steps and there is little doubt of the influence of Rasputin.
  12. 12. The German connection  Queen Victoria’s grand-daughter Irene of Hesse, Princess of Prussia introduced the gene into the German imperial families  She had three sons, two of whom were haemophiliacs
  13. 13. Spanish royal family  Princess Victoria Eugenie Battenberg of England , Queen Victoria's grand daughter married King Alfonso XIII of Spain  She gave birth to 7 children , 2 haemophilic sons and only one healthy son.
  14. 14.  2 sons had haemophilia, one son was stillborn, the second eldest son was death and mute  This news of haemophilia damaged Victoria Eugenia's relation with her husband. It also sent the King into a tailspin of irresponsible behaviour that led him to abandon the affairs of daily government to questionable characters and dictators .  this also weakened the position of monarchy in Spain and discredited the heirs and may have lead to the civil war.  It is impossible to measure exactly the effect of these tragedies on the Spanish throne.The Spanish society was so deeply divided and flawed that civil war might have been unavoidable, but undoubtedly helped to weaken the position of the throne.