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Why do we study Queen Victoria in Bio class? A Case Study in Hemophilia

If someone mentions ‘the Queen’ to you, they are probably referring to Queen Elizabeth II. She has been ‘the Queen’ longer than any previous British monarch. She set that record on September 9, 2015 when she surpassed the previous record set by her great-great grandmother Queen Victoria. During the last half of the 19th century, Queen Victoria was ‘the Queen’ who defined the times.

With the historical drama, Victoria, now being broadcast on Masterpiece PBS there is renewed interest in learning the history associated with her. Queen Victoria has often been the subject of movies and television drama. Biology students don’t need to wait for a movie or a special series to become acquainted with Queen Victoria. Indeed, students are exposed to Queen Victoria when they learn about sex-linked inheritance. Many biology texts have pedigrees analysis charts showing Queen Victoria and her descendants demonstrating the inheritance pattern of (a form of) hemophilia, a sex-linked disease.

                       

The story all starts on April 7, 1853, when Queen Victoria had her 8th child and fourth son, Prince Leopold, Duke of Albany. As a young child, he seemed to be weak, he bruised and bled easily. Could this have been a result of Queen Victoria trying the new anesthetic chloroform during childbirth? Not much was known about hemophilia in the mid-1800s. At this point, we were still 13 years from the publication of Mendel’s work and more than 50 years from understanding sex-linked genetics. While the royal family may have kept Leopold’s condition somewhat quiet, he was attended to by William Jenner, who was well known as the scientist who differentiated typhus from typhoid fever. Probably because of Leopold’s position in society, there was increased interest and research into hemophilia. John Wickham Legg, a student of Jenner, became both Leopold’s personal physician and the author of A Treatise on Haemophilia (1872). In his publication, Legg speculated that hemophilia may be a genetic disease. He even went so far as to warn that:

“Should a bleeder, or one of a bleeder family, be allowed to marry? I think that if the person himself be a bleeder, the question of marriage ought not to be entertained. His sons may possibly escape the disease, but it is almost sure to reappear in his daughters’ sons. The prospect of the certainty of so dreadful an entail of disease must repel every right thinking person from such a step, even at so great a sacrifice to himself; and it seems only necessary for the facts to be known to prevent such marriages among the better classes”

Queen Victoria was quite insistent that her children marry into other European royal families. Through this, hemophilia was distributed through the royal families of Germany, Russia and Spain. Ultimately, she lost a son, two grandsons and five great-grandsons to hemophilia. Perhaps thanks to all the attention that came from Queen Victoria’s mutation, and modern research into the disease, hemophilia is not the fatal disease it once was. Hemophilia is treatable through injections of blood proteins. Research continues, and perhaps hemophilia will be one of the first diseases to be cured through gene therapy. Some promising results have already been published.

Links of Interest:
    Treatise on Haemophilia - John Wickham Legg
    Masterpiece PBS - Victoria
    Timeline of Bleeding Disorders

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Why do we study Queen Victoria in Bio class? A Case Study in Hemophilia

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