Aronova-Tiuntseva's Hemophili The Royal Disease
NATIONAL CENTER FOR CASE STUDY TEACHING IN SCIENCE
Hemophilia:
“The Royal Disease” by Yelena Aronova-Tiuntseva and Clyde Freeman Herreid
University at Buffalo
State University of New York
Hemophilia is an X-linked recessive disorder characterized by the inability to properly form blood clots. Until recently, hemophilia was untreatable, and only a few hemophiliacs survived to reproductive age because any small cut or internal hemorrhaging after even a minor bruise were fatal. Now hemophilia is treated with blood transfusions and infusions of a blood derived substance known as antihemophilic factor. However, such treatment is very expensive and involves the risk of contracting AIDS.
Hemophilia affects males much more frequently (1 in 10,000) than females (1 in …show more content…
An early death is likely. Females, on the other hand, carry two
X chromosomes. If only one is defective, the other normal X chromosome can compensate. The woman will have normal blood clotting; she will simply be a carrier of the recessive defective gene. This fact will be discovered if some of her children are hemophiliacs. Naturally, women hemophiliacs are rare because it takes two defective X chromosomes in order for the condition to be seen.
Hemophilia has played an important role in Europe’s history, for it suddenly cropped up in the children of Great
Britain’s Queen Victoria. It became known as the “Royal disease” because it spread to the royal families of Europe through Victoria’s descendants. Queen Victoria had always been worried about the quality of the blood of the British royal family. Her feelings about the necessity of revitalizing what she called the “lymphatic” blood of their houses are reflected in her letter to her daughter Vicky: “I do wish one could find some more black eyed Princes and Princesses for our children! I can’t help thinking what dear Papa said—that it was in fact when there was some little
Hemophilia:
“The Royal Disease” by Yelena Aronova-Tiuntseva and Clyde Freeman Herreid
University at Buffalo
State University of New York
Hemophilia is an X-linked recessive disorder characterized by the inability to properly form blood clots. Until recently, hemophilia was untreatable, and only a few hemophiliacs survived to reproductive age because any small cut or internal hemorrhaging after even a minor bruise were fatal. Now hemophilia is treated with blood transfusions and infusions of a blood derived substance known as antihemophilic factor. However, such treatment is very expensive and involves the risk of contracting AIDS.
Hemophilia affects males much more frequently (1 in 10,000) than females (1 in …show more content…
An early death is likely. Females, on the other hand, carry two
X chromosomes. If only one is defective, the other normal X chromosome can compensate. The woman will have normal blood clotting; she will simply be a carrier of the recessive defective gene. This fact will be discovered if some of her children are hemophiliacs. Naturally, women hemophiliacs are rare because it takes two defective X chromosomes in order for the condition to be seen.
Hemophilia has played an important role in Europe’s history, for it suddenly cropped up in the children of Great
Britain’s Queen Victoria. It became known as the “Royal disease” because it spread to the royal families of Europe through Victoria’s descendants. Queen Victoria had always been worried about the quality of the blood of the British royal family. Her feelings about the necessity of revitalizing what she called the “lymphatic” blood of their houses are reflected in her letter to her daughter Vicky: “I do wish one could find some more black eyed Princes and Princesses for our children! I can’t help thinking what dear Papa said—that it was in fact when there was some little