Hemophilia and the Royal family

                   Exploration Academy

                           Jaleah C. Price

Photo from Rebecca English For The Daily Mail

                What is Hemophilia?

Hemophilia is a rare X-linked recessive defective gene, in which the body is not able to control blood clotting. It is usually passed from parents to children through genetics. Having hemophilia means a hemophiliac person bleeds longer after an injury than others normally do. They can also bleed internally, causing damage to organs, tissues, which can be life threatening. Hemophiliacs are usually males. 1 in 10,000 males have the disorder and 1 in 100,000,000 females have the disorder. People with hemophilia either have little or no protein called clotting factor to control blood clotting. Small blood cell fragments found in bone marrow, are platelets. Platelets and clotting factors work together to plug injuries on vessels and stop bleeding Clotting factors help the platelets stick together to do so.

There are two types of hemophilia:

Hemophilia A - A hemophiliac who is missing or is low in clotting factor VIII has hemophilia a. 80 percent of people who have hemophilia have hemophilia a.

Hemophilia B - A hemophiliac who does not have or is low in clotting factor IX has hemophilia b.

                    Cause of Hemophilia

Photo from the Centers for Disease Control and Prevention

Hemophilia is caused by a defect in the blood clotting gene, clotting factor VIII or IX, found in X chromosomes. Females (XX) inherit both their X chromosomes from their parents and males (XY) inherit their X chromosome from their mother and their Y chromosome from their father, therefore sons will inherit the disorder from their mother. Clotting factors are only found on X chromosomes. Males with hemophilia have a defective hemophilia gene in their X chromosome. Because males only have one X chromosome, if they do have a defective X chromosome, they right away have the disorder. Females with hemophilia must have two defective X chromosomes. Females with only one defective X chromosome are hemophilia carries and can compensate.

          Treatment of Hemophilia

Photo from Koate DVI

The most common treatment for hemophilia is replacement therapy. Replacement therapy is a concentrate, made of human blood, with clotting factor VIII or IX. It is either injected or slowly dripped into an injured vein. This therapy helps replace missing or low clotting factors. Because this treatment is made of donated human blood there is a risk of getting an infectious disease, but the risk is low. Another treatment is recombinant clotting factors, that is not made from human blood. This treatment can easily be made at home. Next there is a treatment called Preventive therapy that has to be used regularly to prevent bleeding. Finally, there is demand therapy that is used to stop bleeding when it happens. It is a as-needed treatment. There is also Desmopressin, antifibrinolytic medicines and gene therapy treatments.

              Types of Government

Democracy - A system of government where political authority is run by the people, and majority wins. There is also belief in individual liberty and equal rights for all people.

Dictatorship - A system of government in which one person or group is the ruler and has absolute power.

Monarchy - A system of government in which head of state is a royal figure.

Oligarchy - A government where it is controlled by a small group designed to serve their own purpose.

Republic - A country governed by elected representatives and elected leaders, such as a president.

Theocracy - A form of government ruled by religious leaders.

Democratic Republic - A government that is both republic and democracy. Authoritative power is held by citizens and the government is ran by elected officials.

Absolute Monarchy - A form of government where the monarch is not limited or restricted by laws or a constitution.

Benefits of a Monarch Government

Photo from the New York Times

Monarchy is a traditional way of governing. Monarchs show love for their country, its people and are willing to sacrifice in ways elected officials often can not. One benefit of a monarchical government is quick decision making. For example, when it comes to making a decision on how to respond to wars and how the army should act, instead of a long debate the monarch makes what he or she believes is the best decision. Also a monarch brings national identity and nationalism to its country. Decisions made by them are put into effect much more quickly than a republic or democracy government.

Another benefit of a monarchical government is it creates stability. There is not constant elections. Elected officials only have a certain amount of time they have to try to make a difference in their country and because of that they think short terms, whereas a monarch thinks long term. Also in a monarchy government the people are able to predict who the next monarch will be because the line of succession is already set up.

In present day Britain is no longer an absolute monarchy, they are a constitutional monarchy. In other words, Queen Elizabeth II acts as Head of State and does not have the ability to pass legislation. An elected parliament controls legislation. Essentially, Queen Elizabeth has no power as queen. The power is in the hands of David Cameron, prime minister of England; parliament and the court system. Along with that, having a monarch as a ruler draws tourist. About 30 million people visit Great Britain a year, which brings in a great amount of money. Along with bringing in money through tourism, monarchs also spread their wealth, such as putting on shows, etc. for their people.

Finally, another benefit of a monarchical government is there is less corruption. Acts of corruption by officials are great offenses. Monarchs have no reason to embezzle money because everything already belongs to them and they look to use their wealth to better their country.

Hemophilia and the Europe Royal                                    Family

Photo from the National Center for Case Study Teaching in Science

  • Queen Victoria was a carrier of hemophilia and the disease appeared in some her children. She had nine children. One son had hemophilia and two of her daughters were carriers. Hemophilia did not appear in any of her ancestors and it is uncertain how Queen Victoria inherited the recessive gene.
  • Ways the disorder may have entered royal blood:
    1. Spontaneous mutation
    • Most accepted that may have been due to a new germ line mutation in Queen Victoria’s parents or grandparents.

    2. Queen Victoria was not Duke of Kent actual daughter.

  • Most controversial.

                       Royal Pedigree

Particularly between the 16th and 18th century, monarchs believed in what is known as the Divine Right of Kings. Divine Right of Kings is the idea that monarchs and their families were directly chosen by God to be king or queen. Therefore, they did not need anyone's approval, such as citizens or parliament. They also believed that because God specifically chose them, they were obligated to keep their bloodline pure. Relating to hemophilia and the Royal Family of England; monarchs, back then made strides to keep their bloodline pure by marrying within royalty. They either married distant family members or royalty from other countries.

Prince Leopold was the only son affected by the disease. Queen Victoria’s other sons, Edward, Alfred and Arthur did not have the disease. Prince Leopold endured a lot of pain from hemophilia, suffering from ruptured blood vessels that bled out. As a child, Prince Leopold was under strict surveillance to prevent any injuries that may have led to death, such as scraped knees or cuts. At age 31 Prince Leopold died of a minor fall.

Queen Victoria’s youngest child, Beatrice, was a hemophilia carrier and married a Spaniard. The gene, then carried into Spain royalty when she gave birth to four children. Her only daughter was a carrier and two of her sons were affected by the disease.

Alexandra, Victoria’s third child, had two daughters who were were carriers and one son who was a hemophiliac. Alexandra’s son died at age three of internal bleeding. Her daughter, Irene (a hemophilia carrier) married Prince Henry of Prussia, who was from German imperial, and had two hemophiliac sons, which whom died of hemophilia.

Tsarina Alexandra, Alexandra’s other daughter was also a carrier. She married Tsar Nicholas II and carried the disease into the Russian imperial family. She gave birth to four daughters and a hemophiliac son. The two discovered their son had hemophilia when he bled more than two days from a fatal injury and swelling appeared. Hemophilia took such a toll on his body, he was obliged to bed rest and wore a heavy iron brace. A monk named Rasputin was the only person who could soothe their son, Alexei. He was able to create a peaceful atmosphere, which is necessary to slow blood flow. While Tsarina and Nicholas were concerned about their son Russia began to degenerate, which was during the climax of the Russian Revolution. Alexei did not die of hemophilia, but instead was executed alongside his family. Because the family was executed, hemophilia was not inherited by the next generation of Russian royalty.

After Queen Victoria’s reign, Edward VII, her first son, was the next monarch to take over. King Edward VII was not affected by hemophilia and neither was his wife. Queen Elizabeth, who is currently monarch of Britain, and Prince Charles, who is next in line for monarch, are descendants of King Edward VII. Since the present Royal Family of England is Edward’s descendants, they are able to fulfill long reins. It is not clear what type of hemophilia the Royal Family had.

Reference

Aronova-Tiuntseva, Yelena, and Clyde Freeman Herreid. "Hemophilia The 'Royal Disease'" National Center for Case Study Teaching in Science,. National Center for Case Study Teaching in Science, University at Buffalo, State University of New York, 5 Mar. 2012. Web. 25 Feb. 2015. <http://sciencecases.lib.buffalo.edu/cs/files/hemo.pdf>.

"Types of Government." Types of Government. Web. 25 Feb. 2014. <http://depts.alverno.edu/dgp/GEC/Types of Government.html>.

Daniel, Harri. "Benefits Of Monarchy | Benefits Of." Benefits Of Monarchy | Benefits Of. 6 Sept. 2011. Web. 25 Feb. 2015. <http://benefitof.net/benefits-of-monarchy/>.

"What Is Hemophilia?" - NHLBI, NIH. National Heart, Lung, and Blood Institute, 31 July 2013. Web. 3 Mar. 2015. <http://www.nhlbi.nih.gov/health/health-topics/topics/hemophilia>.

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