He gave the example of the attitudes and world views, the policies and programs, even the demands and concessions of President Franklin D. Roosevelt during World War II, contending they were inevitably influenced by his suffering from polio. 2

How different the world would have been if FDR had been a different man, unfettered by the paralysis and its effects on his life. Yet the polio virus is an indescribably tiny thing. It’s almost nothing at all.

So such little things have influenced the course of all history. It wasn’t a tiny virus that brought about the fall of the House of Romanov in the early 20th century. It wasn’t even a smaller and simpler strand of protein! It was the lack of it.

Haemophelia is an inherited disease of the blood . The body depends on the clotting of blood to stop bleeding after injury and to help healing. Clotting comes from the interaction of many substances, called factors, in a specific sequence or “cascade.”

If one of these clotting factors is not present in sufficient amounts prolonged bleeding may occur. A person with haemophilia has less clotting factor than usual.

Haemophilia A is the most common form of the disorder and is due to a deficiency of Factor 8. A person with haemophilia does not bleed any faster than a normal person but the bleeding carries on for much longer.

Haemophilia affects males almost exclusively and is found in all populations about once in 5,000 male births. It is passed on by women who usually have no bleeding problems. In about one third of people with haemophilia there is no family history of the disorder.

The body is made up of millions of cells, each with a central controlling nucleus containing chromosomes. These are paired structures which determine the development of each person. The most obvious characteristic is their sex.

A female has two X chromosomes (XX) and a male has one X and one Y chromosome (XY). One sex chromosome from each parent is passed on by random chance to each of their children, giving four possible combinations for each pregnancy (two males and two females).3

Chromosomes are composed of many units called genes which determine the body/s functions. In addition to sex determination, the X chromosome also carries the gene that controls the production of clotting factor VIII.

In people with haemophilia these genes produce an insufficient amount of FVIII, which explains why the severity of bleeding is constant within families, and why women with the haemophilia gene may have normal clotting as their second (usually normal) X chromosome produces a sufficient amount of factor. In males (with X Y chromosomes) the Y chromosome takes no part in FVIII production.

If a male inherits a haemophilic X chromosome from his mother, he will be a bleeder, as his Y chromosome cannot compensate for the inability of his X chromosome to provide FVIII.

In each pregnancy involving a female carrying the haemophilia gene there is a 1 in 2 chance that the haemophilia gene will be passed on, and when this happens a male child will have haemophilia, and a female will be a carrier of the haemophilia gene. In other words, for each pregnancy there is a 1 in 4 chance that the child will be a male with haemophilia.

England’s Queen Victoria seems an unlikely candidate to have brought the Romanov dynasty to its knees and handed Russia to Lenin and the Bolsheviks; but this monarch among monarchs, to whom her reigning colleagues accorded the title “THE Queen” did exactly that. 3.

Victoria was following a purposeful policy of uniting the thrones of all Europe into one great family by marriage. She transmitted the hereditary gene for haemophilia A to her many offspring. Since there doesn’t seem to be any record of blood disorders earlier in the family , it is assumed the defect originated with Victoria.

Victoria had 9 children. Her son Leopold died from haemophelia. Of her 5 daughters, three transmitted the disorder to their children or grandchildren. Victorias second daughter and third child was Alice, born in 1843. She married Louis the IV, the Grand Duke of Hesse-Darmstadt. 4, 5.

They had 7 children, 5 of which were daughters. The fourth, Alix, was born in 1872. Called “Sunny” for her bright red-golden hair and blue eyes, she traveled, at age 12, to Russia, to attend the marriage of her older sister to Serge, the brother of Tsar Alexander III. It was at the wedding she met 16 year old Nicholas II.

Nicholas was something of an outsider to his family. The Romanovs were exceedingly large and strong. 6. Alexander could bend a coin in his fingers, and once supported the collapsed roof of a railway carriage on his massive shoulders while his family escaped the accident. But Nicholas was only 5 feet 6 inches tall. He was brave, athletic, charming and intelligent, but fatally at the mercy of the stronger personalities of his gigantic Romanov relations.

He had fallen hopelessly in love with the prudish, cold and aloof Alix of Hess. Victoria was all in favor of the relationship, but Alexander III and his wife both disliked Germans. And Nicholas also encountered opposition from Alix herself. She was a devout Lutheran, and had no desire to convert to an “alien” faith. The Tsarina of Russia had to be a member of the Orthodox Church.

In February of 1894, Alexander, then 49, seemed strong and healthy. But he fell ill to influenza and, as it turned out, to renal disease. By April he was obviously dying. Suddenly the matter of marriage for Nicholas became a matter of state magnitude. And as he wanted no one but Alix, the Tsar gave in.

Alexander was gravely ill by October. Alix was summoned and arrived on the 23rd. A formal betrothal took place in Alexanders bed chamber, with the Tsar insisting he be dressed in formal uniform for the occasion.

This entry in Alix’s diary gives great insight into her character, her thoughts regarding Nicholas and proved to be prophetic of their future.

“Sweet child…your Sunny is praying for you and the beloved patient…Be firm and make the doctors come alone to you every day so that you are the first always to know. Don’t let others be put first and you left out. Show your own mind and don’t let others forget who you are.”

On November 1, 1894, Alexander died. 26 year old Nicholas became Tsar, Autocrat and Emperor of All The Russias. Alix was received into the Orthodox Church as Alexandra Feodorovna. The marriage on November 26th was held in deep mourning.

Though the business of state descended upon Nicholas and he had no time for a honeymoon, Alexandra performed her duty. Between 1895 and 1901 she produced 4 daughters for the House of Romanov.

Nicholas transmitted to each the X chromosome, balancing their mothers flawed counterpart in the girls. Then, during the midst of the Russo-Japanese War, 7 on August 12, 1904, St. Petersburg resounded with news that would ultimately change the course of world history. Alexandra had given birth to a son, Alexis, and transmitted to him Victorias mutant haemophiliac gene, unbalanced by a corresponding X chromasome from Nicholas.

The disease first manifested at six weeks, with uncontrolled bleeding from the umbilicus. Then came the endless brusies from the minor bumps of crawling and learning to walk. Alexandra was forced to accept that she had given this most longed for son the bleeders disease. She never recovered from the shock.

The family withdrew to Tsarskoie Tselo, the Tsars Village, in what is today Pushkin, outside St. Petersburg. Here Alexandra lived in a world of hopelessness, self-imposed loneliness and self-loathing.

Evidencing all the process of what today we call the 5 Stages of Grieving, 8 she first resolved to fight, seeking someone somewhere who could make a cure, or declare that an error had been made. But specialist after specialist gave the same sad news, and Alexandra realized she was, ultimately, utterly alone.

Finally she began to prefer it that way. The outside world became cold and unfeeling to her, going about its business instead of screeching to a hault, as her life had, over the tragedy of Alexis.

Like Victoria before her, who hid herself in Windsor Castle from the loss of her son Albert to the disease, Alexandras family became the only refuge. There were no pretensions, no questions. The sadness need not be hidden. This cloistered, inner world became the Tsarinas reality.

1.http://home.pacific.net.hk/~paulchui/sagan.html

http://www.news.cornell.edu/general/Dec96/saganobit.ltb.html

2.COSMOS Carl Sagan Random House,1980. pp.

3.http://www.britannica.com/bcom/eb/article/single_image/0,5716,7184+asmbly%5Fid,00.html

4.http://www.britannica.com/seo/h/hesse-darmstadt/

5.http://feefhs.org/maps/gerw/gw-hesse.html

6.http://www.alexanderpalace.org/palace/alexbio.html

7.http://encarta.msn.com/find/Concise.asp?z=1&pg=2&ti=761552002

8.http://www.death-dying.com/grief/griefstages.html 9. 10.