Queen Victoria of Britain had nine children and most of them married into the royal families of Europe. This was normal in the 1800s as the royal families interbred rather than marry someone untitled. But such inbreeding led to the persistence or magnification of bad genetic traits.

One such genetic defect was haemophilia, a bleeding disease. A gene part (called an allele) codes for hemophilia. This allele is on the X chromosome so if a male inherits the affected X, he gets the rare blood clotting condition. This inherited disease passed through Victoria’s descendants either via female carriers with XX make up or affected males with XY. Read more below. Let’s examine this fascinating topic of Romanovs, Rasputin and the Russian Revolution. It has a lot to do with hemophilia which will be explained below so keep reading.

Hemophilia and the Romanovs

Alexei, the tsarevitch and only son of Romanov tsar Nicholas II of Russia had this game changing hemophilia gene defect. Because of this he led an over-protected life of restricted activity lest he bleed to death from an injury. His four older sisters, the grand duchesses, Olga, Tatiana, Maria and Anastasia being girls, did not have the disease. They could have been carriers of it.

Historian, Helen Rappaport eludes to this in her brilliant books. Modern DNA analysis of the duchesses remains reveal that only Anastatia was a carrier. The gene lottery was more kind to the daughters than the son. The chance of being a carrier if your mother is one is 50% yet three of the girls were not carriers purely by chance.

Hemophilia is caused by a gene mutation

It all started with Victoria it seems. It is important to know that females have two X chromosomes to determine gender and males have an X and a Y. Therefore, scientists denote females by XX and males by XY. Variations to this pattern are very rare.

Victoria carried a gene mutation on one of her X chromosomes that can causes hemophilia to be expressed in a male. This is because there is no other normal X to mask it. Females have two X’s so one affected X will only cause them to be a carrier.

A gene mutation is an accidental change in the genetic code of an individual. It can occur during the copying of genes as the cells multiply and most of the time these small changes do not affect anything adversely. However, if a mutation affects the coding or instructions for an enzyme or metabolic factor this mutation can be deadly for the person who has or inherits the mutated gene code.

In the case of hemophilia, the gene alteration prevents adequate production of clotting factor 8 and 9 which are essential for the body’s response to a hemorrhage of any magnitude.

The gene mutation for hemophilia is on the X chromosome

Such was the case for Queen Victoria’s mutation. How it occurred no one knows. She may have inherited this genetic alteration from a parent or it happened first in her gene code. It was only noticed after the event when one of her sons, Prince Frederick started having blood clotting issues. Because the defect for the gene determining the clotting factor 8 was on one of her two X chromosomes and is a recessive ie not dominant allele, her other normal X chromosome masked the defect.

Queen Victoria consequently did not suffer from the disease. Females with the defective X chromosome do not have hemophilia.

In the late 19th century, genetic science was in its infancy. The occurrence of this gene mutation had been noticed because it was in the royal family. From then on the royal families of Europe were watchful for its appearance in their sons. With the line of succession usually via the male descendants this was a worry. Victoria only became queen because there were no male descendants to take the throne.

The need for a male heir for the Romanovs

Male gender-based succession has led to many lives lost throughout history. There are those who killed to get the throne. Kings like Henry VIII killed his wives because they could not give him a male heir. Then there was the War of the Roses where everyone killed everyone for hundreds of years just to secure royal succession. Much blood was shed. But for one small boy in Russia last century, no blood could be shed. Alexei’s blood could not clot and allow the smallest wound to heal. Yet he was the boy destined to inherit his father’s tsardom.

Clotting factor 8 is the essential factor which allows wounds to heal so if it can’t be made by the body, the individual’s wounds no matter how small cannot heal. As well, any falls or bumps can cause internal bleeding which also can be fatal. Such was the fate of little Alexei the heir to the Russian throne.

It is doubly tragic that his parents anguished about not having a male heir for many years before his birth. Alexandra his mother had four normal healthy daughters over a ten-year interval before he was finally born. A boy! The whole of Russia celebrated. The Imperial dynasty of 300 years could continue now. Nicholas’s brother the standing heir stood aside now Nicholas finally had a son.

But it was only days before Nicholas and Alexandra knew their new baby had health issues. His umbilical cord was still bleeding days after the birth. The dreaded royal disease had raised its head again. Already the two young sons of King Alfonso of Spain had inherited hemophilia. Alexandra realized she had passed the fatal gene onto her son. She was a carrier through her mother, Princess Alice of Hesse. Alexandra’s brother had the disease.

Hemophilia occurs in the Romanov male heir

The hemophilia gene mutation is recessive and only expressed if there is no other normal X chromosome to mask it. As males have an XY chromosome make up not XX as females do, the faulty X chromosome from their mother Victoria expressed itself in Victoria’s sons not her daughters. Prince Leopold inherited this fault and had hemophilia and Princess Alice and Beatrice inherited the faulty X trait but because they had another X chromosome from their father that was normal it masked hemophilia. They were carriers not affected by hemophilia themselves.

The defect in their beautiful baby son must be a secret. If the Russian population knew then the tsardom was in peril. Already there was trouble brewing. The people were poor and hungry, and Nicholas and Alexandra were distant, autocratic and lacking in empathy with their citizens. Alexandra as a German princess bride had been unpopular from the start. But Nicholas had married for love not politics. But politics can be ugly and turn on unpopular monarchs. The Russian people did not like Alexandra, the tsar’s wife. This and the poor health of the tsarevitch contributed to the failure to save the Romanovs.

The hemophilia of the Romanov son had to be kept a secret

Alexandra was an introverted, cold woman in public yet at home she was an adoring mother and wife. Really both Nicholas and Alexandra had the personality for their royal roles. They were a homely, family orientated couple wrapped up in each other and their children. They were negligent of their duties in the royal dynasty. This side of royal life did not interest them. They focused inwards on their family not outwards on the Russian people. This became more so once Alexey was born.

Withdrawal from public life

Alexandra withdrew more from public life as she protected her little son and hid his defect from the world. Even other royal cousins did not know as Russia could not know. She must have realized that even if she could by chance have another son, he too could be affected by the disease.

The chance of another son being affected was 50%. Hemophilia had struck twice in Spain’s royal family. There was no certainty that the genetic lottery would spare any future Romanov sons. Besides, Alexandra already had five children and was not a well woman herself. She suffered sciatica, circulation issues and heart problems. Often she would stay in bed for days or weeks. Her mauve boudoir at the Summer Palace in Petrograd was the centre of her existence.

Rasputin, the infamous monk

The family rarely left the palace grounds to mix with the people or travel abroad. When WWI erupted life became more difficult. She was of German origin and Russia was at war with Germany. Plus, her little son was often ill from bruising due to falls. In desperation for help she sought the services of Grigory Rasputin, the mystical monk with healing powers. His inclusion in the royal household made her even less popular with the Russian people.

Russian Revolution

In 1917, while the war raged, the Russian people had enough of their autocratic, uncaring tsar and his wife. There was a revolt and Nicholas reluctantly abdicated. Alexandra was furious. She was not there when he signed the papers and would have stopped him. Stronger than him mentally, she believed in the dynasty and their rights of succession. Nicholas was weak and not a born ruler. His focus was on his family not his country. But because of his abdication and the ensuing politics, his family would pay the ultimate price.

If their little son had not been so delicate, would the Romanov dynasty been able to survive? The occurrence of hemophilia in the one Romanov child who needed to be robust was a tragedy. It was one of the factors in the dynastic and family tragedy of the Romanovs.

To learn more read my linked Romanov article just above or on my blog joniscottauthor.com. Helen Rappaport’s books on the Romanovs are also wonderful informative reading.