Hemophilia - Joni Scott

Romanovs, Rasputin and the Russian Revolution

By Joni Scott | January 22, 2024

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 marriage with commoners was not allowed so the royal families interbred. But such inbreeding led to the persistence or magnification of bad genetic traits. One such genetic defect in Victoria’s line was the gene part (called an allele) that codes for hemophilia, a rare blood clotting condition. This inherited disease passed through her descendants either via female carriers or affected males. The incidence of hemophilia in the Romanov royal family was one of the contributing factors to the Russian Revolution of 1917. 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 was destined to a short 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 though they possibly were 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 escaped being carriers purely by chance.

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, females are denoted by XX and males by XY. Variations to this pattern are very rare.

Hemophilia is caused by a gene mutation

Victoria carried a gene mutation on one of her X chromosomes that can cause hemophilia to be expressed. 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 prevented 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 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 or kings like Henry VIII who 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 who was 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 he was finally born. 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 the hemophilia was masked, and they were carriers not affected by hemophilia themselves.

The defect in their beautiful baby son had to be hidden. 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 were unsuited for their royal roles. They were homely, family orientated couple wrapped up in each other and their children but negligent of their duties in the royal dynasty.

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 had already born 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.

Why didn’t anyone save the Romanovs?

By Joni Scott | January 11, 2024

The Romanovs were the last of the Imperial royal family of Russia. They were most cruelly assassinated in July 1918 and their bodies thrown into a ditch. If you didn’t already know, the family consisted of the former Tsar Nicholas II, the Tsarina Alexandra and their five children. The oldest four children were all girls named in order of birth Olga, Tatiana, maria and Anastasia. The youngest child was the heir or Tsarevich, Alexey just fourteen at the time of his murder by the then Bolshevik government. Mowed down in a hail of bullets, their bodies thrown without reverence or ceremony into a ditch to rot. Why didn’t anyone save the Romanovs? Helen Rappaport explores this in her fascinating book, The Race to Save the Romanovs.

Since I am enjoying this book, I thought to share the read with you. I know it is a little off topic to my usual but not so far from my latest WWI novel, Time Heal my Heart. the Russian Revolution happened during WWI.

Deposed from 300 years of Royalty.

Noone deserves this fate, especially those born into royalty who had served their country whether adequately or not. This last Imperial family represented 300 years of glorious dynastic rule over vast lands and palaces. But the politics turned against the Tsar and the Tsarina particularly. She was a German princess of Hesse married to Nicholas to secure European alliances. When WWI broke out in 1914 her German connections were not as appreciated by Russians as Russia was on the side of Britain and France and Germany the enemy.

Alexandra the unpopular Tsarina

Adding to this unpopularity was the fact that Alexandra had become obsessed with Rasputin the monk. He was moved into the royal palace to supposedly cure her son of his hemophilia. This genetically transmitted disease was the blight of the family’s happiness. As the only son and promised heir of Russia, this disease made life difficult and precarious. Alexey at any time could bleed to death from any injury that drew blood. His body could not produce the clotting factor necessary to stop a flow of blood like normal people. Hence, he spent his childhood almost literally wrapped in cotton wool. His mother Alexandra fussed and cosseted over him to the neglect of her more robust daughters.

Four daughters and a sickly heir

As a result, these four young girls lived a protected and seemingly dull domestic life within the golden Alexander palace in Petrograd. It was not only their brother who was often ill and poorly but in time their mother too. She suffered from heart issues, sciatica and other health problems that took her to bed for weeks at a time. So, despite her youth, she was an absent mother and perhaps wife as well.

Nicholas II in contrast was slim and reasonably healthy and of a gentle kindly disposition. He was not an aggressive nor militant man so was not a born leader. he preferred the domestic life, loved his family and nature. A quiet life in the countryside would have suited him fine. But he was born into royalty so had to be seen to be active in the affairs of his country. Behind their impressive facade of grandeur, the Imperial family of Russia was like any other loving family with five children. They wanted to enjoy each other’s company and live a quiet and happy life.

Revolution and abdication

When civil revolution erupted during WWI, he seemed too easily convinced to abdicate. But by signing the waver to his royal position he unwittingly signed the death penalty for himself and his beloved family. If any of the two, Alexandra had more ambitions. She was born into an autocratic German family and was not one to embrace the common people. As such she was as distant a royal as she was a mother. The Russian people did not like her.

Royal cousins

Tsar Nicholas however was a royal grandson of Queen Victoria. he had grown up with his royal cousins in a less grand atmosphere. He was close to his British cousins, especially the boy who would become King George V of Britain. They even looked alike and were often mistaken as brothers. Another cousin who was not as well liked was Wilhem of Germany who would become Kaiser Wilhem and a thorn in their sides over the years to come. His ambition and militarism would feed into the progress of the two world wars.

In contrast George V and Nicholas were gentler souls, perhaps ill-suited for their adult roles pitted against the might of Germany and their royal cousin. With regards to possible saviours for the Tsar and his family, history indicates that George V was best positioned for this role. But there were others. Supporters or monarchists within Russia, Alfonso of Spain, Chritian of Denmark and even Wilhem II himself. Why didn’t anyone save the Romanovs?

Politics is a deadly game

The simplest answer to this question is that politics is a deadly game. All the people who could help the Tsar were enmeshed in a web of politics that made any rescue attempt either deadly or suicidal politically. The timing of the revolution in 1917 could not have been worse. Europe was in the grip of war. Britain and France needed Russia on their side to win against Germany. If either of these two countries appeared to be on the side of the old Russian regime then the new Russia could withdraw support. This is mainly why both Britain and France decided not to help. Added to this was their concern over revolt within their population. Fascists and socialists abounded and assisting the old Russian regime would inflame tensions within Britain and France.

Neutral Spain to the rescue?

Then what about King Alfonso of Spain? His country was neutral, could not he have intervened? After all he was another royal cousin descended from Queen Victoria and besides his two young sons also had hemophilia. They must have had an affinity over this royal disease that affected male progeny. But though Alfonso thought long and hard over this issue, he too settled for inaction. His concerns were similar. The socialists and fascists who could turn nasty (and they did in the later Civil War in 1936) could affect his popularity and destabilize the government.

Failure to act

Meanwhile while cousins failed to act, The Tsar and his family were moved further away from the rest of Europe into Western Siberia to a dismal place called Tobolsk. The time to act had passed. From Petrograd especially soon after the abdication would have been best. The Russian people had not yet turned their backs on the family, nor had the new government. Rescue was talked about. There was a way by sea to Finland if the family could be transported by rail past Petrograd. But water exits had to be before ice set in and this chance was missed as the months passed and winter set in.

Britain made a tentative offer via George V in these early days of 1917 but then later took it back due to the British government pressure. The German Alexandra would not be welcome in Britain, tensions would flare, and various other excuses swayed George’s mind. By then forces within the new government were in place to move the family west to Siberia. Out of sight, out of mind mentality.

Escape Options

There, in a rundown government house, the family of seven resided until July 1918 under guard watch. From this location, there was also chances of escape. Roads were mostly impassable, and a rail head was 132 miles away, so water was a better route. A boat down river towards ports that could lead to the sea and a number of possible destinations. The Arctic Ocean and Archangel lay beyond. Bergen in Norway was another option as there was a Norwegian shipper who was willing to help.

A Bergen to Aberdeen escape route had been under British consideration in 1917 before the offer to help was withdrawn. Some Bergen ships operated under British control, so this could have worked once the family were free of Russia. But getting out of Russia was the problem as then, in 1917, the family were near Petrograd which was heavily under government control. Any rail link connection entailed passing through the city first. Another port often considered was that of Murmansk. But this too was a fantasy as this supposedly ice-free port is not really always ice free. Also, its fleet of ships was not exactly a fleet but an old battleship, a cruiser and some fishing trawlers. Plus, German submarines patrolled the waterways and icebergs also abounded to add to the danger.

Why didn’t Kaiser Wilhelm save his cousin?

Of all the royal cousins who could have helped, the one with most power was Wilhelm himself. Word from him in his immense position of power could have saved the family. Why did he not act in sympathy? They were family after all. Wilhelm was even Alexey’s godfather. But no, help was not forthcoming in 1918 either. By then Russia had conceded to Germany in a peace pact and this involved the division of Russia into four governance regions that would serve industrial Germany. Any concessions to a previous monarchy would contravene this treaty. Monarchists could raise the Tsar or his son back to power.

No, Wilhelm did not help. By mid 1918 it was too late anyway. The Russian government with all its powerful bodies, lenin included did not care to meddle with saving the old regime in any way. Turning their back on humanity, they let the status quo sign the death warrants for the ill-fated family, children and all. Nobody helped the once loved royal Romanovs. In July 1918, they were beyond hope.

Joni Scott is an Australian author who blogs about history on her website joniscottauthor.com. Her books are historical and contemporary and based on true stories.

Tag: Hemophilia