The "Royal Disease"

I continue to learn something new every day, and it's so wonderful to do so! I didn't know this before - that there was a so-called "Royal disease" among the royal families of Europe and that it has now conclusively been proven to be hemophelia via DNA analysis. Hemophelia is a horrid genetic defect which, until relatively recently, could not be successfully medically treated. Here is the article, from Science Now: Case Closed: Famous Royals Suffered From Hemophilia By Michael Price ScienceNOW Daily News 8 October 2009 Queen Victoria's male descendants were cursed with poor health. The 19th century British monarch's son Leopold, Duke of Albany, died from blood loss after he slipped and fell. Her grandson Friedrich bled out at age 2; her grandsons Leopold and Maurice, at ages 32 and 23, respectively. The affliction, commonly known as the "Royal disease," spread as Victoria's heirs married into royal families across Europe, decimating the thrones of Britain, Germany, Russia, and Spain. Based on the symptoms, modern researchers concluded that the royals suffered from hemophilia--a genetic disease that prevents blood from clotting--but there was never any concrete evidence. Now, new DNA analysis on the bones of the last Russian royal family, the Romanovs, indicates the Royal disease was indeed hemophilia, a rare subtype known as hemophilia B. Hemophilia prevents proteins known as fibrins from forming a scab over a cut or forming clots to stop internal bleeding. Even minor injuries can lead to bleeding, which lasts for days or weeks and can be fatal. The disease is recessive and is carried on the X chromosome, meaning that men are more likely to develop it, whereas women usually act as carriers and don't show symptoms. Such was the case with Prince Alexei Romanov, son of Tsar Nicholas II, great-grandson of Queen Victoria, and heir to the Russian throne. From an early age, Alexei was prone to prolonged bleeding, and his family feared that he wouldn't make it through his first month of life, says Evgeny Rogaev, a geneticist at the University of Massachusetts Medical School in Worcester. The disease didn't kill Alexei, however: He was murdered at age 13 in 1918 along with the rest of the Russian royal family following the Russian Revolution. Earlier this year, Rogaev and his colleagues reported that, based on DNA analysis, the bodies of two children found near the murder site were indeed those of Alexei and his sister Maria. They further confirmed that the other bodies near the site belonged to the rest of the Romanov family. But Rogaev wanted to solve the final Romanov riddle: Did they really suffer from hemophilia? He and colleagues analyzed DNA from the royal bone fragments again, this time looking for genetic markers of hemophilia. The most common type of the disease, hemophilia A, accounts for about 80% of hemophilia cases and is caused by a mutation to a gene called F8, which encodes a protein involved in blood clotting. They didn't find the mutation. So Rogaev moved on to looking for a rarer form of the disease, hemophilia B, which involves another gene, F9. This time, the team found a mutation in F9, which would have inhibited clotting, in bones from Alexei, his sister Anastasia, and their mother Alexandra. The findings, published online today in Science, indicate that Alexei did indeed have hemophilia B and that his mother and Anastasia were carriers for the disease, bearing out the previous speculation. They also confirm that the other instances of "Royal disease" in the family line were hemophilia, Rogaev says, because they all shared a common genetic heritage. The last carrier of the disease in the royal family was Prince Waldemar of Prussia, who died in 1945. The disease impacted not only the Romanov family but also probably Russian history, Rogaev adds. Alexei's frail condition encouraged his mother Alexandra to keep close company with the Russian mystic Grigori Rasputin, who claimed to wield healing magic. "There was no medication at that time," Rogaev says. "She tried to do everything possible." According to some historians, when Rasputin used his close relationship with the Romanovs to influence bureaucratic affairs in his favor, the public grew increasingly suspicious of the regime, possibly hastening the revolution. Katherine High, a hematologist who studies blood coagulation at The Children's Hospital of Philadelphia, says that the mutation found in the Romanov bones fits an established genetic pattern known to cause hemophilia B, further supporting Rogaev's findings. Tracing this pattern back to the royal family and its history of disease is "very interesting and very exciting," she says. People affected by the disease today should be excited to see hemophilia B step out from under the more common A-type's shadow, says pediatric hematologist Paul Monahan of the University of North Carolina, Chapel Hill. "Now it's clear it's had an enormous impact on Western history." One of my nieces has gone into the field of hematology, devoting herself to lab research in the hopes of discovering clues that may lead to cures for a myriad of blood-related disorders and diseases. I am very proud of her.

Czar Nicholas and Family Vindicated

Hmmmm, interesting. I wonder if this is a Miniputin move that may come back to bite him in the butt (along with the ill-advised invasion of Georgia). How do they KNOW relatives of the Romanovs won't file suit for reparations against the war crimes committed by the Bolsheviks, the benefits of which have flowed to the neo-USSRists? Stay tuned... (Photo: The Czar and his family, 1914). From The New York Times Court Rehabilitates Status of a Czar and His Family By MICHAEL SCHWIRTZ Published: October 1, 2008 MOSCOW — Russia’s Supreme Court ruled in favor of full rehabilitation for Russia’s last czar and his family on Wednesday, officially recognizing the Romanovs as victims of “unfounded repression” 90 years after they were executed. The ruling is the latest step in Russia’s post-Soviet reinterpretation of history, which has seen a new embrace of a monarchy once castigated for brutality and backwardness, accompanied by both nostalgia for and damning reconsiderations of seven decades of Soviet rule. Soviet historians constructed accounts that emphasized blaming Nicholas II, or “Bloody Nicholas,” for famines, wars and social collapse. But as Russian nationalism has strengthened after the fall of the Soviet Union, Nicholas has increasingly been depicted as a thwarted visionary and a beacon of the Russian Orthodox faith. The church, which sanctified the Romanovs in 2000 and was itself persecuted in the Soviet era, welcomed the court’s decision. “It is an important step to remove from our history the heavy burden of this crime against the czar’s family,” said the Rev. Vsevolod Chaplin, a church spokesman. “In one way or another the perceptions of society toward Nicholas II and his family are changing,” Father Chaplin said. “More and more people are becoming free of the sharp clichés that were imposed in the recent past.” In its decision on Wednesday, the court reversed a ruling of last November, when it decided that the Romanovs were not eligible for rehabilitation because their execution was a criminal act, not one of political repression. The new ruling “recognizes their unfounded repression and rehabilitates the members of the royal family,” said Pavel Odintsov, a spokesman for the court. “This is a final decision,” he added. In July 1918, under Lenin’s orders, the czar, his wife, Aleksandra, and their children, Olga, Tatyana, Maria, Anastasia and the 13-year-old heir to the throne, Aleksei, were shot to death in the basement of a house in Yekaterinburg, a city in the Ural Mountains in central Russia. Several members of the family’s staff were also killed. The killings by the Bolshevik government were meant to solidify its hold on power in the midst of an intensifying civil war. The Romanovs’ bodies were likely doused in acid to mask their identities before being buried in secret graves. The remains of Nicholas and Aleksandra and three of the five children were discovered in 1991 in the waning days of the Soviet Union, and interred in 1998 in St. Petersburg, in a special chamber in the Cathedral of Saints Peter and Paul, where many other Russian royals are buried. The remains of the two other children were missing until August 2007, when an archaeologist in Yekaterinburg unearthed bone fragments not far from where the other Romanovs had been buried. The authorities announced this year that DNA testing had confirmed that the remains belonged to Aleksei and Maria. Other members of the royal family had been posthumously rehabilitated. In 1999, four Romanov princes killed by the Bolsheviks, including the son of Aleksandr II — the Russian czar blown up by revolutionaries in 1881 — were found innocent of criminal wrongdoing. In July, thousands of Russians took part in events to mark the 90th anniversary of the family’s execution, and calls for the restoration of the monarchy can be heard despite today’s Kremlin-managed political landscape. The ruling Wednesday seemed to echo that nostalgia. “This decision shows the supremacy of law and the victory of justice over evil and tyranny,” said German Lukyanov, the lawyer for Grand Duchess Maria Vladimirovna, a Romanov descendant who first filed a suit for the rehabilitation three years ago. Mr. Lukyanov said that in the coming months he would file suits on behalf of other Romanovs who had yet to be rehabilitated, including the czar’s brother, Mikhail, and several other members of the dynasty. It is still unclear why the Russian government took so long to rehabilitate the czar. Some have suggested Russia’s current leaders feared that Romanov descendants would seek to reclaim property confiscated by the Bolsheviks, while others have speculated that recent leadership changes in the country could have played a role. Yet, past decisions — including the earlier rehabilitation of those responsible for organizing the family’s execution — are less important than what comes next, said Edvard Radzinsky, a Russian historian and the author of “The Last Tsar: The Life and Death of Nicholas II.” “We have two graves that symbolize the revolution: the dirty hole into which the Romanovs were thrown, and the mausoleum of the one who ordered this,” Mr. Radzinsky said, referring to the red pyramid on Red Square that houses Lenin’s preserved body. “The closing of the first grave,” he said, “should lead to the closing of the second.”

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If you believe in a grand overarching conspiracy theory, then settle in for a long haul yet. The "royals" - descendants of the kings and queens of old, can afford to play a game of wait and see/cat and mouse. Russian politicians will come and go, and so will political systems, but the royals remain forever...

Murder of the Romanovs Started a Diabolical New Era

The mystery surrounding the whereabouts of the remains of two missing members of the Russian royal family murdered on orders of Lenin at the close of WWI may now be put to rest, pending DNA test results:

From The New York Times:

By CLIFFORD J. LEVY
Published: November 25, 2007
YEKATERINBURG, Russia — On the outskirts of this burly industrial center, off a road like any other, on a nowhere scrap of land — here unfolded the final act of one of the last century’s most momentous events.

A short way through a clearing, toward a cluster of birch trees, the killers deposited their victims’ bodies, which had been mutilated, burned and doused with acid to mask their origins. It would be 73 more years, in 1991, before the remains would be reclaimed and the announcement would ring out: the grave of the last Russian czar, Nicholas II, and his family had been found.

But the story does not end there.

Eleven people were said to have been killed that day in July 1918 on Lenin’s orders. Just nine sets of remains were dug up here and then authenticated using DNA. The remains of the czar’s son, Aleksei, and one daughter, whose identity is still not absolutely clear, were missing. Did their bones lie elsewhere, or could it actually be that they had escaped execution, as rumor had it for so long?

Only in the past few months have these questions dating from the Russian revolution apparently been resolved here, and only by a group of amateur sleuths who spent their weekends plumbing the case. In fact, it appears that the clues to what happened to the two children were always there, waiting to be found. All that was needed was to listen closely to the boastful voices of the killers.

Their accounts are in secret reports in Soviet-era archives, one of which offered the most tantalizing hint: a single phrase in the recollection of the chief killer that seemed to suggest where the two bodies might have been deposited.

“All of them wanted to leave a trace in history, for they considered that this was a kind of heroic deed,” said Vitaly Shitov, who lives in the area and undertook a review of the testimony to hunt for the remains. “They wanted to promote their roles.”

Following that wisp of a clue this summer, Mr. Shitov and other amateur investigators went to where the other remains had been found — and they kept walking. Away from the road, about 70 yards from the first burial ground, is a slightly elevated area among the trees.

It is there that the bodies of Aleksei, 13, and his sister were apparently consigned.

The amateurs found the bones, many of them charred by fire, scattered among bullets and pieces of jars that held acid used to disfigure the bodies. These fragments appeared similar to those from the first grave.

So it seems that for all the years since the first discovery, even as people made pilgrimages to the site and wondered what had happened to Aleksei and his sister, their remains were only a short stroll away.

Scientists in Russia and the United States are testing the new finds extensively. The sister is believed to be Maria, 19, though that is not entirely settled.

Others long conjectured that the sister was Anastasia, 17, a theory that fed a belief that she survived. (A woman named Anna Anderson was one of several who over the years claimed to be Anastasia, but DNA testing later disproved her.)

If, as expected, results of DNA tests on the two sets of remains are conclusive, they would put to rest many of the doubts that have arisen in Russia and worldwide about the inquiries into what had happened to the royal family.

Among the most skeptical has been the Russian Orthodox Church, which has never recognized the authenticity of any of the bones here, in part because it said that the missing remains raised questions about whether the nine sets were authentic.

Among some Russians and foreigners alike, the fate of Aleksei and his sister drew intense interest in recent years, as if the inability to find their remains and give them a proper burial was a final affront to the royal family by the Bolsheviks. People looked for bones all over Yekaterinburg, which is in the Russian heartland, 900 miles east of Moscow, on the divide between Europe and Asia.

They painstakingly went over the events of July 17, 1918, when the killers knifed and gunned down Nicholas II, his wife, five children, doctor and three servants in the basement of a house where they were being held after Nicholas was forced to abdicate. It was not easy determining what had occurred — the efforts to dispose of the bodies were poorly planned and inept. Subsequent recollections in the archives are sometimes contradictory.

The killers wanted to conceal the bodies so their graves would not become rallying points for the czar’s supporters. They first dumped them in a mine shaft, then moved them to the burial site off the road.

In recent years, the mine was searched for the missing two sets of remains. People also periodically hunted in the immediate area around the grave where the first set of bones was found.

Then Mr. Shitov and his colleagues decided to scrutinize a statement by the chief killer, Yakov Yurovsky, in the archives. Yurovsky related how he had set aside two corpses, believing that if they were burned and buried separately they would confuse royalists who later might be seeking 11 bodies, not nine.

But how separately? The amateur investigators focused on a Russian phrase that Yurovsky used to describe the sequence of events in the second burial. The phrase — “tut zhe” — can mean “nearby,” “right here” or “right now.” It had often been interpreted as indicating that the second grave was next to the first.

But now a different thought arose. From the context, the experts wondered whether Yurovsky meant that the grave was in the area, but not very close to the first. They also presumed that to burn the bodies he needed to find a place away from the wet ground near the road.

Working weekends this summer, they began searching away from the first grave and road, and first found the remnants of the bonfire that was apparently used to burn the two bodies.

Sergei Pogorelov, an archaeologist who was called in to oversee the work, said that about 15 intact bone fragments were recovered, and more than 40 pieces of charred bone.

Mr. Pogorelov emphasized that many of the reservations about the discoveries at the first site cropped up because the excavation there had been done haphazardly. This time, he said, a professional archaeological dig was done, and the Russian Orthodox Church was invited to observe.
“We have tried to avoid the mistakes that they made in 1991,” he said. “Before, there was simply not any scientific method.”

The nine sets of remains were interred in a lavish ceremony in 1998 at the Cathedral of Saints Peter and Paul in St. Petersburg, which contains the crypts of earlier Russian royals. But the Russian Orthodox Church would not formally take part in that ceremony because of its concerns about authenticity.

For now, the church has declined to say whether it considers the newly found remains genuine, pending further tests. But people who have long sought the remains say they are hopeful that once the results are in, the church will formally conduct a service at the cathedral in St. Petersburg to lay to rest the final remains of the Romanovs.

“This brings closure to a very sad chapter in Russian history,” said Peter Sarandinaki, an American of Russian descent who started an organization to help find the remains and had conducted several searches here. “It is because their murder symbolizes the start of a diabolic era in world history. And now that has all come to an end.”