Holiday in Spala
by Royce Ryton
Royce Ryton - Holiday in Spala
BBC Radio 4: Saturday Night Theatre
Saturday 25th July 1970 @ 8:30 p.m.
The action of the play takes place in the hall of a small shooting lodge in Poland called Spala. A pleasant retreat for Nicholas II, Tsar of
Russia, and his wife and children. It is October 1912 and the Imperial family have just arrived to spend a quiet holiday. But the holiday
would eventually lead the Tsarina Alexandra placing the fate of her son, her husband and his empire in the hands of a wandering holy man.
Though on holiday, Tsar Nicholas II still has an Empire to run as he worries over the state of internal affairs as well as the future of foreign
affairs. With rumours of a war looming with Germany, Nicholas tries to convince his eldest daughter, Olga, now 17, that it would be in the
best interest of Russia if she marry Prince Carol of Romania.
Meanwhile, as the Romanovs try to keep secret Alexei's illness, a crisis occurs when he suffers a life-threatening haemorrhage in the thigh
and there is nothing any physician could do for him. The 8-year-old Heir to the Throne is fighting for his life. To the devastated Alexandra, it
seems that God is not answering her prayers to heal her son and so, in desperation, she sends a telegram to Grigori Rasputin....
Adapted for radio by Alison Plowden from Royce Ryton's stage play, "Holiday in Spala", first produced at the 1969 Edinburgh Festival
Fringe.
With Rachel Gurney [Tsarina Alexandra Fyorodovna], Noel Johnson [Tsar Nicholas II of Russia], Patricia Gallimore [Grand Duchess Olga
Nikolaevna], Elizabeth Proud [Grand Duchess Tatiana Nikolaievna], Rita Staines [Grand Duchess Maria Nikolaevna], Deborah Dallas
[Grand Duchess Anastasia Nikolaevna], Jo Manning Wilson [Grand Duke Alexei Nikolaevich], Margo Boyd [Dowager Empress Marie of
Russia], Hector Ross [Grand Duke Alexander Michaelovich], Madi Hedd [Crown Princess Maria (Missy) of Romania], Sonia Fraser
[Baroness Sophie Buxhoeveden], James Thomason [Prince Nicholas Yussupov], John Rye [Prince Carol of Romania], Hilda Kriseman
[Princess Radziwill of Poland], George Raistrick [Doctor Botkin], and Clifford Norgate [Count Fredericks, Minister of the Imperial Court].
Produced by Norman Wright.
90 minutes.
Did Alexei suffered from hemophilia?
Case Closed: Famous Royals Suffered From Hemophilia
by Michael Price on 8 October 2009 (Science Now)
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 haematologist 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 haemophilia B step out from under the more common A-type's shadow, says
pediatric haematologist Paul Monahan of the University of North Carolina, Chapel Hill. "Now it's clear it's had an enormous impact on
Western history."
Jim
