Download Hemophilia within the Royal Family What is hemophilia? Why is

Hemophilia within the
Royal Family
A Pedigree Study for Recessive Sex
Linked Traits
Why is hemophilia called The Royal Disease?
The marriage of England's Queen Victoria and Prince Albert
marked the beginning of hemophilia in the British royal line.
Queen Victoria, the Queen of England from 1837 to 1901,
was a carrier of the hemophilia gene. She had nine children
and as English royal family members married into royalty of
other countries, the disease eventually infected most of the
royal houses of Europe. Therefore, hemophilia gained its
title of The Royal Disease because it spread through the
royal families of Europe through Victoria's descendants.
What is hemophilia?
Hemophilia is a hereditary (X-linked, recessive) blood disorder that
affects the proper clotting of blood. The disease affects males more
frequently (1 in 10,000) than females (1 in 100,000,000). The gene
carrying the critical blood-clotting gene is on the X chromosome found
on the chromosome set that determines sex. These are the sex
chromosomes. Males have one X and a Y chromosome [XY], females
have two X chromosomes [XX]. Males have one X chromosome, if a
man has the defective gene he will have hemophilia. Females will
have two genes, if she has inherited the mutation one will be normal
the second chromosome will have the hemophilia gene. She will have
normal blood clotting, but will carry the mutation. This means she has
a 50% chance of passing the defective gene to her children. Female
hemophiliacs are rare because she must inherit two defective X
chromosomes from both parents, her hemophiliac father and a mom
who is a carrier for the disorder.
How did Hemophilia enter the royal families of
Europe?
Hemophilia arrived in the house of the Royal Family with
Queen Victoria’s children. The disease has played an
important role in Europe’s history since. Through marriages
to strengthen political alliances, the British family spread
the disease to the Spanish, German (Prussian), Denmark,
Austria and Russian royal families. The traditional
explanation how hemophilia entered the royal family
describes a mutation in Victoria’s gene or the sperm in
Edward Augustus, the Duke of Kent.
Pedigree of Queen Victoria's Descendants
The disease can be followed through the family genealogy
into the Span, Russia, and Germany through Queen
Victoria’s descendants.
Pedigrees of Queen Victoria's Affected Children
Leopold
Queen Victoria's eighth child, Leopold, was her
only child with hemophilia. Two of Queen
Victoria's daughters, Beatrice and Alice, were also
carriers of hemophilia. The daughters, as carriers,
passed the disease to the Spanish, German and
Russian royal families. Figure 1
Leopold, as a hemophiliac, suffered frequent
hemorrhages and died of one at the age of 31. He
was able to have two children prior to his death.
His daughter, Alice of Athlone, was a carrier. She
gave birth to a hemophilic son (Rupert) and two
other children, a boy and a girl. It is unknown if
they were also affected, but in 1928 the boy died
of a brain hemorrhage.
Figure 1 Leopold a hemophiliac was Queen Victoria's son
Alice
Figure 2 Beatrice was a carrier was Queen Victoria's daughter
Beatrice
Beatrice’s lineage is the link between Queen
Victoria to the Spanish royal families and their
connection with hemophilia. Beatrice was Queen
Victoria’s youngest child and gave birth to one
daughter, one normal son, and two hemophilic
sons. Figure 2
Queen Victoria's second child, Alice, passed
hemophilia to the German and Russian royal
families. Of Alice's six children, one son had
hemophilia and two daughters were carriers.
Alice's daughter Irene married her first cousin,
Prince Henry of Prussia. She gave birth to two
hemophilic sons. Attempts were made to conceal
the fact the disease had shown itself in the
German royal family. Unfortunately at the age of
four, Waldemar, the youngest of the princes, bled
to death. Alice's other daughter, Alix, was also a
carrier. If Alice had accepted the offer of marriage
from Prince Eddy, or his brother George,
hemophilia would have been re-introduced into
the reigning branch of the British royal family.
Instead, Alix married Tsar Nikolas II and carried
the disease into the Russian imperial family. Alice
and the Tsar had four daughters, Olga, Tatiana,
Marie, and Anastasia, before giving birth to the
long-awaited son, Alexis, heir to the Russian
throne. During the Bolshevik Revolution, the
family was murdered. The existence of hemophilia
in the Russian Royal family has historical
significance because of its role in leading to the
revolution. Figure 3
Figure 3 Alice a carrier was Queen Victoria's daughter
Hemophilia in the royal families today
Fortunately, Leopold was the only one of
Queen Victoria's son who suffered from
hemophilia. Her other three sons, Edward,
Alfred, and Arthur, were unaffected. The 21st
century royal family, descended from Victoria’s
first son, Edward VII, and is free from
hemophilia. In addition, the family lineages
with hemophilia from the late 1800's and early
1900's have long ago died. Today, there are no
royal family members known to have hemophilia.
Sources—Canadian Hemophilia Society,
Queen Victoria's Family