Nicholas and Alexandra

sex-linked recessive Mendelian pattern. Thus, while women carry the defective genes, they almost never suffer from the disease. With rare exceptions, it strikes only males. Yet it does not necessarily strike all the males in a family. Genetically as well as clinically, hemophilia is capricious. Members of a family in which hemophilia has appeared never know, on the birth of a new son, whether or not the child will have hemophilia. If the child is a girl, the family cannot know with certainty whether she is a hemophilic carrier until she grows and has children of her own. The secret is locked inside the structure of the chromosomes.^*

If modern science has made little progress in finding the cause of or a cure for hemophilia, it has achieved an extensive charting of the scope of the disease. Hemophilia follows no geographical or racial pattern; it appears on all continents, in all races at a statistical ratio of one hemophiliac among every 5,000 males. In the United States, there are 200,000 hemophiliacs. Theoretically, the disease should appear only in families which have a previous history of hemophilia. But today, in the United States, forty percent of all cases appearing have no traceable family history. One explanation for this is that the defective gene can remain hidden for as many as seven or eight generations. A more probable explanation is that the genes are spontaneously changing or mutating. What causes these spontaneous mutations, no one knows. Some researchers believe they are the result of new and rapidly changing environmental factors such as drugs or radiation. In any case, their number apparently is increasing.

   The most famous case of spontaneous mutation occurred in the family of Queen Victoria. The tiny indomitable woman who ruled England for sixty-four years and who was “Granny” to most of Europe’s royalty was, unknowingly at her marriage, a hemophilic carrier. The youngest of her four sons, Prince Leopold, Duke of Albany, had hemophilia. Two of her five daughters, Princess Alice and Princess Beatrice, were hemophilic carriers. When the daughters of Alice and Beatrice—Queen Victoria’s granddaughters—married into the royal houses of Russia and Spain, their sons, the heirs to those two thrones, were born with hemophilia.

The Queen, on learning that her own son had hemophilia, was astonished. Bewildered, she protested that “this disease is not in our family,” and indeed it had not appeared until that point. A spontaneous mutation had occurred, either in the genetic material of Victoria herself or on the X chromosome passed to her at conception by her father, the Duke of Kent. Nevertheless, soon after Leopold’s birth in 1853, the evidence of the disease in the form of bumps and bruises was unmistakable. At the age of ten, he was assigned to tend, during a family wedding, his equally stubborn, four-year-old nephew William, the future Kaiser. When William fidgeted and Leopold reprimanded, the small German boy bit his uncle on the leg. Leopold was unharmed, but Queen Victoria was angry. Leopold grew up a tall, intelligent, affectionate and stubborn prince. Throughout his boyhood and adolescence, his wilfulness often led to hemorrhaging, and he was left with a chronically lame knee. In 1868, the British Medical Journal reported one of his bleeding episodes: “His Royal Highness … who has previously been in full health and activity, has been suffering during the last week from severe accidental hemorrhage. The Prince was reduced to a state of extreme and dangerous exhaustion by the loss of blood.” In 1875, when Leopold was twenty-two, the same journal recorded: “The peculiar ability of the Prince to suffer severe hemorrhage, from which he has always been a sufferer … is essentially a case for vigilant medical attendance and most careful nursing.… He is in the hands of those who have watched him from the cradle and who are armed by the special experience of his constitution, as well as the most ample command of professional resources.”

The Queen reacted in a manner typical of hemophilic parents. She was unusually attached to this son, worried about him, overprotected him, and as a result of her constant admonitions to be careful, she often fought with him. When he was fifteen, she gave him the Order of the Garter at a younger age than his brothers “because he was far more advanced in mind and because I wish to give him this encouragement and pleasure as he has so many privations and disappointments.” When Leopold was twenty-six, his mother wrote to the Prime Minister, Benjamin Disraeli, that Leopold could not represent her at the opening of an Australian exposition as Disraeli had asked. Using the royal third person, the Queen wrote: “She cannot bring herself to consent to send her very delicate son who has been four or five times at death’s door [italics the Queen’s] and who is never hardly a few months without being laid up, to a great distance, to a climate to which he is a stranger and to expose him to dangers which he may not be able to avert. Even if he did not suffer, the terrible anxiety which the Queen would undergo would unfit her for her duties at home and might undermine her health.”

Constantly frustrated by his mother’s attempts to shelter him, Leopold looked for something to do. His older brother Bertie, the Prince of Wales, suggested giving him command of the Balmoral Volunteers, a military company stationed near the royal castle in Scotland. The Queen, fearing for Leopold’s knee, declined, and Leopold thereafter refused to go to Balmoral. When the Queen tried to keep her son sequestered on an upper floor of Buckingham Palace, Leopold slipped away for two weeks to Paris. At twenty-nine, to his mother’s surprise, he found a German princess, Helen of Waldeck, who was unafraid of the disease and willing to marry him. They lived happily for two years and she bore him a daughter. Helen was pregnant a second time when, in Cannes, Leopold fell, suffered a minor blow on the head and died, at thirty-one, of a brain hemorrhage. His mother sorrowed for herself and the family, but, she wrote in her journal, “for dear Leopold himself, we could not repine … there was such a restless longing for what he could not have … that seemed to increase rather than lessen.”

Prince Leopold, the first of the royal hemophiliacs, was the Empress Alexandra’s uncle. His affliction meant that all of his five sisters were potential carriers, but only Alice and Beatrice actually transmitted the mutant gene into their offspring. Of Alice’s eight children, two of the girls—Alix and Irene—were carriers. One son, Alix’s brother Frederick, called “Frittie,” was a hemophiliac. At two, he bled for three days from a cut on the ear. At three, Frittie and his older brother Ernest burst romping into their mother’s room one morning while she was still in bed. The windows which reached to the floor were open. Frittie tumbled out and fell twenty feet to the stone terrace below. No bones were broken and at first he seemed only shaken and bruised. But bleeding in the brain had begun, and by nightfall Frittie was dead.

The Empress Alexandra was a year-old baby when Frittie died, and she was twelve at the death of Leopold. Neither tragedy struck her personally. Her first meaningful contact with hemophilia occurred when it appeared in her two nephews, the sons of her older sister Irene and Prince Henry of Prussia. One of these boys, a younger Prince Henry, died, apparently of bleeding, at the age of four in 1904, just before the birth of Alexis. His short life was lived behind palace walls and his disease was concealed, probably to hide the fact that hemophilia had appeared in the German Imperial family. The older brother, Prince Waldemar, survived to the age of fifty-six and died in 1945.

Under normal circumstances, the appearance of hemophilia in her uncle, her brother and her nephews should have indicated to Alexandra the possibility that she was carrying the hemophilic gene. The genetic pattern had long been known: it was discovered in 1803 by Dr. John Conrad Otto of Philadelphia and confirmed in 1820 by Dr. Christian Nasse of Bonn. In 1865, the Austrian monk and botanist Gregor Johann Mendel formulated his law of genetics, based on twenty-five years of cross-breeding garden peas. In 1876, a French doctor named Grandidier declared that “all members of bleeder families should be advised against marriage.” And by 1905, a year after Alexis was born, Dr. M. Litten, a New Yorker, had had sufficient experience with the disease to write that hemophilic boys should be supervised while playing with other children and that they should not be subjected to corporal punishment. “Bleeders with means,” he added, “should take up some learned profession; if they are students, dueling is forbidden.”

Why, then, did it come as such an overwhelming shock to Alexandra that her son had hemophilia?

One reason suggested by the late British geneticist J. B. S. Haldane is that although the genetic pattern was known to doctors, this knowledge never penetrated the closed circles of royal courts: “It is predictable,” wrote Haldane, “that Nicholas knew that his fiancee had hemophiliac brothers although nothing is said in his diaries or letters, but by virtue of his education, he attached no importance to this knowledge. It is possible that they or their counselors consulted doctors. We do not know and doubtless will never know if … the court doctor counseled against marriage. If a distinguished doctor outside court circles had desired to warn Nicholas of the dangerous character of his approaching marriage, I do not believe he would have been able to do it, either directly or in the columns of the press. Kings are carefully protected against disagreeable realities.… The hemophilia of the Tsarevich was a symptom of the divorce between royalty and reality.”

There is, as Haldane says, no evidence that either Nicholas or Alexandra ever interpreted the laws of genetics to determine their own chance of having a hemophilic son. Almost certainly, both considered the mystery of the disease, of who would and would not be afflicted, to be a matter in the hands of God. This also seems to have been the attitude of Queen Victoria, who apparently did not understand the hereditary pattern of the disease she had spread so widely. When one of her grandchildren died in childhood, she wrote simply, “Our poor family