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Nicholas and Alexandra Page 20


  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 fiancée 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 o
f her grandchildren died in childhood, she wrote simply, “Our poor family seems persecuted by this awful disease, the worst I know.”

  If Alexandra was surrounded by hemophilic relatives before she married, so were most of the princesses of Europe. So numerous were Queen Victoria’s royal progeny—nine children and thirty-four grandchildren—that the defective gene had been spread far and wide. In marrying and having children, hemophilia was considered one of the hazards royal parents faced, along with diphtheria, pneumonia, smallpox and scarlet fever. Royal princes, even those who were heirs to a throne, did not shy away from a prospective mate because there was hemophilia in her family. Prince Albert Victor of England, who, had he lived, would have been king in place of his younger brother, George V, sought Princess Alix’s hand before Nicholas won it. Had they married, hemophilia would have come down through the line of the British royal family. Kaiser William II was surrounded on all sides by hemophilia. He and his six sons escaped, but his uncle and two of his nephews were victims. William himself was in love with Ella, Empress Alexandra’s older sister. Had Ella married William instead of Grand Duke Serge (they were childless), the Kaiser also might have had a hemophilic heir.

  In that era, every family, including royal families, had a long string of children and expected to lose one or two in the process of growing up. The death of a child was never a casual experience, but it rarely brought the life of a family to more than a temporary halt. Nevertheless, in Alexandra’s case the mere threat of death of her youngest child involved her totally, and through her, the fate of an ancient dynasty and the history of a great nation. Why was this so?

  It is important to understand what the birth of Alexis meant to Alexandra. Her greatest desire after her marriage had been to give the Russian autocracy a male heir. Over the next ten years, she had four daughters, each healthy, charming and loved, but still not an Heir to the Throne. The Russian crown no longer passed down through the female as well as the male line, as it had to the daughters of Peter the Great and to Catherine the Great. Catherine’s son, Tsar Paul, hated his mother and changed the law of succession so that only males could inherit the throne. Thus, if Alexandra could not produce a son, the succession would pass first to Nicholas’s younger brother Michael and after that, into the family of his uncle Grand Duke Vladimir. Each time Alexandra became pregnant, she prayed fervently for a boy. Each time, it seemed, her prayers were ignored. When Anastasia, their fourth daughter, was born, Nicholas had to leave the palace and walk in the park to overcome his disappointment before facing his wife. The birth of the Tsarevich, therefore, meant far more to his mother than the arrival of just another child. This baby was the crowning of her marriage, the fruit of her hours of prayer, God’s blessing on her, on her husband and on the people of Russia.

  All who saw the Empress with her infant son in those first months were struck with her happiness. At thirty-two, Alexandra was tall, still slender, with gray-blue eyes and long red-gold hair. The child in her arms appeared to be glowing with health. “I saw the Tsarevich in the Empress’s arms,” wrote Anna Vyrubova. “How beautiful he was, how healthy, how normal, with his golden hair, his blue eyes, and his expression of intelligence so rare for so young a child.” Pierre Gilliard first saw the Tsarevich when his future pupil was eighteen months old. “I could see she [Alexandra] was transfused by the delirious joy of a mother who had at last seen her dearest wish fulfilled. She was proud and happy in the beauty of her child. The Tsarevich certainly was one of the handsomest babies one could imagine, with lovely fair curls, great grey-blue eyes under the fringe of long curling lashes and the fresh pink color of a healthy child. When he smiled, there were two little dimples in his chubby cheeks.”

  Because she had waited so long and prayed so hard for her son, the revelation that Alexis suffered from hemophilia struck Alexandra with savage force. From that moment, she lived in the particular sunless world reserved for the mothers of hemophiliacs. For any woman, there is no more exquisite torture than watching helplessly as a beloved child suffers in extreme pain. Alexis, like every other child, looked to his mother for protection. When he hemorrhaged into a joint and the pounding pain obliterated everything else from his consciousness, he still was able to cry, “Mama, help me, help me!” For Alexandra sitting beside him, unable to help, each cry seemed a sword thrust into the bottom of her heart.

  Almost worse for the Empress than the actual episodes of bleeding was the terrible Damoclean uncertainty of hemophilia. Other chronic diseases may handicap a child and dismay the mother, but in time both learn to adjust their lives to the medical facts. In hemophilia, however, there is no status quo. One minute Alexis could be playing happily and normally. The next, he might stumble, fall and begin a bleeding episode that would take him to the brink of death. It could strike at any time in any part of the body: the head, nose, mouth, kidneys, joints, or muscles.

  Like Queen Victoria’s, Alexandra’s natural reaction was to overprotect her child. The royal family of Spain put its hemophilic sons in padded suits and padded the trees in the park when they went out to play. Alexandra’s solution was to assign the two sailors to hover so closely over Alexis that they could reach out and catch him before he fell. Yet, as Gilliard pointed out to the Empress, this kind of protection can stifle the spirit, producing a dependent, warped and crippled mind. Alexandra responded gallantly, withdrawing the two guardians to permit her son to make his own mistakes, take his own steps and—if necessary—fall and bruise. But it was she who accepted the risk and who bore the additional burden of guilt when an accident followed.

  To maintain the balance which provides adequate protection as well as attempting a degree of normalcy is a cruel strain for a mother. Except when the child is asleep, there are no hours of relaxation. The toll on the Empress was like battle fatigue; after too long a period of sustained alertness, her emotions were drained. This often happens to soldiers in war, and when it does, they are withdrawn from the front to rest. But for the mother of a hemophiliac there is no withdrawal. The battle goes on forever and the battlefield is everywhere.

  Hemophilia means great loneliness for a woman. At first, when a hemophilic boy is born, the characteristic maternal reaction is a vigorous resolve to fight: somehow, somewhere, there must be a specialist who can declare that a mistake has been made, or that a cure is just around the corner. One by one, all the specialists are consulted. One by one, they sadly shake their heads. The particular emotional security that doctors normally provide when confronting illness is gone. The mother realizes that she is alone.

  Having discovered this and accepted it, she begins to prefer it that way. The normal world, going about its everyday life, seems coldly unfeeling. Since the normal world cannot help and does not understand, she prefers to cut herself off from it. Her family becomes her refuge. Here, where sadness need not be hidden, there are no questions and no pretensions. This inner world becomes the mother’s reality. So it was for the Empress Alexandra in the little world of Tsarskoe Selo. Alexandra, trying to control the waves of anxiety and frustration that kept rolling over her, sought answers by throwing herself into the Church. The Russian Orthodox Church is an emotional church with a strong belief in the healing power of faith and prayer. As soon as the Empress realized that no doctor could aid her son, she determined to wrest from God the miracle which science denied. “God is just,” she declared, and plunged into renewed attempts to win His mercy by the fervent passion of her prayers.

  Hour after hour, she prayed, either in the small room off her bedroom or in the palace chapel, a darkened chamber lined with silken tapestries. For greater privacy, she established a small chapel in the crypt of the Fedorovski Sobor, a church in the Imperial Park used by the household and soldiers of the Guard. Here, alone on the stone floors, by the light of oil lamps, she begged for the health of her son.

  In periods when Alexis was well, she dared to hope. “God has heard me,” she cried. Even as the years passed and one hemorrhage followed another, Alexand
ra refused to believe that God had deserted her. Instead, she decided that she herself must be unworthy of receiving a miracle. Knowing that the disease had been transmitted through her body, she began to dwell on her own guilt. Obviously, she told herself, if she had been the instrument of her son’s torture, she could not also become the instrument of his salvation. God had rejected her prayers; therefore she must find someone who was closer to God to intercede on her behalf. When Gregory Rasputin, the Siberian peasant who was reported to have miraculous powers of faith healing, arrived in St. Petersburg, Alexandra believed that God had at last given her an answer.

  For most young mothers of hemophilic sons, encircled by corrosive fear and ignorance, hope is thin and help is uncertain. The greatest support which any woman can have in this lonely torment is the love and understanding of her husband. In this respect, Nicholas’s contribution was remarkable. No man ever was gentler or more compassionate to his wife, or spent more time with his afflicted son. However this last Russian tsar may be judged as a monarch, his behavior as a husband and father was something which shone nobly apart.