Nicholas and Alexandra (25 page)

More than anyone else outside the family, Pierre Gilliard understood the nature of hemophilia and what it meant to the Tsarevich and his family. His understanding developed gradually. He came to Russia from Switzerland in 1904 at the age of twenty-five. In 1906, he began tutoring Alexis's sisters in French. For six years, he came to the palace almost every day to tutor the girls without ever really knowing the

Tsarevich. He saw the boy as a baby in his mother's arms; later he caught glimpses of him running down a corridor or out in the snow riding his sled, but nothing more. Of Alexis's disease the tutor was almost completely ignorant.

"At times, his visits [to his sisters' classroom] would suddenly cease and he would be seen no more for a long time," Gilliard wrote. "Every time he disappeared, the palace was smitten with the greatest depression. My pupils' [the girls] mood was melancholy which they tried in vain to conceal. When I asked them the cause, they replied evasively, 'Alexis Nicolaievich is not well.' I knew that he was prey to a disease . . . the nature of which no one told me."

In 1912, at the request of the Empress, Gilliard began tutoring Alexis in French. He found himself confronted with a nine-and-a-half-year-old boy "rather tall for his age ... a long, finely chiseled face, delicate features, auburn hair with a coppery glint, and large grey-blue eyes like his mother. . . . He had a quick wit and a keen, penetrating mind. He surprised me with questions beyond his years which bore witness to a delicate and intuitive spirit. Those not forced to teach him habits of discipline as I was, could quickly fall under the spell of his charm. Under the capricious little creature I had first known, I discovered a child of a naturally affectionate disposition, sensitive to suffering in others just because he suffered so much himself."

Gilliard's first problem was establishing discipline. Because of her love and fear for him, the Empress could not be firm with her son. Alexis obeyed only the Tsar, who was not always present. His illness interrupted his lessons for weeks at a time, sapping his energy and his interest, so that even when he was well he tended to laziness. "At this time, he was the kind of child who can hardly bear correction," Gilliard wrote. "He had never been under any regular discipline. In his eyes, I was the person appointed to extract work from him. ... I had the definite impression of his mute hostility. ... As time passed, my authority took hold, the more the boy opened his heart to me, the better I realized the treasures of his nature and I began to feel that with so many precious gifts, it was unjust to give up hope."

Gilliard also worried about the isolation which surrounded Alexis. Princes inevitably live outside the normal routine of normal boys and, in Alexis's case, this isolation was greatly intensified by his hemophilic condition. Gilliard was determined to do something about it. His account of what happened—of the decision by Nicholas and Alexandra to accept his advice and of the anguish they and Alexis suffered when a bleeding episode ensued—is the most intimate and moving eye-

witness account available of how life was really lived in the inner world of Tsarskoe Selo:

"At first I was astonished and disappointed at the lack of support given me by the Tsaritsa," wrote Gilliard. . . . "Dr. Derevenko [co-incidentally, the Tsarevich's doctor had the same name as his sailor attendant, although the two were unrelated] told me that in view of the constant danger of the boy's relapse and as a result of the religious fatalism developed by the Tsaritsa, she tended to leave the decision to circumstance and kept postponing her intervention which would inflict useless suffering on her son if he were not to survive. . . ."

Gilliard disagreed with Dr. Derevenko. "I considered that the perpetual presence of the sailor Derevenko and his assistant Nagorny were harmful to the child. The external power which intervened whenever danger threatened seemed to me to hinder the development of will-power and the faculty of observation. What the child gained possibly in safety, he lost in real discipline. I thought it would be better to give him more freedom and accustom him to resist the impulses of his own motion.

"Besides, accidents continued to happen. It was impossible to guard against everything and the closer the supervision, the more irritating and humiliating it seemed to the boy and the greater the risk that it would develop his skill at evasion and make him cunning and deceitful. It was the best way to turn an already physically delicate child into a characterless individual without self-control and backbone even in the moral sense.

"I spoke ... to Dr. Derevenko, but he was so obsessed by fears of a fatal attack and so conscious of the terrible responsibility that devolved on him as a doctor that I could not bring him around to share my view. It was for the parents and the parents alone to take a decision which might have serious consequences for their child. To my great astonishment, they entirely agreed with me and said they were ready to accept all risks of an experiment on which I did not enter myself without terrible anxiety. No doubt they realized how much harm the existing system was doing to all the best in their son and if they loved him to distraction . . . their love itself gave them the strength to run the risk of an accident . . . rather than see him grow up a man without strength of character. . . . Alexis Nicolaie-vich was delighted at this decision. In his relations with his playmates, he was always suffering from the incessant supervision to which he was subject. He promised me to repay the confidence reposed in him.

"Everything went well at first and I was beginning to be easy in my

mind when the accident I had so much feared happened without warning. The Tsarevich was in the classroom standing on a chair, when he slipped and in falling hit his right knee against the corner of some piece of furniture. The next day he could not walk. On the day after, the subcutaneous hemorrhage had progressed and the swelling which formed below the knee rapidly spread down the leg. The skin, which was greatly distended, had hardened under the force of the blood and . . . caused pain which worsened every hour.

"I was thunderstruck. Yet neither the Tsar nor the Tsaritsa blamed me in the slightest. So far from it, they seemed intent on preventing me from despairing. . . . The Tsaritsa was at her son's bedside from the first onset of the attack. She watched over him, surrounding him with her tender love and care and trying a thousand attentions to alleviate his sufferings. The Tsar came the moment he was free. He tried to comfort and amuse the boy, but the pain was stronger than his mother's caresses or his father's stories and moans and tears began once more. Every now and then, the door opened and one of the Grand Duchesses came in on tiptoe and kissed her little brother, bringing a gust of sweetness and health into the room. For a moment, the boy would open his great eyes, around which the malady had already painted black circles, and then almost immediately, close them again.

"One morning I found the mother at her son's bedside. He had had a very bad night. Dr. Derevenko was anxious as the hemorrhage had not stopped and his temperature was rising. The inflammation had spread and the pain was worse than the day before. The Tsarevich lay in bed groaning piteously. His head rested on his mother's arm and his small, deadly white face was unrecognizable. At times the groans ceased and he murmured the one word, 'Mummy.' His mother kissed him on the hair, forehead, and eyes as if the touch of her lips would relieve him of his pain and restore some of the life which was leaving him. Think of the torture of that mother, an impotent witness of her son's martyrdom in those hours of anguish—a mother who knew that she herself was the cause of those sufferings, that she had transmitted the terrible disease against which human science was powerless. Now I understood the secret tragedy of her life. How easy it was to reconstruct the stages of that long Calvary."

CHAPTER TWELVE

A Mother's Agony

Hemophilia is as old as man. It has come down through the centuries, misted in legend, shrouded with the dark dread of a hereditary curse. In the Egypt of the Pharaohs, a woman was forbidden to bear further children if her firstborn son bled to death from a minor wound. The ancient Talmud barred circumcision in a family if two successive male children had suffered fatal hemorrhages.

Because over the last one hundred years it has appeared in the ruling houses of Britain, Russia and Spain, it has been called "the royal disease." It has also been called "the disease of the Hapsburgs"; this is inaccurate, for no prince of the Austrian dynasty has ever suffered from hemophilia. It remains one of the most mysterious and malicious of all the genetic, chronic diseases. Even today, both the cause and the cure are unknown.

In medical terms, hemophilia is an inherited blood-clotting deficiency, transmitted by women according to the 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.*

* At the heart of the problem of hemophilia are the genes which issue the biochemical instructions that tell the body how to grow and nourish itself. Gathered in curiously shaped agglomerations of matter called chromosomes, they

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

are probably the most intricate bundles of information known. They determine the nature of every one of the trillions of highly specialized cells that make up a human being. Scientists know that the defective gene which causes hemophilia appears on one of the female sex chromosomes, known as X chromosomes, but they have never precisely pinpointed the location of the faulty gene or determined the nature of the flaw. Chemically, most doctors believe that hemophilia is caused by the absence of some ingredient, probably a protein factor, which causes normal blood to coagulate. But one eminent hematologist, the late Dr. Leandro Tocantins of Philadelphia, believed that hemophilia is caused by the presence of an extra ingredient, an inhibitor, which blocks the normal clotting process. Nobody really knows.

There is a remote prospect that current research into the structure of chromosomes will help hemophiliacs. If it should become possible to locate the genes responsible—and then to correct or substitute for the faulty gene-hemophilia could be cured. But medical researchers hold out little hope for the immediate future. So far, science has been unable to change genetic characteristics in any form of life except bacteria.

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."