AN INHERITANCE NO ONE DESIRED

What Was the Real Medical Tragedy Faced by Russia’s Last Imperial Heir?

Margarita Nelipa and Helen Azar

Introduction

Hemophilia, a serious disorder that affects the clotting of blood, became known as the Royal Disease in the 19^th century after a number of male members belonging to the European Royal Houses suddenly presented with this incurable and devastating condition. Although its distressing appearance in several family branches of Queen Victoria’s royal descendants made some medical waves during that time, hemophilia gained most of its historical notoriety in the early 20^th century through the well-known legend of Grigori Efimovich Rasputin[1] and his alleged unique ability to stop the bleeding of the hemophiliac heir to the Imperial Russian throne. Sadly the life of Tsesarevich Alexei who suffered from this incurable blood disorder would often hang by a thinnest thread while the best specialists, both in the Empire and abroad, could only helplessly shrug their shoulders in the face of each respective bleeding episode, knowing that there was little that could be done except alleviate his considerable pain.  But, as the legend goes, Rasputin - an uneducated Siberian peasant with no formal medical background seemed to be able to stop the bleeding and appeared to save the boy’s life more that once. As a consequence, Rasputin was to gain the complete trust and confidence of the Emperor and the Empress who referred to him as a “Man of God.”[2]

Many remain convinced that it was Rasputin who was responsible for launching the Russian Empire into its cataclysmic revolutionary downfall, which included the violent demise of the entire imperial family. Even during his own lifetime, Rasputin grew - for better or worse – into a legendary figure, while hemophilia came to be closely associated with monumental historical events ever since.

Although official State documents never mentioned the term “hemophilia” when referring to the heir to the Russian throne, it had become an accepted fact that the boy had indeed suffered from this disease - a condition that put him at constant daily risk of literally bleeding to death. This has been an unquestioned fact until recently, when some surprising doubts as to the nature of the Tsesarevich’s medical condition had arisen in the form of a Historical Perspective investigative article published in a respected medical journal – the American Journal of Hematology (A. J. H.).

The article’s author, John Kendrick, a Canadian journalist argues that Alexei did not have hemophilia after all. Kendrick introduces a number of reasons he believes to be compelling enough to make a case against the traditional historic contention that, he claims, convinced him of the fact that Alexei may have suffered not from hemophilia but a non-fatal blood ailment that was acquired sometime during his short life.

Could it be possible that Alexei did not have hemophilia but another similar condition that was misdiagnosed as hemophilia?  While it is unsafe to provide a clinical diagnosis for anyone who died that long ago, in the absence of complete medical records, we believe that it is possible to form a reasonable assessment from diary extracts, memoirs and other documented historical and medical facts to determine whether Alexei’s condition was indeed hemophilia. This is precisely what we have set out to do in the pages you are about to read.

Birth of the Long Awaited Heir

“An unforgettable great day for us, during which we were clearly visited by the grace of God. At 1.15 during the day Alix gave birth to a son, whom in prayer we have named Alexei” [3]

Such were the first words written into the diary by the new father, who happened to be the Emperor of Russia, Nicholas II, at the Peterhof Palace on 30 July 1904.  Despite the simplicity of these words, Alexei would mature into a handsome youth challenged every day with complex problems throughout his brief life. Few realized why the heir to the Romanov throne was not often seen at formal gatherings. During the times when he was seen by the enthusiastic public, many wondered why he was often assisted, while at other rare ceremonial functions he appeared just like any other little boy.

What was that dark and tragic inheritance which befell the Tsesarevich?

The Russian people became familiar with Alexei mostly by the prolific publication of official photographs in city newspapers and magazines, always showing what appeared to be a bright and exuberant boy. Despite this façade, few outside the immediate family circle were aware of the true nature of Alexei’s condition. The reality was that the Romanov dynasty desperately depended on a male heir, to succeed Nicholas II. How could the Russian people be allowed to learn the painful truth that their Tsesarevitch was born with a disease, which could terminate his life at any moment?  Alexei’s condition would be hidden from public scrutiny. This silence became one of Imperial Russia’s greatest State secrets, which unfortunately only lent to wild conjecture and rumor. Understandably, this secret forced the Imperial couple to rally closer together; and remain secreted away their capital city of St. Petersburg, residing in the Alexandrovsky Palace in Tsarskoe Selo some thirty minutes distant by rail.[4] This deliberate action served only to deepen the Imperial Family’s remoteness from their people.

Although little was understood in 1904 about the nature of hemophilia, it would be incorrect to suggest that families affected by this insidious condition, failed to understand its consequences. Familial experience going back to Alexandra’s maternal grandmother Queen Victoria’s own personal trauma in raising her hemophiliac son, Leopold, provided a basic understanding of the trials which the Russian Emperor and his consort were to endure in the coming years. In the words of Grand Duchess Olga, Alexei’s aunt:

“You might well say that the birth of a son, which should have been the happiest in the event of the lives of Nicky and Alicky, became their heaviest cross.” [5]

The boy’s grandmother, Dowager Empress rejoiced in the knowledge that Alexei’s birth signaled to the world that the Romanov Dynasty would remain secure. Sadly within five weeks her jubilation turned to grief when she learned that her long awaited grandson and heir to the Russian Dynasty was stricken with hemophilia.[6] 

While it is unsafe to provide a clinical diagnosis for anyone who died long ago, in the absence of medical records, it is possible to form a reasonable assessment from family diary extracts and memoirs to determine whether the condition was indeed hemophilia.

When Alexei was born in July, 1904, the condition was not so rare as to remain elusive. His personal physicians, Professors Sergei Petrovich Fedorov, Sergei Alekseevich Ostrogorskii and Dr Vladimir Nicholaevich Derevenko, as we shall demonstrate later in this discussion - knew exactly what the symptoms and consequences of hemophilia were.

What is Hemophilia and why was the condition considered fatal?

Hemophilia is a congenital hereditary blood disorder that affects the clotting time of blood following trauma.[7] However, it is not unusual for the affected individual to experience spontaneous hemorrhage episodes, attributed to the constant physical movement of muscles, which are richly supplied with blood.

1. Historic Notes 

This condition was known to exist for centuries. An ancient rabbinical ruling in the Talmud from the second century A.D. exempted male boys from circumcision if two previous brothers had died of bleeding after the procedure.[8] The first modern clinical description of hemophilia can be attributed to Dr. John Conrad Otto, an American physician, wrote a brief account of persons presenting with a bleeding disposition, in 1803. He emphasized the three cardinal elements of hemophilia:[9]

· An inherited tendency

· of males

· to bleed.

Two decades passed before a German medical student, Herr Friedrich Hopff finally introduced the term “hemophilia” in 1828.[10] Another two decades were to pass before the first formal medical article was presented in the English medical Journal Lancet in 1840.[11] Its true cause was finally discovered in 1939.[12]

2. It’s all in the Genes

Soon after the discovery of DNA was completed in 1953[13] genetic research expanded at a rapid pace. From that point there was a better understanding of inheritance at the molecular level.

Hemophilia is occasionally referred to as The Royal Disease[14] in popular literature. In Queen Victoria’s era hemophilia was referred to as The Victorian Disease.[15] The union between Queen Victoria of England and her consort Prince Albert has been considered by geneticists to be the starting point for hemophilia in the British Royal lineage. Queen Victoria (1819 - 1901) unknowingly passed on the defective gene to three of her nine live births, two daughters, Alice and Beatrice, and to one son, Leopold.

The condition passed down through Victoria's descendants affecting three of the European Royal Houses by reason of marriage into the Russian, Spanish and German Royal families.[16] It was Queen Victoria’s daughter Alice who passed on the defective gene to her daughter, Alexandra. Alexandra became the wife of the Emperor of Russia in 1894.[17] Her only son presented with the symptoms of the condition within his first year. His great uncle, Leopold was diagnosed with hemophilia when he was two years of age.[18]  

Hemophilia affects males more frequently (1 in 10,000) than females (1 in 100,000,000).[19] The reason why males are affected in significantly higher numbers is because the blood-clotting gene is recessive and is carried only on the X-chromosome. Males have only a single X-chromosome as part of the XY complement. If that X possesses the defective gene then that male will always have hemophilia.

Females, on the other hand, carry two X-chromosomes (XX). If only a single chromosome is defective, then the other normal X-chromosome will mask that defect. Although the female will be unaffected, she will be considered to be a carrier of that defective gene. Not surprisingly, it would be very rare for two defective X-chromosomes to be present at the same time during conception. Should this occur, then the female will present with a mild form of hemophilia (susceptible to nosebleeds and menstrual dysfunction).[20] In most cases, the female would randomly pass on the defective gene to some of her children. Should a male be born with the defective gene, he would have hemophilia.

Geneticists today remain intrigued how hemophilia first originated in the British Royal Family. Textbooks often introduce this regal example to provide a neat explanation how hemophilia is inherited by family members. We have compiled a simplified flow chart [Table 1] showing the relationship between Alexei and Queen Victoria:

Table 1      Generational relationship between Alexei and Queen Victoria

There were no sufferers in previous generations, and by this observable fact it can be reasonably assumed that a gene in Queen Victoria’s DNA had mutated spontaneously at the time of conception. Statistically one third of all hemophilia cases have no family history of this condition. In this scenario hemophilia occurs as a result of a spontaneous gene mutation.[21] Table 2 below provides possible scenarios as to how the mutation of the hemophilia gene could have presented in Queen Victoria's children:

Table 2.   Possible theories how Queen Victoria introduced the hemophilia gene to her descendants:

It would therefore be plausible to suggest that Queen Victoria was responsible for providing some of her family descendants with the mutated gene. There is documented evidence that hemophilia had affected her own son, and was passed through two of her daughters, onto their own families.

All congenital blood disorders are inherited. Hemophilia is a term with which we are all familiar, but exactly what is hemophilia?

3. A Few Medical Notes

Simply stated it is the defective production of specific clotting factors located on the surface of the cells called platelets that normally course inside blood vessels.

The integrity of the vascular system depends on three interacting elements: platelets, coagulation factors and blood vessels. All three elements are required for efficient hemostatic function (stopping blood flow). The pattern of bleeding will depend on the specific defect of one of the three elements.

Platelets are activated when a blood vessel is damaged as a consequence of an injury. The blood vessel responds by aggregating platelets around the site of vascular damage by forming a platelet plug. The surface proteins, found on the platelets called Factors, are vital in enabling the blood to clot. When there is a platelet dysfunction, the plugging mechanism will take longer to develop. The patient will experience continued internal hemorrhaging, which causes pressure on surrounding muscle tissue, with excruciating pain and inflammation at the site of injury.[22] Fever may be present.  The consequent swelling at the joint restricts movement of the affected limb, which in the long-term will cause disfigurement of the young developing bones.  Spontaneous bruising after minimal trauma is not uncommon. Such bruising is usually located on the extremities of the body, because of the higher capillary and venous pressure in the skin.

Clinically, hemophilia is a severe congenital defect which is due to the inefficiency of either Factor VIII (hemophilia A) or Factor IX (Christmas disease or hemophilia B).  The most common form of this condition is hemophilia A.[23] Identification of the gene responsible for the coding of clotting factors only began in the 1950’s and their interaction in blood was finally reported on Nature in 1964.[24]

Symptomology for both forms are indistinguishable, and can only de distinguished by genetic testing, which identifies the different position on the gene which has mutated. At the molecular level the mutation is due to a deletion or substitution of amino acids on the long arm of the X chromosome.[25]    

It should be stressed that the affected hemophiliac male will usually demonstrate the presence of this inherited condition within the first year of his birth, although some references contend that it can be within two years.[26] Unusual bleeding in the newborn is the hallmark of hemophilia. The more severe the condition, the earlier will it present. As the infant becomes more mobile minor bleeding events and superficial bruising will appear. With maturity into their teens bleeding episodes will increase in frequency and become more complex after trauma. Spontaneous bleeding, although more rare, can occur often affecting the same area of the body as with previous episodes. Hemophilia is a condition for life, it is episodic, and there is no remission. In 1904 it was considered to be a life threatening condition, because there was very little that medicine could do.

Genetic disorders are incurable, because such a fate is determined at the moment of conception. The only practical course of treatment available when Alexei was born was the application of pressure bandages, and immobilization of the affected limb, which meant bed rest. There was no effective cure available at the turn of the nineteenth century for this condition just as it remains today despite technological advancements. The luxury of infusion of therapeutic products and recombinant technology[27] was unavailable during Alexei’s lifetime.

To better appreciate this discussion it is important to realize that hemophilia is classified by the seriousness of the symptoms presented: mild, moderate or severe.[28] That classification corresponds with the level of Factor VIII.[29] [30] The normal range of Factor VIII is 100% for unaffected individuals. In affected individuals, the clotting time of blood will vary depending on the level of Factor XIII detected. In all cases the clotting time will always be longer than that for unaffected individuals.

The defective gene responsible for the insufficient level of Factor VIII is the same gene passed down the family. For this reason family genetics will predetermine the severity of bleeding, which will be constant within families. If Alexei demonstrated moderate symptoms, then the same degree of severity would have been evident with all his affected male relatives.

Table 3.          Relationship between Severity and bleeding in Hemophilia

For the purpose of this discussion the term hemophilia will be used as a general term, because it is difficult to presume which type of hemophilia affected Alexei in the absence of specific laboratory analysis.

4. Common Symptoms of Hemophilia

The main symptoms that Hemophilia sufferers may experience as children are:[32] [33]

Table 4                          Common Hemophilia symptoms

Clearly all these symptoms are observable facts, which do not require laboratory assessment. With maturity the symptoms become more complex. The most common site of bleeding is the joint, while intracranial hemorrhaging is the main cause of death. A fate which a statistically significant number of Alexei’s maternal relatives met.[34] [35]

Distinguishing fact from fallacy

Today, diagnostic tests for hemophilia provide the clinician confirmation that there is qualitative abnormality of Factor VIII (see Table 2). A century ago, such diagnoses were unavailable. Physicians were however able to identify the condition based on observation of the symptoms, assessing its severity and frequency of events. Physicians at the beginning of the last century understood that that family history played a critical role in the assessment of hemophilia. Its inheritability was the main diagnostic tool facilitating its identification.

1. Mr. Kendrick presumes that Alexei’s condition was never confirmed:

“…no blood test result and no known laboratory test for evidence of a faulty carrier gene in any member of the Royal family has ever been found to confirm that hemophilia had ever existed among the descendants of Queen Victoria.”[36]

While no definitive laboratory assays were available at the turn of last century, what the physicians of the day relied upon were physical observations. Each of those observations would have provided a complete record of events Alexei experienced throughout his life. Cumulatively those observations provided a clear trend that would have provided indisputable proof that Alexei had inherited hemophilia from his mother.

The very first time any parent is alerted that there is a problem is by observation. Bleeding from any site on the body will promptly alert parents that there is a problem.

The first observation that alerted Alexei’s parents that there was a problem was noted simply and precisely by Nicholas II in his diary:[37]

Table 5                            Nicholas II Diary entries for 1904

Despite the clarity of these words, Mr. Kendrick interpreted the diary entries to read:

"The first thing they seized upon was four short entries in the Tsar's diary of 1904 that refer to his newborn son's one brief episode of umbilical bleeding during a single evening in early September[3]."[38]

It is more than clear that Alexei was observed to have bled on two separate days. Judging by his father’s plain words, the pattern of bleeding was intermittent on two successive days. It was also spontaneous. It was certainly not brief, nor is there any evidence that bleeding was during a single evening. Mr. Kendrick also appears to have ignored the entry written on 9 September.

2.  To follow on from the first physical observation the following statement featured on Kendrick’s webpage:

 “The boy's symptoms were episodic but hemophilia is not.  It is always there.” [39]

While the symptoms will not present every single day in the life span of any person, hemophilia does not disappear.  That is why medically speaking the condition is identified as being episodic. It is illogical for Mr. Kendrick to presume that bleeding will be continuous throughout the life of the patient. Taking his contention to its conclusion, no hemophiliac would have lived beyond the first few hours of life!

In her memoirs, Baroness Buxhoeveden, an intimate of the Imperial Court explained:

“Alexei Nicholaevitch was perfectly well and strong between his attacks …” [40]

Alexei’s tutor of thirteen years, Pierre Gilliard wrote:

 “Between each of the attacks … the boy came back to life, recovered his health …at these times it was impossible that he was a victim of an implacable disease which might carry him off at any moment.” [41]

3. In the same vein Mr. Kendrick’s next statement in his Historic Perspective is cause for concern:

 “Many new parents who have witnessed such bleeding will understand that it can be fairly ordinary as many as six weeks after birth, depending on when the umbilical stump falls away, which can take as long as a month [4].”  [42]

Interestingly, Mr. Kendrick’s fourth reference actually stated the following:

“The stump of the umbilical cord, which remains temporarily attached to the navel, should be kept clean, dry ... Usually the stump will fall off seven to fourteen days after birth …*Report any bleeding … to your baby's health care provider.” [43]

The Sutter Healthcare site clearly identifies that "any bleeding" is not a normal event. It specifically warns parents that if such an event was observed in their newborn, it is advisable to seek medical attention. To suggest that any bleeding from the umbilicus of a new born is not alarming contradicts medical wisdom.

4. Mr. Kendrick stated that after the initial bleeding episode, there was:

“…nothing in the private diaries of Nicholas and Alexandra that their only son had suffered any type of blood disorder. …the Imperial parents never mentioned the word “hemophilia” [44]

The Emperor believed that had the Russians been aware of Alexei’s true condition, it would have precipitated serious unpredictable political consequences.[45] Russia at the time of Alexei’s birth was at war with Japan. This State Secret would have precluded any mention of his condition whether written or by verbal expression in the Court.[46] Such a precaution would have dictated that any authorship between or by the Imperial couple would never mention “hemophilia”. This cautious approach would have been a prime consideration at all times for both parents and all entrusted Court intimates.

To suggest that the apparent absence of the term “hemophilia” was proof that Alexei did not suffer from that condition is absurd. Alexei’s real medical condition was never printed in accordance with national security. When the war ended internal security issues due to revolutionary insurgency became a new concern for Russia. The Imperial parents had to continue concealing Alexei’s real condition from the world, a decision which itself caused problems for the Imperial family.

It was politically imperative never to compromise Russia and the heir to the throne. With all the political contingencies at Russia’s doorstep, it would be inconceivable to contemplate that the parents would have acted differently. 

5. Mr. Kendrick continues to dispute that Alexei suffered hemophilia on the grounds that:

 “Even with teething in his first year and again when those first baby teeth had fallen out …there is no record whatsoever of the young Alexei ever having shown any evidence of excessive bleeding.” [47]

Children who suffer hemophilia do not usually bleed any more than normal from scratches or superficial cuts and abrasions. This includes replacement of baby teeth by adult teeth.[48] Alexei would probably not have experienced intense problems with his teeth. Nicholas II was silent about this matter in his diary. With scrupulous dental hygiene and consumption of natural unprocessed foods, Alexei would have minimized the main concern of all hemophiliacs, that being the physical extraction of a tooth.[49] Fortunately, he lived in an era where processed carbohydrates would not have been part of his dietary intake. Alexei’s dentists would have understood about decay minimization.   

6. According to Kendrick, following the Emperor’s abdication in 1917, Anna Vyrubova (Alexandra’s confidant) presented the following testimony at some unspecified interview:

“The child had a rare disease … The blood vessels were affected so that the patient bled at the slightest touch.” [50]

Similarly, Kendrick followed that excerpt with one presented from Lili Dehn’s 1922 memoir:

“…the Tsarevich certainly suffered from the hereditary trouble of thin blood vessels ” [51] 

Both excerpts used by Mr. Kendrick were similarly worded expressions. Their use in his Perspective permitted this conclusive remark:

 “They all indicated that the boy’s problem was not in the blood itself, but in the vessels that carried the blood.” [52]

Neither of the women whose excerpts Mr. Kendrick chose to use was medically trained. It would not be unreasonable to suggest that they attempted to describe Alexei’s condition within the limitations of their knowledge. However both the descriptions provided (above) by the Court intimates should not be discounted for their simplicity.

Historically, fragility of blood vessels was considered to be the cause of hemophilia. Several decades after the memoirs were written, science moved forward. Medical research found that it was not the blood vessels per se but the blood components within those vessels.

When there is vascular damage following injury, there will be a continuous flow of blood that accumulates outside the ruptured blood vessel, until sufficient Factor VIII enables a platelet plug to form after a considerable lag time.[53] In unaffected individuals, following vascular damage, the reverse occurs. Vasoconstriction enables a decrease in blood flow, and sufficient Factor VIII is activated to rapidly form a platelet plug. Bleeding then stops.

To attach reliance on dated descriptions of hemophilia is misleading.  Knowledge about the nature of hemophilia has advanced significantly since the date both quoted excerpts were written.

7. Kendrick was incorrect to suggest that:

“…the Imperial Court had all described a condition that more closely matches … not of hemophilia but of purpura: a disease characterized by purple or livid spots under the skin” [54]

A purpura (Latin word for purple) forms after vascular injury. Capillaries, being the smallest, thinnest blood vessels in the body are located close to the skin. They are the conduit for blood to reach all extremities of the body. When these vessels are damaged, blood will flow uncontrollably for a longer time and pool underneath the skin. What develops is a purpura – purple colored patches. They are temporary lesions, ranging between 2 mm and 1 cm in diameter.[55] A purpuric lesion can persist for weeks and will be absorbed by the body. However it takes weeks to resolve naturally. It is not difficult to imagine that excruciating pain would be present because the blood has nowhere to dissipate.

When hemophilia is also present purpura are associated with subcutaneous swelling. The observable symptom is a dark blue swelling (hematoma), usually on his arms and legs.[56]

Purpura can be due to numerous predisposing factors, not just in hemophilia. Purpuric lesions are a sign of a life threatening condition[57] and have vastly different etiologies.[58]  

Had Kendrick availed himself of a basic medical dictionary, he would have been immediately informed that purpura is a symptom of a pre-existing condition, it is not a disease.

8. Mr. Kendrick contends that persons close to Alexandra never used the term “hemophilia”:

“Just as Nicholas and Alexandra had never used the word hemophilia … neither did the Empress Alexandra’s closest friends.” [59]

A number of references exist that directly contradict Kendrick’s assertion. Not only have a number of persons intimate with the Imperial parents have used that specific definition, but two members of the Romanov family did the same. 

8.1 The first reference came from Anna Vyrubova’s unpublished Memoirs.[60] This first reference clearly proves that the term hemophilia was used by an intimate member of the Imperial Court. In her published memoirs, Vyrubova wrote:

“…it turned out that the child was far from healthy. A medical examination showed internal bleeding. …soon the doctors established that the Tsesarevich had a terrible incurable disease – hemophilia.”

In her 1923 published memoirs, Vyrubova wrote:

“… for almost at once they learned that the poor child was afflicted with a dreadful disease, rather rare except in royal families where it is only too common. The victims of this malady are known in medicine as haemophiliacs, or bleeders.” [61]

8.2 Grand Duchess Marie Pavlovna (Jr.) was a member of the Romanov family who penned her memories in exile in 1930.She noted the following:

 “…from his birth he carried in him the seeds of an incurable illness, hemophilia – a tendency to bleed easily.” [62] 

8.3 Another of Alexei’s relatives, Grand Duke Cyril Vladimirovich became the head of the Romanov Dynasty en exile after the death of Alexei. In his 1939 memoir he confirmed what Grand Duchess Marie Pavlovna stated a few years earlier:

“Unfortunately he was a haemophiliac and whenever he cut or hurt himself in any way which caused a loss of blood, it was impossible to stop the bleeding. …The heir to the throne was doomed.” [63]

8.4 The Commandant of the Alexandrovsky Palace, General-Major Vladimir Nikolayevich Voyeikov was one of the Emperor’s closest personal associates. He noted in his 1930 memoirs that the court physicians informed the Emperor that:

“…the Tsesarevich will never be cured from his illness. Attacks from hemophilia will from time to time appear. It is essential to take the most extreme measures, to protect His Majesty from falls, cuts and even scratches, because every slight bleeding may be for persons, suffering Hemophilia, may prove fatal.” [64] 

8.5 Alexandra’s Chief Lady-in-Waiting, Baroness Sophie Buxhoeveden, was another Court intimate who used the term hemophilia in her 1930 memoirs:

“As the child grew older and more active, he developed the typical swellings that pointed to haemophilia, the dreadful disease from which one of her uncles had suffered …and the Empress realized that her beloved ‘Sunbeam’ had the same terrible weakness.” [65]

8.6  Alexei’s tutor Pierre Gilliard explained in his 1922 memoir, that Dr Vladimir Nikolaevich Derevenko, who was Alexei’s personal physician had informed him during the Spala incident (see below) that:

“…the Heir was prey to haemophilia, a hereditary disease which in certain families is transmitted from generation to generation by women to their male children.” [66]

“He told me that the slightest wound might cause the boy’s death, for the blood of a bleeder had not the power of coagulating like a normal individual.” [67]

This final appraisal that was reiterated by Alexei’s physician correctly identified the cause of Alexei’s illness almost ninety years ago!

Had Mr. Kendrick earnestly consulted a few first-hand accounts it would have become clear that the term hemophilia was indeed used by members who were either the closest employees of the Court or members of the Imperial family. Importantly each author understood the implications of Alexei’s condition. 

To take this matter a little further, a number of memoirists expressed the following insight concerning Alexei’s incurable illness.

The first comment comes from Grand Duchess Marie (Junior):

 “…the parents were quickly advised as the nature of their son’s illness.” [68]

While this second comes from Baroness Buxhoeveden:

“Doctor’s confirmed her fears … Her agony was the more acute as she knew that it was through her that the boy had inherited the illness.” [69]

Anna Vyrubova expressed the following agony that faced the Imperial parents:

“Frequently they die soon at birth, and those who survive are subject to frightful suffering, if not sudden death. …The whole short life of the Tsarevich … was a succession of agonizing illnesses due to this congenital affliction.” [70]

Finally, Grand Duke Cyril provided the following awful truth that confronted the parents:

“All the celebrities of the medical world had been consulted, but in vain.” [71]

Had Mr. Kendrick closely examined a few Russian memoirs, he would have been more than assured that physicians knew what hemophilia was, and that they were compelled to explain to His Majesty Nicholas II the truth of the awful hand of fate extended to the heir of the Imperial throne. Grand Duke Cyril expressed it best with the following:

“The Empress and Emperor were desperate in the presence of this incurable ill and realized that science was unable to save the child.” [72]

 9. Perhaps the most profound misrepresentation made by Mr. Kendrick in his Historic Perspective was the following statement:

“The first real evidence of the Tsarevich Alexei’s blood disorder does not appear in historical records until two months after his eighth birthday.” [73]

Anna Vyrubova, Alexandra’s personal companion provided a significant entry in her unpublished memoirs in which she noted that; in his first year, Alexei had caught his leg in a folding chair and

“...screamed like a wounded animal …the Tsesarevich’s leg was bruised and swollen.” [74]

Normal blood clotting restricts bruising after injury, but in Alexei’s case it is apparent here that he bruised rapidly.

Madame Vyrubova’s in her published memoir expanded on Alexei’s bruising events before he turned five years of age:

“…nothing more heat rendering could be imagined than the little boy’s moans and cries during his frequent illnesses. If he bumped his head or struck a hand or foot …the usual result was a hideous blue swelling indicating a subcutaneous hemorrhage frightfully painful and often enduring for days or even weeks.”  [75]

Everyone would have understood the legacy which Alexandra Feodorovna brought from the Hesse-Darmstadt House. General-Major Voyeikov, expressed the following profound admission in his memoirs:

“…the innocent young boy would suffer from the negligence revealed by the Russian court in the choice of bride for Nicholas II.” [76]

9.1 Appointment of Special Carers

All possible precautions were taken by the family to be prepared for the next crisis. The Imperial’s family priority was always focused around Alexei's health and well being. Doctors were in attendance day and night. When Alexei turned five,[77] two naval personnel were appointed to look attend him by co-ordinated rotation; the sailor Klimenti Nagorny,[78] and Boatsman Andrei Eremeyevich Derevenko from the Imperial yacht Standart,[79] [80] along with two other aides,[81] Maria Vishnyakova – a nurse and Ivan Dmitriyevich Sednev acting as a footman. Alexei was never left alone throughout his life. Each was entrusted to supervise Alexei’s daily activities in order to minimize the risk of falls,[82] but also enable Alexei to enjoy low risk outdoor activities.

Alexei’s tutor, Gilliard described that:

 “…all that could be done was to watch over him closely day and night, especially in his early years and by extreme vigilance try to prevent accidents.” [83]

It would be rather strange that a special contingent of support staff would need to be employed had Alexei not suffered from hemophilia, including an entourage of Russia’s best physicians and pediatricians made available for Alexei’s exclusive use.

9.2    Photographic Evidence

One of the earliest published photographic demonstrating Alexei’s inability to walk shows Nicholas II carrying Alexei on the deck of the yacht in 1906.[84] It is more than apparent that Alexei was unable to walk alongside his father. Another published image shows Alexei enfolding his arms around Fligel-Adjutant Nicholas Sablin neck for support.[85] Other photographs exist with similar images when Alexei was six.[86]  One such photograph clearly shows an obvious large swollen mass on the inner side just above Alexei’s left kneecap. In that image he is being held by Nagorny who himself is standing in the water.[87]

In Vyrubova’s published memoir, there is a photograph of very young Alexei lying clothed on the bed with his mother. This photograph is identified as a “convalescence from a serious illness”.[88]

There is a series of three very telling photographs of Alexei taken in 1910, when the family was visiting their Hesse relatives in Germany. At the age of six he was sitting on a special curved cushioned seat with handles and foot support, positioned at the front of a bike. Alexei was being pushed by Derevenko, who cycled for the both of them.[89] [90] [91]

It is impossible to overlook the photographic evidence that Alexei was indeed experiencing periodic hemophiliac episodes throughout his short life. Because convalescence takes time after each hemophiliac crisis, Alexei was always carried by his caregivers. These are all observable facts. The fact that Mr. Kendrick chose to ignore these obvious pieces of evidence is ignoring the full picture.

9.3 Minimization of Physical Activity

Low risk activities such as swimming and walking[92] that cause relatively little stress on joints were suitable activities, which Alexei was able to enjoy. Any contact sports such as tennis would have been prohibited because of the risk of head or neck injury.[93] Such injuries would prove fatal as discussed previously. Often he would ask tearfully as a youngster why he was unable to play like other boys.[94] Vyrubova explained that when Alexei grew to an age where he could understand; his parents had to explain to him:

“… the nature of his illness and impressed upon him the necessity of avoiding falls and blows.” [95]

Vyrubova described that a few select playmates from the Military Academy School were entrusted to play with Alexei. These boys were warned of their responsibility and avoid rough activities. For this reason the presence of children of Grand Dukes was curbed.[96]

As a child becomes more active with age, the bleeding pattern alters. Hemorrhage into the muscles and joints become a more painful and debilitating problem. This is exactly what occurred in Spala (see below).

There is no need to describe every hemophiliac event that Alexei experienced through out his short life. The evidence was always present every single day of Alexei’s life. Anna Vyrubova stressed in her memoir that while the Empress whose responsibility it was to tend the upbringing of their children:

“The only care she brought to the Emperor was the ever precarious health of Alexei.” [97]

It is quite misleading for Mr. Kendrick’s to claim that Laboratory assays would be the only acceptable form of proof to confirm that Alexei was a hemophilia sufferer. Had Mr. Kendrick examined this matter properly he would have identified that sufficient valid evidence existed. That evidence was based on clear observations. Such observations are the same upon which all clinicians still rely as an adjunct to laboratory assessment.

Had appropriate laboratory assays existed in Alexei’s life, all they would have confirmed is its severity - a factor the family challenged everyday in Alexei’s life. By extension Mr. Kendrick is implying that in the absence of laboratory assays then all historic medical records must be considered unsafe!

The Polish Incident

A Duality of Events

The most harrowing trial for the Imperial Family proved to be the autumn of 1912 when Alexei was turning eight.[98] Whilst vacationing in Bialovieza,[99] Poland, Alexei sprained his ankle jumping into a rowing boat that caused profuse bleeding.[100] [101]  This incident signaled his most serious crisis to date. Within one week of Alexei’s injury he stabilized sufficiently to permit him to be moved to the Spala hunting lodge.[102] However, within days his health rapidly deteriorated.

It is completely misleading for Mr. Kendrick to contend that:

“The first real evidence of ... a blood disorder … does not appear in the historical records until two months after his eighth birthday, with the start of his first and most serious hemorrhage episode … in October.” [103]

Unfortunately, Kendrick appears to have ignored that the first real evidence was published from Nicholas II diary featuring the historically and medically important entries for September 1904. Those records provide detail about the very first bleeding event that appeared spontaneously in the abdominal region a few weeks after birth.

“…there has always been serious doubt about what might have been the initiating cause of that first near fatal episode.” [104]

Kendrick’s assertion here is very misleading. Unfortunately the author fails to provide any clear evidence to support this assertion. The single French language reference that was cited did not have the correct citation; thus we were unable to check it. To suggest that a single reference constitutes a majority opinion is absurd.

The official published Medical Bulletin, signed by the Minister of the Imperial Court, General-Adjutant Baron Fredericks on 21 October, provided a very clear statement. It specified:

“…in the first few days of his stay in Bialovieza …the Tsarevich Heir made a very large step while jumping into a boat.” [105]

The first accident in Poland occurred on September 7, not in October as Kendrick has alleged.

A number of first-hand accounts are available that describe this first episode. Both Alexei’s father and the attending physicians identified the initial crisis, as have Vyrubova[106] and Buxhoeveden[107] in their memoirs. These last three references also provided the cause of that initial crisis. The Emperor wrote in his diary entry of 5 October[108] – that there was a prior event, several hours before the onset of the second near fatal crisis was to take hold of everyone present.

The official Medical Bulletin continued with the following description: 

“As a result of requisite rest and corresponding treatment, within three weeks the hemorrhage became so imperceptible that it could hardly be felt and the patient was beginning to stand up.” [109]

Clearly, the very first bleeding event lasted three weeks however it was not completely resolved. Any reader of that Bulletin will learn that while Alexei was recovering he attempted to walk on the 28 September. Due to his previous prolonged immobilization from the first accident with unsteady gait he fell. This second fall precipitated his re-injury causing a new hemorrhage in the same region as the first injury within days of that second fall.[110]

“… that an unbelievably long period of time passed between the first bruising in the boat and sudden onset of Alexei’s most serious internal hemorrhage” [111]

The Medical Bulletin succinctly described that the second fall compounded the initial September episode in Bialovieza. Due to the proximity of the first crisis, with incomplete recovery and subsequent damage at the same site, one cannot conclude that both falls were distinct and separate events! Alexei’s medical practitioners provided a nexus between the Bialovieza and the Spala crises.

It was a duality of increasing problems.  We believe that this duality of events has essentially been ignored until we investigated the drama in its entirety for ourselves.

Armed with this information it is completely erroneous for Mr. Kendrick’s to allege that:

 “The report had also stated that the newer and far more serious hemorrhage at Spala did not begin until almost a full month later …” [112]

without assessing the documented issues that surrounded the Spala crisis. In plain English, one must examine the whole picture to obtain the correct clinical assessment. To grab selective segments will only serve to cloud the complexity of this series of episodes. What occurred in Spala must not be viewed in isolation.

Both Baroness Buxhoeveden[113] and Anna Vyrubova[114] detailed passionate, but not particularly informative accounts of the dual crisis. It was left to Pierre Gilliard,[115] to provide a well documented daily medical account of those horrendous days in Spala.

There is no need to re-iterate what was written, except to mention that Alexei was delirious, experiencing excruciating spasmodic pain every quarter hour for a period of five days, with a high temperature spike. The crisis proved more complex, because Alexei was experiencing abdominal bleeding. The situation was considered to be so critical, that both the Emperor and his consort believed their son was dying[116] and according to Orthodox ritual; Holy Communion was given.[117] With no strength to cry Alexei was heard to cry out that preferred the peace of death than the pain of life.[118] 

Finally, on advice, the Emperor conceded that Russia was to learn that the heir to the throne was very ill. The Minister of the Imperial Court, Baron Fredericks issued a formal public statement that outlined a general description of the Alexei’s condition.[119] However the true nature of the dual crises that the Romanov Dynasty faced was not expressed. 

Mr. Kendrick describes that there was one official bulletin published that described Alexei’s condition.  To be correct, one needs to appreciate the circumstances that necessitated publication of that Bulletin by the Russian Imperial Court on October 21.[120]

The Medical Bulletin described Alexei’s illness in considerable detail. It is completely misleading to suggest that because the word “hemophilia” was not specified then other factors must have been involved.

“…the official announcement of the Russian Palace. …must be taken to be the most accurate account.” [121] 

It must be emphasized again that the release of the official Medical Bulletin was in keeping with one of Russia’s National Secrets (see above). Buxhoeveden mentioned that to prevent malicious gossip, Alexei’s parents “continued to lead ordinary lives.” [122] It would be absurd to presume that the Russian State would readily admit that the heir to the Crown was suffering a terminal condition. The Bulletin was more than adequate notification to inform the public that Alexei was ill. One only has to remember that President Kennedy whilst serving his nation hid the true nature of his illnesses from the world.

The poignant letter that the Emperor wrote to his mother the Dowager Empress on 20 October[123] was the first dramatic expression of the father’s personal grief concerning Alexei’s imminent death. Fortunately, Alexei pulled through facing an extensive period of rehabilitation. Not until mid November was the family able to return to Tsarskoe Selo.[124] Flexion of the limb eased Alexei’s pain, but because of prolonged immobility he was temporarily unable to extend his left leg.[125]  

Despite Mr. Kendrick’s uninformed assertions, there was a simple medical explanation that explains the course of the two interconnecting incidents.

Joint bleeding and re-bleeding is common in hemophiliacs. Bleeding into joints takes several days.[126] It causes inflammation of the synovium, which is a delicate highly vascular membrane that lines the joint.[127] Chronic synovitis is common in the first two decades of a hemophiliac sufferer.[128] With repeat hemorrhages, synovitis leads to proliferation of the inflamed synovial membrane causing re-bleeding. Thus, a vicious cycle is created. 

Joint bleeding during growing years may cause excess growth at the ends of long bones leading to limb-length discrepancies. Hemorrhage into muscles may lead to atrophy and contracture.[129] Muscle bleeds are problematic for two reasons. It causes agonizing pain, and pressure on the nerves in that area. Continued pressure will damage the nerves. Initially it can cause numbness that decreases the ability to use the injured limb. An additional complication of prolonged periods of immobilization of limbs leg with recurrent hemorrhaging is that it leads to permanent ankylosis (joint stiffness) and deformity.

All these events were meticulously described in the Medical Bulletin. Alexei was beginning to demonstrate this complication in his left leg. Alexei had to wear orthopedic appliances to assist in straightening his leg to its original position.[130]

In Kendrick’s final attempt at rebuttal against recognizing the true nature of Alexei’s illness, it was claimed that:

“…excessively high fevers appearing consistently throughout the record of each of Alexei’s episodes are not a primary symptom of hemophilia …symptoms of delirium, high fever …to be inconsistent with the historically popular …diagnosis.” [131]

A similar comment is also provided on Kendrick’s web page:

“Any modern day physician will readily agree that the symptoms of fever and delirium are not consistent with a diagnosis of haemophilia” [132]

A world acclaimed historian, Suzanne Massie recently affirmed that both her own son and grandson experienced high fevers, concluding that such a scenario was consistent with hemophilia.[133]

There is no reason to disregard Pierre Gillard’s meticulous account of the daily elevation in temperature. It seems incongruous that Kendrick chooses now to selectively ignore the Medical Report - a document that he initially chose to place his reliance on its accuracy.

Alexei’s physicians correctly stated that:

“…the reactive inflammatory process …can be accompanied by a high temperature, which is what was observed in the case of His Highness.” [134] 

The Final Chapter

Alexei’s illness was a very painful one and undoubtedly took a toll on his entire family, emotionally and physically. In April 1918, while in captivity in Tobolsk, just months before he was brutally murdered, Alexei fell down a staircase. He began bleeding at the groin. Following this event, Alexei was never to walk again.[135] During the time that the Imperial family was in Yekaterinburg, the family doctor Yevgeny Botkin wrote a letter to the Ural Regional Soviet Executive Committee. This letter graphically describes Alexis' hemophilia and its life-threatening effects. Part of the letter reads:

“The boy is in such indescribable pain day and night that no one from among his closest relatives, though they do not spare themselves, has the strength to bear looking after him too long, not to mention his mother, with her chronically ill heart...His attendant...after a few sleepless nights filled with agony, becomes totally worn out and wouldn't be able to take it at all.”  [136]

His father Nicholas Alexandrovich was forced to carry his thirteen-year-old son and heir to the Romanov Dynasty to meet his final destiny in the cellar of the Ipatiev mansion in Yekaterinburg in July 1918.[137] Never given the opportunity to mature into a man, never to be Emperor of Russia, his life, though often painful was taken away by another.

A Regal Inheritance Fades into History

In a final attempt to dismiss Alexei’s hemophilia, Kendrick presented the following two statements:

“If the ‘Royal Disease was an X-linked disorder, then how did it apparently appear out of nowhere  … and then vanish again as suddenly in just three short generations?”

“There are no hemophiliacs in all the generation of that same Royal Family immediately following Alexei, nor in any subsequent generations.” [138]

Hemophilia in families tends to disappear.[139] There is a simple explanation for Kendrick’s concerns. Males afflicted with the condition in Alexei’s time had a shortened life span. Some of those males affected lived long enough to reproduce. Their offspring failed to reproduce due to early death, as had occurred in the Spanish and German Royal Houses.

Alexei’s did not die from hemophilia; he was brutally executed before he had the opportunity to have his own family. His entire immediate family lost their lives at the same time in Yekaterinburg in1918. That final chapter in Alexei’s tragic life and those of his sisters (some of whom may have been carriers of the defective gene) eliminated any possibility of the defective gene to be passed on.  

The majority of Alexei’s effected maternal relatives died by their first tri-decade. None of Queen Victoria’s male descendants who were afflicted with hemophilia survived for long periods. Alexandra’s young brother succumbed at the age of three.[140] Throughout his short life, Leopold had suffered severe hemorrhages and died from an intracranial hemorrhage at the age of 31 before he was able to reproduce.[141]

Eventually the Royal Disease diluted to the point where it is now no longer present in the European Royal Houses.

Why was it so?

Initially a few of Kendrick’s excerpts were selected to try and understand his reasoning. Perhaps there was something in Mr. Kendrick’s assertions, which the entire medical and historian community had ignored for almost a century? However on closer examination of Kendrick’s Historic Perspective and Web pages, persistent flaws in both his interpretation of published material and selective omissions of pertinent information became evident. The question first posed by the authors was:

How could such an obvious deviation against the extensively published historic and medical information be explained?

It became apparent that the author of the Historic Perspective article was attempting to set the foundation for his claim in a formal hematology publication. Kendrick’s conclusive remarks clearly enforce his real intention for writing the article:

“…the last Tsar’s only son may have survived the Bolshevik murder of his parents …reaching the age of retirement.” [142]

Kendrick from the very beginning of his Historic Perspective was attempting to reach the above claim that Alexei did not suffer from hemophilia, but some other blood disorder.

A second question now needed to be asked. Why would Mr. Kendrick choose to peruse this unusual path? The answer was found in Mr. Kendrick own Web pages, specifically constructed to draw public attention to his claim that Alexei, had survived into old age using the name Heino Tammet, and conveniently resided in the same city in Canada as the author. To succeed with that claim necessitated some kind of medical explanation as to how a young lad suffering from hemophilia would survive a bloody mass execution, and survive into old age, apparently unaffected by hemophilia, while the other members of his family perished in the same massacre. Kendrick’s publication in a steadfast medical journal has created an unexpected sense of credibility for his claimant’s estate.   

Kendrick’s Historic Perspective attempted to provide a series of “best-fit” pathologies that he believed may be more acceptable alternatives. The authors believe that with the overwhelming evidence against this possibility, there is absolutely no need to discuss what innovative diseases Mr. Kendrick preferred to describe. Despite Kendrick’s earlier contention that Baron Frederick’s Medical Bulletin must be considered to be accurate, then why does he step beyond that document?

To conclude this discussion the authors have compiled a Table (Table 6), which identifies some of Alexei’s known symptoms. When these symptoms are compared with Table 3 (see above), it becomes apparent that:

Table 6                   Summary of Alexei’s Observable Symptoms

Any attempt to apply anything else must be considered only to serve as a distortion of the facts to fit in with Heino Tammet’s cause of death. It certainly knocks out his claim that he was the sole survivor of the Yekaterinburg massacre.

Clearly the Kendrick assertions are nothing but distorted generalizations using several “best fit” medical scenarios; combined with his use of inadequate application of a few random excerpts taken from only a couple of published émigré memoirs. Had he examined his own sources in more depth, he would have realized what each of the memoirist’s statements was actually saying. Otherwise, to pluck out a sentence here and there only serves to provide panoply of distorted impressions – that assist in creating a tortuous argument to benefit Heino Tammet’s original claim.

It would have been more appropriate to apply the principle of parsimony (Occam’s razor), which states:

One should make no more assumptions than needed

To fully understand why such an extensive exercise was conducted in the first place, one need not go beyond Heino Tammet’s reported cause of death. Mr. Tammet died from leukemia[143], in the absence of any other underlying hematological condition such as hemophilia.

Tsesarevich Alexei Nikolayevich

                                      (Photograph from Margarita Nelipa’s personal collection)

Conclusion 

Although more than eighty five years have passed since the murders of the last Russian Imperial family by the hand of the revolutionaries, the public’s interest in the 1918 murder does not seem to show any sign of fading. Moreover, the absence of two of the Imperial children’s remains, including those belonging to Alexei, in the gravesite near Yekaterinburg,[144] added more fuel to the already raging flames of the “survival” theories.

Fate dealt young Alexei an ironic hand. He survived numerous life threatening and painful manifestations of his disease, only to be brutally murdered by revolutionary fire just two weeks before his fourteenth birthday. By many accounts, his naked bloodied body was disposed of in the flames of another fire – as described in an eyewitness report published in the Soviet Union in 1930.[145] There is currently ample physical evidence to support that it indeed happened this way. But the boy’s brutal death has yet again been trivialized by the recent allegations that not only did Alexei not suffer from hemophilia, but that he survived the 1918 massacre and lived out the rest of his life in Canada.

For anyone with medical training in hematology and pathology it is clear that the manner of presentation of Mr. Kendrick’s Historic Perspective leads to unsafe presumptions and conclusions. The connection between his cyber assertions relating to the survival of a pretender to the Imperial Russian throne and the A. J. H. publication is doubtlessly a cause for concern.

Ordinarily an argument against Alexei’s hemophilia would cause some mild curiosity before being dismissed by most as an interesting but completely unsubstantiated claim. However, when the journalist presenting this argument – using a respected medical journal as his academic forum - also hosts a website which supports an individual claiming to be the heir to the Russian throne,[146]the connection between the two authorships should not go unnoticed by the community seriously interested in Russian history.

Distastefully, new conjectures of survival continue to materialize, each with a new twist, taking on a life of its own – even going as far as attempting to re-write history in order to facilitate various claimants.

It would have been more appropriate to apply the principle of parsimony (Occam’s razor), which states:

One should make no more assumptions than needed