Why hemophilia is a royal disease

2024 Author: Seth Attwood | [email protected]. Last modified: 2023-12-16 15:55

This disease has always been considered a sign of the carrier's belonging to the royal family, or even equated with the privileges (very dubious, like gout) of royal persons. In fact, this is not the case: ordinary mortals are not immune from hemophilia either, it is simply unlikely that anyone will enter into historical treatises information that a peasant has died of "liquid blood".

Well, it's not interesting for descendants - perhaps only for doctors.

The life of a person with hemophilia is a constant series of survival tests. What for a healthy person will seem like an ordinary trifle (they cut a finger while chopping onions for dinner, fell off the bike and put their knee on the asphalt, removed a tooth, or nosebleed from high blood pressure) can turn into trouble for a hemophiliacs. No, with this kind of injury, a person will not die from bleeding - this is probably the most common misconception about the consequences of hemophilia, but stopping the blood is very difficult. Internal bleeding becomes a much larger problem, which can occur even spontaneously, without any external influence. Here we already have to resort to special drugs and urgent medical intervention is required.

Royal disease

The cause of the disease is a congenital gene, which is carried mostly by women. The girl takes this gene from her mother, and then passes it on to her son, who will subsequently be sick with hemophilia, or to her daughter, who will also become a carrier of this gene.

The first mentions of "liquid blood" are found in the Talmud. In time immemorial, an old Jew introduced there a rule that a boy was not circumcised if two of his older brothers died due to blood loss caused by the operation. Cruel, in my opinion, but in a different way at that time it was hardly possible to accurately diagnose this disease. Closer to the XII century, a physician from Arab countries noted in his medical diaries that he encountered a whole family, in which men often died of bleeding caused by small wounds. And only in the 19th century, a doctor from America, John Otto, accurately established: constant bleeding, even from small scratches, is a disease, moreover, a hereditary disease that affects mainly men. At that time, nothing was known about women's participation in the “vicious circle”. And the name was different - Otto called her "a predisposition to bleeding", and later scientists from Switzerland gave her a name familiar to the modern eye: hemophilia.

It also has other names such as "Victorian disease" or "royal disease". They did not arise by chance: the most famous carrier of the fatal gene was Queen Victoria.

The woman, most likely, was the first carrier in her family, and the gene developed in her body, since the disease was not found in the families of Victoria's parents. But after it - a lot. Hemophilia also spread because in royal families marriages were concluded between close relatives: this also contributed to an increase in the manifestation of the gene. Victoria herself had a sick son, Leopold, and her daughters became carriers and transmitted the hemophilic epidemic to their descendants, who, in turn, spread it to almost all the royal families of Europe. The fact that Leopold was born with this disease, the ministers of the Church immediately regarded as retribution for the grave sin of the Queen Mother: she broke one of the covenants - "to give birth to children in illness", and when Leopold was born, doctors first used chloroform as an anesthetic … However, if you do not take into account the illness, the young man had a very inquisitive mind and was drawn to new knowledge. He easily graduated from Oxford, and entered the service of his mother as the Queen's personal secretary. Contemporaries argued that Leopold often helped Victoria with the conduct of state affairs, from which it can be concluded that education was not for "tick", but went for the future. The prince even managed to get married, having chosen Elena, the sister of the Queen of the Netherlands, as his wife, the newlyweds managed to give birth to two children (who also suffered from a fatal illness). And then the prince stumbles unsuccessfully and dies of a cerebral hemorrhage.

Despite the fact that people learned to recognize hemophilia in the early stages, no one knew how to treat or prevent it, or even how to make life easier for patients. But they tried as best they could, especially those who had the opportunity to take care of the life expectancy of their descendants. So, in Spain, they tried to protect two sick heirs to the throne from accidental abrasions and scratches in a very peculiar way: while walking in the park and taking "oxygen cocktails", the boys were dressed in a kind of spacesuits on a cotton base, and each branch of the park was wrapped in a thick layer of felt, so that children, God forbid, do not get scratched.

Romanov and hemophilia

When I said that the disease had spread throughout all the royal families of Europe, I didn’t bend my heart at all. At that time, hemophilia could well be considered a deadly disease (and even now there are risk groups depending on the type of disease - A, B or C), and thanks to the descendants of Victoria, she came to the Russian Empire. The only son of Nicholas II, Alexei, suffered from this disease. Alexandra Feodorovna, being Victoria's granddaughter, inherited the ill-fated gene and passed it on to her son.

The prince was not yet two months old when he had his first bleeding, and from that moment the disease began to attack. Every scratch, every bruise led to the fact that the court doctors knocked down in an attempt to "seal" the blood. In the morning, the boy often complained to his mother that he could not feel his arm or leg, and even more often he was tormented by severe pain caused by hemorrhage in the joints.

One can even say that hemophilia had an indirect effect on Russian politics at that time: apart from members of the imperial family, only Grigory Rasputin could drop in to the prince at any time of the day, who somehow managed to stop the bleeding of Tsarevich Alexei in some incomprehensible way. Naturally, this led to the fact that both Nicholas II and his wife infinitely trusted the Siberian, and listened to his words with regard to one or another area of life.

Rumors of illness

Of course, the monarchs exaggerated their concern for children - it was pointless to wrap parks in felt, because a small scratch would not harm the children. On the other hand, it is difficult to give an adequate assessment of such care, because the life of the heir to the throne was at stake, who, moreover, was a small, defenseless child, just like all other children who loved to run and play pranks.

Any large cut, any strong blow can be fatal. That is why surgical interventions are contraindicated in patients with hemophilia: an incision with a scalpel can become fatal. Of course, there are exceptions: in case of emergency and with full provision of the needy with drugs that increase blood clotting, the operation can be performed.

Yes, basically it is a "male" disease, and the stronger sex suffers from it. But the medical archives contain 60 case histories belonging to women who suffered from bleeding, and were not just carriers of the gene. Yes, hemophilia is inherited from mother to child, but sometimes (as in the case of Queen Victoria) this gene mutates on its own, in a healthy adult organism. Such cases are about 30%. It was not possible to find out exactly the reasons for the non-hereditary disease: there are suggestions that in some cases it was provoked by the intake of drugs prescribed for oncology, or in late pregnancy, proceeding with severe complications.

Today, 400 thousand people with hemophilia live in the world, 15 thousand of them in Russia. The world is trying to draw attention to them, and not remain indifferent: since April 17, 1989, World Hemophilia Day has appeared on the calendar. As it was several centuries ago, the disease is still incurable, but modern doctors have a much better chance of saving the life of a person with "liquid blood" by controlling the course of the disease with physical therapy and helping to reduce the duration and frequency of bleeding with coagulation factor injections. These substances, which ensure its clotting, are extracted from donated blood. Together with procedures and medical supervision, they can provide a person with hemophilia with the same long life as a healthy person.

The search for a cure does not stop for a day. Great hopes are pinned on gene therapy, in which changes are made to the genetic apparatus of human somatic cells: it is not yet known how it will affect our organisms, but geneticists managed to cure a couple of mice from hemophilia. For 8 months of continuous follow-up, no side effects were identified. I would like the insidious disease to rather leave people alone, and find its place not in the human body, but on the dusty pages of history.