The genetic bug hidden in the bloodline of European royal families is actually regarded as a symbol of nobility

A small injury will cause bleeding

Replacing a tooth can be fatal

I want to run but I can only stand quietly in front of the window

This is the real life of the "glass people" with hemophilia. Injections and medication have become a common occurrence throughout their lives...

Image source: Huashang Daily

Recently, a 3-month-old baby in Shaanxi was diagnosed with hemophilia. Due to financial difficulties and inability to pay the high medical expenses, the family had to seek help from the society. So what exactly is hemophilia? Is it really fatal?

Hemophilia may have originated from a royal marriage gone wrong

In 1840, Queen Victoria of England married her beloved cousin Albert. It was this grand marriage that started a long royal hemophilia tragedy, and the source of everything was Queen Victoria (her hemophilia may have been caused by a genetic mutation due to inbreeding).

Queen Victoria gave birth to 9 children after marriage, including 5 daughters and 4 sons. Two daughters did not have symptoms of hemophilia, but they were carriers of the hemophilia gene, and the youngest of the 4 boys, Prince Leopold, was a hemophiliac, and at that time no one knew that such a disease could be life-threatening.

Victoria and her nine children, Image source: Wikipedia

Prince Leopold began to suffer from hemophilia at a young age. He would bleed profusely after being injured, especially when he was learning to walk. Bumps and bumps were common, but for him, they were fatal injuries. Queen Victoria, like most parents, loved her son very much and rarely let him go out. Although he was so well protected, unfortunate things still happened. In 1884, Prince Leopold accidentally slipped while on vacation in Nevada, injured his knee joint, and passed away the next morning.

The death of Prince Leopold did not sound the alarm for the royal family. The descendants of Queen Victoria's children were either hemophiliacs or carriers of the disease gene. Through political marriages, Victoria and her descendants passed the disease gene to the royal families of Germany, Spain and even Russia, so that all members of the royal families of Europe more or less inherited this "Victorian legacy."

People found that most royal family members who suffered from this disease were men, so they believed that this disease was a curse of the European royal family and could only be transmitted to men and not women.

In the ancient medical era, even the royal family had to accept their fate when faced with hemophilia. Most of the royal family members who suffered from hemophilia died in their prime due to various falls or bumps.

European royal hemophilia family tree, source: Wikipedia

Is this disease really only contagious to males and not females?

Hemophilia is indeed more common in men, but that doesn't mean women don't get it.

Hemophilia is an X-linked recessive genetic disease. We all know that women have two X chromosomes. To get the disease, the father needs to be a hemophiliac and the mother at least a carrier of the hemophilia gene, and the probability of this is not high. But if there is a hemophiliac in the family, then the woman is likely to be a silent gene carrier, which will increase the chance of male offspring suffering from hemophilia. Because men only have one X chromosome, even if the father is not a hemophiliac, there is a 50% chance of suffering from the disease.

Image source: Wikipedia

Changing a tooth could kill you.

Hemophilia is a lifelong bleeding disorder with bleeding as its main symptom.

Bleeding occurs because there is too little of a protein in the blood that helps the blood clot.

Hemophilia is generally divided into two types, depending on which clotting protein is missing:

Hemophilia A – also called classic hemophilia – is the most common type of hemophilia. About 80% of people with hemophilia have this type. It happens when there is too little of a blood plasma protein called factor VIII. The less factor VIII a person has, the more severe their symptoms. Most people with hemophilia A have moderate or severe symptoms.

Hemophilia B - also known as Christmas disease, is less common. It affects about 20% of people with hemophilia. Hemophilia B is caused by too little of a blood plasma protein called factor IX. The less factor IX there is, the more severe the symptoms. Bleeding symptoms in hemophilia B are usually mild.

What are the symptoms of hemophilia?

Symptoms of hemophilia range from heavy bleeding after trauma or minor surgery to spontaneous bleeding for no apparent reason.

Unlike the common bleeding from gums, tongue, mouth and nose, bleeding from hemophilia is typically manifested by bleeding from the knees, ankles and elbows. Bleeding from small wounds caused by trauma is relatively easy to treat. What is worrying is internal bleeding, especially in the knees, ankles and elbows, as well as internal bleeding in the brain. Internal bleeding can damage organs and tissues in the human body and endanger life.

A 47-year-old hemophilia patient had three joints replaced at the same time. Image source: Weibo

Therefore, if a hemophilia patient has any of the following symptoms, they should seek medical attention immediately:

Signs or symptoms of cerebral hemorrhage: dizziness, headache, limb weakness, binocular vision impairment, etc.

After the trauma , the limbs are not flexible and urination is difficult;

Difficulty breathing when you have a cold;

The swollen joint feels hot to the touch and is painful when you bend it.

Image source: Wikipedia

Can hemophilia be cured now?

At present, hemophilia cannot be cured, and gene therapy that is expected to cure hemophilia is still in the clinical trial stage. However, modern medicine has enabled hemophilia patients to have a higher quality of life, and the treatment center is the replacement therapy of injecting coagulation factors.

For hemophilia patients or people who carry the hemophilia gene, early pregnancy genetic testing can be used to assess the possibility of fetal disease, and the patient can choose whether to terminate the pregnancy or guide relatively safe pregnancy and delivery measures based on the patient's wishes. In vitro fertilization-embryo transfer technology can also be used to provide embryo genetic testing for couples at high risk of having children with hemophilia, and embryos that are not affected by the disease can be selected for transplantation.

I hope that one day in the future, better treatments for hemophilia will be developed, allowing them to run freely...

Audit Expert

Jin Tao, Chief Physician of the Department of Hematology, Shenyang Red Cross Hospital

END

Tadpole Musical Notation original article, please indicate the source when reprinting

Editor/My Neighbor Totoro