All About Hemophilia, The Royal Blood Disease

Summary

The video script delves into hemophilia, historically known as the 'Royal Disease,' tracing its spread through European royal families, particularly due to Queen Victoria's lineage. It discusses how intermarriage among royals, including cousin marriages, exacerbated the condition. The script outlines the tragic consequences for affected royals, such as the early deaths of Victoria's son Leopold and several of her great-grandsons. It also touches on early medical understanding and treatments, including the use of snake venom and the significant advancements that came with the isolation of blood clotting factors, which revolutionized hemophilia care.

Takeaways

• 👑 Hemophilia is a genetic blood disorder historically prevalent among royal families due to intermarriage.
• 🌐 Queen Victoria is known to have passed on the hemophilia gene to her descendants, affecting royal lines across Europe.
• 🧬 The condition was exacerbated by the practice of royals marrying close relatives, leading to a higher chance of genetic disorders.
• 👶 Prince Leopold, one of Queen Victoria's sons, and several of her great-grandsons died due to complications from hemophilia.
• 🔮 Alexandra, mother of Alexei Romanov, sought the help of Grigori Rasputin, who claimed healing powers, to help her hemophiliac son.
• 💉 Early 20th-century treatments for hemophilia were rudimentary, with one method involving diluted snake venom.
• 🩸 Hemophiliacs were advised to lead sedentary lives to prevent injuries that could cause uncontrolled bleeding.
• 🧬 The term 'hemophilia' was coined in the 19th century, derived from 'haemorrhaphilia', and the disease was also historically referred to as 'the royal disease'.
• 📚 Maimonides, a 12th-century philosopher, was one of the first to note the hereditary nature of hemophilia, particularly its connection to mothers.
• 🏥 Modern treatments for hemophilia have significantly improved life expectancy, but the 1980s AIDS crisis affected many due to contaminated blood supplies.

Q & A

What is hemophilia and how is it inherited?

Hemophilia is a rare blood clotting disorder that is genetically passed down from mothers carrying the mutation to their children. It is a genetic condition that affects the blood's ability to clot, leading to prolonged bleeding after injuries.

Why is hemophilia sometimes referred to as the 'Royal Disease'?

Hemophilia is called the 'Royal Disease' because it was prevalent among European royal families due to intermarriage among closely related individuals, which increased the chances of passing on the genetic disorder.

How did Queen Victoria contribute to the spread of hemophilia among royal families?

Queen Victoria was a carrier of the hemophilia gene and passed it on to three of her nine children. Her children's marriages into other European royal families led to the spread of the disease across various royal lineages.

What was the impact of hemophilia on the life of Prince Leopold, Queen Victoria's son?

Prince Leopold, who inherited hemophilia from his mother, Queen Victoria, suffered from chronic pain, swollen joints, and bloody urine. He eventually died at the age of 30 due to a brain hemorrhage after a fall.

What historical treatment methods were used for hemophilia before modern medicine?

Before modern medicine, there were limited treatment options for hemophilia. One such method was the use of diluted snake venom, which was discovered in the 1930s to cause blood to clot and was used to treat patients with blood clotting disorders.

How did the practice of marrying close relatives within royal families contribute to the spread of hemophilia?

The practice of marrying close relatives, such as cousins, within royal families led to a more concentrated gene pool, which facilitated the rapid spread of genetic disorders like hemophilia.

What was the significance of the relationship between Alexandra and Grigori Rasputin in the context of hemophilia?

Alexandra, the mother of hemophiliac Alexei Romanov, developed a relationship with Grigori Rasputin, who claimed to have healing powers. This relationship had a significant impact on Russian history and was a desperate attempt to find a cure for her son's condition.

How did the discovery of blood clotting factors in the 1940s and 1950s change the treatment of hemophilia?

The discovery of blood clotting factors led to the development of real treatments for hemophilia. This breakthrough allowed doctors to understand the deficiency causing the disease and develop therapies to address it, rather than relying on ineffective methods like snake venom or blood transfusions.

What was the impact of the AIDS crisis on hemophilia patients in the 1980s?

The AIDS crisis had a devastating impact on hemophilia patients in the 1980s due to inaccurate and poor donor screenings. This led to many patients being infected with HIV through factor VIII donations, which were not screened for the virus at the time.

How has the life expectancy of individuals with hemophilia changed over time with advancements in treatment?

With proper treatment, the life expectancy of a male with hemophilia has significantly improved. In the 1960s, it was just 11 years, but by the 1980s, it increased to 50 to 60 years. Currently, with modern treatments, their life expectancy is only about 10 years shorter than an unaffected male.

Outlines

00:00

👑 The Royal Disease: Hemophilia's Impact on Royalty

This paragraph delves into hemophilia, a genetic blood disorder historically prevalent among European royals due to intermarriage within royal families. Queen Victoria, in particular, unknowingly passed the condition to three of her nine children, leading to a significant spread of hemophilia across various royal lineages. The paragraph highlights the limited understanding and treatment options of the time, which often involved restricting the affected individuals to minimal physical activity. It also touches on the irony of the disease being called 'the Royal Disease' and the tragic consequences it had on the lives and deaths of several royal family members.

05:01

🩸 Hemophilia's Deadly Legacy: Royal Tragedies and Early Understanding

The second paragraph continues the exploration of hemophilia, focusing on the specific cases within Queen Victoria's family and the broader royal community. It discusses the tragic outcomes of the disease, such as the death of Prince Leopold and several of Victoria's great-grandsons due to complications from hemophilia. The paragraph also touches on early medical observations and understanding of the disease, including its hereditary nature and the naming of 'bleeders' for those affected. It highlights the historical context and the progression of knowledge regarding hemophilia, leading up to the 20th century when treatments began to emerge.

10:03

🧬 Advances and Challenges in Hemophilia Treatment

The final paragraph discusses the evolution of hemophilia treatment from the early 20th century to the present day. It notes the initial discovery of snake venom's clotting properties and the subsequent development of medical treatments. The paragraph also addresses the significant advancements in life expectancy for individuals with hemophilia, particularly with the concentration of clotting factor VIII. However, it also recounts the devastating impact of the AIDS crisis in the 1980s, where many hemophilia patients were infected with HIV due to inadequate screening of blood products. The paragraph concludes with a reflection on the current state of hemophilia treatment and the improved, though still challenged, life expectancy for those affected by the disease.

Keywords

💡Hemophilia

Hemophilia is a rare genetic blood disorder characterized by impaired blood clotting, which can lead to prolonged and spontaneous bleeding. In the context of the video, hemophilia is referred to as the 'Blood Disease of Royals' due to its historical prevalence among European royal families. The video discusses how Queen Victoria's lineage, in particular, contributed to the spread of hemophilia among various royal houses.

💡Genetic Mutation

A genetic mutation refers to a change in an organism's DNA sequence. In the video, the focus is on mutations that lead to hemophilia, which are passed down through generations. The script explains how Queen Victoria, being a carrier, passed on the mutated gene responsible for hemophilia to her descendants, illustrating the concept of genetic inheritance.

💡Carrier

A carrier in genetics is an individual who carries a gene for a recessive condition without exhibiting symptoms of the disease. The video mentions that Queen Victoria's daughters Alice and Beatrice were carriers of hemophilia, meaning they could pass the condition to their offspring without being affected themselves, highlighting the role of carriers in spreading genetic disorders.

💡Royal Lineages

Royal lineages refer to the ancestral lines of monarchs and their families. The video emphasizes how hemophilia became known as the 'Royal Disease' due to its prevalence in European royal families. The script details how intermarriage among royals, such as Queen Victoria's nine children marrying into other European royal families, facilitated the spread of hemophilia.

💡Blood Clotting

Blood clotting is a process that prevents blood loss from injuries by forming clots. In the video, hemophilia is described as a condition that impairs this process, leading to uncontrolled bleeding. The script uses the term to explain the medical complications faced by individuals with hemophilia, such as the tragic outcomes from seemingly minor injuries.

💡Hemophilia B

Hemophilia B is a specific subtype of hemophilia caused by a deficiency or absence of clotting factor IX. The video points out that Queen Victoria carried a rarer form of hemophilia known as hemophilia B, which was passed on to her descendants, affecting royal families across Europe, including the Romanovs of Russia.

💡Grigori Rasputin

Grigori Rasputin was a Russian mystic who claimed to have healing powers and had a significant influence on the Romanov family. The video recounts how Alexandra, the mother of hemophiliac Alexei, sought Rasputin's help to cure her son, reflecting the desperation and lack of medical treatments for hemophilia at the time.

💡Snake Venom

In the video, snake venom is mentioned as a source of inspiration for early treatments of hemophilia. The script explains that in the 1930s, doctors discovered certain snake venoms could cause blood to clot, leading to the use of diluted snake venom as a treatment for hemophilia, an example of how unusual substances were explored for medical purposes.

💡Blood Transfusion

A blood transfusion is a medical procedure where blood is transferred from one person to another. The video discusses the evolution of blood transfusion techniques, which became safer and more effective over time, significantly improving the life expectancy of individuals with hemophilia, especially during and after World War II.

💡HIV/AIDS Crisis

The HIV/AIDS crisis had a profound impact on the hemophilia community, as mentioned in the video. Due to inadequate screening of blood donations in the 1980s, many hemophilia patients who received factor VIII concentrates were infected with HIV, highlighting a dark period in the history of hemophilia treatment and the broader implications of the AIDS crisis.

💡Life Expectancy

Life expectancy refers to the average number of years a person is expected to live. The video tracks the improvement in life expectancy for individuals with hemophilia over time, from just 11 years in the 1960s to nearly the same as unaffected males with modern treatment, underscoring the significant advancements in medical care and the impact on quality of life.

Highlights

Hemophilia is a rare blood clotting disorder, genetically passed down from mothers to their children.

The condition spread through royal families due to intermarriage among European royals.

Queen Victoria is known for spreading hemophilia through her nine children, who married into other royal families.

Hemophilia was dubbed the 'Royal Disease' due to its prevalence in royal lineages.

Queen Victoria's son Leopold and daughters Alice and Beatrice were directly affected by the disease.

The House of Hanover practiced marriages among close relatives, contributing to the spread of hemophilia.

Prince Leopold died at 30 from a brain hemorrhage after a minor fall.

Several of Queen Victoria's great-grandsons died due to complications from hemophilia.

Hemophilia B, a rarer subtype, was carried by Queen Victoria and spread through her descendants.

Alexei Romanov, the heir to Tsar Nicholas II of Russia, suffered from hemophilia.

Early 20th-century doctors used diluted snake venom to treat hemophilia.

Before modern treatments, hemophiliacs were advised to minimize physical activity.

Prince Friedrich of Hesse died at two and a half years old from internal bleeding after a fall.

John Conrad Otto was one of the first to describe hemophilia in an 1803 article.

The term 'hemophilia' was coined in 1828, derived from 'haemorrhaphilia'.

By the 1940s and 1950s, doctors had isolated the cause of hemophilia as a deficiency in blood clotting factors.

The life expectancy for hemophiliacs increased with the development of new treatments and blood transfusion techniques.

The 1980s AIDS crisis affected hemophilia patients due to HIV contamination in blood products.

Today, with proper treatment, the life expectancy of a male with hemophilia is close to that of an unaffected male.

Transcripts

Hemophilia is a rare blood clotting disorder,

genetically passed down from blood mutation-carrying mothers

to their unsuspecting children.

Due to the intermingling of royal families,

the deadly genetic condition found a happy home

amongst several royal lineages through history.

One in particular, Queen Victoria,

was responsible for a long line of royal hemophiliacs,

with three out of her nine children

being carriers or victims of the medical malady.

Without the medical knowledge or technology we have today,

most children with hemophilia were just

reduced to lives full of still activities,

convenient of course, since there is nothing children

love more than standing very, very still.

Today, we'll dive into all about hemophilia,

the Blood Disease of Royals.

But before we get started, it's OK to slightly move your mouse

cursor to subscribe to the Weird History channel.

And let us know in the comments below what

other royal stories you would like to hear more about.

Now, let's proceed cautiously.

This one is a bleeder.

Hemophilia wasn't a snobby disease that intentionally

stayed within royal families, but the life choices of royals

aided in the spread of a rare form of hemophilia

that pass through multiple families.

Royal family dating pools were more of a kiddie pool

versus an ocean.

And they tended to intermingle only with other royals.

With a like-minded gene pool, genetic disorders

spread quickly.

No royal exemplified this better than Queen Victoria.

Queen Victoria gave birth to nine children,

all who were married off to other European royals.

Today, royal families from Germany, Norway, Russia,

Sweden, Greece, and Spain can all trace their heritage back

to this small litter of royal siblings.

Queen Victoria herself was a hemophilia carrier

and passed the blood disorder down several royal lines.

As a result, hemophilia was given the high faluting

sounding moniker of the Royal Disease.

The Queen passed those genes to three of her nine children.

Her son Leopold inherited the disease,

while her daughters Alice and Beatrice were carriers.

Just with these three royals, the genetic

spread, triggering devastating results

throughout European royal families.

For several generations, the House of Hanover,

a house Queen Victoria was a proud member of,

did the perfectly normal thing of marrying their own royal

cousins, which caused the slew of problems.

For one, it's gross.

And for the other, more tragically,

it helped aid the spread of hemophilia.

Between 1680 and 1840, the royal house

saw six marriages between cousins, three of which

being first cousins, which is not exactly

something one brags about.

This disturbing tradition of marrying relatives

was directly responsible for the spread of hemophilia

amongst royals.

Queen Victoria's son, Prince Leopold, died at the age of 30,

due to a brain hemorrhage after he had a relatively pedestrian

fall.

The queen's grandson, who was just two years old, had a fall.

That fall created internal bleedings.

To which he was unable to recover from and died.

All totaled, five of Queen Victoria's great grandsons

ultimately lost their lives due to complications

associated with their unfortunate genetic fate.

And unfortunately for Queen Victoria,

the form of hemophilia she carried

was an even rarer subtype, known as hemophilia B.

As her kin married off into other royal families

and had children, the genetic disease was passed along.

Royal lineages all over Europe showed hallmark signs

of hemophilia, spreading even up to Russia,

where Queen Victoria's granddaughter, Alex, birthed

a son, Alexei, who was cursed with the blood clotting

disease.

Alexei Romanov's flex didn't end that great grandson

of Queen Victoria.

He was also the heir to Tsar Nicholas II of Russia.

Both very cool, obviously.

However, being the great grandson of a queen

had some obvious drawbacks.

Alexei's mother, Alexandra, as mothers often do,

turn to magic to cure her precious baby boy.

Alexandra developed a cozy relationship

with Grigori Rasputin, a man who claimed to have healing powers.

This relationship would go on to shape Russian history.

Alexei would die at the age of 13, but not from hemophilia.

He was shot during the Russian Revolution,

along with the rest of his family.

While magical Russians didn't do much to treat hemophilia,

early 20th century doctors didn't have a lot

of better options, other than--

hmm.

Wish it away?

In the 1930s, doctors discovered that certain snake venoms

caused the blood to clot, leading them to a wacky idea

to dilute snake venom and treat patients with the blood

clotting disorder, selling literal snake

oil as a medical cure.

But in this case, it was actually effective.

Blood transfusions before the 20th century

where dicey procedures, performed only

under the rarest of circumstances.

But before modern treatments and medicine

were around to treat the blood clotting disease,

sufferers of the ailment were told to just lay low

for a while and chill out, man.

Queen Victoria knew her boy Leopold

had an illness that led to persistent bruising

and bleeding.

She did have eyes, after all.

In describing the condition to his father,

King Leopold, the Queen eloquently

described it as, "the peculiar constitution

of his blood vessels, which have no adhesiveness.

Nothing whatever can be done for it.

He may, and it is hoped, will outgrow it.

But since June, he has been banned

from very active amusement."

Despite the Queen essentially telling

her son he was super grounded and keeping his activities

to a minimum, Leopold still spent a life in chronic pain

with swollen joints and bloody urine.

Queen Victoria's genetic rampage plowed through generations

of royals her daughter, Alice, also

married into a German royal family, because royals don't

marry plebeians, no matter what lies Hallmark Christmas

movies try to sell us.

Her son, Prince Friedrich of Hesse and by Rhine,

was one of Queen Victoria's genetic victims

of hereditary hemophilia.

Friedrich was fighting an uphill battle though

with both his grandmother and his mother

being carriers of the disease.

When Prince Friedrich was only two and a half years old,

he fell out of a window as a byproduct

of super good parenting.

Though it's rarely a good outcome when toddlers

fall from windows, the fall was one

a child with good clotting blood would most likely survive.

Unfortunately for Friedrich, the internal bleeding

caused by the blunt trauma was too much for his little body

to handle.

And the boy passed away at only two years old.

Queen Victoria's great grandson, Prince Heinrich of Prussia,

would also pass away and only 4 years old,

also from a simple fall.

Philadelphia man, John Conrad Otto, was one of the first

to describe the malady in an 1803 article.

He observed the affliction tended

to affect men more than women and noted

there was also a clear hereditary component as well.

One couldn't just catch hemophilia all willy

nilly from a royal child falling out of a window, for example.

He was even able to trace one family's tragic line

of hemophilia all the way back to one woman in Plymouth

in 1720.

All of that was more information than most knew at the time,

but he didn't come up with the name hemophilia.

Instead, he called them bleeders.

Head trauma isn't traditionally great for anybody,

but it is especially dangerous for people with hemophilia.

Several of Queen V's descendants succumbed to their hemophilia

after suffering survivable bumps to the head.

Her son, Prince Leopold, died in 1884

after a little fall that caused a cerebral hemorrhage.

Three other of her descendants would suffer similar fates,

Rupert Cambridge, Alfonso of Spain,

and Infante Gonzalo of Spain all passed away

from relatively minor injuries that turned tragic

when their broken bodies couldn't properly heal

and caused massive internal bleeding.

Victoria's genetic reign of terror

ended at last, with Prince Waldemar of Prussia.

A carrier of the hemophilia mutation, Prince Waldemar

died in 1945 while waiting for a blood transfusion

at the end of World War II.

All this tragedy may sound like it's the fault of women,

and it is.

Before genealogy existed and one could spit into a tube

and learn just what percentage of European you truly are,

philosophers were vaguely aware that some diseases

were passed down from family member to family member.

One of those was 12th century Sephardic Jewish philosopher,

Maimonides, who drew the connection

between hemophiliac sons to their genetic mutation-carrying

mothers.

He combined this observation with talmudic law

and wrote up a commentary on the talmudic rule, which

allowed families to bypass circumcisions,

if they tragically lost siblings to the medical procedure

previously.

This rule specifically applied to half siblings

who had the same mom, but not those who

solely shared the same father.

Another Jewish code in the 16th century

added onto the women spread rare blood disorders

clause of circumcisions by extending

it to include the sons of sisters who had brothers who

didn't survive circumcision.

I'll give you a second to work through that one.

Uh huh.

And then, there.

It was an uphill battle for doctors

to treat the condition before advances in medicine.

But rabbinical scholars did figure out

that the blood came from the women.

If you don't have a rare blood disorder,

you might want to thank your mom for not passing one along.

In 1828, University of Zurich student Friedrich

Hoff and his professor, Dr. Scholein,

first drummed up the word haemorrhaphilia

to describe the medical mystery that

was later mercifully shortened to hemophilia.

That's not much of a shortening.

Could've done better.

By the 1940s and 1950s, doctors had finally

isolated the cause of hemophilia,

a deficiency in different blood clotting factors.

With this breakthrough, they were

able to develop real treatments for the disease that just

weren't contact your local magician

or just stay very, very, very still all the time.

New blood transfusion techniques developed during World War II

extended the life expectancy of a hemophilia patient

by seven years.

Only a seven-year increase in life expectancy

in over 60 years of research might sound depressing.

But it really was just the beginning.

The life expectancy for a boy born with hemophilia

in the 1960s was just 11 years.

In the 1980s, with appropriate treatment,

the average life expectancy for a male with hemophilia

was 50 to 60 years.

This was due in large part to groundbreaking advances

in treatments that allowed the concentration of clotting

factor VIII, the crucial component in the blood

that patients with hemophilia A were missing.

Concentrating plasma donations saved countless hemophiliacs

from succumbing to the disease at young ages.

Everything was going swimmingly for sufferers of the disease,

until the AIDS crisis came guns blazing into the 1980s.

Inaccurate and poor donor screenings

left the virus unchecked in factor VIII donations.

Since there was no way to test plasma

for HIV in the early '80s, half of hemophilia patients

in the United States were infected with HIV.

Currently, with proper treatment,

the life expectancy of a male with hemophilia

is only 10 years shorter than an unaffected male.

So what do you think?

Have you ever been more thrilled to not

be in a long line of royals?

Happy your family tree doesn't include

any married first cousins?

Let us know in the comments below.

And while you're at it, check out some of these other videos

from our Weird History.