The Royal Disease of the Victorian Era
Imagine you are the Empress of Russia. You have a loving husband and four healthy and beautiful daughters. But it’s just one problem - you need to give birth to a son to extend your royal dynasty. And finally, you made it, your nation celebrates the heir’s birthday, everyone is happy and you think that your mission is completed. But one day you discover that your son is sick. Even the smallest scratches on his body don’t heal and bleed all the time. Doctors can’t help, every scratch and slip can cause the death of your child.
This is a story of the Russian Empress Alexandra Feodorovna, the wife of Nicolas II - the last ruler of the Russian Empire. Her son named Alexei had a rare and dangerous genetic disease called haemophilia. Generally, the blood of a person with haemophilia can’t clot, which is needed to stop bleeding. How could this happen? The Empress, her husband and daughters were totally healthy.
Alexandra Feodorovna with husband Nicolas II, son Alexei and four daughters (Olga, Tatiana, Maria and Anastasia)
A short lesson of genetics
As you know, everyone has two chromosomes that determine our sex. XX for girls and XY for boys. So everyone gets one X chromosome from the mother and one X or Y chromosome from the father. A genetic disorder that causes haemophilia is located only in the X chromosome.
Girls always have two X chromosomes. If some girl gets one X chromosome with haemophilia, she always has another X chromosome from a healthy parent. So the girl won’t have any haemophilia, but she has this wrong chromosome in her body all the time. If this girl decided to become a mother, in half of the cases, her son would have haemophilia, or her daughter would be a haemophilia carrier. Boys have two different chromosomes, so if some boy gets the wrong X chromosome from his mother, he will have haemophilia. Also his daughter, in any case, will be a haemophilia carrier.
https://en.wikipedia.org/wiki/Haemophilia
Haemophilia epidemic in Royal Families
The Russian Empress Alexandra Feodorovna was a haemophilia carrier and prince Alexei is one of many other haemophilia cases in Royal Families. The first haemophilia carrier from the European royalty was Queen Victoria. Nobody knows how she got the “wrong” X chromosome because she had no ill parents. There are some suspicions, she was a bastard and her real father had haemophilia. Also, there is a chance that her chromosome just mutated. Queen Victoria had nine kids, two of her five daughters (Alice and Beatrice) were silent carriers of haemophilia genes, and her son Leopold also was ill.
Beatrice married one of the members of the Spanish Royal Family. She had two sons and two grandsons with haemophilia, and also one daughter as a carrier. So disease was also present in the Spanish royal family. Alice had two daughters with haemophilia: Irene and Alix. Irene married Prince of Prussia and two from three of her sons also suffered from haemophilia. That means the Prussian family also had disease cases.
Her other daughter Alix married Nicolas II, joined the Orthodox church and got a new name - Alexandra Feodorowna. Consequently, the hidden disease of Queen Victoria caused a real epidemic of haemophilia in all European royalty. Later it was called “the Royal disease of the Victorian era”.
https://en.wikipedia.org/wiki/Haemophilia_in_European_royalty
Questions
1. Have you ever heard about haemophilia?
2. What would you do if someday you found out that your partner has a serious genetic disease like haemophilia or chronic migraine? Would you decide to have children knowing that they could inherit the disease?
3. Can you get any examples of other genetic diseases? Maybe you or someone you know has some?
https://www.sciencemag.org/news/2009/10/case-closed-famous-royals-suffered-hemophilia
https://en.wikipedia.org/wiki/Alexandra_Feodorovna_(Alix_of_Hesse)#Haemophilia_and_Rasputin
https://www.cdc.gov/ncbddd/hemophilia/facts.html
Comments
I think in current times, with our medical knowledge and possibilities of treatments to various diseases, I’d have children with such a person anyway. It’d be more important to me if I loved that person and that person loved me, you know, “in sickness and in health”. In fact I probably inherited a problem with my spine (scoliosis) after my mother; of course it’s a struggle sometimes, but I’m not anyhow blaming my mother for that. You don’t choose to get sick or inherit a disease, especially such a “sneaky” disease as hemophilia.
As I said, scoliosis might be genetic. Also, one of the commonly known inherited diseases is Down syndrome. I also think one of anaemias is possible to be inherited. But I don't think I remember any other genetic diseases.
2. I will sign on every needed examinations to check my partner and my health, DNA and so one. Having child is huge responsibility, so I will take serious it’s future health. I can’t make somebody life miserable just because I wanted kids. If the results will be bad and impossible to avoid inheriting disease, there are also other ways to have a child.
3. I don’t have a huge knowledge about genetic diseases. Only that which was talked through on biology classes. But if the topic is mentioned, Down’s syndrome comes to my mind. It is about extra chromosome. Just a little change like this makes such a huge difference.
The second question is a tough one. I am sure that would be a very difficult decision. I suppose it depends on how and when I found out about my partner's illness. If I found out about that by mistake and when e.g. we're trying to have a baby, I would be devastated. If my partner would tell me about it sooner, then I believe we could've figured out something together.
My mom's friend has a son with autism. I believe he's now either in 6th grade or in Junior High School. At first, he seems normal and behaves normally, but there are some situations that make it prominent. He's great at math, but has some difficulties learning other subjects. From what I know, this is an exemplary issue people with autism have. He sometimes gets bullied by his peers, but who doesn't, right? I don't know him very well, just from his mom's conversations with my mom. Apart from that, I don't think I know someone else with a genetic disease or any other genetic disease. As far as I can tell, some cases of autism are not genetic.
I don’t know exactly the name of this disease, but it happens when you have a condition in which your skin is growing to fast (if I remember correctly). Due to that it cracks open, you have to scrub the excess of it every day, keep your skin moisturized, safe and clean. I even have seen some kind of a documentary about it, where parents have found out about this genetic condition after their first daughter was born and still decided to give it a shot and had another one. They ended up with two cute, but intensively health burdened daughters, as girls had other problems like mental retardation, partial blindness or problems with joints and bones.
Thank You for your comment. I agree that giving birth to a child, knowing that it might have a serious disease is quite a selfish decision. I think a great solution for a pair in this situation is an adoption. Instead of exposing a little person to a life full of pain with a childhood spent in medical clinics and hospitals, they can give a family for a healthy child.
2. Yes, I think I would still like to have children, knowing that they could inherit this disease.
3. I am a hearing impaired person. Not deaf. Hearing can certainly be a genetic disease, but not always. In my case, it's genetic. This means that I can't hear anything without hearing aids. I use hearing aids to hear and communicate with other people. However, hearing aids do not solve all the problems in my case - I still have difficulty understanding in noise or when someone is talking unclearly.
Yes, I have heard of it because I heard about Romanovs.
2. What would you do if someday you found out that your partner has a serious genetic disease like haemophilia or chronic migraine? Would you decide to have children knowing that they could inherit the disease?
I propably wouldn't bother because with nowadays medicine most of then-deadly diseases can be managed and don't affect life significantly.
3. Can you get any examples of other genetic diseases? Maybe you or someone you know has some?
Mental diseases can be heritated. I once watched M.D. House episode in which was said that Huntington's chorea is one of the diseases that is genetic.
Yes, I heard about this disease, as far as I know the Romanov family had such a disease, it is written in the article. We covered this topic in history at school. This is a dangerous disease, you can die from blood loss or hemorrhage.
2. What would you do if someday you found out that your partner has a serious genetic disease like haemophilia or chronic migraine? Would you decide to have children knowing that they could inherit the disease?
I think I would think 10 times before deciding on this. The health of future children is the most important thing.
3. Can you get any examples of other genetic diseases? Maybe you or someone you know has some?
I think a lot of diseases are inherited, such as color blindness, downism. I have not met people with genetic diseases
Yes, I have read about the last Tsar's family.
2. What would you do if someday you found out that your partner has a serious genetic disease like haemophilia or chronic migraine? Would you decide to have children knowing that they could inherit the disease?
From my point of view final decision on that have to be taken by both parents after numerous tests and consultations. If the chances are high I would prefer adoption over than having a sick kid.
3. Can you get any examples of other genetic diseases? Maybe you or someone you know has some?
Unfortunately I cannot recall any similar diseases from the top of my head.
Second question is as abstract as questions gets. For now I'm not even planning a child in the future. It may of course change over time, but it's purely theoretical. In case of chance of inheriting chronic disease, I guess adoption is better option. Even if it's just a promile of a chance, it's gambling with child's life.
One chronic disease from the top of my head is diabetes. It's one of most popular of chronic diseases. Although usually kids inherit not diabetes per se, but strong inclinations toward it.
2. It's hard for me to answer because I don't want children. It seems to me that if I were to make decisions, I would prefer to avoid having children with genetic defects.
3. At school, the biology lesson covers topics about genetic defects. I have heard of diseases such as down syndrome or autism, but I don't personally know anyone with a genetic disease.
I feel sorry for your disease, I hope it is curable. I think such a disease is not a reason to give up on children. It's nice that you heard about haemophilia before, I watched many historical documentaries too.
Answer for Pawel Saniewski
Really, it's an interesting fact. It’s lucky that your mother didn’t become a currier. I think that love is the most important in the decision to have children. My boyfriend has a heavy genetic ADHD among male family members. Such a child needs a lot of effort, but I think we can go through this. Anyway, now my boyfriend is a totally normal and very calm person.
Oh, I’m sorry about your disease. It’s maybe really hard to live with hearing problems. Fascinating, that you are not afraid of having children with such serious genetic condition. Genetically, I have a very big risk of becoming diabetic. In the cause of diabetics, my grandma loosed two legs and my grandpa loosed few fingers. But I also still like to have children, so don’t feel lonely with it.
I never heard about that movie. Few people told me in comments that they watched an animation about Nicolas II and his family. I think I found a film for the weekend.
Yes, life with a haemophilia may be really boring. I can’t remember how many times I bumped my knees during my childhood. I can’t imagine my life with such a disease.
As I noticed you have a really responsible approach to having a child. In the case of genetic diseases, other ways like adoption are definitely worthy.
Of course, nowadays medicine is really advanced and many serious diseases now not affect normal life too much. Once I noticed how my diabetic friend quickly and easily checked sugar level while we waited for the bus. My grandma was a diabetic, and I remember that her hardware for sugar level measuring was big and expensive. Now it’s just a small gadget, a little bit bigger than a smartphone. But there are diseases like ADHD, which strongly affect on life. Sometimes kids with ADHD are punished by teachers in school because they don’t know how to deal with it. And treatment for it is unhealthy because they should take amphetamine-based treatments.
Yes, the decision about having kids is definitely very important. I think people with genetic diseases should think many times before. Sometimes disease not only makes life difficult, sometimes treatment is really expensive. For example, the cheapest pump for diabetics costs 4000 PLN, not counting insulin cartridges, which need to be replaced regularly. I suspect that the treatment for haemophilia also costs.
I’ll agree that decision like that should be taken by both parents. For me is the most important thing in the decision to have children are good relations between parents. I think better to be sick in a happy and loved family than to be healthy with toxic parents. Psychical diseases are often more painful than physical. I think that partners with genetic diseases have a really difficult choice. Maybe many of them should seriously consider adoption.
Yes, it’s hard to even imagine how terrifying life with a haemophilia could be… Of course, I realize that questions about family planning are quite abstract in our age. Many of us just planning to have family in future, someone doesn’t even consider family. But I find such a question quite interesting, also for a person who doesn't even think about the family yet. Many of us one day will find a perfect person with whom they will plan to have children. But we don’t think that our loved one could have a genetic disease and one day some will make such a decision.
Many of us heard about haemophilia on a biology class, but it’s interesting that you heard about it in a historical way. As I understood you a childfree. It’s quite popular nowadays. Several years ago reluctance to have children was seen like something weird and unusual, I like the fact that now it’s something normal. I think that child should be loved and expected. If someone doesn’t want to have kids that desition must be accepted.
I know that many from us heard about Alexei disease, but the fact that he was sick due to disease of Queen Victoria surprised me. I didn’t think that haemophilia was so popular in European royalty. It’s nice to hear that you watched many documentaries. I remember how I was surprised when my friend in middle school told me she never watch any documentaries. It was weird for me because channels like “Discovery” od “National Geographic” were my loved ones.
Thanks for our medicine, because now many diseases are not dangerous anymore. Anyway, sometimes treatment can be really expensive. I may have a big risk to become a diabetic person because many of my grandparents are diabetic. But also I hope it’s not genetic because my grandparents were born a moment before the Second World War. As my granny told me, they mostly ate pure sugar all their childhood.
Also, my boyfriend had advanced ADHD when he was a child. He doesn’t know is it genetic or not. His dad and grandfather also had symptoms in childhood similar to ADHD, but only a doctor can recognize this disease. As I read ADHD can be caused by wrapping the umbilical cord. He was born with caesarean section precisely because the umbilical cord is wrapped, so also that disease can be not genetic. Anyway, in adulthood ADHD symptoms are almost imperceptible. Like many of us, he has a slight difficulty concentrating and also gets drunk faster, but otherwise no different from a healthy person.
I hope your disease is curable. I wish you health:)