What is hemophilia and who has it?
Hemophilia is a rare bleeding disorder in which the blood doesn’t clot as it should. People with hemophilia lack clotting factors – proteins that work with platelets to help blood clot. Hemophilia is inherited and manifests almost exclusively in males. Annually, about 1 in 5,000 boys are born with hemophilia.
Hemophilia can be mild, moderate, or severe depending on the amount of clotting factors in the blood. Hemophilia is incurable, but there are treatments for the disorder such as clotting factor infusions. In modern times, people with the disorder have a normal life expectancy.
What are the symptoms of hemophilia?
Sign and symptoms of hemophilia differ depending on the level of clotting factors in the blood. People with mild hemophilia may experience excessive bleeding from a surgery, dental procedure, or injury. People with severe hemophilia can have spontaneous bleedings.
Symptoms of a spontaneous bleeding are the following:
- excessive bleeding from minor injuries;
- a lot of large or deep bruises;
- constant nosebleeds;
- blood in the urine;
- blood in the stool;
- swollen and painful joints that are hot and tender to the touch;
- in infants, irritability without an obvious cause.
Symptoms of hemophilia and related complications that require urgent medical care include:
- sudden pain, swelling and warmth in large joints (e.g. knees, elbows, hips, and shoulders) and in muscles in your limbs;
- bleeding from an injury, especially if you have severe hemophilia;
- painful, prolonged headache;
- repeated vomiting;
- extreme tiredness;
- neck pain;
- double vision.
What are the types of hemophilia?
There are 3 types of congenital hemophilia:
- hemophilia A, which is the most common, is caused by the lack of clotting factor VIII;
- hemophilia B, the second most common type, is caused by the lack of clotting factor IX;
- hemophilia C, which is often mild, is caused by the lack of clotting factor XI.
Hemophilia A is severe in about 70% of patients.
Hemophilia A and B can’t be passed from father to son, but daughters of men with these two types of hemophilia are carriers of the gene. These types of hemophilia are passed from mother to son; there’s a 50% chance that a boy will inherit the faulty gene from a mother who is a carrier. There’s also a 50% chance a girl will inherit the gene responsible for hemophilia from her mother and be a carrier too. Women are mostly carriers of the gene and rarely experience symptoms.
Hemophilia C can be passed to children from mother or from father. Both boys and girls can have hemophilia C.
Parents who don’t have hemophilia/aren’t carriers can have a child with hemophilia. In such cases, hemophilia is a result of a random change (mutation) in the gene.
Hemophilia can also be acquired – it happens when your body starts producing antibodies that attack clotting factors. This form of the disorder is extremely rare.
What measures can help avoid excessive bleeding and joint damage?
In order to avoid profuse bleeding and joint damage, consider the following:
- people who have hemophilia shouldn’t play contact sports, such as football, rugby, or hockey;
- needless to say, anticoagulants (blood-thinners) are not safe for people who suffer from hemophilia;
- practice proper dental hygiene to prevent bleeding in the mouth;
- children with hemophilia should wear kneepads, elbow pads, helmets, or other protective gear to avoid injuries.
Hemophilia in history
Hemophilia was once known as “the royal disease”. Many of Queen Victoria’s male descendants died prematurely, bleeding to death after accidents. Queen Victoria’s heirs spread the disease marrying into royal families of Germany, Spain, and Russia. Based on the analysis of the Romanovs’ bones, it was established that the disease was indeed hemophilia B.
Hemophilia may even be one of the factors that played a role in the downfall of the Romanovs (Russian royal dynasty) and the Russian Empire. Alexei, the only son of Emperor Nicholas II and Empress Alexandra Feodorovna, suffered from hemophilia. He was in a frail condition, and his mother was desperate to find a remedy. She turned to the mystic Grigori Rasputin for help. Rasputin claimed to have special powers and became close with the Romanovs. He used this relationship to gain influence in Russian politics, and the public grew suspicious about his dealings. This is one of the factors that contributed to the revolution and brought on the end of the House of Romanov.
This post is solely for informational purposes. It is not intended to provide medical advice. Fabiosa doesn’t take responsibility for any possible consequences from any treatment, procedure, exercise, dietary modification, action or application of medication which results from reading or following the information contained in this post. Before undertaking any course of treatment, the reader should consult with their physician or other health care provider.
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