Even today, there are many misconceptions regarding bleeding diseases. Two of the bleeding disorders, with the most misperceptions, are hemophilia and von Willebrand disease. One of the most common myths about patients with hemophilia is their short life expectancy. Since our society lacks awareness about hemophilia and bleeding disorders, the prevalence and sharing of these myths and faulty ideas are quite rampant.
In the present day, more than 60,000 people are living with bleeding disorders in the United States, of which 24,000 individuals have hemophilia.
What are bleeding disorders and hemophilia?
Bleeding disorders occur due to an alteration or decrease in the production of certain proteins called clotting factors. The lack of clotting factors results in poor blood clotting, which leads to spontaneous bleeding as well as blood loss during injuries or surgery. Hemophilia is an inherited bleeding disorder in which there are low levels of factor VIII or factor IX. The amount of these clotting factors present in the blood determines the severity of a bleeding disorder.
Let us have a look at 10 of the most interesting facts about hemophilia and bleeding disorders.
Interesting facts about hemophilia
- Bleed longer, not faster
It is important to note that people with hemophilia bleed longer, instead of bleeding faster. This helps avoid the deadly myth that a minor cut in a patient with hemophilia would lead to their demise. Minor cuts or scrapes usually stop bleeding with the use of a band-aid, similar to patients who do not have hemophilia. However, in cases of internal bleeding in the skull, or hemorrhage in the soft tissue around airways or other internal organs, hemophilia poses a serious threat to the organ and might cause tissue damage.
- Living a normal life
Owing to the advancement of science, people with hemophilia can now live normal, long, and healthy lives. With appropriate care, such as prophylaxis treatment, the life expectancy of patients with hemophilia can be about 10 years less than that of a healthy person. However, it is true that without adequate treatment, many patients still die before adulthood. The death rate for people with hemophilia across the world is twice that of the rate for average people. Though, the mortality rate of hemophilia is also dependent on factors such as whether a person has other diseases or not.
- Most cases are due to inherited hemophilia
There are three types of hemophilia and all three affect both men and women. It would be false to assume that hemophilia only occurs in males. Since the gene coding for hemophilia is present on either of the X chromosomes, this bleeding disease is usually inherited. This way, hemophilia A and B affect the male sex more than the female sex, but females can be carriers of the disease. According to statistics, about 1/3 of women who are carriers of hemophilia tend to experience bleeding symptoms. However, hemophilia C affects males and females equally. Additionally, von Willebrand disease, another type of bleeding disorder, is prevalent in men and women equally.
- Cases of hemophilia with no family history
As mentioned above, hemophilia does not necessarily have to be inherited in all cases, whether mild or severe. Nearly 1/3 of the cases of hemophilia have no link to a family history of the bleeding disorder, as spontaneous gene mutation can also be the cause of hemophilia.
- Hemophilia cannot be outgrown
Owing to the misinformation easily available on the internet, it is widely believed that it is possible to outgrow hemophilia. This is false. Hemophilia is a genetic disease and defective blood factor gene is irreparable by the body. Even though scientists are now searching for a cure for hemophilia via gene therapy but until the cure is officially accessible, unfortunately, people have hemophilia for life.
- Pregnancies in female hemophilia patients are mostly normal
As risky as pregnancies in a female hemophilia patient sound, it is usually not the case. Women with von Willebrand disease often have a normal pregnancy without complications. To be safe, however, it is vital to consult your obstetrician and hematologist to make accurate assessments and take care of any pregnancy risks involved based on the individual’s health. If, in any case, the baby is born with the same disease as their mother, rest assured that it could usually be detected early in life. The diagnosis of mild cases is possible within the infant’s first 18 months whereas it takes a month, at most, to diagnose severe cases.
- Types of hemophilia
The clotting factors responsible for hemophilia A, B, and C are factors VIII, IX, and XII, respectively. Hemophilia A is the most common type of the three and affects one in 5000 people, with its severe cases occurring when an individual has less than 1% of the normal activity of factor VIII. Severe hemophilia A is associated with spontaneous bleeding episodes. Hemophilia B affects one in 25,000 people and hemophilia C affects just one in 100,000.
- The severity of the types of hemophilia
Hemophilia A, B, and C are not all serious cases of hemophilia. Hemophilia C is considered less serious than both A and B. A patient with hemophilia C is only likely to bleed, following a surgical or dental procedure and they do not need regular clotting factor IV drips. In contrast, the health and well-being of people living with hemophilia A and B depend on regular IVs of clotting factor i.e., every couple of days.
- Hemophilia does not cause HIV/AIDS
Before 1985, blood donations did not undergo screening, so transmission of AIDS/HIV was highly likely. This resulted in the contraction of HIV in about 8000 people affected with hemophilia. Since then, the US has made it mandatory for all blood donations to undergo HIV tests. Nowadays, the clotting factor is much safer as people with bleeding disorders use recombinant, or manufactured, clotting factor. This contains little to no human blood, thereby reducing the risk of contracting HIV in patients with hemophilia.
- Complications of hemophilia
Hemophilia and other bleeding diseases, such as von Willebrand disease, are capable of exacerbating complications in health conditions such as joint pain, arthritis, kidney disease, heart disease, and hemorrhage, typically cranial hemorrhage. While joint deterioration continues to play a great role in the chronic complications in hemophilia, efficient and timely treatment can greatly taper the risk of life-threatening bleeding episodes and the severity of long-term damage to joints.
Hemophilia affects people from all ethnic and racial groups. It presents many signs and symptoms in different people. Some may experience bleeding within joints, which causes joint pain and disease. Others may experience bleeding in the head, or even in the brain, causing chronic seizures or paralysis in severe cases. This bleeding disorder, like many others, can be fatal if the bleeding following a surgery or organ damage is not attended by a doctor and stopped immediately.
It is important to consult a medical professional immediately for medical advice, diagnosis, or treatment, as the internet and statistics can be quite misleading with symptoms or numbers. If you or someone you know is showing symptoms of hemophilia, or if you have any misconceptions about this disease, it is ideal to seek advice from your physician or any other qualified health provider.