Growing up, you and I probably used to play a lot - running around, falling down, bruising our knees, elbows and even our heads(at least I did).
Fortunately, children are basically like Wolverine from the X-Men, with the ability to magically heal from most injuries.
But imagine every bruise you suffered - every scrape, every cut, every scratch - could potentially kill you.
That's what it's like living with hemophilia. 17 April 2022 marks World Hemophilia Day.
Hemophilia is a condition marked by your blood's inability to clot properly. This means you could potentially bleed till you're dead, because of an injury that would otherwise be quite normal.
Today we're looking at what happens to a hemophiliac's body in the moments after an injury and what could happen if a bruise or injury is left unchecked.
But first, let's briefly (I promise) look at what causes hemophilia.
What Causes Hemophilia?
Blood has 13 factors which affect its ability to clot. These factors all work together to make your blood clot and save you from bleeding out when you suffer a cut, a laceration or a bruise.
All types of hemophilia affect some of these clotting factors.
Usually the most common cause of hemophilia is genetic. Inherited hemophilia risk or hemophilia usually passes on from parent to child in the X chromosome.
In case you didn't know, all humans have two sex chromosomes. Women have the X chromosome, inherited from the mother and the X chromosome inherited from the father. Men have the X chromosome, inherited from the mother and the Y chromosome inherited from the father.
Having a family member who has hemophilia is one of the most common causes of hemophilia.
The two most common types of genetically inherited hemophilia are hemophilia A, associated with an affected blood clotting factor 8, and hemophilia B, which leads to an affected blood clotting factor 9.
The other type of hemophilia is acquired hemophilia, which happens to people with no famil history of hemophiilia. In acquired hemophilia your body's immune system attacks clotting factors 8 or 9, leading to your blood not clotting properly.
It can be tied to autoimmune disorders, cancer, multiple sclerosis, reactions to certain drugs, or even pregnancy.
So, What Happens If a Hemophiliac Is Injured or Bruised?"
Usually any abrasions or cuts we suffer will stop in a few minutes if you apply pressure to the site.
However, Dr. Prakash Mandal, a hematologist at Kolkata's Nil Ratan Sircar Medical College, tells us that if a hemophilic suffers a cut on their arm or leg, they will bleed continuously without stopping.
For a hemophilic, blood doesn't clot at the site of the injury. This is because of the missing or deficient clotting factors 8 or 9.
The first two hours, called the Golden Hour, are the most critical, he adds.
If a hemophilic has suffered an injury and even if there's no visible bleeding, they must be investigated for internal bleeding. Internal bleeding leads to more symptoms than external bleeding.
"If it's not found and treated immediately, internal bleeding can lead to swelling, called a hematoma or an edema. In cases of an open wound, which keeps bleeding, if they don't get treatment they could enter a coma very soon."Dr. Prakash Mandal, Hematologist, Kolkata's Nil Ratan Sircar Medical College,
He adds that it's critical to get treatment immediately or as soon as possible.
Any delay in therapy could lead to the person entering a state of shock, becoming comatose, and even death.
In some cases, people with a very low level of either factor 8 or 9 can suffer something called spontaneous bleeding. This is because these clotting factors are barely present in their blood.
He adds that in severe cases this will lead to spontaneous bleeding into the joints, or bleeding in the muscles.
What Is The Treatment For Hemophilia?
Treatment for hemophilia is usually preventive and episodic. Modern hemophilia treatment involves administering concentrated doses of the missing clotting factors 8 or 9 at regular intervals.
Doctors call this prophylactic care. Hemophilia sufferers are administered concentrates of the missing clotting factor, which have been treated to kill any bloodborne diseases or infections. This provides the person that missing clotting factor.
Over time, if left untreated, a hemophilic can suffer from bleeding into the joints repeatedly.
This leads to the joints becoming deformed and/or destroyed in a condition called Chronic Hemophilic Arthropathy, says Dr. Mandal.
"A large number of hemophilia patients in India, who have usually had the disease for more than 15 years, are either disabled or deformed because of this."Dr. Prakash Mandal, Hematologist, Kolkata's Nil Ratan Sircar Medical College,
The condition, at that point, is permanent and irreversible. The only option left for the hemophilia patient is to opt for joint replacement surgery, which is neither cheap, nor easy. And even that doesn't replace full function to the joints.
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