Hemophilia The Bleeding Disorder

Imagine this, bleeding without a stop. Ounces, pints, even liters of blood just flowing out like a river. Hemophilia is a severe hereditary bleeding disorder in which factors needed to make the blood clot are missing. There are ways to detect it, such as double vision, prolonged headache, convulsions, and seizures. There are also many treatment options for the disorder.

Hemophilia is a genetic bleeding disorder, due to low clotting factors it doesn’t clot correctly. This certain bleeding disorder causes abnormal bleeding. Hemophilia is rare, and it’s caused by a gene mutation. When a person has hemophilia their blood doesn’t clot normally because it lacks enough blood-clotting proteins. Individuals that have hemophilia have a decrease in factor 8 or 9 depending on what type they have.

In this day and age, there is more technology and knowledge than in the past. But there is still no cure for hemophilia. However, there is treatment for it, the treatment depends on how severe your hemophilia is. A good way to treat it is done by injecting treatment products. People with hemophilia, which get a little scrape or cut can use a bandage and apply pressure to take care of the excessive bleeding. Just like a bandage can stop the bleeding an ice pack can be used as well. That’s if there are little regions of bleeding beneath the skin. Another treatment option for this disorder is medicine. There are medications for hemophilia, some examples are Amicar, which prevents blood clots from breaking down, and results in a firmer clot. Hemlibra replaces factor 8, which is the cause of Hemophilia A. Stimate is another medication for an absence in factor 8. There is also a clotting factor concentrates. The treatment that clinicians prescribe typically for episodic care. It is used to stop or slow down a person’s episodes. Going to a hemophilia treatment center is the best choice. People that have severe hemophilia might be on a treatment routine.

Hemophilia is an inherited disorder, and usually occurs more in males. It’s inherited in an x-linked recessive pattern. In males, one duplication of the gene in every cell is enough to cause the disorder. It’s less common in females because they have two x chromosomes. To cause hemophilia, there must be a mutation in the two x chromosomes. And since males only have one x chromosome it affects them more than it does to females. One in every 5,000 males are diagnosed with hemophilia. Right now there are about 20,000 males in the United States that have hemophilia. Hemophilia affects all people, from all ethnic groups, sexes, and races.

Depending on the level of severity and clotting factors, hemophilia signs and symptoms may vary. Bleeding disorders most likely start at a young age, but some children don’t show any signs or symptoms until they start to walk. There are many ways to detect if a person has hemophilia. One of the ways to detect if a person has hemophilia is easy bruising. Excessive bleeding from injuries or cuts, bleeding into the joints. Tough to stop and frequent nose bleeds, and, unusual bleeding after vaccinations.

There are three types of hemophilia, hemophilia A, B, and C. But there are two common types, those types are hemophilia A and hemophilia B. Hemophilia A, otherwise known as classic or deficiency hemophilia. Is caused by a lack of clotting factor 8. The second is hemophilia B, also known as the Christmas disease. Hemophilia B is caused by a decrease in clotting factor 9. Hemophilia is four times more common than hemophilia B. To know what type of hemophilia someone has it’s determined by the clotting factor the person is deficient in.

With the right treatment, hemophilia can be manageable. However, without the appropriate treatment, it can be hard to control. Treatment is needed to stop the bleeding and blood clots when someone has an episode. Some episodes are weekly and some are monthly. It depends on the treatment the person is taking and how severe it is. Every few days people with hemophilia A and B need IVs that contain clotting factors.

There are many risk factors when considering hemophilia. Having a relative with the disorder is the biggest. Inhibitors are also a main concern and risk factor. Inhibitors make it very hard even impossible to stop an episode. Mainly because inhibitors prevent the treatment from working. Which for hemophilia is very serious, life-threatening. People that develop inhibitors will require treatment, that is until their bodies quit making inhibitors. People with hemophilia use this special treatment called clotting factor concentrates. These products are used to stop bleeding episodes and prevent blood clots. When someone develops an inhibitor, their body is done with accepting the treatment as a normal part of their blood. Which then results in the body thinking the product is an unknown foreign substance. And then the body tries destroying it with an inhibitor. Inhibitors can appear at any time, but most appear during the first time’s someone is treated with a concentrate.

If someone has a history of hemophilia in their family. It wouldn’t be a bad idea to check out genetic testing. With genetic testing, they can see if you are a carrier of the disorder. It’s a good idea to do if they are considering starting a family. A person with the signs and symptoms of bleeding into the brain. If an injury won’t stop bleeding, or joints are painful and swollen. If someone has these conditions then they should see a doctor. There are many foundations that provide support and information for people with hemophilia.

When a person bleeds, their body usually always pools together blood cells. And forms a blood clot to stop the continuous bleeding. Hemophilia is caused when one of the genes mutates. The gene that has the mutation provides guides for making the clotting proteins. This mutation can also prevent the blood-clotting protein from working correctly or just working in general. Hemophilia runs in families, but some families don’t have any past history of it. These genes, that affect hemophilia, are located on the x chromosome.

There are many complications when having hemophilia. Some of the complications may include deep internal bleeding. Bleeding that happens in muscles and swells limbs. Infection, risks of receiving contaminated blood products and more likely to have blood transfusions. And damage to joints, severe pain is caused when internal bleeding puts pressure on joints. If it is not treated it might cause arthritis.

Traveling with hemophilia can be scary and challenging. Because knowing that bleeding episodes are noncontrollable, and can happen at any time. If someone is going to travel they have to plan ahead. Before traveling they should talk to their doctor. To make sure they are in a healthy condition. Along with that, there are some precautions with what to pack. Such as travel letters, medicine, and medical supplies, a list of hospitals, and medical and contact information.

There are key preventions for healthy living. First, get an annual checkup at a hemophilia treatment center (HTC). Second, get the vaccination for hepatitis A and B. Third when bleeding, treat the bleed adequately and early. Fourth, keep a healthy weight to protect the joints, by exercising. Fifth and lastly get tested for infections that may occur in the blood.

“Hemophilia is classified into three severity levels. Depending on the level of severity it could be mild, moderate, or severe”. In mild hemophilia bleeding normally occurs only after major injuries and surgeries. With moderate hemophilia, they bleed infrequently. Long periods of bleeding, and occurs after a minor injury or surgery. Lastly, with severe hemophilia, they bleed often. It occurs randomly in joints and muscles. And happens with surgery or for no real reason.

Hemophilia isn’t a new disorder, it has been going on since we can remember. The people in ancient times told us through writing. They wrote about bleeding problems, and that they could see that people bleed differently. And also from bleeding differently, they saw that people bleed from doing different things. But it didn’t help any that they didn’t know much about how blood clots. Hemophilia is also known as “the royal disease”. It’s known as the royal disease because royal families in Spain, Germany, Russia, and England were affected by it (Nowicki 213). “The British royal family provides a historical example of a sex-linked disorder” (Nowicki 213). Queen Victoria was a carrier of hemophilia, which she passed onto her son. In those days royal families normally married into other royal families. Even royal families in other countries. Such as Spain and Russia also had that allele, the allele for hemophilia. These alleles that the royal families had were traced back to Queen Victoria. Research on hemophilia has come a long way.

Hemophilia is serious and should not be played around with. There are ways to treat it and detect it. Double vision, convulsions, seizures, and prolonged headaches are simple ways to detect it. But it is still very life-threatening.

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