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Hemophilia Hemophilia, also known as the //Royal Disease//, because in Europe many memebers of the royal family fell victim to this disease,is a disorder dealing with the clotting of blood. Hemophilia usually occurs most commonly in males. The mother is the carrier of the disease, and it is passed from mother to son. It can also develop in the womb. What causes Hemophilia is actually the lack of one of the twelve clotting facotors.Hemophilia is a sex-linked recessive genetic disorder, dealing with the X chromosome. Most of the time the clotting factor number VIII(they are numbered using Roman numerals) is missing, unless it is Hemophilia B. Any person of any nationality can have this disease.

Symptoms


 * Big bruises
 * Bleeding in muscles and joints(knees, elbows, ankles)
 * Spontaneous bleeding
 * Bleeding for longer than usual after getting a cut, tooth removal, or having surgery
 * Nosebleeds for no apparent reason
 * Heavy bleeding from a minor cut[[image:hemophilia.jpg width="240" height="151" align="right"]]
 * Serious internal bleeding into vital organs, most commonly after serious trauma

Signs of Internal Bleeding:
 * Blood in urine
 * Bleeding in the kidneys or bladder
 * Blood in the stool
 * Blood in the stool is due to bleeding in the intestines or stomach
 * Bleeding in the brain( this can be fatal )

How Do People With Hemophilia Live? Not everyone with Hemophilia has all of these symptoms. Hemophilia is classified in cases, mild, moderate, and severe. The symptoms are obvious and are caused by the lack of blood being clotted. People with mild cases of Hemophilia live mostly normal lives. They are //cautioned// towards playing sports or doing physical activities that involve the possibility of injury, __but they can still play them__. Those with severes cases of Hemophilia however are not allowed to participate in any sports or physical activities in case of injury. This disorder does allow its victims to live a relatively normal life. Even though it is a life threatening disorder it can be maintained and managed. If treated correctly and often a person with Hemophilia can live as long as anybody else. Hemophilia occurs in 1 out of 5,000 men. || media type="custom" key="3623011" ||
 * How Often Does It Occur?

Does Race, Ethnicity,or Gender Matter?

Race and Ethnicity have nothing to do with the disorder. Everyone from everywhere can be diagnosed with it. Gender is another story. Hemophilia is most commonly found in males, rarely females. This is because in females, there are two X chromosomes, but in males there is only one. If a female has one defective X chromosome, the other X chromosome may not be defective, but if it is defective in the male they only have one X chromosome and it will show the defect.

How Is It Diagnosed?

If your physican is under the impression that you have a bleeding disorder he will check your family's medical history to see if there is a pattern of bleeding disorders that could be passed down. You physician will also do a physical exam and run blood tests to determine which type and how severe your Hemophilia is. The blood test will determine for you whether you are missing any clotting factors, whether your blood has low levels of a certain clotting factor, or how long it takes for your blood to clott.

First Diagnosis

The first modern diagnosis of Hemophilia was by Dr. John Conrad Otto. In 1803 he published "An account of an hemorrhagic disposition existing in certain families." Although the name Hemophilia was not created until 1828 by Hopff, Otto knew how to distinguish the traits of Hemophilia.

Well Known People With Hemophilia


 * Queen Victoria (carrier)
 * Queen Victoria's son Alexis (heir to the throne)
 * Also 4 of her grandsons had Hemophilia

Early Detection? Hemophilia can be detected by testing of blood to see if the person has the proper amount of clotting factors. Also, Hemophilia can be detected prenatally at 9 to 11 weeks by a chorionic villus sampling (CVS) or also by fetal blood sampling at 18 or more weeks. Women can be carriers of this disorder, such as Queen Victoria was. A medical genetics specialist or a specialist in bleeding disorders can run tests to determine whether you are a carrier. It is suggested that you take these tests if you have a family history that involves relatives with Hemophilia.

Treatment? There is no cure for Hemophilia. However, gene therapy holds possibility for a partial or complete cure. It is treated by replacing the clotting factor in the blood. This is done by injecting a product that has the needed factor into a hemophiliac's vein. The bleeding will stop when enough of the clotting factor gets to the bleeding site. If the treatment is administered quickly it will prevent long-term damage.

Where are Clotting Factors Found?
 * Factor Concentrates
 * Cryoprecipitate
 * Plasma
 * Whole Blood
 * Desmopressin (DDAVP)

Drawbacks to Treatments? Cryoprecipitate is less safe from viral contamination than concentrates and is also not as easy to store and administer. Fresh plasma is less effective than cryoprecipitate and large volumes of it have to be transfused which can cause a circulatory overload. Whole blood also has its drawbacks because it must be fresh for the activity of the clotting factors will decrease rapidly with time. Also about whole blood, it must be compatible with the recipient and the large amount of whole blood needed to stop a bleed may overload the circulation and cause heart failure.

Support Groups?
 * Project Red Flag
 * Do the 5!
 * National Hemophilia Foundation (NHF)
 * Gateway Hemophilia Association (GHA)
 * World Federation of Hemophilia (WFH)

References [] [|http://www.nhlbi.nih.gov/health/dci/Diseases/hemophilia/hemophilia_what.html] [|http://www.kumc.edu/gec/support/hemophil.htmlhttp://www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=0&contentid=1http://www.gatewayhemophilia.org/aboutus/committeesandsupportgroups/[[http://www.pathguy.com/lectures/hemophilia.jpg] [] ]]