hemophilia

=**__//Hemophilia//__**=

By Aila and Vivi
Hemophilia is a rare genetic disorder (inherited) when a person's blood clots very slowly or not at all. It takes much longer for a hemophiliac to stop bleeding in comparison to the average person. Hemophilia can also cause internal bleeding, especially in the joints (ankles, elbows, knees). Babies born with the disorder have a low amount of a certain protein or don't have the protein that is needed for blood to clot (protein is referred to as a "clotting factor").
 * What is**

18,000 people in US have hemophilia, & 400 babies are born with hemophilia. Hemophilia can't be passed onto sons from male hemophiliac, yet daughters will be carriers. (Carried on X chromosome) A male child with a mother carrying hemophilia has 50% chance of being born with the disorder. Only males can have the disorder, yet in extremely rare cases, females can too. See the diagrams below.

Clotting is the body's reaction to bleeding, and it helps helps heal wound (a.k.a. Blood Coagulation) The clotting factor works with platelets (small blood cell) and helps them stick together to block, or //plug//, cuts and wounds. With a low clotting factor, the platelets cannot stick together, therefore the blood cannot clot. In result, there is continuous bleeding. Constant bleeding can lead to damaged organs, severe blood loss and even fatality (blood infusions with clotting factor may be necessary)
 * Clotting**

There are two main types of hemophilia; ~ Hemophilia A: Clotting Factor VIII (8) is missing or low (90% percent of hemophiliacs) ~ Hemophilia B: Clotting Factor IX (9) is missing or low

Hemophilia can also be caused by antibodies that develop and prevent clotting factors from functioning.

Hemophilia can be - Mild : 5 to 30% normal factor - Moderate : 1 to 5 % normal factor - Severe : Less than 1% normal factor

70% of hemophiliacs with type A have it in the "severe" form. Normal people have a factor VIII activity or 100%, while people with Hemophilia A, severe, have an activity less than 1%

Signs and symptoms for hemophilia are listed on the table below. Symptoms vary based on the severity of the disorder.
 * Symptoms**

There are signs you should also look for if you suspect bleeding in the joints. If not acknowledge or treated properly, it could lead to disabling arthritis. You may be bleeding in the joints if you experience;
 * **Baby/Toddlers** || **Older Children/Adults** || **Hemophilia Concentrated in Brain** ||
 * may bleed excess amount after circumcision || hemarthrosis (bleeding in joints) || extremely long and painful headaches ||
 * heavy bleeding and bruising in gums from baby teeth coming in || blood in urine or feces || vomiting many and double vision ||
 * bruises or bumps from falls as they learn to walk || continuous bleeding from loss of tooth or cut in mouth || changes in behavior, feeling very sleepy ||
 * swelling or bruising from bleeding in joints, muscles and tissue || nosebleeds without reason || sudden weakness in limbs or difficulty walking ||
 * nosebleeds without reason || bleeding and bruises in tissues and muscles || Neck pain/stiffness or convulsions ||
 * Stiffness or tightness in the joints; no real pain.
 * Tightness or stiffness shall continue with noticeable pain.
 * The joint becomes swollen and is warm/hot to touch, and bending joint causes sharp pain.
 * Swelling and bleeding continues, and movement of joint is limited (if any mobility) along with extreme pain.
 * As joint is finally filled with blood, bleeding slows.



If you have experienced symptoms of the disorder and go to see a doctor, they shall check both you and your family's medical record, give you a physical exam, and conduct a series of blood test. The objective of the blood tests is to discover if you have low or missing clotting factors, and how long it takes your blood to clot. If any of these results are positive, they shall classify the extremity of your disorder as either mild, moderate or severe. (See **Clotting . . .** ) Babies with severe hemophilia are often diagnosed during their first year of life, based on obvious symptoms. Yet people with mild or moderate hemophilia may not be diagnosed until they are older children or even when they reach adulthood, for the signs are not as obvious. Blood tests also confirm if the person diagnosed has Hemophilia A or Hemophilia B. It is important to distinguish this difference, for although they have the same symptoms, they require different treatment.
 * Diagnosis**


 * Hemophiliacs in History**

There have been some well known people through out history with hemophilia. One such person is Queen Victoria, and others in the Royal Family. Queen Victoria of England had been affected by the disease. Her one son and three grandsons had hemophilia. At least two of her daughters, and four of her grandaughters were carriers of the disease. One of her grandaughters, Empress Alexandra, was a carrier of the disease. Later she married Czar Nicolas II in 1894. Her being a carrier of the disase she was able to pass hemophilia to her son Alexis, who was heir to the Russia throne.



1) What is Hemophilia and how is it transmitted? 2) What are the key symptoms of the disorder for both children and adults? 3) What is the difference between Hemophilia A and Hemophilia B? 4) On which chromosome is the disorder carried?
 * Discussion Questions**

"Hemophilia". __National Heart Lung and Blood Institute (Diseases and Contitions).__ 9 May 2007 [|http://www.nhlbi.nih.gov/] "Hemophilia". __Reference.com__. 9 May 2007 http://www.reference.com/ Rose, Lynn A., Ph.D. "Ills and Conditions; Who Gets Hemophilia?". __Caremark Health Information__. 9 May 2007 [|http://healthresources.caremark.com/] "Hemophilia". __Human Diseases and Conditions__. [|http://www.humanillnesses.com/] Friedlander, Ed, M.D., Pathologist. "Coagulation Disorders" __Coagulation Disorders.__10 May 2007 [|http://www.pathguy.com/] "Hemophilia". __Hemophilia.__ 15 May 2007__. [|http://library.thinkquest.org/]__
 * Resources and References**