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Now is your chance to share your thoughts about what or who inspires hope in your life. Whether it’s a teacher or coach who has encouraged you, volunteering your time in the community, or friends and family, share with others what makes you feel hopeful. Complete your entry by following @CHShemophilia, registering on this contest webpage and tweet about what gives you hope. Make sure that you tag #Tweet4Hope to be entered to win.
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Each week, a winner of $500 will be selected via random draw, with the grand prize ($1,000) winner randomly selected on February 15, 2017 at approximately 5:00 p.m. EST.
Dates & Eligibility
Start : January 09, 2017 @ 01:00 am (US/Eastern)
End : February 15, 2017 @ 12:00 pm (US/Eastern)
What is hemophilia and how common is it? The word hemophilia derives from two Greek words: haima, meaning blood, and philia, meaning affection. Hemophilia is a hereditary condition. This means that it is passed on from mother to child at the time of conception. The blood of a person with hemophilia does not clot normally. He does not bleed more profusely or more quickly than other people; however, he bleeds for a longer time. Many people believe that hemophiliacs bleed a lot from minor cuts. This is a myth. External wounds are usually not serious. Far more important is internal bleeding (hemorrhaging). These hemorrhages are in joints, especially knees, ankles and elbows; and into tissues and muscles. When bleeding occurs in a vital organ, especially the brain, a hemophiliac's life is in danger. Both hemophilia A and B are very rare disorders. Hemophilia A affects fewer than 1 in 10,000 people, or about 2,500 Canadians. Hemophilia B is even less common, affecting approximately 1 in 50,000 people, or about 600 Canadians.
Who is affected by hemophilia? Hemophilia affects people of all races, colours and ethnic origins. The most severe forms of hemophilia affect almost only males. Females can be seriously affected only if the father is a hemophiliac and the mother is a carrier, or in the case of X-inactivation when a woman's normal X-chromosome is inactive in the production of factor VIII or IX. These cases are extremely rare. However, many women who are carriers have symptoms of mild hemophilia. We are only now fully recognizing the importance of bleeding in carriers and the degree to which these symptoms affect a woman's quality of life. As hemophilia is a hereditary disorder, people are affected at birth. This means that children can have hemophilia. In fact, hemophilia is often diagnosed in the first year of life.
How serious is hemophilia? Without proper treatment, hemophilia is crippling and often fatal. With modern treatment, most people with hemophilia can lead full, active lives. Hemophilia is classified as severe, moderate or mild.
|CLASSIFICATION||LEVEL OF FACTOR VIII OR IX IN THE BLOOD|
|Severe||Less than 1% of normal|
|Moderate||1 to 5% of normal|
|Mild||5 to 30% normal|
The clotting problem in hemophilia Blood is carried throughout the body within a network of blood vessels. When tissues are injured, damage to a blood vessel may result in leakage of blood through holes in the vessel wall. The vessels can break near the surface, as in a cut. Or they can break deep inside the body, making a bruise or an internal hemorrhage. Platelets are small cells circulating in the blood. Each platelet is less than 1/10,000 of a centimetre in diameter. There are 150 to 400 billion platelets in a normal litre of blood. The platelets play an important role in stopping bleeding by clumping together and forming a plug, thereby beginning the repair of injured blood vessels. Clotting factors like factor VIII and IX are then needed to glue the plug in place thus forming a clot. When a blood vessel is damaged, there are four stages in the normal formation of a clot. Stage 1: The blood vessels are damaged and the bleedings starts. Stage 2: The blood vessels constrict to slow the flow of blood to the injured area. Stage 3: Platelets stick to, and spread on, the walls of damaged blood vessels. This is called platelet adhesion. These spreading platelets release substances that activate other nearby platelets which clump at the site of injury to form a platelet plug. This is called platelet aggregation. Stage 4: The surface of these activated platelets then provides a site for blood clotting to occur. Clotting proteins like factor VIII and IX circulating in the blood are activated on the surface of the platelets to form a mesh-like fibrin clot. These proteins (factors I, II, V, VII, VIII, IX, X, XI, XII, XIII and von Willebrand factor) work like dominos, in a chain reaction. This is called the coagulation cascade.
What is the clotting problem in hemophilia? When one of the proteins, for example, factor VIII, is absent, the dominos stop falling, and the chain reaction is broken. Clotting does not happen, or it happens much more slowly than normal. The platelets at the site of the injury do not mesh into place to form a permanent clot. The clot is 'soft' and easily displaced. Without treatment, bleeding will continue until the pressure outside the broken vessel is equal to the pressure inside. This can take days and sometimes weeks.
What is comprehensive hemophilia care? Comprehensive hemophilia care is: all of the medical services needed by a hemophiliac and his family for the treatment of hemophilia and related conditions. This care is provided at a comprehensive care clinic. This is a place where a person with hemophilia or a bleeding disorder can receive all the care he / she needs at one time. It is called a comprehensive care clinic because it offers a complete range of services. The following people work there: The medical director is often a hematologist who specializes in the area of blood clotting. He / she prescribe the lab tests to find out the exact bleeding problem; prescribes the proper treatment to control and prevent bleeding and monitors the overall health of the hemophiliac. The nurse coordinator is the front-line person in the clinic. He/she helps families deal with the day-to- day problems related to hemophilia; answers families' questions over the phone or at the clinic and provides out-patient care at the clinic. He/she teaches families how to do home therapy; organizes the delivery of blood products for home use; and coordinates appointments with other members of the comprehensive care team. The physiotherapist checks the hemophiliac's joints and muscles to make sure joint movement is not lost and that muscles remain strong; helps the hemophiliac with exercises to regain lost joint function or to rebuild muscles; and helps the hemophiliac to find a sports and exercise program to keep him in top shape. The social worker’s role is to help parents, siblings, and people with hemophilia and other family members, including carriers, deal with the impact that hemophilia can have on their lives. The dentist provides dental care and works closely with the hematologist to prevent bleeding during dental work. The comprehensive care team will add other individuals as needed, such as an obstetrician / gynecologist (to help women with bleeding problems); a genetic counsellor (to give information to carriers); an orthopedic specialist (for joint problems and joint surgery); an HIV specialist (for treatment related to HIV infection); and a hepatologist or gastroenterologist (for treatment related to hepatitis C infection).
What is home care? Home care is the infusion of factor concentrates at home. In Canada most severe hemophiliacs are treated at home. This has major advantages over treatment at the hemophilia clinic or at the emergency room. These are: quicker treatment when a bleed starts; a more normal life for the hemophiliac and other members of the family; a greater acceptance of treatment by the young child and the ability for people to take care of their own health. The comprehensive care clinic team trains the family with a young hemophiliac how to recognize bleeds and then how to infuse the factor concentrate. Children often learn how to infuse themselves at the age of eight or ten. Then, the hemophiliac is able to treat himself at home, at school, at camp or on vacation. Hemophiliacs on home care go to the comprehensive care clinic once or twice a year for a complete check-up.
What is factor replacement therapy? The basic treatment to stop or prevent bleeding in people with hemophilia A and B is factor replacement therapy. This is the infusion (injection into the bloodstream) of factor VIII and IX concentrates to prevent or control bleeding. These concentrates come from two sources - human plasma (a component of blood) and a genetically engineered cell line made by DNA technology, called recombinant. In both cases, the factor VIII or IX protein is nearly identical to the protein which is lacking in the blood of hemophiliacs. After an infusion of the concentrate, all the proteins needed for clotting are in place. A hemophiliac's blood becomes 'normal', at least for a few hours. This allows the time for a clot to form at the site of the damaged blood vessel. (See the clotting problem in hemophilia.) Unfortunately, the replacement of the missing clotting factors is not permanent. Half of the clotting factor activity which was infused is removed by the body every 12 to 24 hours. This means that within 2 or 3 days almost none is left. The hemophiliac's blood is again unable to clot normally.
What are the advantages of today's factor concentrates? The clotting factors available in Canada today have many advantages over products available in the 1950s, 1960s, 1970s and 1980s. These are the advantages.
- • They are very concentrated. This means that a small amount contains enough factor VIII or IX activity to control bleeding, even in major surgery. Thus, they are very effective.
- • They are convenient. The concentrates can be stored in a home refrigerator for up to a year or kept at room temperature for 3 to twelve months.
- • They are easy to mix. A small amount of sterile water, about 5 to 10 millilitres, is mixed with the powdered concentrate. A few seconds to one minute later the preparation is ready to be infused.
- • They are quick to infuse. From beginning to end, the infusion takes no more than 15 to 20 minutes.
- • They are very safe. None of the factor VIII or IX concentrates, whether plasma-derived or recombinant, used in Canada since 1988 has ever been known to transmit HIV or hepatitis.
How are factor VIII and IX concentrates made? Factor VIII and IX concentrates can be divided into two categories - recombinant, genetically engineered factor VIII and IX; plasma-derived factor VIII and IX.
Recombinant, genetically engineered factor VIII and IX
|Product||Albumin as stablizer||Human protein in cell culture||Viral inactivation||Vial Size|