Medication
Hemlibra ® (also known as ACE 910 or emicizumab) Hemlibra ® works by replacing the function of factor VIII (8), rather than replacing the missing clotting factor VIII directly. It can be used to either prevent or reduce the frequency of bleeding episodes in people with hemophilia A. This treatment product can be given by injection under the skin.
Therapy
Treatment Options for Hemophilia. The main medication to treat hemophilia A is concentrated FVIII product, called clotting factor or simply factor. There are two types of clotting factor: plasma-derived and recombinant. Plasma-derived factor is made from human plasma. Recombinant factor products are developed in a lab through the use of DNA technology.
Self-care
Treatment for hemophilia is aimed at prevention of bleeding complications (primarily head and joint bleeds). Treatment may include: Parents may want to purchase soft toys with rounded corners for young children. Padded clothing and helmets may be necessary for the child that is learning to walk or becoming more active.
Nutrition
The main treatment for hemophilia is called replacement therapy. Concentrates of clotting factor VIII (for hemophilia A) or clotting factor IX (for hemophilia B) are slowly dripped or injected into a vein. These infusions help replace the clotting factor that's missing or low. Clotting factor concentrates can be made from human blood.
How do you cure hemophilia?
The current, most-used treatment for hemophilia A is factor replacement therapy. This is done by infusing (giving medication into a vein) a FVIII product into the affected person. When you infuse, FVIII concentrate, the FVIII is immediately available in the bloodstream, and your body can use it to stop or prevent the bleeding.
Are there any cures for hemophilia?
Acquired Von Willebrand Syndrome. Bleeding Disorders. Congenital Von Willebrand Disease. Disseminated Intravascular Coagulation. Hemophilia. Hemophilia in Children.
Can you cure hemophilia?
Comprehensive Medical Care. Hemophilia treatment centers offer treatment for all bleeding disorders through integrated care. Learn more.
Can you test for hemophilia during pregnancy?
For people with a family history of hemophilia, it's possible to determine during pregnancy if the fetus is affected by hemophilia. However, the testing poses some risks to the fetus. Discuss the benefits and risks of testing with your doctor. In children and adults, a blood test can reveal a clotting-factor deficiency.
Is blood testing dangerous for a fetus?
However, the testing poses some risks to the fetus. Discuss the benefits and risks of testing with your doctor. In children and adults, a blood test can reveal a clotting-factor deficiency. Depending on the severity of the deficiency, hemophilia symptoms can first arise at various ages. Severe cases of hemophilia usually are diagnosed within ...
When is hemophilia diagnosed?
Depending on the severity of the deficiency, hemophilia symptoms can first arise at various ages. Severe cases of hemophilia usually are diagnosed within the first year of life.
What is the best way to prevent clots from breaking down?
It can be injected slowly into a vein or provided as a nasal spray. Clot-preserving medications. These medications help prevent clots from breaking down. Fibrin sealants. These medications can be applied directly to wound sites to promote clotting and healing. Fibrin sealants are especially useful in dental therapy.
What is the best treatment for internal bleeding?
Fibrin sealants. These medications can be applied directly to wound sites to promote clotting and healing. Fibrin sealants are especially useful in dental therapy. Physical therapy. It can ease signs and symptoms if internal bleeding has damaged your joints.
Can you use fibron sealant on a wound?
These medications can be applied directly to wound sites to promote clotting and healing. Fibrin sealants are especially useful in dental therapy. Physical therapy. It can ease signs and symptoms if internal bleeding has damaged your joints. If internal bleeding has caused severe damage, you may need surgery.
How to treat internal bleeding?
If internal bleeding has caused severe damage, you may need surgery. First aid for minor cuts. Using pressure and a bandage will generally take care of the bleeding. For small areas of bleeding beneath the skin, use an ice pack.
How to treat bleeding disorders?
Treatment Options for Bleeding Disorders 1 Standard half-life therapies: Standard half-life therapies are used to treat hemophilia A and B, some types of von Willebrand disease, and some rare factor disorders. Dosing can be anywhere from three times a week to every day, depending on the person. 2 Extended half-life (EHL) therapies: EHL contains a molecule that has been modified in some way to delay the breaking down of factor in the body. This results in higher levels of factor in the body lasting for longer, resulting in less frequent infusions. How long the factor is effective in the body depends on the person. Extended half-life therapies are mostly used to treat hemophilia A and B. 3 Bypassing agents are used to treat bleeds in people with hemophilia with inhibitors. These treatments contain other factors that can stimulate the formation of a clot and stop bleeding.
What is a factor replacement?
Factor replacement therapies: Often referred to as “factor,” these products use a molecule that is either similar to natural factor found in humans (recombinant) or use an actual human molecule (plasma derived.)
What is EHL therapy?
Extended half-life (EHL) therapies: EHL contains a molecule that has been modified in some way to delay the breaking down of factor in the body. This results in higher levels of factor in the body lasting for longer, resulting in less frequent infusions. How long the factor is effective in the body depends on the person.
What is extended half life therapy?
Extended half-life therapies are mostly used to treat hemophilia A and B. Bypassing agents are used to treat bleeds in people with hemophilia with inhibitors. These treatments contain other factors that can stimulate the formation of a clot and stop bleeding.
What is non factor replacement therapy?
Non-factor replacement therapies include: Emicizumab (Hemlibra) is a therapy used to treat hemophilia A, to prevent bleeding episodes in people both with and without inhibitors. It is known as a factor VIII (8) mimetic because it mimics, or imitates, the way factor VIII (8) works.
What is emicizumab used for?
Emicizumab (Hemlibra) is a therapy used to treat hemophilia A, to prevent bleeding episodes in people both with and without inhibitors. It is known as a factor VIII (8) mimetic because it mimics, or imitates, the way factor VIII (8) works.
Is emicizumab a vasopressin?
Emicizumab was approved by the FDA to treat people with hemophilia A with inhibitors in 2017 and for people with hemophilia A without inhibitors in 2018. Read MASAC's recommendation on emicizumab. Desmopressin (DDAVP) is the synthetic version of vasopressin, a natural antidiuretic hormone that helps stop bleeding.
How to treat hemophilia in children?
Treatment depends on the type and severity of the hemophilia. Treatment for hemophilia is aimed at prevention of bleeding complications (primarily head and joint bleeds). Treatment may include: 1 Parents may want to purchase soft toys with rounded corners for young children. Padded clothing and helmets may be necessary for the child that is learning to walk or becoming more active. Contact sports in school should be evaluated for risks of injury to the child. 2 Immunizations may need to be given under the skin (subcutaneous) instead of in the muscle (intra muscular) to prevent deep muscle bleeds. 3 Joint hemorrhages may require surgery and/or immobilization. Rehabilitation of the affected joint may include physical therapy and exercise to strengthen the muscles around the area. 4 Before surgery, including dental work, your child's doctor may recommend factor replacement infusions to increase the child's clotting levels prior to the procedures. Your child may also receive the specific factor replacement infusions during and after the procedure to maintain the clotting factor levels and to improve healing and prevention of bleeding after the procedure. 5 Your child's doctor may also recommend the discontinuation of aspirin, and aspirin-containing products, since these products have been linked to bleeding problems. 6 Blood transfusions may be necessary if significant blood loss has occurred. 7 Proper dental hygiene is a preventive measure. 8 Prophylactic self-infusion of factor VIII or IX can allow a child with hemophilia to lead a near normal lifestyle.
How to determine if you have hemophilia?
Specific treatment for hemophilia will be determined by your doctor based on: 1 Your age, overall health, and medical history 2 Extent of the disease 3 Your tolerance for specific medications, procedures, or therapies 4 Expectations for the course of the disease 5 Your opinion or preference
What is the treatment for hemophilia?
The main treatment for hemophilia is called replacement therapy . Concentrates of clotting factor VIII (for hemophilia A) or clotting factor IX (for hemophilia B) are slowly dripped or injected into a vein. These infusions help replace the clotting factor that's missing or low. Clotting factor concentrates can be made from human blood.
What is clotting factor VIII?
Concentrates of clotting factor VIII (for hemophilia A) or clotting factor IX (for hemophilia B) are slowly dripped or injected into a vein. These infusions help replace the clotting factor that's missing or low. Clotting factor concentrates can be made from human blood. The blood is treated to prevent the spread of diseases, such as hepatitis.
What is clotting factor concentrate?
Clotting factor concentrates can be made from human blood. The blood is treated to prevent the spread of diseases , such as hepatitis. With the current methods of screening and treating donated blood, the risk of getting an infectious disease from human clotting factors is very small.
Why is blood treated?
The blood is treated to prevent the spread of diseases, such as hepatitis. With the current methods of screening and treating donated blood, the risk of getting an infectious disease from human clotting factors is very small.
What is replacement therapy?
You may have replacement therapy on a regular basis to prevent bleeding. This is called preventive or prophylactic (PRO-fih-lac-tik) therapy. Or, you may only need replacement therapy to stop bleeding when it occurs. This use of the treatment, on an as-needed basis, is called demand therapy.
Can you get replacement therapy for bleeding?
Or, you may only need replacement therapy to stop bleeding when it occurs. This use of the treatment, on an as-needed basis, is called demand therapy. Demand therapy is less intensive and expensive than preventive therapy. However, there's a risk that bleeding will cause damage before you receive the demand therapy.
What is demand therapy?
This use of the treatment, on an as-needed basis, is called demand therapy. Demand therapy is less intensive and expensive than preventive therapy. However, there's a risk that bleeding will cause damage before you receive the demand therapy.
How to treat hemophilia A?
This is done by infusing (giving medication into a vein) a FVIII product into the affected person. When you infuse, FVIII concentrate, the FVIII is immediately available in the bloodstream, and your body can use it to stop or prevent the bleeding.
What does it mean to be on FVIII?
Being on prophylaxis means infusing FVIII on a regular schedule to prevent bleeds from occurring. Prophylaxis is recommended for children with severe hemophilia, but it can also be recommended for those who have frequent bleeds. Prophylaxis may also be used in advance of activities that are likely to cause a bleed. There are two types of prophylaxis:
Can you infuse FVIII into bloodstream?
All FVIII products need to be infused directly into the bloodstream. There are several ways to infuse FVIII, and each has its advantages and disadvantages. These include peripheral infusion (accessing a vein with a butterfly needle), port-a-catheter (also called a port), a peripherally inserted central catheter (PICC) line, or a BROVIAC® or HICKMAN® catheter.
What is port in medical terms?
A port is a device that is surgically implanted under the skin. It has tubing that is inserted into a central vein. In most cases, it is placed on the chest. Below are some considerations regarding this type of access:
What is a broviac catheter?
BROVIAC® catheters and HICKMAN® catheters (often referred to as “broviacs”) are similar to a PICC line. However, A BROVIAC® catheter is placed directly into a central vein and threaded to a position just above the heart. It is tunneled under the skin and brought out away from the site where it enters the vein to theoretically prevent bacteria from gaining access to the central portion of the catheter. BROVIAC® catheters contain a “cuff” that is under the skin. As the skin grows into this “cuff,” the catheter becomes more stable, reducing the chance of it becoming pulled out. Like the PICC line, his device is easy to use and does not require sticks into the person’s skin. The difference is that these catheters can last for a prolonged period of time. The most common long-term complications of this type of central line are infection, movement that takes the catheter out of the proper position, and damage to the catheter.
What is plasma derived product?
A plasma-derived product is a factor concentrate made from carefully-screened, donated blood plasma. During the manufacturing process, the proteins extracted from the plasma go through a series of extensive sterilization procedures to eliminate viruses and other contaminants.
When did cloning of FVIII and FIX occur?
In the early 1980s, the cloning of FVIII and FIX and subsequent expression of functional proteins occurred. This was during the “bad blood” era (when many individuals with hemophilia being treated with plasma-derived clotting factor were infected with HIV and/or HCV). This led to the development and commercialization of recombinant clotting factors. These initial products were manufactured using Chinese hamster ovary cells or baby mouse kidney cells. Manufacturers injected these cells with the factor gene so that large amounts of the factor protein could be produced. Since the proteins are extracted from animal cells, they are not at risk for containing human viruses. Some factor concentrate products are stabilized using human albumin, while others are stabilized using sucrose. This was life-changing for many with hemophilia, allowing for prophylactic (or preventive) treatment. Some companies are now using human embryonic kidney (HEK) cells to grow the factor.
Diagnosis
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Preparing For Your Appointment
- The main treatment for severe hemophilia involves replacing the clotting factor you need through a tube in a vein. This replacement therapy can be given to treat a bleeding episode in progress. It can also be given on a regular schedule at home to help prevent bleeding episodes. Some people receive continuous replacement therapy. Replacement clotti...