The following information applies to patients with congenital hemophilia A or B with or without inhibitors. Click here for congenital factor VII deficiency or acquired hemophilia, or Congenital Factor XIII Deficiency.
Hemophilia is a chronic, inherited bleeding disorder that primarily affects males. It is sex-linked, meaning that it is passed on from mother to child on the X chromosome. People with hemophilia A lack the factor VIII protein, and people with hemophilia B lack the factor IX protein. These blood clotting proteins are essential for proper blood clotting. Because they are missing these factor proteins, people with hemophilia have a tendency to bleed longer than most.
Bleeds are a big part of hemophilia. They can come from an injury or spontaneously. Spotting a bleed is vital. The more you know about spotting a bleed, the quicker you’ll be able to start treatment.
Before you can treat a bleed, you must know the signs. Bleeding can occur inside or outside the body. Bleeds inside the body can be harder to spot. Kids may be too young to say how they feel. So knowing the unique signs in children can help.
There are many types of bleeds. Bleeds can happen in the joints, muscles, head, spine, and the stomach or intestines.
Joint bleeds are the most common. They can happen in any joint, but the knees, ankles, and elbows are the most common. Not treating joint bleeds quickly may result in more blood in the joint that may cause damage. You could lose your range of motion, have pain when you move, or even lose the ability to walk on your own. Learning the language of your own body can help you identify joint bleeds more easily—making earlier treatment possible.
Common sites for joint bleeds are the knees, ankles and elbows. Bleeds can also occur in the toe, shoulder, and hip joints. Repeat joint bleeds can lead to hemophilic arthropathy or joint damage.
What to watch for: During a joint bleed, there may be a tingling or bubbling feeling. There may also be limited motion, swelling, pain that worsens with time, or general unwillingness to move the joint. You may also feel warmth on the skin over the joint.
What to watch for: During a muscle bleed, the muscle swells and feels warm, stiff, and painful. Bruising may be seen if the bleed is near the skin. Bleeds in deeper muscles can put pressure on nerves and blood vessels. This causes tingling and numbness and may cause permanent damage. A muscle spasm may occur when the muscle tightens up to protect itself.
What to watch for: If you or your child suffers from a persistent headache or feels a lack of energy, it could point to a head bleed. Trouble walking straight or at all, vision problems, bleeding from the ears or nose, vomiting, dizziness, or seizures are symptoms that may occur.
What to watch for: Your arms or legs may feel weak, or you may have pain or tingling. Trouble with urination or bowel movements can also be a sign of pressure on the spinal cord from a bleed.
What to watch for: Symptoms include vomiting blood or black, syrup-like material or red or black bowel movements resembling tar.
Throat bleeds cause swelling. This can make it hard to swallow or breathe. Bleeding in the throat can come from infection, injury, dental procedures, or elective surgery.
Bleeds into the eyes, spine, and psoas muscle can also be serious.
Each person with a bleeding disorder is unique; and individualized treatment is important. Identifying therapies that stop bleeds quickly can reduce the blood in the joint. Work with your hemophilia treatment center (HTC) to design a plan that meets your specific needs. Together, you will find the therapy that works best to stop bleeds.
Inhibitors are antibodies that stop, or inhibit, factor from working. Just as the immune system protects the body from things like bacteria and viruses, it sometimes responds to factor as if it’s one of these body invaders and makes antibodies – or inhibitors – to fight against the factor. Inhibitors only develop after the person with hemophilia has received replacement factor.
People with inhibitors may need to use bypassing agents. These work to bypass the steps where factor VIII or IX is needed so the blood clot can form.
A blood test called the Bethesda assay or inhibitor assay is used to detect inhibitors. The test measures the amount of antibody in your blood.
Exercise and nutrition are important matters for anyone looking to lead a healthy lifestyle. But when it comes to hemophilia, you should keep a healthy body weight. Healthy weight can mean less stress on joints. As you age, maintaining a healthy weight through diet and exercise becomes important as you take care of health issues in addition to hemophilia. You should talk to your treatment team about the timing and exercises that are right for you.
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