Hemophilia A is a type of blood disorder that involves less effective blood clotting. When your blood doesn’t clot adequately, minor injuries or procedures (such as dental work) can cause your loved one to bleed excessively. In some instances, bleeds can even occur without any identifiable cause.
These 10 words about Hemophilia A will make it easier for you to understand prevention and treatments for your loved one.
Clotting factor VIII
Clotting factor VIII is at the root of hemophilia A. If your loved one has this bleeding disorder, their blood has less, or is lacking, a protein called factor VIII. It’s responsible for helping the body develop natural clots to stop bleeding.
Mild, moderate, and severe hemophilia
Hemophilia A is classified into three types: mild, moderate, and severe.
- Mild: Prolonged or excessive bleeding only happens occasionally, usually after surgery or injury.
- Moderate: Bleeding may occur after most injuries and sometimes spontaneously, but not often.
- Severe: The most common type of Hemophilia A. Severe hemophilia is diagnosed during infancy. It can cause bleeding spontaneously multiple times per week.
Knowing the severity of their condition can help you better prevent and treat bleeding episodes for your loved one.
When you think of bleeds, you likely think of external bleeding. But internal bleeding can be an even bigger issue — because you can’t necessarily see it. Internal bleeding may lead to damage to the nerves, joints, and other body systems. Some signs and symptoms of internal bleeding include:
- pain or swelling around a joint
- vomiting blood
- black or bloody stools
- sudden or severe headache
- chest or other significant pain, especially after trauma
With severe hemophilia, internal bleeding can happen even without an injury.
Generally, prophylactic treatment is taken as a preventative measure to keep from getting a disease. Prophylaxis for hemophilia is designed to stop bleeds before they begin. It’s taken as an infusion and includes the clotting factor VIII your loved one needs to form clots on their own. More severe cases require more frequent treatments. These treatments may even be administered at home.
Recombinant clotting factors
In the past, infusion treatments used clotting factor derived from plasma. Now, doctors primarily recommend recombinant clotting factor infusions. These infusions contain clotting factor VIII that is man-made to stop and prevent spontaneous bleeding. According to the National Hemophilia Foundation, about 75 percent of people with hemophilia use recombinant clotting factors, as opposed to plasma-derived factor, as part of their overall treatment plan.
A port-a-cath is a venous access device (VAD) that’s implanted in the skin around the chest. It’s connected to a vein with a catheter. A port-a-cath may be useful if your loved one receives regular infusions because it takes the guesswork out of trying to locate a vein each time. The downside of this device is a higher risk of infections.
Desmopressin acetate (DDAVP) is an on-demand or rescue treatment for hemophilia A. It’s only used for mild-to-moderate cases. DDAVP is made from a synthetic hormone that’s injected into your bloodstream to induce clotting factors in case of a sudden injury or bleeding episode. It’s sometimes used prophylactically before surgeries. Your loved one may need to go to the doctor to receive these injections. DDAVP also comes in a nasal spray for home use. Both the injectable form and the nasal spray product should be used sparingly to prevent building up an immunity to the drug’s effect.
Antifibrinolytics are medications sometimes used alongside infusions. They help prevent the breakdown of a blood clot once it’s formed. These drugs are available in pill form, and they may be taken prior to surgery or dental work. They’re also sometimes used in case of mild intestinal or mouth bleeds.
Some people with hemophilia A eventually stop responding to treatment. The body creates antibodies that attack the clotting factor VIII taken via infusion. These antibodies are called inhibitors. According to the National Heart, Lung, and Blood Institute, up to 30 percent of people receiving clotting factors develop these inhibitors. It’s more common in severe hemophilia A.
This treatment involves genetic modifications that help treat the lack of clotting factor VIII that leads to hemophilia A. While early research is promising, many more studies need to be done on gene therapy to ensure safety and efficacy. Your loved one may even consider participating in clinical trials. The hope is that gene therapy may lead to an eventual cure for this blood disorder.