Hemophilia A

What Is Hemophilia A?

Hemophilia A is a condition that keeps your blood from clotting normally. That means your body has problems stopping bleeding, both outside and inside your body.

Your condition can be treated, and in some cases you can give yourself the treatment at home. You can lead a full life when you have the right treatment plan in place.

There are different types of hemophilia. With hemophilia A, your body doesn't have enough of a protein called factor VIII, which it needs to make clots and stop bleeding.

Hemophilia A can be mild, moderate, or severe, depending on how little you have of factor VIII. More than half of those with the condition have the severe form.

Hemophilia A usually runs in families, but about one-third of people with the disease don't have a family history of it.

Hemophilia A Causes

Hemophilia A comes from your genes. You can inherit it from your parents. Or it can happen if a certain gene changes before you're born. This change is called a spontaneous mutation.

The hemophilia A gene mutation happens on the X chromosome. Women get one X chromosome from their mother and one from their father. Men get an X from their mother and a Y from their father.

Most women with the problem gene are carriers and have no symptoms, but they can pass it to their sons. Therefore the disease nearly always shows up in boys, not girls.

A rare type of hemophilia A is acquired hemophilia. It’s most common in people 60-80 years of age. If you have this kind, your immune system attacks healthy tissue called clotting factors in your blood. Acquired hemophilia tends to happen alongside these conditions:

Hemophilia A Symptoms

The symptoms of hemophilia A depend on whether you have a mild, moderate, or severe case.

Mild hemophilia A. If you're in this group, you may have an episode of heavy bleeding:

  • After you have surgery or another procedure, like having a tooth pulled.
  • After childbirth
  • After a serious injury
  • During your period

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Some people don't get a diagnosis until after one of these things happens to them when they're adults.

Moderate hemophilia A. Moderate hemophilia usually means you:

  • Bleed a lot when injured.
  • Bleed without an injury (spontaneous bleeding).
  • Bruise easily.
  • Bleed a lot after a vaccination.

Severe hemophilia A. In addition to bleeding after an injury, you may also have frequent episodes of bleeding, often into the joints and muscles, without being able to figure out a specific cause.

When you have severe hemophilia A, bleeding can also happen in your brain. It’s rare, but if you have a head bump -- even if it's minor -- and you have any of these symptoms, call your doctor:

  • Prolonged headache
  • Throwing up
  • Sleepiness/tiredness
  • Sudden weakness or problems walking
  • Double vision
  • Convulsions or seizures

Hemophilia A Diagnosis

If you have a family history of hemophilia and are pregnant, tests can tell if your baby has the disease. There are risks though, so you’ll want to talk to your doctor about testing.

In children, doctors usually diagnose severe cases in the first year of a baby’s life. Does your child bruise easily or bleed for longer than normal from small injuries? Make an appointment with your child's doctor. They can tell if it's hemophilia.

It's unusual for a baby younger than 6 months old to be diagnosed with hemophilia. That's because infants this young usually don't do things that would make them bleed.

Once a baby starts moving, crawling, and bumping into things, you may notice raised bruises, especially on places like the stomach, chest, back, and bottom.

The doctor may ask:

  • What happened to cause the bumps, bruises, and bleeding?
  • How long did the bleeding last?
  • Is your child taking any medications?
  • Are there any other medical concerns you have?
  • What's your family's medical history? Does anyone have a problem with blood clotting?

The doctor may also do blood tests, including:

  • Complete blood count (CBC). This test looks at the number of different types of cells in your blood as well as your hemoglobin levels to see if bleeding has lowered them.
  • Prothrombin time (PT) and activated partial thromboplastin time (PTT). Both of these tests check how long it takes blood to clot.
  • Factor VIII and factor IX tests, which measure levels of each of those proteins. Factor VIII is for hemophilia A. Factor IX is for hemophilia B, another type of hemophilia.
  • Fibrinogen test. This test shows how well you form clots.
  • Genetic testing, which can help you gauge your chances of having complications from some treatments. And if you're a woman, you'll learn if you're a carrier.

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Questions for Your Doctor

If you or your child is diagnosed with hemophilia, you'll probably have a lot of questions for your doctor, including:

  • How often will you need to visit the doctor?
  • How long is too long to bleed from a small cut?
  • Are some symptoms more serious than others? What are they? And what do you do if you have them?
  • Are there over-the-counter medicines you should or shouldn't use?
  • What treatments does the doctor recommend?
  • How do you keep your child safe? Do you need to limit their activities?
  • Do you need to let teachers and care providers know about their condition?
  • What can you expect as your child gets older?
  • How do you connect with other families that have kids with this condition?
  • What are the chances your other children will have hemophilia? Or that your grandchildren will have it?

Hemophilia A Treatment

The kind of treatment you get depends on a lot of things, including how severe your condition is, how old you are, and your personal needs.

The best place to go for treatment is a federally funded hemophilia treatment center (HTC). You’ll work with a team of specialists, such as surgeons, dentists, physical therapists, and social workers who focus on the blood disorder.

You might need to get regular treatment to prevent bleeding, which you may hear your doctor call "prophylactic" therapy. Or you may need treatment at the time your bleeding happens, which is called "on-demand" therapy.

The goal of treatment is to give you the factor VIII your body doesn't make. That's called replacement therapy. It's not a cure, but it does help control your hemophilia.

Replacement therapy works in most cases. But there's a 20% to 30% chance your body will reject it. In that case, your doctors may try different sources for factor VIII, or try different amounts.

There are two types of factor VIII that your doctor may suggest:

  • A "concentrated" version that comes from human blood.
  • A "recombinant" version, which is more common, is made from cells that are genetically engineered to make factor VIII. Some of them have "extended half-lives," which means they can stay in the blood longer.

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These products go into your bloodstream through an IV.

If you have mild or moderate hemophilia A, you may be treated with desmopressin (DDAVP). Desmopressin raises the levels of factor VIII in the blood, and you take it through an IV or as a nasal spray.

Drugs known as antifibrinolytics, such as aminocaproic acid and tranexamic acid, can also help if you have a mild form of the disorder. You take them by mouth. They slow the breakdown of clotting factors in the blood.

Emicizumab-kxwh (Hemlibra) is a medicine that can help prevent or reduce the frequency of bleeding episodes in adults and children with hemophilia A. It bridges the gap in the clotting factors left by the missing factor VIII. You take this medication weekly with an injection. A health care professional can give the injection, or you can learn to do it yourself.

Work closely with your doctor if you’re prepping for surgery, travel, pregnancy, or delivery.

Since hemophilia A may mean you get blood donations, you should get vaccinated for hepatitis A and B.

Hemophilia A Outlook

You can do a lot to help manage your hemophilia day to day. Keep up with your treatment, follow your doctor's advice, and do these four things:

  1. Handle injuries correctly. Clean small cuts, scrapes, and injuries. Then apply pressure and a bandage. Serious injuries need medical attention.
  2. Tell all your health care professionals that you have (or your child has) hemophilia. Remind them before you schedule any procedures, like dental work. You may need to take medications to help clot your blood beforehand. Keep good dental hygiene so you can help prevent dental work that could cause bleeding.

Certain medications, like aspirin and other NSAIDs, can slow blood clotting, so check with your doctor about what you should and shouldn't take. Avoid blood thinners. Also check with your doctor about vaccines, like those for hepatitis A and B, that you may need. Most blood products these days are checked for things that can cause disease, but it's still worthwhile to check if you need to take steps to prevent getting infected.

  1. Stay active. If your child has hemophilia A, they can still be active. Exercise makes them stronger, which makes injuries less likely. Exercise also helps by managing weight -- extra pounds strain the body, which raises the risk of bleeding.

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Just take some precautions:

  • Your child should wear knee pads, elbow pads, and helmets during any activities if they're doing something that might make them fall.
  • Use safety straps in car seats, strollers, and high chairs.
  • Check your home and yard for hazards, like furniture or play equipment with sharp corners.
  1. Teach your child about their hemophilia. Let them know when and how to get help so they feel confident, not scared.

There's no cure for the condition, but with the right precautions and planning, people with hemophilia A can live healthy, full lives. Stay active, but learn to recognize the symptoms. See your doctor regularly, and follow your treatment plan.

Hemophilia A Complications

You may experience problems that stem from your hemophilia A, including:

  • Bleeding in deep muscle. This can make your arms and legs swell. The swelling can press on your nerves and lead to numbness or pain.
  • Joint damage. The same pressure from internal bleeding can affect your joints and cause severe pain. If you don’t treat it, you may eventually develop arthritis or your joint could wear away.
  • Infection. Although blood products have become much safer since the 1980s, you’re at a higher risk of receiving contaminated blood because of the number of blood transfusions you may get.
  • Bad reaction to clotting factor treatment. Some people with severe hemophilia have a negative reaction to the clotting factors that treat bleeding. This happens when the immune system makes proteins called inhibitors that turn off the clotting factors. Treatment won’t work as well when this happens.

Getting Support

Many other people have hemophilia, and their advice can be helpful and inspiring, especially if you're just starting to get familiar with the condition.

The National Hemophilia Foundation can connect you to a treatment center near you and to support groups.

The federal government pays for a network of more than 140 hemophilia treatment centers where you can get treatment, education, and other resources you need.

WebMD Medical Reference Reviewed by Neha Pathak, MD on September 03, 2020

Sources

SOURCES:

KidsHealth: "Hemophilia."

Medscape: "Hemophilia A."

National Heart, Lung, and Blood Institute: "Hemophilia."

National Hemophilia Foundation: " "Hemophilia A," "Hemophilia A (Factor VIII Deficiency)."

Medscape: "Acquired Hemophilia."

National Hemophilia Foundation: "Comprehensive Medical Care: HTCs."

Mayo Clinic: "Hemophilia."

UpToDate: "Hemophilia A and B: Routine management including prophylaxis."

National Institutes of Health: "Hemophilia A."

The Haemophilia Society: "Extended half-life (EHL) factor VIII."

CDC: “Diagnosis of Hemophilia.”

National Organization of Rare Disorders: “Hemophilia A,” “Acquired Hemophilia.”

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