Hemophilia A

What Is Hemophilia A?

When you have hemophilia, your blood doesn't clot 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.

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

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 mutation.

The disease nearly always shows up in boys, not girls. Women can be carriers of the troublesome gene but only get some signs of the illness themselves. They pass the gene to their sons, who get hemophilia.

Symptoms

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

Mild hemophilia A. If you're in this group, you may only notice bleeding after a serious injury. Or it could happen after you have surgery or another procedure, like having a tooth pulled. For a lot of people with mild hemophilia, they don't  get a diagnosis until after one of these things happens to them when they're adults.

Moderate hemophilia A. You most likely won't notice any bleeding problems until after you're injured. Sometimes, though, you may have bleeding that starts up without an injury.

Severe hemophilia A. In addition to bleeding trouble 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.

It's important to remember that when you have hemophilia A, bleeding can also happen in your brain. If you have a head bump -- even if it's minor -- and you have any of these symptoms, call your doctor:

  • Headache
  • Neck pain and stiffness
  • Throwing up
  • Sleepiness
  • Sudden weakness or problems walking

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Getting a Diagnosis

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 are you concerned about and why?
  • 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)
  • 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.
  • 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.

One other thing to keep in mind if you're pregnant and hemophilia runs in your family: Ask your doctor about testing to see if your baby has the disease. There are some risks to the child from the tests, so be sure to weigh the pros and cons carefully.

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 his activities?
  • Do you need to let teachers and care providers know about his 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?

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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.

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.

A good place to go for treatment is a center that specializes in caring for people with hemophilia. You'll get to work with a team of health pros, such as hematologists, surgeons, dentists, physical therapists, and social workers.

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 that's made from cells that are genetically engineered to produce factor VIII. Some of them have "extended half-lives," which means they can stay in the blood longer.

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 epsilon 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 works by bridging the gap in the clotting factors left by the missing factor VIII. You take this medication weekly with an under-the-skin (subcutaneous) injection. A health care professional can give the injection, or you can learn to do it yourself.

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Taking Care of Yourself

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.

  1. 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.

Certain medications, like aspirin, can slow blood clotting, so check with your doctor about what you should and shouldn't take. 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, he 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.

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 his hemophilia. Let him know when and how to get help so he feels confident, not scared. 

What to Expect

Although there's no cure for the condition, 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.

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 October 02, 2018

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."

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