What is Hemophilia?
Hemophilia is a problem with your blood. It is a type of bleeding disorder.
A bleeding disorder makes it hard for blood to clot. A clot is a “plug” that stops bleeding. There is something in blood called “clotting factors.” Clotting factors help make the plug. Some people don’t have enough clotting factors. This means you will bleed for too long after getting hurt. Longer bleeding also happens after surgery. Bleeding can even start for no reason. This is called spontaneous bleeding.
This disease is passed down from parent to child. In the United States, about 18,000 people have it and approximately 400 babies are born with it every year. Hemophilia is much more common in males than females.
How will I feel with hemophilia?
Things that can happen:
- Easy bleeding
- Bleeding that starts for no reason
- Lots of bleeding after an injury
- Bleeding that lasts a long time
- Lots of bruises
- Bruises that happen for no reason
- Joint pain and swelling (from bleeding inside the body)
- Blood in your urine or stool
- Nosebleeds that start for no reason
- Bleeding after a shot
Go to the ER if you have:
- Bleeding that isn’t stopping
- Headache that hurts very bad and lasts a long time
- Lots of throwing up
- Neck pain
- Seeing double
What are my treatment options?
There is no cure for hemophilia. There are treatments that work. Treatments will let you live a fairly normal life. The main treatment for most people is called “infusion therapy.” Infusion gives your body the clotting factor it needs. Most people can do this at home.
Your doctor will tell you how to infuse your clotting factor. It will be one of these ways:
1. Demand therapy means taking clotting factor when you start bleeding. You will get this only if you are bleeding. You will also get this if you are bleeding inside your body.
2. Preventive therapy is also called prophylaxis or “prophy.” It means getting clotting factor regularly. You can do this at home. You do this so bleeding doesn’t start. You will probably do this two or three times a week. Prophylaxis is good because:
- Quicker treatment for any new bleeding
- Less chance of problems like bleeding inside your head
- Not as many trips to the doctor or ER
- Joints stay healthier
- Costs less
- Less stressful
Your doctor will probably want you to do prophylaxis. It is better for people with hemophilia. Prophylaxis prevents bleeding. People using it are healthier and have a better quality of life. It can help you live a more normal life. The World Federation of Hemophilia, the National Hemophilia Foundation, and the World Health Organization all say it is best.
Other Medicines Your doctor might have you take other medicines for hemophilia:
Desmopressin (DDAVP) Also called Stimate. This is a hormone. It makes blood clot better. You could get it as a nose spray. It is for mild or moderate hemophilia A. It is not for severe hemophilia A or hemophilia B.
Aminocaproic Acid Also called Amicar. This medicine is used with clotting factor. It stops clots from breaking down. Your doctor could give it for teething, before dental work, or for other bleeding in the mouth, nose, or digestive tract.
Plasma Infusions This stops bleeding in hemophilia C.
What side effects can I expect? Some people can have a reaction to clotting factor. This happens when your immune system thinks the clotting factor shouldn’t be in your body. When this happens, something called “inhibitors” stops clotting factor from working. The inhibitors destroy the clotting factor before it can stop the bleeding. Inhibitors can be a big problem. They can make you bleed more and hurt your joints.
About one in four people with severe hemophilia A get this problem.
About one in 100 people with hemophilia B get it.
You are more likely to get inhibitors if you have:
- Another family member with inhibitors
- African ancestry
- A large amount of clotting factor given at a young age
- A more severe disease
You are less likely to get inhibitors if you are on prophylaxis. It is even better if prophylaxis started when you were very young.
Your doctor will probably find out about inhibitors if your treatment doesn’t work as well as it should. Your doctor will check for inhibitors on a regular basis. You will also be checked before any surgery.
In the past, people with hemophilia sometimes got HIV and hepatitis infections. This happened from infected blood products. In the mid-1980s doctors learned how to keep blood products safer. There is only a very small risk today. Get shots for hepatitis A and hepatitis B to stay even safer.
Today most people use a different type of clotting factor. It is called recombinant factor. There is almost no chance of infection from it.
What will happen in the future? There is no cure. This means that you will have the disease for your entire life. However, treatments and lifestyle changes can let you live a normal life.
If internal bleeding hurts one of your joints very badly, you might need a joint replacement.
Should I make lifestyle changes? Exercise is good for you. Exercise makes muscles stronger. It protects your joints. Do things like swimming, bicycling, and walking. Don’t do contact sports like football, hockey, and wrestling.
You should wear a medical alert bracelet. Some medicines can make you bleed more. Examples are aspirin, ibuprofen, and blood-thinners. Don’t take these. You should take good care of your teeth. You want to have healthy teeth so you don’t need serious dental work. If you need to have a tooth pulled it could make you bleed a lot.
Your child can have a normal life. But some extra care is a good idea. Baby-proof your house. Take away furniture with sharp corners for the first couple of years. Add padding to places like fireplace hearths.
Have your child use kneepads, elbow pads, and helmets during sports. For toddlers, consider knee and elbow pads until they are steadier on their feet.
Tell your child’s caregivers about the disease. Let them know what they should do in an emergency. Tell them to give clotting factor first. This should happen before the injury is checked by a doctor.
How did I get hemophilia?
Most of the time, babies get this disease from their mother. Many more boys are born with this disease. It does not happen often in girls.
Sometimes there is a “spontaneous mutation.” This means the baby has no family members with this disease. The disease starts for no reason. This happens about 30 percent of the time.
You could be a “symptomatic carrier.” This means you have the hemophilia gene but you don’t have the disease. But you can have some symptoms. If you are a symptomatic carrier, your doctor might give you medicine. You might only need this medicine before surgery or dental work.
How will I know I have hemophilia?
All bleeding disorders make you bleed for longer than you should. If someone else in your family has hemophilia, you might find out about your disease when you are young. Your doctor will ask if anyone else in your family has a bleeding problem. Your doctor might even know before your birth.
Your doctor will check your blood for clotting factors. Your doctor will also check how long it takes for your blood to clot.
Some people do not find out about having hemophilia until they are grown up. This is more likely in mild cases.
Could I have something else?
Your doctor will know for sure if you have hemophilia. Other bleeding disorders, such as von Willebrand disease, will be checked for and ruled out.
Can I prevent hemophilia?
Hemophilia cannot be prevented. However, if you are a woman, your doctor can check your baby before it’s born. This can happen as early as 10 weeks into the pregnancy. Some women who know they have hemophilia in their family get a test called “pre-implantation diagnosis.” This checks the embryo for hemophilia. This is done in a lab before the embryo is put in the womb. This means you could have a baby without hemophilia.
What problems can hemophilia cause?
Bleeding inside your body, called internal bleeding, is one of the biggest problems. A bleeding muscle can cause swelling in an arm or leg. This swelling can hurt the nerves. This can make you feel hurt or numb. Bleeding inside your body can also hurt your joints. This joint damage can lead to arthritis.
About my Condition
Patient Infusion Log
Where can I get more information?
National Hemophilia Foundation:
World Federation of Hemophilia:
Hemophilia Federation of America:
LA Kelley Communications:
Canadian Hemophilia Society:
Hemophilia and kids:
Hemophilia for parents:
All About Bleeding: