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Coagulation & Hemostatic Disorders

HEMOPHILIA

What is hemophilia?

Hemophilia is a condition that keeps blood from clotting normally. If blood doesn’t clot normally, people can bleed very easily or much more than normal. The bleeding can sometimes be life-threatening.

People with hemophilia are missing a protein in the blood called a “clotting factor.” Without this, the blood can’t clot normally. There are 2 main types of hemophilia (called “A” and “B”), depending on which clotting factor is the problem:

  • In hemophilia A, factor 8 (also written as “factor VIII”) is missing or very low.
  • In hemophilia B, factor 9 (also written as “factor IX”) is missing or very low.

Hemophilia is a life-long condition that a person is born with. It is caused by an abnormal gene. Sometimes, parents pass this abnormal gene to their child. Other times, a child can get hemophilia from a new gene abnormality that happens before they are born. In some of these cases, parents might not know they have the abnormal gene, because they don’t have any symptoms. Boys and men are most likely to have hemophilia. It is very rare in girls or women.

Hemophilia can be mild or severe. It can be managed with different treatments.

What are the symptoms of hemophilia?

Symptoms depend on how mild or severe a person’s hemophilia is. Some children start having symptoms from birth. For example, a baby boy might bleed much more than normal if he has a procedure to remove the skin that covers the tip of the penis (called “circumcision”). Other children, especially those with mild hemophilia, start having symptoms later on.

People with mild hemophilia usually bleed more than normal after an injury, procedure, or surgery.

People with severe hemophilia also bleed more than normal after an injury, procedure, or surgery. But they can also have bleeding that happens for no reason at all. This type of bleeding usually happens in a joint. The joints most often involved are the ankles, knees, and elbows. Blood in a joint can cause pain, swelling, stiffness, and trouble moving the joint. Over time, repeated bleeding in a joint can lead to long-term joint pain and damage.

Symptoms can also happen from bleeding in other parts of the body. These can include:

  • Blood in a bowel movement
  • Blood in the urine
  • Belly pain (from bleeding into the belly wall)
  • Bruises or bleeding in a muscle
  • Bleeding in the brain or spinal cord

Is there a test for hemophilia?

Yes. Your doctor or nurse can check for hemophilia by doing different blood tests.

What if I am pregnant?

Pregnant women who have the hemophilia gene have the chance of passing the gene along to their baby. Genetic testing can show if you have the gene. If you do, doctors can do a few things to help prevent problems:

  • Do an ultrasound to find out the sex of your baby – This is because only a male baby would be at risk of having severe hemophilia. If the baby is male, doctors will avoid certain procedures during labor and delivery that could cause bleeding. In most cases, the baby is tested after birth to find out if he does have hemophilia.
  • Prepare for a safe birth – Some women who carry the gene are at risk of abnormal bleeding. Your doctor will test your clotting factor levels. This can help them avoid or prepare for procedures that might cause bleeding. Depending on your levels, you might also need treatment for bleeding after the baby is born.

How is hemophilia treated?

Treatment depends on how severe a person’s hemophilia is. It also depends on whether the person is going to have a procedure or surgery.

The main treatment for hemophilia is called “factor replacement therapy.” This involves getting the clotting factor that the body is missing. There are different types of replacement clotting factors. Some are made from human blood, and others are made using cells grown in a lab. Replacement therapy goes into a vein. People can get this treatment in the hospital, or they can give it to themselves (or have someone else give it to them) at home.

Replacement therapy is used in 2 ways:

  • To treat a bleed when it happens
  • On a regular basis, to prevent bleeding

Deciding which treatment is best can be complicated. Hemophilia treatment centers can help people with hemophilia, and their doctors, decide which treatment is best. These centers also help people plan ahead for things like surgery.

Some people have something called an “inhibitor” in their blood. This can make factor replacement therapy not work as well. This is more common in hemophilia A than in hemophilia B. If tests show that you have an inhibitor, you might need other treatments. Your doctor will talk to you about the options.

What else should I know about replacement therapy?

In the past, factor replacement therapy made from human blood sometimes had viruses in it. This could include HIV (the virus that causes AIDS) and the virus that causes hepatitis C, which could then infect the person being treated for hemophilia. Today, replacement therapy is made in a safer way and does not contain HIV or hepatitis C virus.

What else can people with hemophilia do to avoid bleeding problems?

To avoid bleeding problems, people with hemophilia should:

  • See their doctor for regular visits
  • Let every doctor who takes care of them know they have hemophilia, and which type (“A” or “B”)
  • Follow their doctor’s instructions about treatment and which activities or sports to avoid (if any)
  • Learn the signs and symptoms of bleeding, and how to treat it
  • Not take aspirin or medicines called NSAIDs without the input of a doctor – Common NSAIDs include ibuprofen (sample brand names: Advil, Motrin) and naproxen (sample brand names: Aleve, Naprosyn).
  • Plan ahead for any procedures or surgery that could cause bleeding
  • Take good care of their teeth and see their dentist for regular visits
  • Take their medicines with them when travelling

If your child has hemophilia, you should let caregivers, daycare, or the school know. Teach them which symptoms to watch for, and how to treat a bleed.

Choosing an oral medicine to prevent or treat blood clots

What are these medicines?

These are prescription medicines that make your blood less likely to clot. “Oral” means medicine you take as a pill. There are other medicines that are given as a shot or through a thin tube that goes into a vein, called an “IV.”

Medicines used to prevent or treat blood clots are also known as “anticoagulants” or “blood thinners.” But they do not actually thin the blood.

There are several oral medicines used to prevent or treat blood clots. They include:

  • Apixaban (brand name: Eliquis)
  • Dabigatran (brand name: Pradaxa)
  • Edoxaban (brand names: Savaysa, Lixiana)
  • Rivaroxaban (brand name: Xarelto)
  • Warfarin (brand names: Coumadin, Jantoven)

Apixaban, dabigatran, edoxaban, and rivaroxaban are also called “direct oral anticoagulants.” Each of these medicines blocks the function of a specific clotting factor. (Clotting factors are proteins in the blood that help it to clot.) Warfarin is also called a “vitamin K antagonist.” It blocks the body’s use of vitamin K in making some of the clotting factors.

Why would I need one of these medicines?

The main reasons doctors prescribe these medicines are to prevent blood clots or to treat blood clots that have already formed.

Doctors prescribe these medicines to prevent blood clots in:

  • People who have a heart rhythm problem called “atrial fibrillation” – In people with this condition, the medicine helps lower the chances of forming a blood clot in the heart. A blood clot in the heart can cause a stroke.
  • People who have had hip or knee replacement surgery – The medicine helps prevent a blood clot in the legs (called a “deep vein thrombosis” or “DVT”) or the lungs (called a “pulmonary embolism” or “PE”).
  • People who have a mechanical heart valve – This is a device used to replace a damaged heart valve.
  • Other people who are at high risk of having a blood clot.

Nothing can completely prevent all blood clots, but these medicines can make clots less likely to form.

Doctors also prescribe these medicines for people who have already had a clot, to prevent more problems. This includes:

  • People who have had a stroke related to atrial fibrillation
  • People who have had a DVT or PE
  • People who have had a blood clot in another part of the body

How do these medicines help?

These medicines do not dissolve clots, but they keep them from growing bigger and moving to another part of the body. If a clot moves to a lung or to the brain, it can cause serious problems. These medicines lower the chances that the blood clot might get bigger or move to another part of the body, and they lower the chances of new clots forming.

What are the differences among these medicines?

Each medicine is different in terms of the dose, how often you take it, the cost, and how your diet or other medicines might affect it.

The table answers some common questions about the differences. Some of the main differences include:

  • How often you need to get blood tests– If you take apixaban, dabigatran, edoxaban, or rivaroxaban, you will not need regular blood tests.

If you take warfarin, you will need to get your blood tested on a regular schedule to check how it is clotting. This is important in order to make sure you are always getting the dose you need.

  • How often you take the pills – This depends on which medicine you take and why your doctor prescribed it. In general, warfarin, edoxaban, and rivaroxaban are taken once a day. Apixaban and dabigatran are usually taken twice a day. If you are taking the pills to treat a new blood clot, the first week or more of treatment might be different. Your doctor or pharmacist will tell you how many times you need to take your medicine each day.
  • Whether you need to change your diet – This is especially important if you take warfarin, as you will need to be careful about how much vitamin K you eat. If the amount of vitamin K in your diet changes from day to day, this can make warfarin not work as well.
  • The risk of bleeding – Most kinds of bleeding – including bleeding in the brain – are less likely with apixaban, dabigatran, edoxaban, and rivaroxaban than they are with warfarin. But all of these medicines raise your risk of bleeding more than normal. This can be serious internal bleeding, like bleeding in the brain or the intestines, or external bleeding, like after you get a cut.
  • What happens if I miss a dose  If you miss a dose of apixaban, dabigatran, edoxaban, or rivaroxaban, you might not be as well protected from blood clots because these medicines leave your system quickly. If you miss a dose of warfarin, it will still work correctly. Ask your doctor or pharmacist what to do if you miss a dose of your medicine.

Which medicine is right for me?

Your doctor can talk to you about your condition, options, and preferences. For some people, warfarin is the best choice. This includes people with a mechanical heart valve or people with a condition called “antiphospholipid syndrome.”

For others, one of the other medicines might be a better choice.

In women who are pregnant, doctors do not recommend any of these medicines. Pregnant women who need to take a medicine to prevent or treat blood clots have to take a different medicine that comes in a shot.

Von - willebrand Disease

What is von Willebrand disease?

Von Willebrand disease is a bleeding disorder. People with the disorder sometimes bruise easily or bleed too much when they get cut.

Von Willebrand disease is caused by an abnormal gene and runs in families. In some cases, a family might not know about it if the disease is mild. Rarely, people can get a kind of von Willebrand “syndrome” because of another condition, rather than a gene.

What are the symptoms of von Willebrand disease?

The symptoms of von Willebrand disease are often mild. For some people, the symptoms are so mild that they never have problems.

If you have von Willebrand disease, you might:

  • Get nosebleeds a lot or have nosebleeds that last a long time
  • Notice that your gums sometimes bleed for no reason
  • Bruise easily and form lumps under your bruises
  • Bleed more than normal when having medical procedures or surgery
  • Bleed when you take aspirin or other medicines that prevent blood clots

In women, symptoms can also include:

  • Very heavy or long-lasting periods – For instance, your periods might last longer than 7 to 10 days and have large blood clots.
  • Very heavy bleeding after giving birth.

Is there a test for von Willebrand disease?

Yes. If your doctor or nurse suspects you have von Willebrand disease, he or she can check for it with a blood test. He or she will also want to know as much as possible about the way you have bled in the past. Plus, your doctor or nurse will want to know if any of your family members have the disorder or have had problems with bleeding or bruising.

Is there anything I can do on my own to reduce bleeding?

Yes. You can follow these tips:

  • Do not take medicines that have aspirin, or medicines called “NSAIDs” without talking to your doctor first. Examples of NSAIDs include ibuprofen (sample brand names: Advil, Motrin) and naproxen (sample brand name: Aleve). These medicines can make bleeding worse.
  • Be extra safe about preventing injuries if your doctor tells you to. For example, you might need to avoid contact sports, like hockey or football.
  • Plan ahead for surgery or childbirth. If you are going to have surgery or any procedure that might make you bleed, talk to your doctor or nurse about your condition first. Do the same if you are going to have a baby. That way your doctor or nurse can be ready with the right medicines if you start to bleed too much.
  • Talk to your doctor if you are concerned about heavy periods. There are different ways to reduce menstrual bleeding.

How is von Willebrand disease treated?

There are many medicines used to treat von Willebrand disease. These medicines come in shots, pills, gels, and nasal sprays. You and your doctor or nurse might have to try a few different treatment approaches before you find the one that works best for you.

If you have lost a lot of blood, you might need to take iron supplements.

What if I want to have a baby?

If you want to have a baby, you will need to be watched closely during your pregnancy. This way, serious bleeding can be prevented or treated. Also, you will need to talk with your doctor about whether you can get an epidural or spinal block during labor. You might need other treatments to reduce your risk of bleeding.

Most women do not need treatment while they are pregnant, because von Willebrand disease is usually less severe during pregnancy. But after the baby is born, some women can start to have serious bleeding. For 1 to 3 weeks after giving birth, women often need to take extra medicines to prevent bleeding.

DEEP VENOUS THROMBOSIS

What is deep vein thrombosis?

Deep vein thrombosis is the medical term for blood clots in the deep veins of the leg. Deep vein thrombosis, or “DVT” for short, can be dangerous.

If a blood clot forms inside a blood vessel, it can clog the vessel and keep blood from getting where it needs to go. When that happens to 1 of the veins deep within the leg, blood can back up and cause swelling and pain.

Another problem with blood clots in veins is that they can travel to other parts of the body and clog blood vessels there. Blood clots that form in the legs, for example, can end up blocking blood vessels in the lungs. This can make it hard to breathe and sometimes, when they are large, can lead to death. When blood clots travel to the lungs doctors call it “pulmonary embolism” or “PE.”

What are the symptoms of DVT?

DVT can cause the following symptoms in the involved leg:

  • Swelling
  • Pain
  • Warmth and redness

Sometimes clots form in the veins that are closer to the surface of the skin, called the “superficial veins.” Those blood clots cause a different set of symptoms. Blood clots in the veins near the surface of the skin are more painful and cause redness or infection. These clots sometimes also cause the veins to harden and bulge into ridges that look like cords. This is most common with the veins below the knee.

If you think you have a blood clot in your leg, call your doctor or nurse right away. Blood clots in the veins near the surface of the skin are less dangerous. But blood clots in the deep veins of the leg are more serious. Your doctor or nurse can run tests to find out if you do have a clot that needs to be treated.

What are the symptoms of blood clots in the lungs?

Blood clots in the lungs can cause:

  • Panting, shortness of breath, or trouble breathing
  • Sharp, knife-like chest pain when you breathe in or strain
  • Coughing or coughing up blood
  • A rapid heartbeat

If you get any of these symptoms, especially if they happen over a short period of time (hours or days) or get worse quickly,  call for an ambulance (in the US and Canada, dial 9-1-1). At the hospital, doctors can run tests to find out if you do have a clot. Blood clots in the lungs can lead to death. That’s why it’s important to act fast and find out if there is a clot.

How is DVT treated?

DVT is treated with medicines that keep the clot from getting bigger and travelling to the lung. These medicines are called “anticoagulants” or “blood thinners,” although they do not actually thin the blood. Some come in shots and others come in pills. DVT is usually treated first in the hospital.

If you have had a clot, your doctor will prescribe 1 of these medicines to lower your risk of getting more clots in the future. You will need to take the medicine for at least 3 months (and sometimes longer). Some people are first put on a medicine that comes as a shot, called heparin. This might be for a few days, or longer if for some reason you can’t take pills.

The medicines do not dissolve existing blood clots, but they do keep them from getting bigger. They also help keep new blood clots from forming. Taking the medicine for a few months is important because it gives your body time to dissolve the old clot. It’s also important because people who have a clot are at risk of developing another clot, especially in the first few months.

There are different oral medicines (pills) used to prevent and treat blood clots. They include apixaban (brand name: Eliquis), dabigatran (brand name: Pradaxa), edoxaban (brand names: Savaysa, Lixiana), rivaroxaban (brand name: Xarelto), and warfarin (brand name: Coumadin). Each medicine is different in terms of the dose, how often you take it, the cost, and how your diet or other medicines might affect it. Your doctor can talk to you about your options and preferences.

If your doctor prescribes one of these medicines:

  • Take it exactly as your doctor tells you to – If you forget or miss a dose, call your doctor to find out what to do. When you start taking the medicine, you will need to have your blood tested. If you take warfarin, you will need regular blood tests to check how your blood is clotting. This is important in order to make sure you get the correct dose of warfarin for you.
  • Follow your doctor’s instructions about diet and medicines – Depending on which medicine you take, you might need to pay special attention to what you eat. Also, certain other medicines can affect the way these medicines work.
  • Watch for signs of bleeding – Abnormal bleeding is a risk with any of the medicines used to prevent and treat blood clots. That’s because while these medicines help prevent dangerous blood clots, they also make it harder for your body to control bleeding after an injury. So it’s important to try to avoid getting injured, and to tell your doctor right away if you do have signs of bleeding.

People who cannot take medicines to prevent and treat blood clots, or who do not get enough benefit from the medicines, can get a different treatment. This is called an “inferior vena cava filter” (also called an IVC filter). The inferior vena cava is the large vein that carries blood from your legs and the lower half of your body back up to your heart. IVC filters go inside the inferior vena cava. They filter and trap any large clots that form below the location of the filter. Your doctor might suggest one of these filters for you if:

  • You cannot safely take a medicine for blood clots
  • You form clots even while taking a medicine for blood clots
  • You have a dangerous bleeding problem while taking a medicine for blood clots
  • You are so sick that if a blood clot that travels from your legs to your lungs it could kill you

In some cases, a person has a clot that is severe enough to cause gangrene (cut off the blood supply to your leg). If this happens, doctors can give medicine to dissolve the clot. This is sometimes called “clot-busting” medicine, and is given through a catheter (a small tube inserted into the vein). In some cases, doctors will do surgery to remove the clot.

Can I do anything on my own to prevent blood clots?

Yes. People sometimes form clots because they have been sitting still for too long. People who travel on long airplane flights, for example, are at increased risk of blood clots. Here are some things you can do to help prevent a clot during a long flight:

  • Stand up and walk around every 1 to 2 hours
  • Do not smoke just before your trip
  • Wear loose, comfortable clothes
  • Shift your position while seated, and move your legs and feet often
  • Wear knee-high compression stockings
  • Avoid alcohol and medicines that make you sleepy, because they can impair your ability to move around

PULMONARY EMBOLISM

What is a pulmonary embolism?

Pulmonary embolism (or “PE”) is a blockage in 1 or more of the blood vessels that supply blood to the lungs. Most often these blockages are caused by blood clots that form elsewhere and then travel to the lungs. In rare cases, blockages can also be caused by air bubbles, tiny globs of fat, or pieces of tumor that travel to the lungs.

Why are blood clots dangerous?

If a blood clot forms or gets stuck inside a blood vessel, it can clog the vessel and keep blood from getting where it needs to go. When that happens in the lungs, the lungs can get damaged. Having blocked arteries in the lung can also make it hard to breathe and can even lead to death.

Most blood clots in the lungs actually form in the legs or pelvic area (where the legs connect to the body) and then travel to the lungs. Anyone who has had a blood clot in the lungs or who is at risk of a clot should know the symptoms of clots in these areas.

What are the symptoms of blood clots in the lungs?

Common symptoms include:

  • Panting, shortness of breath, or trouble breathing
  • Sharp, knife-like chest pain when you breathe in or strain
  • Coughing or coughing up blood
  • A rapid heartbeat

If you get any of these symptoms, especially if they happen over a short period of time (hours or days) or get worse quickly,  call for an ambulance (in the US and Canada, dial 9-1-1). At the hospital, doctors can run tests to find out if you do have a clot. Blood clots in the lungs can lead to death. That’s why it’s important to act fast and find out if there is a clot.

What are the symptoms of blood clots in the legs?

Blood clots in the legs cause different symptoms depending on whether the clots form in veins deep in the leg or in the veins near the surface of the skin.

Blood clots in the  deep veins of the legs (also called “DVT”) are the most dangerous. Clots in the deep veins can cause:

  • Swelling
  • Pain
  • Warmth and redness in the involved leg

Blood clots in the veins near the surface of the skin (called “superficial veins”) are more painful, and can cause redness or infection. These clots sometimes also cause the veins to harden and bulge into ridges that look like cords. This is most common with the veins below the knee.

If you think you have a blood clot in your leg, call your doctor or nurse right away. Blood clots in the veins near the surface of the skin are less dangerous. But blood clots in the deep veins of the leg are more serious and are more likely to travel to the lungs. Your doctor or nurse can do tests to find out if you do have a clot and where it is.

Is there a test to find blood clots in the lungs?

Yes. There are several tests doctors can use to find out if a person has a blood clot in a lung. The most common tests include:

  • D-dimer blood test – D-dimer is a substance in the blood. The amount of D-dimer often goes up in people with a blood clot in a lung. This blood test is often done together with other tests.
  • CT pulmonary angiogram (also called a CT-PA) – A CT pulmonary angiogram is a special kind of X-ray that involves the use of computers. During this test, a dye is injected into 1 of your veins. The dye shows up on X-rays and can show if any blood vessels are blocked.
  • A ventilation/perfusion lung scan (also called a V/Q scan) – For this test, you breathe in a small amount of a radioactive substance. You also have a radioactive dye injected into 1 of your veins. Then doctors look at how the different substances look on the scan. The scan can show if 1 of the arteries in the lung is blocked.
  • Pulmonary angiography – For this test, you have a small tube called a “catheter” inserted into 1 of the large veins in your body, usually 1 in your leg. Then doctors gently push this tube up into the chest to where the major blood vessels of the lung are found. When the tube is in place, the doctors inject a dye that shows up on an X-ray.

How are blood clots in the lungs treated?

Blood clots in the lungs are treated with medicines that keep clots from getting bigger. These medicines are called “anticoagulants” or “blood thinners,” although they do not actually thin the blood. Some come in shots and others come in pills. Most people being treated for a blood clot in the lung are treated first in the hospital.

If you have had a clot, your doctor will prescribe 1 of these medicines to lower your risk of getting more clots in the future. You will need to take the medicine for at least 3 months (and sometimes longer). Some people are first put on a medicine that comes as a shot, called heparin. This might be for a few days, or longer if for some reason you can’t take pills.

The medicines do not dissolve existing blood clots, but they do keep them from getting bigger. They also help keep new blood clots from forming. Taking the medicine for a few months is important because it gives your body time to dissolve the old clot. It’s also important because people who have a clot are at risk of developing another clot, especially in the first few months.

There are different oral medicines (pills) used to prevent and treat blood clots. They include apixaban (brand name: Eliquis), dabigatran (brand name: Pradaxa), edoxaban (brand names: Savaysa, Lixiana), rivaroxaban (brand name: Xarelto), and warfarin (brand name: Coumadin). Each medicine is different in terms of the dose, how often you take it, the cost, and how your diet or other medicines might affect i. Your doctor can talk to you about your options and preferences.

If your doctor prescribes one of these medicines:

  • Take it exactly as your doctor tells you to – If you forget or miss a dose, call your doctor to find out what to do. When you start taking the medicine, you will need to have your blood tested. If you take warfarin, you will need regular blood tests to check how your blood is clotting. This is important in order to make sure you get the correct dose of warfarin for you.
  • Follow your doctor’s instructions about diet and medicines – Depending on which medicine you take, you might need to pay special attention to what you eat. Also, certain other medicines can affect the way these medicines work.
  • Watch for signs of bleeding – Abnormal bleeding is a risk with any of the medicines used to prevent and treat blood clots. That’s because while these medicines help prevent dangerous blood clots, they also make it harder for your body to control bleeding after an injury. So it’s important to try to avoid getting injured, and to tell your doctor right away if you do have signs of bleeding.

People who cannot take medicines to prevent and treat clots, or who do not get enough benefit from the medicines, can get a different treatment. This is called an “inferior vena cava filter” (also called an IVC filter). The inferior vena cava is the large vein that carries blood from your legs and the lower half of your body back up to your heart. IVC filters go inside the inferior vena cava. They filter and trap any large clots that form below the location of the filter. Your doctor might suggest 1 of these filters for you if:

  • You cannot safely take a medicine for blood clots
  • You form clots even while taking a medicine for blood clots
  • You have a dangerous bleeding problem while taking a medicine for blood clots
  • You are so sick that another pulmonary embolism could kill you

In some cases, a person has a clot that is severe enough to cause low blood pressure and even shock. (Shock is when blood pressure gets too low, and not enough blood can get to the body’s organs and tissues.) If this happens, doctors can give medicine to dissolve the clot. This is sometimes called “clot-busting” medicine, and is given through a catheter (a small tube inserted into the vein). In some cases, doctors will do surgery to remove the clot.

Can I do anything on my own to prevent blood clots?

Yes. People sometimes form clots because they have been sitting still for too long. People who travel on long airplane flights, for example, are at increased risk of blood clots. Here are some things you can do to help prevent a clot during a long flight:

  • Stand up and walk around every 1 to 2 hours
  • Do not smoke just before your trip
  • Wear loose, comfortable clothes
  • Shift your position while seated, and move your legs and feet often
  • Wear knee-high compression stockings
  • Avoid alcohol and medicines that make you sleepy, because they can impair your ability to move around

Factor V Leiden

What is factor V Leiden?

Factor V Leiden (FVL) is a condition that makes blood more likely to form clots in the legs, lungs, and other parts of the body in some people.

Factor V Leiden is a life-long condition that people are born with. It is caused by an abnormal gene. Parents pass the abnormal gene to their child. Often, people might not know they have the abnormal gene because they don’t have any symptoms.

The “V” is the Roman numeral for “five.” Leiden is a city in Holland where the abnormal gene was discovered.

What are the symptoms of factor V Leiden?

Most people with factor V Leiden never have any symptoms. But some people with factor V Leiden have a higher risk of getting blood clots, especially in the leg or the lungs. When clots do occur, they can cause:

  • Warmth, redness, pain, and swelling in the leg – These are symptoms of a condition called “deep vein thrombosis” or “DVT.” This is a blood clot in a vein deep inside the leg.
  • Breathing problems, sharp chest pain, coughing, and fast heartbeat – These are symptoms of a condition called “pulmonary embolism.” This is a blood clot in the lungs. It usually comes from a blood clot in the leg that breaks off and travels to the lungs. This can be very dangerous and even cause death if the clot is large.

Some doctors believe that factor V Leiden can also increase the chance of other problems, including stroke, heart attack, or miscarriage (when a pregnancy ends on its own before the baby can live outside the womb). But other doctors do not. If there is an increased risk of these conditions, it is a small increase.

Should I see a doctor or nurse?

See your doctor or nurse right away if you think you might have a blood clot in your leg. If you have symptoms of a stroke or a blood clot in the lungs, call for an ambulance (in the US and Canada, dial 9-1-1). These conditions are very serious and can be life-threatening. Signs of a stroke include drooping of 1 side of the face, arm weakness, and trouble speaking normally.

If one of your relatives has been told that they have factor V Leiden, you can talk to your doctor or nurse to find out if you should be tested.

Is there a test for factor V Leiden?

Yes. If your doctor or nurse thinks you might have factor V Leiden, they can order a blood test to look for the abnormal gene.

If you do have factor V Leiden, you can tell your relatives (parents, siblings, or children) so they can decide whether to be tested. Their doctor or nurse can help them make this decision.

How is factor V Leiden treated?

If factor V Leiden causes a blood clot, the main treatment is medicines that keep the clot from getting bigger. Some of these medicines come in shots and others come in pills. Doctors sometimes use a stronger medicine that gets rid of clots or do surgery to remove a blood clot in the lungs if it is very severe.

People who have had a blood clot usually take a medicine in pill form to prevent clots for at least 3 months after the clot is found, and often for longer. This medicine helps keep new blood clots from forming. It is important because people who have one clot often have another later on.

Is there anything I can do to prevent blood clots?

Yes. The best thing you can do is move your legs often. This keeps the blood moving. Not moving for a long period of time can increase your risk of clots. Examples are having surgery, being stuck in bed, or taking a long airplane or car trip.

To help prevent a clot on a long plane or car ride, you can:

  • Stand up and walk around every hour or 2 (or if driving, take breaks often)
  • Wear loose, comfortable clothes
  • Change your sitting position and move your legs and feet often
  • Wear knee-high compression stockings, especially if you get leg swelling

Women with factor V Leiden should ask a doctor or nurse if it is safe to take birth control pills or other hormone treatments. The hormones raise the risk of blood clots. This can be dangerous for women with factor V Leiden.

If you are having surgery, let your doctor know you have factor V Leiden. They will decide if you need medicine or other precautions to prevent blood clots.

What if I want to get pregnant?

If you have factor V Leiden and want to get pregnant, talk to your doctor or nurse. You will be monitored for blood clots during your pregnancy and after the birth. You might also get medicine to help lower the risk of blood clots.