Factor V Deficiency

Factor V (factor five) deficiency is a rare genetic bleeding disorder. Factor V is a protein in the blood that helps the blood clot. It is also called a clotting factor or coagulation factor. When you get an injury, blood cells and clotting factors come together to cause a clot at the site of the injury. The clot helps stop the bleeding. When you have factor V deficiency:

  • Your body does not produce enough factor V, or the factor V does not work the way it should.

  • It is harder for your body to stop bleeding, especially after surgery or a serious injury.

Most cases of factor V deficiency are not severe.

What are the causes?

This condition is almost always caused by a flawed factor V gene that is passed from both parents to a child (inherited).

In rare cases, this condition may be caused by the body making a protein (antibody) that causes factor V deficiency. This antibody may develop:

  • After certain medical procedures.

  • After giving birth.

  • Due to an autoimmune disorder. This is a disease in which the body’s disease-fighting system (immune system) attacks the body’s own healthy tissues.

  • Due to cancer.

What increases the risk?

You may be at higher risk for factor V deficiency if you have:

  • A family history of the condition.

  • A family history of bleeding disorders.

  • An autoimmune disease.

  • Certain cancers.

What are the signs or symptoms?

Most symptoms of factor V deficiency involve heavy or abnormal bleeding. Symptoms include:

  • Abnormal bleeding after an injury, surgery, or childbirth. The bleeding may go on for longer than it should or be more severe than it should be.

  • Bleeding under the skin. This may cause bruising.

  • Bleeding gums.

  • Frequent nosebleeds.

  • Menstrual periods that are unusually long and heavy.

  • Internal bleeding.

  • Bloody or black stools (feces).

  • Bleeding from the umbilical cord at birth, in newborns.

How is this diagnosed?

This condition may be diagnosed based on:

  • Your symptoms.

  • Your personal and family medical history, especially any history of bleeding problems.

  • A physical exam.

  • Blood tests, such as:

    • Factor assays. These tests check the level of certain clotting factors and how well they work.

    • Prothrombin time and partial prothrombin time. This test measures how long it takes your blood to clot.

    • Inhibitor tests. These tests determine whether your body’s immune system affects clotting factors.

How is this treated?

Treatment for this condition may include:

  • A nasal spray that increases the amount of clotting factors in the blood (desmopressin). You may need to use this before you have any medical procedures.

  • Birth control pills to help control heavy menstrual bleeding.

  • Transfusions of plasma (FFP) or platelets. This is a procedure in which you are given plasma or platelets from a donor. Plasma is the liquid part of blood that contains many clotting factors. Platelets are blood cells that help the blood to clot. You may need these types of transfusions if:

    • You have severe bleeding.

    • You are going to have surgery and your health care provider wants to take precautions against severe bleeding.

Follow these instructions at home:

Medicines

  • Take over-the-counter and prescription medicines only as told by your health care provider.

  • Talk with your health care provider before you take any new medicines. Certain medicines may increase your risk for dangerous bleeding. These include:

    • Over-the-counter medicines that contain aspirin.

    • NSAIDs such as ibuprofen.

  • You may be given desmopressin nasal spray to use before any medical or dental procedures.

Preventing falls and injuries

  • Limit your activities as told by your health care provider. Avoid activities that could increase your risk of injury or bruising, such as contact sports. Ask your health care provider what activities are safe for you.

  • Follow instructions from your health care provider about ways that you can help prevent falls and injuries at home. These may include:

    • Removing loose rugs, cords, and other tripping hazards from walkways.

    • Installing grab bars in bathrooms.

    • Using night-lights.

General instructions

A medical alert bracelet.
  • Tell all your health care providers, including your dentist, that you have a bleeding disorder. Make sure to tell providers before you have any procedure done, including dental cleanings.

  • To avoid constipation and rectal bleeding:

    • Drink enough fluid to keep your urine pale yellow.

    • Eat foods that are high in fiber, such as fresh fruits and vegetables, whole grains, and beans.

    • Limit foods that are high in fat and processed sugars, such as fried and sweet foods.

  • To limit bleeding:

    • Brush your teeth using a soft toothbrush.

    • Use an electric razor to shave instead of a blade.

    • Do not use enemas or rectal thermometers.

  • Wear a medical alert bracelet that says that you have a bleeding disorder. This can help you get the treatment you need in case of emergency.

  • Keep all follow-up visits. This is important.

Where to find more information

  • Genetic and Rare Diseases Information Center: rarediseases.info.nih.gov

Contact a health care provider if:

  • You have a lot of bleeding.

  • You have bleeding that does not stop.

  • Your skin bruises very easily.

  • Your menstrual periods are very heavy or last longer than normal.

Get help right away if:

  • You have unusual or severe blood loss.

  • You have a head injury.

  • You have a severe headache.

These symptoms may be an emergency. Get help right away. Call 911.

  • Do not wait to see if the symptoms will go away.

  • Do not drive yourself to the hospital.

Summary

  • Factor V (factor five) deficiency is a rare genetic bleeding disorder.

  • Factor V is a protein in the blood that helps your blood clot (clotting factor or coagulation factor).

  • Most symptoms of factor V deficiency involve heavy or abnormal bleeding.

  • Remember to tell all your health care providers, including your dentist, that you have a bleeding disorder.

This information is not intended to replace advice given to you by your health care provider. Make sure you discuss any questions you have with your health care provider.

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