Von Willebrand disease

Description

Von Willebrand disease is a lifelong bleeding disorder in which blood does not clot normally. People with the disease have low levels of von Willebrand factor, a protein that helps the blood to clot, or the protein doesn't work as it should.

Most people with the disease are born with it, having inherited from one or both parents. However, the warning signs, such as heavy bleeding after a dental procedure, it is possible that they do not appear for years.

Von Willebrand disease can't be cured. But with treatment and self-care, most people with this illness can lead an active life.

Symptoms

Many people with von Willebrand disease do not know it because the signs are mild or absent. The most common sign of the disease is the abnormal bleeding.

There are three main types of the disease. The amount of bleeding can vary from one person to another, depending on the type and severity of the disease.

If you have von Willebrand disease, you may have:

  • Excessive bleeding from an injury or after surgery, or dental work
  • Frequent nosebleeds that do not stop within 10 minutes
  • Heavy or long menstrual bleeding
  • Heavy bleeding during labor and delivery
  • Blood in the urine or stools
  • Easy bruising or lumpy bruises

Menstrual signs and symptoms may include:

  • The blood clots more than 1 inch (2.5 centimeters) in diameter in the flow of menstruation
  • The need to change their menstrual pad or tampon more than an hour
  • The need to use double sanitary protection to the menstrual flow
  • The symptoms of anemia, such as tiredness, fatigue or shortness of breath

When to see a doctor

Contact your doctor if you have bleeding that lasts a long time or is difficult to stop.

Causes

The most common cause of von Willebrand disease is an inherited disease of abnormal gene that controls the von Willebrand factor — a protein that plays a key role in the clotting of the blood.

When you have low levels of this protein or not working as it should, small blood cells called platelets do not adhere properly or normally attach to the walls of the blood vessels when the an injury has occurred. This interferes with the clotting process and can sometimes cause uncontrolled bleeding.

Many people with von Willebrand disease have low levels of factor VIII, another protein that helps in the clotting of the blood.

Factor VIII is involved in other hereditary bleeding disorder called hemophilia. But unlike hemophilia, which affects mainly males, von Willebrand's disease, the disease affects men and women and is generally more mild.

Rarely, von Willebrand's disease may develop later in life that people do not inherit an affected gene from a parent. This is known as acquired von Willebrand syndrome, and is likely to be caused by an underlying medical condition.

Risk factors

The main risk factor for von Willebrand disease is to have a family history of the same. Parents pass the gene for the disease on to their children. In rare cases, the disease skips generations.

The disease is usually an "autosomal dominant inherited disorder", which means that you need a mutated gene from only one parent to the disease. If you have the gene for von Willebrand disease have a 50% chance of passing this gene on to their children.

The most severe form of the disease is autosomal recessive," which means that both parents have to go through a mutated gene for you.

Complications

Rarely, von Willebrand's disease can cause uncontrollable bleeding, which can be deadly. Other complications of von Willebrand disease can include:

  • Anemia. Menstrual bleeding can cause iron-deficiency anemia.
  • The swelling and the pain. This may be the result of abnormal bleeding in the joints or soft tissues.

Prevention

If you plan to have children and having a family history of von Willebrand disease, consider the possibility of genetic counselling. If they are carriers of the gene for von Willebrand's disease, can be transmitted to their offspring, even if you have no symptoms.

Von Willebrand disease

Diagnosis

Mild forms of von Willebrand's disease can be difficult to diagnose because the bleeding is common and, for most people, it does not indicate a disease. However, if your doctor suspects that you have a bleeding disorder, he or she may refer you to a specialist in blood disorders (hematologist).

Evaluation for von Willebrand disease, your doctor will likely ask questions about your medical history and check for bruises or other signs of recent haemorrhage.

Your doctor will also likely recommend the following blood tests:

  • The Von Willebrand factor antigen. This determines the level of von Willebrand factor in the blood by the measurement of a particular protein.
  • The Von Willebrand factor activity. There are a variety of tests to measure how well the von Willebrand factor works in the clotting process.
  • The Factor VIII clotting activity. This shows if you have abnormally low levels and activity of factor VIII.
  • Multimers of the Von Willebrand factor. This evaluates the structure of the von Willebrand factor in their blood, and their protein complexes and how their molecules are broken down. This information helps identify the type of von Willebrand disease you have.

The results of these tests can vary in the same person over time because of factors such as stress, exercise, infection, pregnancy, and medications. So you might need to repeat some tests.

If you have von Willebrand disease, your doctor might suggest that the members of the family undergo tests to determine if this condition runs in your family.

Treatment

Although von Willebrand's disease, the disease has no cure, treatment can help prevent or stop bleeding episodes. Your treatment depends on:

  • The type and severity of your condition
  • How have you responded to the previous therapy
  • Your other medications and conditions

Your doctor may suggest one or more of the following treatments to increase its von Willebrand factor, strengthen the formation of blood clots, or control of menstrual bleeding:

  • Desmopressin.This medication is available as an injection (DDAVP). It is a synthetic hormone that controls the bleeding by stimulating your body to release more of von Willebrand factor stored in the lining of the blood vessels. Many doctors believe that the desmopressin (DDAVP), the first treatment for the management of von Willebrand disease. Can be used before minor surgical procedures to help control the bleeding. You may be subjected to a trial of desmopressin to ensure that it is effective for you.
  • Replacement therapies.These include infusions of concentrated clotting of the blood of the factors that contain von Willebrand factor and factor VIII. Your doctor may recommend ifDDAVPisn, is not an option for you or has been ineffective. Another replacement therapy approved by the Food and Drug Administration for the treatment of adults 18 years of age and older is a genetically modified (recombinant) von Willebrand factor product. Because the recombinant factor has been carried out without the plasma, can reduce the risk of a viral infection or an allergic reaction.
  • The oral contraceptives. In addition to preventing pregnancy, these medications may help control heavy bleeding during menstruation. The hormone estrogen in birth control pills can increase the von Willebrand factor and factor VIII activity.
  • Clot-stabilizing medications. These anti-fibrinolytic drugs, such as aminocaproic acid (Amicar) and tranexamic acid (Cyklokapron, Lysteda) — can help to stop the bleeding by slowing the breakdown of blood clots. Doctors often prescribe these medications before or after a surgical procedure or tooth extraction.
  • Drugs applied to the cuts. A fibrin sealant (Tisseel) placed in a cut helps to reduce the bleeding. This applies as the glue with a syringe. There are also over-the-counter products to stop bleeding from the nose.

Desmopressin. This medication is available as an injection (DDAVP). It is a synthetic hormone that controls the bleeding by stimulating your body to release more of von Willebrand factor stored in the lining of the blood vessels.

Many doctors believe that the desmopressin (DDAVP), the first treatment for the management of von Willebrand disease. Can be used before minor surgical procedures to help control the bleeding. You may be subjected to a trial of desmopressin to ensure that it is effective for you.

Replacement therapies. These include infusions of concentrated clotting of the blood of the factors that contain von Willebrand factor and factor VIII. Your doctor might also recommend if DDAVP is not an option for you or has been ineffective.

Another replacement therapy approved by the Food and Drug Administration for the treatment of adults 18 years of age and older is a genetically modified (recombinant) von Willebrand factor product. Because the recombinant factor has been carried out without the plasma, can reduce the risk of a viral infection or an allergic reaction.

If your condition is mild, your doctor may recommend a treatment only when you are having surgery or dental work or when you've had a trauma, such as a car accident.

Self-care

These self-care tips can help you manage your condition:

  • Switch of pain relievers. To help prevent episodes of bleeding, talk with your doctor before you take blood-thinning medications (such as aspirin), ibuprofen (Advil, Motrin IB, others) or naproxen sodium (Aleve). Your physician may instead recommend the fever and pain relievers such as acetaminophen (Tylenol, others).
  • Tell your doctor and dentist. Let your doctors or dentist that you have von Willebrand disease before surgery, starting a new medication or giving birth. It is also mentioned if someone in your family has a history of excessive bleeding.
  • Consider wearing a medical ID bracelet. Make sure that the notes that you have von Willebrand disease, which will be helpful for medical personnel if you are in an accident or taken to an emergency room. Also carry a medical alert card in your wallet.
  • Be active and safe. The exercise is part of reach or maintain a healthy weight. Avoid activities that may cause bruising, such as football, wrestling and hockey.

Preparing for your appointment

It is likely that you see your family doctor if you think you have a bleeding problem. You may be referred to a doctor who specializes in the diagnosis and treatment of bleeding disorders (hematologist).

Here's some information to help you prepare for your appointment.

What you can do

Ask about pre-appointment restrictions when making the appointment. You might need to follow the restrictions in the diet, or fasting for 8 to 10 hours if the blood tests are scheduled.

Make a list of:

  • The signs or symptoms that involve bleeding, including the frequency, severity, and for how long. Include unusual bruising. Your doctor will want to know if you have a history of bleeding or easy bruising since childhood. If menstruation, are your periods are extended and heavy?
  • Key personal information, including major stresses or recent changes, both positive and negative.
  • Key medical information, including other medical problems and close family members that bleed easily. Also the list of medicines, vitamins, or other supplements that you take, including over-dose.
  • Questions to ask your doctor.

Have a friend or family member, if possible, to help you remember the information that will be given.

For the von Willebrand disease, questions to ask your doctor include:

  • What is the most likely reason for my symptoms?
  • What treatment do you recommend, and how will you control if you are working on?
  • What are the possible side effects of the medications you are prescribing?
  • How this condition increases my risk of other health problems you have?
  • What I have to do before having surgery or dental procedures?
  • How this condition increases my risk of health problems during pregnancy and childbirth? Are the treatment options available to reduce that risk?
  • I need to avoid certain physical activities or types of exercise?
  • I have other health conditions. How can I best manage them together?

What to expect from your doctor

Your doctor may ask:

  • Has had bleeding from a small wound that lasted more than 15 minutes, reappeared during the week following the injury?
  • Have you had a nosebleed that lasted more than 10 minutes or you need medical care?
  • Has had unexpected bruising? Have you ever felt a lump under the bruise?
  • If the menstrual period, how many days of your periods last? How often do you change your tampon or sanitary napkin during a period of time? Have you seen blood clots in the flow of menstruation?
  • He has had blood in the feces that is not explained by a medical problem, such as a stomach ulcer or colon polyp?
  • Have you ever needed medical attention for bleeding during or after surgery, dental procedures, childbirth or injury?
  • Has had anemia or need a blood transfusion?

What you can do in the meantime

While you wait for your appointment, avoid painkillers that can increase your risk of bleeding, such as aspirin, ibuprofen (Advil, Motrin IB, others) and naproxen sodium (Aleve). If you need a relief for aches and pains, take acetaminophen (Tylenol, others) in its place.

Stay away from contact sports associated with a high risk of bruising or injuries, such as football and hockey. If you have medical or dental procedures, regular, tell your doctor or dentist about their history of profuse bleeding from minor injuries.

If you are having a procedure that is not urgent, rescheduled until after you have been evaluated for a bleeding disorder.

Symptoms and treatment of Von Willebrand disease