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Glossary

A - E

acquired VWD  A non-hereditary type of VWD in which a person suddenly develops antibodies, or inhibitors, to the VWF normally produced in the blood.

Amicar  An anti-fibrinolytic drug (aminocaproic acid) that helps to hold a clot in place once it has formed by stopping the activity of an enzyme, called plasmin, which dissolves blood clots.

anesthesiologist  A physician who specializes in controlling pain and consciousness during surgery.

antibody  A substance produced in the blood by the body's immune system to defend against other harmful substances.

anti-diuretic  A substance that makes the body retain water.

anti-fibrinolytics  Drugs (Cyklokapron and Amicar) that help to hold a clot in place once it has formed by stopping the activity of an enzyme, called plasmin, which dissolves blood clots.

bleeding disorder  A disease in which the body is unable to form blood clots as quickly or as effectively as normal. The family of bleeding disorders includes von Willebrand Disease, hemophilia A, hemophilia B, platelet function disorders and a variety of rare factor deficiencies. The disorder may be hereditary or acquired.

bleeding time  The time required for a minor cut to stop bleeding. As a test, it is unreliable in diagnosing VWD.

blood clotting  The process of forming a permanent clot to repair a damaged blood vessel. It includes four steps: vasoconstriction, platelet aggregation, platelet adhesion, and the formation of a fibrin plug.

blood clotting proteins  Substances that circulate in the bloodstream, necessary in blood clotting. They include Von Willebrand Factor, and factors I, II, III, V, VII, VIII, IX, X, XI, XII and XIII.

blood type The particular kind of blood each person has. The types are A, B, AB and O.

chromosome  A long chain of chemicals known as DNA, which is arranged into about 25,000 units called genes. Genes determine such things as the colour of a person’s eyes.

comprehensive care  All of the medical services needed by a person with VWD and his/her family for the treatment of VWD and related conditions. This care is provided at a Hemophilia/Bleeding Disorder Treatment Centre.

cryoprecipitate  A blood component made from plasma, containing VWF and Factor VIII, commonly used to treat VWD in the past. However, because there is no method to kill viruses in cryoprecipitate, it is no longer recommended.

Cyklokapron An anti-fibrinolytic drug (tranexamic acid) that helps to hold a clot in place once it has formed by stopping the activity of an enzyme, called plasmin, which dissolves blood clots.

desmopressin  A synthetic drug which is a copy of a natural hormone. It acts by releasing VWF stored in the lining of the blood vessels. Desmopressin is not made from blood. It can be called DDAVP, Octostim and Octostim Spray, Stimate and Stimate Nasal Spray.

dilatation & curettage (D&C)  An operation is to scrape and clean the lining of the uterus.

dysmenorrhea  Pain during the menstrual period.

endometriosis  A condition in which endometrial tissue forms outside the uterus, for example, around the abdomen. When a woman menstruates, endometrial tissue, wherever it is in the body, bleeds.

epidural  A type of local anesthesia in which a needle is placed into the spine to freeze the lower part of the body.

episiotomy  A procedure sometimes done during childbirth in which the skin is cut near the vagina to avoid tearing.

F - M

Factor VIII  A protein in the blood that is essential for clotting. Factor VIII levels are low in people with VWD and hemophilia A.

fibrin clot  The clot which forms in the last stage of the coagulation process.

gene  Tiny structures of DNA which determine such things as the colour of a person’s eyes. VWD is caused by an abnormal gene on chromosome 12.

gynecologist  A physician who specializes in the woman's reproductive system.

hematologist  A physician specializing in diseases of the blood.

hemoglobin  A substance in the red cells of blood, responsible for carrying oxygen.

hemophilia  A term used to describe bleeding disorders caused by low levels of factor VIII or IX (hemophilia A and B). The term can also be used more broadly to describe the family of bleeding disorders, including VWD.

hemorrhage  The escape of blood from blood vessels, either on the surface of the body or internally.

home care  The care of the person with VWD at home, rather than in hospital. This includes the administration of medication by the person with VWD or a family member.

hormone  A secretion in the blood that stimulates organs into action.

hormone therapy  The administration of oral contraceptives or other hormones (e.g. progesterone) to raise VWF levels or reduce menstrual bleeding.

hysterectomy  An operation to remove the uterus, and in some cases, the ovaries.

Humate P  A blood product made from plasma, used in the treatment of VWD, containing concentrated von Willebrand factor and factor VIII.

inhibitors  Antibodies produced to eliminate VWF or other clotting factor proteins, seen as foreign by the body's immune system.

iron deficiency anemia
   A condition caused by low hemoglobin levels because of blood loss, leading to fatigue and lack of energy.

laparoscopic surgery  An operation in which a tiny camera is used to examine the abdominal area. In VWD, this operation is used to remove endometrial tissue that has formed outside the uterus.

leukemia  A form of cancer characterized by an excess of white blood cells.

menorrhagia  Bleeding during the menstrual cycle which is heavier than normal or lasts longer than normal.

metorrhagia  Irregular bleeding throughout the menstrual cycle.

mid-cycle pain  Pain occurring during ovulation, which can be due to bleeding from the ovary at the site of ovulation. Also called dysmenorrhea.

mucous membrane  An extension of the skin inside the body - for example, the insides of the mouth, the nose, the intestines (the gut) and the uterus (the womb).

multimer  A part of the structure of the VWF molecule. In Type 2M VWD, changes in the multimers affect the binding of the VWF to platelets.

N - Z

obstetrician  A physician who specializes in conception, pregnancy and childbirth.

oophorectomy  An operation to remove the ovaries.

ovulation  The release of the egg from the ovary at the mid-cycle of a woman’s period.

partial thromboplastin time  A routine blood test which often gives normal results in people with VWD.

parvovirus  A human virus carried by a large percentage of the population. Normally harmless, in rare cases, it can cause anemia. It can also cause miscarriage.

plasma  The portion of blood that contains clotting factor proteins, including VWF and factor VIII, as well as immunoglobulins and albumin.

plasmin  A substance in the blood that dissolves blood clots after the blood vessels have healed.

platelets
  Small cells less than 1/10,000 of a centimetre in diameter circulating in the blood, which stick to and spread on the walls of the damaged blood vessel to promote clotting.

platelet function tests  These tests measure how well the platelets work to control bleeding.

progesterone  A natural hormone. As a therapy for VWD, it works by thickening the lining of the uterus and making it less prone to bleed.

prothrombin time  A routine blood test which gives normal results in people with VWD.

Type 1 VWD  A form of von Willebrand Disease in which the von Willebrand Factor is present at lower than normal levels, affecting blood clotting.

Type 2 VWD  A family of different forms of von Willebrand Disease in which the von Willebrand Factor, while present at normal levels, does not work properly, affecting blood clotting.

Type 3 VWD  A form of von Willebrand Disease in which the von Willebrand Factor is almost totally missing. This is the most severe form of VWD.

uterine ablation  Also called endometrial ablation. An operation to destroy the lining of the uterus. The operation is performed through the vagina. The uterine lining is burned away.

vasoconstriction  The first stage in blood clotting in which the blood vessel constricts to reduce the flow of blood to the damaged area.

von Willebrand Disease (VWD)  A family of inherited diseases in which the blood clots more slowly than normal.

von Willebrand Factor (VWF)  The clotting protein that is deficient in VWD. The VWF is either present at lower than normal levels or it does not work properly.

von Willebrand Factor concentrate A blood product made from human plasma, used in the treatment of VWD, containing concentrated von Willebrand factor and factor VIII. In Canada, the most widely used brand is called Humate P.

Wilate  A blood product made from plasma, used in the treatment of VWD and FVIII, containing concentrated von Willebrand factor and factor VIII.