Glossary

A

Acquired hemophilia: A form of hemophilia in which the body starts producing antibodies that fight its own blood clotting proteins.
Activated partial thromboplastin time (aPTT): Measures clotting time of the blood to determine the condition of the intrinsic and common pathways of the blood-clotting process.
Activated platelet: Tiny cells in the blood that stick to an injured vessel and to each other to form a plug.
Administer: In terms of hemophilia, it means to apply to the body through infusion.
Albumin: A water-soluble protein found in the blood.
Analgesics: Painkiller medications.
Anamnestic response: An increase in inhibitor level that can occur quickly the second time the body is exposed to factor replacement therapy.
Antibodies: Cells that the immune system produces to fight and destroy viruses and bacteria that can cause infection.
Antifibrinolytic: A type of substance that decreases the breakdown of fibrin, keeping blood clots intact. Antifibrinolytic therapy helps maintain the blood clots that the body forms to stop bleeding.
Aseptic: Free of disease-spreading microorganisms.
Autosomal recessive disorder: An inherited disorder having to do with an autosome, which is a chromosome that is not a sex (gender) chromosome. Both parents must have the disorder to pass it on to their child.

B

Bacteria: One-celled pathogens that reproduce quickly and can cause illness.
Bethesda inhibitor assay: A test performed in the lab and used to measure the strength or titer of the inhibitor. Inhibitors are reported in Bethesda Units (BU).
Bleed: A collection of blood in an area, such as a muscle or joint.
Bolus: A large dose of medicine that is given at the beginning of treatment to raise blood-level concentrations to a therapeutic level.
Bypassing products: A product that skips the need for Factor VIII or Factor IX in treating a bleed.

C

Capillaries: A network of very tiny blood vessels throughout the body that helps various substances circulate between blood and tissue cells.
Cartilage: A tough connective tissue found in various parts of the body, such as the joints, outer ear, and larynx.
Central nervous system: The part of the nervous system consisting of the brain and spinal cord, which coordinate all impulses that circulate through the body.
Chromosome: A threadlike structure, found in every cell, that contains all of a person's genetic information.
Classic hemophilia: Another name for hemophilia A, the most common type of hemophilia.
Clinical trials: Carefully coordinated studies to determine the effectiveness and safety of medicines and treatment strategies.
Clot: The plug your body forms at the place where you've been injured to stop the bleeding.
Clotting: The process of forming into a clot or clots.
Clotting assay: A blood test given to determine what specific bleeding disorder a person has, based on a prolonged PT or PTT previously measured.
Clotting (coagulation) factors: Substances in the blood that work together with platelets to form a fibrin clot in an injured blood vessel and stop bleeding. Clotting factors are named using Roman numerals (Factor I [one] to Factor XIII [thirteen]).
Coagulation: Also known as blood clotting. It is the process of turning some of the elements in the blood into a clot. Coagulation is the result of a sequence of actions and reactions that involve several different proteins in the blood.
Co-infection: To be infected with more than one illness at the same time.
Comprehensive care: Treatment at a doctor's office where patients will see a variety of doctors about different aspects of their health and body.

D

Deficiency: A lack or shortage of something.
Desensitize: To treat a person so their immune system does not react to a certain foreign substance by developing antibodies.
Diluent: A substance that changes the consistency of something else. For example, water dilutes iced tea powder to make tea that people drink.
DNA: Deoxyribonucleic acid. DNA contains all of a person's genetic information and is found in every cell in that person's body.

E

Electrolytes: Various minerals in the bloodstream that regulate the electric charge of the cells.
Eradicate: To eliminate or get rid of.
External bleeding: Bleeding on the outside of the body, such as from cuts and scrapes.

F

Factor: A protein in the blood that helps form blood clots.
Factor IX (nine) deficiency: Absence or shortage of Factor IX (nine), one of the 13 factors that help form blood clots. Also called hemophilia B.
Factor replacement: Replacing deficient or absent factor protein in the body with donated or synthetic factor of the same type.
Factor VII (seven) deficiency: A rare bleeding disorder in which the blood has low levels of Factor VII. Factor VII is important in the blood-clotting process because it binds to blood cells near the location of an injury and to activated platelets, setting the clotting process in motion.
Factor VIII (eight) deficiency: Absence or shortage of Factor VIII, one of the factors that help form blood clots. Also called hemophilia A.
Fibrin: A substance in the blood that combines with blood cells and platelets to form a stable clot at the site of bleeding.
Fibrin clot: Fibrin that works together with a group of clotting factors and blood cells to make a patch (a stable clot) and stop bleeding.

G

Gene: A section of DNA. Each gene on a strand of DNA contains certain genetic traits that have been passed on from parents to child.
Genetics: The science of heredity and how genes transfer to offspring.

H

Hemarthrosis: When blood accumulates in a joint, causing extreme swelling. Repeated bleeding of this type over time can cause a permanent condition called hemophilic arthropathy.
Hematologist: A doctor who is an expert in blood diseases, such as hemophilia, acquired Factor VIII inhibitors, and other clotting disorders.
Hemophilia: A clotting disorder that occurs mostly in males. The disorder makes bleeding hard to control.
Hemophilia A: A bleeding disorder caused by lack of Factor VIII. It is sometimes called classic or standard hemophilia.
Hemophilia B: A bleeding disorder caused by lack of Factor IX.
Hemophilia Treatment Center (HTC): A medical facility that specializes in the comprehensive treatment of hemophilia and other clotting disorders.
Hemophilia with inhibitors: A bleeding disorder where the body fights against the factor used to treat hemophilia.
Hemophilic arthropathy: Chronic joint disease in hemophilic individuals.
Hemorrhage: Excessive and sometimes uncontrollable bleeding.
Hemostasis: The stopping of bleeding through the blood-clotting process.
High-responding: Type of inhibitor that responds quickly with high levels of antibodies to factor replacement therapy.
Histidine: An essential amino acid which is present in many proteins.
Home therapy program or Home treatment (therapy): A program in which the patient or a caregiver administers factor treatment, when needed, in the home setting, thereby avoiding having to travel to a hospital or HTC.

I

Immune system: A mechanism within the body used to protect itself from harmful substances.
Immune tolerance therapy (ITT or ITI): Treatment in which high doses of Factors VIII or IX are used to lower inhibitor levels in patients with hemophilia A or B.
Infusion: The injection of medicine directly into a vein using a needle and syringe.
Inhibit: To hold back, make difficult, or prohibit.
Inhibitor: A substance produced by the body that attacks factor so it cannot work as well. For example, a Factor VIII inhibitor would keep Factor VIII from working with other factors and platelets to stop bleeding.
Insurance caps: The amount of money an insurance company pays to cover an illness or procedure. A lifetime cap is the amount of money an insurance company will spend during the lifetime of the policy holder. Yearly caps refer to a maximum amount that can be spent per year.
Internal bleeding: Bleeding inside the body, in tissues or organs.
Intraoperative: Something that occurs during a surgical operation.
Invasive procedure: Most often referred to as surgery, because the doctor enters the body through an incision to repair damage.

J

There are currently no glossary items for the letter "J."

K

There are currently no glossary items for the letter "K."

L

Low-responding: Type of inhibitor that has a slow, weak response to factor replacement therapy.

M

Mutation: A spontaneous change in the structure of a gene or chromosome, which leads to a new trait not found in parents or any family.

N

Neutralize: To counteract the effect of something so that it isn't capable of producing any change.

O

Obligate carrier: Someone who has a disease-causing gene but does not actually have the disease because he or she has one normal gene.
On-demand therapy: Treatment that responds to a need when it occurs, instead of before it occurs.
Orthopedic surgery: Surgical treatment for diseases, disorders, and injuries involving bones, joints, and tendons. These surgical treatments are performed by orthopedic surgeons.

P

Pathogen: An organism, such as a virus, prion, or bacterium, that causes disease. An emerging pathogen is a pathogen that has recently or not yet been discovered.
Plasma: The fluid part of the blood that contains clotting factors and other proteins.
Plasmapheresis: A process that removes harmful components from the blood.
Platelets: Tiny cells in the blood that stick to an injured vessel and to each other to form a plug.
Postoperative: Something that happens after a surgical operation.
Prion: A type of pathogen that is known to cause diseases that attack brain tissue.
Prolonged: Something that has taken a longer time in duration than normal.
Prophylaxis: Factor replacement therapy that is given on a regular basis to keep a bleeding episode from starting.
Prothrombin: A material in the blood essential to forming stable clots.
Prothrombin time (PT): Measures clotting time of the blood to determine the condition of the extrinsic and common pathways of the blood-clotting process.

Q

There are currently no glossary items for the letter "Q."

R

Recombinant: Genetically engineered DNA. Made without human blood or plasma.
Reconstitution: Adding liquid to prepare a medicine.
Red blood cells: Cells in the blood that transport oxygen and carbon dioxide.
Rehabilitation: To restore to good health.
Resolve: To cause reduction in something, such as swelling or bleeding.
R.I.C.E.: Stands for rest, ice, compression, and elevation. It's a type of therapy that can relieve pain and discomfort during a bleed, particularly in a joint.

S

Spontaneous: Something that happens on its own, for what seems like no reason. Spontaneous bleeding doesn't appear to have a specific cause.
Surgeon: A doctor specializing in surgery, which is a class of operations that involves operating through an open incision in the skin to get to damaged parts within the body.

T

Thrombin: Also known as Factor IIa (2a). It's a protein that binds together other proteins and activated platelets to start forming a clot. Thrombin is created when activated Factor X (Factor Xa) comes in contact with prothrombin.
Thrombosis: An event that takes place when veins or arteries become blocked by platelets that are working to "plug" an injury, preventing blood from flowing normally through the body, and possibly causing a stroke.
Titer: Concentration or strength of a substance; measures the strength of an inhibitor in a person's blood.
Tolerability: Capability of being put up with or endured.
Tolerate: The ability to endure doses of a medicine
Transient: Something that stays in place temporarily and then disappears on its own, such as an inhibitor, which is an antibody to coagulation factors that are brought into the body.

U

There are currently no glossary items for the letter "U."

V

Virus: A pathogen that enters healthy cells and reproduces into large numbers, which then spread through the bloodstream and air passages.

W

White blood cells: Cells in the blood that help protect the body from infection and disease.

X

X-linked: Having to do with gene transmission along the X chromosome in DNA.

Y

There are currently no glossary items for the letter "Y."

Z

There are currently no glossary items for the letter "Z."