A list of glossary terms related to the the management and care of hemophilia.
| Term | Definition |
|---|---|
| Activated prothrombin complex concentrates | Plasma-derived concentrates that contain many activated clotting factors. These activated clotting factors can “bypass” an inhibitor’s actions. |
| Acute pain | In hemophilia, pain which is caused by acute bleeding and not by a chronic joint disease such as arthritis. |
| Adrenaline | The hormone released when your body experiences stress. Adrenaline causes a boost in energy. |
| Amniocentesis | A test used to detect abnormalities in the fetus. A thin needle is inserted through the abdomen and into the uterus to obtain a small amount of amniotic fluid. The amniotic fluid contains cells shed by the fetus, which are examined for defects. |
| Antifibrinolytic agents | Drugs that help hold a clot in place once it has formed by stopping the activity of an enzyme, called plasmin, which dissolves blood clots. |
| Arthritis | Inflammation of the joint. In addition to inflammation of the synovial lining, there is also damage to the cartilage and bones of the joint surfaces. In hemophilia, arthritis is caused by repeated bleeding into the joint cavity. |
| Bethesda assay | A blood test to measure the level of a clotting inhibitor once it is known to be present. The results of the test are given in Bethesda Units (BU). |
| Blood clotting | The process of forming a permanent clot to repair a damaged blood vessel. It includes four steps: vasoconstriction, platelet plug formation, and coagulation. |
| Budget | A plan that outlines the amount and the way you are going to spend money. |
| Bursary | A monetary award that does not have to be repaid, given to individuals who have financial need. |
| Bypassing therapy | A treatment for patients with inhibitors. The factor concentrate that is infused contains clotting factors that work around the inhibitor. |
| Chorionic villus sampling | A type of pre-natal testing for hemophilia. A very small sample of the chorionic villus (part of the placenta) from inside the womb is taken out and tested in the lab. |
| Chronic pain | In hemophilia, pain due to damaged tissues or joints lasting for more than three months and not an acute bleed. |
| Clotting cascade | The chain reaction in which clotting factors, which are tiny plasma proteins, link to form a chain, called fibrin, around the platelets at the site of a break in a blood vessel wall. |
| Complementary and alternative medicines (CAM) | Health-care systems, practices, and products that are not a part of conventional medicine. |
| Comprehensive Care Team (CCT) | The group of health-care professionals who work together to help you manage your hemophilia. This might include doctors, nurses, physiotherapists, social workers, dentists, pharmacists, and psychologists. |
| Diuretic | A substance that causes your body to expel more water than it needs to. |
| Enamel | The protective coating on your teeth. Enamel cannot be regenerated once it is destroyed, so it is important to take care of your teeth to keep your enamel strong. |
| Endorphin | A hormone released in the body that improves one’s mood. |
| FactorFirst card | The Canadian Hemophilia Society (CHS) provides a wallet-sized card that you can carry with you to help you tell health-care providers in the Emergency Room about your hemophilia. A copy of this card can be obtained from your CCT or you can download and print a copy from the CHS website. |
| Factor replacement therapy | The treatment in which clotting factors are infused into the bloodstream of a person with hemophilia to replace those that are missing, and temporarily correct the coagulation disorder. |
| Fibrin | The material making up the clot that forms in the last stage of the clotting process. |
| Fresh frozen plasma | A component of whole blood that was used for treatment of hemophilia in the 1950s and 1960s. |
| Gene | Packages of DNA that determine such things as the colour of a person’s eyes. Hemophilia is caused by an abnormal gene on the X chromosome, one of the sex chromosomes. |
| Genetic mutation | The specific mistake in the gene. |
| Hematuria | Blood in the urine, caused by bleeding in the kidneys. |
| Hemoglobin | A substance in the red cells of blood, responsible for carrying oxygen. |
| Hemophilia A | A genetic disorder characterized by frequent bleeding into joints, muscles and tissues. The prolonged bleeding is caused by low or missing levels of factor VIII (factor 8) or by non-functioning factor VIII. |
| Hemophilia B | A genetic disorder characterized by frequent bleeding into joints, muscles and tissues. The prolonged bleeding is caused by low or missing levels of factor IX (factor 9) or by non-functioning factor IX. |
| Hepatitis C | A viral disease that causes liver damage, which usually takes many years to develop. Hepatitis C is transmitted through blood (e.g., the exchange of contaminated needles and, in very rare cases, by fresh blood components). |
| High-titre inhibitor | An inhibitor that is measured at more than 5 Bethesda Units. The antibodies of a person with a high-titre inhibitor are stronger and destroy the factor concentrate more quickly. |
| Home therapy | The care of the person with hemophilia at home, rather than in hospital. This includes the administration of clotting factor concentrates by the person with hemophilia or by a family member. |
| Iliopsoas | A group of muscles located deep in the hips. |
| Immune tolerance therapy (ITT) | The infusion of high doses of the missing clotting factor concentrate 3 to 7 times per week for very long periods of time—months or years. The objective of the therapy is to allow the body’s defenses to become accustomed to the foreign factor and to stop making antibodies against it, so that normal doses will be effective in stopping bleeding. |
| In case of emergency (ICE) | Store all your emergency contact numbers in an entry called ICE: In Case of Emergency. Many times, emergency personnel or hospital staff do not know who to call if a patient has a cell phone. Similar to 911, ICE is becoming a nationally recognized name. |
| Inhibitors | An antibody that the body produces that recognizes infused clotting factor as foreign and prevents it from being effective/working properly. |
| Intramuscular Injection | A vaccine that is injected deep into a muscle, usually in the upper-arm. |
| Joint | A joint is the place where two bones come together, allowing movement such as bending, rotating and swinging back and forth. |
| Joint bleed | Bleeding into a joint caused by a tear in the lining of the joint (synovium), blood escapes from the blood vessels and gradually fills the joint cavity. A joint bleed is usually because of “pinch or twist” types of injury to the joint. It can happen especially if the activity involves putting full weight and stress on a joint or forceful movements such as throwing and kicking. |
| Joint capsule | The joint space and the tissue membrane that forms a “sleeve” around the joint together form the joint capsule. |
| Joint disease | Synovitis and arthritis. These diseases in hemophilia are caused by repeated bleeding into joints. They are most common in knees, ankles and elbows. |
| Joint replacement | The complete replacement of the joint cavity with artificial (man-made) materials. The joints most commonly replaced are the knee and the hip. However, the operations cannot be performed on young people as the materials used to replace the joint wear out. |
| Low-titre inhibitor | An inhibitor that is measured at less than 5 Bethesda Units. |
| Magnetic resonance imaging (MRI) | An MRI shows the internal structures of your body in tremendous detail, including tiny structures like your veins, arteries and nerves. |
| Muscle bleed | Bleeding into a muscle, which can be caused by a single serious trauma or small but repetitive trauma. A muscle bleed can happen if a muscle is strained or stretched too much. |
| MyHealth Passport | A wallet-sized card that includes all of your relevant health information. A health passport is an efficient way to communicate the details of your hemophilia to new health-care providers. You can create your own here: My Health Passport. |
| Non-steroidal anti-inflammatories (NSAIDS) | Medicines that are commonly prescribed for pain. However, NSAIDS interfere with the ability of blood to clot. When looking for medicines to treat pain, avoid taking NSAIDS, which include acetylsalicylic acid (ASA, Aspirin), ibuprofen (Advil and Motrin), and naproxen (Aleve). |
| Nutrition Facts Table | A label found on packaged food products that tells you the nutritional value in one serving (such as fats, carbohydrates, calories, sodium, and cholesterol). |
| On-demand therapy | An infusion of clotting factor concentrate as soon as the person with hemophilia, or a caregiver, is aware of a bleed. The goal is to promptly stop the bleed. |
| Physiotherapy | The use of exercise to stay fit or rehabilitate weakened muscles and damaged joints. |
| Plasma-derived factor | Factor that is collected from a large group of human blood donors. |
| Port | A central venous access device that is surgically implanted just under the skin. It allows easier infusion of clotting factor concentrates if access to the veins is more difficult. |
| Post-secondary education | The level of schooling that follows high school. University, college, and trade school are all forms of post-secondary education. |
| Prerequisite | A requirement one must fulfill. |
| Prophylaxis | Regular infusions of clotting factor concentrates. This is done in order to prevent bleeding episodes from happening. |
| Psoas | The muscles located on both sides of your lower spine. The psoas extends through your pelvic area to your hip joint, allowing you to freely move your hips. |
| Radioactive synovectomy | An operation to shrink the synovium in a joint. A radioactive chemical is injected into the joint. The swollen synovium dies and is eventually replaced by a healthy one. |
| Recombinant factor | Clotting factor that scientists and engineers make artificially inside a laboratory; cells that produce factor are grown inside a controlled environment in the lab. |
| Release of medical records | A form that gives health-care workers from other institutions access to a patient’s medical records. |
| R.I.C.E | An acronym that stands for Rest, Ice and Immobilization, Compression, and Elevation. These are the steps you can follow in case you have an injury that causes a bleed. |
| Scholarship | A monetary award typically given to individuals who present high academic or athletic achievements or who have made a contribution in the community. |
| Self-infusion | The administration of clotting factor concentrates by the person with hemophilia. This is done intravenously using a syringe and needle. |
| SMART | An acronym to help set good goals: specific, measurable, achievable, realistic, and time appropriate. |
| Standardized test | A test administered by the government or school system that is used to evaluate students of the same grade level in a fair manner. |
| Stress | The physical and emotional response of your body to pressure. |
| Stressors | Situations or demands that cause pressure. |
| Surgical synovectomy | An operation to remove the synovium in a joint. It is used when more extensive joint damage with cartilage erosion makes an arthroscopic synovectomy impractical. The surgeon makes an incision in the joint to remove the synovium and smooth any rough spots on the bones themselves. This operation is also called an open synovectomy. |
| Synovial membrane | Also called the synovium. It is a layer of special cells that line the capsule of the joint. |
| Synovitis | A type of joint disease caused by repeated bleeding into the joint cavity. The synovial membrane, in an attempt to remove old blood and debris from the joint, grows new blood vessels and becomes thicker and prone to repeated bleeding. |
| Three-sentence health summary | An easy way to discuss important health information with your health-care team. |
| Transition | The process of becoming self-sufficient and learning to manage your hemophilia on your own. Transition may involve the movement from a paediatric centre to an adult centre. |
| Tuition | A fee charged by post-secondary schools that covers the cost of the courses a student enrolls in as well as maintenance of facilities and services. |
| Ultrasound | An imaging technique that uses sound waves to take pictures of the inside of your body. These pictures show information about the size, shape, and texture of the body part being scanned. |
| Vaccination | Medicines that are injected, usually into the muscle, to help protect you from serious infections and diseases. |
| X-ray | A type of radiation that passes through the body. It’s a quick way to visualize the internal structures of your body. |
