There can be a lot of jargon involved in discussions of treatment and care. This glossary aims to explain what some of the more common word and phrases that you might come across actually mean.
- Acquired Immunodeficiency Syndrome (AIDS)
- An illness which affects the immune system, making people with AIDS more likely to get infections. It is caused by Human Immunodeficiency Virus (HIV)
- Acquired von Willebrand's
- Von Willebrand's can be acquired rather than inhereted, but this is very rare. This means a person is not born with the condition but develops it later in life.
- A measure of the amount of working clotting factors in the blood
- Acute Bleed
- A bleeding incident that is currently happening
- Acute Hepatitis
- The term for hepatitis that lasts less than 6 months
- When red blood cells do not contain enough haemoglobin, the substance that carries oxygen round the body. The usual cause is that there is not enought iron to make red blood cells, and can be the result of heavy bleeding. Anaemia can make you feel tired to iron supplements may be prescribed to boost your iron levels.
- A protein that is produced as part of the immune system’s response to ‘foreign invaders’, it is designed to kill any potential infections
- This means having a condition or illness without any symptoms.
- Asymptomatic Carrier
- A carrier who displays no symptoms of the condition at all, but still carries the affected gene.
- Autosomal Dominant
- In Autosomal Dominant disorders, only one parent needs to have an affected gene in order for their child to have the disorder
- Autosomal Recessive
- In Autosomal Recessive disorders, both parents need to have an affected gene in order for their child to have the disorder.
- A name given to any chromosome that does not determine the sex of the person
- Batch Release Testing (BRT)
- Testing a batch of clotting factor product by regulatory authorities before it is officially released to ensure that the product specifications are met.
- A procedure where tissue, cell or fluid samples are taken to be examined. Liver biopsies are a common test of the extent of cirrhosis damage, or scarring.
- Bleeding Disorder
- When one of the clotting factors does not work as it should, is missing, or is present only at low levels. The blood does not clot the way it should or may not clot at all. When this occurs, we say someone has a bleeding disorder.
- Bleeding Time
- The time required for a minor cut to stop bleeding
- Blood Clot
- A blood clot - made of a jelly-like substance - acts like flue and completely covers the hole in a damaged blood vessel, preventing any more blood from splitting out.
- Blood Product
- Any treatment product derived from blood or plasma
- Blood Transfusion
- The transfer of healthy blood or blood components
- Blood-borne viruses
- Replacement factor made from human blod has been known in the past to carry blood-borne viruses. The most well know for people with bleeding disorder have been HIV and the hepatitis C virus. Effective heat treatment was first introduced in the UK in 1985 for state-produced products (1987 in Scotland for state-produced products).
- Someone who carries an affected gene but doesn’t have the condition itself.
- Carrier testing
- These are tests whihc show whether or not a woman carries the gene for a bleeding disorder which she may, in turn, pass on to her children.
- Central Venous Access Device
- An implant that allows for easier access to a vein for infusion of treatment products.
- Centre Audits
- Every three years the UKHDCO leads in a process of auditing haemophilia centres to see how well they are delivering the National Service Specifications.
- Analytical measurements which allow a detailed understanding of the composition and other attributes fo a clotting factor product.
- Clinical Commissioning Groups (CCGs)
- A group that commissions services, taking over from primary care trusts.
- Clot See: Blood Clot
- Clotting factor
- Certain proteins found in the blood are needed to stop bleeding and are known as clotting factors. Clotting factors work together with other proteins and cells in the blood to help stop bleeding.
- Having two viruses at the same time, this can happen with HIV and Hepatitis C
- The process the NHS uses to decide where it should be spending money. Commissoners decide the size of the relevant budgets in their local area.
- Comprehensive Care Centres (CCC)
- These Haemophilia Centres have a broader range of services and specialists to diagnose and treat people with bleeding disorders.
- Comprehensive Care Centres See: Haemophilia Centre
- Cykllokapron tablets and liquid
- Tranexamic acid tablets and liquid are useful in reducing blood flow. They slow down the body's natural process of breaking down a clot. This helps to stop the bleeding and promotes the healing process.
- DDAVP See: Desmopressin
- DeoxyriboNucleic Acid See: DNA
- Desmopressin (DDAVP)
- A synthetic hormone and one of the most frequently used infusion treatments for von Willebrand's Disease.
- The acid in the chromosomes that helps decide your genetic construct and function.
- Donor Screening
- Individual donatations of blood are screened to ensure that blood-borne viruses do not enter the plasma pool. Screening is currently available for HBV, HCV, and HIV.
- Donor Selection
- Procedures designed to identify and exclude donors at risk of being infected with any blood borne pathogens such as viruses.
- Enveloped Viruses See: Lipid Enveloped Viruses
- European Medicines Agency (EMA)
- The agency that evaluates medicines used in Europe
- Factor Concentrates
- A concentrate used to treat a deficiency of any of the clotting factors. It helps by increasing the number of working factors in order to create the clots needed to block any damage.
- Factor Eight See: Factor VIII
- Factor VIII (FVIII)
- Factor VIII is a protein needed for blood clotting. Thei protein is the one commonly missing, or at low levels, in people with Haemophilia A.
- Factor VIII concentrate
- Factor VIII clotting factor concentrate is made from human blood plasma. This concentrate is effective in treating most cases of sever von Willebrand's Disease.
- Fifth Disease
- A common childhood disease, caused by parvovirus B19.
- Finished Product Testing (FPT)
- Testing done on finished blood products to allow maufacturers to characterize their products and to demonstrate compliance of every batch with the licensed specification.
- The process of seperating and processing human blood plasma into a range of products for therapeutic use.
- Freeze-drying See: Lyophilization
- Genetic disorder
- A genetic disorder is a condition that is passed down through the genes from parent to child.
- Genetic testing See: Carrier testing
- The complete set of genes for a particular organism.
- Good Manufacturing Practices (GMP)
- All the elements in established practice that will collectively lead to final blood clotting products that consistently meet expected requirements as reflected in product specification. These include traceablity, segregation of product manufacturing steps to avoid cross-contamination, training, documentation, change control, deviation reporting.
- Bleeding into a joint.
- A doctor who specialises in disorders of the blood, including bleeding disorders.
- An escape of blood from a broken vessel
- Haemolytic Reaction
- A reaction in recipient blood (from blood transfusion) due to the development of antibodies which destroy red blood cells.
- A bleeding disorder in which clotting factors Factor VIII or Factor IX are either absent or don’t function properly.
- Haemophilia A
- A lack of, or problem with, Factor VIII.
- Haemophilia B
- A lack of, or problem with, Factor IX.
- Haemophilia Centre (HC)
- Haemophilia centres provide care, information and support for people with a bleeding disorder.
- A procedure to stop bleeding
- The time taken for half the infused clotting factor to disappear from a person’s bloodstream
- Home treatment
- Home treatment involves having infusions at home rather than in a hospital of medical setting. Your treatment team will teach you how to infuse yourself, or how to infuse your child or other member of your family.
- Homone-releasing IUD (IUD)
- IUD is short for 'intrauterine device', a small device that is placed in the womb to prevent pregnancy. The hormone-releasing IUD releases a hormone that may reduce the blood flow.
- Immune System
- A group of cells and proteins that work together to fight infections. Through a response known as the immune response it builds antibodies to combat the infectious substances.
- The breakdown or loss of function in part of the immune system, making people more susceptible to diseases.
- The number of new cases of a disease occurring over a period of time.
- Incubation Period
- The period of time between someone first being exposed to an infection and the symptoms occurring.
- When part of the body swells and can become hot, red and painful. This can be caused by bleeding in the joint or muscle.
- Medications or blood products injected into a vein.
- Inherited Condition
- A condition which is carried in the genes and can be passed from one, or both, parents to a child.
- Inherited disorder See: Genetic disorder
- An inhibitor is the name given to the antibody that attacks and destroys clotting factors, which helps form blood clots. It is called this because it inhibits the actions of factors.
- The infusion of a treatment directly into the vein.
- Inventroy Hold
- The retention in storage of plasma for fractionation while processes designed to assure donor safety are undertaken.
- Limit Testing
- Testing of a blood plasma pool using NAT in which a maximum level of viral contamination, rather than an absolute elimination, is the aim.
- Lipid Enveloped Viruses (LEV)
- The common tranfusion transmitted viruses HIV, HCV and HBV, which are all characterized by a lipid viral envelope and are all highly infectious.
- The process of isolating a solid substance from solution by freesing the solution and evaporating the ice under vacuum.
- Marketing Authorization
- The formal permit from a regulatory authority that allows a manufacturer to market a product following that authority's scrutiny.
- Plasma samples pooled from several donations, and then tested for viral markers.
- A process whereby protein solutions are passed over small pore filters which can remove viruses while allowing therapeutic proteins to pass through.
- National Haemophilia Database (NHD)
- The United Kingdom Haemophilia Doctors' Organisation (UKHDCO) maintain a database of information about everyone diagnoised with a bleeding disorder in the UK. Access is carefully restricted and it is primarily used to improve clinical care.
- National Speciailised Commissioning Team (NSCT)
- A group established to commussion services on a regional, or in rarer conditions a national, basis.
- Non-enveloped Viruses See: Non-lipid Enveloped Viruses
- Non-lipid Enveloped Viruses
- Pathogenic viruses (for example HAV or parvovirus B19) which lack a lipid envelope and therefore are not susceptible to viral inactivation techniques such as solvent-detergent treatment.
- Nucleic Acid Testing (NAT)
- Testing for viral nucleic acid, thus allowing detection of a virus which may cause disease before the development of immunological markers of infection.
- Oral contraceptive pill
- The oral contraceptive pill regulates periods, and may be very effective in reducing blood flow. The hormones in these pills cause an increase in von Willebrand factor and factor VIII levels.
- Parvovirus B19
- A human virus which affects a large part of the population, it can cause fifth disease and in extreme cases, leukaemia, anaemia and miscarriage.
- The action of drugs in the body over a period of time, including the processes of absorption, distribution, localization in tissues, biotransformation and excretion.
- The liquid component of blood which makes up about half of its volume. It contains protein as well as hormones, fats, dissolved salts and gases, but no cells. It can be used in the treatment of various bleeding disorders.
- Plasma Master File
- A dossier of information compiled according to European guidelines, which allows the manufacturer of plasma derivatives to fully describe the source material.
- Plasma Pool
- Plasma from a number of donors to be used to make one lot of product.
- A method of collecting plasma from donors wherby only the donor's plasma is removed. This method allows a donor to donate a larger colume of plasma per donation and donate more frequently than is possible when donating whole blood.
- Platelet Function Test
- Tests that measure how good the blood is at stopping bleeding.
- Platelets are special blood cells which form a plug to stop the blood flowing out of the hole in damaged blood vessels.
- Port-A-Cath See: Central Venous Access Device
- Possible Carrier
- Any daughter of a haemophilia carrier has a 50% chance of carrying the gene, making them a possible carrier.
- The biological activity which may be measured in the laboratory which is best related to a clotting factor product's actual therapeutic effect.
- The number of cases of a disease in a population at a given time.
- Primary Care Trust (PCT)
- Product Specification
- The properties of a clotting factor product. They can be measured in the laboratory, allowing a manufacturer to assess and demonstrate fitness of purpose.
- Prophylactic Therapy
- Where the treatment method is taken regularly to prevent bleeds, rather than on demand once bleeding has started.
- A large molecule composed of one or more chains of amino acid, they perform vital functions such as supplying energy and repairing damage.
- The process of removing impurities from the blood.
- The proportion of the desired ingredient (e.g. factor VIII) in concentrates, relative to other ingredients present.
- Quality assurance system
- A mechanism for achieving, sustaining, and improving product quality.
- Recombinant Clotting Factors
- Treatment products which aren’t made from blood, but instead are manufactured to replicate healthy blood.
- Recovered plasma
- Plasma collected as a by-product of donated whole blood. Recovered plasma is generally procured from unpaid donors.
- Self Management See: Home treatment
- Service User
- Term used to refer collectively to everyone using a particular service. Often used as a shorthand for patients, parents and carers.
- Shelf Life
- The period of time during which a product may be stored under specified conditions and retain its characteristics.
- Source Plasma
- Plasma collected from donors through a process known as plasmapheresis, which removes only the donor's plasma. Worldwide the majority of this plasma is obtained from paid donors.
- Specialised Commissioning
- Some conditions or proceedures are too expensive or complicated to be commissioned in the normal way. These are commissioned over larger areas with specialist teams of commissioners. Haemophilia Services are commissioned in this way.
- Specialised Commissioning Groups (SCGs)
- Groups established to commission services on a regional, or for rare conditions a national, basis.
- Symptomatic Carrier
- A carrier who displays mild symptoms of the condition, such as signs of bleeding and low factor levels.
- The pill See: Oral contraceptive pill
- A protein in the plasma which induces clotting.
- Tranexamic acid tablets and liquid. See: Cykllokapron tablets and liquid
- United Kingdom Haemophilia Centre Doctors' Organisation. An organisation of medical practitioners who work in Haemophilia Centres across the UK.
- United Kingdom Haemophilia Centre Doctors' Organisation See: UKHCDO
- The action of proving that any material, process, procedure, activity, system or equipment used in manufacture of control can and will reliably achieve the desired and intended results.
- Variant Creutzfeldt-Jakob Disease (vCJD)
- variant Creutzfeldt-Jakob Disease, a rare and ultimately fatal progressive degenerative brain disease, caused by a build-up in the brain of an abnormal form of the ‘prion’ protein.
- von Willebrand factor (vWF)
- von Willebrand factor is one of the proteins in the blood that works to make blood clot. It acts like a 'glue' to make platelets stick to the areas of blood vessels that are damaged.
- von Willebrand factor concentrate
- von Willebrand factor concentrate is made from human blood plasma. This concentrate is effective in most cases of sever von Willebrand's Disease.
- von Willebrand's Disease (vWD)
- von Willebrand's is a blood clotting and bleeding condition.
- Window Period
- The period between thwn a donor is infected with a cirus or desease-causing agent and when infection can be detected by an immunological marker. During this period the donor is infectious but the infection is undetectable. With nucleic acid testing (NAT), the window period is shortened.