Fibrinogen is one of the clotting factor proteins that are needed to help the body form a blood clot to stop bleeding. People diagnosed with Dysfibrinogenemia have enough fibrinogen in the blood, but the fibrinogen does not work correctly. This can delay the time it takes to make a blood clot and for this blood clot to be weak, causing bleeding to last longer or to stop and then start back up again. Though Dysfibrinogenemia is considered a bleeding disorder, there is also a risk for increased clotting because the poorly working fibrinogen can also cause problems with the normal breakdown of a blood clot the body has made. Increased clotting can cause a thrombosis (a blood clot that prevents blood flow) to form.
Dysfibrinogenemia is a very rare inherited bleeding disorder. One or both parents can pass the abnormal gene to their child. If one parent passes the gene to their child, it is called heterozygous and usually symptoms are minimal. If both parents pass the affected gene to their child, it is called homozygous and makes the risk for much bleeding higher because both genes are affected.
- Increased bruising and/or bruises that take a long time to heal
- Prolonged bleeding
- Excessive bleeding from a circumcision
- Excessive bleeding from surgeries
- Bleeding into the joints
- Heavy Menstrual Bleeding
- Getting multiple blood clots
Diagnosing Dysfibrinogenemia
When testing for Dysfibrinogenemia, a series of blood testing will need to be done and a detailed bleeding history will be taken. People who should be tested for a bleeding disorder include:
- Someone with a parent or sibling diagnosed with a bleeding disorder
- Someone with bleeding symptoms who also has a family member(s) diagnosed with a bleeding disorder
- Someone born female that is experiencing heavy menstrual bleeding without a known cause
- Someone experiencing signs and symptoms of a bleeding disorder
Fresh Frozen Plasma
One treatment for Dysfibrinogenemia is to give Fresh Frozen Plasma. Fresh frozen plasma is a transfusion of plasma, donated from another person, which is given in a vein. It contains fibrinogen and other clotting factors that will help the body to stop bleeding; but it also contains anti-clotting factors that will help to prevent a blood clot that prevents blood flow through veins. Mostly, people diagnosed with Dysfibrinogenemia will only need treatment when injury occurs, before more invasive dental work and before surgeries.
Cryoprecipitate
Cryoprecipitate is a transfusion of plasma, donated from another person, which is given in a vein. It contains fibrinogen and three of the clotting factors. Cryoprecipitate will provide fibrinogen that works correctly to aid in making a blood clot to stop bleeding. It does not contain anti-clotting factors so should not be given to someone who has a strong family history of blood clots or has had blood clots themselves.
Amicar/Lysteda (Aminocaproic Acid/Tranexamic Acid)
Amicar and Lysteda are oral medications that work very well for bleeding of mucous membranes such as nose and mouth bleeding. Lysteda is also used for heavy menstrual bleeding. When bleeding occurs, the body tries to heal the area by making a clot that will plug the hole to stop bleeding. This clot will stay there for about four days. After these four days, chemicals in the body are triggered to breakdown the clot. This is called fibrinolysis. Amicar and Lysteda are called anti-fibrinolytic medications because it stops fibrinolysis. A person with a bleeding disorder needs more time for healing because they cannot make a strong clot to heal the area of bleeding. Amicar and Lysteda stops the body from breaking the clot down. This gives the blood vessels more time to completely heal and prevents prolonged bleeding and rebleeding.
Bleeding can occur anywhere in the body. Usually bleeding is caused by injury, surgery or dental procedures.
Complications from Dysfibrinogenemia can include:
- Prolonged bleeding leading to anemia
- Bleeding in joints leading to arthritis or damage to joints
- Damage to the brain (Head injury resulting to bleeding inside the skull)
- Difficulty breathing (Due to bleeding in the neck or lungs after injury)
- Paralysis (Due to bleeding in the spinal column after injury)
