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Dysfibinogenemia

 

Fibrinogen (fy BRIH neh jen), also called Factor I, is a blood plasma protein produced by the liver. It plays an essential role in blood clotting. When a blood vessel is injured, the body forms a clot to stop bleeding. Fibrin, which is converted from fibrinogen, is the main protein helping form the clot.

When there is a problem with fibrinogen—either it is missing or does not work properly—this can affect the clotting process. It can result in either too much bleeding or too many clots.

When fibrinogen does not work properly, the patient has dysfibrinogenemia (dis fy BRIN oh jeh NEH mee ah). This disorder is passed down from either the mother or father. Those affected rarely suffer major bleeding problems. 

With dysfibrinogenemia:

  • Bleeding may be absent (no symptoms);
  • The patient may show a tendency to bleed (usually minor); or
  • The patient may show a tendency toward thrombosis (clotting).

What treatment is needed?

Many people with dysfibrinogenemia do not need treatment, and any treatment options must be tailored to the patient. If treatment is needed, the following might be used:

  • Aminocaproic Acid (Amicar®) for treatment of mucosal bleeding (nosebleed, gum bleeding, heavy menses, mouth/lip bleeds, etc).
  • Cryoprecipitate (made from frozen blood) and fresh frozen plasma may be needed in the event of surgery or trauma.

While some people receive infusions of fibrinogen concentrate for other conditions, it is not recommended for people with dysfibrinogenemia.

For bleeding prevention

  • Never take aspirin. Aspirin increases the risk of bleeding by decreasing platelet function.
  • Prevent dental problems with proper brushing and routine dental visits.
  • Always contact St. Jude if you have surgery or a tooth extraction scheduled. The staff has guidelines for before and after the procedure.
  • Always wear a medical alert bracelet explaining your condition.
  • Always wear protective equipment when doing certain physical activities.
  • Contact sports such as boxing, soccer, hockey and football must be avoided because of the high risk of bleeding. 

Questions?

If you have questions about dysfibrinogenemia, call the nurse case manager at 901-595-5678 and leave your child’s name, MRN number, your name, and phone number for a return call.

If you have a concern about a bleeding problem, call the nurse case manager at 901-595-5678. If you cannot reach her, call the clinic at 901-595-5041 during working hours. For after hours, weekends, or holidays, call the St. Jude operator at 901-595-3300 and ask for the hematologist on call. 

To learn more about this disorder, visit www.hemophilia.ca/en. In the left menu choose Bleeding Disorders, then Other factor deficiencies. From the list choose Factor I deficiency (Fibrinogen deficiency)


 

This document is not intended to take the place of the care and attention of your personal physician or other professional medical services. Our aim is to promote active participation in your care and treatment by providing information and education. Questions about individual health concerns or specific treatment options should be discussed with your physician.

St. Jude complies with health care-related federal civil rights laws and does not discriminate on the basis of race, color, national origin, age, disability, or sex.

ATTENTION: If you speak another language, assistance services, free of charge, are available to you. Call 1-866-278-5833 (TTY: 1-901-595-1040).

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