Factor XIII (13) is one of the clotting factor proteins that are needed to help the body form a blood clot to stop bleeding.  People diagnosed with Factor XIII (13) Deficiency do not have enough factor XIII (13) clotting protein in the blood.  Not having enough of any of these clotting proteins 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.

Factor XIII (13) Deficiency is a rare, inherited bleeding disorder.  One or both parents can pass the abnormal gene for Factor XIII (13) Combined Deficiency 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.

SIGNS AND SYMPTOMS

  • Increased bruising and/or bruises that take a long time to heal
  • Prolonged bleeding from injuries
  • Nosebleeds
  • Bleeding gums
  • Excessive/prolonged bleeding from a circumcision
  • Excessive/prolonged bleeding from surgeries
  • Heavy or prolonged menstrual bleeding
  • Bleeding into the joints (Rare)

 

Diagnosing Factor XIII(13)
When testing for Factor XIII (13) Deficiency, 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

TREATMENT

Clotting Replacement Factor
The treatment for Factor XIII (13) Deficiency is to replace the Factor XIII (13) clotting protein that the body is lacking.  Factor replacement is given through an intravenous (in the vein) infusion.  Frequency of treatment is dependent upon disease severity and bleeding history.  Treatments are either provided when a bleeding episode occurs (episodic) or to prevent a bleeding episode from occurring (prophylaxis).  Mostly, people diagnosed with Factor XIII (13) Deficiency only need treatment when injury occurs, before more invasive dental work and before surgeries.  Those who experience frequent bleeding and/or are diagnosed with severe Factor XIII (13) Deficiency will need to have treatments to prevent bleeding (prophylaxis treatments).  If prophylaxis treatments are needed, patients and caregivers will be taught how to give the factor replacement through the vein so treatments can be given at home.

 

Fresh Frozen Plasma
Not all hospitals have clotting factor replacement products available.  If severe bleeding or injury occurs, fresh frozen plasma will need to be given. Fresh frozen plasma is a transfusion of plasma donated from another person.  It contains all clotting factors and will help the body to stop bleeding.

 

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.  [/vc_column_text

COMPLICATIONS

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Bleeding can occur anywhere in the body.  Usually bleeding is caused by injury, surgery or dental procedures.

Complications from Factor XIII (13) Deficiency 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)