ACHIA

Factor IX Deficiency


COMMON BLEEDING PROBLEMS AND CLINICAL SITUATIONS

Following are specific recommended plasma levels and actions for common bleeding problems and clinical situations which occur with Hemophilia B, also known as Factor IX Deficiency:

 

A:  Mild Bleeding Episodes:

 

Bleeding Problem

Action

Suggested Factor IX Level (Dose)

  • Mild Bruises
  • Minor cuts or scrapes
  • Brief nosebleeds
  • Brief mouth bleeds
  • Observation, no treatment
  • In pts 3 yrs old or younger, or newly diagnosed pts, physician visit may be necessary until parents learn to assess 
  • No replacement needed

 

B:  Moderate Bleeding Episodes:

 

Bleeding Problem

Action

Suggested Factor IX Level (Dose)

  • Hemarthrosis
  • Muscle bleeds of arms and legs
  • Prolonged mouth bleed, especially in toddlers
  • Prolonged nosebleed, any age
  • Hematuria
  • Assess situation
  • Treat promptly with I.V. factor
  • Repeat I.V. factor in 24 hrs if symptoms continue or re-occur
  • Call Pediatric Hematologist if uncertain or question need for further factor
  • 40-50% plasma IX levels (40-50 units Factor IX/kg)
  • Note: Recombinant Factor IX (Benefix) may have a decreased bioavailability and require increased dosing for equivalent hemostasis. Consider increasing the Factor IX dose by a factor of 1.4 if Benefix is utilized.

 

C:  Serious & Emergency Bleeds:

 

Bleeding Problem

Action

Suggested Factor IX Level (Dose)

  • Suspected intracranial bleed
  • Head bleeds w/trauma
  • Bleeds of the eye
  • Threatening the airway (neck, throat), GI, or iliopsoas
  • Go in ER promptly
  • Treat with factor promptly
  • Pursue emergency management as needed
  • Reassess frequently
  • If CNS hemorrhage possible, give factor prior to head CT/MRI imaging
  • Notify Pediatric Hematologist of event to discuss subsequent management
  • 80-100% plasma IX activity (80-100 units Factor IX/kg)
  • Note: Recombinant Factor IX (Benefix) may have a decreased bioavailability and require increased dosing for equivalent hemostasis. Consider increasing the Factor IX dose by a factor of 1.4 if Benefix is utilized.

 

MINOR/MAJOR SURGERY:

If sutures are required or if any surgery, elective or emergency, is anticipated, please contact the Pediatric Hematologist on call at 205-939-9285.  (NOTE:  Plans for elective surgery should be discussed one or more weeks before the planned surgery with the Pediatric Hematologist.)

 

DENTAL INTERVENTION:

Dental care requiring tooth extraction, stitches, or nerve block should be discussed with the Pediatric Hematologist.  For elective dental work, this discussion should occur $1 week prior to the intervention.

 

IMMUNIZATIONS:

Children with hemophilia should be given complete immunizations on schedule, if possible.  We recommend they be given as follows, to avoid muscle bleeds and complications:

  • Never give in buttocks
  • Use 25-27 gauge needle
  • Follow individual immunization instructions for route of administration (intramuscular or subcutaneous)
  • Apply direct pressure 20-30 minutes following injection
  • Check the site the next day, and have nurse check the site or make phone contact with parents for next three days

 

Rev 7/27/2009