Factor IX

Registered South African indications:

• Factor IX deficiency

Contraindications

• Hypersensitivity to the active ingredient or any of its excipients
• Known / history of heparin-induced thrombocytopenia [HIT] type II

Cautions

• Pregnancy
• Breastfeeding
• Hepatic impairment
• Repeated dosing
• Neonates
• Risk factors for thromboembolic events
• Risk factors for disseminated intravascular coagulopathy
• Cardiovascular risk factors
• Central venous access device placement
• Immunodeficiency
• Increased erythropoiesis
• Patients on a low sodium diet

Forms avail. in SA and covered in this monograph: inj

Congenital (Haemophilia B) or acquired Factor IX deficieny

• Minor bleed: IV: 20-40 IU/kg
• Major bleed: IV: 60-80 IU/kg

Patients undergoing surgery

• Minor surgery: IV: 20-40 IU/kg, 30min before surgery; Repeat dose at 6hrs post-operatively then dly thereafter
• Major surgery: IV: 60-80 IU/kg

Factor infusion for major surgery should continue for 7-14 days; Venous thromboembolism prophylaxis using elastic stockings should be considered in all high-risk surgery.

Continuous infusion of Factor with a pump may be used.

Keep peak maintenance Factor level at 50% until healing has started.

Introduce postoperative rehabilitation and mobilisation gradually under Factor prophylaxis.

Use of antibiotics post-operatively is mandatory.

Ensure that patient receives adequate analgesia -NOTE: avoid intramuscular analgesia

Prophylaxis

• IV: 25-40 IU/kg, twice a week

Rationale for prophylaxis is to maintain Factor activity above 1% converting a bleeding pattern from severe to mild/moderate

Treatment should be advised by a Comprehensive Haemophilia Treatment Centre found across South Africa. See Care Co-ord section for contact numbers

Paed. forms avail. in SA and covered in this monograph: inj

Congenital (Haemophilia B) or acquired Factor IX deficieny

Younger than 6 years

• Not established for use

Older than 6 years

• See adult dosage

Pregnancy 

• Caution; not recommended
• Animal reproduction studies have not been conducted with factor IX
• Based on the rare occurrence of haemophilia B in women, experience regarding the use of factor IX during pregnancy and breastfeeding is not available. Therefore, factor IX should be used during pregnancy and breastfeeding only if clearly indicated

Breastfeeding 

• Caution; not recommended 
• Animal reproduction studies have not been conducted with factor IX
• Based on the rare occurrence of haemophilia B in women, experience regarding the use of factor IX during pregnancy and breastfeeding is not available. Therefore, factor IX should be used during pregnancy and breastfeeding only if clearly indicated

Renal Impairment

• No dose adjustment recommended

Hepatic Impairment

• Caution; no dose adjustment recommended

Elderly

• No dose adjustment recommended

Porphyria Information

• Probably not porphyrinogenic (PNP)

Central Nervous System Disorders

• Anxiety
• Hyperaesthesia

Cardiovascular Disorders

• Hot flushes

Respiratory Disorders

• Dyspnoea

Gastrointestinal Disorders

• Nausea

Musculoskeletal Disorders

• Back pain

Immune System Disorders

• Hypersensitivity

Skin and Subcutaneous Tissue Disorders

• Dermatitis allergic
• Urticaria
• Injection site reaction  

Others

• Feeling cold

Factor IX complex

Coagulation analysis

Treatment should be initiated under the supervision of a physician experienced in the treatment of haemophilia and Factor IX deficiency

Factor IX is a vitamin-K dependent coagulation factor and it is synthesised in the liver. It is activated by factor XIa in the intrinsic coagulation pathway and by the factor VII/tissue factor complex in the extrinsic pathway. Activated factor IX, in combination with activated factor VIII, activates factor X. Activated factor X converts prothrombin into thrombin. Thrombin then converts fibrinogen into fibrin and a clot is formed.

Injection (per 5ml) 250iu

• Octanine F250 (Octapharma) originator

Injection (per 10ml) 500iu

• Haemosolvex Factor IX (National Bioproducts)
• Octanine F 500 (Octapharma) originator

Injection (per 10ml) 1000iu

• Octanine F1000 (Octapharma) originator