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Coagulation factors

Coagulation factors are proteins, which in the blood, cause clotting. The factors are manufactured either from human blood (plasma derived) or genetic engineering (recombinant).

Once administered the products stay in the blood for a relatively short time (half life) thus they are given frequently when bleeds occur or when a child is having surgery or acute bleeding (Rationales 1 and 2).

Preparation - child and family

Inform child and family of the following (Rationale 3):

  • that a coagulation factor is necessary
  • the reason for the infusion
  • what it entails
  • the likely duration of the procedure

Ensure the following pre-transfusion observations of the child are recorded (Rationales 4, 5 and 6):

  • temperature
  • respiration rate
  • heart rate
  • blood pressure

Ensure venous access has been established (Rationale 7).

This should be according to intravenous transfusion guidelines.

Preparation - prescription

Coagulation factors must not be administered unless prescribed on the child’s prescription chart (Rationale 8).

Ensure the coagulation factor is prescribed according to the Drug Policy (Rationales 8, 9 and 10).

This should be in international units or micrograms.

Ensure you have a prescription or instructions for what should happen after the transfusion (Rationales 11 and 12).

If administering an intravenous pre-medication of an anti-histamine and steroid do so prior to commencing infusion (Rationale 13).

Preparation - injection/infusion

Planned administration

The coagulation factors should be available in the drug refrigerator on the ward (Rationale 14).

Emergency administration

The coagulation factors can be collected from the refrigerator in the Haemophilia Centre.

The person collecting the coagulation factors from the Haemophilia Centre must record their removal in the treatment book (Rationale 15).

Check the child’s details are the same in the treatment book & their health care records ie (Rationale 16):

  • hospital number
  • first name and family name
  • date of birth

Gather the equipment required to administer an intravenous infusion or injection (as appropriate) (Rationale 17).

Coagulation factors must not be filtered (Rationale 18).

Prepare injection/infusion using standard (universal) precautions & an aseptic non-touch technique (ANTT) (Rationale 19).

Infuse/inject product - general

Check the child’s name band and prescription according to the Drug Policy (Rationale 16).

Inject/infuse product according to prescription according to the relevant intravenous therapy guidelines using an aseptic non-touch technique (Rationales 9 and 19).

Infuse/inject product - observations and recordings

Record the following in the child’s health care records:

  • Date and time of starting administration (Rationale 20).
  • Sign that you are responsible for its administration (Rationale 21).

Establish the frequency and type of observations that the child may require during the transfusion according to their clinical condition (Rationale 22).

Observe child for minor reactions to coagulation factor eg (Rationale 23):

  • sweating
  • mottled appearance
  • dizziness
  • rash
  • redness on chest or flushing
  • fever
  • tachycardia
  • nausea
  • chills

Observe child for major reactions to coagulation factor eg (Rationale 23):

  • rigor
  • laboured breathing/wheezing
  • chest pain
  • back or loin pain/darkened urine
  • loss of consciousness
  • sudden collapse

Infuse/inject product - reaction management

If the reaction is unexpected:

  • Stop transfusion (Rationale 24).
  • Infuse 0.9% sodium chloride (Rationale 25).
  • Obtain medical assistance (Rationale 26).
  • Record incident in child’s health care records (Rationale 20).
  • Complete an incident report form. 
  • Continue administration when possible.

If child had potential for a reaction:

  • Stop transfusion.
  • Infuse 0.9% sodium chloride (Rationale 25). 
  • Give prescribed steroid and anti-histamine (Rationale 24).
  • Continue administration when child has recovered.
  • Inform doctor.
  • Record in child’s health care records (Rationale 20).
  • Complete an incident report form. 

If a severe reaction occurs:

  • Stop transfusion immediately (Rationale 27).
  • Infuse 0.9% sodium chloride (Rationale 25).
  • Seek urgent medical assistance. 
  • Give steroid and anti-histamine if prescribed (Rationale 24).
  • Do not recommence administration.
  • Record incident in child’s records.
  • Complete an incident report form.

Completing infusion

If the prescription is complete: 

  • Dispose of all used equipment according to the Waste Policy.  Do not put into sewage/water system (Rationales 8 and 28).
  • Wash and dry hands (Rationale 19)
  • If large volumes of coagulation factors are required, inform the Haemophilia Centre (Rationale 29).

Appendix 1 - Coagulation factors

 Coagulation factor
 Rationale for use
 

RECOMBINANT FACTOR VIII

KogenateBayer
Advate
Helixate
ReFacto AF
Gena-03*, A-Long*
* = in clinical trials

Available in 250, 500, 1000, 1500, 2000 and 3000 iu vials.

For use by children with haemophilia A (Classical haemophilia/factor VIII deficiency).


Recombinant technology reduces exposure to human blood borne infections, both those that are currently known, eg hepatitis, HIV and potential diseases, eg Creuzfelt Jacob Disease.
 

RECOMBINANT FACTOR IX
BeneFix
B-Long*, Inspiration*
* = in clinical trials

Available in 250, 500, 1000 and 2000 iu vials.

For use by children with haemophilia B (Christmas disease/factor IX deficiency).


Recombinant technology reduces exposure to human blood borne infections, both those that are currently known, eg hepatitis, HIV and potential diseases, eg Creuzfelt Jacob Disease.
 

RECOMBINANT FACTOR VIIa:
NovoSeven

Available in 1, 2, and 5 mg vials.

For use by children who have developed antibodies to Factor VIII or IX, or those with severe platelet disorders, and rarely by those with intractable bleeding.

It acts by bypassing the factor VIII or IX activation in the clotting cascade.

 

Recombinant technology reduces exposure to human blood borne infections, both those that are currently known, eg hepatitis, HIV and potential diseases, eg Creuzfelt Jacob Disease.

Can also be used in congenital factor VII deficiency but has a very short half life and requires frequent administration.
 

Plasma Derived Factor VIII
Haemate P
Wilate

Available in 500 and 1000iu vials.

Contains high levels of von-Willebrands factor.


The presence of von-Willebrands factor in high concentrations makes this an ideal product for treatment of children with von-Willebrands disease, for whom there is no recombinant product available.
 

Plasma Derived Factor VIII
Fandhi
Octanate

8Y
Optivate

Available in 250, 500, 1000 and 1500 iu vials.

For treatment of Haemophilia A.

Inexpensive when compared to recombinant products.

 

As effective at preventing bleeding as recombinant products but risks exposure to blood borne infection.

Is used in children with intractable inhibitors where the recombinant FVIII has failed. Is thought that the von-Willebrand factor makes the factor VIII more stable in the blood.

Used for self-funding (non NHS eligible) overseas children only.

8Y is used (off label) for children with congenital thrombotic thrombocytopenia purpura (TTP) as it contains the missing cleaving protein that they require.
 

Factor XIII
Fibrogrammin
rFXIII* (* = clinical trial use only)

Available in 250 and 1250 iu vials.

Used for children with congenital factor XIII deficiency.

 

Plasma derived and heat-treated to reduce risk of viral exposure.

Recombinant product available for use by children in clinical trial only.

Factor XI

Available in 500iu vials only.  (The vial size may vary by 20%).

For use by children with severe congenital factor XI deficiency and for those with liver disease.


Plasma derived & heat-treated to reduce risk of viral exposure. Factor XI is thrombogenic therefore must be given in small closely monitored doses. It is more usual to give virally inactivated FFP (Octaplas) – see FFP guidelines.


Fibrinogen Concentrate
Hemocomplettan/Riastap

Available in 1G vials only.

For use by children with congenital hypofibrinogenaemia as well as children with consumptive disorders, eg histiocytosis/DIC.

 

Plasma derived and heat-treated to reduce risk of viral exposure.

No recombinant product available.


Prothrombin Complex
Octaplex

Availabile in 250 and 500iu vials

Used for children with coagulation factor II or X deficiency as well as Warfarin reversal.

Factor X concentrate available for use by children with factor X deficiency as part of clinical trial only.

 

Plasma derived and heat-treated to reduce risk of viral exposure.

Contains factors II, VII, IX X.

No other source of factors II or X.
 

AntiThrombin
Kybernin P

Available in 500iu vials.

Used for children with congenital or acquired antithrombin deficiency disease, eg veno occlusive disease following bone marrow transplant.


Plasma derived and heat-treated to reduce risk of viral exposure.

Recombinant product currently in clinical trials.


Rationale

Rationale 1: Children with inherited or acquired bleeding disorders are treated with coagulation factors to correct their underlying bleeding disorder.
Rationale 2: In children with severe bleeding disorders these are given prophylactically, usually three times per week, to prevent bleeding occurring.
Rationale 3: To obtain "consent".
Rationale 4: To establish the baseline parameters for a child.
Rationale 5: To rule out pre-existing diseases processes if a reaction is suspected.
Rationale 6: These could alter in a reaction.
Rationale 7: To enable infusion to commence.
Rationale 8: To maintain Hospital Policy.
Rationale 9: To prevent infusion errors.
Rationale 10: To reduce wastage.
Rationale 11: Doses may need to be repeated.
Rationale 12: To ensure planning takes place.
Rationale 13: To prevent a reaction to the blood product in children who have suffered reactions in the past.
Rationale 14: Coagulation factors must be stored at 4-8°C.
Rationale 15: To ensure product tracing can take place.
Rationale 16: To ensure the correct product is given to the correct child.
Rationale 17: Coagulation factors are administered by intravenous injection except for Fibrinogen which needs to be infused over 20-30 minutes.
Rationale 18: Coagulation factors will be removed by filters leading to the administration of a suboptimal dose and result in bleeding.
Rationale 19: To minimise the risk of infection.
Rationale 20: To ensure accuracy of records.
Rationale 21: To determine accountability for the transfusion.
Rationale 22: To monitor for potential complications.
Rationale 23: To ensure safety of child.
Rationale 24: To stop reaction.
Rationale 25: To maintain patency of IV access device.
Rationale 26: To obtain prescription for steroid and anti-histamine.
Rationale 27: To minimise impact from reaction.
Rationale 28: To reduce risk of infection by safe disposal.
Rationale 29: To assist ordering and stock delivery to ward areas.

Further reading

Reference 1:
UKHCDO (1997) Guidelines on the therapeutic products to treat haemophilia and other hereditary coagulation disorders. Haemophilia 3: 63-77.

Reference 2:
Bolton-Maggs PH, Colvin BT, Satchi BT, Lee CA, Lucas GS (1994) Thrombogenic potential of factor XI concentrate. Lancet 344 (8924): 748-9.

Reference 3:
Khair K, Richardson J, Susman-Shaw A for Haemophilia Nurse Association (1995) Standards of care for haemophilia nursing. London, Royal College of Nursing

Reference 4:
Bedford M, Elliott D, Khair K, Mackett N for Haemophilia Nurse Association (2002) A competency framework for nurses caring for people with haemophilia and related bleeding disorders. London, Royal College of Nursing

Reference 5:
Department of Health (1993) Provision of haemophilia treatment and care. Health Service Guidelines (HSG) 30: pages not given.

Document control information

Lead author(s)
Kate Khair, Nurse Consultant, Haemophilia

Document owner
Kate Khair, Nurse Consultant, Haemophilia

Approved by
Clinical Practice Committee

First introduced: 30 January 2004
Date approved:
20 July 2011
Review schedule:
Two years
Next review:
20 July 2013
Document version:
3.0
Replaces version:
2.0