Management

General measures

• If bleeding is likely, explain this to the parents
• If a significant bleed is a possibility benzodiazepines ± opioids should be readily available (see below) and the use of dark towels and blankets may be helpful in these circumstances
• If a tendency to bleed occurs in a girl who has started menstruating consider the continual use of the oral contraceptive pill to prevent menstruation occurring
• If coagulation is abnormal secondary to liver dysfunction, consider Vitamin K either orally (prophylaxis) or intravenously (acute treatment)

Medication

Bleeding gums

• Use soft toothbrush and consider gentle regular antibacterial mouthwash to prevent secondary infection
• If low platelets are a contributory factor and the symptom is very distressing consider platelet transfusion
• In some areas of the UK it is possible to give platelets at home, contact your local SPC or Haematology team to discuss this if appropriate

Tranexamic acid

Form

Tablets: 500mg
Injection: 100mg in 1mL, 5mL ampoules. Oral suspension only available as ‘special’

Dose (mouthwash)

Use undiluted preparation for injection to apply directly to bleeding point or dilute 1:1 for use as mouthwash/oral use.

Dose (oral)

1month - 12yr: 15- 25mg/kg (max 1.5g) 2-3 times a day.
>12yr: 1g t.d.s (max 4g daily)

Caution: reduce dose in renal failure; caution in haematuria because of the risk of clot retention.

Licence: licensed for use in children.

Vitamin K

Form

Tablets Konakion 10mg (Discontinued January 2010)
Injection: 1mg/0.5ml ampoules (can be given IM), Konakion MM® 10mg/mL (must not be given IM, give IV)

Dose (IM, IV bolus over 15-30mins) :

1 month - 18yr: 250- 300µg/kg stat (maximum 10mg)

Topical Adrenaline

Form

1:1000 solution

Small external bleeds: soak gauze, apply directly to bleeding point

Sorbsan dressing

Haemostatic dressing: apply directly to bleeding point

Platelet transfusion

Consider if bleeding is problematical and related to low platelet count in a child with a reasonable prognosis where transfusion would improve quality of life, or in any child where on balance the burden is outweighed by the benefit. In reality there often comes a point at which platelet transfusion is no longer helpful, but the decision to stop must be carefully discussed with the child & family, and the wider team of carers and professionals. It is helpful if the possibility of stopping transfusions is discussed when they are started/early on. Some areas in the UK have established protocols for platelet transfusions to be given to children in their homes.

Catastrophic haemorrhage

The fear of catastrophic haemorrhage is typically out of proportion of its actual likelihood, so we can often reassure the family.

Since most children with this die at home, buccal Midazolam is the drug of choice as it can be given very quickly without medical staff present and reduces the fear of the situation for the child.

Buccal Diamorphine can also be given for the associated breathlessness due to volume of blood loss - not because of pain.