#paeds
See: [RCH haemophilia](https://www.rch.org.au/clinicalguide/guideline_index/Haemophilia/)
See also: [[Von Willebrand disease]]
> [!key points]
> Haemophilia A : Clotting Factor VIII deficiency
> Haemophilia B : clotting factor IX def
>
> **Treatment**
> clotting factor replacement (8 for A, 9 for B)
>
> **points**
> ==do not give IM injections==
>
# Severity
|severity| concentration of clotting factor % | features|
|---|---|---|
|mild | >5-40 | bleeding with major trauma and surgery |
|moderate| 1-5| can bleed after minor injury. can go into joints |
|severe| <1%| frequent bleeds common, into joints and muscles|
# Treatment
Rest
Ice
Compression bandage
Elevation
Splint
avoid NSAIDs
- Factor VIII >10% (mild cases) can be Tx with **DDAVP** → releases vWF → transports factor VIIII
- DDAVP dose is 0.3 μg/kg in 100 mL saline over no less than 45 minutes
- side effects: face flushing, headache
- tachyphylaxis develops after three or four doses
- antidiuretic properties can last 24 hours, can cause fluid retention and [[hyponatremia]]
- More severe cases need factor VIII replacement
# Treat bleeding
Minor bleed:
factors
Major bleed
more factors
# consequences
- compartment syndrome
- head bleed