Haemophilia
What is the Factor deficient in Haemophilia A?
Factor VIII
What type of bleed is characterised by pain, swelling, warmth and reduced movement.
Joint bleed (haemarthrosis)
What is the most common bleeding disorder?
Von Willebrand Disorder
What medication should generally be avoided due to its effect on platelet function.
Aspirin
Only males can have haemophilia?
True or False
False
Around 20-30% of females with the haemophilia genetic change have bleeding symptoms and may have haemophilia
What is the Factor deficient in Haemophilia B?
Factor IX
Even without symptoms what type of injury requires immediate treatment with factor replacement and urgent assessment.
Head Injury
How many people in Australia have been diagnosed with a bleeding disorder?
a. 3543
b. 2934
c. 2010
d. more than 8400
More than 8400 people
What treatment strategy do we use to prevent bleeding episodes?
Prophylaxis
Which factor assists platelet adhesion and stabilises Factor VIII
Von Willebrand Factor
What are the early signs of a joint bleed?
Heat, tingling, bubbling or aura sensation.
These early sensations can occur before visible signs
Groin and or hip pain, lower back pain and inability to straighten the leg may indicate what serious bleed?
An iliopsoas bleed.
Iliopsoas muscle spans between the hip bone and femur.
Hip flexion, walking, climbing, running
second most common site of major muscle bleed
What does Christmas Disease have to do with Haemophilia B?
It is named after the first person diagnosed with the condition in 1952
What treatment product do we use for preventing bleeds in severe and moderate haemophilia A?
Emicizumab - bispecific monoclonal antibody administered subcutaneously.
Used to prevent bleeding in those with FVIII inhibitors
What percentage can a father pass on the haemophilia gene to his daughters?
100%
All his daughters will have the genetic change and are termed obligatory carriers
What term describes a joint that has bled three or more times within six months.
Target Joint
What life-threatening bleed can present with headache, vomiting, confusion or seizures.
An intracranial haemorrhage
When was von Willebrand disease first discovered?
VWD was discovered in 1925 by and named after Finnish doctor Erik von Willebrand.
What treatment product do we use for VWD for both prevention of bleeding and treatment.
Biostate - plasma derived FVIII/VWD concentrate.
Name the treatment products for Haemophilia A, Haemophilia B and FVII deficiency held in the WCH Transfusion Lab.
5 products
Haemophilia A: Eloctate & Adynovate (FVIII - EHL)
Haemophilia B: Alprolix & Idelvion ( rFIX - EHL)
FVII deficiency: Novoseven (also used for inhibitors)
What complication of haemophilia treatment makes standard factor replacement ineffective.
Inhibitors
Antibodies to FVIII/IX
These four bleeding sites require urgent specialist review due to their severity.
Intracranial, neck/throat, gastrointestinal and iliopsoas (retroperitoneal) bleeds
How many people with haemophilia have not yet been identified and diagnosed?
WFH estimates that over 75% of people living with haemophilia worldwide have not yet been diagnosed.
identification and diagnosis rates are even lower for women with bleeding disorders and for those with VWD and other rare bleeding disorders.
Which conditions can we use Desmopressin to prevent and treat bleeds?
Name 4
Mild Haemophilia A
VWD Type 1
VWD type 2 variants excluding 2B
Platelet Function Disorders
What treatment product is contraindicated in treating bleeding in patients with inhibitors on emicizumab?
FEIBA
Factor Eight Inhibitor Bypassing Agent - plasma derived product. (aPCC - activated prothrombin complex concentrate)
Alerts on Emergency Treatment cards
Can cause Thrombotic Microangiopathy and Thromboembolism