hemophelia
Terms
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- 2 most common forms of hemo
-
Factor XIII (hemo A, Classic)
Factor IX (hemo B, Christmas) - Basic defect of Hemo
-
Lack of antihemophilic factor (AHF)
less AHF, more severe the disease - define hemothorosis
- bleeding in the joints
- which joints are affected first
- ankles b/c of walking
- what labs associated with hemo?
-
Ptt, platelet, pt=normal
specific assay factor - goal of theraputic management
- control and prevent
- replace clotting factor, relieve pain, exercise, primary prophylaxis
- theraputic management
- what should you do rather than a fingerstick in theses kids to draw blood?
- venepuncture
- what is the best sport? what kind of sports can't they do?
- swimming, contact sports
- how should you give an IM
- sub q
- managment of bleed
-
#1 start factor
RICE, ROM not complete - what should they do before any type of dental work and after
- factor
- pooling of blood in the liver-can lead to shock
- sequestration crisis
- don't make RBS's
- aplastic crisis
- accelerated rate of RBC destruction
- hyperhemolytic crisis
- most frequent problem with SCA
- vaso-occlusive crisis give advil
- cold, infection, dehydration, stress, fatigue
- triggers for vaso-occlusive crisis
- what med should sca kids NEVER get?
- demerol
- dysnpea, pain, pneumonia like symptoms
- chest syndrome
- symmetrical swelling of hands and feet, very painful joints
- hand and foot syndrome
- increace fluids, give new blood, plenty of rest-conserve 02 do not undermedicate for pain, use heat
- crisis interventions
- pt taking INH should avoid what foods
- tryamine and histamine: red wine, aged cheese, soy sause, tuna, yeast extracts