An overview based on a contemporary clinical question: eculizumab and paroxysmal nocutrnal haemoglobinuria

  • introduce a difficult clinical issue
  • formulate a treatment question
  • consider what evidence we need to answer our quesiton
  • Look at some EBP resources
  • do a systematic search
  • resources to help with clinical appraisal

PNH rare acquired haemolytic anamia stemn cell mutation cauding a lack of compleement inhibitor on RBCs

  • haemolutic anaemia
  • free hg in blood

Eculizumab monocloncal anitbody bind and inhibits complement activation effects prevent haeolusis and haemolutic anameia

but susceptible to infection especially meningococcal

current reccomensation is to give it

grad 1b reccomentation

grade 1 is strong recommensation and that the goverment believes that if you follow it you will do more harm than not

grade b means best estimates of the critical benefits and risks come from RCTs with important limitations

why did pharmac turn it down.

they daid that most evidence was from observational studies.

5 a’s of evidence based practive

tools for finding information

there was a lack of evidence that it prevented thrombosis and therefore did not make financial sense as it was being looked to for the purpose of anti thrombotic