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Haemolytic Uraemic Syndrome

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One way that pre-reg differs to Univerisity: there is a patient at the end of every decision you make.  Now that is a scary thought!  Yes, we have had a lot of “practice” during our 4 year MPharm but it is a very different feel when you have a real patient in front of you and the information they are giving you is not from a spiel, carefully constructed to give you just the right amount of information to enable you to do a set task.  Oh no, they have their own stories, own way of telling you and sometimes can’t tell you anything at all because they are off the ward having a chest x-ray or are so short of breath that they can’t speak very much at all.  Nothing really prepares you for that.

 


So, after 3 weeks as a pre reg, I am more determined than ever to be the best pharmacist I can be and that starts with reading the policies and procedures for the trust in which you are working and getting stuck in with as much as you possibly can!

 


One area which needed further work on my part was antibiotics.  Looking at a patients drug history on the ward, seeing an antibiotic prescribed and having little idea what it actually covered was the wake up call I needed.  Luckily, there is an antibiotic formulary, outlining first, second and third line antibiotics for each indication along with route and dose – very handy!

 


After having a brief flick through, I noticed that gastroenteritis, caused by E.coli, should never be treated with antibiotics because of a rare syndrome called HUS, haemolytic uraemic syndrome.  It can cause haemolytic anaemia (destruction of red blood cells), acute kidney failure and low platelet count in affected patients, both adults and children.

 There are 2 main types of HUS; atypical (aHUS) which is caused by a genetic defect and results in chronic, uncontrolled complement activation, or Shiga-like toxin-producing E. coli HUS (STEC-HUS).  Both of these can cause endothelial damage, leukocyte activation, platelet activation, widespread inflammation and TMA (multiple thromboses in small blood vessels). There are no known treatments for HUS but the symptoms can be managed which allows the body to overcome this syndrome, hopefully successfully.  Antibiotics may stimulate further verotoxin production and so should never be used in a patient with HUS.  There are multiple tests that should be carried out to gauge a patients disease state; LDH (a marker of cellular damage), creatinine (if elevated, is indicative of kidney injury) and platelets, which are commonly low.  In patients with aHUS, a monoclonal antibody has been trialed and has shown to be of some benefit.  Eculizumab can block the overactive complement system.  It does this by inhibiting the terminal complement activation at C5 protein which in turn reduces haemolysis and thrombotic microangiopathy (TMA).  At £3150 per vial it is not cheap, but can be used under specialist supervision when a patient cannot overcome aHUS without intervention.  It is reserved as a last resort and has shown to be of huge benefit, especially in children and may be rolled out further if more research can be done into its use.


HUS is only something I stumbled upon when looking through the antibiotic formulary and hopefully something that I will never see in my career but anything that looks at future uses for drugs and expands my knowledge is just fine by me.  After all, we never stop learning and this nugget of information may be of use in some medical based pub quiz…..…surely?!

 

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