Term 4 week 7 (w/c 15/10/12)
Lots of hits on the case story last week. We are pleased that we there has been a worthwhile discussion. We will update our new The Expert Says section as soon as we can.
This week looks a little more complex at the outset.
A 26 year old female has presented to Emergency Medicine with a vague story related to a fit. She has been brought in by her mother who she lives with. Her mother came home to find that she appeared a little confused and had wet herself.
Because we have a mixed crowd of followers you may chose to answer either question:
1 What other information do you want?
2. What specific actions will you take?u
Different specialties approach this kind of question in different ways. Assuming that the practical ABC assessment with IV access has been carried out and the patient is in a monitored bed we can now consider the history.
No previous fits. Generally well. Non smoker, social drinker.
Appendicectomy 6/12 previously
Mum says she hasn’t been right for months. Has been unwell non specifically with general pains which has been diagnosed as fibromyalgia for which she has been taking increasing doses of analgesia. Her mother says the pain had been particularly bad through the night for the last week and she has started taking her mothers oxycodone.
She had been lethargic in the morning and her mother had gone out to the shops. On return the patient was confused and had wet herself.
How do we move forward with this case? Do you want to give anticonvulsants?
Sadly we haven’t had too much tweeting on this case.
The results available reveal a normal CT head and relatively normal blood results.
Key numbers Hb 10.6 WCC 12 Na 132 K 4.0 U 7.0 Cr 76 Pa O2 10.0 on air pH 7.42 PCO2 3.9
Our patient has become more lucid and whilst she is still relatively confused she is complaining of generalised pains, worst in the abdomen, and appears a little breathless.
Time for a few guesses. What is the diagnosis that you should try to rule in or out?
It’s good for anaesthetists to have a think every now and again. Acute Porphyria is a fairly rare diagnosis that interests us a group because it is possible that it can arise as a result of our pharmacological interventions. The history is often vague and confusing and a high index of suspicion is needed in order to make the diagnosis. Local biochemistry labs can analyse blood and urine in order to make a diagnosis but further management needs to be directed by specialist centres. The presence of porphobilinogen in urine is diagnostic but it’s absence can be due to the effects of light on the container or even different types of Porphyria.
Assuming we have now got the correct diagnosis what are the key management principles?
The Expert Says
The first point of treatment is to remove the precipitant. In this case the precipitant may well have been the oxycodone which is known to be unsafe. The chronic grumbling history may reveal problems precipitated by the general anaesthesia for appendicectomy some months back. It is also possible that some intercurrent illness may be implicated and infection is a high possibility. Many thoughtful diagnoses from #gasclass brought attention to sepsis and that needs ruled out.
Feeding, glucose and fluids are important as supportive measures. However IV haem arginate is the definitive treatment which should lead to a rapid improvement if started early enough. Specialist help should be sought.
An excellent review of the implications of porphyria in anaesthesia critical care and pain medicine is in CEACCP (BJA) 12:3 June 2012
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