We don’t get a lot of Southern European Pain and Suffering Intolerance Syndrome (S.E.P.S.I.S.) in Tasmania, but I saw a very severe case tonight in the ED, and I thought I would review some of the evidence on the subject, in light of the recent focus on the much less interesting condition of boring old infective sepsis…you know, Manny Rivers’ original 2001 NEJM Early Goal Directed Therapy study, the 2011 Jones et al. paper on lactate clearance from JAMA and the podcasts which are getting a bit of airplay on emcrit.org at the moment…all very dull…
Instead – S.E.P.S.I.S., A VAST TOPIC…HERE GOES!
Disappointingly, entering “s.e.p.s.i.s.” as a search term in google merely leads one to the usual suspects, ignoring the acronymic nature of “the other sepsis”. Things improve a little, however, if one enters the term: “the other sepsis” or indeed the longhand version: “Southern European pain and suffering intolerance syndrome.” With these searches, we find good old litfl.com‘s glimpse of the same subject by my old mate Peter Allely MB BS FCEM F.UCEM. Peter does not really go in detail into management of the condition, and the literature is lacking on a rigorous approach to emergency department/critical care of S.E.P.S.I.S.
With this in mind, it would seem that there is a potential role for an Early Goal Directed Therapy approach in severe S.E.P.S.I.S. as defined by M.A.D.A.M.S. (Mediterranean Area Demographic Associated Melodrama Syndrome) plus a proven or suspected source of Irritation (Often exacerbated by relocation to a primarily English-speaking region of the world, although there is some evidence that this is observer-bias). This definition is analogous to the concept of S.I.R.S. plus a source of infection in common-or-garden sepsis, such that:
S.E.P.S.I.S. = M.A.D.A.M.S. + SOURCE is analogous to SEPSIS = SIRS + SOURCE
Of particular note is that using systolic blood pressure or serum lactate to guide therapy in these patients adds nothing, therefore surrogate indicators of severity are required. Although case series data only are available, it would seem that a reasonable assessment of severity can be estimated by carefully counting and documenting the number of body regions in which the patient complains of severe pain, and multiplying this by the highest value for pain score stated by the patient (on the McGill pain index from 0-10 – if accompanied repeatedly by the phrase “doctor, I’m dying,” this value should be multiplied by the number of times said). The really useful thing about the number thus arrived at (the MADAM index) is that it is also inversely proportional to the probability of finding an actual organic pathology of significance that requires specific treatment. Clearly this requires further validation.
[(BODY REGIONS IN PAIN) x (PAIN SCOREmax)] x n = MADAM INDEX
= 1/(probability of significant organic pathology)
(where n = number of times “doctor I’m dying” is said, usually in a heavy accent with accompanying dyskinetic but symmetrical gesticulations of the upper limbs primarily involving shrugging of the shoulders and opposition of thumbs and forefingers)
Once again, analogous to infective sepsis care prior to the Rivers’ study in the NEJM, the lack of a rigorous and evidence based approach to S.E.P.S.I.S. means that these patients are not actively screened for and prioritised for early aggressive ED treatment, such that there is undoubtedly a subgroup who deteriorate during their admission and we would postulate that mortality rates are likely to be in the region of 20-30%. However, these numbers are mere speculation as this condition has not been well recognised and is not even available as a diagnostic option on most versions of EDIS, a widely used ED IT management system in Australian Emergency Departments.
Therefore, we propose a pilot study to retrospectively apply the diagnosis of S.E.P.S.I.S. to appropriate patients through a chart review of all patients presenting to EDs with vague symptoms and surnames suggestive of Mediterranean ethnicity. Mortality rates and outcome data can thus be gathered in order to assess the real extent of the issue, which we postulate to be one of the most common unheralded killers of first and second generation Mediterranean Australians in the world today. The second phase of this research will be to investigate for associations with poor outcome and produce a score (a la the rigorous and well-respected ABCD2 score in TIA/CVA) which would allow us to identify those at risk, followed by phase 3 which aims to construct an evidence based “bundle” of interventions which we hope will reduce the morbidity and mortality from this terrible affliction, and ease the pain and heartache of the families, carers, and most of all, the doctors of these unfortunate souls.