Emergency Referrals

How to refer to the
national AHUS service?

If you suspect aHUS:

Call the Newcastle upon Tyne Hospitals switchboard on 0191 2336161 and ask for the on-call consultant for aHUS.

Download our referral [diagnostic checklist] and send to the on-call consultant following discussion.

We will also advise which sample request forms to use on our forms page.

If we approve the use of eculizumab – all the necessary instructions to obtain eculizumab will be shared in an email and are available here.

Forms Page

Obtaining Eculizumab

Why refer to the National aHUS Service?

The National aHUS Service offers a complete diagnostic toolkit to assess a potential new case of aHUS. This helps to rapidly and reliably exclude ADAMTS13 deficiency (TTP), STEC-HUS and other secondary causes of aHUS.

The National aHUS Service is then able to offer treatment to patients who are likely to have aHUS as early as clinically appropriate to maximise the chances of recovery of renal function.

Differentiating ADAMTS13 deficiency (and other causes of HUS)

When aHUS is suspected, the first critical diagnostic step is to exclude ADAMTS13 deficiency. We recognise the importance of prompt use of plasma exchange until ADAMTS13 deficiency has been excluded. For further guidance see https://onlinelibrary.wiley.com/doi/full/10.1111/j.1365-2141.2012.09167.x

We recommend all samples relating to the possible diagnosis of ADAMTS13deficiency, STEC-HUS and complement-mediated aHUS are taken prior to plasma exchange. We therefore encourage EARLY REFERRAL to the National aHUS Service to access our diagnostic toolkit (that includes 7-days a weekADAMTS13 testing) even before (though not delaying) ADAMTS13 testing and initial plasma exchange

Next: Differentiating ADAMTS13 deficiency (and other causes of HUS)

Timing of treatment with eculizumab

With respect to the timing of eculizumab in a possible case of aHUS, and prior to the exclusion of ADAMTS13 deficiency, our recommendations are in accordance with KDIGO guidelines:

In adults, we recommend that plasma therapy is undertaken until ADAMTS13 deficiency has been reliably excluded.
In children, it may be possible to commence eculizumab prior to the availability of the ADAMTS13 activity result.
In all cases, early referral to the National aHUS Service allows timely clinical discussion, investigations and decision making.

Please note: The decision to initiate Eculizumab (following exclusion of ADAMTS13 deficiency) may be made prior to the availability of diagnostic tests that would support a diagnosis of STEC-HUS or a secondary TMA.

A decision to await additional diagnostic tests before commencing eculizumab will depend on the clinical presentation with the ultimate aim to maximise clinical efficacy in patients who subsequently are confirmed to have primary complement mediated aHUS.

There is currently no evidence as to the efficacy of Eculizumab in STEC–HUS or secondary TMAs and consequently NHS England do not fund its use in this situation. Therefore in some cases where initial doses of eculizumab have been given, additional doses are not authorised once confirmation of an alternative diagnosis has been made.

Prior to the approval
of Eculizumab

As part of the assessment and approval process for funding of Eculizumab in a potential new case of aHUS, we will require a diagnostic checklist and a signed shared care protocol. Once the patient has been referred, complement profile testing and ADAMTS13 activity can be accessed from our combined aHUS lab diagnostics service in Newcastle.

Ideally all bloods relating to the possible diagnosis of ADAMTS13 deficiency, STEC and complement-mediated aHUS should be taken prior to plasma exchange.

Please note that our assay for ADAMTS13 activity testing is available 7 days a week allowing minimal delay to exclude ADAMTS13 deficiency prior to commencing eculizumab.

More information

Please note that our assay for ADAMTS13 activity testing is available 7 days a week allowing minimal delay to exclude ADAMTS13 deficiency prior to commencing eculizumab.

Additionally, STEC testing can be accessed.

Please note that as of October 2017, approval for funding of Eculizumab will only be possible via Blueteq as mandated by NHS England