Emergency Referrals

Emergency Referrals

We recommend that all patients with a potential new diagnosis of aHUS are offered treatment with eculizumab. The earlier treatment with eculizumab is initiated the greater the chance of recovery of renal function. For this reason we recommend that the only investigation listed on the diagnostic checklist that must be available before the onset of treatment with eculizumab is the ADAMTS13 activity. Until this is available we recommend that plasma therapy is undertaken. Provided that the ADAMTS13 activity is greater than 10% we would then recommend that treatment with eculizumab be started.

If you have a patient with a potential new diagnosis of aHUS, please call the Newcastle upon Tyne Hospitals switchboard on 0191 2336161 and ask for the on-call consultant for aHUS.

Please note: There is currently no evidence as to the efficacy of Eculizumab in secondary TMAs or STEC–HUS and consequently NHS England do not fund its use in this situation. The decision to initiate Eculizumab may be made prior to the availability of diagnostic tests to maximise clinical efficacy in patients who subsequently are confirmed to have primary complement mediated aHUS. An alternative diagnosis will necessitate withdrawal.

 

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. In addition, clinicians can request complement profile testing and ADAMTS13 activity from our combined aHUS lab diagnostics service in Newcastle.

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

 

Diagnostic checklist for aHUS (download here)

A renal TMA can be the presenting feature in a number of other diseases and these should be excluded before a firm diagnosis of aHUS can be made. Most tests in the diagnostic checklist can be performed at the referring centre. An important exception is testing for STEC-HUS that will require sending of samples to a national reference laboratory in Colindale.

 

Shared care protocol for Eculizumab in aHUS (download here)

Responsibility for the care of patients receiving eculizumab is shared between the National aHUS service and the referring consultant. The responsibilities of each are outlined in the shared care protocol. All clinicians must agree to the shared care protocol before eculizumab can be authorised by NHS England.

 

Complement profile testing and ADAMTS13 measurement

Download for Adult / Download for Child

We have established a combined aHUS lab diagnostics service in Newcastle upon Tyne Hospitals that encompasses a comprehensive genetic and immunological evaluation of the complement system (that includes ADAMTS13 activity) to facilitate diagnosis of aHUS and exclusion of TTP.

Full instructions for sampling and transporting to the combined aHUS lab diagnostic services at in Newcastle upon Tyne Hospitals are on the request form. ALL tests are paid for by NHS England as part of the diagnostic and follow-up pathways in patients referred to the national aHUS service for consideration of Eculizumab.

 

Following approval of Eculizumab

Key information at time of initial commencement of Eculizumab has been summarised.

 

Before Eculizumab can be given

  • Vaccinate with a tetravalent (ACWY) vaccine and Bexsero (against serotype B). 

  • Send oropharyngeal swab to microbiology to exclude carriage of antibiotic resistant Meningococcus.

  • Prescribe prophylactic antibiotic for the duration of Eculizumab treatment.

 Full advice on reducing the risk of menigococcal infection is available in the here.

 

The dose of Eculizumab is different between adults and children

Full dosing regimens are available in the eculizumab dosage section. The initial dose only is summarised below.

 

Initial Dose of Eculizumab

Adult dosing schedule

900 mg via 25-45 minute IV infusion

Paediatric dosing schedule

Patient body weight 40 kg or more

900 mg via 25-45 minute IV infusion

Patient body weight 30 kg to less than 40 kg

600 mg via 1-4 hour IV infusion

Patient body weight 20 kg to less than 30 kg

600 mg via 1-4 hour IV infusion

Patient body weight 10 kg to less than 20 kg

600 mg via 1-4 hour IV infusion

Patient body weight 5 kg to less than 10 kg

300 mg via 1-4 hour IV infusion