Forms and Protocols

Forms

The following forms are for use following discussion of a case of suspected aHUS with the on-call consultant at the National aHUS Service.

Diagnostic Checklist (Referral Form) | Shared Care Protocol | aHUS Laboratory | Complement Genotyping | STEC Testing | Meningococcal Titres

 

Diagnostic Checklist (referral form)

Download diagnostic checklist 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. Many tests in the diagnostic checklist can be performed at the referring centre. Please use the forms below for complement profile testing / ADAMTS13 measurement and STEC testing as soon as aHUS is suspected and PRIOR to plasma exchange.

 

Shared Care Protocol

Download shared care protocol 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.

 

AHUS Laboratory: 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. Requests using these forms should be made following discussion with a consultant at the NRCTC and NOT if the patient has already (recently) received plasma products.

In certain situations, it may be more appropriate to use our complement genotyping form  – available below. If in doubt, please discuss with the on-call consultant.

 

Complement Genotyping Formclick here

 

STEC testing

Download forms for STEC testing here.

Testing for STEC-HUS is performed in the national reference laboratory in Colindale. This can now be requested via the NRCTC using the form above. Simply follow the sampling instructions and send to Colindale. STEC testing is 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.

 

Meningococcal Titres

Download forms for requesting Meningococcal titres here.

Patients receiving eculizumab must be vaccinated against meningococcal infection using the vaccines ACWY and Bexsero. As part of the monitoring of serological response, we recommend annual testing of ACWY titres. NB Due to the nature of the assay, B-titres cannot be tested whilst patients are receiving eculizumab. Our specialist aHUS nurses will usually be in touch to confirm the timing of these titres. Samples for testing should be requested using the following form, requesting measurement of ACWY titres. These tests are paid for by NHS England as part of the follow-up of patients receiving eculizumab within the National aHUS Service.

 

C3G referrals

If you have a case of recurrent C3G following renal transplantation and wish to refer to the National C3G service for consideration of eculizumab, please contact us on C3.glomerulopathy@nhs.net and complete the following C3G checklist.

For more details about the this policy (NHS England 16054/P) please follow the link here.

If you wish to discuss a case of C3 Glomerulopathy please contact the team at C3.glomerulopathy@nhs.net.

 

Protocols

Further details on the following and specific protocols currently used by the National Renal Complement Therapeutics Centre can be found here.

Dosing of eculizumab

Vaccination against meningococcal infection

Patient monitoring whilst on eculizumab

Monitoring following withdrawal / stopping of eculizumab

Recommendations for prophylactic eculizumab to prevent aHUS relapse in renal transplantation