Just over two years ago the alliance reported on an event in India where key aHUS clinicians met to discuss and agree how HUS/ aHUS could be managed there. That article can be seen here.
aHUS Patients were represented at the conference by the parents of Ananya whose died through lack of access to eculizumab. Annaya’s harrowing story featured in a blog by aHUS India which can be read clicking here.
This week the findings of that consensus conference have been published.
It reveals the chasm of treatment potential for aHUS between the different worlds that aHUS patients find themselves . Some times the wrong genes in the wrong place.
The full publication abstract can be seen here.
It can be seen that the conference was called because international treatment guidelines are now centred on eculizumab but access is limited in most developing countries including India. Specific guidelines crafted to India’s needs were required.
The situation in India is unclear the incidence and prevalence of HUS uncertain but one key difference is the extent of HUS resulting from anti-factor H antibodies in young children and adolescents.
The conference broke up into four working groups focusing on :
-Diagnosis
– Shiga toxin ( E. coli ) , pneumococcal and infection HUS
-aHUS
-Complement blockade
A classification and evaluations algorithm was developed to aid diagnosis.
Treatment protocols using Plasma exchange supportively for Shiga toxin HUS and therapeutically for aHUS.
Those with anti-factor H antibodies would be managed with immunosuppressive medications to reduce or eliminate the antibodies.
Eculizumab is needed for incomplete remission , when life is threatened, PEX complications or vein access , known inherited complement defects and following transplant re onset.
Capacity to undertake this would need attention. ( So does access to an affordable Complement Inhibitor.)
The conference’s conclusion was that the proposed guidelines were not only relevant to India but might be applicable to others in the developing world.
In many ways this sets the basis for hope for those in India affected by aHUS. Yet it is also something to build upon on to give hope to probably more that 50% of the world’s sufferers of aHUS, who live in developing countries.
Note: The featured image is of Aadhyan Faiswal an Indian aHUS Patient
