Ethics Case Study 2

A 3 year old girl was diagnosed with nephrotic syndrome secondary to FSGS. She was initially treated with steroids and Cytoxan later converted to cyclosporine A. Despite treatment she slowly deteriorated into ESRD and started dialysis. At 10 years of age, she underwent a deceased donor kidney transplantation and was managed with triple immunosuppressive protocol (CyA, MMF and steroids) without pre-transplant plasmapheresis (PP).

At the 14th post op day with creatinine levels dropping to 1.0mg/dL she developed proteinuria of 17g//24hr. and was placed on PP sessions 3 times/ week followed by Rituximab (single dose of 375mg/m2). The following months despite aggressive continuous sessions of PP maintaining her protein urine level < 10g/day she developed hypoalbuminemia (albumin levels 2.2g/dl) associated with severe leg edema, skin infection and convulsive disorder secondary to PRESS. At six months after transplant her mother requested to discontinue immunosuppressive treatment and she returned to dialysis.

Six years later she was fed up with dialysis and asked to be listed for a second transplant.

At this stage would you consider a second transplant using one of the parents who was a match donor knowing that this scenario might be associated with a high risk of recurrence?

We thought that we shouldn't offer live donation and listed her for a deceased donor transplantation. She had a PRA of 60% and waited almost 9 years until she received her second transplant. Rituximab was given as part of the induction protocol and PP was initiated at the second post-op day as a preventive measure. Despite these measures on post-op day 7 she developed again a high level proteinuria of 14g/24hr. She continued with PP sessions X3 for another two weeks followed by gradual reduction of the PP interval to once a week. Again, she presented with a full spectrum of complications secondary to severe proteinuria including anasarca, abdominal fluid collections requiring drainage to relieve abdominal tension and hypoalbuminemia.

Is there any benefit to continue PP and maintain the graft?

Should we give her a chance of a third transplant with our current inefficient treatment for recurrent FSGS?