“De Novo” aHUS is bad luck

It has been known for decades that what is called ” de novo ” aHUS can happen to a kidney donor who is predisposed to aHUS.

It is why those who are known to be predisposed to aHUS were not allowed to be a living donor for a family member needing a transplant.

The loss of their own kidney and subsequent “adjustments” of the remaining kidney can trigger off an episode of aHUS in the donor.

The decision to donate must be based on proper medical advice even to day when aHUS patients can access complement inhibitors.

Here is a story of a son getting “de novo” aHUS as a result of donating a kidney to his father, who had end stage kidney failure and was on haemodialysis.

It happened in New Zealand in the 2020s just a month ago. ( Read the full article HERE )

As always with media stories about aHUS there are some inaccuracies and some important facts are missing.

Although it is natural enough for the recipient to feel guilty when something happens to the donor this story shows how the two not helped by their health care providers. In more ways than one.

The article does not tell us what caused the father’s kidney failure but in a previous article states it was stated it was due to diabetes, something that happened in the family. Diabetes nephropathy is thought to have a link to complement. Did he have aHUS too?

That would have been the red flag to any clinician considering a kidney transplant donor.

It did not and would explain why a living donor of a family member was considered.

When the son is diagnosed with aHUS there is no mention of a genetic cause. No genetic test conformation. Only that aHUS is an autoimmune disease. It is not.

If the father is genetically predisposed to aHUS too then his transplant may trigger another episode of aHUS and he will lose the kidney graft. The article does say whether any genetic tests have been undertaken.

Although the son says he had plasma infusions, plasma therapy ( plasma exchange ) would temporarily provide some complement control . Also the son’s current campaign is to raise awareness of “plasma” donation not blood donation.

To compound their situation New Zealand health authorities do not fund eculizumab for aHUS . Its health authority declined to fund eculizumab for PNH in 2013 and Alexion consequently did not make a application for aHUS to be funded.

Nor has ravulizumab been considered which is even more cost effective.

It looks like the health authorities are open to an application as they say “we will be seeking advice from our Haematology Specialist Advisory Committee soon to gain a better understanding of the health needs and therapeutic options available for people with this condition. Depending on the advice that we receive, Pharmac may request that the relevant pharmaceutical suppliers submit a proposal with supporting evidence for funding consideration.”

If the father did have aHUS and had been treated with eculizumab he may not have needed a transplant and the son would not have needed to donate a kidney. Two lives blighted and two costs of the alternative treatment to eculizumab.

Or is it all a coincidence? The son has a “de novo” genetic pathogenicity to aHUS , never experienced in his family, and which was not revealed in the months of extensive tests of his suitability for being a donor, that caused his own “de novo” aHUS onset?.

That just would be very bad luck.

Better luck would be to access clinically effective treatment in his own country. Now that he needs it for only as long as he needs.

It would be a good day too if any Pharma with proven or “in trial” treatments for aHUS shows him the compassion that he showed for his father. And he gets the treatment he deserves.

Articled No. 546

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