Hi I heard your recent vaccination conversation with someone about deciding whether to vaccinate or not when a predisposition to aHUS is known.
Yes Hi, I have had people tell me since how because of their experience they are very much against vaccinations, in particular the COVID one. Also others who had the infection and became a victim of aHUS when a vaccination would have helped. Neither knowing about it before it happened to them.
That is my experience too I did not know about aHUS until my three-year-old daughter recently had a COVID infection. It triggered aHUS
Sorry to hear that.
Thank you but the experience has made me think as a health care worker and to look deeper into the matter.
And what are your thoughts now?
Firstly, that if my child had not been triggered by this infection then another later in her life might have.
Yes most patients face many triggering hits without onset and then one comes along which does. Go figure no one knows why that is.
Yes I also thought why didn’t it present earlier particularly at the time of her birth.
Why then?
Because I had a COVID infection at around the same time. I was symptomatic when I gave birth.
So, you too faced a simultaneous double hit which if you were predisposed could have brought it on but it didn’t.
Yes but although the COVID virus can rarely cross the placenta, and no checks showed that my child did have an infection, neither did it trigger aHUS in her. Maybe an undeveloped complement system did not react. It could be a year or so before complement is fully developed.
We don’t know the state of their complement development, but new born children can have aHUS within weeks.
Or it could be, because of my job, I had to have COVID vaccinations , two by then, without reaction. Although I still had an infection with symptoms at the time leading up to delivery, my vaccinations’ IgG antibodies probably helped protect her.
Very likely as it would from your previous vaccinations or other diseases you have had through your life, but these would only be a transitory protection for her.
She had a COVID infection at 3 years of age then it triggered aHUS. She was relying on her own immune systems. Complement inhibitor treatment worked. It was more than a self-limiting infection TMA.
So, was she genetically predisposed?
We don’t know yet testing is in progress, here it could take three months to get the results. But even these don’t confirm an aHUS diagnosis. Like others who have what is a non-genetic complement mediated TMA. She will still be treated.
Indeed, something is not right in her complement if an inhibitor works. But discontinuation when there is no infection effect is more likely not to result in relapse.
But it is very distressing.
It is.
She will be getting all her vaccinations. As a family we will also be too. We have been lax. I have had three vaccinations then stopped.
And it is likely that you would have given her meningococcal vaccination much thought too given it was needed to have access to a complement inhibitor, eculizumab. Now on a complement inhibitor she is not in an uncontrolled complement activated state. Though immunosuppressed it might take longer for vaccinations to be effective. Though not as long as it is for aHUS patients with kidney transplants when vaccinated who are doubly immunosuppressed.
Yes we have seen what a meningococcal infection can do another child we know. We have worked out that the timing of the vaccination while on eculizumab is important too. Up to seven days before vaccination is better for her than the day before infusion which she had with her first vaccination.
Personalising these things is important. But am I right that so far no one else in your family has had COVID and each of them including you, pregnancy being the most common female trigger, have experienced some of the lifetime hits known to trigger aHUS.
The family is being tested. So that is an unknown variable for now.
Moving away from the genetics do you think COVID is a strong trigger.
I do; there is something about it.
Yes before vaccinations and all that, COVID was an extreme killer. We have to remember that for many affected in the pandemic it was not aHUS that killed them. Some serious stuff was going on in their lungs. Complement played a part in that, but when it lost the battle for the linings of the lungs, which not only made breathing difficult but released pneumoniae and sepsis into the blood stream, deadly killers. We should take that into consideration too however bad aHUS is, that did happen to many people who did not make it.
I also think it for each and every one to choose the right path for themselves. I can understand from experience you can get stuck into an endless ruminative trauma loops, questioning the endless hypotheticals how many more years we could have had without the burden of aHUS and her going through childhood if we had NOT gotten the vaccine. This is an impossible question which drives the brain crazy as there are limitless possible outcomes- it could have been several years or just several weeks that we would have saved her knowing now what the multitude of infections and potential triggers might bring to our life.
Yes, the risks and benefits evaluation can do that. It is not just a case of vaccinations trigger aHUS and based on that recommend no one should have them. We need to know what if anything is different about those who are triggered by it and those who aren’t. It needs more explanation and evidence. Other non complement diseases like TTP are triggered too. You could have aHUS, but you do not want full blown COVID. But you might not get COVID, but that COVID hit may not be one. You follow the thinking like a line on a Mobius Strip ( see article image)
But I am aware that I may feel differently if it was the COVID vaccination and not the COVID infection which triggered aHUS in me or my daughter. We are also lucky that at this stage my daughter most likely and fortunately does not have evidence of significant permanent kidney damage from her first aHUS episode. If this were the case, I would feel more strongly about those who do not get vaccinated.
It is a matter of where it is perceived from.
Undoubtedly, I think that in a clinical, emotion stripped appraisal of the current evidence, – the evidence is stacked in favor of still getting the COVID vaccination (unless other intolerable side effects) in the setting of true genetic aHUS and active protection from the risks of relapse while on complement inhibitor treatment.
It is the right thing to do in your mind. You are comfortable with that.
Yes but ultimately the individual and/or parent needs to be comfortable with the risk/benefit of their choice. I have had the COVID vaccine three times with no significant side effects, and no aHUS being triggered in myself. Again, I might feel differently about the COVID vaccine if it had been an optional vaccine that I had chosen for her and that it contributed to permanent kidney damage for her at the age of three. Then it would be guilt and the endless “what if we had not got her vaccinated and avoided the different scenarios about her new childhood”.
It can be like the so-called Russian roulette, what if the next chamber has the bullet. But more like a chance from 150 chambers for the vaccine trigger and six for the infection trigger . Play it long enough and you will find out.
OK so we have covered many more perspectives on the vaccine/infection decision, here let me summarise them.
| Naïve to aHUS | Experienced aHUS |
| Known Genetic predisposition | Not known or not predisposed |
| Vaccinated before | Not Vaccinated ever |
| Infected | Not infected |
| Discontinued complement inhibitor | Considering discontinuing |
| complement inhibitor never needed | On complement inhibitor |
| With a family | Without a family |
| No kidney transplant | Kidney transplant |
| COVID Infection TMA version | Complement mediated TMA version |
| COVID still an extreme killer with other severe consequences, including sepsis | COVID is now mild illness for the vast majority |
With the various permutations it is by no means a binary decision.
Absolutely you can say that about it.
Article No. 797
