Any HUVS suffers with experience of being treated with C1 inhibitor - e.g. Cetor, Berinert Ruconest?

[Rofa]

I have suffered from HUVS for over 5 years with attacks generally occurring every 3 months - generally severe or very severe. I have now been treated for about two years with Cetor and subsequently Ruconest. Attack interval is about 8 months and attacks have been extremely mild. Treatment is not in the UK.

[PatriciaAnn]

Hi Rofa

I don't have HUVs but a few of our members do, so hopefully they will be along soon to comment from their personal view point.

In the meantime, have you seen the Vasculitis UK webpage about Urticarial Vasculitis? vasculitis.org.uk/about-vas...

I've looked at the page and the drugs you mentioned are not noted there. I'm sure those with HUVs will be interested in hearing about different treatments which have worked for others. I think it will be interesting to know where you receive your treatment.

Kindest regard. PatriciaAnn

[RichardE Volunteer]

Hi.

I have HUV and like Pat am not familliar with the drugs you mention. As you probably know HUV is a pretty rare form of Vasculitis so it's exciting to hear from you ! But also exciting to hear that you are having a treatment that we are unfamilliar with Can I ask what country you live in / are treated in please ?

Reading up about the drugs you mention I see that they are described as C1 esterase inhibitors and normally prescribed as a treatment for hereditary angioedema (HAE). Can I ask if you have also been diagnosed with this or do you know if the drugs are being used "off label" as a treatment for angiodema caused by your HUVS ?

It appears to me that the drugs are normally prescribed as a treatment of acute attacks of angioedema. Can I also ask if you administer yourself in order to prevent an oncoming attack or whether you have the treatment in some regular form to prevent attacks starting in the first place ?

Sorry to ask so many questions but this is so interesting and I know of at least one other HUVS sufferer who might benefit from your knowledge.

Many thanks,

Richard.

[Rofa in reply to RichardE]

Sorry Richard and Pat for the delay in replying. I have not been diagnosed with HAE but my consultant for some reason just decided to try Cetor when I was having a bad attack and it was amazing how quickly the attack subsided. We moved to Ruconest because there was a suspicion I had developed antibodies to Cetor (human blood product). I currently receive one injection (takes 5 mins) twice per week as a preventative measure - and when the hospital is closed my wife administers. She was a nurse and a small needle has to get into a vein - there is a self admin kit - so it is certainly possible to do it oneself with a little training. It is a very expensive medication and the chance of getting it through normal channels (NICE) is I suppose remote. I am fortunate in having financing for this privately - and what works for me may not work for others - it is to say the least not a very well understood condition - and a clinical trial on one person is certainly not possible! However even if you can't get it as a prophylactic Richard, you may persuade someone to try it during an attack especially if your attacks are particularly severe - teaching hospital with appropriate research program maybe??

[Rofa in reply to RichardE]

Sorry Richard and Pat for the delay in replying. I have not been diagnosed with HAE but my consultant for some reason just decided to try Cetor when I was having a bad attack and it was amazing how quickly the attack subsided. We moved to Ruconest because there was a suspicion I had developed antibodies to Cetor (human blood product). I currently receive one injection (takes 5 mins) twice per week as a preventative measure - and when the hospital is closed my wife administers. She was a nurse and a small needle has to get into a vein - there is a self admin kit - so it is certainly possible to do it oneself with a little training. It is a very expensive medication and the chance of getting it through normal channels (NICE) is I suppose remote. I am fortunate in having financing for this privately - and what works for me may not work for others - it is to say the least not a very well understood condition - and a clinical trial on one person is certainly not possible! However even if you can't get it as a prophylactic Richard, you may persuade someone to try it during an attack especially if your attacks are particularly severe - teaching hospital with appropriate research program maybe??

[Rofa]

Sorry for the delay in replying Pat and Richard. No I have not been diagnosed as having HAE. My consultant who had no experience of HUVS but was very experienced in HAE decided to try it when I was having a severe attack - it was amazing how quickly the attack subsided. I now receive one injection twice per week either in hospital or at home - a needle has to get into a vein. I am fortunate in having financing for this and the chances of getting it through NICE must be remote - unless you can maybe swing a change in diagnosis. And what works for me won't necessarily work for everyone. But if your attacks are severe maybe you can persuade your consultant to try it when one starts??? And if it does work then maybe that is a good reason to modify the diagnosis

[Rofa]

Sorry for the delay in replying Pat and Richard. No I have not been diagnosed as having HAE. My consultant who had no experience of HUVS but was very experienced in HAE decided to try it when I was having a severe attack - it was amazing how quickly the attack subsided. I now receive one injection twice per week either in hospital or at home - a needle has to get into a vein. I am fortunate in having financing for this and the chances of getting it through NICE must be remote - unless you can maybe swing a change in diagnosis. And what works for me won't necessarily work for everyone. But if your attacks are severe maybe you can persuade your consultant to try it when one starts??? And if it does work then maybe that is a good reason to modify the diagnosis

[RichardE Volunteer]

Hi Rofa, thanks for the update. And sorry for my delay in replying, I lost track of this post whilst moving house over Christmas !

I'm personally in a good drug controlled remission but I do know of another HUVS sufferer who is struggling with frequent angiodema attacks. I'll mention your treatment and see if she's heard of it.

Richard.