Hello

In Belgium (and I suppose in other countries) patients with a weaker immunity will have the oportunity to receive a 3th injection, a so called booster vaccination.

On this subject I have 2 questions.

1 I wonder whether SCLS patients can be considered as having a weaker immunity. In Belgium a list will be made up but SCLS is not very known as there are only a few patients

2. Is it useful for us and is there any danger? I had no adverse reaction on the 2 vaccinations I received in the past.

Thank you for your reactions and maybe some people already have experiences.

Hans,

Thank you for bringing up this issue of Covid vaccine booster shots, because priority patients in Israel and the United States already have, and in France and Germany will soon (September) have, the luxury of receiving an extra vaccination to prevent Covid-19.  And as you say, other European countries like the United Kingdom and possibly Belgium are likely to follow soon after.

I was going to bring up the matter today anyway, because yesterday I received my Covid booster shot -- the third Moderna vaccination so far this year, having received the first in January and the second in February.  I may well be the first SCLS patient in the world, or the first in our RareShare community, to get it, so let me now answer your two very good questions, slightly restated.

1) If Covid booster shots become available to immunocompromised patients, do SCLS patients qualify as such?  This is a highly relevant question because in the United States immunocompromised patients are on top of the current priority list, see https://www.cdc.gov/coronavirus/2019-ncov/vaccines/recommendations/immuno.html  And, sure enough, SCLS is not listed -- and it will very likely not be listed in any forthcoming list, anywhere. 

Therefore, I consulted with several knowledgeable physicians about the matter and in summary I was told as follows: On the one hand, SCLS patients typically have a normal complement of the usually measured immunities, but on the other, there is something in the immunoglobulin "chicken soup" that is not being measured and yet we know that this unidentified ingredient is remarkably effective in preventing episodes of SCLS.  Consequently, it can be concluded that SCLS patients are immunocompromised, which is why most receive immunoglobulin infusions on a regularly scheduled basis. 

In sum, if an SCLS patient who is on a regular therapy of immunoglobulin wishes to receive the Covid vaccine booster, and is asked whether they are immune-deficient or immune-compromised, he/she may answer "Yes" or may answer more factually "I regularly receive immunoglobulins for my illness."

2) Can Covid vaccines trigger episodes of SCLS?  This is a highly relevant question because the answer is Yes, there are reported cases where individuals who had a history of SCLS, or in retrospect probably had SCLS but had not been properly diagnosed as such, have had an episode of SCLS following their vaccination, see https://rareshare.org/topics/2131 or https://www.acpjournals.org/doi/10.7326/L21-0250 

Therefore, the recommendation from the physicians most knowledgeable about SCLS is that SCLS patients should get vaccinated with the Pfizer or Moderna vaccines, and NOT with the Johnson & Johnson or AstraZeneca vaccines, and they should receive their Covid vaccines soon after they have received a full-dose (2 gr/kg) infusion of IVIG.

In my case, I received my usual, full-dose IVIG infusion on Monday and Tuesday of this week, and then the Moderna booster on Wednesday, and so far, some 30 hours after the vaccination, I have no side effects to report.

Update: By now five days have passed since getting the third dose, and I have had no side effects.

So I'm one of the lucky ones who had a severe attack after my second dose (Moderna). This was back in February, and it actually led to my SCLS diagnosis. (I'd actually had two prior attacks that were misdiagnosed as anaphylactic shock and septic shock).

I'm very much on the fence about receiving the booster. COVID in and of itself could lead to another attack, but so could the COVID vaccine, even though I'm now on IVIG. There's just no way to know if the IVIG will protect me since I have a history of attacks post-vaccine. I've been talking with Dr. Druey, who recommends the the booster shot. I said I'd only be comfortable if I could get the booster in a controlled, clinical setting at NIH. He agreed that this was a "prudent plan of action." (Luckily, I live nearby.) 

That doesn't mean I'm not still anxious. I'm getting an antibody test soon which should help me understand just how at risk I am for COVID in the first place.

I'll keep you posted.

Stay well! Jen

I have an appointment for the Moderna booster Wednesday, the day after my IVIG infusion. I had no reaction to the first two injections.

Any advice or concerns?

Susan

Hello all

Thursday I received my 3rd Pfizer vaccination (Tuesday I received my IVIG) and I had no real side effects.

Hans 

Hello Hans , Hello all

What is the dose of IVIG (2 gr/kg), (1.5 gr/kg), (1 gr/kg), (0.5 gr/kg) when you are vaccinated against COVID?

This is a problem because I can only get IVIG (0.5 gr/kg) 

I feel worried!

Hello Wangtai, hello all

I know that several patients receive 2gr/kg  every month, I personally receive 1gr/kg every 3 weeks. I think -maybe I am wrong- that in Europe that 1gr is the standard. Altough 2gr is maybe preferable, I feel until now rather comfortable with the 1gr every 3 weeks.Before my IVIG session my blood is controlled and my IgG values are measured and they are good 15-16 while they need to be between 6 and 18. That value was very low after my severe attack in september 2019. I do not know if this is a false feeling... Maybe Arturo can tell more about this?

Some weeks ago (June) I had a serious fever (>40° Celsius) and I went to the hospital nearby (Mechelen, altough not a small hospital) and they sent me to the University hospital of Louvain. I was monitored all the time to see whether a new SCLS attack was happening. Fortunately there was no attack, so I also felt comfortable with the fact that both hospitals took no risk with SCLS. My IgG level at the next IVIG session decreased to 13.5. Was this decrease due to the fever and infection, so I guess that IVIG did its work . The fever was 2 days after a IVIG session. I spent 3 days at the hospital and felt good again.

Hans 

PS my first Pfizer vaccination was 14 days after an IVIG session.

Dear Hans

Thanks for replying!

Best regards
wangtai

Hello everyone

My husband has received his 3rd Pfizer vaccination and his treatment is back to 1gr/kg every 4 weeks (while he was waiting for the 2. vaccination he got 2 gr/kg) 

Is anyone still getting higher dose after receiving the booster vaccine (3.)?

(Omlkron spreads alarmingly fast i Denmark right now and we are worried). 

Best of wishes for your all. Kristine 

Hi Kristine

I have had my 3rd dose (1 Astra 2x Pfizer). In Australia the 3rd dose for an at risk person is not considered a booster but part of the base vaccination.  No troubles at all with the vaccine. I remain on 2gm/kg and will do so for as long as its possible.  The consequences of failure are too severe. Seasons greetings and best wishes to all.

I received my Booster vaccine(Pfizer) in October without any issues.  It was administered the day after my monthly infusion of 2g/kg. The possibility of getting COVID as an SCLS patient is terrifying.  My physicians highly recommended that the vaccines were important to help in the prevention on contracted COVID.  Good Luck to everyone and Happy, Healthy Holidays.

Thank you very much for your answers, it means so much to know we are not alone and there is help to find in this forum. 

We have now contacted the hospital and hope the dose can be raised to 2 gr/ kg. 

Best holiday greetings and wishes for 2022
Kristine