Dr. Kirk Druey of the U.S.National Institutes of Health (NIH), the world's leading authority on SCLS, see https://www.niaid.nih.gov/research/kirk-m-druey-md-0, has asked me today to convey the following information to the members of this SCLS community:

Dr. Druey has recently been made aware of several cases of severe and/or fatal episodes of SCLS following COVID-19 vaccination. The common denominator in these unfortunate patients was that they (a) had not been diagnosed as suffering from SCLS or (b) had been diagnosed with SCLS but for one reason or another were not receiving IVIG on a regular basis.

While most COVID-19 vaccines on the market are not made from attenuated or dead forms of the coronavirus, they can trigger an inflammatory reaction which could spark an episode of SCLS.

If you are or know of any such patients, please have their physician contact Dr. Druey before proceeding with the COVID-19 vaccination.  He can be reached at kirk.druey@nih.gov

So far, SCLS patients who are receiving IVIG on a regular basis and who get vaccinated against COVID-19 have not experienced such an inflammatory reaction.

Hi Arturu,

thanks a lot for the information. 
 

My English is not good enough for the sentence "While most COVID-19 vaccines on the market are not made from attenuated or dead forms of the coronavirus, they can trigger an inflammatory reaction which could spark an episode of SCLS."

Are the vaccines there made from the attenuated or dead forms are the problem, or are they the ones that make no problems? Or all kinds of vaccines?

Good is, that I get my IVIG regularly in the recommended dosis (2g/ kg weight). Bad is, that I am not on my countries priority list to get the vaccination at the moment. SCLS is a rare disease and I had to apply at the local authorities to get the vaccination (no answer so far). 
 

Bye,

Andreas

Hello Andreas,

It is strange that you as a SCLS patient are not on the priority list for vaccination for Covid. Here in Belgium SCLS is on the list, while it is known as a rare disaese and is listed on the European ORPHANET as Orpha188. Since yesterday we can see if we are on the list of people that will get their vaccine sooner (in Europe, and Belgium this is relative...) In Belgium they normally start as from April the 19th with the vaccination of patients with certain diseases.  I am on the list, but I already had my first vaccine, my doctor hematologist arranged a vaccine of a leftover in the hospital. I told him that I preferred Pfizer or Moderna while they give the best protection also against mild symptoms. So he arranged a Pfizer vaccine in the hospital while the personel of the hospital , other then nurses and doctor- administrative personel etc.got their 2nd vaccine. In the comming weeks i expect to receive my 2nd one. On RaDiOrg it is mentionned that the European  policy is to give people that have a raredisease priority. So I recommend to talk about this with your doctor.

PS You can also contact me on hansdewit3010@gmail.com and you can do his in German. I understand quit good German, but writing in German is more difficult.

I personally receive "only" 1gr/kg IVIG but every 3weeks not 4 weeks.

In reply to Andreas, there are four types of COVID-19 vaccines on the market and/or in development: Whole Virus, Protein Subunit, Viral Vector, and Nucleic Acid (RNA AND DNA). Some of them try to smuggle the antigen into the body, others use the body’s own cells to make the viral antigen.

The Whole Virus vaccines are the ones that use either a weakened form of the virus that can still replicate without causing the illness or viruses whose genetic material has been destroyed so they cannot replicate, but can still trigger an immune response.  For more information, see https://www.gavi.org/vaccineswork/there-are-four-types-covid-19-vaccines-heres-how-they-work

All four types of vaccines are trying to achieve the same thing: immunity to the coronavirus, and some might also be able to stop transmission. They do so by stimulating an immune response to an antigen, a molecule found on the virus. My understanding is that all of them have the potential to trigger an inflammatory reaction.

Since the principal Whole Virus vaccines are manufactured in China (the Sinovac vaccine already in circulation within China and Indonesia, which was tested among volunteers in Brazil, Indonesia and Turkey, plus Chinese Sinopharm vaccines in the pipeline), I am pretty sure that the problems Dr. Druey has been informed about are associated with the other types of vaccines that are being administered here in the United States and in Europe, which are NOT of the Whole Virus kind.

For more background and updated information on COVID-19 vaccines, see https://www.gavi.org/vaccineswork/covid-19-vaccine-race and https://covid-nma.com/vaccines/mapping/ and click on any particular country on the map, for example on China to see the full list of vaccines.

Many thanks for this information. It is interesting as my first and near fatal SCLS attack happened days following a travel vaccination I had (Japanese Encephalitis) and it was considered to be a reaction to the vaccination as there was no other apparent trigger. I have therefore thought vaccinations could be a trigger for initiating SCLS. That was the last vaccine I had before going for my covid vaccine on the 1st Feb this year. I had the Astra Zeneca vaccine 3 days after having a 1g/kg infusion of IVIG and had no adverse reaction. I'm due my second dose soon and hoping all will be well with that too.

Best wishes to all.

Caroline  

Hi,

thanks for your answers.

Shortly after Arturos post, I read about the issue, that a vaccination can cause an attack in a reputable German news magazine on the internet. They wrote, that the affected vaccine was AstraZeneca - a Viral Vector vaccine. (The ggod side effectcould be, that more doctors know or at least have heard about SCLS....,)

Hans, as you probalbly know, the whole vaccination issue in the EU is a big mess., compared to the US, the UK and many other countries. In Germany, Im am definitely not on the priority list. However, crrently I try several ways to get a vaccination. The problem is, that my doctors are not allowed to overrule the priority list on their own. I have to apply for that at the federal state adminisrration. And it seems, that the work slowly.

Best wishes.

Andreas

To follow up on Andreas's message, here is an extract from the bulletin issued on April 9, 2021, by the European Medicines Agency (EMA), the European Union's equivalent of the Food and Drug Administration (FDA) in the United States, following a four-day meeting of their Pharmacovigilance Risk Assessment Committee (PRAC):

"PRAC reviews signal of capillary leak syndrome with Vaxzevria (AstraZeneca’s COVID-19 vaccine)

PRAC has started a review of a safety signal to assess reports of capillary leak syndrome in people who were vaccinated with Vaxzevria (previously COVID-19 Vaccine AstraZeneca).

Five cases of this very rare disorder, characterised by leakage of fluid from blood vessels causing tissue swelling and a drop in blood pressure, were reported in the EudraVigilance database.

At this stage, it is not yet clear whether there is a causal association between vaccination and the reports of capillary leak syndrome. These reports point to a ‘safety signal’ - information on new or changed adverse events that may potentially be associated with a medicine and that warrants further investigation.

PRAC will evaluate all the available data to decide if a causal relationship is confirmed or not. In cases where a causal relationship is confirmed or considered likely, regulatory action is necessary in order to minimise the risk. This usually takes the form of an update of the summary of product characteristics and the package leaflet.

EMA will further communicate on the outcome of the PRAC’s review."

See https://www.ema.europa.eu/en/news/meeting-highlights-pharmacovigilance-risk-assessment-committee-prac-6-9-april-2021

Thanks for the information update Arturo.

This is now in the mainstream British media.  

Hello Arturo and SCLS patients,

Thanks to Arturo  he gave us, as always, again some very interesting information for the SCLS community. We are very grateful to have such a helpful community leader.

I have a general question on vaccines.: is there also a risk for us SCLS patients receiving vaccines as flu vaccines, pneumonia vaccines etc? I receive a flu vaccine every year and pneumonia vaccine every 5 year. I presume that the benefits of all vaccines are more important than  the potential risks. I personally receive every 3 weeks 1gr/kg IVIG and my immuneglobulines IgG (last week =15.59g/L   needs to be between  5.40-18.22 g/L) are also monitored every 3 weeks . To everybody take care.

Hans

Hello everyone! I'm new here. Just diagnosed with SCLS in February after what was most likely a third attack in 18 years. This time the trigger was the Moderna COVID vaccine. Imagine my surprise when I found this group and saw that the most recent post was about me! ;) I live right outside Washington DC and saw Dr. Druey at NIH last month. He'd told me about the other cases of vaccine-induced attacks among SCLS patients. Obviously, I was one of the lucky ones.

When I saw the news from Europe on Friday I felt a sense of relief. I knew this was happening but the world didn't know it yet. This thing that I'd been trying to explain to everyone in my life was suddenly a headline. I know Dr. Druey is working on a paper about me and the other patients who had attacks after getting the vaccine. I'm guessing that after it comes out the American news media will be more inclined to pick up the story, especially since none of us received the AZ vaccine, which isn't even approved here yet.

I've had one round of IVIG so far and go for my second tomorrow. Dr. Druey has made it very clear that I should remain on IVIG. He did mention an injection option instead of the IV drip. Has anyone tried that? Finally, Dr. Druey recommended that I should continue to get vaccines (including any future COVID boosters) as long as I remain on IVIG. I hope this is helpful!

Hello Jen ... at this precise moment I am at home, with the second infusion of the month, I started yesterday and it has been almost 4 years now.  Regarding the Gammaglobulin injection, I understand that it is the subcutaneous injection, which does not suit us much due to the large amount we demand.  But if Druey commented on it, he would not stop reviewing it, perhaps technology advanced on this.  In my case, my 3 episodes occurred in 14 months, so 3 in 18 years at first glance I would take it in a positive way.  Let's keep informing each other, I think we owe it to Arturo, to the rareshare site and to this small community.

EDIT - Got through to our specialist today and got my questions answered. Wishing you all good health.

L
 

Thank you Arturo. Do you know if there is anything documented about similar reactions to the other vaccines? My husband won't be getting the AstraZeneca vaccine (in Canada, we aren't giving AZ to anyone with underlying health conditions), and will instead get Pfizer or Moderna. Unfortunately, if the information about other vacines is only anectodal at this point, we may have difficulty getting our physicians to take our concerns seriously. Any information is helpful, and thank you.

L

I would like to share a newsarticle that was published today in "De Standaard" a Flemish newspaper. Here below you can find a (by Google) translation

EMA is also investigating a second possible side effect AstraZeneca vaccine.

The European Medicines Agency EMA is also investigating a second possible side effect of Vaxzevria, AstraZeneca's corona vaccine. It is about reports of capillary leak syndrome, a very rare condition characterized by leakage of fluid from blood vessels leading to tissue swelling and a drop in blood pressure. The FAMHP reports this on its website on Friday.
Until now, five cases of this condition have been registered in the European pharmacovigilance database EudraVigilance.
"At this stage it is not yet clear whether there is a causal relationship between the vaccination and the reports of capillary leak syndrome," it said. The PRAC, the EMA's risk assessment committee, will investigate the matter further.
The PRAC previously confirmed that a combination of very rare cases of blood clots and a low platelet count is a very rare side effect of Vaxzevria. A study of thromboembolic events following vaccination with Johnson & Johnson vaccine in the US is als

Hans,

With regard to your latest entry, it repeats the news dated April 9 which I reported on six entries above yours, on April 10.

As concerns your earlier question regarding vaccinations generally, the consensus among physicians is indeed that the benefits of all vaccines are more important to us SCLS patients than their potential risks.

Since I was first diagnosed with SCLS more than 15 years ago, I have received every vaccine available (e.g., against Tetanus, Diphtheria, Influenza, Pneumonia, Shingles/Herpes Zoster, and now Covid-19) without any significant side effects.

However, since being on a tremendously successful IVIG therapy during the past more than 10 years, my physicians have advised me to get any and all vaccines soon after getting my IVIG -- to minimize the potential risk of any SCLS-episode-inducing side effects.  It's a timing recommendation that makes very good sense, but which all of us should discuss with our own physicians.