I have been on a steady therapy of 2 g/kg of IVIG every 4 weeks since November 2009.

In the more than 10 years from then until January 2020, I never experienced another episode of SCLS.  

And this despite my working full-time, thus being in permanent physical contact with many people -- never mind friends and family members -- which implies that I must have been exposed to viral infections and other potential triggers of SCLS episodes.

Indeed, during that decade I had my fair share of upper respiratory tract infections -- common colds of viral origin -- yet none of them triggered an episode of SCLS.  And, believe me, every time I had a cold or flu, I diligently monitored my hemoglobin levels using a HemoCue analyzer, so I would have detected any hemoconcentration.

This picture of complete preventive IVIG success during 2010-2019, unfortunately, has been marred by events during the past 3 years.  

In January 2020, an influenza infection triggered in me an episode of SCLS requiring hospitalization and treatment -- despite my having been vaccinated against the flu, see https://rareshare.org/topics/1967

Then, in December 2021, a Covid-19 infection triggered in me an episode of SCLS requiring hospitalization and treatment -- despite my having been as fully vaccinated as then possible against Covid.

And now, during the last week of April 2023, a human metapneumovirus (HMV) infection triggered in me an episode of SCLS requiring hospitalization and treatment.

[Incidentally, all 3 of these episodes were treated only with extra IVIG, as per the recommendations in the recent article "Management of Acute Episodes of Clarkson Disease (Monoclonal Gammopathy-Associated Systemic Capillary Leak Syndrome) With Intravenous Immunoglobulins," downloadable from https://www.acpjournals.org/doi/epdf/10.7326/aimcc.2022.0496  For more background information, see https://rareshare.org/topics/2192]

Therefore, my main doctor and I are wondering if we are witnessing in me a loss of effectiveness in the preventive capabilities even of high-dose IVIG therapy for SCLS, for which purpose I'm turning to this community to see if anyone else -- especially those who have been on IVIG for a decade or more -- have also witnessed anything similar.

The explanation we came up with for my first 2 post-IVIG episodes (Jan. 2020 and Dec. 2021) is that they occurred when I was just days away from getting IVIG, namely, when the level of immunological protection I had was low.  However, this latest episode took place just one week after my monthly infusion, when the level of protection I had should have been sufficiently high.

Hi Arturo. Very sorry to hear of your episodes. I have been on IVIG at 2mg/kg since 2011 and while I have not had a full SCLS episode in all those years, recently, towards the end of the 4 week period I am regularly experiencing swelling. Sometimes reasonably significant. I will keep monitoring and report if there are any changes. Good luck. Warren King

Hi Arturo, I am so sorry to hear about your latest episode.  I hope your recovery is going well.  When SCLS patients have an episode that results in hospitalization, are they mild or severe in nature?  As new members to the community, looking to get a baseline on if episodes occur while on IVIG are they as sever as the past two we have had (not on a monthly treatment of IVIG) requiring ICU an or fasciotomy?   

You mention the use of a home hemoglobin test kit.  Do you have one you recommend?  

Sending you good thoughts for a speedy recovery and so thankful this channel has you and your willingness to share to help others.

All the best,
Angie

Hi Arturo.  I am sorry for what happened, for you and the entire community.  As I recounted in other posts, 2 of my 4 vaccines have caused minor leaks and one that required hospitalization on Christmas 2022. Since the covid began I have a theory that I would like to share.  I understand that the antibodies we receive to gamma globulin are derived from blood donors for its manufacture.  I understand that we are going through some particularly changing years in terms of viruses and their varieties.  That is why I understand that it is possible that even the blood of the donors does not have a sufficient amount of antibodies for the gamma globulin to protect us.  If this theory that I share, without any scientific rigor, should stabilize over time.  I greet you all with the constant hope that a cure for our disease will appear.  Greetings.  Ariel

I have not had any problems since I started on IVIG over 13 years ago until I got Covid in October of 2022. Since then I have struggled with mild "leaking" episodes that include swelling and decreased blood pressure with dizziness every few weeks regardless of my monthly IVIG. In January, we decide to try a half dose every 2 weeks. It did not really help stop the periodic leaking but the leaking has gotten milder. I have found that hard physical work does bring on a mild episode. All this said, my doctor and I decided with my last treatment to go back to a full monthly dose. So far it has worked. I will let you know if it continues to work. Thanks for keeping us up dated.

Nolan Peterson

I have started getting IV. I G treatments every two weeks at half the monthly dose. Due to travel, we separated them by three weeks two treatments in a row and I ended up having an attack. My doctors have me sticking to a strict two week schedule. I am a little concerned as I have developed an iron deficiency, and I'm currently on a supplement.

Rita Wood

I have been receiving monthly IVIG infusions 2g/kg for the past 10 years without any SCLS attacks.  Occasionaly I felt the onset of a slight attack that included minor leaking, lightheadeness and blurry vision.  Within one day, the symptoms abated and I returned to normal, without the need of any meds.  Overall, I consider myself "one of the lucky ones" who have not had any SCLS episodes, especially with the circulation of COVID and all other airborne illnesses.  I am able to maintain a very active lifestyle, however, at times I need to throttle back my exertion especially on hot, humid days.  Best Wishes to everyone.

Hi Arturo so sorry for your episode hope your recovery going well 

This conversation isn't helpful. Haven't we all been through enough? Now we have to worry about our one hope for treatment and prevention? Though we are all diagnosed with SCLS, we are also different. I'm unsubscribing to this. I don't need another panic attack like the one induced from another conversation here during the pandemic. I wish you all the best. 

Hi Arturo, Very sorry to hear about these episodes .Thank you for the updates as all this information is so important .Sending you all the best . Ann

Case studies have suggetest that SCLS may have a viral trigger. We know that the COVID virus, and its viral variants, can be deadly and debiltating. We also know that IVIG lessens the intensity of crises and flares in most SCLS patients.

What we don't know is whether high mortality risk viruses erode the efficacy of IVIG.

I think that it's a topic ripe for research. However, I don't think that it's a cause for worry at this time.

As for my recent viral and other history:

1. Late 2021, I had a heavy Upper Respiratory Illness in late 2021.
2. I had more frequent flares after that time.
3. December 31-January 2, I was hospitalized for 3 days for rapid fluid shifts that triggered autonomic dysregulation, a near fatal event.
4. July 4-12, I was hospitalized for aspiration pneumonia. A near fatal event.
5. December 18-30, 2022, I had a viral illness that developed into COVIS pneumonia. I was in Florida where no one masks, including health care workers. Though masked and only using curbside businessess, I contracted COVID when I dropped my mask for an oral temperature reading by an unmasked nurse. I left Florida as soon as I was able.
6. I have had chronic edema since the COVID episode.
7. Susan Tedrick 

Hello SCLS Community,

I am sorry to hear that many patients are having the same problems as me, but I am grateful to know that I am not going crazy. I too have been struggling with more frequent leaks ever since February 2023. I have had the Covid Vaccine, two booster shots, and infected with Covid two confirmed times. I believe I had Covid again this past February, but I wasn't tested and recieved an extra treatment to combat the leak.  I have been on IVigg since 2011. I teach fitness classes and personal train, so I always have a heavy activity load. I have had intermittent small leaks over the years, but they were always preceeded by an illlness and physical activity. Prior to Februray 2023, I had only been hospitalized three times for severe leaks in a span of 12 years. However,  since my last illness in February I have synncope symptoms (fogginess, fast feelings of faint that go away quickly) almost daily and I have been hospitalized twice. I also have had a leak two to three days after every infusion with no preceeding illness. I receive 55g every two weeks. Most of the leaks are not too serious and resolve on their own in a few days, but they still slow me down where I can't work and have to rest for 3 to 4 days. I have changed IVigg brands thinking that might help, but to no avail. I have stopped teaching cycle classes, which are the most intense classes I teach, in hopes to stop the leaks. This hasn't made any difference either. My doctor also tried increasing my IVigg dose to 80g every two weeks hoping this might stop the small leaks, but this trial actually sent me into the hospital with a severe leak. I have spoken to Dr. Druey and he suggested trying subcutaneous. My doctor and I are in the process of working this out with my insurance company. Has anyone had success with SQ and if so what brand? I am hopefull that two shots a week will minimize the amount of fluid I am getting at one time and will stop the leaking.

I have no basis for my next statement except for what I have been experiencing these last five months. I know it is not safe to hypothesize, but there is so little known about our disease and the effects of Covid on an SCLS patient that I think searching for ideas can be helpful. I believe Covid has compromised my capillaries rather than my immune system. From what I was told when I visited the NIH in 2011, our capillaries thicken up and pull apart  during an SCLS attack which allows the plasma in our veins to leak. I feel that after my last infection with Covid and my capillaries were compromised, they have never fully recovered to a normal state.  So now when I get my treatment the fluid that comes with an IVigg infusion is too much and forces my compromised capillaries to leak. I have not been sick since February, so it is not my immune system trying to fight an illness that is sending me into the leak. Please share your thoughts on my hypothesis.

Praying for the best for all of us,

Cristina Burns

Cristina,

I think your theory is logical, for no other reason than there's a lot we still don't know about covid and there is a cardiovascular component that sets it apart from other upper respiratory infections. A friend who happens to be a nurse at NIH (different department than Dr. Druey, but she knows a nurse on his team) told me after my SCLS diagnosis in 2021 that "there's something about covid and blood vessels that we're starting to see." The attack that led to my diagnosis was triggered by the covid vaccine. Both the vaccine and the disease have been known to cause blood clots, myocarditis, pericarditis, and SCLS attacks. I agree that more research is needed. This isn't just about SCLS patients, yet we could be an important clue in helping solve some of the mysteries of covid.

Jen

Cristina, as concerns your hypothesis that "Covid has compromised my capillaries rather than my immune system," I think if that were true then many more people -- and well beyond just SCLS patients -- would be reporting capillary leaks, but I haven't seen that mentioned in the medical literature about Covid.

With regard to subcutaneous IG, I don't think that any adults with SCLS have gone down that route because the amount of IG that we need is so large that one would have to be shooting themselves daily, or every other day, in order to receive the dosage that one can get in one IVIG session.  Subcutaneous IG is therefore usually given to children or to adults (with other conditions) who need only a small amount of immunoglobulins per month.

Cristina, your question has piqued my interest. I did a quick search ("covid capillary damage") and came across these stories from last month. They're using terms like "capillary leakage." It sounds like the leakage isn't as dramatic in covid patients as it is for those of us with SCLS, but it's still leakage, and enough of it that it has real consequences. And if our capillaries are already damaged from prior leaks or simply prone to leakage, it makes sense why covid is so serious for us. It could also explain why a lot of us are expeiencing more leaks all of a sudden. It might not be the IVIG; it might be covid. Because this theory even explains long covid! And covid toe! (Remember covid toe from the early days of covid??) I'm a little surprised I haven't seen more of this reporting in the mainstream media. Definitely not a doctor here, but it seems like this theory explains a whole lot. I found other stories hinting at this theory from as far back as January 2021. That would explain my friend's comment about the link between covid and blood vessels, which she made in February 2021. The good news is that they're suggesting that the more recent strains of covid don't cause the same kind of leakage.

Has anyone ever had a conversation with Dr. Druey or any other doctor about endothelial cell damange? Cristina, I wonder if that's the "thicken up and pull apart" that you mentioned.

https://www.bhf.org.uk/informationsupport/heart-matters-magazine/news/coronavirus-and-your-health/is-coronavirus-a-disease-of-the-blood-vessels

https://www.science.org/content/article/blood-vessel-attack-could-trigger-coronavirus-fatal-second-phase

https://pubmed.ncbi.nlm.nih.gov/33523608/

https://www.defense.gov/News/News-Stories/Article/Article/2468664/covid-19-may-invade-damage-blood-vessels-not-nerve-cells/

Thank you Jen and Arturo for responding. Jen, in 2011 I had a conversation with Dr. Druey about endothelial cells. In my telehealth appointment with him this past week, we didn't bring it up. He was pretty perplexed about what is happening to me at the moment. I urge all of those suffering with more frequent leaks, after Covid infection, to reach out Dr. Druey. He was surprised when I told him that there were other SCLS patients experiencing more frequent leaks as well. Because everything we do is exploratory, I am going to keep him informed on everything my doctor and I try. I am going to try the subq route next. I will let you all know what my experience with it is. 

Cristina

Hi Arturo,

I'm sorry to read that your IVIG treatment isn't working well anymore. 
I have a theory that the covid vaccine is responsible for many changes in our inmune system in both healthy individuals as in SCLS-affected patients. 

A research Doctor in Barcelona, Spain, who completed his specialization studies in the Cleveland clinic, would like to try a biological treatment with Anakinra known as Kineret. Since I have been stable so far, and with no severe episodes since my 3rd, and last one, back in December 2021, we decided to give it a try whenever the next episode arises. 

Best of luck,

Valeria. 
 

Update: I have been swollen for 3 weeks now.

My family recently drove to North Carolina from Kansas City. I had no trouble with the drive there and the week in North Carolina with leaking.  I wore my compression socks to avoid swelling from sitting for the long drive. However, on the return trip my legs began to swell, even though I had the compression socks on. I also had an ulcerative colitis flare up while in NC. I thought the swelling would go down after a day or two, but it has been three weeks now. It had been a month since my last Ivigg treatment. I began subQ treatments two weeks ago (20g every two days) and have not seen any improvement with the swelling. I still have 4 more treatments to finish out the month, and am hopeful that once my Ig levels are stable the swelling will go away. We have had horribly hot temperatures in Kansas City for the past two weeks as well. I am also unable to do my job well. I swell more with activity. I have not been light headed, but I am very uncomfortable with the 15lbs of extra water weight on my body. My thoughts are all over the place, becasue there are so many variables as to why this is happening. I do not know what is causing this swelling to linger. Is it COVID complications to my immune system or capillaries making the ulcerative colitis send me into a leak? Is the heat causing me to swell? Is exercising making the swelling last longer? Is it because my Ivigg levels became too low?  I have had ulcerative colitis flare ups in the past, and they don't send me into a leak. I have tried taking ice baths which help for a short period. Has anyone experienced this type of swelling before? If so, how long did it last? What did you do to relieve pain in your joints from swelling? I am curios about trying diuretics? Thank you for any insight.

Cristina Burns

I hope that Dr. Drury is able to follow this string for possible anecdotal data and case study development.

I began IVIG in February 2010 and felt significantly better. In order to decrease side effects, I have a half-dose every 14 days.

My observations:

1. Most SCLS sufferers have comorbitities [other conditions] whose pathology and/or treatment may cause edema and fatigue, and thus cloud the IVIG picture.

2. Many of us have suffered COVID episodes whose longterm, systemic impact is just now being studied.

3. Patients who experience longterm edema, such as SCLS, heart failure, and medications may also experience venous insufficiency over time.

4. Since I was diagnosed in 2010 and confirmed by Mayo Clinic in 2014, I have had several serious SCLS flares. I have also persistent mild edema that resolved overnight.

5. Over the last few years my edema has increased in my lower extrmities during the day and has resolved less and less overnight.This may be simply gravity and venous insufficiency.

6. During the pandemic I developed crushing fatigue and increased dysautonomia. I isolated but had 2 acute upper respiratory infections, and 3 episodes of pneumonia, one of which was acute COVID pneumonia.

7. I can tell the difference between an SCLS flare, where I swell all the way up my body, including my face, gain 8-15 pounds of "water weight", and daily edema where my swelling is mostly in my legs. In both circumstances, my feet are the size of American footballs and extremely tight. I try to elevate as much as I can.

8. It's impossible for me to say whether my daily edema and crushing fatigue are related to COVID or other chronic illnesses.

9. I have found very few academic studies correlating COVID with decreased IVIG efficacy; or COVID with various comorbidities. However, I have found learned articles that identify dysautonomia as a symptom of COVID and/or COVID as a trigger of dysautonomia. A recently announced study is an attempt to treat "long" COVID, but does not mention IVIG nor SCLS.

Bottom Line: There is not enough data to surmise or conclude that, for SCLS patients, IVIG loses efficacy over time. The concern certainly deserves to be studied along with the interplay between SCLS and other comorbities.

Susan Tedrick

stedrickmedlaw@gmail.com

Susan and Cristina, so sorry to hear about your persistent troubles!  If you wish to let Dr. Druey know about your troubles, you (or preferably your physician) must let him know directly, because to my knowledge, no, he does not read our posts here on RareShare.

Hello everyone. Recurrence has definitely increased in my case. In August of this year I traveled to Italy for 4 weeks. The next day after arriving with my full gamma tank, I started to have a typical leak of mine. Severe leg pain and fatigue with a flu-like state. Once again the plane seems to be present. Supported only on high doses of prednisone (I don't know if they are useful or not) he completely reverted only on day 9 (the worst are the first 4). As I said before, I also had an episode last December, 3 days before the gamma.
Greetings, Ariel.

I feel that both COVID and it's vaccines are problematic for me as an SCLS patient. My own history is:
2014 - started treatment for SCLS with 2g/kg of IViGg
2015 - dosage dropped to 1g/kg
2018 - SCLS flare due to going 6 weeks between doses. Moderate symptoms brought under control by receiving IViGg
Jan/Feb 2021 - got Pfizer covid vaccines. Felt exhausted, lethergic, and brain-foggy for 8 months.
Oct 2021 - Realized I was 20 lbs. heavier than the dose of IViGg I was receiving was calibrated for and asked my doctor to raise the dose  - that made a big difference.
Jan 5 2022 - I came down with COVID (the Omicron varient) less than 2 weeks after an IViG infusion. I didn't have much edema, maybe some mild swelling, but did have major exhastion/lethargy that took over 4 weeks to get rid of.
Jan. 2024 - currently fighting with a sinus infection that is causing lethargy and brain fog. And making me cranky! I am so tired of being tired!!

I feel strongly that both COVID and its vaccines are hard for my body to deal with. I know we are supposed to get all the vaccines, but I have not gotten another covid vaccine since 2021 - but I fully acknowledge I have crackpot beliefs about my immune system and what's best for it, as I am someone who always refused to get flu vaccines before covid.

So I wonder: Is it that IViG is becoming less effective? Or is it that our immune systems are getting overloaded with stuff that's hard for the body to deal with?

Arturo, I sincerely hope that you are completely over the virus you caught last spring, and that you are feeling well now. You're a gem of a person and I wish the very best for you :)

Cathy

After 4 years of avoiding contracting COVID, I had my first episode in late December.  I have been fully vacinated including booster shots, the flu vaccine and RSV.   When I contracted COVID, it was week 3 of the monthly IVIG infusions (2g/kg), so my antibodies were diminished.  I quickly started Paxlovid and my symptoms starting getting better within days.  Fortunately, COVID did not require any hospitalization or another SCLS attack.  The combination of IVIG and Paxlovid were beneficial in my case.  Without getting my regular IVIG infusions, I fear the worst case scenario would have occured and another violent episode of SCLS would have occured.  Consulting with Dr. Pecker at Weil Cornell (who Dr. Druey at NIH studied under)  and discussing my situation with Arturo, these recommendations re-assured me that the infusions and taking Paxlovid avoided an episode.  It has now been 14 since my last attack after having 7 major attacks which included stays in ICU.  IVIG treatments began 11 years ago.  Without IVIG, my life would be totally different.  Someone once described it as "Liquid Gold"  I am a true believer that over time IVIG has not diminished its capicty to diminish or prevent future episodes of SCLS.

Hi eveyone!, 
Upon reading your experiences and theories about episodes triggered by both covid vaccine and covid infections, I'd like to share that:
1.- Both of my doctors here in Barcelona (the one that diagnosed me with SCLS after my 2nd severe SCLS episode back in 2019 and the one that conducted the research for another treatment apart from IVIG) think that covid vaccines may be as harmful as a covid infection for SCLS patients.

2.- I am not vaccinated and I did contract covid once which resulted in mild symptoms that resolved without hospitalization just as the ordinary chronic and mild leakage episodes that include swelling and decreased blood pressure with dizziness I struggle with every other week since my 2nd severe episode in 2019.
3.- As many of you know, IVIG did not work for me. For the ones who are new: IVIG did not not work for me as I continued to struggle with recurrent and mild leakage episodes that included swelling and decreased blood pressure with dizziness every week/10 days regardless of the IVIG infusions which I received every other week from May up until November 2019, and because my kidneys presented with signs of chronic failure which resolved when the infusions were ceased.
4.- I started daily treatment with ANAKINRA in mid-December 2023, in the midst of one of the recurrent-mild episodes I usually struggle with, which eventually started to escalate to a more severe one. As planned, we gave Anakinra a try and the leakage stopped completely. My Hematocrit levels decreased within hours, my blood pressure normalized within hours too and the 7lbs-oedema resolved within 2 days. I now inject it every morning since that day. 
*Anakinra sold under the name of Kineret, consists of 100mg subcutaneous injections of a prescription medicine called an interleukin-1 receptor antagonist (IL-1Ra) used to reduce the signs and symptoms and slow the damage of moderate to severe active rheumatoid arthritis (RA) in people aged 18 years and older when 1 or more other drugs for RA have not worked, also to treat people with a form of Cryopyrin-Associated Periodic Syndromes (CAPS) called Neonatal-Onset Multisystem Inflammatory Disease (NOMID) and to treat people with Deficiency of Interleukin-1 Receptor Antagonist (DIRA).
5.-I continue taking terbutaline, theophylline, furosemide and bilastine daily.
 

Cathy, I wonder if you're suffering from long covid. I believe some people get long covid from covid vaccines. 

8 months is way too long to feel exhausted and brain-foggy. And now your sinus infection is putting a severe drain on your immune system again.

My husband has SCLS and has gotten all Covid vaccines and boosters with no issues. 

He had COVID a few months ago, took Paxlovid, and recovered within a few weeks.

IVIG continues to work for him after almost 5 years with no leak episodes.

On the other hand, our son has Myalgic encephalomyelitis, also called chronic fatigue syndrome or ME/CFS.

He got sick 19 years ago after a severe flu that was not diagnosed. He has been steadily declining and is now housebound.

We're thankful he's not bedbound on feeding tubes. 25% of people with his disease are.

We opted for him to not take any COVID vaccines, concerned he might have adverse reactions as he has to other vaccines and medicines.

ME/CFS caused by a virus like his (some cases have other causes) is very similar to long covid, they are both post-viral syndromes.

Extreme fatigue and brain fog are among the many possible symptoms.

A few years ago my son had an issue when the COVID virus we've all been exposed to, joined with the Epstein Barre virus in his system that chronically reactives.

Our local doctor worked with Bruce Patterson MD, a long covid specialist, to treat him with antivirals.

The medicine finally helped, but in his case that meant going from 20% functionality to 30%.

Dr. Patterson's protocol may be more helpful for long covid patients because they've only been sick for a few years at most.

I highly recommend Dr. Patterson to anyone who has long covid. https://www.covidlonghaulers.com/home

Hope this helps!

(Mrs.) DavidS - thanks so much for the information. I will certainly look into it.

:)
Cathy
ps - it took me a long time to see your post due to the struggle to change my email address. I finally gave up and established a whole new account with rareshare.