When I reported back in October the bad news of the death from an episode (since fully confirmed) of SCLS of a fellow RareShare member who was compliant for 4 years with his IVIG therapy, see https://rareshare.org/topics/1950, it got me thinking whether the rest of us, who have had no episodes of SCLS for many years, ever since we gained access to IVIG treatment, were similarly vulnerable to new episodes that might get us killed. I'm sure I wasn't the only one who was shaken up by that piece of news.

So today it's my turn to report that two weeks ago I had a fully confirmed episode of SCLS, the first in more than ten years on IVIG, with the big difference being that I survived it with no evident damage to my limbs or organs.

Here are some reflections on what happened that might be useful to you.

1) To state the obvious, IVIG does not cure us from SCLS. It merely helps to prevent the chain of chemical and biological events that lead to an episode of SCLS. It hasn't helped everybody or all of the time, but it has worked wonderfully for almost all of us, almost all of the time. Therefore, consider and plan for the possibility that the therapy might fail you at least once. I fortunately did that in recent months, and it's probably why I'm here writing to you.

Implication #1: It’s really true that “an ounce of prevention is worth a pound of cure.”

2) Before I went on IVIG, most of my episodes were triggered by a viral infection, like the flu, a stomach flu, or the common cold. This has been commonly reported by other SCLS patients, so I wasn’t an exception. There is something about viruses as triggers of capillary leaks in patients who are prone to such leaks. However, I became complacent because during the past more than ten years I have had the flu and the common cold several times, and yet IVIG protected me from the usual chain of events that trigger an episode of SCLS. This time I had the flu (Influenza A, confirmed) but I had it during the last (fourth) week before my IVIG infusion, and that was probably what led to an episode of SCLS five days before my regularly scheduled infusion. My conclusion is that it’s best to assume that we are vulnerable during the last week of our infusion cycle, when there is relatively little IVIG circulating in our veins, and therefore we should avoid being far from home and our doctors and hospitals at that point in the cycle.

Implication #2: Arrange your work and vacation schedule such that you are close to knowledgeable family and medical help on the week before your next infusion.

3) Before I went on IVIG, what turned out to be my last several episodes were minimized by large, upfront doses of steroids (starting with my taking a strong dose of Prednisone at home and before getting on an ambulance, followed by additional strong doses of steroids upon hospital admission), and by minimal to zero fluid support during the leak phase while in the hospital, to reduce the chances of swelling and compartment syndrome needing surgery (fasciotomies) in all four limbs. The available medical literature says that steroids have helped some SCLS patients but not others, so it’s not a universal solution. Failure to provide intravenous fluids could lead to organ damage, so while the medical literature agrees that “less is more” when it comes to fluid support during episodes of SCLS, giving little or no fluid is also not a universal solution. And then there is the issue of what to do about IVIG: Should it be given up-front during an episode of SCLS, only as a last resort, or not at all? Here too, the medical literature is inconclusive.

Implication #3: Agree on a plan with your family and main doctor as to what you want, and don’t want, done if you happen to have an episode of SCLS.

While hospital emergency-room and intensive-care doctors are not used to getting guidance from other doctors, never mind from patients and their family, when it comes to someone with a rare disease, they will read written instructions on a doctor’s stationery and will listen to a doctor’s advice given over the telephone or in person. It’s always better to have a plan, or even a Plan A and a Plan B, than to arrive at a hospital with no specific instructions for your care. 

(It's also a good idea to have written backgroud information on episodes of SCLS, and I recommend always having a copy of this article to give out: https://journals.lww.com/ccmjournal/Abstract/2017/07000/The_Clinical_Picture_of_Severe_Systemic.15.aspx) 

In my case, I was very lucky that (a) I was at home and I was not alone; (b) my main doctor was in town and working at the hospital; (c) we had an agreed Plan A, we followed it, and it worked well.

Plan A consisted of my taking a strong dose of Prednisone before leaving home – and in recent months I’ve been carrying Prednisone with me wherever I go – plus a second dose while waiting to be processed at the ER; and then giving me IVIG within a few hours after arriving at the ER, followed by the usual second dose 24 hours later, with the IVIG being the only fluid support received during my 3-day hospital stay.

We observed that the Prednisone boosted my blood pressure back up to near normal levels; the first infusion of IVIG did not stop the leak episode (I continued leaking for another 12 or so hours), but it caused no harm; and the second infusion, which took place after I stopped leaking, also served mostly a preventive function and caused no harm.  (Remember that I was near the end of my cycle, so I was supposed to receive IVIG anyway.)

I’m not recommending any of this to anybody. Use it to start or continue a conversation with your own family and main doctor about what you want done if you should ever have another episode of SCLS. My doctor and I really wanted to see what difference, if any, IVIG delivered early and fast made during an episode, and we’re happy we did. I had made it crystal clear to my family and my doctors that I didn’t want to risk suffering organ damage and dying without having given IVIG a chance to work its usual magic, so my doctor made sure that I got it in the ER within a few hours of arriving there.

Sorry to hear about your recent episode Arturo but glad you are well again .Thank you so much for all this information which is greatly appreciated and so important for us all to know .

Continued good health and every best wish . Ann.

I  am so sorry to hear about this Arturo but I'm relieved that you are ok. Thank you for sharing this valuable information. I'm going to share it with my Dr who manages my SCLS, ( Dr Carl Lauter). 
wishinh you good health,

Susan 

Hi Arturo.. I am very sorry to hear news. Of course my first thoughst are for you and my second thoughts are for our community. It seems we must be on a hightened awareness as you point out. Thanks very much for your thoughts and insight into your management plan. I think we will all need to be vigilant and of course provide feedback to our community as this may become an even greater concern with time.

I am very compliant with my IVIG infusions at University of Florida Shands Teaching Hospital, where the most recent death has occurred. This and Arturo's news have been very unsettling.

I have a chronic cycle of significant swelling and resolving every several days. I am planning to spend much more time in Maine because the colder climate seems to lessen my cyclic swelling.

What advice, if any, should I provide my hematologist?

Susan

Hello Arturo and Comunidad all ... looking at the half full glass, there is my joy that you are well and that it is only a scare.  My desire, ... may God preserve your health, energy and your analytical mind forever.  Our health also depends largely on this happening.  Thanks Arturo.  As time passes and we move away from our last attack, we have the need to want to believe that we are almost normal, which obviously is not yet the case.  Hopefully medical science will advance soon.  And that each one can get this feeling of anguish that generate this news inside us.  Let's be on alert and don't trust each other.  And above all, let us share the details in the rare share community if there are any in this way.  Finally, I consult Arturo.  Finally he received two doses of 2gr.  Kg separated 24 hours.  Right?

Yes, I have always received 2 gr/kg every 4 weeks, two different brands, usually every Monday morning and Tuesday morning.  This leak started on a Wednesday afternoon (before the Monday when I was scheduled to get my first infusion) and it lasted almost 48 hours, until Friday afternoon.  I got my first IVIG infusion in the hospital on Thursday afternoon and the second on Friday afternoon.

Arturo,  

Thank goodness that you survived the most recent SCLS attack and you are OK.  It is comforting to know that you did not have a life threatening episode nor one that comprimised the use of your limbs. For that we should be grateful.  Unfortunately, we all know the risks of having this terrible disease. While IVIG helps prevents having more episodes, it is not a cure, as you can attest to. The most important take-away from your last episode, is that we can never become complacent with our care, even while being treated regularly with IVIG. Like you, I too keep a ready supply of Prednisone nearby, wherever I am.  Your advice of staying near home the week before the infusions is so valuable and a great reminder for all of us.  Unfortuntately, as time and years pass without an attack, it is easy for us to let our guard down, ever so slightly.  We mus remain vigilant in everything we do and never forgot how terrible these attacks are.  Personally, having the IVIG literature with me upon going to the hospital during my last attack (thankfully, now 7 years), I feel it saved my life. The doctors were able to follow the recommended course of action. Although, it still manifested into a major attack, I came out of it without any long-term issues.  Arturo, we are glad that you are doing well and ever appreciative of all the great things you provide for the SCLS Community.

Hi Arturo, I'm glad you're fine, but I'm sorry to hear this news because you live a false stability with the ivig and that makes us relax in the care.

1) I would like to ask you what symptom you had that allowed you to realize that this was not a common cold ... was the home measurement of hemoglobin or hypotension?

2) In other cold episodes already treated with ivig have you also taken prednisone at home? This year Ariel for the first time in almost 3 years of treatment with ivig had a common cold, but with fever and I just had him take 150 mg of pregnisone, luckily nothing happened.

3) Continue daily with hemoglobin measurements? We were very constant in the first months, but now this false stability that the medication gives us means that we do not measure it daily.

4) Could you talk to Dr. Druey or Dr. Amoura about his episode and about the October patient? The October patient also received 2g / kg monthly?

Finally I tell you as a fact that Ariel in his last episode 3 years ago, in which the doctor made the diagnosis of capillary leak, automatically also started to pass ivig 2g / kg for the first time in 2 days and the picture gave way. So with your story I would also like IVIG if you go to the emergency room.

Again, I'm glad you're well and I'm very sorry for the scare that you, your wife and your children had to go through. I send you a kiss.

Arturo, I'm sorry to hear you had another attack but happy to know you were well-cared for and didn't experience the most severe consequences of an attack. I have yet to have a full attack while on IVIG, but I switched to getting it every other Friday because I always felt ill during the last week leading up to my treatment when I was getting treatment every four weeks. Whenever I get a cold, I tend to get edema and gain water weight that I lose after a few days. My blood pressure doesn't drop and my numbers are in the normal range. I'm convinced these are mild episodes of SCLS. Thanks for sharing your story and thanks again for everything you have done for all of us.

To Lolaudesi, here are the answers to your questions:

1) What symptom you had that allowed you to realize that this was not a common cold ... was the home measurement of hemoglobin or hypotension?

I thought I had a cold and did not find out it was Influenza A until I was tested positive at the Emergency Room.  I had no idea I was leaking until I experienced syncope, frozen-cold extremities, and related drop in blood pressure.

2) In other cold episodes already treated with ivig, have you also taken prednisone at home?

I have never taken Prednisone except when pretty sure I was having a capillary leak and I was in fact on the way to the hospital.

3) Continue daily with hemoglobin measurements? We were very constant in the first months, but now this false stability that the medication gives us means that we do not measure it daily.

I recommend testing for hemoconcentration only to confirm/deny a suspected episode of capillary leak, e.g., when experiencing cold extremities (my case), general malaise, lightheadedness (and of course syncope, which was my case), drop in blood pressure (also my case), but from now on I will always test myself during a suspected viral infection (e.g., cold or flu).

4) Could you talk to Dr. Druey or Dr. Amoura about his episode and about the October patient? The October patient also received 2g / kg monthly?

Dr. Druey was fully informed by my doctor and by me during and after the episode, and we sent him episode blood and tissue samples for his research; the October patient did receive 2 gr/kg monthly and Dr. Druey received and examined all his pre-mortem medical records.

Hello Arturo,

I am sorry to hear about your recent SCLS attack. I am relieved that you are alive and had no lingering complications from this attack. I posted in May about my most recent attack. I have had two full-blown attacks since my very first one in 2011, with some small leaks in between. You can read the history of my attacks on my profile page.  I thought my attacks, although triggered by an illness, were mainly because I was not getting my IVIGG correctly. But with this new news of others having attacks while being treated correctly with IVIGG, that may not be the case. My history with IVIG is as such, and I write it out because it pertains to my upcoming question. After my 2011 attack, I was prescribed 1g/kg IVIGG treatments 1x a month every three months. I had another attack a year later in 2012. My doctor changed my IVIGG treatments to monthly treatments but the dose still only 1g/kg. I was having side effects from the long infusion, so my doctor changed my IVIGG treatments to 2x month. I was still only receiving 1g/kg broken into two doses a month. My most recent attack was in May of 2019. My doctor changed my infusions to 2g/kg and I still get them broken into 2x a month. My question is this: I understand the IVIGG does not stay level in our systems, but I am curious if breaking the treatment into two infusions a month keeps the IGG levels steadier? Or are my IGG levels on week 2 of each of my infusions, similar to the IGG levels of week 4 for a person who gets a full dose 1 time a month? I hope this question makes sense, and I ask it only to inform us all.  My first two attacks were preceded by an upper respiratory infection, and the May 2019 attack was preceded by a stomach bug. I have been falsely thinking that I was finally protected with the correct dose of 2g/kg IVIGG treatments and not too worried about the flu this season. I will now be hyper-vigilant. Wishing good health to all. 

To gandcburns, here is a simplified answer to your question:

Suppose that you drive a car and you usually use up a full tank of gas per month.  You can wait to refill the whole tank until the end of the fourth week, in which case you'll be driving around with a quarter of a tank or less in the final days; or you can buy half a tank of gas every two weeks, in which case you'll also be driving around with a quarter of a tank or less in the final days. 

(It's simplified because the IVIG protection we get does not decay in a linear manner, namely, it's not "consumed" at a rate of one quarter per week.)

Therefore, unless I'm mistaken and will be corrected by some of the physicians we have in our community, you have the same, relatively low level of protection in the final days of a monthly, full-dose infusion of IVIG as you do in the final days of a biweekly, half-dose infusion of IVIG.

In sum, the best thing you and your doctor have done to keep you episode-free is to have increased the dose of IVIG over time until you (finally!) started getting the recommended 2g/kg after your May 2019 episode.  I hope you don't have any more episodes but, well, my cautionary tale also applies to you.

Hi All. I am an engineer and so my understanding of the rate at which IVIG depletes may be incorrect but here goes. As Arturo points out  IVIG does not deplete in a straight line and indeed from my very quick look at some studies on this matter the half life (the time at which only half the material remains) varies greatly. Anything from 10 to 40 days. I have not yet been able to understand why the variation but intend to get some reseach papers on the matter because I think this is very important for us to understand. I assume therefore that the 2mg/kg dose rate has been established on the basis of the normal decay rate that a person experiences and leaves us with adequate protection at the end of the 4 week period.

On this basis alone it would be better to get a half teatment every 2 weeks rather that a full treatment every 4 weeks. However there may be other factors at work. For example (and this is not based on ANY medical knowledge) perhaps having the full dose every 4 weeks has a type of "knock out effect" which provides better overall protection than 1/2 dose every 2 weeks. I have used both regimes without any noticable difference in the effectiveness of the treatment. If I find anything further in the research papers I will post the information.

Welcome back, Arturo, to direct sunshine, wet sidewalks, the sound of birds singing and rivers flowing. 

Thanks for sharing your experience, the information and your suggestions. Good to have you back. 

Hi Arturo,

I am sorry that you had another attack, but I am happy  and relieved that you are still alive and your organs and limbs did not suffer any damage.

To all: My doctors sometimes take blood, and sometimes they determine the Level of IgG. Even about a whole month after I got my infusions, and shortly before the next one, my IgG level is rather high, higher than the reference level (this is always the case, when they do, they just do not do it always].

Do you know your IgG levels and have the same findings?

Andreas

I did the math once and the level of IVIG in your blood is much stabler when you do it every other week. The lowest level of IVIG on the fourth week is lower than the lowest level when you do it every other week. This is all driven by how long the half life of IVIG is and I've seen different numbers in different articles so this is by no means a definitive answer; I suspect the half life might even vary by individual. I also found that getting IVIG two days in a row was much harder on my body than getting it every other week. I experience fewer side effects on my current schedule.

Sorry for the attack Arturo, and I'm glad you came out the other side.  You're needed around here!

Glad to hear you are well Arturo - and a good reminder to us all to take no risks!!

Bonjour Arturo.

je suis vraiment désolé de lire que tu as eu un épisode de fuite cappilaire. par contre heureuse que tout a été pour le mieux pour toi par la suite.  il y a deça quelques années, j'avais des petites épisodes aussi, surtout à la suite d'un rhume comme toi.  Suite à cela, je reçois maintenant mes traitements IVIG chaque trois semaines. et depuis ce temps, aucune épisode.  j'espère que tout ira bien pour toi aussi

Google Translate:

Hello Arturo.

I am very sorry to read that you had an episode of cappilaire leak. however happy that everything was for the best for you afterwards. a few years ago, I also had small episodes, especially after a cold like you. Following this, I now receive my IVIG treatments every three weeks. and since that time, no episode. I hope everything will be fine for you too

Hello Arturo. I'm sorry to hear this. Thank you for letting us all know. I'm also happy that you have recovered. This must have been scary for you and your wife, and I feel much empathy.

Hi Arturo and others -

Haven't been on here for a while and I'm so sorry to hear that you experienced an episode - very glad that you were able to recover!

I'd like to stress again that I have only been on IVIG for about 2 years; for the previous 18 if I had an episode then I followed an albumin infusion protocol when going to the ED. I have used this protocol at least 25 times and it WORKS to abate the leak until it reverses itself. I want to stress this - it immediately makes me feel better and I spend a night in the hospital to be monitored. Then I am home with no side effects other than some IV scabs. I am more than happy to scan the document and send it to the group. 
I did meet with Dr. Druey last summer and have entered into the study - he and his team have this same protocol. 
 

Please let me know if there is a place on here upload the document and I'll be happy to do it.

Good Health to all during this pandemic!

- Jeff Zielinski

Thanks Jeff! If you could scan the document we'd appreciate it. We'll speak to my husband's hemotologist about this as well.

Hi Arturo!  I am sorry to hear about another episode but am so glad to see that you are ok.  It sure makes me nervous to think this even with the ivig therapy, this is a possibility.  Qamer has not had any episodes since 6 years & we have let our guard down.  I will let him know of this news.  Our routine is usually to travel right after getting ivig to reduce the risk.  Qamer is working at a hospital that has 8 corona virus patients & it makes me nervous.  He was able to opt out of caring for them because of the capillary leak syndrome.  You all take care & I pray that everyone stays safe.