I had my first episode May 22 and had a second in Feb 23. After fighting with the insurance company to get it approved I finally got my first infusion of IVIG in May and my second Today. I am curious how you all live your daily lives. Do you wear masks, stay away from people, or do you live a normal life? Both my episodes were tramatic and I don't want a third.
Hi,
I was finally diagnosed in 2016 and receive IVIG since then. I live a rather normal live, with no extra precautions (since Covid is more or less over).
if I did not do so, I am pretty sure, that I would get huge psychological problems.
There is one exception: I do not travel to far away abroad, because I would get in trouble with the health insurance abroad. Probably they would not cover SCLS-related treatment (e.g. an attack, I am not talking about the monthly IVIG), and an ICU stay would be extremely expensive.
I live in Germany, and traveling to France, Austria etc is no problem. But I hesitate flying to the US or so.
Yours
Andres
I was diagnosed in March 2022, and started IVIG in Oct 2022. For the most part I've been doing things normally, and wore a mask up until a few months ago. I've never been diagnosed with Covid so I'm not sure how I would react. Probably about 50% of my SCLS attacks (6-7 out of 12) were related to illnesses or vaccinations, so I'm still kind of cautious when it comes to large crowds. I still avoid those for the most part (concerts, large work related meetings), all though I don't have any scientific reason to do so. It's mostly just playing it safe.
On the other hand, I probably had a small, continous leak, and since the IVIG started, I'm feeling so much better in almost every aspect of my life and have been much more active in getting out, exercising, cycling, hiking, etc. So that has changed, for the better.
Welcome to our SCLS Community, Rick!
I read your profile page and I'm sorry to learn that you've had two episodes and twice underwent eight-compartment fasciotomies! I also suffered through the same experience back in 2007 and 2009, so I have some idea of what you and your loved ones endured.
The only good thing about the fasciotomies is that if they performed them in time, they should have prevented the impairment or loss of muscles and nerves in your four extremities. In my case, they didn't perform them quickly enough during what turned out to be my very first episode in 2005, so I suffered permanent damage in all four extremities and thus I'm partially disabled. (They didn't realize that I was bleeding internally into my arm and leg muscle compartments until much damage was done.)
In those days 15-20 years ago, there was little to no awareness about SCLS in the medical community -- never mind there being a good protocol for how to manage our episodes, not to speak about how to prevent them. Getting a diagnosis of SCLS was the equivalent of getting a death sentence, because the natural progression of the syndrome was for us to go through more, and more frequent, episodes until one of them killed us, due to heart or brain damage.
But you and most current members of this SCLS community are fortunate that we've come a long way since then, and now we know not only how our episodes should be managed, but also how to minimize the incidence of episodes. Therefore, most of us are able to live 10, 15, 20 or possibly more years after surviving our first episode. So that's the first piece of good news.
To make a long story short, if/when we have a confirmed episode of SCLS, we are to be given two doses of IVIG (intravenous immunoglobulins), 1 gram/kilogram of body weight each infusion -- ideally, the first one in the Emergency Room within the first 2 hours after arrival, the second 18-24 hours later in our hospital room.
And that's pretty much the only fluids we should receive, because it is the fluids (usually saline) administered in the hospital that harm our muscle compartments -- because we're going through the leak part of the cycle.
Therefore, even if you experience additional episodes of SCLS, the two fasciotomies you endured can and should be the last ones you ever suffer through. So that's a second piece of good news.
Please go to https://rareshare.org/topics/2192 and read all about it, then download the pioneering article at https://www.acpjournals.org/doi/epdf/10.7326/aimcc.2022.0496
As it advises there (https://rareshare.org/topics/2192), read the article, then email it or print it out and give it to your main physician and also to your immediate family members, and then print out extra copies for yourself and carry them with you wherever you go -- so that you or your family can give it to the first nurse or doctor you meet the next time you experience an episode of SCLS.
Now with regards to the prevention of episodes of SCLS, the third piece of good news is that most episodes can be prevented by regular infusions of IVIG, 1-2 gr/kg every 4 weeks or alternatively 0.5-1 gr/kg every 2 weeks.
Most of the episodes of SCLS are triggered by viral infections, and chances are pretty good that if you get a viral infection in the days or few weeks after your infusion – when your “tank” is pretty full, so to speak – then the extra immunities you’ve received should be able to prevent the cascade of internal events that triggers an episode of SCLS. See discussions at https://rareshare.org/topics/2183, https://rareshare.org/topics/2204, and https://rareshare.org/topics/2223
However, experience shows that (a) in the wake of a viral infection (especially the early versions of Covid, which were very powerful), it is unusual but possible to get an episode even soon after you’ve received an infusion of IVIG, and (b) it’s always true that “an ounce of prevention is worth a pound of cure.”
Therefore, in my case, for example, since in recent years I’ve had 3 episodes of SCLS in the wake of 3 different viral infections, see https://rareshare.org/topics/2223, I practice prevention and put on an N95 mask when I know or realize that someone I have to meet has a cold or some other illness, and I avoid large crowds in indoor settings especially during wintertime, when viral infections tend to thrive. It’s a small price to pay for the privilege of living as episode-free as possible.
I hope this information helps you and others wondering the same.