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.