Hi Guys, my name is Gene and apparently I have been told that I am case 151 with SCLS. My first episodes started in July 2012 completely out of the blue. The episodes would come on very fast over a matter of hours with no indication that it was on the way. Each episode required that I got to Emergency as quickly as possible and because of the unknown factor I would generally stay in ED for around 18 hours before they gave up trying and passed me onto ICU. ICU would then give me fluids and within a few hours (literally), all the symptons would disappear and all would be back to normal. Liver, kidneys, blood count etc. The episodes got more frequent over the following months and got to the stage where I was having episodes almost on a weekly basis. It seemed to me that each episode was triggered by an argument with my manager at work. Generally I would say that 3 to 4 days after an argument, I would start my day with a visit to the Emergency dept. Fortunately for me, a trainee doctor at the time, saw a study from the Mayo clinic about SCLS and recognised that the symptoms were almost identical to to my case. In July 2013 I was diagnosed with the SCLS and started on a treatment of Theophylline and Terbutaline. Over the next 2 months I had episodes, but the onset of each episode was slowed down significantly, but not stopped. The treatment I received when arriving at ED was as far as I can remember simply to have 3 litres of normal Saline which settled everything down within a couple of hours, followed by observation for an hour or 2 to be sure everything was stable before being discharged, This happened 2 times and the whole episode from admission to discharge lasted 4 hours. Finally in September 2013 I was started on a treatment of IVIG (39mg every 4 weeks). Since starting on the IVIG, I have had no episodes at all. However, in June 2014 I started a new job which involves long haul travel on a regular basis. With this in mind and the fact that all had been good up to that point, I missed 2 treatments of the IVIG. All went well up to one week after missingmy 2nd treatment. The SCLS came back with a vengeance and put me back in ED in a different hospital. Despite my trying to inform them of my condition and what was done during my last episodes, it ended with me receiving 18 litres of fluids (ICU were knocking at the door) before being discharged. The following morning I attended ED again but at the original hospital I went to when all the episodes began and they gave me Diuretics to remove the fluids that had started to fill up in my lungs. My 2nd episode happened within 2 weeks of this, but this time the doctors listened to what I had to say and gave me 3 litres of fluids which stabilised everything, but then they went back into normal mode and stopped listening. This meant that I required an overnight stay with the night-shift (never a good time in my experience) resulting in the normal doctors starting from stage 1 when they arrived to see me in the morning.I am happy to say that I am now back on the IVIG and now realise that it is a long term situation so I won't be missing any more treatments.

Hello Gene... Wow! That episode with 18l sounds traumatic... Mine was 10 l in 6 hrs and that was bad enough. I suppose it is in all of us to become complacent.... I explain to family that it is like insulin for diabetics. You do not have an option. Good news, I have just received approval for free supply of IVIG! Hoping for a positive response. Any clues from anybody about how IVIG works? John

I received info from Arturo this afternoon containing a report from May with really good news on the effect of the IVIG on SCLS. So hopefully good news all round. The does not say how it works. Just that it works. Good enough for me.

Gene, I have been undergoing IVIG Therapy for over 3 years and often wondered what would happen if I missed an infusion. That is scary. I am sort of retired (photojournalist) and love to sail so I have put my "around the world" adventure on hold until a cure is found for this condition. Hang in there & don't miss anymore treatments! Photodonn

After 12 months in the clear and not knowing that this maybe a long term situation, it was something I had to try. It's unfortunately in my nature. I now know that at least on this occasion I managed to go 9 weeks before it came back and kicked me up the arse for being an idiot. But at least I now know that it is not a good idea for the future.

I'm lucky enough to live near NIH and got to talk with Dr. Druey about SCLS. He said they don't really know why IViG works, but that the theory is that SCLS is an autoimmune condition in which our antibodies gang up on us and cause some chemical to be released into the bloodstream that causes the capillaries to open. By flooding the body with lots of other antibodies (IViG), our antibodies don't get a chance to build up an attack against us. He said they'd done a test where they took blood that was sampled from someone during an attack and put it on epithelial tissue (same stuff capillaries are made of) in a petri dish, and the cells all seperated from each other. Blood taken from the same person when they were not having an attack had no effect on the tissue. In other words, if we're not having an attack, we're perfectly normal.

p.s. Gene - I was told I was 151 and I got attached to that number. I have to concede, though, because you were diagnosed before I was. (signed) 152 :-)

It seems to be a popular number and obviously comes from the report on the number of recorded cases since 1950. Ive actually been told by Arturo that I am more likely case 1351 or something, nobody knows. In any case it seems neither of us is 151.