Hi all, Because it might be important for everybody I decided to write down some of my story , especially about the effects of treatment. Since 2010-2011 I'm suffering from an ongoing disability to perform any kind of exercise, in combination with swelling of face and trunk (abdomen). Normal life is completely disturbed. The episode was started with à clear swelling, as some other periods already passed before. Beside of this in investigation was proven that exercise caused severe acidosis after a short period and a mitochondrial disease being ruled out. In 2012 diagnosis was set to SCLS, chronic persisting and Terbutaline and Theofyllin was started. À dramatic improvement was initiated, but faded away after three months. My doctor- hematologist/immunologist prof Kramer discussed about IVIG, but in the end proposed to start with imatininib (glivec, /gleevec) together with T&T. Another patient with à similar clinical presentatiion, started already with positive results. Imatinib is à proteine kinase inhibitor and theoretically could influence SCLS bij downregulation the activity of VEGF, without introducing strong vascular side effects. This autumn was started with à positive result, but still situation could be disrupted very easily by triggers as walking short distances, cycling 10 minutes, cold a.s.o, so the daily dose was elevated up to 400 mg daily. that started the most stable period for about 8 weeks untill now in the last two years. Energy is increasing, and a trigger gives a less strong disruption for a shorter period (1-2 days instead of a week or weeks) and à social life is regained. It is still fragile but we are hopefull that more progression can be made. As far as known in literature almost no one is treated succesfully with this type of medicin, and it is not likely to be tried out in an acute situation. But knowing that this might be effective, at least in chronic SCLS, mentioning will maybe attribute to the benefit of all. I'm willing to give more information when needed. I'll keep you informed. Hiltjo scls@xs4all.nl

Hiltjo, Interesting that your doctor is using Gleevec. It will be interesting to see how it goes for you. Unfortunately anything we do carries some risks and unknowns. That makes me wonder how many people are being treated for SCLS with drugs/treatments other than T&T or IVIG? I am currently on IVIG every 4 weeks with excellent results. I do not have the chronic form, but more acute attacks when they occurred. If this treatment ever fails me, my doctor always talks about using Rituxin. He is very positive on the results of this drug with other diseases. He believes there is some correlation with the other diseases treated by Rituxin and our SCLS. So my questions to the group: Other treatments besides IVIG and T&T? And has any other doctors discussed or tried Rituxin? A link to info on Rituxin - https://www.navigatingcancer.com/chemotherapy_drugs/rituximab and another : http://www.rituxan.com/index.html?cid=rth_PS_00001065&c=00001065&moc=MBRTNH7005&gclid=CO3MmuahmLcCFYOo4AodkhIAvg Thanks, Barney