I very much regret to inform this community that I have just learned that one of our members, Bruno Galien (see _www.rareshare.org/users/bruno_), aged 55, from France, died this past February 25, 2012, from the consequences of a severe episode of SCLS. What is particularly disturbing is that I have also learned from his widow that the late Bruno had 3 severe episodes of the illness (in December 2010, July 2011, and the fatal one in February 2012) -- despite being on a regular, monthly therapy of IVIG at the recommended dosage (2 gr/kg/month) since early 2008. The news that an SCLS patient on IVIG for four years running was still having serious episodes, and even had one that took his life, is very disturbing for all of us SCLS patients who in recent years have been able to normalize our lives thanks to this medication. Coming as it does on the heels of Liesa's post that her husband also recently had a serious episode (see Discussion Topic "An episode despite receiving IVIG" ), including a mere 2 days after receiving his monthly infusion, the implications are clear: *We must not become complacent.* Apparently, Bruno started to feel abnormal on Monday-Tuesday of that week (2/20-21) and kept getting worse in the following days, but he did not end up going to the hospital until that Friday evening, after he had lost consciousness while at home. Despite the supportive care he received upon arrival, he died the next morning (2/25) from pulmonary and cardiac failure.

I am very saddened by this news, and wish to send my sincere sympathy to his widow and his family. translate.google.com Je suis très attristé par ces nouvelles, et que vous souhaitez envoyer mes sincères condoléances à sa veuve et sa famille.

I too am so saddened to hear of Bruno's passing, and my heart goes out to his family. Arturo, are there any new discoveries in the way of treatment beyond IVIG that you might have heard about? Thank you, Ann

Nope, Ann, IVIG is state-of-the-art, and to our knowledge it works better than anything else for those who are having episodes despite having taken other medications. What we are realizing, however, is that it's not 100% effective, and thus how an episode is managed -- or mismanaged -- matters a lot.

Thank you Arturo. Ann

Hi guys! I am so sorry to hear of Bruno passing. I have had 4 IVIG fusions. Dec, Jan, Feb and March. After first one I felt so great!!!! Second one I got sick with virus, was ill the whole month. After my fourth one March 12 and 13 I got very ill. three days after fusion I woke up and my head, neck and chest were bright red! Like a terrible sunburn. The very next day swelling in my chest, head neck began and continued down my arms and hands! It was all concentrated in upper body. My head swelled so bad I looked like monster!! My doctors thought I had to have blood clot!! They said they had never seen reaction to IVIG 3 days post fusion so severe, so it must be a clot? Well after 3 very extensive tests for clots they all came back negative!! They did not know what to think and they still do not. I was put on 80 mg of steroids and kept dosing down. I am currently off for 1 week now. Swelling better still some. What a mess. I am going to see Dr. Druey May 30th. I sent him a picture, he could not believe it!! I know I am very lucky to be here. We are talking about IVIg and will revisit dosing and IVIG when I am better. I was very glad to read that you also had an issue 2 days post fusion. With SCLS people our doctors must think outside of the box!! We are unique! Good health and best wishes to all for a great holiday week-end!! I live in Indy!!! Home of the 500 race this week-end!!! Take care and blessings to all!

Arturo Could you find out what brand of IVIG Bruno used? My insurance switched the brand of IVIG in December, 2011. I used the original brand for the last two years. By March of 2012, my condition returned to a state that I was in before receiving IVIG. 3 weeks ago I receieved the original IVIG and am doing very well. I will post my experience and details after I get a little more time on the original IVIG. For me, it does matter which IVIG you receive. Not sure if this is relevant, but worth mentioning. My thoughts are with Bruno's family.

Hi Walt. I'm sorry to hear that your condition declined again. I look forward to hearing more about your experience when you are well enough to share. My husband used Privigen for 3 months with no episodes. He switched to Gammunex for his April and May transfusion, and had an episode right after his May transfusion. In our case, I don't feel the brand of IVIG was a contributing factor (as my husband had a severe stomach virus), but mentioning it just in case, so we can all compare our notes. :)

I should also mention that my husband did have an episode last year shortly after an infusion with Privigen. He wasn't on monthly treatments at the time, but for us it demonstrates that in our case brand may not be a factor.

Walt: I'm so glad you're back on Privigen and doing much better! As to Bruno, he was receiving CLAIRYG, a French brand of IVIG produced by LBF Biomedicaments which comes in a 5% concentration, rather than the preferred 10% concentration. I don't think it's used much outside France.

Sorry Walt, I mis-read your message the first time around and thought you said IVIG "doesn't" matter for you. To echo Arturo's sentiments, I'm very happy you are doing better since switching back.