Systemic Capillary Leak Syndrome Questions & Answers - Arturo C. Porzecanski

Arturo C. Porzecanski ( aporzeca )

About me

I had my first episode of SCLS in November 2005, and was very lucky to have survived it (though with permanent disabilities) and to have been diagnosed correctly within days.

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claude53 asked "

According to the french medical literature concerning the treatment of the SCLS by intravenous Immunoglobulines (IvIg), there are 2 different approaches: 1. preventive treatment (by monthly injection of Ig). 2. treatment of an acute crisis per perfusion of Ig as soon as the first symptoms appear. Which is the American experiment on this subject? Do You know how many Patients with SCLS profit from the treatment by IvIg in the USA? I am not informed of another Patient in Switzerland (7 million inhabitants) exept me. Which treatment do you have personally? I thank you for your answer.

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As far as I know, there is no ongoing “American experiment” using IVIg either during acute episodes or for prevention purposes. There are 4 reasons for this: 1) Dr. Greipp of the Mayo Clinic, the country’s leading authority on SCLS with whom everyone consults, has not endorsed it; 2) no long-term, convincing study of experimental treatments (in Europe) has been published as of yet; 3) IVIg infusions often lead to unpleasant and costly medical complications; and 4) last but not least, the cost is prohibitive (at least $10,000/injection) and no U.S. insurance company is likely to cover it unless conditions 1-3 have been met. Thus, most patients take various combinations and dosages of Theophylline, Terbutaline and a leukotrine antagonist like Singulair to minimize the frequency and severity of episodes, and are given stress doses of steroids (e.g., Prednisone) as early as possible during an episode.

meldumais asked "

I see in your discussion you talk about the Mayo Clinic and Doctor Greipp, so how can we get in touch with him? Do we need to be referred to him by a Doctor? Do we know if they other people in Canada have this disease? Thank you so much.

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Yes, the proper way to approach a consultation with Mayo’s Dr. Greipp is to have your own doctor contact him directly by telephone, so that he/she may be able to tell him precisely what diagnostic, treatment, prevention or other issues need his expert help.

I don’t recall from the literature that any doctors in Canada have published about SCLS cases in recent years, but that doesn’t mean that there are no cases. For example, most of us survivors of SCLS have not been written about in the medical literature, so I’m sure the case you know about is not the first or only one. SCLS is very rare but also often misdiagnosed.