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

Arturo C. Porzecanski ( aporzeca )

About me

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

I have had 2 other life- and limb-threatening episodes since then, in April 2007 and March 2009, requiring 2+ weeks of Intensive Care hospitalization to keep my organs alive and emergency fasciotomies to preserve the muscles and nerves I still have.

I have also had 7 episodes of lesser severity (Dec. 2007, June 2008, June 2009, July 2009, September 2009 and two in November 2009), because I realized I was having them early on, which allowed me to get a massive dose of steroids (Prednisone pills and/or injections of Solu-Medrol and Albumin) that effectively stopped the capillary leak phase of SCLS.

Given the increased frequency of my episodes of SCLS, despite having taken the recommended doses of the traditional medications (e.g., Theophylline, Terbutaline and Singulair), I was put on IVIG in November 2009 and have had monthly infusions since then with no adverse side effects whatsoever. So far, so very good: I have had no more episodes of SCLS.

Therefore, I hope that my insurance company will allow me to keep getting infusions of IVIG every month for prevention purposes, because there is growing evidence (especially out of Europe, Canada and now the United States) that IVIG works better than anything else.

My email address is aporzeca@american.edu

You must be a member of this community to ask a question.

ciro asked "

I have SCLS for three years now. At first I only got episodes about once a month, but now they come about every three days. What wil happen when I get them every day?

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I’m so sorry to hear about that, but welcome to our SCLS community! You and your doctors will find a great deal of useful information in the “Disorder Details” and “Disorder Resources” sections of this site.

The short answer to your question is: If you really have SCLS and whatever medications you are taking are not stopping your increasingly more frequent episodes, you are at high risk of organ and/or limb damage — and, ultimately, of death.

Therefore, your doctor should consult and make an appointment for you with one of the experts in this rare illness, and the nearest to you is Dr. Mark Pecker in New York City, see his contact information at the bottom of the “Disorder Details” page. After examining your medical records, he could (a) confirm that you have SCLS and not something else and (b) recommend the appropriate therapy, which would likely be monthly infusions of IVIG.

In the meantime, please put as much of your medical history as possible in your profile page, so we may know more about what you have experienced and what treatments you have already tried, and also pull together all your medical records so they can be studied by one of the specialized doctors you should see.

Amy J asked "

All of my vitals (blood pressure, sugar, etc.) drop except for my pulse, and I retain fluid. It’s very painful most days. The Mayo Clinic in Mankato (MN) wants to start me on physical therapy with compression wraps. Do you know anything about this? Does it work?

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No I don’t, I’m sorry. I hope it works for you. We have several members of this community with chronic fluid retention and swelling problems that do not conform to the usual short-lived, self-reversing episodes typical of SCLS.

Susan F, Vainik asked "

Have you heard from community member Marilyn (maire602)? Her profile reveals that she has not logged in for 4 months, and she has not responded to my email. She was a regular participating member of this virtual community, and I am concerned having not heard from her this long.

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You are right that it is concerning. I have also written to her recently without success. I last heard from her in mid-August, because I was helping her to get on an IVIG therapy. Let’s hope she reconnects with us soon.

Renee asked "

Is there a place where ALL of the SCLS member profiles are listed?

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Unfortunately, no, that’s not how this website was set up — by two young men who did it out of the goodness of their hearts. You have to click on SCLS community user names and see their profiles individually, and then refresh the page and new members will appear, and then refresh as many times as it takes until you see all of them.

Annamarie asked "

I have been through my 3rd set of IVIG infusions and still have many issues. I don’t have the severe swelling as before, but my hands have never returned to normal. I still have much pain throughout my body, and I feel sick to my stomach all the time. My rash-like itch has spread to most of my body. Several joints in my hand feel like they have frozen, and my knees pop when I walk. Bottom line: I feel awful most of the time. My doctor has suggested that I go on disability. Do you have any suggestions on what can help me?

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I am truly sorry to hear about your situation.

It is hard to tell from your description which were pre-existing problems that may or may not be related to SCLS, and which are the more recent, possibly IVIG-related side effects. These effects are generally mild and self-limiting, and occur in the first hour after the infusion begins.

In particular, skin reactions to IVIG are uncommon, and of all the reported rashes, urticaria (red patches and weals in the skin) and a blistering type of eczema are the most usual. This last one often begins a week after exposure to IVIG and goes away 1 to 4 weeks later, depending on the use of topical steroids.

My advice is to see an allergist or reumatologist or immunologist, because they would be able to isolate IVIG-related problems from others and help you, specifically, with your skin and joint problems.

As to going on permanent disability or not, that is a major decision that you should take only once you know which quality-of-life problems are likely to be solved (e.g., by your body becoming used to IVIG) and which do not go away even after trying other medications. Sometimes people on disability become isolated and depressed, but I know of someone in this RareShare community who is on disability but is having a good time — but then, he doesn’t suffer from pain like you do.