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 recently put on IVIG and have had eight monthly infusions already with no adverse side effects whatsoever. So far, so 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.

irerolper asked "

I have searched the internet for information concerning SCLS for many years and have now become a member of this site. I have some questions but first I would like to ask if it`s OK for me to write in Swedish?

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I’m sorry but I can only answer brief questions in English. We have a lot of information in this site, so please read as much as you can before asking a question. You should realize that I am not a medical doctor and, even if I was, I could not give you a long-distance diagosis or recommend any treatments.

cmanmommy asked "

My 4-year-old son had his 1st undiagnosed attack in March 2009 and his 2nd in December 2009, and it was much worse. Hindsight being 20/20, we now know he had smaller attacks that never escalated out of control. How can you tell withough bloodwork that he has a small leak? My son has a cold, cough or ear infection at least twice a month, not to mention really bad allergies with runny nose, and my husband are I are trying to figure out the difference between them and episodes of SCLS so we can do the right thing for him. Any advice and insight would be great.

Thanks – Jen Thompson

"

As you will read in the Discussion Topic I posted entitled How to Know For Sure When You Are Having An Episode, on the 2nd page of the Discussion Forum listing, the only way for you — in this case, your son — to know for certain is to have his hemoconcentration measured: If it is elevated abnormally, he is having an episode; if it is not, then he is not.

Therefore, you have 2 choices. You can ask your doctor to write out a standing order for a hemoconcentration test (or a more comprehensive so-called CBC test, since both require the same vial of venal blood) and run to the nearest hospital or commercial lab facility (like Quest), and wait for a day or so to get the result, every time you suspect that your son might be having an episode.

The other choice is to ask your doctor to get you a HemoCue device, which allows you to prick your son at will, in the privacy and comfort of your home or wherever he is, and measure his hemoconcentration and get a result within seconds — so you know what is really going on inside of him in real time.

I used to rely on the former approach, as I suppose do 99% of all SCLS patients, but I grew tired of waiting to get my doctor’s paperwork, then waiting in line to have my blood drawn, then waiting for the results to come back, etc. every time I suspected I might be having an episode.

And then, a year ago, I had an episode and didn’t know it, because I had no obvious symptoms, and so by the time I showed up at the hospital, I was nearly dead — and sure enough, I nearly died. So my doctor rightly told me: “Never Again!!! If diabetics can get used to pricking themselves all the time and measuring the sugar in their blood, you can get used to measuring your hemoconcentration.” (At the time, I couldn’t even stand the sight of blood!)

So ever since then I now have this HemoCue device, I’ve overcome my psychological aversion to blood, and I know exactly what is happening inside of me at any point in time. It’s like having a thermometer to know for sure if you’re having a fever or not — and whether it is a life-threatening fever or not.

In sum, I really, really recommend this 2nd aproach to all SCLS patients. Don’t take it from me: discuss it with your own doctor. Until we know how to prevent SCLS, we must try to catch all SCLS episodes early, before our blood pressure collapses, because they are so much easier, cheaper and faster to treat when we do.

[And in case anybody our there is wondering, No, I own no stocks in the company that makes the HemoCue device, or in any medical or pharmaceutical company for that matter, and I neither seek nor would accept any compensation from anybody to promote anything.]

scls asked "

I was first diagnosed in 2007 after 2 weeks in ICU and had a second attack in Jan.2009 which put me in an induced coma and the hospital for 30 days. Just recently (Dec 09 and twice this year) I have been having mild attacks resulting in five to ten lbs of weight gain in 24 hours. So far I have avoided going to the hospital except for the first one in Dec. Have you heard of or experienced any connection between these type of attacks and exercise or over exertion? My recent attacks have followed moderate workouts. I am on the recomended medicines which I think have kept the attacks moderate but was wondering if there was any connection to physical exertion.

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There are reports that stress, upper respiratory infections and other factors may trigger episodes of SCLS, but as I read the latest evidence, the underlying reason for SCLS is an immunological deficiency.

So, of course, you can try to avoid possible triggers like overexertion and other people’s colds — or you can treat the underlying immune deficiency, in which case you can lead a normal life (exert yourself all you want and mingle with people who have colds) without having to worry about getting an episode.

And the best way to do the latter is to get monthly infusions of IVIG, a medication that is the ultimate immune booster. Talk it over with your doctor or have him/her consult with Dr. Greipp at the Mayo Clinic, because IVIG is expensive and most insurance companies at first don’t want to pay for it, so you’re going to need all the support and testimonials you can get.

Also, you may want to obtain through your MD a HemoCue device, which allows you to prick yourself and measure your hemoconcentration on a daily (or more frequent) basis, so you know what is really going on inside you.

Check out the two most relevant Discussion Forum topics: on IVIG and on How to Know For Sure When You Are Having An Episode, on the 2nd page of the Discussion Forum listing.

kbas719 asked "

Hello Arturo, thank you so much for all the information you have provided. My daughter was discharged 1/27/10 from the hospital, and started on theophylline, and terbutaline. Is Dr. Pecker familiar with SCLS?

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Of course! Otherwise I wouldn’t recommend him! He’s the most knowledgeable person about SCLS in all of New York. And don’t forget to ask for a tranquilizer for your daughter if you notice that, as the dosage is increased, she starts to get very nervous. It’s a well-known side effect.

kbas719 asked "

Hello, I am new to RareShare. My 16-year-old daughter is currently in the ICU diagnosed with SCLS. When do you start these traditional medications?

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I’m so sorry to hear about your teenage daughter. SCLS in young people is very rare, so once she is discharged from the hospital, I suggest you get a 2nd opinion to make sure she was diagnosed correctly. I see that you live in the state of New York. If by chance you are within driving distance of Manhattan, I recommend you seek an appointment with Dr. Mark Pecker of Weill Cornell/New York Presbyterian Hospital, tel. 212-746-2210. Once confirmed, your MD will probably suggest that she start taking Theophylline and Singulair, perhaps with a tranquilizer to offset the side effects of the former. If she still has episodes despite taking those meds, the treatment of last resort is IVIG. In the meantime, you must educate her, yourself, and your personal doctor and the ICU staff at the hospital nearest your home about how to treat an episode of SCLS without causing any collateral damage. To start, have them go through this site (see Disorder Details and Disorder Resources).