I recently just barely survived my third very serious episode of SCLS, and since I have learned a few things along my four-year journey coping with SCLS -- by reading most of the relevant literature, speaking to many scientists and medical doctors, and from the information that you all have provided -- I thought I would share with you my tentative, personal conclusions about this mysterious illness. Keep in mind that I am no scientist -- never mind a medical doctor -- so please do not consider the following to be medical advice. These are ideas for you to discuss with your own doctors.
1. How is it that we catch SCLS? I think we experience some kind of mid-life genetic mutation, that all of a sudden weakens our immune system and makes us vulnerable to colds, the flu, stomach viruses, stress, etc. and triggers these (curiously self-reversing) systemic capillary leak episodes.
2. Can SCLS be cured? I think it is best to think of SCLS as incurable, and thus it behooves us to remain forever vigilant. To those of you who have not had an episode in several years I say: Congratulations and I wish you the best – but don’t become complacent, as I became, and do plan for a possible new episode. As you can read in this website, and in various published articles, SCLS patients have tried all kinds of medications and procedures and most of us, sooner or later, have been disappointed by the results. In my case, I have now stopped taking Theophylline and Terbutaline because, as is also true of so many other patients, these medications neither prevented nor lessened my last episode -- and, besides, they have unpleasant side-effects.
3. Can an episode of SCLS be stopped or minimized once it has started? I think it can be, provided it is identified during the first 24-48 hours, before the capillary walls leak massively -- which they tend to do in the following 24-48 hours. Since most of us are given strong doses of steroids and other medications once we are so sick that we must be hospitalized, it may make sense to take one or more of these medications at home as early as possible. In my case, my doctors decided to have me take high doses of a steroid (Prednisone) early on, and in so doing I have succeeded in minimizing two episodes of SCLS. I now carry Prednisone with me wherever I go.
4. If early detection matters, how can we confirm that we are having an episode of SCLS? I used to think that I could recognize it by its symptoms (in my case, a runny nose and a flu), but ahead of my last episode, I had gastrointestinal discomfort (a stomach flu) – and therefore I missed it until it was too late and I fell unconscious. I also thought that I could spot an episode by measuring my blood pressure, but I have learned that it usually does not drop much until very late in an episode, when the blood pressure collapses -- and so do I. Therefore, blood pressure readings do not provide me, at least, an early-enough warning.
5. So, do I have any new ideas? Yes, what I am trying now, at the suggestion of my doctors, is a portable device that allows me to prick my finger, take a blood sample, and measure hemoglobin concentration in seconds. It is supposed to allow me to detect the kind of upward trend in hemoconcentration that takes place during every episode of SCLS right from the beginning. These devices are accurate enough to be used in blood banks and hospitals, but they are expensive (about $1,000, though you may be reimbursed by your medical insurance company if you have a doctor’s prescription for it) and they are not licensed for home use in the United States. (I purchased mine via a compassionate doctor who ordered it for himself.) The one I bought is called HemoCue, is made in Sweden, you cannot buy it directly, and you can read about it at www.hemocue.com/index.php?page=3004. It is sure better than waiting for hours at an outpatient laboratory for a blood draw and the results, never mind going to and waiting for hours in the admissions area of a hospital emergency room during the night or a weekend -- just because you don’t feel quite right and you suspect you might be having an episode.
6. How does one make sure that the ER and ICU doctors will admit you and will know how to help you during a serious episode of SCLS? Since I tend to live in and travel between New York, Washington DC, and central Pennsylvania, I have a doctor in each location that I go visit for a check-up whenever I am in town. That way, there is someone I can call on in an emergency who knows me and will get me admitted to the ICU without delay. The next problem is how to make sure that the ICU doctors will know what to do. This is, I have found, the bigger challenge, because ER and ICU doctors rotate, so chances are you will not get the same doctor who helped you last time -- and thus they will be completely ignorant about SCLS. What I do, especially when I visit a new area, is take with me photocopies of the material on SCLS that is now in the NORD website (http://tinyurl.com/omdvew), in the Mayo Clinic website (http://tinyurl.com/qtkqt6), and in this RareShare website (www.rareshare.org/communities/systemic-capillary-leak-syndrome/details), and I give it to the ICU doctor who is on duty. I also have a one-page instructions sheet written by one of my doctors, which we are perfecting and will soon make freely available to the RareShare SCLS community. I suggest you prepare yourself and do something similar in case you are admitted to the hospital -- even one you have been to previously -- but I can tell you from painful personal experience that there is no guarantee that the ICU doctors will take the advice they are given. My last set of ICU doctors nearly killed me trying to save me.