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How to Know For Sure When You Are Having An Episode

aporzeca Message
15 Oct 2009, 11:58 AM

I don't know about the rest of you, but in the past year my symptoms of SCLS have kept changing and most of my usual early warnings have disappeared. For example, I no longer get a runny nose and flu-like symptoms, or have gastrointenstinal problems, at the start of an episode. Thus, I have missed some episodes until they got to a very late, life- and limb-threatening stage -- namely, until I became unconscious. And at that point you are usually in shock, are taken to the ER by ambulance, and the doctors on duty there often panic and they start giving you way too much fluid to bring your blood pressure back up, thus putting your limbs at grave risk of destruction (through compartment syndromes) -- or else your life at risk because they have to perform emergency surgery (fasciotomies) to preserve your muscles and nerves. The results are lenghtly, costly, dangerous, painful, scarring and traumatic hospitalizations. This is the worst-case scenario and we MUST try to avoid it. At this point, the several doctors I have consulted in the United States are united in the belief that (a) there is currently no medication capable of fully preventing episodes of SCLS in all patients; and therefore (b) until a cure is found, the realistic goal is to catch episodes of SCLS early -- namely, when the hemoconcentration rises to the 17-19 gm/dL range and blood pressures are not much below the normal range of, say, 120/70 -- and to manage them in a safe, quick, painless, and cost-effective manner. I would like to report that I have recently made good progress in identifying the presence or absence of an episode of SCLS in the following way. First, I now have (and use whenever I feel like it) a sophisticated medical device that measures my hemoconcentration within seconds: I prick one of my fingers and use the 2nd or 3rd drop of blood to fill a so-called microcuvette with some active chemicals in it, which when inserted into the device gives me a fast, objective hemoglobin (Hgb) reading -- no matter where I am or whether it is a night, weekend or holiday. The device is made in Sweden, approved by the FDA for institutional or medical use, and is available to doctors, hospitals and blood banks around the world. (It is used mostly for the opposite purpose, namely, to spot persons with anemia.) It is not yet FDA-approved for home use in the USA, so one of my doctors bought it for himself and then resold it to me at cost. (I later submitted the bill to my health insurance company for reimbursement and, after denying payment at first, they ended up covering most of the cost once they got a letter from my doctor explaining how use of the device would help minimize the kind of frequent, expensive hospitalizations I had been needing – and they had been having to pay for.) The device is called HemoCue Hb 201+ (see it at and it can be obtained by a medical professional in many countries around the world. The device has a one-time cost of about US$850 and a recurring cost consisting of a package of 200 microcuvettes (with 4 containers of 50 each, with a shelf life of about one year when unopened and 3 months once a container is opened) that costs about US$200 each, and which you have to buy one or twice a year, depending on frequency of use. The reading you get is usually a few decimals different than the one a hospital or professional lab gets using venal blood drawn at the same time, but it is accurate enough to give you and your doctors a sufficiently approximate level – and to catch increases in hemoconcentration as they happen (for example, by taking a sample every 3-4 hours when you suspect you may have started a capillary leak episode) at home or on the road. As a result, I no longer need to guess whether I am or am not having an episode, and if by chance I am having an episode, I can get the medical help I need, wherever I am, long before my blood pressure starts to collapse and I go into shock. This may not work for you, but once I identify an abnormal level of Hgb, I call my doctor and he authorizes me to take a stress dose of Prednisone. (I carry Prednisone with me wherever I go.) If my reading is in the 17.5-19.0 range, I take 100mg of Prednisone every 4 hours and test my blood every 3-4 hours, and then go to an outpatient lab for a daily confirmation reading from venal blood for the next 2-3 days, and also go see a doctor in his office. Usually, my hemoconcentration stabilizes during the first 12 hours and then starts to drop back to normal levels, at which point my doctor, after seeing me in person, authorizes me to taper off the Prednisone (e.g., 2 days of 60 mg/day, 2 days of 40mg/day, 2 days of 20mg/day, and 2 days of 10mg/day). If I miss an episode early and my reading is in the dangerous 19-21 range, I call my doctor and he authorizes me to take an even stronger stress dose of Prednisone (150mg of Prednisone) and then he has me go to the nearest Emergency Room, with which he leaves the following instructions: That to prevent my going into shock, they should give me an initial dose of SoluMedrol (methylprednisolone 125mg via IV STAT) and then they should take a blood sample to confirm my HemoCue Hgb reading. If the high reading is confirmed, then I am to be admitted to the hospital’s ICU and are to be given identical, repeat doses of SoluMedrol every 4-6 hours until my Hgb reading starts to come down. If my blood pressure does not collapse, that is it: I am released after 24-36 hours and transitioned to Prednisone on a tapered basis. If my blood pressure starts to drop meaningfully, I am put into the ICU and also given simultaneously, during the initial 12 hours, at least 50ml of albumin, Phenylephrine (or another vasopressor) titrate against blood pressure readings, and less than 2 liters of Saline fluid. Once my blood pressure is restored and my Hgb readings come down, I am released after 36-72 hours and transitioned to Prednisone on a tapered basis. Feel free to discuss these ideas with your own doctor, but my message to you should be clear: Until we know how to prevent SCLS, let us try to catch the episodes early, because they are so much easier, cheaper and faster to overcome when we do.
Jaycee Message
18 Oct 2009, 11:12 PM

I read your post with interest and i believe her in lies the biggest problem - we wait for others to find a cure. I do believe we should take responsibility ourselves as well - I manage my scls quite well myself these days and get complete remission when I watch my diet carefully - chinese medicine takes a different approach and would say the condition is related to too much inner damp which is not transformed adequately - if I stop mucus/damp producing food for at least a month (no wheat, meat or dairy) then only eat them in very small amounts and not every day after that I have no trouble. I would definitley suggest people give it a shot for a month - what have you to lose after all - if it works for you then you wont need the drugs and you wont lose your limbs for sure by being overhydrated by medical staff
tiggrrr027 Message
18 Nov 2009, 07:56 AM

Thankyou arturo for the detailed information. My brother allen does not have episodes as often so this information is invaluable. The details are helpful to us and the doctos in establishing a protocal for the docors to follow. We appreciate the suggestions and history as a lot of treating scls is trial and error and everyone seems to differ a little in frequency of episodes, response to treatment, and preventative medicine. Jaycee, thank u as well for the suggestion. There seems to be some connection to diet so u make a valid point. It is great that everyone is taking time to help each other. Thanks to all who have made it to NIH for study as well. Sharing information is invaluable in the search for a cure and minimally disease management. So thanks to all!!
greipp Message
20 Nov 2009, 04:24 AM

Arturo Thank you again - Early detection goes right to the heart of being able to thwart attacks of SCLS. This approach may become part of our armamentarium for monitoring - not only those with frequent attacks but those who may have infrequent clinical attacks but more frequent subclinical attacks of the sort you have shown us. Your guidelines setting the bar at 18-19 seem wise in your situation and with more testing in more patients may prove to be so for others. As a clinician, I like this approach, We remain in your debt, Phil Greipp
jisenhour Message
23 Nov 2009, 01:18 PM

FYI as a result of Jaycees message above I've stopped eating or drinking anything with Dairy and I've noticed a difference.
clswalt Message
26 Nov 2009, 02:08 AM

Arturo, My primary physician ran a spreadsheet of all my blood tests from December 2007 to present. Thanks for the e-mail on your Hgb references values. I have had at least one blood test each month since they diagnosed me. My doctor insists on a monthly blood evaluation. The Doc plotted all values on the tests and found some interesting things. It turns out that my Hgb levels shot to 17.6 to 18.5 each ER visit last year and all 8 this year. All test values in between ER visits are normal except for aminophylline levels. I have now had 16 weeks of normal life with no episodes what so ever. The daily 1200 mg of aminophylline are working very well for the 16 weeks. I am sleeping as well every night. Again, for some reason, I know when to go in with the strange feeling. The Hgb levels confirmed that I go in in advance of major episodes but not unecessarily. The blood test right after albumin IV also shows that it works well. Hope everyone has a great Thanksgiving Walt and Nancy