Arturo, I was wondering if you have the actual number of patients diagnosed so far with SCLS. (within the US and outside of the states) I realize that not all members of this forum have SCLS, but are family, close friends, and physicians connected with this disorder. thank you. marilyn

As per my count, we've attracted 54 adults who claim to have SCLS, of whom 2 are now deceased, plus the parents of 4 children and 1 teenager. Since quite a few of these 59 have not checked in for a number of months, there may be others who are deceased -- or perhaps they have discovered that they don't have SCLS, after all, and therefore they have lost interest.

Arturo, I have been going through "all" the past topics for the last few days. I do this, once in a while, to see if I missed anything back then that may help now. It is also amazing how many members read the topics. I thought that only the members who reply were reading the new entries. I was very wrong. I think a lot of the members just read, which is great. And since a lot of you read the topics, see below: To ALL: Please do something special for Arturo, as well as yourself. By Christmas, make an entry to this topic and report how you are doing. You have plenty of time between now and Christmas. Since Arturo has done so much for this site, it seems fitting that we ALL (58) report in. Don't worry about translation for some, we have translators. Like the NIKE add, "JUST DO IT"

Walt Breidigan 9/28/10 Now on home IVIG, doing very well. Back to work full time and about 85% back to normal. No episodes since 3/10. Thanks to my Dr. Barrett. Now there is only 55 more to go.

Ruth Nolan 29/09/2010 Two acute attacks last year since my first major attack in October 2005. No medications other than regular pain relief due to surgeries from first attack.(No medication is my choice for now :-) Teaching part time as this helps not to get too tired and run down. Mini attacks of swelling in lower limbs and abdominal area unsure if ISCLS ? 85% health and a positive attitude some days and others 30% health and still a smile :-) Ruth Nolan (Christchurch New Zealand)

When at NIH in August I asked Dr. Druey if I did indeed have scls and he replied that "Yes, I was one of 150 confirmed cases." Of course there have probably been many others but it was felt that a large number had been misdiagnosed as sepsis and didn't make it. Not sure if the "150" number is USA or world but that gives you an idea of the rarity of this disease.

I'll check in for Arturo, Glenn was diagnosed at Mayo by DrG 11/05. He died 4/19/06. I still follow all of you and hope to have something to add on the occasion that it may help someone else.

Hi I am doing okay, I don't let the syndrome run me. I really appreciate this group. I don't responed much because I don't feel I have much to contribute. Take Care Rita

Ritz A lot of members don't say anything by not replying. They still read and keep up with the topics. Anything that you go through is worth reporting. Glad to see that you are OK. 53 more. See you added to our future. Arturo is starting to get a count.

Sharon I hope you are doing well. It breaks my heart when we lose a CLS patient. I do believe that Arturo's efforts will help those of us that have survived. My family (and me) had serious doubts about my future this year until the IVIG. I caught myself giving up early this year as well. Walt If I know how to use rareshare, 32 CLS patients have at least signed in to read topics in the last 30 days. There are 5 that have not checked in for a year, which worries me about there health.

I am so sorry for your loss as well. I was at the Mayo and diagnosed in December 2004. I had 3 very close calls this past December 2009. I really thought I would not be here. It is such a comfort to be able to hear from all of you via this forum. Thank you for all your contributions, it means more than you know to all of us. Arturo, how can we say thanks for all that you contribute to this forum and to us all!!!! You are a blessing to us and I pray you stay in great health!! I am holding for now, a few issues, but I am holding, thank the Lord. Blessings to all, Mary Jane

Just checking in. my husband Jim has been out of hospital for just over three months now, and we are finally settling into our new home (we had to sell our old condo to accomodate Jim's disabilities, and that snowballed into three moves since last spring). I still log in occasionally to read, but don't comment much because I'm exhausted and perhaps a bit depressed. Right now, avoidance is how I cope, It's flu season and I don't want to think about it. Jim is working hard in therapy to regain as much mobility as possible. The permanent wheelchair ordered for him sits unused in the corner of the room and is now the world's most expensive cat bed. Jim has been using crutches instead (although he does need the chair for longer trips as he has no endurance. I've posted a good video of him in therapy. You are welcome to check it out his progress (youtube.com/liesaevans). I hope you are all doing well and staying healhy.

Arturo, My last episode(fourth in two and a half years), was in May 2010. We caught it early and i was checked into the hospital from his office. My icu stay was 4 days, and 5 days on the cardiac floor. Weight gain was 15 pounds. ( in icu a combination of dopamine, dilaudid, iv steroids and ivig, along with controlled fluids. This time rather than a central line in the chest, they did a pic line thru my arm. I feel my internist is tweaking the response to my episodes each time and this has made a big difference compared to the first few episodes. I am now working out (2-3 days a week), mostly cardio to strengthen my heart, seeing a counselor for depression and learning coping skills because of this life altering change with SCLS. I really feel hopeful at this time. sincerely, marilyn meaux

Arturo, Since I have been able to identify and avoid my triggers my episodes have decreased in occurrence and severity. My swelling usually appears in my lower extremities, hands, face and abdomen. I have been able to handle these episodes at home with potassium sparing diurectics. I have never had an episode that required hospitalization. After reading posts by other members I still question my SCLS dignosis at times.

Claude Pfefferlé, born 11.18.1953. Switzerland. SCLS since May 2003 (first schock). 6 other severe attacks in spite of different med. On April 2005 introduction IVIG-treatment : from then on no new leak! Recently operated for shoulder injury without any problem. For Ruth Nolan : a precise BP control is very important to prevent vascular complications (damage of heart- and peripheral arteries, ventricular hypertrophy...) and is of significant value in case of an attack of SCLS. Normal BP is <140 mmHg/<80mmHg. The BP should be controled by drug with short half-life : in case of an attack of SCLS, BP would not be droped too long. With the same idea : normal(-low) weight, normal muscular development, regular sport training, absence of diabetes, no smoking, no alcohol,... are beneficial factors in case of a resuscitation during an attack. Best greetings to Prof. Arturo, all SCLS-Patients in the world and helping community members. Claude (cn.pfefferle@bluewin.ch)

Nolan is doing great. He has had no new attacks since July of 2009, that is when he started on IVIG. He is on dialysis because of the kidney failure he got from low blood pressure. He is on the transplant list at this time. He is now also on high blood pressure medication. It might be his kidneys that are causing his high blood pressure. Thanks to every one that has written. Wendy

HI all, I am doing pretty good overall. I had one bad attack back in Sep 09 and was in the ICUf for 2 days and the hospital for 4 days. I attribute the last attack to thinking I could scale back on my dose of theophylline and also not paying attention to the warning signs. My attacks one in 2003 and then in 2009 seem to be more major but think there are warning signs several days ahead that I need to pay more attention to and get in to the ER for fluids. I am back to taking 600 mg twice a day and in general I seem to be able to tolerate the side effects the insomnia and irritability (my family and friends are pretty tolerant when I get in bad moods and I am lucky to be pretty easy goin which helps ). I hope this helps and Walt thanks for bringing up this topic and Arturo you have realliy done a lot for our community. Hopefully you all and I will stay symton free! All the best, Allen

My son Andrew, 9, has been "initially diagnosed" with SCLS. Although we have been through a lot of tests and 2 attacks in the last 6 months, we are still not sure that this is what it is. He has not had a lot of swelling; his main symptoms are severe stomach pain followed by vomiting, a quick drop in blood pressure and then anaphylactic shock. We now carry an Epi-Pen everywhere and know to have blood drawn during an attack in order to confirm. He has started terbutalene, but theophylline has been hard to come by around here. Will keep you informed!

Oct. 4, 2010 update: I suffered my first attack in May, '07, spending two weeks in ICU of which 8 days was in an induced coma. Was told by my doctor that I had had capillary leak and if I became critically ill again it could reoccur. I was also told it was unlikely I would ever have another episode like that again. I had swollen up but suffered no lasting physical after effects. In January, '09 I had a second attack and again almost died. I was in the ICU three weeks (induced coma) and a total of thirty days in the hospital. I was diagnosed with SCLS and put on theophylline (400mg twice a day) and terbutaline (5 mg daily). Although kidney and liver organs failed during the attack all functions returned to normal. It was difficult bringing me out of the coma; the liver failure causing amonia to leak into my brain. Again no after effects but was told to avoid drinking alcholic beverages. I then suffered heart problems, high blood pressure, tachycardia, etc. but with the placement of a stent, meds. and dieting those are no longer an issue. This year (2010) I have been in the hospital three or four times overnight with mild attacks which required IV's. I also have periodic mild attacks which I ignore when my blood pressure drops to 90 over 60 and I get very fatigued. These reverse themselves in 24 to 48 hours with 5 to 8 pounds of weight gain and I am back on the golf course the next few days. For those of you fighting depression with this disease (and it sounds like many are) I suggest you read Richard Block's book, "Fighting Cancer." Richard Block is the "R" in the H & R Block tax firm (since deceased) but dealing with SCLS carries a lot of the same issues faced by cancer patients (I was diagnosed with lung cancer in 1998; with a less than 4% five year survival rate) and found the book of great help. Finally, for those of you still considering whether or not to go to NIH to participate in the research they are doing on this disease, please contact them and get the details. They need another 15 or so volunteers. I too, would like to add my sincere thanks to Arturo for his tireless efforts to bring us the latest news and his support on this very difficult disease.

Diagnosed w/ SCLS in May 2010 after having many diferent tests done. (Everything from blood, urine, feces & other fluid testing to CT, PET & MRI, to Angiogram, Endoscopy & Colonoscopy & Liver & Bone biopsies done) I was at Cleveland clinic for about three weeks & this was the diagnosis they came up with for me. I am not 100% convinced that SCLS is correct but am mantaining comfort with medications such as 2 types of diuretics, antidepressant & plenty of supplements as well as low salt diet & watching my fluid intake by mouth. I do appreciate all that both Arturo & Walt have done for this group though. Still waiting to hear from NIH about study for SCLS in Maryland. Holding my own - Amy :)

Note to Allenoverland, I too battled with the side effects of theoph. and terabut. Anxiety, irritability,and insomnia were very difficult for me. After 6 months, i took a chance and got off of both, but continually monitored my bp two times a day. The side effects were interfering with any sort of normal life I had had. So this was definitely a personal decision for me and my family. I also informed my internist prior to my decision. By no means am I recommending you to get off of your meds. If you are responding well to them, you should definitely continue this plan. Everyone is different when it comes to these foreign chemicals being introduced to our bodies. One thing you may want to consider( for help with the tera. and theoph. side effects) is asking your doctor about klonozapem, which is a benzodiaprine. (check my spelling) I initially was prescribed this medication years ago to address restless legs syndrome. The newer meds now used for RLS were not yet out at that time. And the side effects of these newer options are much more chancy for me, since i am so sensitive to all meds. I take a very small dose of Klonopin at night. It helps me to rest better, which prior to that, i really wasn't sleeping very well at all. (my mind tends to race, with or without meds) I now can fall asleep more easily and after waking during the night, do not have to watch the clock for hours, until i can drift back off. ( I do not do well with Ambien, promethazine, or antihistamines, in order to help with sleep. All of these add to my RLS.) In addition to relief from RLS, and better sleep, i discovered my frequently occurring migraines had reduced in number per month dramatically. I use Relepax for these.(a very pricey drug) Keep in mind this is a controlled substance. I do not abuse it, nor increase my dosage. My dosage remains the same as it was 8 years ago when i was first prescribed this. And if one uses it on a regular basis, and chooses to get off of it, he must be gradually weaned, as the body has now become dependent upon it..(google these symptoms of withdrawals) Because of my sensitivity to meds, i actually can only take a certain manufacturer's(generic) brand or the" name brand" only. Others, cause depression and crying. (You don't want that). I am also not good with Valium( one dose was given to me on two separate occasions for closed MRI's, again....constant crying... just horrible.) After all of this, i may have actually scared you from even going near the stuff. But, i think it is worth reading up on to help ease the irritability, and insomnia side effects. It may be of some help. You can discuss this with your doctor and see if he is in agreement with this added med. I hope i am not steering anyone in the wrong direction. I just offer what has helped me. YOu know, just comparing notes with one another as we all deal with the changes from SCLS. sincerely, marilyn

Note to Marilyn : Thank you for making me laugh.You have a great sense of humor. I always enjoy and look forward to reading your posts when I sign on nightly. I also look forward to contributing to the discussion forums ( I could write a book on the hypertension discussion)when I am feeling better and not so fatigued. It sounds like we suffer from similiar symptoms. Stay strong, Susan

Note to Marilyn : Thank you for making me laugh.You have a great sense of humor. I always enjoy and look forward to reading your posts when I sign on nightly. I also look forward to contributing to the discussion forums ( I could write a book on the hypertension discussion)when I am feeling better and not so fatigued. It sounds like we suffer from similiar symptoms. Stay strong, Susan

Dear Susan, The fatigue is definitely a downer. It seemed like as soon as i tried to walk the block each day for exercise, within a week i would be down with something and back in bed. It's so discouraging when you finally get the "umph" to actually commit to do something and it falls thru, so quickly. It's like "go to jail, go directly to jail, do not pass go, do not collect $200!" or basically: take one step forward, then two steps back. Since i started an exercise class, i have been doing so much better along with the counseling. Huge difference. I am seeing how much i can do. Often i have an underlying fear of pushing myself too much and ending up with an SCLS episode. But, I can't live in fear, nor can i ignore my body when i am doing more than what i should. It's all about moderation. I personally needed to push thru. i had given up on my life or any hope of joy in my future. These changes, i finally made, may seem small to others, but the direction i have chosen to go in is a huge victory. I thank God, my husband, my family and friends, and all of you who are my partners in battling SCLS. Have a blessed day, Susan.

Hi Marylin, Thanks for the advice. I must admit that I feel very, very lucky that 90% of the time I am able to tolerate the theophylline and my body seems to process it well with only some elevated blood pressure readings. I take a very small dose of lisonopril to counter to counter my mildly elevated blood pressure. And like you I am blessed with a VERY supportive partner Jim, sister Linda (on the Board), Mom and brother as well as a good network of friends. Also, I excercise in moderation and am happy to hear you have started to excercise too. If anything exercise release endophines, our natural "happy drugs" which helps a lot! Take care and know you have a place,thanks to Arturo, where we can all share our stories and thoughts and feelings! All the best, Allen

This a great start in the quest we have in hand: Quest: Let Arturo know how you are!! elganzory from Rome Italy: It is really great to hear that you are still with us and well. Great English too. What is your first name so we can address you. Is it Yaser? To all: Being a guy, it is hard to find the right Christmas present for anyone, let alone a person like Arturo. If everyone replies, we may have found the perfect gift for Arturo. If you don't want to post, click on Arturo's picture and his e-mail can be found. He keeps a good record of all CLS members. We are a small group, as far as syndroms go, so please don't feel awkward.

I would like to report that I have been very fortunate and have had no major attacks in the past two years. Since I began my daily regimen of Terbutaline and Theophylline, my condition has not flared up. I recommend and echo the comments from others who have gone to the Mayo Clinic to see Dr. Greipp and to NIH to see Dr. Druey. These trips are very important to help potentially uncover the root cause of SCLS, not to mention how wonderful and thoughtful both individuals are. Robert Nuara

Robert I have been keeping a time line from everyones comments and you seem to be the one who has endured CLS the longest. Would you please e-mail me your e-mail address. I have a few questions for you in particular. Nothing personal or diving into your private life. I am just really glad to know that there is one of us with 20 years under their belt with CLS. You give us hope for the future. I also have your birthday on my timeline as 2 days younger than I am. Please let me know. wnbreidigan@verizon.net Walt Breidigan