The new year did not begin as I had hoped. After four years of not having an attack,I had a full blown attack on New Years Day. This is my seventh attack since 1988. Like the other episodes this one was accompanied by an upper respiratory infection. Fortunately, the ER and ICU doctors followed the protocols. If you have not downloaded it, I recommend you do so and keep it with you. The doctors at the hospital consulted with Dr. Mark Pecker at Weil Cornell. They coordinated my treatment. I spent 6 days in ICU and was released two days later. I followed up with Dr. Pecker and he is contemplating putting me on IVIG. We may begin with monitoring my blood levels with the hemocue machine before a final determination is made. Dr. Pecker is consulting with Dr. Druey at NIH. I have a scheduled visit with Dr. Druey ( this will be my visit to NIH) I have had no complications from my episode and have returned to my normal schedule at work and exercise.

I'm so sorry to hear the news of your recent episode and hospitalization, rnuara! Despite the scare, consider yourself extremely lucky to have suffered, yet again, no harm to your extremities or organs. You are in the tiny minority of SCLS patients who have defied the odds, namely, you have survived this life- and limb-threatening illness for nearly a quarter of a century; you've had episodes only once every 3-4 years on average, but without ever sustaining permanent damage; and for the most part you have taken no medication to prevent the episodes, except that lately you've been taking Terbutaline and Theophylline, as per your Profile page. So now you face a choice that many of us never had the luxury to contemplate, because we had frequent and damaging episodes which Terbutaline and Theophylline were unable to prevent -- so we HAD to go on an IVIG therapy because otherwise we were surely going to die, as many before us had died before the news of IVIG's benefits arrived. I don't envy you. On the one hand, you can keep playing Russian Roulette, hoping that future episodes will likewise be infrequent and benign. To be brutally frank, almost all the patients I've known who went for this choice ended up dead -- sooner or later. And in a scientific article published in 2011 describing the experience of French doctors with 28 different SCLS patients over many years, they came to the same conclusion: 7 of their 8 patients who died were taking no medications prior to the episode that killed them, and the 8th had only been taking medications for just 3 weeks. On the other hand, you can go on an IVIG therapy, which has proven highly effective in stopping all episodes over many years except in a couple of patients that we know of, both members of this community: one in Canada, see _Josephite_, and another one in France, _Bruno_, who died last year of a severe episode -- despite being on IVIG and episode-free for several years. However, receiving IVIG is inconvenient and time-consuming; some patients have had to fine-tune the dosage and the time interval between infusions; and some others have had allergic or other reactions. Good luck to you!

So sorry that this has happened to you but the silver lining you can take away is that we have the protocol information available to print from the website ( thanks to arturo) and doctors like dr druey and dr pecker and dr greipp before he retired that have stepped forward to help with investigating and finding treatments for this condition. If you are proactive and informed and bring that information with you and are lucky or insistent enough to have the emergency room doctors read the protocol and follow up with the experts you might come out " relatively unharmed". This is a big improvement from when my brother had his first episode and he is like you with only theophylline and no ivig and may have a minor episode every few years but nothing like his first near death experience. I credit alot to him being more aware, the information and contacts from this website and the correct protocol at the er. I agree with arturo that there are few of you that get to make a choice. If everything seems fine except for a bump in the road here and there why upset the apple cart? At one time I ivig wasn't needed for everyone according to the experts and it was a fight to get the insurance to pay for it. I believe that is changing and the majority of patients are choosing ivig just for the piece of mind and being able to have a normal life. Both ivig and theophylline have side effects and risks and these should be discussed in regard to the benefits outweighing the risks on an individual basis. I would encourage you to follow the recommendations of dr druey and dr pecker. I will also tell you that arturo is very highly thought of in the medical community in regard to his efforts for everyone in this website community and most including me would highly value his opinion. Trust me I have asked and he has graciously offered in times of emergency and I will be eternally grateful to him. Interestingly there used to be alot more interaction on this website before ivig, life threatening episodes were frequent and the prognosis for scls was not good. Because of people coming together ( that includes arturo, dr druey, dr greipp, dr pecker, walt with his tv appearance and every person that wrote in ) having scls is something that people are living with- definitely not an easy road but living. Ivig is definitely a big part of that. Good luck to you.

Dear rnuara, Sorry to hear of your recent attack, but glad to hear you came through it in fine fashion. I am currently on IVIG and have been now for about 1.5 years. I suffered my first episode in Sept 2010, at 43 years of age. I was lucky, as I did not have have any limb damage that was lasting, although I came close, as I had excruciating pain in my lower left leg while in the hospital. The doctors didn't think it was compartment syndrome, but after my later diagnosis from Dr. Greipp, we all realized it was the beginning of something like that. I will never forget what the lead ICU doctor's words when I was leaving the hospital, "We didn't think you were going to 'make it' this week." I had not realized how close to death I had been and how I had been 'the talk' of the hospital as none of the doctors could figure out was causing me to present in this manner. I was a frequent jogger and the doctors agreed that my heart health was probably what allowed me to live that week. My blood was so thick that week that it was hard for them to believe my heart could pump it, I guess. After the diagnosis, I spent the next few months writing letters and meeting with the doctors who had treated me while in the hospital. I wanted all of them and their colleagues to know what SCLS was and if I showed up again, they would know better what to do. I also found a sympathic and knwoledgable hematologist/oncologist here locally that is now monitoring my health. I had a couple of flare ups over the next year that were cured with rest and drinking fluids. I went to the NIH for the study in June of 2011 and Dr. Druey recommended Theophylline as he was concerned about the minor episodes I was having. I finally ended up going to the ER in July of 2011, two days after a routine colonoscopy. With the help of Arturo and Dr. Greipp, I started IVIG at that time and have been on it since - taking the recommened amounts every 4 weeks. I have been completely symptom free ever since. I consider myself very lucky so far, since my insurance is covering it very well. Rnuara, due to your excellent physical health, I would think(as a health novice, of course) that this is very helpful in your battle against SCLS. I wish you well in your decision around starting or not starting IVIG. To be honest, I hope you 'join us' so to speak as a measure for your safetly, but I do understand your hesitancy. I don't have a lot of confidence in the Theophylline regiment, but again that is my opinion, since it did not seem to work for me. If you have any questions about the pros and cons, feel free to reach out.(sorry about the length). Marc

First I realized that my profile did not have my fist name listed it only had my user name. My first name is Robert. Much more personal to have or names. Sorry for the oversight. I appreciate the postings. I too amazed the doctors with my recovery. They did not think I was going to pull through. In my episodes, my blood has been so thick that it was clotting before they got it to the lab. They were actually running with the vials to the lab. In some instances due to the low BP they had trouble finding a vein to draw blood. I am personally in favor of IVIG, waiting to speak to the doctors this week after they have consulted with each other. Can you share with me the side effects from IVIG? Thanks Robert

Robert, The main side effect for me is headaches. Since being given Solumedrol(sp) before each infusion, my headaches are much less than the first couple of times. Now if I get treatments on Thursday and Friday afternoons on one week, I get a headache the following Tuesday, Wednesday or so that lasts for a couple of days. I take Tylenol or Motrin for them and it helps. Other than that I feel kind of 'hung over' and my face is a little flushed the day or two following the IV treatment. I guess another issue is the IV itself. Some nurses have a hard time finding a vein. It really doesn't bother me that much, but a few of them try to convince me to get a "Port" to make it easier. I don't want one at this point but may change my mind in time. Overall I am learning to live with the issues and find much peace of mind knowing the IV is working for me. I must admit that I am battling some mild depression as well, but that got worse when my doctor talked about taking me off the IVIG at one point, plus was having some family problems on top of the SCLS. It gets to be a lot to handle at times. I definitely prefer the IVIG over living in fear, regardless of some minor side effects. Marc

My brother, Allen, was also extremely fit, age43,when he nearly lost his life to his first scls episode In 2003. He had 4 fasciatomies and was in a drug induced coma for about a week fighting for his life. He was also told by the icu doctors the fact that he was very healthy and in above average physical health probably saved his life. He was not diagnosed with scls until 2004 by Dr. Greipp at the mayo clinic. Since my brother had only had the one episode and seemed to be getting better and had been told that his excellent physical health had saved him he asked Dr. Greipp if it would be possible that this was a one time occurrence. After all, anyone faced with a diagnosis like scls would have the tendency and hope to think that if you just go back and take even better care of yourself, get your flu shot( he had missed his that year and had flu like symptoms when it occurred) that this could be a fluke and maybe Dr. Greipp was wrong. We wanted even the tiniest chance that this would never happen again. I can still remember Dr. Greipp's reply and even though I didn't like what he said I now appreciate his brutal honesty. He said " there is no doubt in my mind that it will happen again" . Lucky for my brother he is like you Robert and subsequent episodes have been minor and every few years with only taking theophylline. As I stated above, part of that is being informed, proactive, taking care of yourself and being lucky enough to have knowledgable doctors take care of you and follow the protocols. He has never used a hemocue because his episodes are years apart and the theory is that his results would be normal for years at a time. He does have periodic blood tests with a hemotologist and those are fine.If you decide to use the hemocue on a daily or fairly frequent basis I would be interested on behalf of my brother and maybe others like you all to find out what your results are since you are similar to him in frequency but have had episodes for 24 years as opposed to his 9 years.He also thinks he can tell when one is coming on and if he hydrates and rests that feeling subsides alot of times. He uses a blood pressure cuff to monitor or if he has any doubt he goes to the er, gets fluids and blood drawn and most of the time is fine. It worries me that this might give him a false sense of security and become overconfident in predicting and managing episodes. Those that have frequent life and limb threatening episodes have no choice but to go on ivig but it is frightening to think that the ones that don't may indeed be playing "russian roulette"'. As Dr. Greipp said " this will happen again" and now knowing that ivig is very effective and becoming the treatment of choice for prevention and I believe is given during episodes then maybe everyone should be on it? Another thing I wonder which arturo brought up to me is if repeated episodes, although minor, over a long period of time may wear down your body's ability to recover especially as you age. Is ivig is a good idea just to preven"'wear and tear"? If your immune system isn't as effective and you happen to have a life and limb episode will you be able to recover?From what I understand the protocol , while extremely valuable and for a time it was all we had,only manages and hopefully minimizes the events of an episode,giving guidelines for doctors to follow in how to treat and not to over treat but it is your body that reverses the leak. For someone like you or my brother would ivig help "preserve"'the resistance to that you both appear to have in matters of frequency and severity by only taking theophylline? Or are you both just extremely lucky?These are good questions for the doctors. As more people are surviving with scls for years and decades beyond expectations in the past I am sure these questions will be answered. Arturo said you are lucky to have a choice as he did not. I can tell you in discussing these things with my brother it is a difficult choice because for the most part everything is fine just taking theophylline and a minor bump in the road here or there. Ivig seems like a big inconvenience, costly and is it really necessary for everyone? But then peace of mind has to be worth something not only for you but your family even if your episodes are infrequent. Is there any way to predict how severe the next episode might be? Maybe it is better to take ivig and never have to take that risk. After all Dr . Greipp was right about it happening again and will your luck just run out?? For you lucky few like my brother I hope you take this into consideration, talk to your doctors and weigh your options maybe before your next episode. It would be awesome to prove Dr. Greipp's prediction a thing of the past.)I wish you all the best.

Hello Robert, I just recently had my second attack. I was first diagnosed in Dec. of 2011 at the age of 40. I had a life threatening attack that began as an upper respiratory infection that resulted in a week coma, and fasciatomies on all four limbs. I too was told that the only reason I survived was because of the physical condition my body was in. I am a fitness instructor. I am also one of the patients that did not want to believe this diagnosis. How could I have such a rare disease when I take such great care to be in shape, watch what I eat, and stay current on my physicals and medical health? I visited both the Mayo clinic and the NIH. Both doctors I consulted had very different ideas on treatment. I am lucky that my doctor where I live is a family friend. He is very proactive. After reading all the information on SCLS, he was pushing for me to begin Ivigg treatments. I waffled on the idea of being treated with Ivigg, after all I had only had one attack. I do have four children though, and I wanted peace of mind for them and my husband. So I decided to try a combination of the recommendations from Mayo and NIH and try a longer time between Ivigg treatments (every 3 months) and continue on the theophylline pills. It didn't work. I had my second attack this past November. Staying away from germs with 4 kids is next to impossible. This attack began with the same upper respiratory infection. I was in the hospital for 5 days. This attack did not end up resulting in fasciatomies since the doctors knew what I had and did not flood my body with fluids. Thanks to the efforts of Arturo and the information he armed me with, this time around my recovery was quick. Regardless, my hemoglobin levels were extremely high and once again my organs were in distress. Needless to say, I am now doing monthly Ivigg treatments. I still have trouble with headaches, but we are continually tweaking the system. This month I am switching to splitting my monthly dose in half and getting a dose every 2 weeks. The idea is to keep the Ivigg levels constant in my body, cut down on infusion time, and hopefully alleviate the headaches. My original infusion time took from 9am to 3:30pm once a month and then I was down one to two more days with a severe headache. I do not have a problem with getting an IV. My veins can be tricky sometimes, but I do not want a port as that would interfere with my lifestyle too much. I am still fighting with my insurance company on a hemocue machine. I would just buy it, but I am also fighting with insurance on covering my initial visit to the Mayo clinic. The expense those with diseases must deal with is so unfortunate. Anyway, I wish you luck in your decision on how to treat your SCLS. I hope all of our stories help you make the informed decision you are searching for. I know I consult this site daily in the management of my SCLS. Sincerely, Cristina

Could I just point out thst we should not be lulled into a false sense of security of IVIG eliminates this syndrome. People have died whilst on IVIG so vigulance is still really important.

Thank you all for sharing your stories of SCLS and your individual treatments. I am anxious to speak to Dr.Pecker again later this week after he has consulted with Dr. Druey and the other physician in France. I will post what my treatment plan is. Like everyone else, right now the mental aspect of the attacks and not knowing what may occur inthe future is exasperating for me and my family. Again my sincer appreciation for your posts Robert

Hi all, Arturo once asked me why I participate on this discussion forum being that I am only a sister of someone who has scls especially when he does pretty well with infrequent episodes. As I said previously I am eternally grateful to arturo as well as Dr. Druey,Dr. Greipp and all of you that have taken the time to share and collaberate on their experiences including family members whose loved ones lost their fight. As my brother had his first episode before we had a protocol i witnessed the devastation of scls. An overload of iv fluids complicated things. The emergency / icu personell had very little if any information on scls. After his diagnosis was made 10 months later i became his advocate and have gone " to battle" with emergency room/icu medical personell to insure the protocol was followed. As a result his episodes have been manageable with no obvious permanent damage but that is not without a few frantic calls to arturo, Dr. Druey, the mayo clinic with Dr. Greipp and Dr. Haymons. I have also threatened to tattoo the protocol to my brother's forehead as I do not live in the same state(although I was with him on his first episode and visit to mayo) and he travels quite a bit so much of this has been on the phone. Fortunately he never loses consiousness but there is always some panic involved and as some of you may know it is good to have an advocate that is educated on scls to help you make informed decisions and to help facilitate the emergency personell getting in touch with the appropriate experts and information. A big reason things have become easier with subsequent episodes for those with scls diagnosis, a big part of participation with Dr. Druey and NIH , a good part of why scls is being diagnosed more efficiently and more often (lives are being saved because of awareness and correct protocol)) and new treatments such as ivig are being explored is because of this website. Arturo is our expert and when he was fighting for his life every few months before he started on ivig he had personal reasons to be our hero although I know that is not why he does it. His episodes have subsided( thankfully ) but he knows how far we have come and that the fight is not over even with ivig. Unfotunately it is probably not the cure but it sure has saved a lot of lives and improved quality of life.It gives hope and some sense of peace. Scls does not only take it's toll only on your body. It takes incredible mental toughness to fight especially when you and most of the medical community do not know what you are fighting. There are financial burdens and relationships are affected. Everyone handles the realization of having scls a little differently. The evolution of this website has certainly made it easier. Before ivig there were more postings because people were in crisis more often. I can tell you that my brother does not post often because his episodes are infrequent and benign( thankfully) and he only takes theophylline.He told me he has a hard time reading through all of the stories. I think he tries to go on with life as best he can and to be honest tries not to think about scls every day although he has a splint on one foot and the scars of his fasciatomies so there is always a reminder. Those on ivig that are doing well probably post less for the same reason. I certainly dont blame them and it is the reason i post- maybe as a stand in for my brother although sometimes all I have offer is encouragement. Arturo( and there are others)is quite remarkable in his vigilance with this website. He is very brave and above all extremely compassionate. Even though it is extremely hard to face the reality of scls he is always here gladly offering his expertise especially for the newly diagnosed. I guess for me and probably for him all the rest of you I just don't want the conversation to be over and I want scls to be a big enough deal that the research continues until the cause and cure is found. Robert, you are quite the exception with little treatment and infrequent episodes for great deal of time and I guess I just want to know " how did you do that?". Wishing good things for all of you! Linda

Linda I wish I had something unique to share with the community as to why I have been able to survive the attacks with no lingering effects. The only permanent damage I have is a slight dropped foot. It has not hindered my activities at all. Other than exercising a minimum of 5 hours per week, including my cycling, I have not done anything special. Perhaps Cristina can comment on her physical condition post the episodes? I am curious if anyone else gets a serious bout of uncontrollable chills during the resuscitation phase? Robert

Thank you for your reply Robert. If things are going well there isn't much to say( which is awesome).I know you participate at NIH and so does he. Dr druey has also taken blood samples during an episode as my brother lives in that area. Hopefully that helps them identify why this condition affects people differently. My brother fully recovered from his first episode except for a dropped foot which he wears the splint for. He is still in above average physical health,exercises, eats healthy and continues to travel quite a bit overseas and in the us. He says the theophylline is like drinking 20 cups of coffee I can believe it as he runs circles around the rest of us. He does not waste one minute of life. Other than his first life and limb episode the only other classic episode( with a small amount of swelling, the hypotension and hemoconcentration) happened when he dabbled with his theophylline dose. He was late or missed taking it a couple times as he had worked late but then didn't want to take it at bedtime because it would cause him to be up all night. That episode also progressed somewhat and we suspect it was because they wouldn't let him take his theophylline. In subsequent very minor leaks when he takes the theophyline and follows the protocol the episode is very minor with just a little fluid,maybe some prednisone, albumin and he does not leave the hospital till the blood results and vitals are normal. He has good insurance which allows him to go to the er when needed and he does not hesitate to go if there is any doubt. He also wears a medic alert bracelet that lists scls and contact information and has multiple copies of the protocol everywhere and lots of stashed theophlylline at work, in the car,etc. For those that take or used to take theophylline has a late or skipped dose possibly contributed to an episode. Did you take theophylline during the leak and did it help? Thanks to all that write in Linda

Linda I take 2.5mg of terbutaline twice per day and 450 mg of Theophylline twice a day. I have been on both meds for the last four years. My recent attack was identical to prior episodes. The major difference was that the doctors followed the protocol and were in communication with Dr. Pecker. I still gained about 35 pounds (all fluid) and have since lost all the weight. I am just about back to my full strength. Robert

Thanks Robert . During my brothers first episode they gave him enough fluid that he blew up like a sumo wrestler similar to walt in the "mystery daignosis scls episode". His rhabdomyalasis was severe, he could not even lift a spoon and he could not walk without a walker for a few months.He had dialysis to remove the fluid as there was so much . The second episode about a year and a half later was when he dabbled with the dosage of the theophylline. The protocol was mostly followed( one of my battles because it was the same hospital and the nurse told me on the phone they had his records from the first episode and were going to repeat that treatment- yikes!) . This time he gained maybe 15 pounds due to fluid. He did not have any noticeable physical weakness.. Any other time after that the fluid was not noticeable except for slight swelling in hands or feet or maybe abdomen and the blood tests while being a little off initially( no extreme hypotension or hemoconcentration like a classic scls episode) went back to normal with the prednisone, albumin, theophylline and a small amount of iv fluid and a little wait and see monitoriing vitals, blood levels, urine output, etc. So maybe you are alike only in frequency. Actually I don't know if there are any identical scls cases out there but that may be because it depends alot on what protocol is followed during an episode. I would still be interested to see if they recommend ivig or not and what you decide. Thank you again for your input. Linda

I asked my brother and he said he also has had times where he had the chills. He said he had some issues infrequently with them even before scls diagnosis. I guess if it is possible a virus may be a trigger then fever and chills would be expected. With scls the mobilization of all that fluid and the fact that your immune system has gone into "overdrive" could be a factor in severity. Also could be whatever they are putting in your iv. Linda