Hello. My name is Cristina, I am 40 years old, and I was diagnosed with SCLS on Dec. 8,2011. After a long day in and out of the ER and doctor's offices with no diagnosis, I ended up in the ICU where faciotomies were performed on all four limbs. I was in ICU for 5 days in an induced coma,intubated. 12 hours after being admitted and operated on, the doctors found SCLS through the web and diagnosed me. Luckily during the recruitment phase my organs kicked in ,and I have been recovering ever since. I spent another 7 days in the hospital and was released on a regimen of Theophylline (400mg 2x a day) and Terbutaline (5mg 4x a day). I have an appointment with the Mayo clinic in Feb. I am still battling nerve damage in one of my feet, and have no strength in my lower body muscles. I participate weekly in Physical and Occupational Therapy. A friend in the medical field sent me the information about the clinical study at the NIH. I am in the process of making my doctor aware of the study to see if I qualify. Does anyone have any advice on how to proceed? I am also curious, after reading some of the forums, as to the variety of episodes. I read some stories where people have episodes, but do not end up in the ICU. How often do the attacks happen?( I know it must be different for everyone) I am the first known case in Kansas City and none of the doctors here are familiar with SCLS ,even a little. I don't want to be steered in the wrong direction ,and I definitely do not want to have another attack like the one on Dec. 8th. I am sorry that we are all a part of this group, but I am relieved to not be alone.

Welcome to our SCLS virtual community, Cristina! We are very sorry to hear about your traumatic first episode, but it's great to hear that you survived it with your organs intact and got a diagnosis so quickly! Most of us were by no means as lucky. In these many pages you will find out just about everything that is known about SCLS, and compared to the state of knowledge that prevailed just 5 years ago, believe me, we have come very far, very quickly. I'll just answer one of your questions to begin with, about the different experiences with episodes of capillary leaks. We believe that the severity of the episodes has to do with 3 things: (1) how much plasma the capillaries leak (measured indirectly by how concentrated your blood's hemoglobin, or Hgb, becomes) and for how long they leak; (2) to what extent your organs (and particularly your brain and heart) are capable of functioning despite the drop in oxygen delivery that takes place during an episode; and (3) the dosage and type of medications taken before and during an episode. For example, I had episodes when my Hgb rose from 16 to 19 gr/dL and then subsided, and others where it rose to 21 or even 23 gr/dL before trending down. (The normal range for an adult is somewhere between 13 and 17.) Initially, even the former episodes would land me in the hospital, because I would lose consciousness -- my brain couldn’t function properly. After a time, however, my organs seemed to get used to the episodes, and I wouldn’t pass out unless I had one of the latter, severe leak episodes. We also know that some medications can help to abort or minimize an episode, such as steroids (e.g., prednisone taken orally if an episode is just getting started, otherwise methylprednisolone administered intravenously) or albumin. However, it’s not advisable to take steroids every day to prevent episodes, because their long-term side effects can be worse than the consequences of the episodes themselves. Episode prevention is best accomplished by means of monthly infusions of IVIG (intravenous immunoglobulin), but some patients seem to be helped by a far cheaper and less invasive set of medications called beta agonists, such as theophylline and terbutaline (T & T) – though they have nasty side effects, including irritability, insomnia and fast or irregular heart beats. Since nobody has conducted blind, randomized trials with either medication – and probably never will – we don’t know for sure how or when these medications are effective. In general, most patients are given T & T to begin with, but if they keep having episodes anyway, they are (increasingly quickly) being transitioned to IVIG. And then there are a few lucky patients that go for many years without having an episode even though they are taking no medications at all. We can't explain that as of yet.

Hi Cristina, welcome to the community. I am sorry to hear that you have been diagnosed with SCLS. Your episode is so similar to the last one that i experienced. I had experienced 9 separate episodes where i was hospitalized for a few days in ICU to help recover from the leaking episodes. All this started in June 2007 for me. I was diagnosed in Aug 2010 at the Mayo Clinic in Rochester, MN by Dr Greipp. My last episode was on Dec 8, 2010. You can read my profile for more detail, but in short, I too was placed in a coma and received emergency fasciotomies on all 4 limbs. I was finally released from the hospital on Jan 14, 2011. Had to learn how to stand and walk again. I have been on IVIG since March of 2011 and have not experienced any leaking episodes. Feeling good ever since. I also visited the NIH for testing. If you can get there, that would be a good thing. I think you need your doctors to submit the request to Dr Kirk Druey. If you take a look at the resources included on the website, you will find them very helpful in managing this syndrome. Glenn

Hi Christina Sorry to hear that you have scls but great to get a quick diagnosis. Learn as much as you can from this website as arturo has done a great job of keeping it updated and as he said we have come a long way in a short time. There is a protocol for the emergency room care as well as important information about scls that u will want to make copies of to keep at work, in the house,in your car and try to file it with your local emergency room. This is found under "disorder resources" and arturo just told us it is now published in a medical book and he could send you the pages. Inform your family and friends as sometimes emergency rooms can be busy and you may need an advocate to insure prompt and correct treatment. Dr Kirk Druey at the national institutes of health is/was doing the study. If possible I would encourage you to participate as you will gain a wealth of information as you participate. Dr greipp at mayo clinic in Rochester is retired and I believe dr Susan haymons is no longer seeing scls patients but last I heard they were working on a replacement doctor. These experts could confirm your diagnosiss and also help you and your doctor find the best treatment / prevention for you. My brother take theophylline and does pretty good as long as he takes it regularly and stays hydrated. He has had one major episode like you so far. Others seem to leak more often and the ivig has been a lifesaver for them. I know you have been through alot but just get yourself better(neurontin works for the nerve pain if you haven't tried that),educate yourself and your doctors and hopefully you will do well with managing/preventing future episodes. Good luck. Our thoughts are with you Linda

Christina, I would also print the information under "disorder details" in addition to " disorder resources" and have several copies so u have one close at hand to give to emergency personell. Linda

I have a copy of it in my handbag in a clear plastic ziplock bag. I have also given copies to my close relatives just in case.

Thank you Linda, Glenn, and Arturo for your responses. My doctor is sending all of my information to NIH. He is also wanting to start me on IVIG.I will start the research with my insurance today. I may keep my appointment with the Mayo Clinic because it is next month. They may be able to answer some questions before or if I can get into the NIH. Do you think making an appointment with Dr. Pecker is something someone with SCLS should do? I have many questions that are probably better suited to ask a doctor, but I know many of you have been living with SCLS for a while and may have some answers through experience. Should treatment be either IVIG or T&T? Does anyone do both? Do most attacks present themselves in the same way? In other words will I know if I am having an episode. I will never forget how I felt the day of Dec. 8th, but wonder if my body will warn me like that again? Thank you again for taking the time to reach out to me. Cristina

Hi Christina The trip to mayo could be beneficial as they have treated scls patients in the past and other than dr druey at NIH will have information in regard to that. Dr greipp and dr haymons worked in hematology so I assume you would want to see a doctor in that department. When my brother went there he was treated very well. They are very organized and do a good job of communicating between the specialty doctors within the clinic and overseeing your patient care as a " whole person" They gave him his a scls diagnosis and put him on theophylline. He has also seen dr druey a few times for the study and has had a great experience there. The theophylline has worked well for him and so because he has had only one life threatening episode they have not put him on ivig. He takes good care of himself, rests when he gets tired, gets a flu shot every year ,and does have a blood pressure cuff and an epi pen at home. He has gone to the er a couple times when he just did not feel right and as long as they give him judicious fluids iv along with steroids and sometimes albumin and later a little a diuretic to help get rid of the excess fluid he does ok with them just monitoring him. His symptoms are cold hands or feet, fatigue, swelling in extremeties and also a drop in blood pressure. If he is not sure he just goes to the er where they can check his vitals and do some blood work to see if he has hemoconcenration of his red blood cells,'check his albumin levels and some other things to see if it looks like he is having an episode. His first episode was like yours but because they probably over hydrated him in attempt to save his life he had the fasciatomies etc. I don't think he has ever felt as bad as that first time but that is where prevention and you educating yourself, your family and your health care providers come into play. Some patients have episodes more often and the theophylline does not prevent them or they cant tolerate the side effects so they take the ivig which has been very successful in preventing episodes and giving frequent "leakers" peace of mind and mostly a pretty normal life I believe you only take one or the other and a few patients take nothing. Everyone seems to be a little different. Your doctor seems like he is proactive and that is great so you are on the right track. Thanks to this arturo, this website, dr greipp and the mayo doctors and dr druey the treatment and awareness of scls has come a long way. Good luck with everything Linda

I am the person on this site that keeps trying herbs and supplements. Several people have tried horse chestnut...it helped me but I have not heard back from the others on this site. I also think I have been helped by Ceylon Cinnamon. But don't risk your life. I have naturalpath that I am working with. A new supplement Germanium sounds promising and I might try it. But you need really good care. Leg swelling can also signal congestive heart failure or a blood clot. There is one lady from this forum who wrote a great letter about being helped and it was reprinted on the 26blue.com website. She went to Dr. Tran. "Dr Tran website":http://www.wellspringseminars.com Just keep in touch on this website so that others can dialog with you about their own experiences. Years ago, I went to one hospital, was admitted for a few days, and then discharged with the comment that they did not know what was wrong with me so they were not able to help me. I went to another hospital two days later, almost dead. That doctor did not know what to do either but his approach was simple...keep the vitals working as best as possible and hope the body can repair itself. It may not have been the best but at least I received some medical intervention.

Rivcal I tried the horse chestnut but for me it did not help unfortunately. That is not to say it won't help for anybody else. I have not tried anything else natural except raw egg whites in large quantities which may have helped. It is very difficult to know if it was regression to the mean and would have been improved anyway, or the actual egg whites. Being raw, fresh egg whites, I was a bit concerned about the possibility of getting salmonella.My theory was that I may be receiving albumin hypothetically but it is a far call..... I hope you have printed out the ICU instructions and protocol that Arturo has been working on,on the other thread-it is invaluable

cristina, my husband was diagnosed this past feb 2011 with CLS. he was having episodes for about three years prior until we started seeing a hemo doctor.she diagnosed it in feb and after joining the community board, Arturo, we paid a visit to Dr. Pecker in ny cornell. unfortunately, but fortunate for us my husband had an episode right in his office. was admitted immediately across in the hospital and received his first IVIG within two days. dr. pecker insisted we had to visit NIH. we had another treatment in beginning of jan and then left for NIH. he is feeling great., slight headache but Arturo says that will pass. our suggestion definately see Dr. Pecker, definately NIH, and use all information available. the community board and this website is the best thing that happened for us. Good luck and keep positive. we'll be thinking of you mr & mrs. Custom.

Trying to connect to Window....you don't have an email on file. Germanium claims to be good for lots of the symptoms we have. One should do a hair analysis to know for sure. I have put off doing one because I don't want to spend family money. After writing it here, it sounds penny wise with dollar foolish but I still don't want to spend the money. Because there are different types of germaium, a person has to buy from one of the top health food brands. Germanium is also in garlic and shitake mushrooms. Of course, these have to be grown in rich soil. "Website for it":http://www.janethull.com/newsletter/0207/the_health_benefits_of_germanium_1.php I know you tried horse chestnut. How many bottles did you use before you decided it was not improving anything? Have you tried Food Grade Diatomaceous Earth? It is a health booster and cures almost everything. It is very inexpensive. "Website for it":http://www.wolfcreekranch.net/diatomaceous_earth.html I am not saying that it is a cure for us but it gets rid of so many other problems that our lives are better. Plus, it sweeps the arteries, veins, and capillaries.

I tried just the one bottle of the horse chestnut and saw no improvement.Does it require more than one bottle to have an effect? I have not tried any of the others you mention. The trouble is there are so many natural health remedies out there, I could spend a fortune and get nowhere. I met somebody the other day who said a product called Limitless may help- a relative of Prolain but I could be throwing money down a dark hole too Thanks for your suggestions. I will certainly give some of these a try but it will be one by one otherwise I won't know what is helping and what is not.

To windows... you are right that it is easy to pay out a fortune. Food Grade Diatomaceous Earth is cheap. The horsechestnut should be on the cheap side also. You took one bottle of horse chestnut...did you have an episode while on it? Unfortunately, health does not feel like anything. Being sick obviously feels bad. So the chestnut could have worked and felt like nothing. I wish health felt exhilerating but it doesn't. People become drug addicts to feel exhilerated. Feeling bad and feeling exhilerated both are not health. As it says on the 26blue.com website, swimming appears to be the only exercise that works for us.

You are quite right. health does not feel like anything. It is like happiness. You only know when you don't have it.But if you never had sadness, you would not appreciate happiness:) Sorry Arturo, I digress. I have leaks several times a day as I am a chronic leaker so it was difficult to tell but I still had chronic leaking whilst on it I have started IVIG now so that confuses the issue even more