Hi everybody,my name is Greg,I'm from Southend(nr London),England. It appears I was struck with SCLS in November 2014. I say 'appears' only because we have no specialists on this condition in the UK,and all the symptoms I experienced pointed toward SCLS and this is what the NHS have diagnosed. I spent a total of 6 weeks in hospital,4 of which were in intensive care and high dependancy wards. I had extensive swelling of my lower legs and both forearms to such an extent it caused a lot of muscle tissue to die,requiring surgeons to remove the dead tissue to the point of almost losing a limb,having read some articles it appears this may be due to myself being administered too many fluids in the first instance(please correct me if I'm wrong. About anything. I often am). The other 2 weeks were spent in another hospital to have skin grafts on the extensive scarring caused by the debrading. I was unable to walk without a zimmer frame for approximately 4 months and had no follow up treatment in this time,other than daily dressing changes. I was eventually contacted and put on a course immunoglobulin infusion (ivig?) of 300cl every 4 weeks. I must point out that I had no subsequent attacks during this time and none up to now. At this point,although my mobility has been quite severely effected I can walk unaided,I do however have 'drop foot' caused by the tendons in my left ankle being damaged and have lost a good deal of dexterity in my right hand(I am,of course right handed). Although I am currently having the immunoglobulin,the NHS want to take me off of it and prescribe me theophylline instead,this sounds like bad news for me and would appreciate any thoughts on this. Anyway I apologise for droning on quite so much and am very pleased to have found somewhere I can refer to and garner support from. Just wish I'd found you sooner. Thanks.

Hello Gregwilkin. You're young and married; you have a dependent family; you made a severe episode of SCLS; your treatment appears effective : do not change it! Claude Pfefferlé

Thanks Claude,great to see you've had no attacks in over 10 years(I read you profile),I fully intend to fight the NHS to keep me on my current treatment and any evidence that immunoglobulin infusions work or even help will only strengthen my argument.

Hey there... greetings from Canada. I am on IVIG and have been since 2012. It is working and life has returned to a new normal for our family of 6. After consulting with the specialists in the States, my Canadian specialists believed the only effective course of treatment was IVIG. There are some side effects but they are manageable for me. I pray that you will get full approval for IVIG. Take care...

Greg, Welcome to our Community! You seem to have had a classic episode of SCLS I'm very sorry for all that you have gone through, but many of us have a lot of scars also -- in both arms and legs -- and have lost muscles, nerves, and thus some mobility, and some of us even wear braces on our legs to help us walk without tripping and falling. But at least you are a survivor, and now you have a 2nd chance. If you would like to receive a package of medical articles about SCLS and about IVIG therapy, please write to me at aporzeca@american.edu After I've emailed it to you, you need to email them on to your primary physician and encourage him/her to read through the articles and then make contact with Dr. Kirk Druey at NIH for any additional assistance. Go to the tab "Disorder Details" and near the bottom you will read about him and find his contact information.

Thanks Lisa and thanks Arturo. Incidentally I have already initiated contact with Dr Druey,initially due to having a friend of a friend who works at NIH,and I'm about to embark on trying to gather all my medical records for them. I will certainly contact you shortly for that package,Arturo. Many thanks

Hello Greg, So sorry to hear of your episode which sounds like classic SCLS. Your main issue is one which is becoming increasingly prominent, that is to prove that theophylline and terbutaline are ABSOLUTELY NOT an alternative to IVIG. It is a fallacy that these might work. They don't. There is NO alternative to IVIG. On another tack, you may bear in mind that the dropped foot may be due to peroneal nerve injury, which may recover with time. Persevere with this. The other issue is that the dose of IVIG. Can only be measured by grams per kg. The cl dose does not make sense as there are varying concentrations of IVIG.. 5%, 10%. Etc. John

Hi Greg, My brother is Guy Allen Overland(allenoverland) and he passed away from a severe episode of SCLS one year ago. He is the last one of our community to have passed from this and I hope it stays that way! He had his first episode in November of 2003 at age 43 with all 4 limbs requiring fasciotimies, a lengthy ICU stay with a ventolator and a few months at a rehab center learning to walk again. I was there and was his medical advocate for the rest of his life. He recovered quite well, although he wore a leg brace, he returned to full time work and travelled the world. Prior to that he was in excellent health and we credited that for saving his life. Back in those days a diagnosis was difficult even though he lived in the washington dc area and had his first episode at a prominent university hospital. In search of a definitive diagnosis we travelled to the Mayo Clinic in Rochester,MN in 2004 to see Dr. Greipp, now retired,who was the only researcher of SCLS in the US at that time to confirm the diagnosis. There was no cure and we were told that the episodes would reoccur. At that time there had been maybe a dozen cases reported to Dr greipp and maybe one or two had survived past the 5 years. The only experimental and somewhat helpful medications were theophylline and terbutaline so of course that is what he took. Somehow he managed to survive for over 10 years only taking the theophylline with it's horrible side effects and only had minor episodes until the one that took his life last year. During that time the SCLS website on Rareshare was created, he became friends with it's founder, Arturo and the studies at NIH were started. He visited Dr. Druey and participated in the studies. Since his episodes were not frequent or severe( he had 3 minor elisodes in 11 years) when IVIG became available he hesitated to change. This hesitation cost him his life. When IVIG first became available it was very expensive,insurance companies did not want to pay for it and it was somewhat inconvenient. The first to try IVIG were those who were having severe, life threatening episodes a few times a year and they really had no choice. Thanks to their bravery it has now become the standard of care for anyone with SCLS, insurance companies pay for it and it has saved many lives. Arturo, Dr. Druey and myself had been trying to persuade my brother to start IVIG and ironically he had the episode that cost him his life about 10 days before his apointment to discuss this with Dr. Druey. I started a thread one year ago with his name " Guy Allen Overland"telling his story because I want to make sure no one else falls into this false sense of security that they are different and do not need IVIG. Everyone with SCLS needs to be on IVIG. I am very sorry that you have what sounds like a classic SCLS diagnosis and have suffered the damaged limbs that my brother also had. However, consider yourself lucky to have survived,that your doctors have started you on IVIG and that you have knowledge and treatments available to you. Do not let anyone tell you that theophylline works- it does not and was never anything more than an experimental treatment. My brothers death confirms this. Arturo will help you with the information needed to make sure they continue to pay for this. It is good that you have contacted Dr. Druey. These are the experts so please take their advice and make sure you stay on the IVIG. My best to you. Linda

Linda, thank you for your post. I am at a crossroads and this helps me with my healthcare decision. I have been off IVIG since November 2015 as I had attacks after treatment in both Oct and Nov. I have been feeling better than normal and my last blood work was the best it has been in years, so I was thinking of stay off IVIG and taking my chances. Your post has changed by mind. Reading your brother's story confirmed what I was thinking the consequence of my actions could be. Thank you Rita

Hi Rita, I read your profile and apparently the IViG stopped your episodes for 5 years but then they started again so I can see your dilemma. I am not a doctor but do have a medical background and my opinions are based on what Arturo and Dr.Druey have told me along with personal experience with my brother. I think another of the reasons my brother was hesitant is because his local hematologist/primary care who monitored his theophylline levels told him he did not need IVIG. This same doctor was not available during any of his episodes and never returned any of my calls even with his final episode. It is so important to have a competent primary care that will keep up to date with SCLS treatments,will reach out to the experts,is available in case of emergency and is dedicated to finding the best treatment for you. Arturo and Dr. Druey who are the experts and were at his bedside for every episode really pushed for him to start IVIG especially in the last couple years. I will tell you that my brother described taking theophylline like " drinking 10 cups of coffee". His blood pressure ran high and my sweet brother was somewhat irritable especially right after he took it. I realize IVIG has side effects and is not 100% effective and there is a small population that it doesn't seem to work for and that unfortunately you may be one of those. I know sometimes they have to adjust the dosage which sounds like your doctors have tried. I know you post alot so I assume your doctors have contacted Dr. Druey and you have contacted Arturo. I would take their advice because they are the experts. My point to Greg and anyone else is that theophylline was always considered experimental and Dr.Greipp told us the episodes would continue even with it. IVIG is the current treatment of choice. If it doesn't work then what?? Doing nothing seems terrifying. Did the IVIG lessen the severity of the episodes or did it make them worse? If it worked for 5 years why did it stop working? Can they adjust the dose again? There is still work to be done in finding the cure for SCLS because of people like you and people like Greg who still ended up being flooded with fluids leaving him with damaged limbs. Even the protocol to follow during an episode is not 100% effective but generally it makes it less severe. I know Dr. Druey and Arturo are committed to finding a cure. Do not give up and find your answers. Like any disease each case is a little different. I think that doing nothing is not the answer. I wish you the best and hope that they can get the IVIG to work again for you. Linda

Hello again folks,sorry I've not replied sooner,have had some Internet problems. Thanks John,I have requested a copy of my medical record(which can take up to 40 days) so I can get a better grasp of what I'm actually being given dosage wise. I have submitted all the relevant forms to my consultant along with pages of info about the condition in order for my records to be released to Dr Druey so that I can participate in the clinical trials at nih. Thankyou,Linda. Sad to hear about your brother and your experience has certainly reinforced my resolve to get the best possible treatment. It can be difficult due to the rarity of it and the lack of any expertise in England only makes it harder. Greg

You are welcome Greg. Hopefully you will be able to continue IVIG and it will continue to work for you. I read your post on " methylene blue" and saw what Arturo had written which I agree with. Just so you and others know the person he was talking about was my brother. Arturo, to whom I will be forever grateful, was at his bedside every day and part of the team that tried everything to save him. I don't want you or others to be alarmed thinking it was another person who had lost the battle. It is good that you are educating yourself especially because there are no experienced doctors near you. Take care! Linda