Just wanted to update every one on Nolan's use of IVIG. It has been a miracle for him. He was having bloating and low blood pressure every five to six days. Some times the episodes were not too bad and sometimes they would send him to the ICU at our local hospital or life flights to a major medical center. After trying all the medications recommended for SCLS he was started on IVIG. His first dose was given in July of 2009. He gets it every month now. He has had no episodes since then. It has been a literal life saver! If you are still having frequent or severe episodes you need to get on IVIG. Nolan was started on it during an episode. Our insurance approved it when he improved after the the first dose. This is an expensive treatment but difinately worth it. Wendy

Bonjour Wendy, c'est exacetement la même chose pour moi. Durant 8 mois, soit de Janvier à Août 2008, j'avais des épisode à tous les semaines ou presque. Depuis Aout 2008, je me rend à l'hopital à tous les mois afin de recevoir une dose de IVIG. J'ai même du plaisir durant mon séjour, j'apporte des films, des livres... je prends du temps pour moi. Mon traitement est d'une durée de 5 heures par jour durant 2 jours. comme Nolan, les IVIG ont transformés ma vie: maintenant, je passe de bon temps avec mon conjoint et mes enfants, je travaille, je fais de l'exercice tous les jours et j'ai énormément d'énergie. C'est merveilleux. Josée.

Rough translation of what Josée, from Quebec, wrote: This is exactly the same thing I experienced. During 8 months, from January to August 2008, I had episodes every week or thereabouts. Since then, I go back to the hopital every month in order to receive an infusion of IVIG. I have a good time during my stay, because I bring movies or books with me, and it's like time I have for myself. The [intravenous] infusion takes about 5 hours a day during 2 days. As in Nolan's case, IVIG has transformed my life: now I have a good time with my spouse and children, I work, I exercise every day, and I have enormous energy. It is wonderful!

So far my experience has been the same. I had weekly episodes that ranged from having a hard time walking up to time in the ICU. I'm now in my second month of IVIG treatments and I've had no episodes that cause any impact at all since I started. My treatment has been administered in a single day over 5 or so hours and I've felt no ill effects from the treatment. It is extremely freeing as Arturo has said. I can't get over how normal I feel. Its pretty amazing.

Bonjour, je vais à l'hopital ce mercredi. cela fait maintenant 1 an et 9 mois. et tout va très bien. c'est encourrageant. Josée

Rough translation of what Josée, from Quebec, wrote: I'm due to go to the hospital [for my IVIG infusion] this Wednesday. It is now 1 year and 9 months that everything has been going well. It's encouraging.

Hello everyone from Walt. I hope all are feeling well. First I have to give a personal thank you to Arturo, my friend. Thank you for the path that I am on. Saturday 8/20 will be my 5th IVIG treatment. Things are so much better and life has some additional quality that has been missing for some time. I am now on my way back to working full time again. I have waited to post, because the first two treatments really did not change things for me and the way I have been feeling. Fatigue, dizziness and confusion have given me a hard few months. After the 3rd IVIG, it was amazing, Things have since been getting better each week. I am also weaned off of my aminopylline. I take only the IVIG treatments. The major benefit of the IVIG was the decrease in fatigue and the stopping the leaks. Now the other symptoms seem to be reduced as well. The IVIG IS THE BEST thing right now for the CLS. And with that, I thank Arturo for doing such a great job in his search for helping us all. My last episode was March 20. I spent two days in the ICU. The albumin they give me did not seem to help me as in the past. No episodes since that day with the IVIG. Arturo told me to be patient and that it may take longer than others. He was right. So if anyone is taking the IVIG, you may need several treatments before you see the benefit. Each treatment so far has given something back. My physical status is much better. In my case, there is no hemoglobin level to check. my Family Doctor hung in there with me and now it has payed off. I am not sure how long I will get the IVIG but I will deal with it as it comes. Walt Breidigan

Walt, Its great to hear you're on the treatments and its working for you. Every day for me is a gift now and I agree that IVIG treatments can change a life.

Hi Walt, I am currently in the ICU, second time this year. They have already used over 10 viles of albumin. Now they have said that I will need a port as my veins are collasping even during insertion of a centeral ine. The doctors have said it is getting to dangerous to stick me. Hopefully the port will help with this. I am starting to wonder if they will have to change me to IVIG in the future. I know they were considering it in the beginning but decided to go with the t & t pills instead. I am still working part time but am I starting to wonder about Socail Security. Has any one looked into this as an option to working? Ritz

Dear Ritz: Please don't give up on your job and your life! Wendy's husband, Josée, Walt, Jisenhour, I and many others have been at the point you find yourself in, and we have moved on for the better. If theophylline, terbutaline and albumin are not working for you, the time has come for you to insist that your doctors and insurance company give monthly infusions of IVIG a try. There is growing evidence that IVIG has worked for many SCLS patients in Europe, and is proving largely successful among patients in North America. Start by pointing your MDs to a new case report that has just been published: Pierre Abgueguen et al., “Immunoglobulins for treatment of systemic capillary leak syndrome,” American Journal of Medicine, 2010, 123 (6), pp. e3-4, and also to Marc Lambert et al., “High-dose intravenous immunoglobulins dramatically reverse systemic capillary leak syndrome,” Critical Care Medicine, 2008, 36 (7), pp. 1-4. Links to these and other items appear under Disorder Resources in this website. And there are other articles favorable to IVIG treatments in the pipeline. I will gladly send you these and other supporting materials and have your doctors speak to mine or to others who have witnessed the excellent results that IVIG can have when nothing else seems to work. My email is aporzeca@american.edu.

Ritz Give me a call some time to discuss your comments. 412-831-3526. This is my two cents on your comment about IVIG. We are the same age. I am 4 months older according to your bio. My first episode was 2/05, yours 2007. My first 3 years of CLS were episode free. I had no symptoms. After the 2nd, the last 2 1/2 years have been episodes which are coming more frequent. In fact i have been off work because they did not stop this year. I noticed that there are many that have continuous problems like fatigue, dizziness and a not so clear feeling. I am concerned that we take hits during low blood pressures that may affect us later. My guess is that they will come more often. I agree with Arturo that the IVIG is the best way to stay alive. In 2008 and 2009, I thought that my Albumin IV's would keep me feeling normal and ICU free. That was not the case. I was in the ICU March 20. I have not been back since IVIG. The more stable blood pressure is why i think IVIG makes that much difference. Except for Robert, I have not read about anyone that has had cls for more than 7 years from today. So we are all the new group of CLS patients as far as I have read. I would strongly suggest having the IVIG approved for 4 months minimum and then see where you are. Your comment about work has not been discussed before. It should be, it is an important topic. The most important thing to us is health care. You need some means of keeping your health care, wether it be employment or disability or medicare.

Hi Gentleman, Thank you for your replies. I was mostly just wondering what others have done about work. I am not ready to throw in the towel yet. I work with college students and I have way to much fun with them. Walt I will call went I am doing better. I am still in the ICU. They used 13 + bottles of albumin and those were in the ICU, I was on a general floor first and I don't know the count there. THe leak is under control,but my left lung keeps filling with fuild. They using laxtics and take oxgen on and off. It is hard for me to talk right now. I will check with my doctor and Dr Greipp on the IVIG. If remember correctly my insurance is oaky with the IVIG if needed, Take Care Rita

Rita Just checking on you to see how you are doing. Hope your home and safe. I can honestly say that you should try the IVIG. My 2 days a month are worth the result. After my 4th treatment, I would say this is the best I have been for 8 months. I am trying to get it in one day like Jeff H is. Walt

HI Walt, I was in for a total of 12 days for the two visits. I have several follow up appointment set up, Now that it is jsujt after an attack I remember that me eyesight gets affected after each attack, it takes a month or two for it to go back to normal. Now that you are in the IVIG you are feeling much better. My doctors are still reluctant to make the change just yet. They are going to install a medi port as it took iver an hour to start a center line, They couldn't get a pic line started. I am slow getting my engery back this time. Twisting a knee isn't helping matters. Rita

IVIG Update - Walt Breidigan - treatment 5 I received my 5th IVIG treatment in my home the last 2 days. Nancy worked out the details and it makes it much more convenient than the infusion center. I keep the IV machine at home now. This is great and it is less expensive for the insurance company. I returned to work full time last week after being off for 4 months. I could ramble on about IVIG, but this is my update: For those of you that leak continuously or frequent, PRESS HARD to get the IVIG now. Arturo pressed me very hard for months about trying the IVIG. He is clearly, without a doubt, correct. THERE IS NO REASON FOR A CLS PATIENT NOT HAVING THE IVIG TREATMENTS. If you are having trouble with the approval, have your Doctor keep pressing the insurance company. If you do not have insurance or can't get the approval, contact your representative. They will help. I also think that Mayo and the NIH should take a serious look at CLS and IVIG. I also believe our number of known CLS patients will reach 150-200 by Christmas looking at the number of new patients over the last few months. To Arturo, My family and I again thank you for your persistance on putting pressure on me about the IVIG. Nancy is now 100% on board with the IVIG.

I would like to know from everyone who has been on IVIG how many months of treatments did it take to stop your attacks. I was on IVIG for three months with no decrease in my attacks, every two weeks. After consulting with Dr. Greipp we decided to stop the IVIG. If there is more information out there suggesting that it may take more than three months to work I would be willing to try it again. I will be seeing Dr. Greipp next month to discuss this. Thanks Heptagona (Steve)

I got your email the other day, Walt. thanks for the advice. And i am so encouraged to hear how well you have responded to the ivig. I, like you, need to be pushed. When you battle with scls for a while, its almost like you don't want to get your hopes up- only to be dashed again. Like the last ditch effort doesn't even help. Does that make sense to anyone? I am going to really get my papers in order, Arturo's, Dr.Greipp, my internist..again. I should not put this off any longer with the holiday's coming upon us, as this seems to be a stressful time which adds to my episode occurrence. Thank you Arturo, Walt, and Nancy. We all need this type of support and guidance when we begin giving up the fight against scls.

To Steve (Heptagona): I'll give you a longer but more informative answer. When I met more than three years ago with the French doctor (Zahir Amoura) who had pioneered the use of IVIG in European patients with SCLS, he told me that in his experience (a) it didn't work in every case, but it worked more often than anything else; and (b) that it didn't always work from the first infusion, but sometimes it required many months of infusions -- up to a year, he told me, before the treatments finally kick in. I understand that Dr. Amoura is trying right now to publish his results, which encompass experience with more than 40 European patients, many of them on IVIG during more than a decade. (Incidentally, one of his patients is a member of this community, Claude53.) The results detailed in his paper reportedly are consistent with what he told me back then. Other than that, all we have to go by is 2 published papers by other French doctors (see the Disorder Resources section); a paper that my doctors are drafting and which will cover my experience and that of 2 other North American patients who are members of this RareShare community; and the testimonials freely provided by several of us here. For this random group of about 10 patients in Europe, Canada and the USA, the improvement with IVIG has either been immediate or became evident within, say, 3 months. To my knowledge, IVIG boosts one's immunity and does no harm (except for minor side effects), so to the extent that your insurance will pay for some additional infusions, what have you got to lose from getting some more of them?

Steve In my case history, there are major leaks and minor leaks. My last major was in March (passed out and taken to the ICU), with IVIG starting in April. During March, April and May, I leaked continuously to some extent, but what I explain as a minor leak (no hospital visits). My veins were somewhat collapsed during those months, very fatigued and usually dizzy. After the 3rd IVIG, this feeling started going away. I always wished for a normal day. The normal days started after the 3rd treatment. Now I have a very close to normal day, everyday. My question is: What kind of episodes were you having after the IVIG treatments versus before the treatments. It is obvious that we all have slightly different symptoms of CLS. Walt

I am replying to Ritz I applyed for Social Security back in June - as I too enjoyed my part-time job but was off for 6 months. I received notice last week that I WILL receive benefits to some extent and you ARE able to continue working part time! My advise about that is to APPLY - be very detailed, and do everything that they require for the evaluation! It is definitely worth is as this will help with medical costs while I was hospitalized & the time I have been out of work. If possible - try to get unemployment for the time you were off too. I was denied claim for that but every case is different. On another note: the NIH has received my liver biopsies & they are testing them. We should know something this week as to wether I am going to NIH for the CLS study being conducted. Has anyone gone thru this that is reading this now? I am curious as to what kinds of tests & procedured thay will be performing? God Bless - Amy (jofusmuffler)

Walt, Thank you for the information. When I leak my head starts to tingle, my mouth becomes very dry, I am extremely fatigued and I have orthostatic hypotension 50's to 90's. I usually go to bed wake up in the morning and go to Urgent Care for IV fluids. I usually take 4 to 6 liters of fluids over 3 to 4 hours. I go home and rest and will feel stronger in a couple of days. My attacks occur on my weekends off from work. Rarely do I have to miss any work. Since my work schedule has changed to having every weekend off I have started to have minor attacks on the opposite weekends. These attacks only require bedrest for 24 hours and PO fluids. All of these comments from this commumnity will help me when I see Dr. Greipp in October. Fortunately for me is that Dr. Greipp is only an hour or so from my house. I have been to Mayo numerous times to see him. In the past I have tried all of the known meds (terbutaline, theodur, singulair and zocor. We even tried Revlimid for 6 months. I have had this disease for 13 years. Thank you to all of the community members, and especially Arturo, who continue to strive to help each other out.