I am about to begin IVIG treatment, insurance permitting. I have only had one acute attack, no other symptoms ever that I am aware of, that landed me in the hospital for 12days, fasciotomies on both arms and legs, induced coma for 5 days, and still trying to recover. My doctor is proactive and wants me to start IVIG treatment right away. I am ready, because I do not want to have another attack like my first one. My question is to those of you already on IVIG. Are there any markers that let your doctor know that you are improving ( other than no more attacks)? What should we be looking for as a sign of improvement. Also is IVIG treatment for life? Have any of you been able to stop IVIG treatments and still have no attacks? I am visiting the Mayo Clinic at the end of February and may have some of these questions answered. I thought I would check with you all, who have first hand experience, first. Thank you for taking the time respond. Cristina

Here are my best answers to your questions: 1) The scientific way to know whether your episodes are over (once you are on IVIG, some other medication, or no medication) is to monitor your blood on a daily or more frequent basis, to determine whether your hemoglobin numbers stay within their normal range. This can be done by pricking yourself and then placing a drop of blood in a portable analyzer like the HemoCue device, see the Disorder Resources tab, under Diagnostic Tests. To my knowledge, I may be the only one who did this every day for months before and after I went on IVIG, and I stopped checking my blood once it became clear that my numbers were, for the first time, remarkably stable month after month after month. Most patients simply go by how they feel, and if they feel normal, they conclude that they are not having episodes of SCLS. 2) IVIG treatments are for life or until someone -- most likely, Dr. Kirk Druey at NIH -- comes up with the answer to the question: What immunological or other factor are we missing, such that we become vulnerable to episodes of SCLS? There are a couple of SCLS patients in Europe who have been on IVIG for a dozen years, but most SCLS patients have been on it for 5 or fewer years. 3) SCLS episodes are so dangerous and traumatic that I'm not familiar with anyone who, after enjoying the benefits of IVIG or other treatments, has decided to play Russian roulette with their lives and tried going off their IVIG or other medications to see if the episodes come back. Good luck with your visit to the Mayo Clinic, and let us know if they tell you anything different. Chances are that they will not, because to my knowledge they don't even have first-hand experience with SCLS patients who have been on an IVIG-based therapy.

Hello Cristina, I totally agree with the responses of Arturo. I am very pleased to read that emergency physicians diagnose SCLS very quickly. I am even more pleased to read that patients receive a preventive treatment with IVIG very early. From my point of view IVIG-treatment will become the Golden Standard against SCLS. In contrast I am sad to learn that so many patients require extensive fasciotomies during resuscitation. We need to make progress about the volume of infusions during an acute attack. Claude Pfefferlé

I had an attack after being on IVIG for 6 months. I suspect it was because my dose was to low for my weight. I've had no minor or major attacks since.

You may want to look at the discussion topic,"IVIG user facts" which gives a brief summary of some of us using IVIG. It is also helpful to click on the individual "profile" name for their history with SCLS. I have been on IVIG for seven months at half the dosage most of the others are on, ie one gram per Kilogram of weight instead of two grams so I only go one day a month. I also have a Hemocue machine that Arturo mentioned and do weekly blood tests which have been normal since IVIG treatments. I am also curious to see if some moderation of the frequency of treatments can still keep me attack free but have not discussed this yet with my doctor. Good luck with NIH. PS. I too am from Kansas City although it has been a few years since I moved. Gene

Hello Gene, Your are just a little bit older as me, because born in October, 1943 in France. By me, the diagnose of SCLS was done in April 1988, after a severe collapse and a first hospitalization in distress and 3 more other until January 1989, so as I think I will, probably, be at time the “oldest” survivor so as more than 20 (twenty) years later I am always alive in spite of a very dark prognosis and a life expectancy of about 6 months. After reading all the messages on this forum, I want to share my experience in this very fearful fatal disease without codified therapy at time and send to all a message of hope. On this time, IGIV treatment seemed not be existing for SCLS, and a treatment by acupuncture initiates on me by a world-famous acupuncturist, followed without interruption since June 1989, keeps me alive in satisfactory conditions without any major crisis at date. At my knowledge currently several other patients in the world are successfully treated at the same manner and still alive in good life conditions . Since August 1990, after a new severe crisis induced by aspirin, I never had any other and in 1995 a treatment by CELECTOL and ALDACTAZINE began for additional hypertension.Look on my profile for more details. I follow with a very great interest all informations and researches conducted by MAYO and NIH in particular, but IGIV is at time not necessary for me so as I am in a good general state of health. Best whishes to all from the Cote d'Azur and for the Valentine's day ! Maggy

I apologize for not responding to all of your messages. I used to get an update on my email when there was a new response, but that does not seem to be the case anymore. To date I have had only one IVIG treatment which was as the end of March. My doctor and I are trying a variation on the IVIG treatment. I am still on theophylline and will be doing IVIG every 3 months. My first infusion was done at the hospital over 2 days. The infusion rate was started very slow and sped up to a faster rate. My infusion at the end of this month will be done at home and in one day. So far, I have had no other attacks or symptoms of SCLS. I continue to recover from my attack and am back to teaching fitness classes. Due to my nerve damaged feet I am limited in what I can do, but I am thankful to do what I can. I will keep you posted on the effectiveness of my treatment plan. Cristina

I would think twice about once every three months.

Jisenhour, I am aware that most people who suffer from SCLS only find relief with monthly treatments, but Dr. Druey and my doctors kept telling me that I was not a clear cut case to recommend IVIG as a treatment. I am not what is called a "chronic leaker". I have had one attack, an extremely dangerous one, with no precursors and nothing since. Most doctors have told me to stay with the Theophylline only. I pushed for the IVIG ,and we decided to go with a 3 month plan. Our thought is that the IVIG potency fades at the rate of 1/2 the potency per month. So by the third month I have 1/8 of the potency in my system. There are some patients who find relief from only the Theophylline and Terbutaline, so I am thinking I have a little more protection then them. This may be a dangerous way to go about things, but in the mean time I am being hyper vigilant about how I feel and hope that this treatment is right for me. If not and I have another attack, that GOD willing is not as severe as my first, I will go to monthly IVIG treatments. I would not recommend this treatment to anybody else, but it is what my doctors and I came up with due to the circumstances surrounding my case. Thank you for the advice, and hopefully I will have only good news to share. Cristina

Ok, that logic sounds reasonable. And you have the best guy in the US consulting so I wish you very good luck. Jeff

You may want to have your doctor order you a HemoQue machine which allows you to measure your red blood cell counts at home. Arturo mentions it above and under "Disorder Resources." Then you can monitor fluctuations and know when to head for the emergency room as opposed to "just not feeling well." Its not cheap (about $600) but worth it for peace of mind, especially with the IVIG schedule youre on. I also had one major attack then went almost 2 years with no other symptoms before I had a second major attack (see profile) so patterns are hard to predict. Good luck.