Arturo, Claude, Jeff and IVIG users. Today is one year on IVIG for me. It is also one year since I have been to the ER/ICU. I have had *NO* episodes since March 2010. Since becoming a cronic leaker last March and then seeing what IVIG has accomplished, I would like to have the IVIG users reply with their current status. So far this year I have been able to install kitchen cabinets and a deck. I feel normal again and do not fatigue as in the past. Claude, what do you think is next beyond IVIG? This IVIG treatment is great. Arturo and Allen, hope to see you April 25/26 while at the NIH.

In my case, it's now 16 months of IVIG infusions and it's 16 months with no more episodes of SCLS! Truly a new lease on life, and an incredible improvement in the quality of my life and that of my family. In fact, I was telling one of my doctors the other day that while I am aware of six SCLS patients who have died during episodes of SCLS during the past five years -- the latest one in recent weeks, see Community Expert -- I haven't heard of anyone who has died since starting to receive monthly infusions of IVIG. By the way, others from this community who are also on IVIG include Josée and Nolan (nwbsaw). PS: I'll see you when you come to NIH.

I've had some problems. I was on 1000 ml of ivig per month and seemed to be fine until late last September when I started to have minor attacks again. In late January I had a major one which put me in ICU and on a respirator for 3weeks. I got out in late Feb. And am well on the way to recovery. We've doubled the IVIG dosage to see if that has any impact.

Arturo and friends, It has been a long time since I last logged in to this forum. What fantastic news to learn of the success you have found in IVIG. Even though Judy did not live to benefit from IVIG therapy, I know that she would be thrilled that your tireless pursuit of progress on behalf of SCLS patients has at last born fruit. Congratulations on your improved lifestyle, and here's to hoping for an eventual cure! Thomas Hammond Cambridge, MA, USA

Jeff Are you taking the 1000 in one day? Check on taking it in a two day dose. I found that IVIG (for me) is 750 ml (75g) each day for two days a month. I see the difference at the end of the month, which I believe, in the last week of the month, there is only about 5% of the IVIG in my system. Take care the week before your treatment. I also never change the flow rates for the treatment. If we change the rates, I get headaches and my blood pressure spikes. For the first 1 1/2 hours, I start with rates at 30, 60, 120 ml. Then I switch to 220 ml per hour. I have tried 230 and 240 ml, but it doesn't work for me. Hope it works out for you. How far from the NIH are you? I plan to be there end of April. Walt.

Hi, I started IVIG on April 2005. I get 2g/kg polyclonal immunoglobulins on 11th of each month (in memory of the fall of the twin towers). The infusion runs in my home during 4-5 hours without complication. I’ve never changed the medical dose of IG and the various products I used have allways had the same efficiency. I had no new attack of SCLS for exactly 6 years. I’m very happy my experience will benefit to all Patients with SCLS around the world. IVIG is great and is probably the best prophylaxis and acute treatment against SCLS. Claude

Nolan has been on IVIG since July of 2009. He went from having episodes of some kind every 5 to 6 days to no episodes. It is truely a blessing to us.Nolan gets IVIG every month. I give it over 8 hours at night for two nights while he sleeps. He sometimes gets headaches but they are mild. we wish everyone the best. Wendy and Nolan

Ma dose de IVIG est aussi de 2gr/KG mais sur 2 jours donc, je reçois 1gr/KG la première journée (en 5 heures) soit : 30 ml/ hre pour 30 min. 60 ml/ hre pour 15 min 100 ml/ hre pour 15 min 200ml/ hre pour le reste des bouteilles. et le lendemain, je reçois la même dose. je pèse 67 kg donc je reçois un peu pus de 3 bouteille de 200 ml. aussi, la veille de mon traitement, je dois prendre 150 mg. de predinsone. soit 50 mg. à 6h00 le soir 50 mg à minuit et 50 mg à 6 heure le matin. bonne chance à vous !!!!!!

hello alsso for me i start one year ago i havent any epsoid and i fiel olweyes good i us 2 gr/kg i ll vist paris next 10 april to meet DR.AMOURA and may be he well chang the dose i ll going to inform you after , i am alsso some time git a contact with DR, GRIEP ,hi sayed that is ok the dose that i us right now - i hope for all of you a good health yaser italy,roma

Hello Elganzory, Sono realmente felici di apprendere che vai bene con le Ig da un anno. Penso che non debba cambiare la quantità del tuo trattamento e restare a 2g/kg quindi che tu lo sopportate bene. Quando sarai a Parigi, dà i miei migliori saluti al Prof. Amoura e alla Dressa Gousssef. Ciao. Claude Hi, I am very happy to learn that you are going well for 1 year. I think that you don't have to change the dose of your treatment and remain to 2g/kg becouse you support it well. When you'll be in Paris please give my best regards to Prof Amoura&Dr Gousseff. Ciao Claude

Further to Claude's point, and as an important clarification on Jeff's' (_jisenhour_) troubling post, above: At first sight, Jisenhour's entry appears to be the first reported failure of IVIG therapy from any one of our many RareShare patients who have been on IVIG for 1-6 years. It is also troubling especially since IVIG had stopped his previously frequent episodes for many months. However, I spoke with him yesterday and it turns out that, as Walt wondered in his post above, Jeff had been getting only half of the recommended dose, namely, 100gr of IVIG once a month (for someone who weighs about 100kg). Jeff told me that neither he nor his doctor knew that this was only half the recommended amount, but now that they know, he has received two 100gr doses -- which is what explains his comment that his previous dose (of 100gr once a month) has being doubled. While we are all sorry for his January relapse and for his pain and suffering (e.g., he underwent some preventive abdominal surgery during his hospitalization, and his lungs were at risk), Jeff may have unwittingly provided us all with an extremely valuable data point: namely, he may have confirmed that a single monthly dose of 1gr/1kg of IVIG is NOT enough, and the proper amount is 1gr/kg of IVIG for two days in a row (namely, 2gr/kg per month), as the rest of us is getting.

hi claude thankes for replay i am happy alsso for you ,i ll send your rgarders when ill be a paris to Dr. A moura have anince day hoping for our commuinty the best wishes to be alweyes in a good health . yaser

Hi Arturo, I have to precise this very important concept : THE FULL DOSE OF IG IS 2 G/KG and not mg/kg. If your weight is 100 kg you have to recieve 200 g of IG. You have to recieve the total dose of Ig within 1 or 2 days, it doesn't matter. BUT YOU HAVE TO RECIEVE THE TOTAL DOSE EVERY MONTH becouse the half-live of Ig is 28 days. Prof Amoura said to me that he knew a turkisch Patient who died becouse he didn't recieve the full dose (too expensive). In case of an attack of SCLS you have to recieve half a dose as soon and as quickly as possible. If the leak doesn't stop within 12 hours, you will recieve the second half dose of IG. Best regards to you and everybody. Claude

Thanks for catching my mistake Claude. I fixed my post, above, accordingly.

I just wanted to say that Arturo is correct in how he characterized my situation. I have since received from him some information, and done some of my own research and will talk to my Dr. About it. Thank you very much. I really appreciate this resource. Jeff

I am still only on Prednisone, but am going through some testing. I am to have some blood and urine tests tomorrow. After I get all the results together from my hospitalizations, ER visits, and labs, I am hoping they will be able to put me on either the asthma meds or the IVIG to prevent future problems. I am feeling very well on the Prednisone. Do you know of any Doctors in the South Jersey or Philadelphia area that may be able to help my docor with questions? Thank you very much for all the useful information I've gotten in the past few weeks. Mary Eileen

my son had 3 episodes in 2010 from may-dec. the last 2 episdoes were only a few weeks apart. which makes you wounder if the episodes were getting more frequent. the last episode he ended up having cardiac arrest and nearly a month in the hospital. during his visit he had a double treatment of 4g/kg. he has made a full recovery since the episode and we have been going for monthly treatments of ivig. it has been close to 4 months since he has had a episode. he has gotten sick a few time but has not leaked, which is unusual for him because any sickness normally kick started his episodes. thanks everyone for their info. and a special thanks to Arturo C. Porzecanski for all the extra info he gave me to help inform myself and my doctors.

Jeff Glad to see you found the dose issue for IVIG. Good luck from this point. I am sure this will make a big difference. Keep us updated as to the following months. At least you found out before something worse. All the luck. Erintaylor It really is tough hearing about the children with this syndrome. When I found out, it was only people around 50-60. I don't think any of us considered children having CLS. I hope he grows out of this soon. Walt

Jeff How are you doing? Just wondering if everything is OK.. Send me an e-mail if you get a chance. I am visiting the NIH April 25/26. Hope to get a chance to meet you. Walt

Thanks for asking Walt. I'm still pretty weak in the arms and legs (lost 70 lbs) but overall I'm recovering nicely. Got back on my bike for a bit today and plan on going to the pool for some laps this weekend. It's been a chore but I've come a long way already. I'd love to join you all for dinner and I'll keep both days open. Here's wishing continued good health to you as well. Jeff

Arturo, Perhaps under disorder details or disorder resourses there could be a fact sheet stating the details of ivig to include general dosage recommendations based on weight,how the dose is dispersed ie 1 to 2 days,andthe frequency it is given ie once a month. Also recommendations on how to give ivig during an episode ie half dose to begin then half dose later. then when do you go back on the monthly maintenence dose? It would be handy to print off to show the primary care doctor,hemotologist and er doctors. I am so glad that the ivig is working for you and all the others. We have come a long way and the best part is that lives are being saved and the quality of life is so much better. You,dr greipp,dr druey and everyone that participates on this site has made this happen and I am so grateful for that and thank you all Linda

OK, Linda, I have acted on your suggestions, adding a paragraph at the end of the Disorder Treatment discussion and expanding the insert on IVIG right under it, to which attention is now called. There was mention of IVIG and its recommended dosage already in the Prognosis section. All the new text refers to reliance on IVIG to prevent or minimize future episodes. The use of IVIG during an episode (to abort or lessen its severity) has not been discussed much in the literature; thus, there is no evidence that it shortens a capillary leak attack. However, I have not heard that it does any harm, either, and it is often that doctors have been moved to give the first of many doses of IVIG to patients while they are having an attack that has not been prevented by other medications.

Arturo, Thankyou so much for expanding the ivig info in those resourse sections. While theophylline may work for some it seems that the ivig is working for almost everyone in preventing scls episodes with tolerable side effects. I know giving ivig durimg an attack may need to be researched further and it may just be a matter of trial and error to see how to proceed with this. with many takimg the ivig monthly hopefully there will be less episodes occurring. The resource sections are invaluable. Great for the patient and the healthcare provider. Thank you for keeping them up to date and in order. You are our hero! Linda

Hello all. While it can't exactly be considered solid medical proof, my husband has had two episodes this year that were halted with the administration of IVIg. Our specialist gives him the standard dose in an ER setting, and then admits him to a ward for a day of observation. With this round (we are still in hospital), the break inbetween doses was skipped. The hubby's hemoglobin counts start going down within hours of IVIg administration. We aren't yet taking IVIg monthly as a preventative therapy, but I suspect it's in our future. Fortunately, we have a very intelligent and compassionate specialist following our case. Liesa

Hello all. While it can't exactly be considered solid medical proof, my husband has had two episodes this year that were halted with the administration of IVIg. Our specialist gives him the standard dose in an ER setting, and then admits him to a ward for a day of observation. With this round (we are still in hospital), the break inbetween doses was skipped. The hubby's hemoglobin counts start going down within hours of IVIg administration. We aren't yet taking IVIg monthly as a preventative therapy, but I suspect it's in our future. Fortunately, we have a very intelligent and compassionate specialist following our case. Liesa

I'm glad to get this update from you, Liesa. I remember that your husband could not tolerate theophylline in high enough doses, so if he has been getting new episodes he should be transitioned to receiving IVIG on a monthly, preventive basis. Have your doctor read the latest article on treating SCLS with IVIG, see "New Article on SCLS from Europe" in this Discussion Forum section. And please update your/his profile entries to reflect what has happened since the initial, terrible episode.

hello like i sead before i just come back from paris when i met dr, Amoura he visit me he found every thing is ok i tell him that when i start the IVIG tratament one year ago i am stell ok now he make some blod test he recomand now to start to gite 1g/k just for tray every month if every thing is going well we well going like that if not we well ngo back to the old way as 2g/k hope for all of you a god health yaser

Yaser Can you tell us why Dr.Amoura suggests a half dose of IVIG treatment each month? Are there other CLS patients on 1g/kg? I am worried about your health since Jeff reported an episode at 1g/kg dose level. Plese be careful. Please also read Claude's replies. Walt

hi walt thank you for your replay it very kind of you as i write DR Amoura said its to way 1g/kg month or 2g/kg every 2 month but he said that we just have to tray by using 1g\gk and he recomand if i have a problemes i have to going back to 2g\kg monthely , as we know this tratement is using in trail so thay tray if its ok we well be happy for that i was just was wont to inform oll of us about that alsso i taoked with Dr, Amoura about the case was pobicied by cloud i ll goig to the hospital at next 19/04 to git my IVIG SO i ll tray if i going to have any probelmes i ll inform alwyes all of you in the end i pray god to help and saport all of us yaser

hi Claude dopo aver leto la resposta di walt si ti possbele di conttarmi per sapere di piu il mio e mail e yelganzory1@fastwebnet.it per che ci sono di lati scoure grazie