Hi Group, So I guess I need a little help here as both my doctor and I are not sure how to proceed. I was on 5% IVIG every four weeks, the infusion center changed brands and went to 10% IVIG. Apparently my records were never changed so the Doc thought I was on the 5% the whole time. On the 10% I kept feeling worse every time I received treatment. I had an attack 3 day after treatment in October 2015 so we switch to one bag of 10% IVIG every two weeks but I had another attack 3 days after the first dose of IVIG, so the Doc is holding treatment. As of today I have found out that all the IVIG is out of my system. I am more or less running on faith to see what happens next. My Doctor is looking for a specialist in SLCS. I have been to NIH and and have seen Dr. Griepp at Mayo. The Doc is consider trying the 5% solution again. I live in Milwaukee, Wisconsin. Any ideas would be appreciated.

Ritz, Thanks for the update and I'm so sorry to hear. Have your doctor inform and coordinate with Dr. Druey at NIH. He needs to know what exactly has been tried and what the outcome has been (laboratory results, etc.), in order to provide potentially useful advice.

Thank you, I don't know what my Doctor has sent but I just emailed him a 50 page health history.

Hello Ritz, Your Doctors have been in an unfortunate position, what with the dose changed without their knowledge. You must wonder what exactly is reflected in those 50 pages? It's a wonder he/she didn't have a stroke! You need to be quite clear. The actual percentage is a matter of fine tuning. The real issue with dose is the total in Grams. It seems to be ideally at 1G/kg given fortnightly. I get 90G each fortnight. Now, the levels can actually be measured in your blood, but be aware that you are measuring the Total IgG... That is the sum of what your body makes and what is infused. It is your IgG. This level should be well over 16, preferably over 20. If the level is below 16, you can reasonably assume the infusion has worn off. The level should be measured just before the next infusion. The only difference between the 5 and 10% is the volume used to achieve the result. Some people will be less tolerant of higher volumes. While waiting for definitive answers from NIH, please consider getting back onto fortnightly doses of 1G/kg. Only when you are totally confident of what you are getting can you reasonably decide on whether there are side effects. John

Hi again Rita, After I posted on the " new guy" thread with a response to you i went back and found that you had started this thread. I totally agree with John and Arturo. If your dose/volume was changed that explains the episodes. I wonder if the brand makes a difference which you also mentioned had changed. Even though medications are essentially the same sometimes there may be inert or inacitve ingredients that you may not tolerate. If the other brand worked maybe they should try finding that for you again. It sounds more like you just need to find an IVIG regimen that will work for you and perhaps john's advice regarding continuing the IVIG while your doctors figure things out makes the most sense. Best Wishes! Linda

Hi just tried IVIG again and have been in the hospital for a week. My local dr is going to send me back to Mayo. She is not comfortable continuing my treatments. I guess I will be seeing what is next.

Ritz, Thanks for the update, but what happened in the hospital? How did you react and what did the doctors do?

Hi I had what appeared to be a classic CLS attack. I was treated with fluids and albumin. I was in the leak phase for at least three days and I am just now finishing the recovery phase. I discussed brands and preventages of IVIG and was told that there is no research supporting that that would make a different. If that is true I volunteer to be the research. Dr.Qamar specialize in oncology and is looking for some one to help with my case. I am still not feeling back to normal so I now see that my last post was not specific, it was more fustration.

Hello ritz, This may help. Twice in 18 months, I have had a " bad week" after IVIG. The first episode came on during the infusion, and to all extents, it looked like a viral illness. It had a mild fever, with severe muscle aches, progressing that night to severe night sweats. (The muscle pain and night sweats are also the prodromes for my SCLS attacks). When I was alomst at the point of hospitalising, I took 15mg prednisolone, and within an hour, I could feel this all starting to abate. It lasted 2 days. The second episode came on 2 days after the infusion. The same thing, - fever , severe sweats, and muscle pain. I took prednisolone 15mg daily for 4 days, and got better. At the time, I didn't have time to be ill! lol. These are unlikely to be allergic reactions to the IVIG, as they only happen very occasionally. They are possibly viral contaminants in the batch.. Unlikely. It is possible that the batches had less of the relevant immune globulin, and they were thus ineffective. At this stage, I continue my IVIG, and get trigger happy with the steroid at the very first prodomal signs.. Body aware is good. John

Thanks John, My doctor is afraid to proceed. I have talked to my insurance company and they stated that they would help me find a research doctor in the area. Maybe I can help form a procedure for problem patients. I don't have steroids that I can take if I start feeling ill.

Hi Rita, So sorry to hear about this and applaud your courage and persistence. Did they reduce the percentage/ volume back to what it had been when ivig was working for you? Seems like that would be a logical thing for them to have tried. The brand could make a difference and John has made some valid points. My brother also had the prednisone on hand and took it occasionally when he felt like he was getting an episode and it seemed to work most of the time and it saved a trip to the hospital. The only thing I would add is that if you take prednisone for a period of time you are supposed to taper off so make sure you ask your doctor how to take it. I am glad that you are recovering and I hope you can find some answers. Please keep us posted. You are not the only one that has issues with IVIG so any information you can share is valuable. Take care! Linda

bonjour, j'ai aussi ce problème depuis quelques temps. lundi 22 fév. après une journée de traitement 1 gr/kg, j'ai dû retourné à l'urgence, arrivé là, hémoglobine à 184, le médecin a décidé de me redonner un autre traitement ivig 1gr/kg durant la nuit. à 7h00 le matin: hémoglobine à 145. par la suite, un autre traitement de ivig. 1gr/ kg. et aujourd'hui, je vais bien. Mes médecins ont dit qu'ils suivraient la situation de près. pour l'instant, je vais recevoir mes traitements chaque trois semaines. et je me demande si je ne devrait pas essayer un autre sorte de ivig. présentement Privigen. espérant que tout va redevenir à la normal. merci et bonne chance a vous Ritz. Hello, I also have this problem for some time. Monday, February 22 after a day of treatment 1 g / kg , I had returned to the ER, got there, hemoglobin 184, the doctor decided to give me another IVIG treatment 1g / kg overnight. at 7:00 in the morning, hemoglobin 145. thereafter , another IVIG treatment . 1g / kg. and today , I'm fine . My doctors said they would follow the situation closely . for now, I will receive my treatments every three weeks. and I wonder if I should not try a different kind of IVIG . Privigen now . hoping that everything will become the normal . thank you and good luck to you Ritz.

Thank you Josee. I am trying to get as much information as I can to give my Doctors. I hope thing go well for you too.

I saw my local hematologist today and was taken aback by his statement that IVIG is not covered by insurance as the treatment isn't really proven as effective for SCLS, it sounds like many of you are encountering similar issues. Any suggestions on dealing with Insurance?

Hello Arturo Is there by now a register of people who have had several attacks, and totally stopped by IVIG? If not, I would be happy to keep a data base. I accept the shortcomings associated with a lack of uniformity in diagnostic criteria, but we could surely stablish a protocol for inclusion? This would not impact on Kirk's decisive work, rather be labelled as 'ancdotal' John

Anecdotal

IViGG has been a godsend for almost all. Insurance companies would love not to pay for it. I would suggest that anything posted include the exact mg/kg dosing, frequency, duration, degree of swelling, if any, nature and dosage of premeds, etc. Let us not risk identifying iGG as a "red herring" for other causes of edema. John's careful, informed, medical approach is greatly appreciated. Best to all, Susan PS I am slowly developing a template document for Medicare/other insurance/NHS appeals for IViGG coverage that I will share for all to consider. It is slow due to my mom's end-stage frailty and my low cortisol levels. Medicare law was my specialty prior to my medical retirement due to SCLS. Susan

Update: I went to Mayo Clinic in Rochester MN last week and visited with Dr Kapoor. The plan is to try Singulair and lisinopril (2.5 mg a day). I will still be off of IVIG. If I continue to have problems Dr. Kapoor wants to try adding IVIG back in with the Singulair and Lisinopril. The third stage of the plan is to try chemo if the above doesn't prevent attacks. I will let the group know how this goes.

BEST WISHES! Ma it all be excellent

Ritz, I will keep you in my prayers. I have been "running out of steam" between bi-weekly IVIG infusions, with the thought of a major episode always in the back of my mind. Stay strong and Thanks for keeping everyone updated. Dawn

Well so far so good. I have been off of IVIG since February. We started the Singulair a month ago, no problems. I started the Lisinopril last week, I am having a few side affects but I am going to give this a little time. I am still having minor "swells" but nothing that has required the hospital. I do feel much better than I did when I started reacting to the IVIG. Right now it is worth the better quality of life for me. We will see what the future holds.

Hi Ritz, how long were you on the IVIG before you started experiencing the issues ? Dawn

Hi Dawn, About 5 years. I always had a reaction but not so serve as the last several infusions.

I collapsed in a hardware store yesterday and was taken by ambulance to a small critical access hospital which in turn transferred me 80 miles to the hospital where my hematologist is located. I had over-exerted during the 14-day interval between infusions and was very ill two days early. Thankfully, I did not have a dangerous amount of swelling. However, the stress of the collapse exacerbated my secondary adrenal insufficiency. I was infused overnight with IViGG and one-on-one nursing care and felt much better this morning. I am experiencing deep fatigue but I am no longer sick. Now to rest and hope that my baseline returns to normal and that I make it to my next scheduled infusion. Any thoughts?

Hi Ritz. It sounds like your situation is similar to that of my husband. Best of luck with the new treatment plan. We are on a similar path. Question: When your episodes returned, how often were you having them? I

Update: So my BP has been dropping again. I checked with my doctor. My creatine and albumin were off a little but not bad. I got a bags of fluid, and I am doing better today. This reminds me a lot of when I was undiagnosed, and trouble was brewing. I have an appointment soon, so I will see how it goes. This is a little unnerving.

Responding to the question about a database of people who have stopped having attacks after IVIG: I stopped IVIG about 2 years ago after being on monthly doses - I think 1g/kg, for over 2 years. It seems like I have more of a chronic form now, maybe just residual effects, scarring, from having had so many severe attacks for years. (Just chronically leaking capillaries with excess fluid moving with gravity) My first attack was in 1999. 2 weeks in hospital. The next attack was several years later. By 2010, I think I was having around two severe attacks a year. And between them, I stayed so swollen and sick and weak. Finally went to Mayo and saw Dr. Greipp and started IVIG. I had one really severe attack right after first dose but most of this was result of the local infusion center mistakenly giving me a double dose and no premedications. After barely surviving that, I had only one, maybe two more attacks severe enough to be hospitalized. I elected to stop the IVIG after around 2.5 years of monthly treatments. And no attacks for about a year. I haven't had IVIG in over 2 years and, as I mentioned, I haven't had an attack yet. I still see my hematologist every 6 months. Although it sounds a bit risky to have stopped IVIG, in retrospect, I could just tell that I felt better. The chronic feeling sick and weak even between attacks seemed to lessen over time. Sorry this may not be a very cohesive history. I'm attempting to multi task this morning so... Carole Thorn

Hello Carole This is a very informative post, and while it seems risky for you, I have to admire your courage of your own convictions. If you could find time,mInwould be very grateful if you could complete the survery at www.capillaryleak.com. This will help us understand the role of IVIG so much more. John

I need to make an important correction to my above post. I incorrectly stated that I had been on 1G/kg IVIG. It was 2G/kg given over two days. (Which can be confusing - 1G/kg x 2 days would be the same. Key words are "over" vs. "times") I will absolutely fill out the survey! Thank you!

I have typed in the link to the survey a few times but have been not been able to reach it. What am I doing incorrectly?

Hello try to copy and paste this: http://www.capillaryleak.com/survey/ The password is alaska John

Hello John. I tried this also Maybe it is because I am 'downunder'?

Update I have been in the hospital since 12/21. Major attach. ED doctors would not look at nor follow the CLS instruction sheet. Before going into arrest got my cell and called my husband and Doctor for help. I feel the lack of quick care caused things to be worst. I had a stroke and went into cardiac arrest. I was in icu for several days. I am now on a rehab floor. I am starting to walk again. I am on dialysis and am have intestine problems.

OMG, Ritz!!! You are very lucky to be alive. Well, now your doctors and you know the downside of stopping your SCLS therapy completely and, as you put it months ago, "running on faith to see what happens next." You do remind me of the problems that Josephite's husband had with IVIG. He too went off but we haven't had an update for quite a while. There are no easy answers, but I reiterate my advice to have your doctors coordinate ASAP your care plan with Dr. Druey at NIH and with Dr. Kapoor at the Mayo Clinic. They also need to know all the details of your situation, just in case any other SCLS patient encounters similar problems as you did.

Hello Ritz Our thoughts are with you and your happy smiling face John

Dr. Druey has been great. He worked with my Doctors over the phone. He has a plan for IVIG. I am home but it looks like this is going to be a long recovery. I lost my drivers license, so I need to take a rehab class and pass a driving test to get it back. The stroke that happened mess with my eye sight.