I have had SCLS for over 10 years (I signed up on rareshare back then, and entered my profile), and have spent those years mostly maintaining a chronic leak with diuretics. I would have an episode every 18 months or so where I would get up (dehydrated) in the morning, pass out, and go to the ER for 2 or 3 liters of fluid and then take lasix to bring the fluid back down.  I was pretty much in a constant state of puffiness. 

This February, I was in the ICU for 6 days because I had a very different kind of episode.  Luckily, the Intensivist on duty listened and investigated, and after about 10 liters of saline, put in a central venous line and administered Levophed, and then lasix.  It took me 2 months to feel like myself again, and we drove to Mayo Clinic in MN for a consult (I went 10 years ago).  It was recommended that I begin IVIG 2 g/kg given every 4 weeks. 

When I returned to Maine, I had my first treatment over 6 hours, came home sleepy and with slight headache.  Woke up feeling ok the next morning, but diarrhea all day and into Sunday, which was when I also had a migraine and was panicked about becoming dehydrated.  I went into the ER for 1 liter fluid and a 'migraine cocktail', came home & slept it off.  For the following 8 days, I had intermittent headaches/migraines and diarrhea. With the shortage of IVIG, no one was calling to set up another appointment, and I was ok with that.  I did realize, after about 6 weeks, that I had not needed to take any diuretics in this time.  My muscles were also tender most of the time, and that is gone. I have pretty much stopped eating white flour (I would notice my muscles more sensitive after it, and the February episode happened after an overload - and - they may have absolutely NOTHING to do with each other).  My husband keeps telling me I look 'so healthy!' because I've had a consistent look for these 9 weeks now.

We are hesitant to get on a schedule with the IVIG.  I asked my Mayo doctor about waiting until I start retaining fluids again, and he would prefer going to 6 or 8 weeks, not wait to treat symptoms - which I completely understand.  It sounds like my reaction to the IVIG was unusual.  What are others' experiences with IVIG?  Any other 'chronic' leakers?  

I am so glad to be able to put this out to people who have had similar experiences - thank you for any input!!

I'm glad that you got the right advice from the Mayo Clinic.  If you don't trust them, I suggest you make an appointment ASAP with Dr. Kirk Druey at NIH and get a second opinion from him.  I came to know (in person or via email and phone, mostly thanks to RareShare) several "chronic leakers" in various countries who managed themselves by drinking or getting IV fluids when they were leaking, and then by taking oral or getting IV diuretics once the episode was over.  Some of them I knew back in the mid-2000s, when there wasn't widespread knowledge of the benefits of an IVIG therapy.  Others are from recent years; they knew about the benefits of IVIG, but they didn't want to bother with it and thought they could keep riding their episodes.  They all eventually died from an episode they couldn't manage, probably because the body's organs -- particularly the heart -- are weakened by each episode.  The writing is on this wall.

Your quick response is much appreciated, and this is exactly what I was looking for.  I completely trust the Mayo Clinic, but wanted some idea of other patients receiving/not receiving IVIG. Your points make perfect sense, and I'm afraid I was in the group thinking I could just ride this out.  Thank you! 

Dear, although it seems a bit rough Arturo's response is nothing but reality, today 2 gr / Kg.  Every 4 weeks is the recommended safety dose.  I don't think it's crazy to take it to 6 weeks if necessary.

When I started IVIG I also got very server migrane/intense headaches.  These quite quickly improved after a few month of having IVIG.  I have also recently noticed having even small amounts of alcohol the day or so before IVIG makes this headaches worse.  Since then I have ensured I am very well hydrated (literally drinikng a extra few pints of water of the preceding  24 hr) before infusions and this has eliminated all symptomes after IVIG.

You really should go on IVIG - the consequenced if you have a sever attacked are too dangerous to take a chance.

I just had my 18th straight month of IVIG. I have had no more episodes since I started the treatment. Basically, I made it through the winter without an episode. No flu to test it, though I did get some kind of virus/cold and managed to be ok without a visit to the ICCU. After my first infusion I had a serious migraine. Since then I’ve only experienced a migraine here and there (several times, but not every time). I’ve had diarrhea as well, just as few of times. It seemed like one or both of those symptoms might occur during my first few infusions. But I had my infusion 2 days ago and I’ve felt fine. All in all, the IVIG gives me peace of mind. So as long as my insurance is covering it I’m sticking to the plan, and advice from Dr. Druey at the NIH. It’s the only way I can convince myself that I am ok. And that I will be ok for a long time. Having a migraine or diarrhea is much better than being on life support and going through the physical and mental trauma.

Thank you everyone for the added info - I am getting my 2nd IVIG on Friday and will definitely take the advice on fluids.  My husband was extremely hesitant about another treatment, so having this opportunity to hear others' experiences is definitely a blessing :-) 

I am also from Maine, though I spend winters receiving my infusions in Florida to avoid the ice and snow of medical travel. I have been very successful in reducing my IVIG side effects by splitting the dose, i.e., receiving 0.5 gram/kg every 14 days. It is time-consuming but my quality of life has improved as a result. Side effects may be unavoidable for some of us, but reducing them to a tolerable level and remaining on IVIG is definitely worth the increase in life expectancy.

Susan