Question for the community. Today, I am getting my Day 1 infusion of IVIG, however due to the impending snow storm tomorrow, I may not be able to get the Day 2 infusion. Has this happened to anyone else? Should I skip my Day 2 treatment and resume my next infusion in 4 weeks? Thanks.

Dear rnuara, You definitely have to receive the full dose of immunoglobulin each month. You can ask to receive the dose no 2 within 2-3 next days, as soon as possible after the snow storm. You must not wait until next month without receiving 2 g immunoglobulins / kg. Claude Pfefferlé

I am also from N J and am due for my infusion on thur. and fri. . In the passed I have Skipped a day and went the next one and have had no problem, but I would not wait till your next treatment.

You must get your 2g/Kg per month. However I have been getting mine at 1g/KG every two weeks and I find this optimal. Better level of IVIG throughout the month and less reaction to large dose of IVIG

Thanks for your replies. Much appreciated.

Hello, I had my first two infusions on an inpatient basis with no apparent side effects. However, my first outpatient infusion didn't go as well. I had Benadryl, Zofran, and 100 mg hydrocortisone each of the three days as premeds. On the third day I felt very poorly and they offered an ambulance, which I declined. For several days after the infusion I had very high blood pressure that fluctuated with low normal readings. Normally my BP runs quite low. Has anyone else experienced post-infusion BP spikes? It is a common side effects? Could it be one of the premeds? Thank you for your perspective, Susan

Hi Susan, It is a wonder there is not more of a BP spike. After all, a large infusion of hyperosmolar fluid is going to suck extracellular fluid back into the CVS. For this reason the infusion is progressed very slowly. It is amazing how the body can adapt! I am sure that your physicians can deal with this. John

Thank you, John. Written like a wise physician and makes perfect sense. My struggle with dehydration had decreased significantly, perhaps explaining my less frequent low readings. There go the joys of my high salt diet! Again, thanks, Susan

Hello out there I just found out my precious 2 1/2 year old grandson has SCLS and I am so scared. The data is so limited with the rarity of this disease. I guess any insight or info anyone can share would be most appreciated and welcome. The few case studies I found were not for one so young

Hello kgoldade, First, let me introduce myself who has had four REALLY severe episodes. Second, let me say that the medical attention I have had is SUPERB. It follows exactly what Dr Drury and those on this site propose. I had four attacks in 6 weeks, and haven't had an attack since then after starting proper treatment. Third, as a grandfather of 12, I really treasure these youngsters. They make the world go round. Without them, the Earth wouldn't rotate !?! Last, try (if possible) to see the glass half full. There is SOOO much to be gained by including your grandson in Dr Drury's work in order to help him, and also contributing to helping other kiddies worldwide. I call this my Shrek disease. Perhaps he may be lucky enough to meet Fiona? John

Hi Kgoldade - Just want to suggest you post this as a new topic. There are a few people on here who have children with SCLS and they'll be amongst your best sources of support. I don't think everyone reads every topic thoroughly, so your question might get missed by people who could speak to the issue. BTW, I called it "Violet Bouregard Syndrome", after the girl in Willy Wonka who blew up and turned purple :-D Cathy