Hello, I'm writing in for my wife who has SCLS and is back in the hospital for the tenth time in one calendar year. She has been through several life threatening episodes one of which took her life. On that particular episode she was resuscitated and given a pacemaker. Most all of her episodes start at home with redness & swelling a week prior then followed by a trip to er, then up to I.C.U. for 10-14 days. Every one of these episodes starts off with treatment of pressers & fluids due to mid 60's b.p. After a day, she develops plural infusion, which most times requires her lungs being tapped. During the first 8-10 days her b.p. is in the mid 80's to low 90's. After all the trauma she begins to recover back up to 100's where at that time the kidneys, bladder, breathing etc. all return to normal. The heart was previously effected but is somewhat protected by the pacemaker. She definitely is paced during each episode. This reprieve usually lasts 4-5 weeks only to begin the same episodes all over again. On her last episode at the hospital she received her first IVIG treatment which assisted her in her recovery of about 5 days stay at hospital. She still had all the same things happen but much quicker when IVIG was treated. Her doctor who has diagnosed her, starter her on monthly IVIG infusions of 30 grams. Well, we made it past August, but only half way through September. Her next infusion was 5 days away. She didn't make it. So here we are back in hospital again getting full dose of IVIG. With all the same issues as in the last 9 times. Why is this so severe with her? Is there anyone else diagnosed with SCLS have the same severity? We have been very fortunate to have a world class facility in our back yard that can help us, but what are we doing wrong that other people can recover quicker or even at home. I read where most people can make a month or longer in between infusions. Her doctor is suggesting every 2 weeks in between infusions. The IVIG has helped, we see results. I was just hoping for these monthly infusions to last, well a month. Hopefully, we won't be in the hospital as long as we have in the past, but it hard to say. Can someone let me know if they are aware of any other cases as ours. We are really down as this shows no signs of letting up. Thanks for reading... Knackll

Dear Knackll: I'm so terribly sorry to learn of your awful ordeal! However, if your misery wants some company, I can assure you that yes, plenty of others in this community have also suffered a series of terrible episodes leading to permanent damage to extremities and/or vital organs -- and even to death. Here are some suggestions for the two of you: 1) Have your hospital send to Dr. Druey at NIH refrigerated blood from the day of your wife's admission. (They usually keep any blood drawn for a week.) Her blood needs to be studied with the powerful tools available at NIH to determine what (if anything) is wrong -- or especially wrong -- with her. For instructions on how to ship the blood, which must be chilled but not frozen, have the hospital contact Dr. Druey and/or his team, see "Disorder Details" in this site, and scroll towards the bottom for contact information. And do this every time she lands in the hospital. 2) If she indeed has been properly diagnosed as having SCLS, and if her doctors are going to give an IVIG therapy a fair try, then they must give her the recommended dose of 2 gr/kg/month during two consecutive days. In other words, if she weighs 60 kilograms, she should get 60 grams on Day 1 plus 60 grams on Day 2. If it is true that she was started on a dose of 30 grams of IVIG, whether for one or two days, she was seriously shortchanged -- unless she weighs in at 15 or 30 kilos, of course! If you wish, insist that her current doctor consult with an immunologist; they are most familiar with IVIG and how to administer it. 3) Have her doctors make arrangements to have your wife seen at NIH (in Bethesda, MD, just outside Washington DC) as soon as possible. For this purpose, they must contact Dr. Druey and send him all of her medical records along with a referral letter. Dr. Druey will be able to confirm her SCLS diagnosis, or else he will have her examined by his colleagues until the right diagnosis is obtained.

Dear Aporzeca, Im trying to understand your calculations on the recommended IVIG. So, if she weighs 120lbs. That converts to roughly 55kg. Are you saying she should receive 220 grams of IVIG per month? 110 on day one and 110 on day 2? If i understand 2gm/kg of patients weight of 120lbs. Should be 110 grams/month or 55 grams day 1 & 55 grams day 2. Please advise back Knackll

Sorry, I confused you by writing "pounds" on one occasion when I meant "kilograms" -- now corrected. If your wife weighs 55 kilograms, the recommended dose is 55 grams of IVIG on Day 1 and another 55 grams on Day 2.

Currently in the hospital she is receiving the correct dose of 110grams. Unfortunately she is not tollerating anything more than 25 grams a day. Her bp plummets and they have to stop it and restart after waiting an hour in between for bp to come back up. She does receive the premedication of steroids, Tylenol, benadryl.

Also, i will make every effort to obtain and send her blood. My experience with situation is very limited and I'm not quite sure how they will receive this request. Your replies are very much appreciated. Knackll