My daughter is 7 yrs old. It seems like each episode is almost exactly the same . She has had 3 requiring PICU. So first , Leila seems to get a normal illness, like parainfluenza ( sore throat, fever and runny nose) then she seems to get better after a couple days. Almost 2 weeks after the start of the initial illness she develops again a fever, sore throat , except this time with croupy cough, stomach pain and muscle aches and eventually vomiting, during the entire two weeks she also suffers from constipation. So total from onset of initial virus to start of capillary leak ( shock) is 2 weeks . The last attack / sept. 2012, was slightly different being that she had right sided abdominal pain for weeks leading up to her " shock" scls episode. Which was very upsetting because I knew something was wrong, but no one could help us.( repeated visits to doctor and an abdominal sonogram) Leila has had a very difficult 2 and 1/2 weeks , as she had a fever and sore throat Aug. 17th. She has been having many of the symptoms - besides leaking ! , over the last 2 weeks. I think we are now " out of the woods" she seems better today . Leila suffers from side effects from igg therapy , headaches and exhaustion, she also has very achy feet since her terrible episode( oct.2011) . Which the doctors say is due to hypermoblity and tendinitis. But I'm almost certain it's from nerve damage since it started immediately after her 2nd and most terrible episode. Looking for support and also wanting to get opinions on the timeframe of onset of illness to actual leaking episodes. Thanks again- hope all are well, Kristy

Dear Kristy: Welcome to our SCLS Community! The only scientific way to tell whether your daughter is having episodes of SCLS is for her blood to be tested. The markers for a definitive diagnosis are episodes of hypotension, hemoconcentration, hypoalbuminemia and the presence of the MGUS. In particular, if those tests were to reveal a level of hemoglobin that is above the normal range, then that would be a good indication that at the time she was tested she probably was having an episode of SCLS — depending on how far off from the normal range she was, of course. And such evidence would then allow her doctors to adjust her medications for the purpose of eliminating said residual episodes. You should also speak to her doctors about taking advantage of a unique opportunity: Dr. Kirk Druey at NIH is currently doing a study of SCLS in children. For further details, see the Discussion Forum titled "Calling Parents of SCLS Children." Since you live in Maryland, it would be a shame if you didn't take advantage of the opportunity to participate in this study. Good luck and please keep us posted.

We actually have seen Dr . Druey at NIH for scls and also been part of the mastocytosis study which turned out negative . She is currently on igg subq therapy as the Ivig could not be tolerated . Her health care is managed by a doctor at CNMC and he is guided by Dr. Druey. It's been my understanding that typically the episodes come about quickly - but Leila's symptoms seem to build up over weeks - and then she starts " leaking" and then becomes hypotensive and hemoconcentrated . Also she seems to have smaller episodes where her eyelids swell and she does not become hemoconcentrated. Also I have read on here that Ivig has been given very slowly , perhaps over 2 days? When Leila would get large infusions monthly and when she was hospitalized for acute attacks they did infusions over approx. 6 hours. The treatments had to be stopped because Leila would getterrible headache vomiting and high fever-and high Bp . They did try steroids but that didn't help, it just pushed the side effects out a couple days and when the steroids were over the fever and other side effects would happen just the same. We were never offered slower infusion rate? Also when she was in PICU and received Ivig over Approx 6 hours , even when she was really improving we had to stay a couple extra days because her Bp was very high. , Now I understand that the high bp was probably a sude effect of ivig .In the event of another episode, god forbid, should I insist that they do the infusion slower? Thanks Kristy

Also she does not have mgus

Kristy, Now it is clear that you are on top of the situation as best you can, and that you have availed yourself of the expertise that there is in your geographical area. Dr. Druey and colleagues should be able to come up with a definitive diagnosis. As concerns IVIG infusion rates, yes, they are supposed to be slowed down or speeded up in accordance with how patients react not just to the medication mixed in with the fluid, but to the amount of fluid itself. (It is the fluid being pumped in that usually raises a patient's blood pressure temporarily, not the medication inside.) And the recommended dose is usually infused over two consecutive mornings or two afternoons. In general, patients get used to IVIG and the side effects, if any, tend to diminish and disappear over time. Also, allergic reactions and headaches can usually be minimized or prevented with Benadryl, Tylenol, or if necessary some Prednisone. These medications are routinely offered prior to the infusion, and they can also be taken after the infusion under medical supervision.