Hi All, I am out of the ICU but still in the hospital after my first SCLS attack since I started IVIG. It was a much milder attack that in the past and responded quickly to albuim and fluid. I think the key was that I went in at the right time and the ER started treatment as soon as my blood pressure dropped. Hopefully this was just an odd occurrence.

Rita, This is disturbing news. You are not the first or only patient to have had an episode despite being on IVIG, but we need to get to the bottom of these relatively rare incidents. First, request and authorize in writing that the ICU send your 1st and 2nd day blood samples to Dr. Druey at NIH. He is always looking for episode samples -- especially now, that most of us are not having episodes anymore. Hospitals usually save blood samples for a week. He can instruct whoever is going to do it how to go about it (e.g., the blood should be chilled, but not frozen). Second, please write, and have your main doctor write, an email to Dr. Druey informing him of what happened. You can also request and authorize the hospital to send a copy of your hospital records to Dr. Druey directly. I wish you a speedy and complete recovery!

Thank you, I will talk with the staff here at St Luke's in Milwaukee about shipping the blood. Even though no one here specializes in SCLS, they do a wonderful job of caring for me. I do know that they rely on the information I share from this group to help with my care plan. So I greatly appreciate the group. I am wondering if any one else that has had attacks while on IVIG is sensitive to IVIG? I am and the doctors here are wondering if I am reacting to the IVIG.

Well, yes, actually. The husband of "Josephite" has had multiple episodes of SCLS following his infusions, such that they've recently stopped his infusions to see what happens. Check out her page, where she wrote several months ago: "UPDATE: June 5, 2015: We are discontinuing monthly IVIG treatments as almost every treatment triggers a severe capillary leak episode. We are out of treatment options." I really hope that you've had a one-off episode, and that's all.

I had the hospital send what they could. I have to attempt I am a little scared for the first since I got sick.

I started having attacks on a weekly basis even though I was receiving my monthly IVIg. It seemed to start up for no apparent reason and went on for about 4 months until my doctors doubled my dose from 48mg to 96mg. I have been OK since although I have now started to get a severe rash during my infusion. Very itchy, but it disappears as quickly as it starts as soon as the infusion is finished. They are going to try another IVIg product next time to see if it helps. At the moment I am on Intragam.

Thank you for the information. Question are you still able to work.

Hi Ritz, praying for a speedy recovery for you. I'm wondering if you have been under more stress than usual ?

I am still able to work and I try not to let my scls interfere with my work. I generally get a few hours notice before each attack and as long as I am at the emergency before my kidneys start to hurt too much, it is manageable. At the time I was having these attacks, I was working in Papua New Guinea. A really poverished place which normally I would not recommend to go to, but this is part of my work.

I have had a couple of attacks on IVIG, and the current theory is that my IVIG prescription was low for my body weight. Dr. Druey has been VERY helpful talking to my Dr. to get my order modified.

I have had significant swelling between infusions but it may be due to low dose Actos for hyperinsulin, or daily hydrocortisone for adrenal insufficiency. My endocrinologist is very slowly decreasing the hydrocortisone in hopes of awaking my adrenal glands. I am wary of the Actos and hope to discontinue it as well.

Hello Ritz. My husband had to discontinue montly IVIG as they stopped being effective as a preventative measure. In the past year, 100% of his transfusions have resulted in an episode. His doctors theorize that his body has likely developed an antibody to the treatment (which is a documented risk of IVIG), and this triggers an episode. I wish you a fast recovery and hope you get out of the hospital soon. Arturo's advice about getting in touch with Dr. Druey is good advice.

Hi I hope your husband is doing well. We are going to try one bag of IVIG every two weeks and see if it helps.

Thanks Ritz. Best of luck with the treatment adjustment. I've heard that can help. Hope you feel better soon and can get away from that hospital food. :)

Back at my hospital again. Had another attack shortly after my IV I G treatment. Dr. is going to take me off of the I V I G treatments and we're going to see how that goes.

So sorry to hear, Rita! I hope it's a mild and fleeting one. As mentioned last time, don't forget to inform Dr. Druey and to have him sent lab results and episode blood samples for his analysis. We've got to get to the bottom of this. Arturo

I hope you feel better now? I haven´t had any attack since that really heavy in March. I´don´t know if it may interesting for you. I take Oktagam 10% monthly. The blood is controlled between the dosises. Everything is ok, inspite of not taking Therbutalin, L-Thyrox and other tablets I have taken before. All the best for you!

Rita, can I ask what treatment you receive when you have an attack? I presume that you arrive at the emergency department of the hospital. When I have an attack, I have a treatment plan. The plan is very simple and is effective for me. I do not know if this plan will work with everyone, but I have found that 5 litres of saline over 5 hours catches the attack and limits it to 5 or 6 hours depending on the level of my Hb when I arrive at the hospital. I have mentioned this before, but I did not get a response from anyone. It seems that for me at least, putting in the fluids at 1000ml/hr seems to overtake the leak and pulls the levels of Hb back to normal levels. The leak episode normally stops at this point and the 5 litres of fluid is easily dispersed with a simple diuretic like lacyx.After the 5 litres, I am able to leave the hospital and return to normal duties without any issues. This normally gives me 2 weeks before my next episode. I have found in the past that if the fluids are administered too slowly, I end up with 16 or 17 litres of fluid and a visit to ICU followed by massive risk of pulmonary oedema. In response to the post of Liese, I stopped taking Theopholine and Terbuteline shortly after starting IvIg. I found that the Ivig was enough to stop the episodes when my dose was sufficient. The theopheline and Terbuteline only slowed the episode down and did not prevent it.

They use a combination of albumin and saline. They are given separately. The amount of albumin depends on the Leak.