I was diagnosed this summer after feeling ill for quite some time. I had been feeling fatigued on an off for almost a year . I am the mother of a four year old and usually very active. I had an episode of swelling around the abdominal area summer 2014 and in July this year i started feeling ill again . It was like a was about to get the flu. The my stomach and lower back swelled. That summer they found fluid around my heart and lungs and in my abdomen . My nose was ( and still is) runny. I was hospitalized for three months while the doctors tried to figure out what was wrong with me. The bottoms of my feet swelled and made it hard to walk. One foot turned blue! I hade stabbing pains in my back , hips and chest and it was hard to breathe. Face and limbs swelled . My doctor finally landed on SCLS / Clarksons and I have had ivig treatments every month since that. Now it is starting to come back and it scares me!! I thought the treatment would take the symptoms away! Since this summer there has only been about a week when I have been 100% well, it seems like the swelling is always lingering . And now it is ale almost back to where it was this summer. Have any of you experienced the same?? Would you recommend seeking a second opinion from a doctor on fex the U.S. ? ( I live in Scandinavia ). My parents are doctors and they are both scratching their heads! My doctor is amazing and very well renowned but I can't fight the feeling that the diagnosis might be wrong? Does this seem like SCLS ?

Dear Silje, Welcome to our Community! There are a variety of swelling disorders, and while swelling tends to occur during episodes of SCLS, it usually subsides after 48-72 hours. Moreover, such swelling is normally concentrated in the extremities and not in the abdominal area. It is also usually provoked or aggravated by fluid (typically, saline) injected as part of a medical treatment process. Therefore, you are right in wondering if the diagnosis your doctor gave you is the correct one. My suggestion is that you have your doctor send your medical and laboratory history to Dr. Kirk Druey at the U.S. National Institutes of Health for a second opinion on your diagnosis. He is the most knowledgeable person on SCLS and he is familiar also with other swelling disorders. His contact information appears towards the bottom of the page "Disorder Details." Good luck to you!

Hello Silje, The first question you must ask is whether the oedema is associated with haemoconcentration. Does your haematocrit go up? The second question is whether your ivig dose and regime is correct. The ivig wears off after a variable period! Mine does not last a full month, so I have had to change to fortnightly dosing. I would strongly suggest that you change to 2g/kg/month, given in fortnightly doses. Do this before coming off the treatment. John

My IVIG treatments started causing attacks. They are currently on hold however this is the first month. I wish you luck with your search for care.

Jcarson when you get your ivig every 2 weeks now is it still over 2 days or did they take the 2 day dose and you get 1 one day and 1 2 weeks later? Shannon

Hello Shannon, I toddle off for my 5 hours of infusion every fortnight, from my favourite staff in the oncology ward. What they do for evrybody is amazing! I get half of the monthly dose.. i.e. 1g/kg each time. The infusion startes slowly at 90ml/hr, and is stepped up to 270ml/hr. So at 90kg, give or take, I get an infusion of just shy of 1l. I can feel, and measure the extra kg., but am coming to cope with it. The joy is that I start in the am, and have the afternoon free, and that I am healthier than all others in the ward! John

Hi I started IVIG in February and have chosen to have half the dose every 28 days. While it's taken time for my body to adjust with very few side effects now I am happy with the decision. Feeling energized and healthy is great. Take care and find a compassionate health professional to help support you in the correct path for you. Ruth Nolan NZ

Jcarson, I continue to receive my IVIGG q28 days, with 50mg hyrdrocortisone, 50 mg Benadryl, and some Phenergan as premeds. I am also weaning off daily hydrocortisone in hopes of awakening my adrenal glands. Because I often have a week of headaches after the infusion, my questions are: 1. Do you receive hydrocortisone before each biweekly infusion? If so, how much? I am aiming for the minimum. 2. Are your side effects different or less severe since changing to biweekly infusions? 3. Does the frequency of infusions interfere with travel? I am hoping to travel internationally again, with a safety person. Thanks in advance, [The ever curious] Susan

Hello Susan, You seem to have it worked out! I had to change from q28 because the ivig didn't last. If my level drops below 16, I get an attack. Side effects are not a great problem. Sure there are headaches, ( much less since taking daily aspirin). Perhaps these are inflammatory, and hopefully not thrombo-embolic. Still, they are no longer an issue. Terfenadine is less drowsy for me than Benadryl, and I am much better on it daily. Steroids come with me everywhere, but it is a year since I needed them, and even then I may have been trigger happy. Currently planning a trip... 5 days immediately after ivig in outback Australia on planes and trains.(should anything go wrong here, the outlook is not pretty). Then 2 weeks cruise on a liner with full medical facilities.. Thus creating a 20 day record between treatments. Gene is the person to ask about travel...now, He is a man who pushes travel! Curiosity is good! John

Hello Susan, You seem to have it worked out! I had to change from q28 because the ivig didn't last. If my level drops below 16, I get an attack. Side effects are not a great problem. Sure there are headaches, ( much less since taking daily aspirin). Perhaps these are inflammatory, and hopefully not thrombo-embolic. Still, they are no longer an issue. Terfenadine is less drowsy for me than Benadryl, and I am much better on it daily. Steroids come with me everywhere, but it is a year since I needed them, and even then I may have been trigger happy. Currently planning a trip... 5 days immediately after ivig in outback Australia on planes and trains.(should anything go wrong here, the outlook is not pretty). Then 2 weeks cruise on a liner with full medical facilities.. Thus creating a 20 day record between treatments. Gene is the person to ask about travel...now, He is a man who pushes travel! Curiosity is good! John

️Thank you so much for the response ! It is very helpful both to me and my doctor!!

John, Extremely helpful. Do you still receive IV hydrocortisone with your infusions? Susan

John, As I reread your post I note again your lack of steroid use. I now realize that you must also mean that you haven't required them in conjunction with your infusions either. This is very encouraging to me. I will discuss biweekly v. q28 day infusions with my hematologist. I will also try to talk her out of the IV hydrocortisone, furthering my attempts to revive my adrenals. Lighter side effects would be icing on the cake. I would love to spend a few days in Morocco where I lived as a child. A bit optimistic but we need to have dreams, n;est-ce pas? Susan

Hello Susan and silje, Thankyou for your encouragement. I don't get steroids at all... But still have them handy! It's like wearing a belt and also braces..lol. Aaah, Morocco! Can one ever replace or meet the dreams of our childhood? My time receiving infusions is spent learning Italian... The people around wonder why I am mumbling sometimes.. Use a program called Duolingo.. It's great! Enjoy life John

Dear Silje, Are you getting the recommended dose of IVIG? My attacks didn't stop completely until I got the full dose of IVIG. Also, after a few months of being on the full dose, I would get minor attacks towards the end of the month because the medicine would start to wear off before my next infusion. In May, I went to every 2 weeks, getting 1g/kg at each infusion, for a total of 2g/kg each month. This was because of minor swelling, not feeling well towards the end of the month, and also because I was prone to aseptic meningitis from getting all the medicine at once. I was on high doses of prednisone to try and prevent it for a long time and still getting massive headaches. Since going every 2 weeks, no more minor attacks and no more prednisone. I still feel tired and nauseous after my infusions and get a slight headache, but nothing compared to before. Also, I had major abdominal swelling when I had attacks. My stomach was my most problem area. I did have a lot of swelling in my extremities, but my swelling was not limited to those areas. Everyone is different and every disease can present itself differently in each person. If every disease was text book, everyone who was sick would be easily diagnosed. There is no definitive test for SCLS, it is a disease of exclusion. Also, you have capillaries everywhere in your body, not just in your extremities. This might just be where your body is prone to leak. Don't get discouraged, sometimes it takes a while to feel better. Your body has been thru a lot. I thought I would feel back to normal after I started my treatments. I have finally come to the conclusion that I will never feel my old normal again. I do have a new normal though. Not a perfect normal, but way better than it used to be :) I really hope things start to get better for you, Maria

Dear Silje, and others, Please take notice it is not only the extremities that are affected. By definition means 'systemic' in SCLS: "all over the body". As Arturo stated in reaction to Silje, the extreme swelling in extremities is one of the well known recognized abnormalities of wich suffer a lot of members within this community. On the other hand case histories with remarkable swelling within trunk and belly are also written down by some of the members as well as in literature Combination of both seem to happen as well, pleading for a gradual scala of presentation forms. It seems the clinical picture of SCLS has may faces, which makes it very difficult to analyse, and to deal with, as individuals and as community on this site. That might also give rise to different solutions for therapy. As we can read on this site in both therapy-regimen on IVIG as wel as T&T recurrencies can occur, and both help to change survival rates! Puzzling it is, and difficult for sure. Some of those things Silje mentioned I do recognize. Hiltjo

Dear Silje, I now have acute abdominal and lower back swelling with each attack. I have always started each attack with swelling in my face and hands. Now swelling in my abdomen and lower back, begins a few hours after the start of the swelling in my face and hands. My lower back swells so much I am unable to stand up straight. I also began having frequent attacks ( I was hospitalized 4 times in six months and then once a month) after an increase in the rate of my IVIG infusion. I had no idea why I started getting so sick all of the sudden. I am never really well, but being that sick, really put things into perspective. My 19 year old son is the one who helped me discover what was, all of then sudden, putting me in the hospital once a month (the last two times I went by ambulance). I did as my son suggested and requested that my rate of infusion be lowered and I am happy to report that I have avoided the ER so far this month! I don't know if there are definitive right or wrongs for any two people, but there is a mountain of research to support which medical approach is the most successful, so try to find a way to make IVIG infusions work for you. My advice is to ask your doctor if they have changed any anything concerning your infusions and work to solve your mystery. Good Luck Kimberly