To all, Chronic systemic capillary leak syndrome might be à specific entity, but on other hand combination of acute attacks and chronic leak seems to occur in some people as well, as we can read on this forum. In literature about 10% is documented as chronic SCLS, and their data are not included in studies or general reports. I would like to have contact with those patients who are suffering from chronic leak alone as well those experiencing a combination with acute attacks. The aim is to discover which complaints and problems we have in common and which not, in à more systematical way. I'm a patient myself as well as a medic. On this topic à specific (personal) e-mail box was created to collect data. I would be pleased if you want to sent à message to scls@xs4all.nl. But feel free to discuss on this forum as well, on this topic!! Hiltjo.

Hi, Many thanks for your initiative being, just like you, confident that as all these symptoms apparently commons do not have the same origin. I will send you more explanations per message on scls@xs4all.nl.

Dear Maggy, Thank you for sending your mail, which I answered to you. I hope more of us will reply, so later on it is possible to sent all of them à questionnaire. Hiltjo

I have a chronic form of capillary leak but have not responded to the IVIG so there is some doubt as to whether it is SCLS now. I don't know whether my information is any use to you but you are welcome to it if you wish. I have also had acute episodes.I am at present under no treatment regimen......

Hi, ( reaction on Windows), Thanks for replying on this topic. Like you I have à more chronic form, and recognise the triggers you mentioned. By the way, in New Zealand, I suppose it is possible to take terbutaline bij inhalation, I'm using it as well. You are wondering if your informatiion is helpfull. yes it for sure it van be, especially because chronic SCLS is even à less frequent described disorder, we should gather the information to hopefully find à clue. I think it might be possible, Maybe we find out the common triggers and presenting symptoms in the group as à whole, and this subgroup too. So may be you will sent some information or even just your e mail adress to scls@xs4all.nl (which is my account, used for this purpose), so I can sent you à questionnaire later, on, to that account. If you have any questions about my plan I am willing to explain, of course Hiltjo

Hello, I am new to this forum but felt compelled to reply to your post. I, too, suffer from cscls. I was diagnosed by a physician at the Mayo Clinic last year. However, I have not participated in the study at the NIH with Dr. Druey yet to confirm 100% that this is my condition. I'm not sure what I have to offer to others in this forum, but would like to participate nevertheless. I am interested in finding out others' symptoms and triggers and how they compare to mine. Thank you, Annette

Hiltjo: I emailed you . I was diagnosed with SCLS ( chronic form ) by a physician at the Mayo Clinic as well as by my Infectious Disease Doctor who ran his own tests . I am currently receiving 30 grams of flebogamma IVIG every other Saturday at Beaumont Hospital . It takes 6-7 hours as i receive some fluids after . I cannot tolerate an infusion rate above 100/ml . I premedicate with Benedyrl , Zyrtec and extra strength Tylenol and double the dose of my diuretic and blood pressure and migraine meds. The goal is to increase the dosage but because of reactions I have had to the IVIG itself ( and the infusion rate ) including aesptic meniginitis ( which landed me in the hospital ) , hives , migraines , allergic reaction , etc , we are taking it slow. I am extremely fatigued for 36 hours post IVIG . I sleep throughout the transfusion and through the next day . I have lost 14 pounds since starting Ivig . At first I was receiving IVIG once a month but 3 weeks post IVIG I was showing symptoms of capillarily leak . We went to every 3 weeks . My Dr continued to test my blood and moved the transfusions to every other week . I have not had any major episodes since , but prior to my next scheduled transfusions I become weak , fatigued , swollen , and hemoglobin is high . I still experience swelling with exercise , stress , air travel , but not to the extent I did previously . Previously i was having attacks on a weekly basis . For the most part they were dealt with at home with lots of fluids and rest . I do have neuropathy in my right foot from damage from an episode of capillary leak . I have a photo of my foot from that episode . Even though I sleep away every weekend due to the fatigue from the IVIG , the benefits are still worth it . I am looking forward to increasing the dose of IVIG to obtain the full benefits . Please advise me of your research results regarding your study of Chronic SCLS. Thank you .

Hi Annette, Susan and others, Sorry for not being on the forum for some time . The coming days I'm travelling home and, I will start up again. Hiltjo

Hi all, Untill know I haven' t been able to make à lot of work on the subject., due to lack of energy. It also forced me to officially retire next july as an active medical professional, in fact I haven't been able to work the last two years. On the other hand, because of the ongoing disability my immunologist started with à new approach, starting with imatinib which indeed improves my situation. Maybe it is à suggestion especially for those having à chronic SCLS to discuss this with their doctors's. It is potentially not free of side effects, but I'm tolerating is very well. See the topic. With kind regards. Hiltjo

Hello, I have a more chronic form of SCLS. I was diagnosed Jan/Feb of 2011, started 1g/kg IVIG in May 2011, 2g/kg IVIG in October 2011, and was doing extremely well. No attacks at all except for one after using a belt sander for 8 hours straight. Won't do that again. Besides having 2 bad days after my infusion and maybe 1 or 2 days during the month that I feel extremely tired and worn out, I feel pretty much normal for the most part. I get tired a lot quicker than I used to but am learning to adjust to that. In January my doctor decided to reduce my IVIG dose down to 1g/kg/month. He told me that this was due to the fact that I was doing so well, and he had spoken to one of the french doctors that originally helped with my diagnosis and it was possible that this dose would be adequate to keep me from leaking. He also told me he was not sure what the long term effects of being on such a high dose of IVIG for the rest of my life would be since I am fairly young. Well unfortunately it did not go so well. Within 2 weeks of decreasing my dose I had a fairly severe swelling attack and was pretty much sick everyday for 10 weeks. I didn't swell every day, but felt extremely sick and weak every day and had swelling attacks every few days. Some were minor and some more severe. I missed a lot of work and was miserable. I forgot how bad I used to feel. At the end of March, my doctor finally decided to put my dose back up to 2g/kg/month and I feel almost completely back to normal. Well my new normal :) So for me, I need the full dose to prevent my attacks. My big triggers were exercise, stress and heat. My biggest complaint now is that I am hot all the time. I have been this way ever since I got sick. It's not from the treatment. I was hoping it would go away with my treatment but it didn't. Hiltjo, Maybe your doctor could try IVIG for a 6 month trial and see if this is what you need. I'm not sure if he is just not willing or it's an insurance issue, but being so sick that you are being forced to retire is terrible. I guess I was just really lucky that my doctor decided to go straight to IVIG and had it approved so quickly. I hope things start to improve for you and the new medicine helps you feel better. Maria

I am sorry for your troubles, Maria, but thank you for telling us about your recent experience with a reduced dose of IVIG. Now we know that it can be dangerous to experiment like that. I'm passing the news on to the doctors who help many of our community's patients.

Maria, Thanks for your story and advise, which I will keep in mind. Our stories (chron. leak, triggers, feeling exausted in long periods) seem to have a lot of simularities. I discussed with my doctor IVIG and we agreed ( on his initiatieve) first to try out imatinib (gleevec). as you can reed on this forum, is seems effective, especially now we found the appropriate dose.(2 months) The reason to wait with IVIG is because of the uncertainty of the mechanism (why does it work), especially because of the chronic leak, and the possibility of side effects. Your story tells us again it is not free of potential severe hazards (your aseptic meningitis is an example of dangerous problems and I understand the dosis being reduced because of that, and now being elevated in small steps), but IVIG on the other hand can be a crucial treatment.. As far as I know for now the choice is not an insurance issue, but a made for theoretically reasons. Both IVIG and gleevec are in the Netherlands medicins with some restrictions because of the high price, but avaliable. Thanks Hiltjo

Hi All, I am new to the site and am desperately trying to find a diagnosis for what I have been experiencing for the last 13 months. It started with my calves swelling, then my thighs, then upper-arms, forearms, hands, face and nose. The swelling is by no means as acute as many of the people posting here, but happens every few days beginning with me having a sore throat and feeling flu-like, followed by sensations in the soft tissues which start to swell and have a reddish tone- particularly my palms and nose. This seems to be triggered by heat, stress and certain foods and exercise. The swelling never fully goes away, and although I have managed to lose most of the stone of weight (fluid?) I put on in 1 month of this starting, I am still feeling very unwell and my health definitely seems to be deteriorating despite immunologists and endocrinologists telling me there's nothing wrong with me and all my blood tests coming back clear. Please could you tell me how I can be tested for a chronic form of this condition and if any of this sounds similar? Many thanks in advance.

The areas that swell also ache incredibly, particularly my arms, which feel very soft and 'squidgy' at the tops, along with my thighs, which feel weak and heavy. When the swelling starts up I feel particularly faint and unwell. I really apologise for posting on here without a diagnosis, but am truly desperate to discover what is happening to me. (I live in Birmingham, England and was wondering if there is anyone over here that has any knowledge of this condition) Many thanks, Rebecca.

Hi Rebecca, First of all, welkome, and no need to apologise. It is difficult to diagnose SCLS, as well as some other swelling disorders, and a lot of people experience long time while having complaints but living without clear diagnosis . I'm sorry for you to feel not healthy and insecure. There is no possibility to test on SCLS directly, especially for those not having acute attacks, but rather a more chronic leak. The latter category seems the smallest. The majority but not all patients have an elevated kind of protein (monoclonal gammopathy) in their blood, During classical attacks haemoglobulin (hb and Ht)will risé and albumin will be lowering. Please check also the disease description on this site( SCLS Details) It is very important to exclude other diseases as well, wich may be, has already been done? You describe reddishness, ( and may be kind of itching??) , I'm not sure if it is recognised by others. you can verify with your doctors things like allergy, complement disorders,mastocytosis and some other rare diseases. I hope that some other British can tell about the situation in your country. Hiltjo

Hello rebeccajane, there is on this site one patient who lives near London his name : krogers . Ask him how to do to find a good doctor to have a good diagnosis. Keep us in touch, Anne laure.

Thank you so much, Hiltjo for your kind response. I am consulting a Vascular Surgeon in 2 weeks' time and will ask if he can check the protein levels in my blood, as you suggest. Is there also a scan that would show up capillary leakage? Regarding the areas of my body that look slightly red and swollen - these are only my palms, nose and the top of my ear, which all feel warm to the touch. My arms and legs are not red. I have never experienced any itching at all, which is what has confused the Immunologists and has ruled out any allergy. The swelling is also worse on the left side of my body and is confined to my limbs and face. Many thanks for your help.

Hi Rebecca, Succes while visiting your docters. Conclusion should not depend on just a test, but an integral judgement of all data, including your recent history ,by an experienced clinician with interest in this subject and related matters, which will often be (but not obligatory) immunologists or docters in Internal medicine. Testing on monoclonal gammopathy, can be usefull, embedded in this context. Good Luck Hiltjo

Hi Rebecca, I was diagnosed with SLCS in Dec 2011 after 5 years of problem. Where are you based in the UK? I go to King's College London but I am probably the UKs expert on this disease as I have read up on it. There is also another published case in the UK in Berkshire and somebody else in the UK on here called systemicLisa but she seems to have dropped of the site. First read this site the resources part is an excellent starting point to understanding and hence confirming if you have this disorder and hence how to go about getting the best treatment. If you get in touch I would be glad to help you. My email address is: kevinrrogers@yahoo.co.uk Best of luck, Kevin

Thank you Hiltjo, Anne and Kevin for your kind and helpful messages. I will post again once I have consulted the Vascular Surgeon and perhaps e-mail you Kevin, depending on the outcome. Best regards, Rebecca

Best of luck Rebecca but remember your vascular surgeon/doctors will have no idea or even of heard of SCLS so make sure you have the information with you from this site so you can use it to confirm if your condition fits. The most important thing is does you albumin levels fall and your heamoglobin level increase during a bout of your condition. Kevin

Hello , I am new to the site and am delighted to find an opportunity to share my experience and learn of others lives dealing with this very baffling disorder. I was diagnosed with a-typical capillary leak syndrome at Mayo late 1999, there was no doubt for me that it was systemic leak when I read the scant literature available at that time. I have a very definite trigger.....any virus, be it a minor head cold or major flu will invoke a swell. The swells are not consistant in severity but are always horrible, very painful, very ill, urination slows to nothing if a major swell, and the illness lasts for weeks. I am now faced with a dilemma. I must have a aortic valve replacement and am told that it can provoke a severe attack. I would like to ask if any of you have had any surgery since your diagnosis and if there were any difficulties? I thank you for your insight. Patti

Dear Patti: Welcome to our SCLS virtual community, but I hasten to say that no definitive diagnosis of SCLS can be made unless you are measured and tested and it is confirmed that *during your swelling episodes* you suffer from hypotension, hemoconcentration, and hypoalbuminemia -- because there are several other swelling disorders out there. The fact that your swelling episodes last "for weeks" and not for 2-4 days at a time raises doubts that you have, in fact, SCLS. As to your question, no, there is no evidence that surgery triggers episodes of SCLS. Many of us have undergone all kinds of surgeries without any problem. I suggest that your physicians and surgeons get in touch with Dr. Kirk Druey at NIH to ask him about this, and see the bottom of the Disorder Details section for his contact information. Good luck to you!

Thanks for your response. The "sick for weeks" I refer to is the weakness and sore muscles and joints that takes 2-3 weeks to totally dissipate. Dr. Kapoor at Mayo Rochester informed me that heart surgery in particular can cause devastating results in leak patients (in fact any auto immune disease can be triggered with heart surgery). I have had a couple of minor procedures with no problems, but go into this with great trepidation. I have contacted NIH in hopes of participating in the study. I know the frustration of searching for answers so would like to be a part of gaining more knowledge for others as well as myself. Patti

Hi, I have just found this group and I am in the UK. My wife was diagnosed with this condition a few years ago after several years treatment for C1 Inhibitor deficiency. She has almost continuous cyclical symptoms that are thankfully under some control with Slophylin and Bricanyl. Before this treatment regime and before the treatment she received for C1 in. def. she had 2 attacks of anaphylaxis - very scary. Symptoms are, as I say, almost continuous. Shifts in fluids are very noticeable to me as it has an effect in appearance - facial swelling etc and in energy levels. Sleepless nights because of trips to the bathroom are a regular occurrence. But the severity of the cycles is much reduced on the drug regime She is currently under the care of Prof. Carrock Sewell at Scunthorpe Hospital. He is very knowledgeable about the condition and regularly visits the Mayo Clinic. My wife was his first patient, but I believe he now has a few patients under his care.