Hello SCLS community. I am a 54 year old female from Sydney Australia. After 35 years of struggling with increasing symptoms, of what I now think is Chronic Capillary Leak Syndrome. It is such a relief to find this group and realise that I am not crazy or alone. Thank you to those of you who have created this group. I have already gained much needed information. Please see my profile. I have a few questions, and would appreciate any responses. 1. Are there any members who have been treated with IVIG for Chronic SCLS, globally and/or within Australia. If there are ,did it work? and how difficult was the process. Any advice ? 2. Are there any members who have nonstop episodes without any off time for recovery. My symptoms started 35 years ago but have been nonstop now for about 3 years. 3. Are there any members who suffer from migraine from possible cerebral oedema? 4. Do any Australian members know of any doctors in Sydney with any experience of SCLS

Welcome to our Community, Annmarie! My general advice is that you should have the physician who knows your medical history the best write to Dr. Kirk Druey at NIH with as much information as possible about your case, asking him the kinds of questions you have raised. He has become the de facto clearinghouse of information on SCLS, what with knowledge of nearly 40 cases and him having published 10 articles on various aspects of SCLS in the past five years. (In the process, he even partnered up with physicians in Australia to write an article on SCLS in children, since published.) You'll find information on him towards the bottom of the page "Disorder Details."

Hi aporzeca, Many thanks for your advice. It is much appreciated.

Hello Annmarie, Welcome to the club! Just about everybody on the site has come after many episodes, some minor, and other very debilitating. There are probably 6 or 7 Aussies with CLS.. Brisbane, Canberra, Melbourne, ?Perth,a couple of kiddies in Sydney (PoW), and yourself. I believe most of us are on IVIG, (thankyou Medicare), and it is readily available through our system, as long as it is justified. Yesterday I celebrated my first year free of symptoms after starting IVIG. It's a life saver for me. Dr Rabbolini in Canberra has published his experience. I have a presentation for the Australian College of Rural and Remote Medicine coming up in Oct (Adelaide conference). Dr Michael Tong (Melbourne Pathology) has experience. I am not sure of the identity of the PoW immunologists. Dr Kirk Druey is undoubtedly the repository of cases in the US, but transferring documentation into the US system can be fraught. Of note is the fact that myalgia occurs in 56-76% of cases. It is not all about overt systemic oedema. However the major adverse effects cause compartment oedema or visceral oedema including pulmonary, pericardial, or cerebral. In order to document it, a meticulous daily weight is important. Showing haemoconcentration through haematocrit reading is a great help. Hypoalbuminaemia, in my experience, is a sign of really severe attacks, and not present often. IVIG can almost be seen as a therapeutic trial. If you don't respond to IVIG, it is unlikely that you have it. If you respond, it is a good sign. However one of the adverse effects of IVIG can be debilitating headaches. We also do not know correct guidelines for dosage and frequency of IVIG. I get mine at 2g/kg/fortnight aiming at a trough level of IgG of 20G/l. Which ever way, keep strong. Feel free to contact me at john.m.carson@bigpond.com John

Hi John, Thanks for the reply. I am glad to hear that IVIG is working , and that it has been a lifesaver for you. I do hope to be able to trial it, in the future. The one advantage of having 4-5 mild attacks a month is that a one month trial will give a lot of information. I do hope it won't aggrevate the existing migraines, but I guess one step at a time. I am awaiting an appointment date with Dr Katrina Randall in Canberra. I hope she is more able to assess and hopefully if appropriate offer a proper diagnosis. I intend also to ask my Vascular Physician or Dr Randall to be in touch with Dr Druey. I am very curious to know if there are others, where SCLS induces migraine. I am yet to read of anyone else experiencing this. Daily weight, blood pressures and blood testing have been done over a week, however temperatures dropped suddenly on the week this was being done, which made for less swelling than usual. There were some considerable movements in the blood work but only from one end of the range to the other. Weight varies generally 3-4 kg between the leak and retrieve stage, upper and lower BP can both vary up to 50 points between stages, however I also have orthostatic hypertension, probably from venous pooling. I will obtain a Hemocue as that might give valuable information. Thank for the info on you Dr Rabbolini, I will spend some time tomorrow looking him up. Thanks again Annmarie

Hello Annemarie, Just to clarify. I get IVIG at 2g/kg every month but have to receive half every fortnight as it does not last the 4 weeks. I used a hemocue daily, sometimes twice, for 4 months but was unable to extract any useful information. There has been a recent change in the hemocue agency arrangements. They have been taken over by Radiometer Pacific, although hemocue still trade as The Cosset Group. Radiometer is at 1800247254. Cosset is at 02 43846855. Prices between the two may vary greatly. There are othe Aust contact esp Brisbane, on the notice board. John

Hi John, Thanks for the extra information. I wonder, if maybe the hemocue is maybe not as useful for the more chronic versions, where haemoglobin levels vary less ? Does anyone have experience using it with a more chronic condition. Many thanks Annmarie

Hi Ann Marie. I am in New Zealand and of you look at my profile you will see I am a chronic 'leaker'. I had IVIG for about 6 months in 2012 and had some success with it. Unfortunately it gave me hugely raised liver enzymes (which is not a common side effect) though one which people should be aware of, so I had to discontinue it. I also had all the other drug treatments first to varying degrees of success. Pseudoephedrine also helped me to a degree. It seems the IVIG is the treatment of choice and may require a bit of individual tailoring as John said above. All the best!