Hi there I have just been diagnosed with SCLS after experiencing approx 1 attack every month since September, with some being mild but the last 3 being much worse, although still mild compared to what I see is possible. Feeling very isolated here in Western Australia and wondering if there are any other members from Western Australia or Australia, and if so can they can point me in the direction of a clinician experienced with this condition. My doctors are lovely but have never encountered this condition before. I am an otherwise very fit and healthy, life loving 48 year old Mum of 3 and keen to do whatever I can to reverse this condition or it least get it manageable. Many thanks for any replies.

Hi jodono. I am very sorry to hear you have been diagnosed with SCLS. I am a resident of Canberra and was diagnosed with SCLS about 4 years ago. Fortunately due to the good work of Arturo, NIH and others there is now a very effective treatment available and in Australia it can be supplied free through the NBA. My treating specialist is Dr Katrina Randall at the Royal Canberra Hospital who has been magnificent and has produced a paper on my condition and treatment. Be positive, my life has almost returned to normal with the treatment. My private email is warrenlking@gmail.com.

Thankyou so much for your reply - feeling much more positive already! I am awaiting my next appointment and will make sure my doctor is aware of the work your doctor has been doing. Will email you privately if I need any more information - thanks again. Julie

Hello Julie Just feel the positives! I am in Melbourne, and you make up number 6 in Australia as far as I am aware, - including two kiddies in Sydney, with Warren in Canberra, and Gene in Brisbane. As much as we all would prefer not to have drawn the short straw, the therapy works well, - I am approaching my 1st anniversary without a trip to ICU! Whoopee Doo! I too was very fit, completing Kokoda with ease. Sometimes I think I brought the condition on by overdoing things! The treatment by the Dept of Health, through the National Blood Authority gives us Aussies VERY HEAVILY subsidised, free IVIG infusions, providing we meet the criteria. (Warren was largely responsible for getting this to happen.) If it was any consolation, I was diagnosed by a second year resident, after my third admission to ICU. My treating physician is Dr Michael Tong in Melbourne. I have found that I have to have fortnightly treatments, as monthly did not work. I am presenting a paper on the condition to my colleagues at the Australian College of Rural and Remote Medicine in Adelaide in Nov. Keep smiling John Carson john.m.carson@bigpond.com

Thanks John appreciate your reply - already feeling a bit buoyed by the positive results both you and Warren report . Never expected to be a member of such an exclusive club !! Thanks again Julie

I too was very active with running, bike riding, skiing and diving. In addition I had a busy job involving large amounts of air travel both domestic and international. I still wonder if there is not a link here. Everyone of my major attacks followed significant exercise and air travel.

Hello again - been doing lots of reading and interested in community opinion re my blood work and any ideas for differential diagnosis that I could pursue with my doctor.. During my most recent episode I ended up in hospital for 3 days and was treated just with I/v fluids slowly- my albumin dropped from 41 to 30 on day 1, my haemoglobin went up from 146 - 156 , my white cell count went up from 6.25 to 12, red cell count up a little from 4.81 to 5.14 . However, my haematocrit stayed very stable throughout the whole attack. I do have MGUS protein but unfortunately that wasn't measured during the course of the episode. Interested in any feedback/ comments / ideas to pursue. Thanks Julie

Hello Julie, Unfortunately, SCLS is just that, a Syndrome, not a completely defined single entity. The essential triad includes hypotension and haemoconcentration; oedema; and hypoalbuminemia. (refer Kapoor et al, (2010); Rabbolini et al (2013); Druey et al (2015)). Without a fuller picture of your personal condition, it is hard to help, and herein lies one of the major difficulties. In Australia, most ED notes and ICU notes are written on A3 or bigger paper, and irregular sizes. This makes it hard to collate notes into the total picture. X/Ray, and Blood test notes are easily assimilable. I suggest it would be in your interest to request copies of all your tests ASAP, and on an ongoing basis; and obtain full copies of your inpatient notes, - not just discharge summaries. Now, armed with a full picture, you can make contact with Dr Kirk Druey at the NIH, Maryland, (even through this site) as his experience is valuable. If French is your bag, then Dr Amoura at the Pitie-Salpetriere Hopspital in Paris published the European registry results in 2011. There is an adequate differential diagnosis list in Druey's review of 2010, which may be an interesting starting point. If you wish I can send these publications, - you may have them? John

Hospital, not Hopspital!

Much appreciated John - would love to obtain the publications. My email is jodono@bigpond.com. I have the feeling that I am going to have to have a few more attacks to" collect data" before I get a definite diagnosis but would really much prefer to avoid this if possible! Julie

Hi Jodono, John and Warren. I can add to the list of doctors in Australia and PNG with experience on the treatment of SCLS. In Brisbane we have Dr Peter Wood, Dr Sally Mapp and Dr Robert Bird Dr Wood has more hands on experience but all are very familiar with the condition. They are based in the Princess Alexander hospital in the Haematology Dept +61 7316 211 (PAH Switchboard) In PNG: Port Moresby - PIH Dr Ronny Koli +675 70120309 Lae - Angua Memory Hospital - Dr Kari Seki +675 76629928 AMH is a public hospital and is not much to look at, but the staff are friendly and they get the job done. I would absolutely not recommend the Lae International unless your emergency can wait for a day or 2. It is quicker to fly back to Australia. I have been treated with IVIg since Sept 2013 on a monthly basis, which worked well up to February this year when for some reason it stopped working. I started to have episodes again and was visiting ED every 3 days before my dose of IVIg was increased. To cut a long story short, my dose has been increased 2x since then and now finally seems to be OK again after not having had an episode for the last 3 weeks. IVIg due in 2 weeks, The plus side of this if there is a plus, is that I now have a regime when arriving at ED which means that I am in and out in 6 or 7 hours instead of enduring the usual minimum overnight stay. I now carry a letter which I have put together with the help of my doctors at the PA. This letter basically takes away the decision making process that has always aggravated the situation when arriving at ED. I don't want to drag on, but basically my treatment is 5 litres of normal Saline fluids with each litre delivered at 1000 ml/h. This is delivered continuously one litre after the other beginning with a blood sample to test my Hb, followed by another Hb test after 3rd and finally after the 5th. The 4th litre is infused immediately after the blood sample has been taken for the Hb, not when the result arrives. I have usually returned to a comfortable position after the 2nd litre and after the 5th litre, Hb levels are returned to normal.I have found that depending on the level of my Hb on arrival (>230) that a 6th litre is sometimes required to top up my waterworks to return to normal operation. At this point I am ready to go home. This treatment is based on the fact that after all the visits I have had to ED armed with all the information and experience that I have of SCLS and my particular symptoms. After explaining all this information, slowly and clearly about flow-rates and the reason for the high flow-rate to the ED staff and the treatment normal beginning well with the infusion rate of the saline at 1000ml/h as requested after a test of my Hb. Problems normally start with the 3rd litre because the consultant does not like to see such a high flow-rate. Then the 4th litre is not infused because they are waiting for the result of the test and so on..... Trying not to blab, but this works best for me. If any body would like to hear more about my treatment and the reasons behind it my email is genecridge@bigpond.com. Warren, I too fly very regularly and although my longest flight for a while has been 4 hours, I have never felt that it has had an influence on the onset of an episode.

HI Genecridge - thanks so much for your thoughts - we travel to the Gold Coast regularly to see family so is very reassuring to have the contact details for PAH- am tossing up at the moment whether to go at Xmas or not but this makes it more appealing! The idea of a letter is also a great one as it is scary to think of fronting up to an ED where staff don't know about SCLS - Thanks again Julie

quick correction to the number for Brisbane PAH. Switchboard number is: +61 73176 2111