Just to keep you up to date on the 30 June I was hospitalized with my second major acute ISCLS episode. After feeling unwell a few days previous ( with flue like symptoms) I collapsed called the ambulance and was taken to hospital. I clearly had begun to leak, my blood pressure droped and I became unconscious. Rushed to ICU where they put a breathing tube in and placed me into a coma. I then started to gain 37 liters and with a purfect balance of fluids some adrenaline and wonderful medical care I made it through another episode. Eleven days in ICU and a few days on a ward I have made it home. Special thanks again must go to this site especially the 5 minute consult paper which was supplied on this forum. I always carry these papers with me and they proved invaluable this time around. So thank you to those who helped put it together. A question please can you tell me if any of you or all of you have the flue injection? I have always be very weary about having one and thought I had read here on this site not too? It appears that when they tested me in hospital I had both stains of flu A and B which they say in impossible. This is possibly what triggered my episode? The mystery of what triggers an attack and what stops me from leaking remains. All I know is I had the best of medical care the love, prayers of family and friends which now has me on the road to recovery. With not having to be cut this time I am so much stronger and feeling well again although still sore internally. Ruth Nolan Christchurch New Zealand

Ruth I am very sorry to hear of your attack but very pleased to hear your treatment was successful. Were you receiving the recommended treatment of IVIG leading up to the attack and did you receive IVIG during the attack?

Hi no I am on no medication for ISCLS due to the fact that most of the time I keep very good health. Apart from my first attack in 2005 I really have only had 2 other hospitalizations and unlike others on this site. It appears I only ever have acute attacks? Although at times I have other symptoms like itchy spots on my hands and small amounts of swelling in my legs I am really un sure they are related to ISCLS? However they always appear together and my doctor seems tho think they are related to my MGUS? So many questions and so many unknowns it appears it goes with living with ISCLS. Ruth

I get flu shots every year, and it hasn't seemed to trigger an attack. A quick note. Even if you are not on regular IVIG treatments getting a treatment while you are having an attack has worked wonders for me.

Best wishes and prayers for your full recovery. I have found '5 minute consult paper" in the Resources section of the site. Could someone provide a synopsis of the contents? It is quite pricey and I hope not to duplicate the materials that I carry. Thanks so much, Susan

Hi Ruth, I am very happy to hear that you made it through this most recent attack and are home recovering. I am like you in that I only have acute attacks. I am one of those people who never made it a priority to get a flu shot prior to my SCLS diagnosis. Pre -SCLS diagnosis in years when I had a flu shot, I always ended up with the flu. Based on a few repeat years of flu shot= flu for my body, I chose not to get the flu shot. I had my first SCLS attack in November of 2011 (beginning with symptoms of flu) and that was a year I did not get the flu shot. The following year my doctor insisted that I get a flu shot. I received my IVIGG treatment ( important to note that I was only getting an IVIGG treatment every three months at this stage in my treatment) and the following day received my flu shot. One day after the flu shot my legs began to swell, but fortunately the attack did not progress from there. I believe the IVIGG kept me from going into an acute attack. That same year that I received the flu shot I had another attack that also was preceded with flu like symptoms. This second acute attack was in December of 2012. Thanks to this site, I too fared much better than I did in my first attack. I was switched to a treatment of IVIGG monthly. I receive the treatment in 2 doses a month. Every two weeks I have a treatment. I chose not to get a flu shot this past year, and due to monthly IVIGG I have now completed one year and 8 months with no attack and no flu like symptoms even through the winter flu season. I also have an mgus and am not sure how that impacts my SCLS attacks. I believe that we all respond differently to the flu shot. For some on this site it seems to have no negative impact or is helpful to their bodies. For me it seems the flu shot puts too much stress on my immune system and shakes my peace of mind. Best wishes in your recovery and your search for answers. Cristina

I get flu shots every year with no side effects (check my profile for my history). Also if you click on "Ask a Question" on the home page, Arturo has addressed this question from another patient. It is also listed under the discussion topics but might be difficult to locate due to the sheer volume of topics.

Thanks for that info Ruth

Hello molan, Happy to hear you get better. I had already 3 attacks at home without to know wath was it. I would like to know how did they treat you ? I am curious to know that , I am like you, beetwen the crises I get well , I don ´t receive any medecine no IVIG. Thank you, Anne Laure.

Ruth, So sorry to hear about your recent episode! I'm glad that you had in hand a copy of the "5-Minute ICU Consult," although it doesn't sound like the doctors followed its advice on *cautious* fluid resuscitation -- or else you wouldn't have needed to be hospitalized for 2 weeks! You write that you "started to gain 37 liters" and perhaps you meant to write that you gained 37 pounds or kilos, or else that they gave you 37 liters of fluids. Either way, the proper treatment of an episode of SCLS should *NOT* involve or cause any such massive fluid infusion or weight gain. If you did not suffer from compartment syndromes, you did not need fasciotomies, and you did not have fluid invade your lungs, thereby putting you at risk of death, you were exceedingly lucky, indeed. Since a 2nd serious episode often leads to a 3rd, you may want to start educating the doctors at your hospital on how to manage that eventuality better in the future. And I echo Jisenhour's advice that if they are going to give you fluids, they should start by giving you IVIG. On flu and other preventive shots, feel free to have your physician double-check with Dr. Druey at NIH, but as far as I know, there is no evidence that flu shots cause episodes of SCLS, so most doctors encourage their SCLS patients to have all of their vaccinations up to date. Incidentally, the flu doesn't cause episodes of SCLS, either. Many of us get flu-like symptoms as we enter an episode of SCLS, but that's another matter -- a consequence, and not a cause, of our illness. I have been getting my flu shots yearly both before and since being on a successful IVIG therapy, and they have helped to keep me free from the flu.

Susan (Stedrick): Welcome to our SCLS community! I would appreciate your filling out your profile page more completely, starting with your birthdate and location -- never mind your relevant medical history. We need for everybody to contribute information to this community, because in the case of exceedingly rare diseases, information is what we are lacking the most. As to the "5-Minute ICU Consult" on SCLS, I would be pleased to send it to you by email. Write to me at _aporzeca@american.edu_ Arturo

I have updated the demographics this morning and will work on the medical history soon. I'll contact you by email as well. Thanks!

So good to have support of this group. I am a recently retired remote physician, previously very fit,trekking New Guinea's Kokoda track easily last year. So when I developed acute pulmonary oedema, I was more than a little surprised. Then the Hb of 220, and hct 64 became puzzling while I was battling for breath. The ED consultant saw an empty left ventricle, and gave me 10 litres of crystalloids in 4 hours with nor adrenalin. I survived without a diagnosis! Trouble was that blood cultures showed a possible septicaemia, possible contaminant. 3 weeks later the second attack included coronary angiograms, but only 1 litre of fluid. I survived without a diagnosis. A further 2 weeks later I caught another attack early, and by this time I had assembled a hot shot team who were right onto SCLS. Hb of 196 only, and I felt right to go home after 3 hours, but they have kept me in for the dreary investigations including marrow etc. I am about to go onto IVIG. I blame the extreme exercise... But then maybe not. This is the first year I have missed the flu injection. Are there any plaudits for other treatment modalities?

Hello Ruth.. Seeing you are only just across the ditch, and having spent happy times in Christchurch I empathise well. Last time there, we were in Oxford cooking! I am pinning much hope on IVIG, though I find my acute episodes managed best by minimal fluid replacement, morphine for the pain which is excruciating, but most of all BPAP respiration. Each time the attack finishes as quickly as it began, and within 12 hrs my biochem is near normal! Hope all is well John

Hi All I had a follow up Hepatitis A shot a week before my first episode of SCLS Wondering if anyone can relate to this. Kind Regards Andrew Davis