I would be interested to hear about the treatment that you receive at the onset of an episode of SCLS. I have found that what works for me is an infusion of 5 litres of normal saline administered at a rate of 1000ml/hr. This seems to catch the episode in its tracks and everything returns to normal after the infusion. This means that my episodes last for 5 hours max before returning to normal. I have found that depending on the value of Hb on my arrival to the hospital, 5 litres is normally OK. If I arrive a little late and my Hb is slightly higher than normal, it may be necessary to administer another litre. The general rule for me is that if my waterworks are not OK after 5 litres, it normally takes one more litre to complete the job. After this treatment, I am able to leave the hospital and continue my life as normal. I understand that this may be something that is good for me and not for everyone else, but I have found that most hospitals do not like to administer fluids at this rate. For me this is when my problems start and I receive massive levels of fluid and then a visit to ICU. Please post your treatments here so that we can compare the treatments received by everyone.

In 2009, when I was taking theophylline and terbutaline before initiating my IVIG infusions, which have been successful in preventing the onset of episodes of SCLS for the past 6 years, I had a half-dozen classic episodes. These allowed my doctors to experiment with various combinations and dosages of steroids and fluids in an attempt to abort the episodes and minimize the risk of compartment syndrome, while still preventing damage to vital organs. Receiving 5 or more liters of saline, and as quickly as you note, would have led to compartment syndromes, so if I were to have a new episode in the future -- God forbid -- I would urge my doctors NOT to follow the approach you describe. What worked for me was to take large doses of steroids. I would take at least 150 mg of oral prednisone on my way to the hospital, and once there get 125 mg of injected methylprednisolone every 3 hours, supplemented by a drip of epinephrine, a vasoconstrictor. I would get albumin and no more than a liter of saline. As a result, compartment syndromes were avoided, my organs survived undamaged (despite very low readings of blood pressure and urine production), and my hospitalizations were cut short from several weeks down to less than 36 hours, thanks to the avoidance of surgical and other fluid-management complications. So what would I encourage my doctors to do if I had an episode now? To give me both steroids and IVIG. In fact, I still carry my high-dose prednisone pills with me.

This is such a complex disease, but perhaps I can shed some light. Firstly, at presentation, leakage leads to hypovolemic shock, with acidosis and organ failure. The quicker this is fixed, the better, rembering that the acidosis further aggravates the condition. Fluids work as fast as they are given. Adrenalin and inotropes work immediately. Prednisolone takes 4-6 hours at least. Hydrocortisone IV starts working immediately. Secondly, we are faced withe the juggling act, and this is best done in ED/ICU. The moment you are haemodynamically stable, renal funtion must be attended to. As long as your renal function can cope, you need to have the extra fluid eliminated so as to avoid overload/compartment syndrome. Third, it is difficult to assess any treatment regime as the episodes are possibly self limiting. What would happen if you received no treatment other than having your legs raised above your heart level? I had one episode with 8l in 6 hours, but suddenly after 4 hours, I sat up in bed and felt fine, normotensive, sats normal, and acidosis gone. The next 2 episodes required only 1-2 l saline over an hour before I came right. The fourt episode needed more fluid. I don't see this as a major issue. Whatever happens, the shock needs to be corrected ASAP, and the treating physician needs to be VERY aware of the resorption phase, when overload/compartment can occur. What can shed more light on your particular progress? A daily early am weight, and weighing in ED can give some idea. It is possible to measure blood volume, though not routinely available. Above all, whatever works for you... AND IVIG.