It has been a little while since I have been on here. Hope everyone is well and had a lovely Christmas and New Year. From what I have read, their has been a lot going on in the world of SCLS.
I was wondering if I could get some opinions on how to raise the topic of bring my treatment closer together with my doctor? And what is the most common treatment period? I am from Brisbane, Australia. I am the only known ISCLS in Queensland to my doctor knowledge and he really is learning as we go along, just like me.
The condition first showed up as acute form when I was 28yo. From then to when I was 47, I had a number of acute episodes where I was hospitalized and ICU admission occurred. I was always released from hospital without a diagnosis and no one having any idea what was going on with me. Pretty much the same things kept occurring My GP stumbled on SCLS in early 2018 and I was referred to an Immunologist and to a physician. At first they were very sceptical. It took them 18 months to finally give it some serious consideration.
Last year the Immunologist finally diagnosed me with ISCLS that appears to be chronic and ordered IVIG treatment. I was cycling ever 7 – 10 days, prior to being put on IVIG and was not able to function in a normal lifestyle. About once or twice a year these attacks would be severe and cause bilateral plural effusions, acute renal failure, liver enlargement and dysfunction and bouts of rhabdomyolysis, peritonitis and pancreatitis, amongst the usual pain, fatigue and weakness caused by the condition. Gain was commonly 10kg however when I have the severe bouts can be up to 20kg. I do not have MGUS.
I have been on Privigin IVIG 2g/kg for a little over 7 months now. I don’t think anyone was more surprised that it worked then my Doctor. I originally started on a double dose the first time and then every 4 weeks. This works amazingly for the first 2 weeks without any symptoms or leaks. Then around the 16 - 18 day mark I start to feel fatigued, dizzy and become hypotensive again. I then get all the other symptoms. I leak again and struggle to get to my next treatment. As a result I had another severe attack and was hospitalised. My doctor changed my treatment frequency to every 3 weeks after I was released.
The same thing is still happening, however it is just a shorter time that I have to get through between the 16-18 day mark and the 21day IVIG treatment. I am still trying to work, however for those 5 or so days, I am very often quite unwell and my work has to change my shift due to my dizziness, need to lie on the ground and how sick I get. My work is very physical and I cannot afford not to be on my game.
If I am lucky enough not to have a leak between treatments, the next treatment I am extremely well and life moves on amazingly. If I do have a leak before the treatment, the treatment stops the leaks, however it takes me a little while and medication (steroid & lasic) to recover, bounce back and feel well.
Has anyone had to raise the subject of bringing treatments closer together with their specialist? And if so, how did you do it? I am also worried that one day I will have that attack that I don’t come back from.
I have started to keep a very detailed medical journal to document my progress.
What is everyone’s opinion? Should I pursue this?