Hi everyone, 

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?

Hi,

Dr Druey at the NIH in the US discussed it with my doctors. I receive IVIG every other week. I react to the IVIG even with premeds so I have a slow infusion rate. My dosage was increased due to the covid pandemic but I hope to reduce it after I am vaccinated. Perhaps your doctor could get in touch with Dr Druey, he and his research team are wonderful.

Take Care

 

Yes, I agree with Rita that your lead physician should seek a telephone or email consultation about this matter with Dr. Kirk Druey at NIH, see contact information at https://ned.nih.gov/search/ViewDetails.aspx?NIHID=0010170114

We have a number of patients who, for one reason or another, receive 1 gr/kg every two weeks, rather than 2 gr/kg every four weeks.  And I think there are a couple of patients who receive 2 gr/kg every three weeks, because they were continuing to have episodes towards the end of their month.

Thank you both so much for your for your replies. I will get the details for Dr Druey and take them with me the next time I see my immunologist. He is the one that is managing my treatment and condition. Hopefully he will agree to contact him and have a chat. I have premeds for a super itchy rash that the IVIG seems to causes and panadol for 2 days to stop the headach. It makes me really fatigued for 24-48hrs where I just sleep heaps. But then I bounce back and I am fine. 

Do you know anyone that has the IVIG and still needs other medications to help control it? Does everyone still work and did there lives return to normal? Sorry for asking so many questions but the fact it only last 16 - 18 days and I become unwell again is having a big impact on my work and life. 

I am very grateful for everones time and help.      

I was just released from the hospital yesterday because I had the second mild episode in two weeks. I often don't feel well in the days leading up to my infusion, and my current regimine is 1 gr/kg every other Friday.

When I started getting IVIG, I was getting 2gr/kg every four weeks, but I started feeling sick 4 or 5 days prior to the infusion. I made an adjustment to 1gr/kg every other Friday, which actually seemed to help, but over time I noticed I was growing inceasingly tired and light-headed prior to my infusion, and would start to gain a few pounds from retaining water. The symptoms would usually start sometime on Wednesday, prior to the Friday infusion. I always get my blood checked prior to the start of the infusion and despite my symptoms, I was never out of range until recently.

For the last two infusions, my hemoglobin levels have indicated I was having an episode prior to starting the infusion. My doctor ran a series of tests to see if there was some kind of precipitating factor, like a viral infection, but the results came back negative. As a consequence, I will probably need to adjust my dose to a higher level.

I'll see my doctor this week to finalize the new dosing regimine and I'll let you know what we decide to do.

Hi Di, 

I would say yes raise it with your doctor. I started having 2g/kg over 2 consecutive days each month and this also worked brilliantly to start with but in the 8th month I started feeling ill a day or two before my appointment. The following month in March 2020 I started an acute leak 3/4 days before and went to A&E. Unfortunately they did not get the on-call pharmacist out in the night and the following morning I had to go to ICU. However I was then carefully monitored and given IVIG and was out in a few days. This was amazing as my blood results were the same going into ICU the year before when they didn't know my diagnosis and I nearly died. IVIG seems very effective in both preventive measures and a treatment for an acute episode. 
After this March episode my doctor moved my infusions to 3 weekly which worked for a while but again I started feel ill beforehand. Then he changed them to be 2-weekly with a slightly increased dose and again I started getting symptoms and hospital admissions again beforehand. The final place we've arrived at is that I now have weekly infusions at 1g/kg each week. I am also having some steroids daily to further support me though reducing the amount slowly. With this treatment I am the best I've been for sometime and can function much more normally working part time and homeschooling my kids during lockdown. I few things I have learned along the way... the first is to go to hospital for IVIG treatment as soon as I feel symptoms of an acute episode, I'm more confident with this now I've realised my blood pressure gets lower but then starts to raise and significantly my pulse is 20/30 higher than it normally is at rest. It never raises like this at any other time when I'm at rest, only when starting an episode. Now I go in earlier to hospital without getting too ill and have 1g/kg and go home the same day and then usually fine to work the next day. This is a lot better than when I used to leave it till I got much worse which would need longer recovery. It was hard to live a normal life at that time. I have a care plan now which explains this and that my symptoms may seem mild but to be treated immediately. Another thing that has helped a lot in more recent months is that I've had a PICC line put in my arm for instant access for taking blood and receiving my infusion. This saves a lot of attempts getting canulars into me and taking bloods. I was reluctant to have it for some time but my arms are very glad of the break from needles as I've been in hospital many times this year. It is completely painless. Lastly just to mention for me as well as viruses, physical and emotional stresses on my body seem to trigger my acute episodes. I do less physical activity and rest more than I used to. I was very active before getting SCLS. I would also get both physical and emotional stress in my body with my monthly periods and found that I would often get an acute episode around the first day of my period. I have therefore consulted with a gynaecologist who also spoken with my hospital doctor and now am taking the progesterone only contraceptive pill. This has reduced the reduced the stresses a lot and seems to be helping.

Sorry that was long winded, but I think you might also reduce the number of episodes you are having if you increase your dose and frequency. Once I've been stable for a while it's likely my doctor will reduce my dose again and maybe that will be ok for me at some point, but he is able to be reactive to do his best to keep me well and avoid episodes and stop them progressing to far if they start. This has been a journey though. 
I do hope some of this is helpful and that you are able to discuss things further with your doctor so that you can enjoy living more normally and with less worry for your health.

Very best wishes,

Caroline  

Hi Mark,

I do hope you are also able to have your IVIG dose increased also to keep you well and prevent these episodes happening before your infusions. 

Best wishes for your discussions with your doctor also.

Caroline