Hello all,

I am very grateful to Auturo and this group as it has provided a great source of information and support these last 18 months since being diagnosed with SCLS after my second leak episode. 

I have two different questions I would be glad to hear anyones thoughts on if possible...

1) Has anyone had the IG levels monitored in their blood to help identify the dose of IVIG they need to stay well? - Possibly if your doctor has looked to reduce your dose? If yes then do you know what levels have proved too low for you? Or what levels work for you?

After 8 months on 2g/kg every 4 weeks and keeping well I had a leak episode 3/4 days ahead of my planned infusion and went in to ITU in early March this year. (I had felt symptomatic the day before the previous month also) My doctor increased my dose of IVIG after that but unfortunately I had further leak episode and emergency admission in June a day ahead of my planned infusion. My doctor then split the same higher dose but a 1 day infusion every two weeks but I had yet another emergency admission the night before my planned infusion last week. My doctor is now taking blood test every Monday and Thursday to monitor the IG levels in my blood to see if that helps understand the level that will work for me.

2) My 3 children age 11 and under will be going back to school in September. As for so many, it's been hard for my family with my husband and I working from home and caring for children since mid March so we are all looking forward to this change. However like many of you we have been shielding in recent months. I am now wondering whether to live separately to them when they go back to school to continue shielding in the hope of a vaccine for Covid 19. My husband can get childcare after school to help with this. I wonder if anyone else with young children is thinking of taking similar measures to help their family live more normally? Or perhaps people may continue to keep their children at home and not to school? I realise everyones situation is so different and we are all just trying to do the best we can in the circumstances we find ourselves in during the pandemic.

Very best wishes to you all.

Thanks Caroline

Hello, I will start with the children.  I will first extend your return to class for a while.  Then I will stay in my room in isolation until I get a vaccine.  I admit that it is extreme.  What you tell us about IVIG is very complex, and disturbing for those of us who try to see a certain normality in IVIG.  I would think about doing a weekly app, a little bit higher, let's say 10%.  I clarify that I am NOT a doctor.  Do they always use the same brand?  I find it strange that for 10 years IVIG has worked perfectly and 2020 finds us with 3 cases of failure.  I ask that you please keep us informed and keep Dr. Druey and Dr. Amoura informed who are the people who study leek.

Hi Caroline,

I am sorry to hear IVIG has not stabilised your condition.  Is there any clues to what is triggering the episodes?

My children are a little older than yours but I have exactly the same dilemma.  I have no real options to live seperately but will try to isolate as much as possible in our house.  Luckily we have quite a large home with 4 floor levels which will makes this to some extent feasible.  Things are not help my myself and my wife being teachers, although I will be teaching from home.

I think it is now well established that children under 10 years old are weak spreaders of the covid disease as thhey have low levels of the ACE2 receptor in the lungs which the virus uses to enter cells.  Hence the risk is much lower in younger primary school aged children. 

These are really difficult decisons but my youngest got a fever  few days ago and it was really worrying but we have all tested negative for covid but this alone was very stressful. 

I have no idea about the IgG levels although these are monitered but probably varies with the different people.

Best wishes,

Kevin

Thanks very much for your messages Areilbatt and Kevin. Yes I think I will take the approach you mention and try and live separately inside our home in the first instance and reconsider if the infection rates increase but our children still in school. Kevin your situation does sound tricky with older children at home and also your wife in educaton too. I'm glad to hear you are able to continue working from home and you have good space to isolate as much as possible at home. Yes as my children are young, the risk should not be high, that is something I can bare in mind. The school are also staggering start and finish times which should help with drop off/pick up of the younger ones.

In terms of these episode while on the full recommended amount of IVIG and higher I have a few ideas but not sure. I realise it is very strange as it works well for others. One thing, although I haven't had any Covid symptoms it's possible that I came into contact with it at work. Following my first emergancy hospital admission while on the recommended dose in March, I returned to work from home as my workplace had sent employees home to work. I found out then that a colleague who sits a few metres from me had Covid symptoms and later tested positive for the virus. This is therefore a possible reason I've been having problems although I had felt symptomatic the day before my infusion the month before also. When it became possible to have an antibody test in hospital in June I took the test but this was negative. I understand now that a T cell test is more accurate but I don't think they are available. My only other idea is that it has been quite stressfull in lockdown and I know emotional stresses as well as physical stresses seem to trigger my leaks. My doctor has updated Dr Druey though not made contact with Dr Amoura before, though I have now passed on that contact to him. 

Yes perhaps the IG levels varies with different people. I have had two types of IVIG since I started depending on which is available either gamunex or flebogamma, also my dose had reduced by a bottle after the first 6 months as I'd lost some weight. One thing I have notice through this time is that I know very clearly when a leak is starting. As well as the thirst, heavy/achy arms and legs and feeling tired, my resting heart rate increases. I took my blood presure and heart rate daily over a number of months and for me my resting heart rate is always in the 60/70's. The only time when it went into the 80's was when a leak was starting and if it went into the 90's I know I should be in hospital. My heart rate is rising to support my blood pressure which had been dropping - then my blood pressure goes up a little. Also IVIG is very effective to stop the leak when it starts.  When I went into ICU in March apparently I was in the same condition when I went in the very first time when I nearly died, This time they had started the IVIG treatment and I quickly got better and was out in a few days. I do wonder though if I have been in a cycle that the IG levels in my blood are reduced as it has somehow got used up treating a leak and then not lasting as long to act in a preventative way until my next infusion.

Thanks again for your response.

Best wishes,

Caroline

Hi,

Stress has certainly been a trigger for me too as well as viral infections. 

T cell immunity is a seprate way the body fights infection to the production of anbtibodies.  Some scientist are specvulating that the reason for fairly low levels of people with antibodies to covid is due to T cells being the mechanism to fight covid 19 infection.  Its all rather speculative at the moment.  Where you test for the virus when you went into hospital by the normal RT-PCR test?

Best of luck,

Kevin