Hello all. My wife was recently diagnosed with CLS. Initially her Immunologist prescribed IVIG infusions at 2g/kg, which seems to be the standard protocol. Unfortunately due to the sudden approval of the IVIG through insurance we could only fit the infusions as 3 smaller infusions across an entire month. I graciously had a lengthy discussion with Dr. Mark Pecker and he confirmed that the 2g/kg protocol was correct, but they should be accomplished as close together as possible.

Yesterday we were able to get the infusion clinic to schedule 2 longer infusion sessions for next month to get it all done across 2 infusions (one day on one day off).

Today, out of nowhere our Immunologist informed the clinic and us that he wanted to do 1g/kg going forward across 3 infusions throughout the month going forward. The only reason given so far was that he he heard that "1g/kg should be tolerated well".

This was shocking as the original IVIG script was accurate (2g/kg) and now it's been changed for unexplained reasons.

Has anybody here had trouble educating their doctors on the appropriate protocol for infusions?

My wife tolerates the IVIG very well and we just want to continue on the recommended protocol and try and live a somewhat normal life. It is quite frustrating seeing some hope on the horizon and then getting one more unexpected obstacle.

Hello 

I live in Belgum and was diagnosed in September 2019 having CLS.   In Belgium the social security approves the treatments for everyone, no (private) insurance is needed. Hospital insurances are most of the time only concluded if you are employed in a medium or big company (offered as an extra of your wage, and I do have one by my employer and the employer of my wife) and these insurances cover supplements for single room stays in hospital (with higher honoraria). My "specialist" only approved to receive 1g/kg, arguing that that is enough. I receive it every 3 weeks instead of every month. Until now I did not have any health problems receiving that dose.  About a year ago I had a common flue just a week after my normal dose of 1g and received an additional dose of 2g in my hospital. So 2g a month is not everywhere the standard and I think that some members of this community even get less than I do (0.5g). 2g will give a better protection and I hope you can arrange it with your immunologist but please do not panic if you receive less. Until now (for 4,5 year) I had no problems with "only" 1g. I admit that if I would have new attacks that I will argue with my "specialist" to receive a higher dose ...

Wishing you all a good health,

Hans

jweb84:

Depending on how they measure the dosage, mine is probably less that 1g/kg.   So far after about 15 months I've been doing ok, no futher attacks.   I wonder if its prudent to talk about actual dosage, as I get 65g  (650ml) of IVIG every 4 weeks.  I am about 104kg in actual weight. The infusion center has told me the dosage is based on "ideal body weight", but I've gone from an initial dosage of 70g (700ml) to the 65g after losing a little weight. So it's a little confusing to me on how that's supposed to work.

I have my first in-person visit scheduled with Dr Druey at the end of next month, and that is one of my top 5 questions I will have for him.

I received a question on my comment by Kevin. I tried to "Reply on Rareshare"  but that did not work. So I answer here. The additional 2g IVIG that I revceived after a flu was given precautionary..So I did not have a SCLS attack, but the doctor who was on duty that weekend did not want to take the risk and gave an additional (for me)  double dose. My normal IVIG dose I receive in my hospital under "supervision" of a hematologist (the only one in my hospital) . When he is absent some oncologist take over the supervision. One of these oncologists was on duty that weekend so she knew my situation and prescribed the double dose. She also did follow-ups of my blood data (f.i.. hematocrit and hemoglobulins).   There is also an hemtologist (professor) of another (bigger and university hospital  of Louvain) hospital that is in contact (if necessary) with my hemtologist.The professor hematologist of the university hospital  has also only 2 or 3 cases of SCLS. So his expertise can not be compared with dr Druey or dr Aziz  Amoura (Paris)

Hans

Hans,

I hate to say, in the US, getting a precautionary extra double dose probably would have resulted in a $65,000 bill being sent directly to the patient. Or a pre-approval for something like that most likely would have needed an extra appeal or two, and taken 3+ weeks under emergency circumstances. 

Please note that Hans had his extra IVIG given by a physician in a hospital.

In the United States, and I presume in most countries, medical insurance companies don't usually second-guess the decisions made by physicians in a hospital setting, so whatever you get in a hospital is not subject to pre-authorizations.

Therefore, should any SCLS patient ever need any extra IVIG in a hurry, the way to get it is to call your main physician, explain the situation, and have him/her make arrangements for you to get the IVIG in the nearest hospital Emergency Room.

I started with an IViGg dose of 2g/kg every 4 weeks in 2014. After 1 year, it was decided to put me on 1g/kg. I went along with it because (I think) IViGg can strain the body in some ways, for instance it can be hard on the kidneys, and I have had mild to moderate reactions to it. While my prescription said "every 4 weeks", I usually scheduled myself every 5 weeks. I had no problems with leaks until 2018 when I went 6 weeks between doses - that was a bad idea that landed me in ER.
So 1g/kg of IViGg worked well for me all the way up to Feb. 2021, when I got the COVID vaccines....that resulted in chronic lethergy and mild edema that lasted 8 months, when I finally thought to ask that my IViGg dose be raised a little because I had gained 20 lbs in Jan. 2020 and hadn't had my dosage increased to match my weight. The increased dose made a big difference.
My conclusions from this: 1) I believe 1g/kg is a good dose, generally speaking. 2)  It's improtant to get the doses on the schedule they set you on. 3) COVID and it's vaccines are a stressor for me.
wishing you the blessings of peace and hope - as these are good things that strengthen the immune system :)
Cathy