There are many protocols likely due to the fact that the disease is really rare and very, very variable in different people. So different ways will work in different people. The one common thing about most of the protocols is that the first dose (or loading dose) adds to a total of 2 g/kg (as your 0.4 g/kg for five days did). Most of the rest of the protocols call maintenance doses a total of 1 g/kg. there are arguments from pharmacokinetics that a smaller dose twice a month is more effective which makes logical/mathematical sense, but I have never seen anyone actually document in a person that this works by levels. I think the greatest argument in all of this is that if something works for you, then this should be used and then slight modifications considered for reasons of quality of life–so I think you are right on to ask to go back to the way that worked with “loading doses” of 2 g/kg for the three times and see if you are significantly improved as before and if so, then consider a maintenance schedule.

Regarding steroids, one thing to think about is pulse steroids–in which steroids are given once a week or once every other week or even once a month in large doses. there are fewer long term complications from this and there are a number of papers showing that this can be effective. However, immunoglobulin worked for you to improve you a significant amount and does have fewer long term effects, so try this first.

Remember the goal is to be aggressive enough to stop continuing damage and to maintain function so you want to try to get as much back to normal as possible and try to stay there. You will not be 100% normal because some damage just will not heal well enough, but this is a chronic illness for which there are few possibilities of cure and immunoglobulin and steroids are not stopping the fact that there is disease there, just limiting damage. They are not like antibiotics that get rid of the source of the illness–rather more like glucose controling medication that keeps the disease from getting severe and then causing terrible damage.

Good luck to you.
WithHope for a cure of these diseases