SCIG Trial

[floopyjim]

When I was first diagnosed with a specific antibody deficiency, I was trialled on many different prophylactic antibiotics which either causes lots of bad side effects or didn't help my many infections (3-4 a month).  I was then put on a 1 year trial for SCIG which, to be completely honest, has changed my life for the better.  I have had 2 infections in 7 months.  I am now at the 10 month mark on SCIG and I saw my immunologist this morning who wants to take me off scig for a period of time to see if it has made a difference (!?).

I would be lying if I said i was not scared.  in fact, I am terrified.  I really don't want to go back to the many, many infections I had (the sum of which caused permanent damage to my lungs).  I don't know what to do.  I don't appear to have a choice in the matter.

The consultant asked me how many days off work and how many hospital admissions I had in the 18 months before scig and wanted to use them as a baseline to see how many I would have when I'm not on it anymore!

He did say that sometimes, people with specific deficiencies can come off scig after a year and don't need it again as it kickstarts the immune system - I hope it does.  He also said that if it's obvious I need scig again then I will be put back on it so it's not necessarily a permanent thing.

Just when things were starting to settle down and the infections were getting fewer and further between...  I know it was a trial but I am still rather confused and slightly in shock.  I guess having far less admissions and infections isn't enough for them?

Has anyone else heard of people with specific antibody conditions getting 'cured' with a year on immunoglobulin therapy?

[john]

Morning Jim,
I was a bit concerned when I read this post and have been trying to think of a reply that would make you feel better about this.
Firstly the fact that SCIg makes such a positive difference to your life must be proof that you still need it.
If your levels are increasing beyound that wich is supplied externally, then I would accept that as proof that somehow you weree producing your own antibodies and might not need to infuse.
My own levels are totally dependent og the infused product...they give me more and my levels increase, and if I have less they drop.
We all know the the therapy is expensive...ask whether this test is not just to save money.
To just stop, cold turkey, would scare the daylights out of me too.....in my experience I don't think I know anyone who just came off Ig and found themselves cured, although that would be a marvellous thing for all of us who are dependent on having this weird and incomprehensible therapy every week!!
If I can get you more information I'll post it ASAP
Good Luck, John

[floopyjim]

Thanks John. I knew it was a trial but one of the other consultants in the clinic appointment in January said that they wouldn't take someone off therapy if it was helping. That was echoed by a specialist nurse (who has since retired). I feel like they are playing games with my life and jeopardising my future health. What happens if I get a really bad infection that ends me up in icu? I think I would go down the route of medical negligence if that was the case.

I have an appointment with a respiratory consultant who saw me in hospital on my last admission in a month and I will mention to her that I think taking me off scig would be a bad move as any additional lung infections would damage them further. I think I will chat to my GP as well.

[prickles]

Hi Jim,

My daughter (18) who has Hyper IgE Syndrome has been on IG's for the last 8 years or so, 6 of which were done sub-cut. For her syndrome this is not a standard therapy because her IG levels are not low. She threw a wobbly as teenagers do a couple of years ago and refused to have them for a few months but ended up with lots more infections so was persuaded to go back on them via IV and she is so much better.

As John said unless your levels are increasing more than by what are being infused then you obviously need them. I am sure your respiratory consultant would prefer you staying on them.

Sue

[hlh]

My father has Specific Antibody Deficiency and Lymphopenia. He was also started on SCIG this year after 7 urinary tract infections and two hospitalizations in a short period of time. He is a complicated case though, and has some other medical problems that also contribute to his infections. He has been doing well since starting SCIG 4 months ago, with no infections. However, his doctor is like yours and will be stopping the SCIG after about 6-8 months or so to see how he does. I was also surprised, but it is my understanding that this is done. In fact, my Dad's insurance company may insist upon it, as it is a very expensive treatment.

We're nervous like you. But this may be more common for Specific Antibody Deficiency since it seems like SCIG is not uniformly used for treatment.

But when my father stops his SCIG, he will still be on a variation of prophylactic antibiotics. I'll make sure his flu and pneumonia shots are up to date in the fall. So he wont be on nothing. And we'll be watching very closely and all of his doctors will know.

I think it is reasonable to talk with all of your doctors about it, so they are aware. Keep us posted on how it goes.

[floopyjim]

Thanks All - it's good to know I'm not the only one - I won't miss stabbing myself but I am worried about how bad the infections will get because, even with the prophylactic antibiotics, I was still pretty much unable to function properly.

[s162216]

Hi Jim,

Sorry that you're so worried and scared about this, and I can perfectly understand why you would be when things have finally started to settle down. When it comes to your own health I would try anything to prevent future problems.

john, prickles and hlh, I do think it is important to understand the use of IgG in the context of specific antibody deficiency (SAD), as it is very different really to it's use in CVID, XLA etc. With SAD there isn't actually a general deficiency in total IgG or IgG subclasses (so levels are usually normal) like with more classical general antibody deficiencies, but instead there is just a deficiency in specific antibodies against certain organisms (such as pnumococal strains) which obviously makes the person at increased susceptibility to those individual organisms.

IgG infusions are usually helpful if antibiotics or repeated vaccinations don't work, but essentially they are like using an enormous great fire hose to put out a single burning match in that only a very tiny subset of the infused antibodies are actually needed to replace those that are missing - it's not really a targeted treatment. Because IgG levels are usually normal in those with SAD, there is no real way to determine if the infusions are working correctly or not, except for measuring the number of infections. You can also measure the immune system activity against the specific organism in question by doing a vaccine challenge (introducing a tiny amount via vaccination and then shortly after measuring the antibody titre), but whilst having the infusions you obviously can't do this as the infused antibodies will act against the vaccine so you can't tell whether the body is actually producing it's own immune response.

There is always the chance that the immune system may randomly invoke a response against the organisms in question (the same as with any immunodeficiency), which is quite common in children with SAD but less so in adults, which is why in order to test this it's necessary to come off the IgG infusions for a while in order to test it. The problem is that it's unknown as to how long any protection may last, which is why continual monitoring afterwards is needed. I don't know whether this may be what your doctor is proposing to do Jim, but I would ask them as to what they want to do and their course of action.

Keep us updated here as to what happens, and remember that we're here for you always.

Sam.

[floopyjim]

Hi Sam,

Thanks for that - I had a Pnumovax II vaccine test before I was put on SCIG which showed no reaction to any of the 32 Serotypes - the infections did not reduce even when I was only on prophylactic antibiotics (I currently take Doxycycline). I did have low IgG levels (less than 5 I think) before SCIG and 15 during (assuming I remembered the numbers correctly). I'll keep you posted on my progress.

[hlh]

Thanks for the nice explanation Sam.

My Dad has also had the vaccine challenges multiple times in the past, and failed them. His doctors actually wanted to start him on IVIG years ago, but he held off because he was still doing fairly well. The infections weren't frequent enough and had other contributors. His doctor was even a bit ?annoyed my Dad wouldn't start IVIG. Now that he has started them, and is doing well, it just surprised us how quickly he wanted to stop them again.

His situation is also a little tricky with the lymphopenia, and if that drops much lower he will need to take a prophylactic antibiotic as well.

[floopyjim]

Now I'm very confused - just had my clinic letter stating that the July MDT meeting approved continuing therapy as diagnosis was not Specific Antibody Deficiency ? Well, my consultant kept refering to a specific antibody deficiency during the clinic appointment?!

Later in the letter, he states that if the MDT meeting approves to continue therapy I will have an elective change of product at next prescription (I've been getting lots of headaches and fatigue)

The letter also says I had low IgM, IgG1 and IgG3 levels and they went to 11.2g/L in my June blood test (they were 15g/L 6 months prior).

I wonder if the MDT meeting was before my clinic appointment and he will be proposing stopping at the next one it on the basis that I have a specific deficiency...??!! I think he's rather confused but not quite as much as I am now! I think I will be ringing his secretary tomorrow.

[s162216]

Jul 23, 2015 17:04:04 GMT floopyjim said:

Now I'm very confused - just had my clinic letter stating that the July MDT meeting approved continuing therapy as diagnosis was not Specific Antibody Deficiency ? Well, my consultant kept refering to a specific antibody deficiency during the clinic appointment?!

Later in the letter, he states that if the MDT meeting approves to continue therapy I will have an elective change of product at next prescription (I've been getting lots of headaches and fatigue)

The letter also says I had low IgM, IgG1 and IgG3 levels and they went to 11.2g/L in my June blood test (they were 15g/L 6 months prior).

I wonder if the MDT meeting was before my clinic appointment and he will be proposing stopping at the next one it on the basis that I have a specific deficiency...??!! I think he's rather confused but not quite as much as I am now! I think I will be ringing his secretary tomorrow.

Hi Jim,

That is confusing! I (this is my own personal opinion as I am certainly not a clinical immunologist!) personally did think it a bit odd that you had specific antibody deficiency and also have lowered IgG levels (between about 7.0 and 16.0 g/L is the normal range) as to me that sounds more like a general antibody deficiency (such as CVID) than a specific one.

I think I would get onto your doctor to demand an accurate and up to date picture of what is actually going on, and to find out what condition they think you actually do have.

Keep us updated,

Sam.

[floopyjim]

The confusion is over...it turns out the MDT meeting was right after my appointment on the same day and the letter was very badly worded. They have approved continuing therapy now as my levels are looking more and more like CVID - my specialist nurse said I was in the middle between SPAD and CVID. I should have contacted my specialist nurse in the first place - she really is a star.

So, after I have used up the current stock of subcuvia, I will be changed to a different product as this one gives me headaches and fatigue.

Thanks to everyone here who, after another panic, helped me out again. I don't know what I would have done without you.

Back to the stretchy trousers!

[s162216]

Jul 24, 2015 13:25:33 GMT floopyjim said:

The confusion is over...it turns out the MDT meeting was right after my appointment on the same day and the letter was very badly worded. They have approved continuing therapy now as my levels are looking more and more like CVID - my specialist nurse said I was in the middle between SPAD and CVID. I should have contacted my specialist nurse in the first place - she really is a star.

So, after I have used up the current stock of subcuvia, I will be changed to a different product as this one gives me headaches and fatigue.

Thanks to everyone here who, after another panic, helped me out again. I don't know what I would have done without you.

Back to the stretchy trousers!

Good to hear that you've managed to get a good result out of this Jim, and I'm glad that you don't have to worry about the prospect of not having SCIG anymore.

Sam.

[floopyjim]

Cheers Sam. Next step - change of product. I was wondering how many bad habits I have picked up whilst doing scig at home. I must make sure I do things by the book when I'm back in the hospital. It will be nice to see everyone again - they are a lovely lot...

[perfectlypeg]

First, let me thank Samuel for his excellent explanation of the differences in immune treatment, I had not known that info.
Now....let me just say that I get so angry when I read this kind of treatment to someone who has put their heart and soul into the battle of their life. And then you go to a regular appointment and this person who probably doesn't know as much as you do about your disease decides he/she wants to play the "let's see how low we can go" game. Maybe I am off base here as I haven't been around in a while, but I am so tired of having people make decisions about my life that really have little interest in the results of their actions.
Yes, that sounds harsh, but it is true. I have reached the point where I believe that chronic disease is a result of the medical system passing patients around while their body systems are fighting to help each other out, all the while buckling under the pressure of not receiving the support they need. I didn't start out 16 years ago with 6 diseases, I only started out with one. But as time passed and I was passed around for this consult and that test, each of my major organs began to fail. In the adapting/preserving life manner of the human condition, all other organs came to the rally cry of the one in trouble. This only caused other systems to fail.
All the while I attended all the scheduled appointments, the procedures that usually did little good. I played the dog and pony show with all my might believing that this was to be my role to bring to medicine the generational changes that were occurring amongst members of society.
But the one thing that has never been addressed has been the role of medicine in causing these diseases to escalate and compound in multiples.
Maybe everything will turn out okay if we have to go off the protocol now and again, but that is not the point. The whole point is that neither medicine nor society recognizes the "lack of consideration" for our choices in these matters. Basic decent consideration is all I ask for and is the one thing that seems to be so hard to get.
Perfectly Peg