IgG levels up, no improvement in symptoms

@525gooze Welcome to Mayo Clinic Connect! I think the members will be better able to hep you with your concerns. I doubt that many members will be able to really understand all of the lab values. No one here is a medical professional. They can best help by relating their own experiences as compared to yours. But we’ll see; we’ve got some real intelligent people on Mayo Connect!

Thank you. I have never joined a forum before and wasn’t sure what I should initially add to get started. It has been impossible to get information about real experiences in dealing with autoimmune disorders, IVIG experiences etc. I receive mixed information from various sources, infusion nurses, Dr’s etc and while I do appreciate the work being done research and treatment wise I am ready to hear from others as to their thoughts etc.

Hello,
Does your doctor have any ideas on why levels are not increasing? I'm sorry this has been difficult.

I have a lot of experince with changing doses to try to get to the threshold level that reduces symptoms. Hypogam myself.
We worked with a few doctors on threshold levels of IGG. We got symptom relied over 1000 IGG. We had to both increase dose and increase frequency. That is move from 30/35 gr to 45 and go from every 28 to every 21 days.
For an overview -- there is a statement allergy and immunology association on this -- Eight Guiding Principles for Effective Use of IVIG for Patients with Primary Immunodeficiency (includes Hypogam).. The crux of this, in my reading, is the doctors seem to urge the insurance companies not to use the blood levels b/c the doctors really need to have the flexibility to evaluate the clinical symptoms. Some people get better- symptom relief- at 800.. others at 1200 trough,
I empathize with the fear of side effects. I was stuck there for a while and I found doctors too can have a wide spectrum of acceptable risk. The doctor who convinced me to try said .. I understand you're scared, but how is this working for yur lifef. She said we don't know anything really about how you will do, you just have to try for 3 months. Think of it as a trial. See if you feel better. This helped me get over the hump of only imagining side effects. She told me... We really don't know all of the risks., but there could be rewards.

Some ideas.. Maybe your doctor would be receptive to this statement from the association of doctors on how to think about it. I hope this is helpful. My other advice, is sometimes people are very set in their ways, and even if they are an expert, they have different comfort levels. For a long time, I was afraid, and it turned out okay. I feel for you because the whole thing can be scary. Sometimes finding a doctor who can balance our fears and has a bias to action can help. In my own case this really helped. The challenge is understandable and natural for the doctors and patients.. it is hard to know how an individual would react, and everything has risk. It took me 2 years to make the move, and I'm glad I did. Maybe seeking out some other opinions and another doctor would help.

one more idea.. you're doing great in asking for help... The other doctor who helped me was a very senior researcher at a medical school. She had to face the decision of whether to put her son on IVIG.. it was agonizing for her to think about the side effects. She did and it changed everything for her son. She told me to re-frame it as "liquid gold". I needed a lot of feedback from others and you might need that too. Then, the second step is to try to figure out if you're with the right doctor. What is the right time frame. Before IVIG, we did a trial of daily antibiotics for 18 months- total failure.. and another doctor said, you should have made a new choice after 6 months of failure. It's very hard to tell in the moment, but I feel like 12 months feels a bit long. I have been given advice of more like 3 months. Would they be willing to try to increase in a step wise fashion.. try 5 more grams for example? Sometimes if large moves are hard, then, reducing the increments can help with decision making and also shorter trial periods. These might be 2 frameworks that help with decision making.

I want to thank you more than I can say for taking the time to write and most of what is mentioned yes has been discussed or done.
I am speechless as the information, questions, you answered is more than I could have hoped for and much more so after six or more years of getting along and thanks to my husband who doesn’t give up, a diagnosis, confirmation then start of IVIG with initially great results but now back to square one I somehow found this forum and most fortunately for me, and luckily found you. Please believe I am not kidding!
I will read the article mentioned and hope many others find the information (if fitting) you provided.

With your hypogammaglobulinemia, do you have additional immune defects? Were you able to build an antibody response to the pneumonia vaccine challenge? I'm only asking as once upon a time I had hypogammaglobulinemia and at the time did not qualify for infusions. No one even gave me the vaccine challenge at that point. But it transitioned to CVID (common variable immune deficiency) which is when IgG is low along with low IgA or low IgM or both. That just makes me curious if something like that is occurring here. Do you have all of your IgG's measured when you do your troughs? If so, then it would show up there.

Just confirming that you have been on Asceniv for a year now and only in the last 3 months have you seen your IgG trough number rising? I'm assuming you're having your blood drawn only right before your infusion, correct? I would think that after 12 infusions and only getting into the 800's means the drug is not working well for you. It might be helpful to increase the dose by a reasonable amount. I'm not familiar with this brand of IVIG you're using but increasing by single grams seems really low when the strategy is to boost it adequately. It's possible your immunologist is being conservative since IgG has moved some lately-just not enough. But another option open to you is trying a different brand. That is done quite often, at least with those with CVID and this can't be much different.

IVIG can be hard on the kidneys and I wonder if switching to sub-q would be an option for you. Do you already have some kidney disease happening? Sub-q also stabilizes your IgG levels more evenly without creating the big dips at the end of the 28 days or whatever monthly cycle you're on.

I would also say that some of your symptoms may not be related to your immune deficiency and/or infusions won't help a lot. Migraines are not part of CVID though people can get side effects from infusions causing migraines. If that's the case, switching brands, slowing the infusion rate or switching to sub-q can be helpful. Are you getting pre-meds like Benedryl or steroids? Body/joint pain isn't part of CVID either unless it's being caused by an infection that creates those symptoms. It wouldn't be surprising for you to have these pain issues from inflammation either because your trough is so low. I developed whole body lymphadenopathy and after a big medical work-up, the doctors believe it's from the CVID itself where I am constantly fighting infections causing the lymph nodes to swell. And fatigue is terrible issue to deal with. But also be aware that people with primary immune deficiency are prone to autoimmune disease though they're very difficult to test for. This is because we lack the ability to create antibodies as the basis of an immune deficiency. And once on infusions that becomes more difficult as the donor pool antibodies will show on lab work giving some false positives. Biopsies and imaging become important tools.

I would suggest you look at the Immune Deficiency Foundation website. Community support can be invaluable for those like us. It has a vast amount of information on immune deficiencies with a lot of videos and presentations on many different aspects of the conditions including symptoms. You can look up immunologists, get help with insurance or financial help for cost issues just to name a few. I hope in the next months you'll find yourself improving and if not, keep pushing your immunologist. Make sure if you need antibiotics they cover a longer period of time. A regular course typically won't work.

Best of luck and wishes.
https://primaryimmune.org/

I would also suggest to switch to subcutaneous. I always had the “dips” when I got infused monthly. Now I switched to Hizentra and I feel phenomenal. No dips, my levels remain constant.

Thank you... that is so kind of you. I was thinking about your notes last night.
It really means a lot to me.. When you go through these things it is so hard, and it is a joy to be able to help anyone else, because if you can save anyone a little pain, it helps relieve everyone.

I also re read your note about already trying 3 grams. That for my risk tolerance is too slow and too little. There was a time when my son had gained 70 lbs.. and they didn't change his dosage, and somehow facts did not help, and he got really sick. So, he was underdosed. We finally got to move it 5 grams.. then, see if he has a reaction.. for 1 month or maybe 2.. then move it 5 more grams. Later on, because of a move to a new city, a very senior doctor just said, they were wrong. It seems like being with a doctor who is afraid of the IVIG medicine is like being with a surgeon who is afraid of the scalpel. I have a lot of empathy for the fact that

I was trying to think about a framework.
I think finding a doctor who is willing to be open minded to 1. information - what are best practices 2. risk/reward framework and 3. a good match for your personal risk profile is helpful. I also thought it might help that there is more data about the dosages for specific conditions. That is, the protocol for inflammation is often Other guidance I got was to ask questions like 1. How we will know it is working? How long would you give it to work.. at the end of the day, it seems like you have tried so much and that itself is exhausting and overwhelming. Trying to convince someone that you need help is hard. .. and here you are so close to the finish line and you're on the medicine. I would push hard to change or get to a therapeutic dosage. I understand that is easy to say, and making it happen it harder. It seems like you have done all the reasonable things and maybe it's time to move on.
It's not just you...

One more thing that helped me was from a Mayo talk on rare childhood diseases, and the woman who spoke and the team were amazing. They referenced this link David Cameron who had a son with a rare disease, and it took 7 years to get a diagnosis for most people. Here is this very powerful person who has the access the best of medicine and he has empathy for the doctors, and yet, his son was still suffering. What I take away is this.. there is a not some huge data base of pushing a button, and so try someone else who might be more open to working with you to get the clinical symptoms managed

I think of all the parents around the world going through what we did — not only the anguish of seeing their child suffering, but also the frustration at the lack of diagnoses, treatments and knowledge about what has happened and why. In many cases the doctor is unlikely to have seen a patient with the same condition before. They are left to rely on intuition and antiquated tests to determine which of the 7,000 rare diseases may be affecting the child.
https://www.linkedin.com/pulse/what-i-learnt-from-our-sons-rare-disease-david-cameron/
Even the most experienced doctors can't predict your specific outcome, and it is exhausting trying to convince someone. As a personal note, I had to put my son on IVIG when he was young, and I was too risk averse, and I needed to partner with a doctor who was more clear that we're just guessing. My son had a life changing experience for the better. He grew 3 inches in 3 months and started reading entire books. His entire system came back on line and he was able to hit milestones. Now, of course no doctor would ever promise IVIG cures dyslexia, and you will get taller too, but it happened and everyone was happy. It turns out the other doctors were clinically good, but one had seen bad outcomes with young kids being traumatized. He was trying to protect my son from that.. I would say even the best intentions .. like trying to protect your kidneys can lead to bad outcomes.

Just to add to the other comments here.. We also had to do a brand switch. We found out.. that A+ blood in our case was reacting, to the IVIG, and eventually led to hemolytic anemia. I asked the doctor and they said they don't test blood type, but another brand did more A+ antibody screening. I moved from Gamunex C to Privigen. I was getting migranes during the IVIG as my dose increased. The doctor said never heard of it.. but, was willing to try. I think the bottom line is in the realm of rare diseases, you are going to rely on more trial and error and ofen the patients have more experience. We also found the primary immune foundation helpful.. that's where I found our doctor who convinced me to go on it. She was giving a talk at a for IDF at a Marriott on a Saturday.. Anything you can do to feel more connected to that community could be helpful. Again, your doing do much. It's very brave to post and to take the first step and put out there that you don't know what you're doing. Hang in there.

I do IVIG once a month, now for just over a year. In September, my doc ordered a bag of saline to be run with the IVIG for the headaches... decreased the flow to a max of 4... and added a second dose of Tylenol at the end (usually just about 4 hours after the pre-med dose)... It all helped. I receive my infusions 28-30 days apart at Mayo Jacksonville which is where both my Rheumatologist and Immunologist are. It was a simple fix with steady labs.

Thank you for your response. I had none or very little response (from what I remember) to pneumonia vax trial.
IgG’s are checked every 3 months but not all of them each time along with basic panels and whatever she may want depending on prior results. She will check all before next infusion.
The concern with provider is kidneys which bloodwork sometimes show on lower end/normal range with last result much improved so this is why increase from 20 gr to 25, which is a really the lowest amount given with the brand.
My husband said recently, the provider is more worried about kidneys than treating your problem. She is going on numbers and algorithms which don’t always work. When I first saw this provider she said the med I am on was easy to get started with and adjustments, change of brand are to be expected but last several months has said this can be a long process lets stay with same brand, dosage, and kidneys. I am not sure how much experience the few Dr’s have with these problems as it seems allergy patients are the majority of their practice.
The migraines, body and joint painI have had for many years before but after second or third infusion lessened by 80% or so. This is another reason we don’t understand why the waiting game where symptoms are now as bad as before first infusion and IgG levels dropping along with. I also do not experience any side effects from the med. I do take Benadryl/500 mg Tylenol before as directed.
In writing this it is very apparent I am not in the right place yet. I have this forum and knowledgeable people like yourself to thank.
I have a referral to another Dr (found out yesterday) so I see him in January, fingers crossed he is a better fit.
If not there is another Dr about four hours away as another possibility. I will stay proactive, thanks again, and many good days for you in your journey.