Ivig reactions

My daughter has had ITP for over ten years, the first blood episode was anemia and they had a very difficult time finding a donor due to her antibodies. Will never forget the BP 50's over 30's for two days and on a Dopamine drip. The next blood issue was ITP around 18 months later, she gets IVIG and platelets, and dex. Her second to last ivig caused aseptic meningitis, the last reaction started within ten minutes, beet red eyes-face-body tight chest, retreated with Benadryl and more steroids. Later that night around 3 hours after platelets her blood pressure dropped quite low. We are almost a year out and I am getting nervous about her treatment options. She bottoms out very quickly, norm bloodwork Wed and in ICU Friday with no measurable platelet count. Has anybody else developed more severe reactions as time goes on?

Wow! What a nightmare! My wife had aseptic anemia from IVIG and ended up spending four days in hospital. I know how scary it was for a grown woman. It must have been terrifying for it to happen to your child.

Was the anemia "autoimmune hemolytic anemia"? Does she have Evans syndrome?

Did they say her reaction after the last IVIG was anaphylaxis? The contraindications for IVIG say, "GAMMAGARD LIQUID (immune globulin intravenous (human) 10%) is contraindicated in patients with known anaphylactic or severe hypersensitivity responses to Immune Globulin (Human).

Yes that is what they called her anemia. They did not call it anaphylaxis, but I sure think it was.
I am not familiar with Evan's Syndrome, at one point she was diagnosed with Sticklers Syndrome by an Ophthalmologist at the Cleveland Clinic, but that was just one of dozens of things they say she has had over the years.
The hematologist told me before the last transfusion that some people don't think you should receive IVIG after a bad reaction (meningitis)that you should not get it again, but he didn't feel that way.
She did tolerate the drip after it was slowed down to 10 and steroids, so I do believe the speed of the drip makes a difference, so does the ER nurse, now! I told him I had read that a lot of people blamed getting the meningitis after too fast of a drip and he did not believe me, told me sometimes the internet is not our friend.

I'm glad it went better for her. What a silly thing for him to say....the internet is not our friend! I get that, but there is no harm whatsoever in slowing down the drip.

No one called it aseptic meningitis, but my daughter had a severe headache and nausea for 12-24 hours after she had IVIG. It started just as she was being discharged from the hospital, so we dealt with it at home.

If she needs another IVIG, I will definitely ask them to slow down the drip. They told us her's would be about 8 hours, but it was finished in 4. I don't know if someone made a mistake or what, but that was too fast for her.

Evans syndrome is defined by the simultaneous or sequential development of immune thrombocytopenic purpura and autoimmune hemolytic anemia. In about 50% of cases, it is associated with systemic autoimmune disease, such as systemic lupus erythematosus, lymphoproliferative disease, or primary immunodeficiencies. - www.ncbi.nlm.nih.gov/pmc/articles/PMC4463807/

The diagnosis of Evans syndrome can affect the choice of treatment compared with ITP by itself.

As I understand it, the doctors are only treating your daughter when platelets are too low, rather than treatment to prevent the drops. That is fine if the drops are not too often or too rapid, and if rescue treatments are effective. You might want to reconsider that strategy now that she is having trouble with the IVIG; much more is known, and there are more options, today than ten years ago when your daughter first began treating for ITP. But, I realize that your daughter's case is far more complicated than what we usually see here.

Her drops have been happening every 12-18 months ever since the anemia, the treatments usually help within 24-36 hours. They have no idea what causes them to drop I was actually told by more than one doctor that sometimes we have to accept that there is no diagnosis and just be glad we can treat the symptoms when they occur. It has been many years but she went through the Cleveland Clinic, University Hospital & Rainbows Baby & Childrens Hospital with bloodwork sent to labs in California.

She was sick often as a child and one time they thought she had pertussis despite having the appropriate vaccines, so they gave her live vaccines and a pneumonia vaccine and took blood samples several times in the days and weeks following. After two weeks she showed no sign of ever having the vaccines.

She has been a medical mystery since she was small, nobody ever knew what was going on with her and she spent a lot of time in the hospital. It seems I have stuck with the doctors who treated her symptoms the best and gave up trying to find an answer.

I will say that her family history is quite extensive, back when I was 14 my family was in a study that was published in JAMA when they were just realizing that arthritis was genetic. She has RA, Scleraderma, Chrone's, Osteo, Fibro in her immediate family and my nieces and nephews have issues as well.

She went through a study at Rainbow's where they concurred with Sticklers Syndrome but told me we might want to stop short of DNA testing so she would not be labeled for insurance purposes, so we did not opt for the testing. While some of her symptoms fit, many do not so I never truly accepted the diagnosis. However look up Lyme Disease and she has 95% of the symptoms!

It took a year and a half to get her Interstitial Cystitis diagnosed and in the mean time she had a breakdown mentally because nobody believed her, so she was sent to a mental ward while she was peeing blood. Poor kid has been through the ringer and back.

I will look into Evans Syndrome a little more, thank you.

I think Katie is starting another episode, I was wondering if I started to get Benadryl into her system for a few days could it possibly lesson the reactions? I hope I'm wrong but she is acting like it is starting, and she has a few more bruises than usual (new puppy doesn't help)the bad thing is that when she starts bruising and I take her in her blood work is OK, so we have learned to wait until the purpura starts. Probably right before Thanksgiving, poor kid has missed so many holidays

I really don't know. It probably doesn't last long enough to have an accumulative affect and the recommended dose might be too small to make much difference. You should ask her doctor.

From what I've read the purpose of Dex premed is that it allows Benadryl to get into the brain better. Also, steroid reactions can very from person to person. Is it possible that one of the other steroids may be better for her like Methylprednisolone if she is not metabolizing Dex as most others do?

When I took IVIG I had a headache two days after treatment - which was when the Dex wore off. Unlike Prednisone, Dex takes a while to dissipate from the body. Since your daughter had a headache immediately, it makes me wonder if the Dex was working at all for her.

You mentioned she got more steroids on reaction. It is possible she got Methylprednisolone instead of Dex then? If so, maybe she needs to start out with it instead of Dex.

Ask to try a different brand of IVIG - here in the UK the preferred brand is Privigen.

I have reacted with a tightening chest and getting itchy to Flebogamma in the past.

I now have a litre of Privigen in one hit without pre meds once a fortnight without too much issue apart from generally feeling a bit rough/tired for a couple of days and occasional slight muzzy but bearable headache.

Speed of infusion is critical for people who react - ever so slowly does it!