IgM deficiency

[2boyzmama]

Does anyone have any experience/information about IgM deficiency? Connor's most recent labs came back with normal IgA and IgG (which he's always had) but low IgM (35, dr told me the lower limit was 47).

The dr (Infectious Disease) isn't concerned, he thinks it's likely a one-time thing. He said that he'd be more concerned with a high IgM vs a low one. I did a quick google and found this link http://emedicine.medscape.com/article/137693-overview and towards the bottom it actually lists my son's syndrome as a possible cause of IgM def. It also lists several infections in the mortality/morbidity section that he has had (and he had a really hard time recovering from a H. flu. infection almost two years ago, which is one bacteria listed).

So at first glance, I'm not so sure that this is as benign as the dr said.

Connor's t-cell and b-cell tests aren't back yet, previously he had a low t-cell (TD4 and T-4) categorized as "moderate primary immune deficiency" but I don't know what his most current levels are.

The issue of prophylactic abx has been discussed several times, but his labs and illness history have never warranted it. Aside from that invasive H. flu. infection he had, every illness has been easy to treat, and since his immune system is partially functioning and capable of "learning", we haven't gone out of our way to protect him from infection. The drs have all explained to us that it's actually beneficial in this type of immune deficiency to let him get sick and hopefully build antibodies (as long as he recovers well, which he so far has). But does this possible IgM def change things?

[Satori]

I am really scatter brained today but if I remember right IgM basically programs the B cells and if you have low IgM your going to be shooting blank B cells so your likely to not hold vax titers.

[2boyzmama]

Hmm...that's interesting to note, Satori. Because his b-cells have always been normal in COUNT, but we suspect they're not normal in FUNCTION because of his illness history. Particularly the H. Flu. infection he had...his t-cell deficit supposedly only makes him susceptible to viral infections, not bacterial, yet he's had so many bacterial infections. I'm anxious to get his new t-cell and b-cell test results next week.

And it's interesting you note vaccine titers...we haven't done any vaccines with him (only Synagis, which is an immunoglobulin, and he DID get RSV while receiving the series, although it was only a moderate illness). The Infectious Disease dr has been after me to allow him to do vaccine titers, but other drs (including his Pulmonologist, who is really better at explaining all this immune stuff to me) say that it's highly unlikely that he'd mount any kind of immune response to a vaccine anyway. So vaccinating him will only prove that, won't do him any good (as far as protection from disease) and of course still holds all the risks of vaccines (to whatever degree you believe they have risks. His pulmonologist, for example, doesn't think there are any real risks, but agrees that there's little to no benefit, so he has never once pushed me to vaccinate against anything).

I found several more articles talking about Selective IgM deficiency, but many said that usually t-cell counts and functions were normal. Which his has never been.

This is all fascinating to me...

[Satori]

This is one of the problems we had with my older dd, her counts were normal but no one had ever tested function since she wasn't vax'd. With my little one she got her 1st pneumonia at 2 months old in the middle of summer no less and it was one thing after another. I decided I was NOT going though the hell with her that I did with older dd and allowed her to get HIB and Prevnar which would test both sides of the immune system. She had ZERO response and between that and her constant infections they ordered IVIG to start. Unfortunately that got us kicked to a major PID treatment center which prefers to take a wait and see approach and make sure its not the transient form.

Anyway, honesty I would allow them to do HIB and Pnumovax (assuming he's 2 or over) to get the function test done and get treatment started. This is one situation where benefit outweighs risk big time. Leaving a PID untreated can be very dangerous and seriously impacts quality of life. Treatment really improves things and not being able to do the function tests really drags things out.

[2boyzmama]

I guess I need to research more...I was under the impression that he wouldn't be a candidate for IVIg unless he had a specific Ig deficit (and IgM deficit doesn't appear to warrant IVIg according to what I've found so far) but you're saying that if we discover (through vax titers) that he has a b-cell dysfunction, then there's something that can be done??

His first pneumonia was at 9 weeks old and in the summer, plus he had a UTI at that time. I truly can't count how many ear infections he's had, he's had two more pneumonias, two cases of RSV, one severe case of rotavirus, one invasive H. Flu infection (that started as an ear infection), Chicken Pox, Impetigo twice, an umbilical infection (at 2 years old, not as a newborn), H1N1, and countless other "colds" (manisfesting as URIs). He had IV abx for the first pneumonia/UTI, he had a PICC line for the H. Flu., and he's had oral abx for every ear infection (cultures are H. Flu. or staph, sometimes several different types of staph). He's also had "cradle cap" since an infant, and is very prone to yeast.

It sounds like a lot (and I guess it is) but with the exception of the H. Flu. and the recurrent yeast, everything has been easy to treat. I've been told that this is just what to expect with t-cell deficits and that although it sucks, every infection helps to "teach" his immune system and eventually it will function better. From what I've read of other 22q kids, that appears to be the case, that they are sick frequently in the first couple years, but it gets a lot better when they're older.

I asked whether we should be sheltering him more through these early years, but I was told (by several drs) that it would only put off the illness "phase". That his t-cell count will probably never go up (because it's caused by his underdeveloped thymus) so we just need to get the function better and we do that by exposure to germs. And since he's breastfeeding now, better to expose him now when he has the assistance of breastmilk to get him through an illness.

Does all of this make sense? Have you heard/been told differently?

[momtoalexsarah]

Reading that article is makes sence in terms of Megan as well - her numbers are on the lower end of normal - when she is healthy, and when she isn't they take a huge hit.
She has repeated URI - always catches the same one at least twice (is the first to get it - gives it to the rest of us and gets it again on the way out)
Megan has had the HIB and prenar - She did not origenally hold good titers for these, but they where given at a time when her immune system was stressed by chronic ear infections. We did an extra booster on both this summer - and had titers redone in late sept when they had to do a pre surgical blood draw. Her titers where within range at that point so we know that she needs repeated exposure to build responce.
Strep is Megans issue - it was strep that caused the sepsis after the cleft surgury, and she is considered chronicly infecteted - and it can't be treated unless we want to risk C-Diff with abx again. At this point we are waiting - and she is doing ok.
She had bronchitis w/ bronchal spasms about 2 weeks ago - but that was after a single vomiting episode that caused so much acid damge it irritated the airway that bad. He airway is still very reactive to stomac acid. She is refluxing again, so I am going to have her pead look into more advanced allergy testing. She has the digestive wheat reaction (possible celiac) so other allergies would not be a far stretch.

[heatherdeg]

You might want to x-post this in Health & Healing. I'm almost positive I found info on ds' IgA deficiency there.

[DaughterOfKali]

My son's doctor actually said it would be a GOOD idea for me to homeschool my son this year to try to give his body a rest and a chance to 'recover'.

We did do the Pneumovax and then tested his blood a month later to see if his body made antibodies from it. His body did make them. They were a little low, but it was a positive sign. I recommend that test, if possible. It gives the docs an idea of how severe the deficiency is.

[heatherdeg]

Originally Posted by DaughterOfKali

My son's doctor actually said it would be a GOOD idea for me to homeschool my son this year to try to give his body a rest and a chance to 'recover'.

You can get a 504 (like an IEP) to cover home instruction where they send a teacher to your house to teach your child if your doctor writes this up. I'm not saying anyone needs a teacher to teach their child (I'm a former teacher and extremely pro-hsing ) but for those who really aren't sure they can do it--you should know that the option exists.

Some districts like to do this via IEP because they get funding with an IEP that they don't with a 504. Since new legislation allows a parent to end IEP services without consent of the "team" as of earlier this year, it shouldn't make a difference to the family/parent.

[DaughterOfKali]

My town will not send anyone out. Not a teacher, nor his therapists.

[preemiemamarach]

Originally Posted by heatherdeg

You can get a 504 (like an IEP) to cover home instruction where they send a teacher to your house to teach your child if your doctor writes this up. I'm not saying anyone needs a teacher to teach their child (I'm a former teacher and extremely pro-hsing ) but for those who really aren't sure they can do it--you should know that the option exists.

Some districts like to do this via IEP because they get funding with an IEP that they don't with a 504. Since new legislation allows a parent to end IEP services without consent of the "team" as of earlier this year, it shouldn't make a difference to the family/parent.

Yep, we are going to be doing this with public preschool this fall (DS turns 3 in July). Mainly I'm doing this because it will allow us to have therapies at home continue as well.