Immunogloblin sub class defiency

[HollyHeski]

Hi, just had a lenghy discussion with my son who had for the 1st seven years of his life had the above! He now has several health issues, including cluster headaches and on top of my issues Ive asked him to see his GP and get tested - Question is - exàctly what blood tests should he be asking for?

[HollyHeski Administrator]

Hi, he is now 26, under my old GP, before a I moved so they know all my history so wont be hard to get him tested. Just wondered if just to ask for him to be tested for Hughes or should I ask for percific tests?

[MaryF Administrator]

Two of my children have Hughes Syndrome, one also has Lupus, and my son is tinged with Hughes Syndrome and also a thyroid problem, (thyroid antibodies). MaryF

[AnnNY]

I was tested for CVID because I had two chronic infections, autoimmune diseases and frequent viruses. I went to an immunologist in NYC. She tested me for things I never was tested for before, the levels of the different immunogloblins, T cells, natural killer cells, and titers for illness and vaccinations. It turned out that my Immunogloblins were normal, but a few other things were off. I really tried to get an answer to what my abnormal tests meant, and even the immunologists resident didn't know. (Low natural killer cells).

I don't know about your access to immunologists, but I really don't think any other doctor would be that helpful. The treatment seems to be to replace the missing immunogloblin through infusions. The titers to various illnesses and vaccinations mean that the immune system is protecting the body despite any deficiency. Also, it seemed important to the immunologist that I didn't have a history of pneumonia. It seemed to me that if I hadn't had pneumonia and I had good titers to childhood diseases, my immune system was working well enough.

[ndstephens49 in reply to AnnNY]

Hi AnnNY! I tested positive for CVID 2 years ago. I have APS.

Turns out my CVID is due to defective lymphocytes--they are unable to detect infections and then to make proper antibodies to infections.

My blood was tested at Cincinnati Children's Hospital and showed defect was congenital. CVID can be acquired due to other issues, as having taken Adriamycin for lymphoma. There are also other causes of CVID.

Every 4 weeks I spend 4 hours st hematologist office and have immunoglobulins (a blood product) (antibodies) infused so I can fight off infections. I have not had a sinus infection or bronchitis/pneumonia for almost 8 months.

Yes, the Immunologist felt several bouts of pneumonia were significant.

Nancy

[AnnNY in reply to ndstephens49]

It is great that it is helping for your sinusitis and bronchitis! My sinusitis has been pretty good lately, but I'm always waiting for the next one.

The doctor I saw was a top immunologist, but she was two hours away, so I didn't see how I could have infusions regularly anyway.

[ndstephens49 in reply to AnnNY]

In the 3 year period from January 2014 to December 2016, I was on antibiotics 20 times. Mostly Levoquin as I am allergic to many antibiotics. I start out with sinus infection and within 24 hours I would have bronchitis. I have lung damage from so many infection and am now on oxygen at night. I also had at least one bout of pneumonia and possibly more. With CVID I cannot mount a normal response (antibodies) to infections and therefore nothing may show on chest x-ray or cat scan yet I still have pneumonia. I rarely get a fever also. In fact, my temp runs low when infected.

I frequently get ulcers on fingertips. During this 3 year period one ulcer was unhealed for a year. It was cultured and I had MRSA. That's the second time I had MRSA.

Much organ damage can occur from chronic infections. Chronic infections can be fatal in CVID due to multiple organ involvement.

This us why I have to put up with IV infusions every 4 weeks.

Nancy in West Virginia

[HollyHeski Administrator]

Thanks everyone, we will get him tested. I have all his notes and blood test results from Gt Ormand street hospital, while he was under the immunologist. So when we get his results for Hughes we can take it from there.