Hi all. I just found out my 3 yr old has Hypogammaglobulinemia. He was diagnosed with c4 deficiency at 2 yrs due to having ? atypical Kawasaki disease at 16 months. I know that he is prone to bacterial infections and has a 75 % chance of developing Lupus due to the c4 deficiency. I also know that he didn't respond to his prevnar and meningegate vaccines and has to revacinated, for the 3rd time. What I don't know is, what is Hypogammaglobulinemia and what does it mean for him? I have a 13 yr old with the C4 deficieny and he is pretty healthy, as is Luke. They are never sick, or so it seems. My 13 yr old always has sinus infection but aside from that he is pretty healthy. Does anyone have any information for me on what this all means?? Thanks
Hi Wendy ... I know its hard not to panic when you are the parent (I have four kids!!). I am not a doctor, but I do have hypogammaglobulinemia (also known as an antibody deficiency) so I might be able to offer some insights to help you out until you get some solid answers from an immunologist. Hypogamma means that the patient is not producing enough immunoglobulins - that's where we get the antibodies from to protect us from disease. The most prolific one in our bodies is IgG and your son is likely deficient in this and perhaps some others. Those of us lacking IgGs tend to be very prone to infection, although there is a huge range in how this affects different people. The good news is that it is treatable and most of us with hypogamma lead long, relatively healthy lives, especially if the condition is discovered early in life before there is long term damage to lungs etc. The prognosis for your son would be very good I believe. The treatment might involve regular use of antibiotics and agressive treatment of infections ... or it might at some point involve monthly infusions of gammaglobulins (this is known as ivig if you are doing a search). IVIG is a blood product that essentially gives us someone else's immune system for a month. It is very effective, but wears off in a few weeks, hence the regular infusions. There is an organization in th US called the Immune Deficiency Foundation. You might want to check them out. I hope you are seeing an immunologist about this and that you get the problems and treatments worked out soon. It might be a good idea to have your 13 year old's immunoglobulin levels checked as well. Chronic sinus infections are typical with many kinds of immune deficiencies. Let us know how you are making out and if we can help with any other questions.
hi there and thanks for replying. Luke is low in Iga and Igg and is not producing antibodies. He sees and immunoligist for his complement deficiency and he is the on that told me that luke is low in these areas but he didn't tell me the name of it. I read a letter that he sent to my GP and in that he stated that Luke had hypogammaglobulinemia. I think he also tested my 13 yr old for this but I don't think he has it but the c4 deficiency will also cause the cronic sinus infections. When did u get diagnosed with this? Do you take IVIG for illness? Are you sick very ofter? Luke had IVIG at 16 months when he became very sick. They thought he had cancer and actually told us he did and then said that they were pleasantly surprised that he didn't have it and diagnosed him with atypical kawaski disease. Atypical because it caused bone marrow failure. a very scary time. thanks for the info and will look forward to hearing more.
Hi Wendy - sounds like you're on the right track with the dr. That must have been very scary when Luke was so sick. I was diagnosed with an "immature immune system" when I was 4. This was in the 60's and there wasn't a lot of information or treatment options then. I was supposed to 'outgrow' the deficiency, and to some extent I did, but it started causing problems again about 5 years ago. It took 4 years for the drs to figure out what was going on. I have brittle asthma and I trigger very badly off of respiratory infections so when I started getting serious infections every month things got a little ugly. I started on ivig in January 2004 and I am doing much better now. I still get infections that land me in hospital but its about once every 6 months instead of every 6 weeks. Big improvement. Its very positive that your son was diagnosed so early. As with many other conditions, an early diagnosis is a huge benefit.