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What Is Hypogammaglobulinemia?

Hypogammaglobulinemia is commonly treated with injections of gammaglobulin which has been extracted from donated blood.
Hypogammaglobulinemia is a disorder in which the body’s immune system does not make enough or any antibodies.
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  • Last Modified Date: 20 October 2014
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Hypogammaglobulinemia is a disorder in which the body’s immune system does not make antibodies, or makes a reduced amount. It has a variety of causes, but the most common is a primary immune deficiency, usually the result of a genetic mutation that prevents the immune system from making antibodies.

In a healthy immune system, cells called B lymphocytes make antibodies in response to bacterial and viral infection. Antibodies have a variety of roles, the most important of which is to destroy invading microorganisms. This is achieved through a process called opsonization, in which antibodies coat the microorganisms and make them vulnerable to destruction by other types of immune cells.

Someone with hypogammaglobulinemia, therefore, is highly susceptible to bacterial infections, and to a lesser extent, viral infections. Symptoms such as chronic bacterial or viral infection such as colds, flu, tonsillitis, and sinusitis are common. Chronic diarrhea, inflammatory bowel disease, weight loss, and fatigue may also result. Children with this immune deficiency often fail to thrive, and may be underweight for their age, and smaller than their peers.

Many different conditions may cause antibody deficiency. One of the most widespread is called common variable immunodeficiency, or CVID. This immune deficiency can be caused by a range of different genetic mutations, each of which results in antibody defects that increase susceptibility to infection. Another antibody deficiency disease, called X-linked agammaglobulinemia, is a genetic disorder which is inherited on the X chromosome.

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The most common treatment is regular injections of IgG immunoglobulin, also known as gammaglobulin. This is an antibody preparation that is extracted from donated blood. Gammaglobulin contains antibodies extracted from people who have been vaccinated against or exposed to common diseases, such as mumps, chickenpox, influenza, and bacterial infections. These antibodies provide what is known as passive immunity; it is passive because it has not been generated by the recipient’s own immune system.

This treatment is given every three to four weeks, for the remainder of the recipient’s life. It does not cure hypogammaglobulinemia, but reduces susceptibility to infection in people with the disorder. The recipient’s quality of life and lifespan are both markedly improved.

Gammaglobulin treatment can prevent many common infections, but not all can be avoided. People with antibody deficiencies will also generally take antibiotics in any case of bacterial infection, no matter how minor it may be. In the case of chronic infections, such as sinusitis or urinary infection, long-term treatment with antibiotics may be necessary.

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anon972128
Post 27

I have had hypogammaglobulinemia since 1991 and I was told If I did not take my these treatments I would not have lived to be 35. I took them for a while and started having problems with the gammaguard and quit taking it. Well, then I started getting repeated infections in my lungs and it had done so much damage to my lungs that I started taking it again to keep from destroying my lungs any further.

I take 35 mg IV every two weeks and have a doctor who premedicates me so there are no issues. I am on 3 liters of oxygen from the damage of not taking the treatment. So a word from the wise: make sure you take your treatments!

anon942904
Post 26

I have had hypogammaglobulinemia since I was a baby. It's been 20 years and I hate getting IV injections for six hours at a time every four weeks. Doesn't make me feel better afterwards. I usually feel terrible the next few days after getting treatment.

anon350836
Post 25

I was diagnosed with hypogammaglobulemia as a child. I was always sick. And they would always say I had allergies etc. Finally a very wise doctor figured it out. Anyhow, they told my mom I probably wouldn't live past 10 years. I started having gammaglobulin shots every Friday during the school year because I seemed to get sick mostly during school time, as a cold turned into bronchitis or pneumonia always.

I had shots at the doctor's office, the shot would come out of the fridge and bam! I was done for the week. I stopped having shots when I was a teenager in the 80's, because my doctor at the time was worried that I could get AIDS or something from the shots.

I still get sick mostly in the winter, and it almost always turns into bronchitis or something else. I usually am sick at least six to eight weeks. I don't usually start to get well until I convince my doctor to give me antibiotics, which they don't like to do nowadays.

I have a flu shot every year and I get a pneumonia shot every five years. I stay away from sick people, I wear gloves at the market, I wash my hands all the time, etc. I am now 46 and I have only had one winter since I stopped the shots at 16-17 years old, that I haven't been sick. One winter in 30 years, and that was two winters ago. But the following winter I made up for it because I was sick for almost three months.

Sorry this was so long, but everyone hang in there and there is hope and I guess a lot of advancement since those Friday shots all those years ago.

anon337084
Post 24

I was diagnosed with this a couple of months ago, and after seeing the results of the rest of my blood work, my neurologist is finally sending me to a hematologist. I have been making all sorts of excuses for why I am so lazy, but it is not laziness or depression or stress. It is this disease that I am just reading about.

I didn't think much of it until I found this web site. Now I am really fatigued. I have like, dozed off while driving and had fatigue. I don't have to apologize anymore - yay! That alone is helpful, and explains so much.

Question, though: Do any of you have a skin rash on your hands that comes and goes? After reading here, I am now assuming that it is an infection that just won't go away. Also, I have this awful pain in a couple of my fingers, and behind my knee. I have more pain elsewhere due to DDD, but this pain is the same in these three areas; like someone is twisting my nerves in my fingers and behind my knee. And, as one poster stated, my family is horrible to me. They know that I have been being tested for MS, am on social security disability from the DDD and Optic Neuritis, yet every time I don't show up to something or get aggravated that I know they think I am a hypochondriac, and I find it extremely disrespectful. I really just don't talk to any of them anymore. But now, maybe I should forward this site to them. Or maybe not.

Does anyone have any similar pain symptoms as I have? Also, I have been running a fever for several weeks - low grade - and they found a small enlarged lymph node on my neck last week. Could I actually get so sick as to cause lymphoma? Thank you!

anon322387
Post 21

I have a student whose husband has hypogammaglobulinemia and wants to know what foods would be appropriate for this disability. What can you share as to how I should approach this malady and help this family?

I am a registered nutritionist but have nothing in my medical and advanced clinical nutrition texts to address nutrition maintenance.

anon318289
Post 20

I am 39 and finally, after fighting this for years, I have been diagnosed with Hypogammaglobulinemia (CVID just today. I also have Mannose-binding protein deficiency. My doctor is telling me about the IV infusions that are done monthly or the one done weekly. He says we have to get approval from my insurance first. Does it hurt? Will it make me feel better?

anon299212
Post 19

I was diagnosed in 1983. Now I self infuse with subcuvia every week. It takes 2 1/2 hours but hey, life is for living. No worries. You rule your deficiency; don't let it rule you. Just stay positive minded.

I wasn't going to live past 18 months then, as I fought it, the doctors gave me to the age of 21. Ha. Were they wrong! I'm now 59 and have four kids and eight grandchildren and all are well. Live life to the full.

anon298519
Post 18

My problem is my family. I actually have given up on ever connecting with them as there are too many issues in the way. But they don't believe me or my apparent symptoms. My mother has narcissistic personality disorder, and if life doesn't revolve around her, she freaks. She has my father and three of my four brothers convinced I'm crazy. That's what narcissists do. If anything takes attention away from them, they connive, manipulate and lie to make you look like a bad person.

I am just headed to therapy to be helped with what she has done to me throughout my life, but I am disabled from other serious illnesses and mostly bed bound and live with her and my father. The stress of living with her has made all my illnesses worse. (look up daughters of narcissistic mothers online) I am moving out soon, but am so scared. I don't know how I am going to find an apartment, how to get someone to move my stuff, or how I am going to unpack. I have mobility issues, pain issues, balance issues, frequent falls so on and so on. How do I find the strength, where do I go for help, how do I find a future for myself?

anon288924
Post 17

I was finally diagnosed after suffering for more than 30 years. I get the IVIG infusion treatments every four weeks and have been doing this for over two years. I get them on Friday (at the outpatient infusion center for four or five hours), go home and stay in bed through Saturday and feel better on Sunday. While I am not 100 percent on Monday, I start bouncing back and feel good for the next four weeks. While it is not what I would like for my life, it works and has allowed me to have a life and a very busy and demanding career.

anon287207
Post 16

I have had blood infusions for six years. I feel tons better.

anon285277
Post 15

Can you get disability for this disease? I am finding I am off work more than I am working. I am waiting to see if my insurance covers the treatments but I am so tired and have a constant fever. Also, working in the medical field, I am exposed to everything! Is the social security process a difficult process for this disease?

anon284088
Post 14

My son started SubQ Ig and he gets it at home once a week. He doesn't need steroids or any other meds before or after the treatment. He's been on them for six weeks and is already healthier than he has been for 10 years. The treatment takes about an hour and a half and is a piece of cake compared to being sick all the time. He doesn't even mind it.

anon274257
Post 13

I have just been diagnosed as having the "hypo-goblin" disease as we have named it. It has certainly taken a toll on my body/finances (I have currently stood down as I work in a school) but due to waiting on other tests like a bone marrow biopsy and extra blood tests to find any other autoimmune diseases that may have caused this, my treatment won't begin for at least another four weeks.

I say, bring it! I am so glad I haven't been told my stomach infections are yet again "irritable bowel syndrome" or 'just in your head. You need to meditate."

anon271551
Post 12

I have been doing subcutaneous injections of Hizentra for over a year. I have experienced a year of almost no antibiotics or infections! It has been unreal. However, I am extremely fatigued most of the time. Is this normal for most of us with hypogammaglobulinemia?

anon263625
Post 11

I have three children: two boys a-gamma and one girl who is hypo-gamma. They all receive the four to five hour treatments every 28 days (four weeks).

My main problem is I can't seem to find info on the long-term for them. What kind of life span will they have and is it going to get worse with time? I know they used to put these children in those clean environments (bubbles) but thank god that's not what's done now, but my kids still stay sick monthly. For the most part, it's diarrhea, vomiting, viral infections, asthma and middle ear infections. The boys have ports but they did not diagnose my girl until she was five and she's always got breathing problems.

Are there other things I can do to boost their immune systems, like foods, certain supplements, etc.?

anon240200
Post 10

My husband has hypogammaglobulinemia for over five years. He used to sit at the infusion center for hours. Now he has found a doctor so he can administer the IVIG at home. It has worked out great. He is not exposed to all the other sick people at the doctors office or iv center. Once you start the therapy at home you will wish you had done it sooner. Good luck and god bless.

anon225203
Post 9

I have been receiving treatments since April and it has helped with my infection levels. I used to end up in the hospital every other month - no lie - since I started in April I have only been in the hospital twice. They had a hard time getting veins with me, so on my last round of home IV antibiotics I requested a Smart Port be installed. It is great! One stick and it is done.

It is under the skin on my chest and it can be accessed by the infusion center, hospital staff and labs for blood draws or medication administration. It can be left in indefinitely and it only took about an hour in the doctor's office to have it put in.

anon174271
Post 8

@anon156284: I've had the same problem, but presented my evidence of testing (ongoing) and records proving my illnesses, they (my professors)had an option to grade my work and testing despite my chronic absences. Each professor worked with me accordingly.

However, make sure you know your last day to drop the classes. The illnesses and fatigue may not allow you to perform your best on a full time schedule, but maybe drop a couple so that you can keep your focus and your grades up!

They don't even know what is wrong with me yet. My first blood tests show low IgE, low IgA, and low IgM. All else looks normal and skin allergy testing is negative while an immediate histamine response is positive. Any ideas would be much appreciated!

anon171497
Post 7

Their is another alternative that is not quite as intrusive. You can be trained to self administer a subcutaneous version. This means you insert small needles into yourself weekly and spend two hours at home hooked up to a small pump. Although this does not sound very appealing, the alternative is not attend my classes, or work full time.

I refuse to give up having a life so I prefer this route. If you suffer from a PIDD then I personally recommend discussing this with your doctor.

anon160507
Post 6

I have treatment very three weeks. It starts off at six hours but over a few months reduces as your body gets used to the infusion. I now travel an hour into the city, get hooked up to a machine for 1hour 40 minutes, then travel home again.

I was ill for 32 years (from 4 years old), every day I heard myself saying, "i don't feel well". I won't go into all the problems.

The illness and the treatment is life long and depending on the level of illness you will be put on the infusion. You can opt out but I recommend taking it. I still feel tiered but I have not been ill for months, it feels great.

I am now fighting for disability payments, they don't recognise the illness. It is really hard.

anon159688
Post 5

elizabeth 2: It's not an injection. It's 4-5 hours hooked up to an IV with the chance of adverse reactions. To prevent reactions they give you steroids and benadryl. Oh boy. Doesn't that sound like fun. Yippie

anon156284
Post 4

I wish someone would explain this to my school. They say they don't discriminate against anyone with a physical disability.

But they will not accommodate the idea that I have Hypogammaglobulinemia and cannot control the fact that I get sick a lot and can't fight of surface bacteria. They fail me in my college courses when I miss four classes. I have F's on my transcripts and I have to chance it every semester paying for the class and trying my best to not miss too many. How does one fight a University on this?

elizabeth2
Post 3

Any problem with the immune system can be very dangerous. While the hypogammaglobulinemia treatment doesn't sound desirable -- I sure wouldn't want to have to get injections every three to four weeks for the rest of my life -- it is a lot better than suffering from the disease.

We owe a lot to the amazing people who discover treatments for all of the horrible diseases out there!

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