Hypogammaglobulinemia - Primary Immune Deficiency

by Amy
(Anaheim, CA)

I was diagnosed many years ago with Hypogammaglobulinemia.


It is a rare, genetic immune deficiency. My mother, my brother, and my son also have it. My antibody IgG levels are the lowest of all of us, but I am the last working adult in the household. They are on permanent disability.

I receive IVIG infusions once a month, but I can't stay well. In the last 6 months I've had appendicitis (appendectomy), an abscess and infection as a result of surgery, full blown influenza, (and just in the last month and a half) a cold turned into bronchitis which became pneumonia, and I currently now have strep throat.

During this time I also got a yeast infection from the antibiotics...the over the counter medication was failing me and I was desperate! I looked up remedies and found a specified vitamin list of what to take and not to take..I was amazed!! It was gone in less than 24 hours.

I currently take lots of vitamin c (6000 mg) and a multi-vitamin. I find myself desperate once again for a solution. I have worked too hard and come so far to be this chronically sick.

I love my job as a chef and don't want to give up my dream. I just can't go on like this. If this keeps up I'm going to end up on disability or I could even die...it's getting that serious!

The doctors are at a loss of what more they can do. I have to take my health into my own hands and start doing some research.

Is there some aggressive vitamin regiment that you recommend for people who suffer from chronic immune deficiency? Any info would be a big help.

Also, please post this to raise awareness for this disease. I believe it is more common then they say...most dr's don't even know how to write the lab slip to test for it.

Also, I want to encourage others to donate blood! Without my monthly blood infusions I would have been dead years ago! Thanx for taking the time to help others! I wish there was more people in the world like u!! -Amy


RESPONSE


Hi Amy,

Thank you very much for sharing your story and raising awareness of this particular immune system disorder.

Let me explain briefly what hypogammaglobulinemia is so others may understand what you are going through.

People with hypogammaglobulinemia (pronounced hypo-gam-ma-glob-u-li-ne-mia) produce very low levels of antibodies, which makes them highly susceptible to common bacterial and viral infections. A minor infection that would be a simple annoyance to those with normal immune function could prove deadly to someone with this disorder.

I am more familiar with this condition being called common variable immunodeficiency (or CVID).

People with CVID have defective adaptive immunity, which is also known as the acquired immune response. This immune response is specific to the particular pathogen that is encountered and depends on specific white blood cells called lymphocytes.

There are two main types of lymphocytes – T cells and B cells. T cells recognize pathogens that have infected our cells. B cells recognize pathogens that are outside our cells. People with hypogammaglobulinemia either have defective B cells, or have defective T cells that do not properly alert B cells. Much more research needs to be done to fully understand this disorder.

What we do know is that people that suffer with this have B cells that simply cannot make enough antibody. Low levels of antibodies means that the body cannot properly clear bacterial and viral infections.

Typical treatment involves regular injections of antibodies that have been extracted from the blood of immune sufficient donors. Without this life-saving treatment, bacteria or viruses would grow out of control.

As for recommendations on staying well with this kind of condition – I would look to make the most out of what you do have. I'm talking about improving your innate immunity, since your adaptive immunity is what is flawed.

Having a strong innate response will cause you to rely less on your adaptive immunity (actually, your blood transfusions). Innate immunity is your first line of defense against infections. It isn't as targeted as the adaptive response, but it does mount different responses against viruses and bacteria.

Strong innate immunity can clear infections without help from lymphocytes.

Boosting your innate response can be achieved by taking a high enough vitamin C dosage and maintaining proper levels of vitamin D. Good nutrition also plays a part in both immune responses.

Also check your body pH, as immune cells have trouble functioning optimally in an acidic environment.

Hopefully this has given you some information that you can use to move forward.

Wishing you the best of health,

Melissa
Approach Wellness


Comments for Hypogammaglobulinemia - Primary Immune Deficiency

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Something different? ?
by: Dawn Rogerson

I was diagnosed years ago and my first treatment resulted in my feeling deathly ill like you. I immediately contacted the hospital I was treated at, as well as my immunologist. Both said that what I was experiencing was not related to that first infusion. Boy, were they all wrong! !
When I went to receive my second infusion, I mentioned what had happened, and I was a bit confused when she said that I was supposed to receive some simple over the counter medication first and foremost in advance of your treatment ! She went on to say that without these meds (Tylenol and Benadryl ) , one would suffer like we have.
What a difference in how I felt. So, if you are not being given 2 Tylenol with 1 Benadryl, please make sure to talk to either the hospital staff or your doctor.
I truly hope that this helps.
Praying for Cure!!
by: Anonymous

I was Diagnosed 6 years ago in my mid 20's after years of Constant Infection and Viruses!! I get monthly IVIG treatments. I can probably count on one hand in 6 years the number of times I did not get side effects! I would try to think what did I do different; rest more, not as stressed, more hydrated, etc. I think I just got Lucky! It's hard losing a week out of a month to feel like heck! I still get infections but not as many!! I guess I'll take the side effects to get a sinus infection or cold once a month than be sick Every Day of My Life!! I look back and think "how did I make it?" Before getting diagnosed and treated!! I was the walking dead!! I pray every day that it goes away as quickly as it came! I pray for a cure even though I am Soooo Grateful for the treatment! I try to stay Positive by counting my Blessing and thinking how worse it could be... But I'll Never get over "Why Me" until they can tell me!
Concerned Laura
by: Anonymous

I am constantly cutting things out to see what helps or hurts. Gastrointestinal issues are the number 1 symptom of immune deficiency according to my immunologist. I have always had digestive issues as well as other family members with this disease, and I've learned that everyone is different. My brother and (also immune deficient) says gluten upsets his system (probably a gluten allergy) and he does better without it. I find sticking to a vegan and low cholesterol diet helps me as well as my mother. But, I must add, that even though we are eating digestive friendly foods we still have issues. It just comes with the territory. You can try cutting one thing out at a time and see if it helps. But overall I would just focus on healthy, clean eating for both you and your son. Lots of veggies, fruit, and whole grains. Avoid processed and refined foods. I have learned that it takes more energy for the body to process meats and cheese, and if I eat those I am tired for days. Hope that helps. And prayers to your sweet angel!! My son has it and my daughter just got diagnosed as well, so I know how hard this is for you. You are doing great!
Concerned mom
by: Laura

My 1 year old son just got diagnosed with Hypogammaglobulinemia. He had a mediport put in place and receives infusions. He also takes medication for gastroesophigial reflux. My question I guess is I have read here or there that diet can help improve his over all health with his condition. By that I mean eliminating gluten and dairy. I myself am a celiac. They have done blood work and it came back negative for celiac but at his age he's not really eating a ton of gluten filled foods anyway. Due to his reflux he still eats a lot of jarred baby food because the consistency is easier for him to tolerate. Has anyone had similar issues? I believe he is gluten intolerant and when he does eat gluten it bothers his tummy more than if I were to keep it away from him. But I don't want him to have to have that life if I'm just being over protective.
Been there, still there
by: Kat

I was diagnosed back in 2001. I had my first IVIG October first that year. No one warned me what was in for just bring something to do. I was the first patient in and the last patient out. I went home and slept the rest of that day. The next morning I had the worst migraine in my life. Had to stay the whole day in my room, total darkness, total silence, no smells etc. My husband had to take care of me as well as the kids. The next day was only slightly better...I could function with in the house but that was it. By the third day there were no groceries in the house so I had to go shopping. My husband and kids came along and we would send the kids to find items (two older were elementary age) and my youngest would find things for me in the isle we were in. The walking around was good for me but I didn't have much energy so I had to keep it simple. The next day was church and I was still weak but went anyway. I gradually got back to my routine and feeling well. Then my next IVIG and it all started again. After a year of this I wanted to just stop.loosing a full week out of four was the pits and I did not like it at all. The doctor told me without them I would get more and more sick each time and eventually die. So I had to work within my self and make loosing a week part of my routine. I dreaded the treatments, I had a bad attitude about what I was dealing with which only made things worse for me. I am not sure when I changed or how I changed but I now explain it as "this is my life such as it is." Part of what caused my poor attitude was trying to explain my condition and why I needed a week to recover from a treatment that was supposed to make me better. But at some point my brain flipped or something because I found myself embraceing my life such as it was. Then one day the nurse who hooked me up did it different than everyone else. Some how the saline dripped during the treatment. I felt great. Went to dinner with the family had a great evening got up the next morning just as if it had not been treatment week. Next time I went I told them to do that a again and was told it was not protocol and the previous way was repeated. Yes I was down and out like before. So I told my Dr to make it protocol. He did and I do pretty good. Yes there are still treatment weeks I come home and crawl into bed but the next day I am hood to go. I take vitamin D supplements and fish oil but it is the high quality refrigerated probiotics that help me push back yest infections I get when I do have to take antibiotics. Something I observed recently was I had gotten bitten by a cat on my hand that immediately infected (4 hours or so after the bight I could see the redness growing) we traced the redness with a black sharpie pen and called the doctor. I then hit my system hard with the probiotics and by morning the redness had already began to receded. So keeping the probiotics going apparently helps my system fight off infections too. Not stopping the IVIG because I don't think probiotics alone could fight off everything. Advice in life changes. First attitude about your diagnosis. You are not sick you are not a victim it just is what it is make the best of what you have. Wipe down surfaces constantly. Shopping cart handles, computer keyboards and mice, pens pencils. Outsides of purses, handles too, and diaper bags, arms of chairs, door knobs, door frames desk tops, counter tops. If you might touch it wipe it off first. Wash your hands, for real,constantly. Wash your hands looks like warm water rinse, add soap, scrub for two or more seconds getting between fingers too...then let the water rinse them off no less than 15 seconds. Wash your hands before eating and minimize touching your food. Finger foods try to use a fork instead... Yes eating French fries or a piece of bread is quite difficult and awkward at first but you get used to it. My husband love the all you can eat places....I see germ factory. So when we go I get my food, then I go wash my hands then eat. I do use hand sanitizers but I don't trust them much so when water is available I wash with water first choice. Hang in there. So far there is no cure for this, but with resolve and attitude you can make the best of this and not let it shut you down. Welcome to your new life, buckle you you are in for one huge ride.
Will it get better? Can you work? Response.
by: Amy

IGG infusions will help your body to fight off some infections. It doesn't work like a normal immune system though. You will still frequently get ill, the infusions alone can make you feel ill. IGG has improved my quality of life substantially, but I still have to be so careful with germs, rest, stress, allergies, and especially exposure to public places. Everyone reacts differently to IGG therapy...some run marathons, work actively, and can function normally....others (like me) can't go in public (without mask, gloves, Clorox wipes), still get infections, still get hospitalized, develope autoimmune issues, and end up on disability. Social security disability is finally starting to recognize CVID and PIDD as disabilities. I did everything in my power to avoid disability, but it became necessary. There is no cure for this disease. IGG therapy is the only treatment.
hypogammaglobulinemia
by: Anonymous

hi i have just been told that the reason for my recurrent chest infections is because of this immune disorder...will it ever get better with thr transfusions??? and will i be able to get disability payments or will i be able to keep working?? i am confused and i have not as yet started the treatments..thanks
Raising kids with PID or CVID
by: Amy

Hypogammaglobulinemia, CVID, or PID affects different people in different ways. I know some that just get IGG infusions and are fine and active!! Some children grow out of it. Some kids you have to be careful with like setting limits, but otherwise they can participate in school and activities. Then there are some that will be very fragile. Be careful with live vaccinations!!! Ask your pediatrician for the non-live vaccines!! I recommend joining a support group on Facebook...tons of information and zebra love out there on social media! Best of luck and prayers for the kids.
hypogammaglobulinemia
by: Anonymous

My son who is only two was diagnosed about a year ago. He takes weekly infusions but he also takes an antibiotic every other day even when he is not sick. This has helped tremendously in bringing down the number of infections. I also give him green powder supplements and drops of vitamin D in his juice. With all of the above he seems to be fairing pretty well. He almost died twice but now we have been at least a month with no infections!! Good luck to all of you! !
Gillian and everyone who has this disease
by: Amy

Just to let you all know you can go on the IDF website and request information. They have sent me a ton of literature and a quarterly magazine absolutely free!! It's been a great tool in educating myself and dr's! Also I have found loads of support on Facebook support groups for CVID!! Lots of answers out there as our community grows!! Zebra strong!!!!!!!
i need help
by: dennis

i have a 9 month old daughter who has hypogammaglobulinemia. will my daughter be able to go to school when its time and what all am i in for i had to quit work to take care of her im a single parent
Response
by: Amy

Don't be frightened! I've learned dealing with this disease that I had to make some life changes and change my outlook. I was on IVIG and now I currently am doing IGG subcutaneously. I recommend that you google "the spoon theory"! It helped me a lot! In the last few months I've had to go on disability and weigh the quality of life vs. the chef job that I love. I chose to let myself heal and listen to my body. I am an extreme case. The IGG will make you stronger, but doesn't work like a regular healthy immune system. You will still be susceptible to infections but the Igg gives you a better chance at fighting it off. After your first IVIG write down all the side effects, so the dr can properly pre-medicate. They use to give me a steroid shot or Benedryl shot before each infusion. Although, a rare disease you are not alone. Take care of yourself and get to an immunologist ASAP...also important to have a great PCP that you trust! God bless and keep commenting if you need more answers
Newly diagnosed, and a bit scared
by: Gillian

Well hello all. I am in the early stages of Hypogamaglobulinea. I have had 2 tests of my blood, and the latest shows the following; IgG 532, IgA 55. The research I have seen, states its Hypogamaglobulinea.
As I am in the early stages, have not seen a Immunologist, Or Hematologist. Reading the complications, and treatments themselves is quite frightening.
Should I be frightened? The IVIG procedure I am really not looking forward to.
Thank you
ps, Is there anything any of you can help me with, in regards to this?
Hypogam.
by: Anonymous

Hi there,
I was diagnosed with Hypogammaglobulinemia when I was in the 8th grade. I couldn't go to school at all, I was sick 24/7 (2 bouts of tuberculosis and many cases of pneumonia). My immunologist put me on immunotherapy via a port they implanted in my chest. Right now I am currently a college freshman, and running track for my school and my country. This disease really is limiting to people because of the symptoms.

Best of luck to you all!
HypoGammaGlobulinAnemia
by: Steve

Hi:
I was diagnosed with this condition several years ago. I am now 46. I went through a lifetime of infections, constantly on antibiotics and finally losing tonsils and appendix. Without the right treatment, you will never really feel well. Changing your diet and vitamins will not help overall. The problem is your immune system and how it does not work. Generally, your B-Lymphocytes are not producing IGg's. If you are having hight levels of bacterial infections, this is the cause.

You should be seeing an immunologist, who will place you on a IV treatment of IG's every 3-4 weeks. If you have alergies as well, you may be placed on a treatment of Privigen, which is a purified IGg treatment. Otherwise, you will need Benadryl or steroids before having each treatment. The problem with IVIGg is that the treatments are far apart and you will probably go through a roller-coaster effect on your immune system (highs and lows). I was on these treatments for some time but now use a SQ Igg, subcutaneous infusion into the fatty cells of your body. You do this yourself, it is rather painless, and you will get used to it. There are a couple of products out there such as Vivaglobin and Hizentra. In my opinion, Hizentra is better because it can be stored at room temperature and you can travel without having to refrigerate. The infusion is done with a 22 gauge (can be changed) butterfly needle and a syringe. The "push" method is used, meaning that you deliver at the rate that you are comfortable with. I have to infuse four times a week, three times a week with 20ml and once with 5ml. It takes anywhere from 5 - 20 minutes an infusion.

You should investigate further and not rely on diets and vitamins to fix you because they won't! Seek treatment from an immunologist. I will be happy to answer anymore of your questions if you reply here.

Steve

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