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Ig G1 Deficiency

Syd_Mum
0 Recommendations

Hi, I was wondering if anyone else has been diagnosed with Primary congenital Ig G1 specific deficiency. My daughter and I were diagnosed with this about 18 months ago.

We have had a really hard time getting useable information in regards to this. Most information seems to cover G2, G3 and G4. Mostly, it just says G1 is very rare. I would love to hear from anyone who has been dianosed with this, or anyone that would have much information in regards to this.

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Diphtheria Tylenol Meningitis Falls Penicillin Tetanus Pneumonia Acne Probiotics Prednisone Benadryl Naproxen Stress Cefzil Asthma Arthritis PFAPA Creutzfeldt-Jakob disease Allergies Physical therapy Fever Rubella Constipation Rashes Job syndrome Depression Acetaminophen Diarrhea

40 replies

SickOfBeingSick

Tamyra,

Thank you so much for the referral. I will contact this doctor and see if he can get me the name of someone in my area.

I talked to Baxter the other day and they told me that with Medicare I couldn't go to the outpatient infusion center I had been to before, I could only go to a hospital. They were much more knowledgeable, willing to help, and useful than the IDF.

This could work in my favor because a hospital wouldn't be as likely to turn me down simply because I'm on Medicare and whatever immunologist I see would only be worried about payment for their office visit, not for the infusion since all they would be doing would be writing the prescription for the infusion.

I agree with you about the IDF. In the US they seem to be nothing more than a political action group in bed with the pharmaceutical manufacturers of IVIG. They completely refused to help me and had a horrible attitude towards me before I started IVIG.

This last time that I called them, they again asked about my history of receiving IVIG and as soon as they found out I had been receiving IVIG their attitude was completely changed from when I had called them before and they were very sympathetic. However, they weren't able to do anything productive for me.

It's a shame, because their sister organization in the UK, the PIA, seems to be truly patient-oriented and real patient advocates, I hear accounts all the time of how much they have helped people.

The values of the IDF go right along with the values of US society. The almighty dollar rules and Doctors, Insurance Companies and Pharmaceutical Companies are gods above reproach. It shouldn't say "In God we Trust" on our currency, it should say "In the Almighty $$$ we Trust". I want out of the US, now!

Cathy

Tamyra

Cathy,

I am sorry to hear you are having problems. If you contact Zuhair K Ballas, M.D. Allergy/Immunology University of Iowa Hospitals and Clinics, he is on the Internet. I believe he can help you, if you can't get to the hospital he will give you a name of a doctor closer to you. I sent him an email, scanned my last consult with blood work results, He called me, I went to see him, I now have a doctor 20 minutes from me home.

Most doctors do not know enough about IgG to stand up to insurance companies.

I hope you find help. Yes, IDF is no help at all, if you think your bank balance is to high, you can send them you money. LOL

SickOfBeingSick

Hi,

Haven't tried the Jeffrey Modell Foundation. Just really, really tired of calling agency after agency, getting no help and banging my head against a brick wall.

Just looked at my last test results and I was low on total IgG, IgG1 and IgG4. I have been low on the other subclasses in the past (IgG2 and IgG3 at different times). I think I don't produce any IgG4 (it is almost zero every time without fail), and produce suboptimal amounts of the other subclasses, so different subclasses dip below what the call the low limit at different times.

Cathy

Syd_Mum

Cathy What about the Jeffrey Modell Foundation, they state that one of their goals is to "To serve as a tireless, compassionate advocate on behalf of patients and families to assure their access to excellent and comprehensive care".

They might be able to assist?

TwoToeheads hi,

I had a look at the website its interesting. It never ceases to amaze me what is out there. The challenges ones have to overcome.

SickOfBeingSick

Hi two toeheads,

I have heard of Hyper-IgD before but not PFAPA. Hyper-IgD falls under the broad umbrella of Primary Immune Deficiencies which includes about 150 different disorders.

Yes, the effects are very similar to IgG deficiency except that hyper-IgD can't be treated with IVIG, unfortunately :( Hyper-IgE, also known as "Job's syndrome" (the "E" is not a typo, IgE is yet another immunoglobulin) is another Primary Immune Deficiency that causes chronic infections. Funny how both having too little and having too much of an immunoglobulin can both cause fevers and chronic infections.

Welcome, and I hope you receive the support and information you need here.

Cathy

SickOfBeingSick

Hi Syd_Mum,

Prion diseases are things like mad cow disease and Creutzfeldt-Jakob disease. They are very rare.

I don't think the insurance companies will change without political action. Unfortunately, they are a business and only care about saving money. As long as they can get away with it, denying care will be their modus operandi.

The big difference between the US where medicine is a business and countries that have socialized medicine is that in the US the goal of insurance companies is to make as much profit as possible. With national health systems, the goal is to spend money on health and medical care in as thrifty a manner as possible.

This subtle difference in philosophy creates a huge difference in the delivery of healthcare. Countries with socialized medicine care about the health outcomes of their people because healthy people will utilize the nation health care system less. In the US, they could care less about things like quality of life and quality of healthcare. All they care about is making a buck, and they will do that no matter how unethical and immoral their methods, even resorting to outright fraud because they can get away with it due to lack of government oversight.

I have contacted the IDF in the US and they haven't done anything to help me except give me a referral to a new doctor, whom I haven't been able to get in to see.

I recently lost my insurance coverage and now have only Medicare. The IDF couldn't even get the Medicare guidelines for receiving IVIG for me, so I could fight my doctor's decision not to give me IVIG because Medicare POSSIBLY might not reimburse them. Medicare won't give me the criteria because I'm not a doctor (which should be illegal, as a patient I have a right to that information). So, I have no way to rebut her decision if I don't even know what Medicare's criteria is.

The last time my levels were taken in October I had low total IgG and I think low IgG1, maybe one other subclass, I don't remember now. I am currently pretty much bedridden and have constant infections. But, I will fight this in the political arena and hold my demonstration against the state of healthcare and social services in this country, even if it kills me doing so.

Cathy

TwoToeheads

I'm new to inspire because I am looking into HIDS and PFAPA which are recurring fever diseases. HIDS is a very rare syndrome related to Hyper-IgD. There is a website containing info www.hids.net. Some of the symptoms described on this discussion board are posted as symptoms of HIDS. Just thought you might want to check it out.

Syd_Mum

Cathy hi,

What are prion diseases?

And yes your right things do need to change. I know I have heard that they are spending a lot of money in america educating doctors and the public about primary immune disorders. Hopefuly this will have some benefits for you via the insurance companies as it becomes more known and ones are educated about it.

I wonder if one of the primary immune organisations might be prepared to step in and take up the fight with them? It would be good publicity for them in getting the information out there, and also the benefits could then flow down to you and others.

Syd_Mum

Blue jeans hi,

I dont know much about cvids or the others. I know the G1 deficiency we were born with, so it stays the same from birth till death. It can never be cured or go away. Its passed on through the mothers line. You are either born with it or not.

Its a scary thought that ones can develop it...I imagine too, in a way worse. If your born with it, you dont know any different. We kind of ingore most of the symptoms as we have had them forever. Its nothing for me to have fairly constant fevers and white blood cell count up, with no obvious problem. Just another infection somewhere :) Not that you get used to it but you just deal with it as its what we are used to. Its only when it gets really bad we go to the doctors, most of the time we self medicate. But to have been really healthy and then get it, would be horrid.

With the Ivig, do you like music? Maybe an ipod would help, or one of those small dvd players. It would be frustrating, reading is one of my fav ways to spend time if confined too.

foreverinbluejeans

Primary immune deficiencies can have onset at any age. I didn't become ill with CVID until I was 32. I got a haemophilus lung infection from my stepson, he got it from day care. My 8 year old and 5 year old also got the infection and were diagnosed with CVID at the same time and started IVIG.

If my children wouldn't have gotten that infection, they may not have gotten CVID until they were adults. There are certain bacteria that can bring on or make immune deficiencies much worse. The bacteria aren't rare.

Your body doesn't reject IVIG. If you take Tylenol and Benedryl before the infusion it will help. It's normal to feel worse a couple of days before the IV and a couple of days after. Running the IV slow will decrease side effects.

You don't want to put your children and grandchildren through the life-threatening infections you can get. My youngest son is afraid that while he is at work I could get an infection or blood clot and die before he gets home. I get very sick, very quick.

I'm doing everything everything I can to stay healthy and alive. I endure the IVIG every 3 weeks. My current Medicaid won't let me do it at home and I have to go to an infusion suite 45 minutes away. They don't have a TV and it takes almost 12 hours. I can't read, it gives me headaches. I can't eat much. I itch the whole time. It sucks to have immune deficiencies.

I have an IV port and the nurses have trouble accessing it. I did my IVs myself for 15 years and now I have to let nurses stick me multiple times. It's worse in the hospital. They try to start IVs in my arms even though I tell them I don't have veins that will hold up for IVs, they don't listen. I used to be a Medical Lab Technologist and worked as a phlebotomist while I was in college.

SickOfBeingSick

Hi,

You should be able to look for information on hypogammaglobulinemia and descriptions will be pretty much applicable to your IgG1 deficiency. Hypogammaglobulinemia is the general term for any of the Ig subclass deficiencies. The only difference, as noted by ForeverInBlueJeans, is that the different subclasses fight different things, like viruses vs. bacteria.

Decontamination of IVIG is very thorough and about the only concern nowadays are prion diseases. Pretty miniscule risk compared to catching pneumonia or meningitis or some other nasty from not receiving treatment.

Your lack of response to vaccines is diagnostic for this disorder. Vaccinating and checking response to vaccinations are used to diagnose Primary Immune Deficiencies, in combination with checking immunoglobulin levels.

I tried to appeal my insurance company's decision to the state agency that oversees insurance companies and was told they fall under ERISA which in the United States means that they can pretty much do whatever they want with no recourse for those covered by these policies. This needs to change!

And yes, IVIG is much easier to obtain in Australia, Canada, the UK, the Netherlands, Cuba and almost anywhere other than the US and very poor third-world countries. Insurance companies here typically cover a few months to a year and then proclaim you "cured" from this GENETIC disorder because your health has improved and your immunglobulin levels have gone up (duh, that's the whole point of treatment and CONTINUED, REGULAR infusions to keep levels up).

Cathy

Syd_Mum

Cathy that is really sad about you not being able to recieve them. I am pretty sure our system out here is a little different. There seems to be more control over there about who your allowed to see and who you arent.

We had no problem getting the gamma globulin, but in saying that I spoke with a lady that had been trying to get it for her child and was unable to.

Is there someone you could contact, an authority on this that might speak up on your behalf?

Syd_Mum

hi Blue Jeans.

Thank you for sharing.

I have read those notes online re Selective IgG1 subclass deficiency being 60 - 70% and very rare. Thats my where my frustration comes from as I cant seem to find anything else on that. :) Both myself and daughter have Selective IgG1 subclass deficiency. I am the sort of person that likes to read and be really informed, and its hard when I cant seem to find much specific information on it other than its very rare.

I have never tried the Ivig only the gamma globulin injections. The chance of disease from this was another reason I was not happy having it, aside from how my body reacted to it and how it made me feel.

I honestly dont know if I am doing the right thing in not having this or not, as in not having any at this point in time. I am 46 years old, so reading your post I am doing pretty well. Hopefully I am doing something right. I have 3 children and 2 grandchildren. So far only my daughter has the same condition. I was told it could not be passed down through males, only females. Males can get it but do not pass it on to their children.

In australia most the shopping centres have air conditioning and yes legionaires can be passed that way. But also normal infections too as they hold large groups of people. We do have shopping areas the same as your strip malls and no air conditioning there. So I tend to use those if I have to.

Same as working in offices, how colds, flu's etc pass around the offices through air conditioners.

Normal household airconditioners are fine as far as I know, with the exception of the water cooler evaporative types, as these commonly bread bacteria if not maintained perfectly.

My hope is, as new information is discovered it will be passed on to us.

I actually am curious about a couple of things, primarily immunisations etc. I have had 4 rubella injections through my life, each time, one month after receiving it all antibodies to it were not present. It was as if I never received it. I had the same happen with desensitization injections years ago. In being tested its as if my body has never had them. I am guessing this has something to do with the condition, but am not sure.

If true though I am wondering if there is any point to pneumonia or flu vacines if they work in the same way. I have never had one as yet.

foreverinbluejeans

This is some info I found that may help -

"While all the IgG subclasses contain antibodies, each subclass serves somewhat different functions in protecting the body against infection. For example, the IgG1 and IgG3 subclasses are rich in antibodies against proteins such as the toxins produced by the diphtheria and tetanus bacteria, as well as antibodies against viral proteins.

In contrast, antibodies against the polysaccharide (complex sugar) coating (capsule) of certain disease-producing bacteria (e.g. the pneumococcus and Haemophilus influenzae) are predominantly of the IgG2 type. An inability to produce antibodies of a specific subclass may render the individual susceptible to certain kinds of infections but not others.

The IgG circulating in the bloodstream is 60-70% IgG1, 20-30% IgG2, 5-8% IgG3 and 1-3% IgG4.

In general, the infections suffered by patients with selective IgG subclass deficiencies are not as severe as those suffered by patients who have combined deficiencies of IgG, IgA and IgM (for example common variable immunodeficiency). Very rarely, IgG subclass deficient patients have suffered recurrent episodes of meningitis or bacterial infections of the bloodstream (e.g. sepsis).

Selective IgG1 subclass deficiency is very rare. IgG2 subclass deficiency is the most frequent subclass deficiency in children, while IgG3 subclass deficiency is the most common deficiency seen in adults. IgG4 deficiency most often occurs in association with IgG2 deficiency. "

I have CVID and very low levels of IgG subclass 2 and 4. I've been getting IVIG for 21 years except when I couldn't get it because of insurance issues. Getting IVIG doesn't make you feel better, it keeps you alive so you can see you child graduate from grade school or high school or if you are lucky, college.

Having an IgG subclass 1 is a life-threatening condition. Since viral infections are a problem there may not be treatment like there are for bacterial infections. It isn't responsible to refuse IVIG if recommended by your doctors if you are a mother, the benefits outweigh the risks.

I am allergic to 6 different groups of antibiotics and there is only one group of oral antibiotics left that I can take for sinus or respiratory infections. Antibiotic allergy is another important reason to get IVIG. It's better to avoid the infection.

There are some things to worry about with long-term IVIG. There is concern about IVIG and the large number of other drugs people with PIDs often have to take and liver damage. Some doctors recomment blood liver tests every 6 months. Kidney damage has been reported and seems to be linked to speed of transfusion. I get my transfusion very slowly - over about 10 hours. There may be exposure to disease from the product, including disease we don't know about yet.

I'm not sure why you were told to stay away from air conditioning. The usual warnings are crowds, places where sick people are, church, movies, & children. It's good to avoid thermostress, high or low temps. So, air conditioning as far as I know is good.

The only thing I can think of is the rare case of the large building air conditioners that are linked to the very rare outbreaks of Legionnaires disease. As rare as it is, I was exposed to this twice. I was at IU as a student before I knew I had CVID. The second time I was hospitalized with pneumonia at Methodist Hospital in Indianapolis and could see the offending AC system from my room. It was a couple of months before I was diagnosed. I didn't get Legionnaires either time.

SickOfBeingSick

I received IM injections back in 1980. My doctors won't even consider giving this treatment anymore because treatment with IVIG or sub-Q has replaced it. A lot less painful, but MUCH more expensive and harder to get approved. I would rather at least get the shots that the insurance company doesn't scrutinize as they do IVIG so I could at least get SOME treatment. I am currently being denied treatment.

I feel so much better when I am on this treatment, it is like I have a new lease on life, like someone flipped a switch. It is really a miracle treatment for me and makes me really angry that an insurance doctor who isn't even an immunologist, much less an immunologist specializing in immune system disorders is denying my care when I meet the insurance company's WRITTEN criteria for treatment. It's fraud.

As for the canned and jarred fruit - I think it's the canning process that kills bacteria and breaks down some of the fiber and acids making the fruit easier to digest. I try to stay away from the more acidic fruits in cans, though, because they tend to leach metal into the contents of the can. You can taste it. So I try to get these fruits in plastic or glass jars, instead.

Syd_Mum

Cathy hi,

When I had the gamma globulin it was via injections, very painful ones iwth a large guage needle :( lucky needles dont phase me anymore. It was given intramuscularly. I never received any other medication with it, just the needles. Which because of the amount had to be split over 2 needles.

We attended the professors office again and they are starting daughter on prophylactic antibiotics so shall see how that goes too.

I know eventually we may have to go back to the gamma globulin but I am trying to hold off and see if we can find a better way for us.

With the fruit in cans, I had not thought of this till recently, but you are right. I actually wondered if it was something to do with having it too fresh... maybe enzymes that are present etc? Only a thought. We noticed recently that daughter can eat corn, but not if its straight out of her grandfathers vege garden. He uses no pesticides, but it has started me noticing that she can eat tinned fruit such as peaches but not the fresh ones.

I will look for the ultra-high pasteurized milk, as I am not sure if I have seen it here.

And yes thank you! Any advice like that is good and can help others.

SickOfBeingSick

Hi,

I have low Ig (different ones at different times). You are right. This is a genetic disease and if your body can't produce appropriate levels of gamma globulins because of a genetic defect, no supplement or herb or diet is going to help that. They might help by improving your health in general, but they can not substitute for IVIG and replacing what is not there and what the body can't produce!!

Two months is way too short of a trial for IVIG. It typically takes much longer than 2 months to feel good, often even up to a year or two. You didn't get to this run-down state overnight, and it takes time to rebuild immunity.

There are many things that can affect your reaction to IVIG. Brand has a BIG effect. Different brands work for different individuals. There are people that have horrible reactions to one brand and almost no reaction to another brand. Mild headaches and flu-like symptoms for a day or two post-infusion is common, but people on IVIG almost universally agree that the temporary discomfort is well worth the improvement in energy and decrease in infections and auto-immune problems. When your immune system kicks in and finally starts fighting the bugs that are there, you will have temporary mild fevers, aches and pains, etc.

The reactions can be reduced by using a very slow infusion rate, switching brand, infusing sub-Q instead of IVIG, drinking LOTS of water (IVIG makes your blood thicker temporarily), taking acetaminophen or ibuprophen, taking benadryl (very important) and if necessary, a day or two of steroids at infusion time. You should have received IV benadryl along with your infusions. If you didn't, you need to INSIST on this, it is the accepted, most common treatment protocol for reducing side effects. Also ask what infusion rate is being used. Infusing too quickly is a very common medical mistake that can cause severe side effects. Many people keep a record of their infusion rates.

I highly recommend that you check out the site www.pia.org.uk , especially their discussion forum. You will find a lot of very knowledgeable, supportive people there and can get a lot of your questions answered.

About your daughter's problems with fruit - I have a lot of GI problems. It is typical of this disorder. I find that I can tolerate fruit in cans and jars a lot better than raw fruit. I know, it's not as healthy. But it's better than not eating any fruit at all. I also can't drink regular milk. The small amounts of bacteria left after pasteurization that don't affect most people, leave me with nausea and diarrhea. I have to drink ultra-high pasteurized milk that comes in the unrefrigerated cartons in the drink aisles of grocery stores.

Hope some of this information helps.

Cathy

Syd_Mum

Just a note for ones who are wondering. Air conditioners such as ones used in large shopping centres and offices spread infection very easily. So best to avoid to avoid infections.

The desensitization injections are for allergies. You may find that your daughter is allergic to many things. We had allergy tests run by the professor and found this out to be true with us. The injections, initially weekly and later monthly, stop the immune system overreacting for the allergies. We found that she had massive sinus problems, which were then going secondary and causing chest infections. As you know when they get those types of infections then its many many weeks recovery. With the desensitization injections her infection rate has gone down dramatically.

The prednisone was given to her because of the asthma that would occur with the chest infections, when it would get to a point where she could not breathe.

She still cant attend school. We have to home school her. We attempted again January this year to see if her health was up enough to attempt going to high school but she only made it about 4 weeks before becoming extremely ill. We are lucky as she has some very good friends male and female who understand, and for example: They wont visit if they feel unwell, or have been in contact with anyone sick. Once teens understand they are probably more understanding than many adults! Let her know its ok to let them know and ask them to be aware. Its probably more the parents that need to know that their child cannot catch this as they don't understand and get scared. At this age they NEED the social contact with others their own age.

The rest I have put in email :)

Syd_Mum

Ajax hi and thanks I shall send you through an email :)

AjAX

I guess I should have read everything at once. What is desensitization? I've noticed my daughter is sensitive to sound certain touches (socks, etc.). Also I'll try the South Beach Diet. Our dr said medi because her gall bladder was flaring up-he thought too much protein but since Igg treatments no gall bladder problems. As someone said earlier - it all depends on the day what she can eat. never any red sauce or garlic or dairy. Protein seems to be good. And yes she does go to school. Missed a ton at the beginning of school (expose I guess) but I talked with the school and teachers and they were great. She needed that touch of reality. It's been hard with friend and relationships. In Feb a boy broke up with her after her first treatment so she tells everyone upfront but it looks scarey for a teen and alot to ask of a person not in your family to stand by you through the hard times. She has had a hard time making friends because they think she is a hypochondiac and you miss out when you aren't in sports with your connections.

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