Just diagnosed CVID please help me understand?

[ronnie]

Hello everyone I hope you don't mind a member from Australia I am desperate for some advice. My name is Ronnie and I have just been finally formally told I have CVID with IgA deficiency. I've known about the IgA with low IgM and IgG subclasses for about two years but have never had any options explained to me, just that I am susceptable to bugs. I also have low thyroid and take replacement and have Von Willibrands bleeding disorder which has only just been worked out even though I've had lots of big bleeds afer minor surgery. I would be truly greatful to hear from anyone willing to give me a little of their time. I am very confused about my options and am not sure what step to take next in managing my life with this condition...even though I've had it from birth!

My story is probably like a lot of others except that I have an identical twin sister who has the condition also. I have had infections one after the other since early childhood. My sister and I caught whooping cough even after all my immunisations at age 5. (I still remember it!) My mother fought for years for doctors to listen that there was something wrong with out immune system to no avail. I had my tonsils out at 10 (they grew back) and my adenoids out at 24 hoping it would change my life.

I am now 37 and currently am 21 weeks pregnant with my third child. I have just gotten over a month long ENT infection. I never know when I'm going to get 'the flu' again and generally have around 10 ENT (with bronchitis but never pneumonia) infections of varying intensity per year but haven't been hospitalised since my 20's. I have shingles a minimum of twice per year. I also can get bladder infections repeatedly but haven't had one for a year now. I have tried covering antibiotics to try and prevent infection but I get terrible yeast infections while on them so it isn't really an option.

Inbetween infections I feel OK but do suffer from bouts of fatigue. I've just lived like this for so long it feels strange to think that I'm not a hypochondriac after all!

I can't predict when I'm going to get sick although winter is generally a lot worse though not always. Even though I get roughly 10 ENT infections per year I can go months without getting sick and then other times it seems relentless.

At the moment even though I am feeling weary from being pregnant I don't feel too bad. Soooo, here comes the question...IVIG? I had an appointment with my specialist last week and he said that my overall IGg levels are low enough now to entitle me to medicare funded IVIG which is a big deal here. I've lived like this for so long it suddenly feels like a really big step to start it. What is the norm to start on IVIG. I might not get another infection for months (or next week) but how bad does a person with this usually get before they choose to start it?

I have a fear at the moment that is worrying me. I have to have a c-section to birth my baby in May. Usually my flu's start in April for our winter. When I had my second baby by c-section two years ago I had the worst bronchitis I have had to date. I could barely breath (being heavily pregnant) and then when I had my c-section I was weak and poorly and coughed and coughed and coughed. A nightmare situation when you've just been cut open hip to hip. The nurses just looked at me like I wasn't trying hard enough to be healthy, I was in agony! I have found medical staff over here have minimal (ie. no understanding) of this condition at all. The main nursed who looked after me had a streaming cold and could not understand why I kept asking her to wash her hands before she touched me and refused to wear a mask and told me I couldn't possibly catch and infection because I was already on antibiotics for the infection I had!

I am wondering if I should start IVIG to try and prevent a repeat situation this time around. If so does anyone know anything about starting IVIG for the first time while pregnant?

I know I have a lot of questions and obvious anxiety I just don't know where to start in having my situation explained to me in plain english. I trust my doctor as he is the first doctor that has ever listened...he just isn't that great at talking and explaining things. I have faith that he is educated well in this area and he works half his time in pathology and research. He has basically said that if I want the IVIG let him know and he'll set it up swiftly but otherwise it's up to me and we can 'wait and see'.

In a nutshell my questions are: When does a person with this condition usually choose to start IVIG? ie. How sick do you have to be? What about IVIG for the first time in pregnancy? Could it afford me a healthier birth/c-section? I understand these are round- about medical type questions and I understand that any answers I get are general and not from medical professionals I just need a better insight than what I have.

Any advice would be truly appreciated. Thank-you for your time at least in reading this,
Ronnie.

[john]

hi ronnie,
welcome to our forum!
australia eh? you're going to be our farthest flung member, without doubt.
i am sure you will get lots of input after this post, i am in the process of getting away to the country for a few days to give my mum a treat on her 92nd birthday, but i will come back to your post when i get home.
what i found interesting and unique is that you share your diagnosis with an identical twin - just when i thought i had heard every variation on this rare condition we all share here!!
take care - john

[mali42]

The main problem for us is late diagnosis and the longer it goes on the more damage we do to out bodies,

since I have been on ivig I average 1 or maybe 2 infections a year if I,m unlucky and the infections are less severe,

My immune system was virtually nill when they found me and I started ivig right away and like you I have had this all my life but it slowly got worse and I started ivig when I was 46

If I was in your shoes I would jump at the chance to go on ivig and it does sound scary at first but once you get established on it you will feel a lot better.
Malcolm

[liz2mac]

Hi Ronnie,
I have been diagnosed with CVID for a long time and treated with IVIg for @37 years. You have some interesting parts of CVID that I don't have which I will ask about and get back to you with answers. In general I would agree with all that's been said. IVIg is as safe now as it's ever been from the point of view of reactions to it etc. You probably will react a bit for your first few infusions, i.e. you may get shivers, headache, feel sick. This is normal and your medical/nursing team will be looking out for it and will be able to help by slowing down the rate of infusion, giving you simple medication such as paracetemol (which I understand is perfectly safe in pregnancy when taken within regular guidlines). Why will you react? Well, people with a normal immune system have antibodies which, with the help of other parts of the immune system, can actually recognise the bug that's invading us and make antibodies to fit that actual bug.........clever eh? It attacks & kills or disables it and then the immune system sends in another part of it's army to gobble up the dead bug and get rid of it from our system. Now all this warfare within our system is what usually makes us feel ill. That's one of the reasons that those of us without a functioning immune system can walk around looking relatively well whilst having a quite serious infection. No warfare going on within our systems. So, when your body is finally given antibodies it is suddenly subject to a lot of work going on within it and "POW" we shiver, feel sick, have headaches. Actually though, in one way this is a good signal as it shows us things are happening. Once you are established on IVIg you will be utterly amazed at the energy levels you have. I can actually recall thinking "is this how everybody else has been feeling all my life? No wonder they were able to look after their kids, keep their houses clean, have a good relationship with their husbands and go out with the girls!" Is it safe to have whilst you are pregnant? Yes, yes, yes. This baby will be the first one to be born with antibodies. My kids weren't and spent the first two years of their lives with throat, chest& ear infections. Would I take IVIg if I were (miracle) to be pregnant now? without a doubt.
So, I hope this answers some of your questions. I'll look into some of the other issues and get back to you. Meanwhile I'm sure others on the site will have lots of help, advice & experience to offer you. Best wishes.
Liz

[emsy]

Hi Ronnie

I am relatively newly diagnosed too (3 and a half years ago) but only started infusions last week. My IgG droped dramatically when I had routine blood tests in August when I was 7 months pregnant but my Immunologist wanted to wait until I had my baby before starting me on infusions. My baby is 3 months old now.
I dont know whether this caution is what is recommended or if its because in England health care professionals are extremely cautious (and cost conscious ;-) ) I am on sub cut infusions now which I should be able to eventually administer myself at home.
During the birth of both my babies (I also have a nearly 3 year old) my Immunologist made sure that I had IV Antibiotics during Labour (Although the 3 month old arrived so quickly there was no time to set up a line so I had to have oral antibiotics straight afterwards!) This was to prevent me getting infections during the act of giving birth as apparently that is when you are most at risk of picking up infections.

Hope this helps

Emma

Emma

[s162216]

Hi Ronnie,

Looking in my copy of the British National Formulary (which all doctor, pharmacists etc in Britain use when prescribing drugs), there are no dangers or warnings mentioned for any IGG solutions when used in pregnancy; although there are some small risks of side effects with having IVIG/SCIG as with any drug (and the great majority of these can be easily controlled) I think that they are outweighed by the positives in this case as your less likely to get infections if you start having it and your baby will additionally receive IgG antibodies like normal, so rather than 'stealing' all of yours you'll have enough to prevent you from getting infections and keep your future child healthy. Whether or not to give IVIG is decided by your doctor based on your IgG levels, if their only slightly low then daily antibiotics can be enough but too low and your at risk from infection. Its extremely expensive (I cost the NHS about £830 every 3 weeks for instance) and in short supply sometimes when plasma/blood donations drop, so doctors are careful before deciding to start IVIG.

Sam

[ronnie]

Thank-you so much everyone for your responses. I am overwhelmed that you all care enough to read my post and respond. At the moment I'm emotional and in a way grieving for the years that I have thought that there was something psychologically wrong with me and that I perhaps thought myself sick like some kind of self fulfilling prophecy that I would one day learn to overcome. Oh and the great jobs I've lost because I couldn't go more than three weeks without taking another day off with the flu...I could go on and on.

Liz, your post made me cry! Really could I possibly get through life not feeling like my body weighs that of a small elephant? (even though I'm slim). Could I have the energy and zest for life of 'normal' people? For so long I've called myself a procrastinator or lazy because I'd rather lie down than go for coffee with friends or repot my sad and neglected potplants.

I'm also more than interested in the health of my baby so I will definately be following up the IVIG in pregnancy.

I'm interested what all of you have to teach me about this condition and your experiences with it. So, please keep your posts coming.

Could having IVIG really give me better daily energy levels? That excites me more than anything.

I know it's a hard question but did any of you start IVIG without being hospitalised regularly for infection. Because although I get sick so far I've gotten by without anything really serious happening to me. Should this matter or is it only a matter of time before it does?

Thank-you again for your responses,
Ronnie.

[mali42]

Hi Ronnie
All I did before ivig was work, lay on the sofa until bedtime and then repeat. I used to do all the gardening but my wife had slowly taken over that roll and I did feel lazy but I was just so exhausted with continued inections.
I was never hospitalised but I think I was very close to that towards the end, I was lucky because when I got an infection the antibiotics worked well for me,
I had to wait a month while on permanent antibiotic and then started on ivig once a week on lower amounts to get me used toit and after3 weeks it was every 2 weeks, years later I am on 3 weekly ivig, but we all vary,
I now do home infusions with my wife assisting which makes life so much better, I hate hospitals as you can imagine.
since ivig my wife has had a cold which I usually catch but one time she had one I didn't catch it which was amazing.

[liz2mac]

Oh no, two weepy women on each side of the world. You WILL have more energy. You WILL understnd that you are not lazy or have some weird syndrome by which you soemhow "make yourself ill". I will try to get mys husband to put up on the net I made YEARS ago with my then Immunologist, Dr Matthew Helbert. If John manages this I'll put up the link.
Keep talking to us. Somre of us have been there before, some of us have specific medical knowledge, some of us have access to Immunologists who will answer questions we don't know the answers too. Some of us are experienced in acting as advocates; all of us suffer ourselves and all of us care. No, it most certainly doesn't matter that you've not been hospitalised with an infection. The greatest risk is that we are left undiagnosed/untreated and develope damage in lungs and other organs. We need to get as many people treated at an early stage before that happens. Without organ damage and with replacement therapy there are many people out there living as close as damn it to a "normal" life.
I'm sendimg you virtual hugs, a cup of coffee and seriously bad for us cjhocolate cake.xx
Liz.

[s162216]

Hi Ronnie,

I was only ever in hospital with an infection once (when I was 3, about 16 years before) before I started IVIG, I found out that I had CVID after seeing my new immunologist and within 3 weeks I was starting IVIG (at a different hospital though and with a different consultant as I was going to university). I've never really suffered from serious infections but did used to have recurrant chest and skin (from my insulin pump cannula) infections and with the level of IgG that I had it would have only been a matter of time before I got a serious one, especially being at University and living in quite close proximity with people.

For once I didn't have a cold all winter either which was a great bonus!

Sam

[gilliem]

Hi Ronnie. I was only hospitalised once in my whole life, and saved that up for when I was 52! I came out with a diagnosis of CVID and, like Malcolm, the unwelcome news that I had 0.04% ivig trying to battle any infection I came in contact with! Not surprisingly, I was more ill than I care to describe! I have had ivig every 2 weeks for nearly 10 years now and I am rarely ill, have enough energy for what I want to do and have rediscovered the enjoyment of life. You will too.
Best wishes,
Gillie.

[john]

hi ronnie,
i'm back and am proud to see the responses our wonderful members have made to your post.
all i can add is that 20 years ago i was sure i was not going to pull out of a constant stream of crippling coughing and agonising sinusitis, and then i was diagnosed and began the path back to pretty much full health, with IVIG the main reason. as sam says, they don't easily prescribe it here due to the cost (mine costs the nhs £2300 every three weeks), so we only get it if they're sure we will benefit. i do a volunteering befriender job at my infusion clinic, and no patient has ever told me that their life wasn't transformed after IVIG or SCIG kicked in.
it's great to have you on board ronnie, feel comfortable to ask about ANYTHING to do with immuno deficiency and you WILL get a reply!
take care - john

[ronnie]

Thank-you again everyone. I'm still amazed that so many of you care about how a 37y.o married pregnant woman from Australia is feeling about cvid! I'm new to using discussion boards also. I have a couple of more questions...I'm just going to keep on asking as they come up, I hope you don't mind. Do I keep doing it in this one column or am I supposed to post a new topic for each question? Please let me know if I'm not putting these in the right place.

My General Practitioner told me I was IgA deficient a couple of years ago after finally doing a test (even though I'd been seeing him with 12ish infections per year for 12 years to get yet more antibiotics). He said that I was also IgM deficient and had a deficiency in IgG subclass 3. My overall IgG at the time was low but within normal limits. Like I've already said earlier I've been very prone to infection since forever!

I had an obstetrician appointment today and I overheard that my overall IgG results were 6.2 with normal lower limits starting at 6.9. So it looks like I'm not super low. What does this mean? (IVIG here in Australia is restricted also to the most extreme cases) But, even with these results I am still apparently entitled. Does IgG being below normal limits (even a smidge) mean the same thing for everybody?

I also heard that for the first time my IgA was within normal ranges on the day I was tested with the lower normal limit being 0.7 and my result being 1.2. I this a low normal result? I was sickening with a nasty cold the day I was tested does this affect IgA levels?

And if I start IVIG with IgA deficiency does this mean that I will still be prone to infection but that the IVIG will help me fight it better?

From what I have been finding on the internet if I have low igG then my baby will have a lower than average immune system at birth because of my low IgG. When I asked about that today my obstetrician said 'nawww. I don't think the baby will be affected because it's in it's own protective bubble.' How helpful. These are the sorts of answers I get to nearly all my questions to the doctors.

Last but not least. What does low IgM mean? Does everyone have this too? It is not hugely below normal but still down a bit.

I know I'm asking a lot of basic and probably tedious questions and I appreciate your patience.

Thanks, Ronnie.

[slf]

Dont worry about posting,just hope we can help.
I have'nt been pregnant,but i do know that non immunology doctors dont recognise or understand CVID.
I have no detectable Iga,and ivig seems to keep infections at bay for me,not sure about the other levels,but yes we do have Igm,i know Igg and Igm are low on me but am a bit vague.
Hope that you will get much better advice soon
Take care
Steve

[liz2mac]

Hi Ronnie,
I'm a bit out of my depth here, so I'm asking questions from some one who does know. It may take a day or two to get back to me but as soon as I hear I'll post again. As to the baby being in it's own little bubble...........words fail me. Sometimes Doctors don't use their common sense. Yes your baby is in it's own little sterile bubble, but the nature of pregnancy is that it comes to an end. Babies have their maternal antibodies for about two years, after which time they begin to manufacture their own. Breast milk contains the mother's antibodies as well. So, your baby will be born with whatever antibodies you do have, but will not get those you don't make until it is a toddler and can make its own. I really think the best thing for you would be for you to see an Immunologist. I will find out where the Immunologists are in Australia, what there names are and how the Australian system works for you to get to see him/her. I had a friend years ago who was Australian and her CVID was treated by an Immunologist. As to replacement therapy. The thing I'm hesitant about is the fact that you appear to be producing some IgA. Sam will probably be able to tell you more about this than I can, but I know that people who do produce some IgA have to be careful which replacement immunoglobulin they go on because, for reasons I don't fully understand, the IgA in your body may react with certain products. It's not a reason not to have replacement therapy, just to be careful over which product. As to IgG helping the baby, yes replacement will help the baby and will help you with all the things we've previously discussed.
Please don't worru about asking questions. The immune system is so complicated that (as you are finding) most Dr's don't get it. Then here are we flung in at the deep end, HAVING to know, as we then in turn have to teach all our family & friends and our Dr's! We've all experienced things like you are now. Getting diagnosed and properly treated is not easy. As to whether you have to start a new thread......I don't know, I'm new to all of this as well. Sure John & Sam will tell us though. I've been so heartened by this forum, its friendly, informative and caring. Thank you everybody for making my exploration into this type of support such a good experience. Liz.

[anilla]

Jan 16, 2012 9:45:55 GMT liz2mac said:

I will try to get mys husband to put up on the net I made YEARS ago with my then Immunologist, Dr Matthew Helbert. If John manages this I'll put up the link.

i'm interested in seeing this! hope you can post it soon.

[emsy]

Hi Ronnie

You seem to be going through the exact same anxieties as I did when I was pregnant. I was diagnosed during my 1st pregnancy so had loads of questions and the same ones that you have. I kept getting the same kind of answers that you seem to be getting, telling me that it was unlikely that I will pass CVID onto my baby as its not hereditory. However, reading about peoples experiences on this forum and also the facebook one, there seems to be a heck of a lot of people who's children also have CVID for it to be a coincidence. My nearly-3-year-old was born with a cold and constantly has a runny nose- I worry that he has picked up my condition but I guess only time will tell. My 3 month old......so far no problems at all but then its early days.......

Continue to ask the questions anywhere you like is what I say as I can guarantee someone else would also like to know whatever answers are forthcoming. Thats the beauty of these forums :-)


Read more: primaryimmuno.proboards.com/index.cgi?board=general&action=display&thread=12#ixzz1jwPWPRGR

[liz2mac]

Hi Anilla,
Yes I'm going to do my best. Trouble is that one of our Immunologists was in it as well and he's away at the mo. Can't post it until I get his ok.
Liz

[ronnie]

Hello everyone.

Thanks everyone. My 5 year old daughter was a very healthy baby and didn't get her first cold until she was 8 months old. BUT, I was also a 'single parent' at the time because my husband was away (he's a soldier in the Australian Army) and I seriously lived like a hermit with my daughter waiting for him to get back. She only caught that first cold when he got home and I started catching everything she had. I'd been relatively healthy and so had she because we rarely went out! My second daughter got her first cold (of many) at 7 days old even though I was breastfeeding her. It was definately obvious that my antibodies in my breastmilk were not the best. I can't help but think that with two young siblings in the house our new baby doesn't have much chance unless I start the IVIG.

I've burned my brain thinking in this last week..... there is so much to adjust to in finally getting a proper diagnosis... and so little to adjust to because nothing has changed I just know I'm not crazy anymore! I've been asking a lot about whether IVIG will make me feel better and when/why did others start on it blah blah but in reality this is not a fair question for me to ask because the only way I'm going to know if I'll feel better on it is to start treatment.

I've realised that my questions are related a lot to my fear (terror) that maybe I'm not 'sick enough' to notice a great difference after IVIG if that makes sense. I think that my expectations are in danger of being too high and I've been hoping for some kind of guarantee that I'll feel amazing. So I've decided I need to keep a lid on that! Next week I will be scheduling an a appointment with my immunologist asap to try and get started on the IVIG.

I have started a new topic to ask some basic (less fortune teller type) questions so please have a look at it for me? I appreciate your time and I will keep everyone updated on how I get on. I'm sure I'll have heaps more questions to fill in gaps for me as I go on. I will also share anything I find out that could help others struggling with this.

Ronnie.

[s162216]

Hi Ronnie,

Be aware that it does usually take several months before the IVIG makes you feel less tired or ill, after 3 months I certainly started to feel 'different' although I couldn't quite work out how as had always felt the same and couldn't remember anything different.

BTW, in your other thread I've given you a quick overview of the parts of the immune system affected in CVID.

Sam