Good luck tomorrow! Have all your questions ready!! You have already empowered yourself SO much by learning as much as you can before hand :) Keep us updated!

I just logged on to message you good luck and saw this. No need to worry. The hardest part will probably be finding parking around UAB. In Birmingham they say it stands for the Untiversity that Ate Birmingham. Lol.

Let us know how it goes. Though in all honesty you won't know all that much for another month. But for about a week after getting the pnuemo vax I actually felt a boost. But it only lasted a week or so. :( You're seeing a good doctor so ask all the questions you can think of, bring a list if you need to. Good idea to bring the folks. It's always good to have extra ears, we can get overwhelmed with it all.

Good luck.
ZzZappergirl

Ok - so today was pretty epic for me. I am so very glad I went to see this doctor. He is absolutely brilliant - but there is no trace of arrogance. He was personable and very 'real'. Every person working in that office is friendly and sincere - and very professional.

He said there is no question that I have CVID - he is working to identify the sub type. He says my rapid loss of lung function is very serious. I am not yet to the 'point of no return' but if we do not act to treat this very aggressively, then I will be in a few short years. The good news is that with treatment, he believes we can not only stop the decline but regain some ground.

Having mom there was very handy. The detail of the medical history was mind numbing. Mom was able to remember a lot of what I couldn't - not just about me, but about extended family members. They gave her a screening free of charge and we are both now a part of two research studies. Our blood cells will be immortalized! LOL Any treatment or tests related to research are free of charge. Non-research related tests/treatments, of course, are not. I'm pretty sure that they will do some genetic testing in the research study part.

I got vaccines for pneumonia, diphtheria, and tetanus (all pain free) and they also injected two different types of fungal nasties (one being candida and I can't remember the other one). They also injected a 'control' substance. They have also given me a TB test.

They took 26 vials of blood. I am the vein-less wonder and there was no pain and no floundering around for a vein. I am so grateful.

Gotta retest for titers in 3-4 weeks, of course, and can't start treatment before that (although they will contact me immediately if they find something in the tests they just took). I will be doing IVIG (I think he said Gammanex? Gammunex? -- anyway...starts with 'Gama')..and that normally they start people at 4 g/kg and ramp up to 6..but in my case we would start at 6 and ramp up from there. The good news is that they have some home health people that they use so I don't have to check into the hospital - that these people are more than prepared to handle any negative reactions. He mentioned that we will have more risk of negative side effects with this aggressive schedule but that he thinks it is important to do so.

I spoke of my concern about being moved to a cube environment at work (all the germs...people bringing in illnesses from day care, etc.). The doctor told me that once I can get these treatments going - I might just be the 'healthiest' person there. :-) So - there is reason to have hope. It's just going to require a little patience at first and I'm going to make an Olympic effort to get better just as fast as I can...I'm going to stay positive.

I know I am leaving out a lot of information but it's been a long day and I have a pretty nasty lung infection at the moment. I think I've earned a day off work tomorrow. I just need to absorb all of this and get some rest. Haven't had a vacation in years and years.

They gave me a huge book about primary immune disease (complete with bright colors and pictures) - but it contains a lot of real information. I want to re-read everything I consented to w/ the research studies and look at my notes. Mom will remember parts that I've forgotten.

Oh - I asked him about this. In the past, I've had my IgE levels tested and they were WAY high. I asked how (if at all) this related to low IgA and IgG (IgM is near the bottom of the 'ok' range). Of course, the IgE is related to hypersensitive allergies (I've been tested for 80 separate substances - I tested positive to ALL of them). He spoke of it in terms of one part of my immune system trying to compensate for the weaker parts. He talked about this being related to selective IgA deficiency. I need to look at my notes and use my google degree on this one. I thought that was interesting anyway.

My IgG is low too but I got the impression that rather that aim for a target IgG number - the goal was to target better lung function.

He talked about how once you get Immunoglobulin replacement - it can throw off blood tests and give you some false positives if you are tested for other things...that you should always let a doctor know if they are doing blood tests or tests related to research.

Oh - he said that I absolutely should not get a flu shot...but that people around me should get a flu shot. I'm not sure if that is for their protection or mine.

I'm sure I'll think of a lot more - I'm just mentally and physically exhausted right now. Thanks for listening and thanks for being there. I know a lot more now than I did this morning...and I'll know a lot more in a few weeks. When the doctor does his big write-up for my pulmo doctor...he will also send me a copy.

Wow he sounds amazing :)

Interesting about the flu shot as it is NOT live vaccine. I believe it was the CDC and/or Immune Deficiency Foundation that recommends people with immune deficiencies receive the flu shot. Maybe he thought it wouldn't help you?

He was correct about getting tests after starting the IVIG. Most docs do not realize we can throw false positives to antibody tests as we are receiving thousands of others peoples blood and in some situations we can throw a false negative because we don't make our own antibodies.

The 2 brands that start with Gamma - Gammagard and Gamunex. Just remember each brand affects each person differently and read on here on how to prep for IVIG and you will do great!

Are they doing a ct scan to check the lungs? PFT's? The top docs recommend a baseline CT scan when dx with CVID.

Yay for you!

WOW! I am so jeleaous, I need to go see this guy for sure. I learned more through your visit than I did through three of my visits with my doctor.

I haven't heard about the false positives on blood tests, what's that about? Is that just for antibody tests or would that be for other tests too, since as you say we have thousands of other peoples blood in us. I never thought about it. Though I guess we are really getting just the antibodies and everything else is filtered out... Hopefully, right? Idk. Just thinking out loud. Any insight on that?

I "believe" this is just on antibody tests that they can be very inaccurate on us. We make little to no antibodies. This is kind of why they give the vaccinations as a confirmation of CVID. If you do not produce antibodies, or very low antibody response, that confirms cvid.

I think its a good thing we get other peoples antibodies. They (the professionals) say once we start receing treatment we shouldn't get pneumonia again. I think that in part is because we are getting other peoples antibodies to it. We also get previous flu strain protection. So although I will get a flu shot this year, I should be protected from previous strains from my ivig. Gosh, did any of that make sense? Did I even answer your questions lol

Kelli - yes to the PFTs. My FEV1 is 44% right how. Funny thing about that - he gave me the print out and I was going over all the numbers, graphs, text - there was a little line of text that said, "Incentive: Birthday Cake". I said, "WHERE is my birthday cake!!" ;-) (i knew it was on the screen but it was just fun to make them laugh)

I've been getting PFTs every couple of months - sometimes more often...he had all those records too.

I had to go to National Jewish Research Center in Colorado (premier place in the world for that - let me tell you) for my asthma/allergies a few years ago and they did a CT Scan (I may have had one since too). They did an 8 day workup on me and this doctor had all of those records so this is probably why we didn't do a CT scan.

One interesting thing - when mom and I went to the lab with our bucket of vials for the vampires -- the doctor's right hand nurse went with us. She didn't go with us to show us the way or to keep us entertained either -- she went with her big bag of vials too. She is the 'control'. She has the CVID genetic markers but she doesn't manifest any of the symptoms. Mom talked to her about this - she says she has to come down to the lab and do this about once a week...and that sometimes the doctor himself was the control. That tells me - these are people who are serious about some research...and this really is their life's work.

My appointment started right on time - and I mean to the minute. One of the nurses took me back to get vitals, height, weight, etc. - and we went over my paperwork (which was voluminous - let me tell you). I felt like I was buying a house LOL But - when we got through and she walked me back to my room...I fully expected to wait in that room for the doctor. Imagine my surprise when she opened the door and the doctor was sitting right there at a computer in the exam room and his right hand nurse was there too. We started immediately.

I can totally understand why it takes six months to get in. It is totally worth it. In my case - I got in much sooner because I was on a waiting list and someone cancelled.

Oh - and all the exam gloves were purple...and so are the blow covers for the PFTs :-)

One thing though - if you are already getting gamma globulin replacement....it could skew the tests there (as mentioned earlier). The nurse mentioned them having a problem like that earlier that day...so if you want to get in it would be worthwhile to talk to the nurse in advance (she is very helpful and informative) and see what to do. In my case, I was already scheduled for hizentra - but I called the nurse to ask the day before my appointment and she told me it would be ideal if I could wait.

I asked about differences in one gamma globulin product over another. He mentioned differences in the number of doners, whether or not the doners were volunteer, the country/population where the doners lived, the binding agents used in the product. Apparently there is a great deal of difference from one product to another.

He said that for me, he really preferred the IV method over the sub-Q just because we really needed to get my levels up in a hurry. He mentioned, however, that for people on medicare, hizentra is really the only option available...and it isn't because of the affordability of the drug so much as it is the cost of having a person in the loop to administer the IV.

Kelli - I got the impression that it could skew more than CVID tests.

Sorry I must not have explained myself well. It can skew certain rheumatology tests, for example (in addition to multiple other tests) that check for antibodies - so its very important to have a rheumatologist this is not just lab results driven - they need to be able to really take your symptoms into consideration. Thats what I mean by antibody tests being inaccurate. We are however given the pneumonia vaccine to see if we mount an antibody respone.

I went to National Jewish as well, for a week in 2011. It was quite an experience. I believe they took 26 vials of blood from me one day. They are the ones who officially diagnosed me with my rare lung disease caused by CVID. I had SO many weird tests there, it was crazy, very thorough :) My current doctor worked for NJH for years and took a particular interest in this specific lung disease and has several patients with it.

Thanks! Just never heard that referenced to, but it makes perfect sense.

Kelli - at National Jewish...did you have to do the 'phone booth' test? OMG -- such fond memories of that (NOT!)

Phone booth test? Hmmm I don't think so. Lets see I had:

Bronchscopy (develped a nasty fever after blech)
Blood Gas? Where they strap a freaking wet suit thing over your face and attach a hose for you to breathe out of, slam a needle into your arterial line (your wrist), miss, poke you again and then put you on a bicycle and tell you to pedal away? LOL
Barium Swallow tests
Respiratory therapy
CT Scans

Man I could go on and on haha

OMG'sh are you talking about the PFT? I do those every two months and I have to sit in a phone booth thingy for like an hour

There's one test in the phone booth where they remove all of the air...and you can't breathe in no matter what you do. That was not fun.

Re: Blood Gas -- are you sure they weren't trying to extract intelligence information instead? Sheesh!

Ok so I clearly have never played phone booth with the docs and thank gawd for that.

LOL too funny...gotta laugh right? I was stuck on o2 the entire time...I was so traumatized. I told them I will NOT use it when I get home. My daughter is 8 for peets sake and will freaking disown me