A lot of people like to say "Common VARIABLE Immune Deficiency... because it is different with every patient!" It is and it isn't... and here's what I mean:
It's variable not because the presentation is so different (even though it does vary somewhat), but rather in large part because patient experience is so different. I have lung problems. Not all CVID patients do. I don't have GI problems, in large part, but a lot of CVID patients do. Some of us have sinuses that are worse than others, and no one really knows why some of us are so much sicker than others. That I will grant you.
BUT... and this is a BIG BUT...
The diagnostics are pretty straightforward. You do not have CVID if you respond to pneumovax. You do not have CVID if your IgG level is not 2 standard deviations BELOW NORMAL. This is a pretty low number, not just "out of range." Many many people are out of range and have no issues. It must be marked decrease in your serum levels. Ok? Ok. Now, with this reduction in IgG, you must also have reduced (or nonexistent) IgA or IgM. Not low IgG paired with normal IgA and high IgM. That's not CVID. Not. No. It isn't. Just stop. Also stop saying you have hypogammaglobulinemia when you have normal serum levels. Thanks.
I understand diagnostic criteria can change, but this has been standard for like 10 or 15 years. There are tons of folks who decide they have the symptoms (uh, what? You have fatigue? Definitely CVID ermmm no) without having reduced serum immunoglobulins and they respond to vaccine. I'm sorry that you feel crummy, but your feeling crummy is NOT THE SAME as mine. NOT THE SAME AT ALL. Yes, this makes me a little outlandishly ragey, but it has taken a long time to find people who truly understand, because so many people are like "I HAVE THIS" when they don't. You can insist all you like, but that doesn't change your blood chemistry.
I'm not saying you don't necessarily have a Primary Immune Deficiency, but what I'm saying is what you have is NOT what I have. So. In summary... JUST STOP.
Showing posts with label bitter. Show all posts
Showing posts with label bitter. Show all posts
Thursday, May 30, 2013
Wednesday, April 3, 2013
The B Word
Sorry I've been bad about updating. There has been a lot going on that has taken a great deal of my brain space.
It's been a rough six weeks since I returned from vacation. My breathing never returned to normal and I feel really run down. At my March infusion, I discussed this with the doctor and we agreed that I should have a high-res CT scan. That scan turned up some reasons for my feeling crummy that I was not even slightly prepared for...
I have the first spots of bronchiectasis in my lungs. That's the B word. The word which scares me more than I can fully express. I know many people who have it and, just from seeing them, I have an idea of where this leads. The saddest part is that my infections have been well-controlled and reduced by proper IG replacement, so there's not any reason I should be getting this. I should be fine. My lungs should not be getting damaged. Yet, here we are. Why? Who knows!?!?! My doctors certainly haven't shared anything enlightening.
Now I must go to a pulmonologist to get further evaluation, to include investigating the "opacity" in my lung... aka what could be a fungal ball. I've had fungus and yuck in there before, and I've had a bronchoscopy before, but this time I'm really frightened and tempted to just ignore it as long as possible. As it turns out, the medical establishment is with me on that. I called to try to get a doctor's appointment with the pulmonology group I (and my PCP and my rheum) think is most qualified to treat me. I had a particular doctor in mind, so I called. They didn't want to get me in until May 1, and not with the doctor I wanted to see....
Enter my beloved primary medical group. She talked to them and let them know my situation... They will be calling to schedule me ASAP. I feel much better since she seems to be taking me seriously and doing her best to help me as she can.
The immunologist, on the other hand, is making me feel like I'm crazy right now. I know that's not the intent, but that's the result. Telling me that I just "didn't get along" with the last pulmonologist makes me realize that he really wasn't paying attention when I told him what happened and when I cried in his office. I definitely will not be using his recommendation of another "nice guy" pulmonologist. Especially since my primary cautioned me against using that doctor specifically and the rheumatologist cautioned me against his practice generally.
I hope to get in some time in the next week or two so I can find out what is going on and get whatever testing needs to be done. I just want to feel better.
It's been a rough six weeks since I returned from vacation. My breathing never returned to normal and I feel really run down. At my March infusion, I discussed this with the doctor and we agreed that I should have a high-res CT scan. That scan turned up some reasons for my feeling crummy that I was not even slightly prepared for...
I have the first spots of bronchiectasis in my lungs. That's the B word. The word which scares me more than I can fully express. I know many people who have it and, just from seeing them, I have an idea of where this leads. The saddest part is that my infections have been well-controlled and reduced by proper IG replacement, so there's not any reason I should be getting this. I should be fine. My lungs should not be getting damaged. Yet, here we are. Why? Who knows!?!?! My doctors certainly haven't shared anything enlightening.
Now I must go to a pulmonologist to get further evaluation, to include investigating the "opacity" in my lung... aka what could be a fungal ball. I've had fungus and yuck in there before, and I've had a bronchoscopy before, but this time I'm really frightened and tempted to just ignore it as long as possible. As it turns out, the medical establishment is with me on that. I called to try to get a doctor's appointment with the pulmonology group I (and my PCP and my rheum) think is most qualified to treat me. I had a particular doctor in mind, so I called. They didn't want to get me in until May 1, and not with the doctor I wanted to see....
Enter my beloved primary medical group. She talked to them and let them know my situation... They will be calling to schedule me ASAP. I feel much better since she seems to be taking me seriously and doing her best to help me as she can.
The immunologist, on the other hand, is making me feel like I'm crazy right now. I know that's not the intent, but that's the result. Telling me that I just "didn't get along" with the last pulmonologist makes me realize that he really wasn't paying attention when I told him what happened and when I cried in his office. I definitely will not be using his recommendation of another "nice guy" pulmonologist. Especially since my primary cautioned me against using that doctor specifically and the rheumatologist cautioned me against his practice generally.
I hope to get in some time in the next week or two so I can find out what is going on and get whatever testing needs to be done. I just want to feel better.
Thursday, April 5, 2012
An Aware World
I would be remiss if I didn't mention that April is Primary Immune Deficiencies Awareness Month. Whoop tee doo. Every year I post a bunch about it, every year it's the same. People ignore it because it's not their issue. Ok whatever. That's fine.
This year, I've decided to go with a different thought path. What would the world look like if it were more aware? More specifically, what would the lives of my friends be like if we lived in a world where PIDD wasn't so strange or foreign or scary or... insert your favorite negative adjective here. It's always going to be a life altering diagnosis, but it doesn't have to be a life-ending one.
My friend G would be studying for her PhD in vocal performance and pedagogy. Her coloratura would be known in the opera community as one of staggering power and emotion. She would travel the world, in demand for her pitch perfect interpretation of Rossini. She would draw crowds wherever she went. Her classical albums sell consistently well and she lives quite comfortably. The adoring crowds love her vulnerability and are impressed that she balances her busy career with motherhood and chronic illness.
My friend L would be doing a number of things. What I most see her doing? I think she'd have her own dance studio, where she'd share her love of ballet with students young and old, teaching them the techniques she learned at ABT and Joffrey. The positive and creative atmosphere would inspire her students and attract world-renowned talent for masterclasses in her studio. She would host special performances of her studio's award-winning performances for children from the local hospitals, knowing that, because of her great team of doctors, she was able to live her dreams.
Why are these scenarios not the case? My friends were not diagnosed or treated properly. In some cases, they still aren't. They could have been. It could have been a different world for them. I hope that, someday, little girls like G and L will be able to fulfill their dreams and not feel so limited. I hope that PIDD becomes something that can be more benign and not cause lung damage, ridiculous infections that require a year on antibiotics, and will be something that is more manageable. I want PIDD patients to have full lives. All of them. Not just the lucky ones who are blessed to be diagnosed early or have uncomplicated presentations.
This year, I've decided to go with a different thought path. What would the world look like if it were more aware? More specifically, what would the lives of my friends be like if we lived in a world where PIDD wasn't so strange or foreign or scary or... insert your favorite negative adjective here. It's always going to be a life altering diagnosis, but it doesn't have to be a life-ending one.
My friend G would be studying for her PhD in vocal performance and pedagogy. Her coloratura would be known in the opera community as one of staggering power and emotion. She would travel the world, in demand for her pitch perfect interpretation of Rossini. She would draw crowds wherever she went. Her classical albums sell consistently well and she lives quite comfortably. The adoring crowds love her vulnerability and are impressed that she balances her busy career with motherhood and chronic illness.
My friend L would be doing a number of things. What I most see her doing? I think she'd have her own dance studio, where she'd share her love of ballet with students young and old, teaching them the techniques she learned at ABT and Joffrey. The positive and creative atmosphere would inspire her students and attract world-renowned talent for masterclasses in her studio. She would host special performances of her studio's award-winning performances for children from the local hospitals, knowing that, because of her great team of doctors, she was able to live her dreams.
Why are these scenarios not the case? My friends were not diagnosed or treated properly. In some cases, they still aren't. They could have been. It could have been a different world for them. I hope that, someday, little girls like G and L will be able to fulfill their dreams and not feel so limited. I hope that PIDD becomes something that can be more benign and not cause lung damage, ridiculous infections that require a year on antibiotics, and will be something that is more manageable. I want PIDD patients to have full lives. All of them. Not just the lucky ones who are blessed to be diagnosed early or have uncomplicated presentations.
Thursday, December 1, 2011
"Is that like AIDS?"
Today is World AIDS Day. I used to work in an AIDS org. This day was once a huge deal to me.
Right now, though, I feel pretty bitter about the whole thing.
Once upon a time, I used AIDS as a way to explain my immune deficiency. Those days have passed since it seems that no one can understand that, no, I don't have AIDS, and no, what I do have is not communicable. Hell, I probably won't even give it to my kids.
It frustrates me now that I'm older and understand the system a little better because AIDS makes PIDDs less visible and siphons away a lot of research money and immune system attention. there have been major developments in AIDS in the past 30 years. Not so much with PIDDs. The latest and greatest development for us is adding a tissue expander to the sub-q administration method. This is hardly a treatment breakthrough, I'm sorry.
Furthermore, there will be a cure for AIDS in my lifetime. There won't be one for PIDD. There might be a better treatment option available, which is my hope, but they don't even understand the mechanism enough to even begin knocking on the door of a cure. It's sad, but true.
Right now, though, I feel pretty bitter about the whole thing.
Once upon a time, I used AIDS as a way to explain my immune deficiency. Those days have passed since it seems that no one can understand that, no, I don't have AIDS, and no, what I do have is not communicable. Hell, I probably won't even give it to my kids.
It frustrates me now that I'm older and understand the system a little better because AIDS makes PIDDs less visible and siphons away a lot of research money and immune system attention. there have been major developments in AIDS in the past 30 years. Not so much with PIDDs. The latest and greatest development for us is adding a tissue expander to the sub-q administration method. This is hardly a treatment breakthrough, I'm sorry.
Furthermore, there will be a cure for AIDS in my lifetime. There won't be one for PIDD. There might be a better treatment option available, which is my hope, but they don't even understand the mechanism enough to even begin knocking on the door of a cure. It's sad, but true.
Monday, October 10, 2011
Bone Marrow Transplants and PIDD
I got in a rather heated discussion on Facebook today about bone marrow transplants . It seems we're talking a great deal about transplants lately. Here's the thing: it's not for the PIDD I have.
How do I know? I've asked every expert I could corner if there was a chance it would work for me. There's basically a greater chance of death than a chance of cure for me. This makes the whole idea of BMT kind of difficult to talk about for me. It is even more difficult when I see people giving information I know to be false to other people to raise their hope.
Fortunately, I think most adult patients realize that this is life. There isn't much escape from the diagnosis, once you have it and it's confirmed. My diagnosis has been confirmed by 3 doctors. The pulmonologist who initially gave me the diagnosis, a Fellow of the Royal Society (the first allergist accepted) who started me on IVIG, and the current group of doctors, lead by the former head of allergic disease at NIH. It's not going away anytime soon.
The folks most vehement about BMT being relevant/good for CVID patients were parents. Some of them have kids with more severe immunodeficiencies who were cured by BMT. Good for them. It's not going to cure the CVID kids and they wouldn't even have the chance unless they have something else requiring BMT. At least that's what the current medical literature states. Maybe there are other doctors who want to risk a patient who could live well for 90 years on a treatment that may kill them. I doubt these are the kinds of physicians you want to see anyway. I guess hope springs eternal, but I hate to see these people get their hopes up about something like this when I know it doesn't work this way.
One mother has a kid who has selective IgA deficiency. Talking about whether her daughter would be a candidate. NO! That's a TERRIBLE idea. All she has to do is take prophylactic antibiotics! I would never dream of risking her life on an experimental BMT. That seems absurd. Gene therapy? Maybe. BMT? Yeah, that's nuts. I can't even begin to tell you what a bad idea I believe that is. Maybe I've missed something, but the people I've talked to are all in agreement: no BMT for adult CVID and 90% sure none for CVID kids, either.
Now, there is GREAT evidence that SCID and several other PIDDs, CVID is just not one of them. Maybe it will be someday, and I'll be the first to get excited about the medical literature that comes out about it.
You also don't win friends with the truth.
How do I know? I've asked every expert I could corner if there was a chance it would work for me. There's basically a greater chance of death than a chance of cure for me. This makes the whole idea of BMT kind of difficult to talk about for me. It is even more difficult when I see people giving information I know to be false to other people to raise their hope.
Fortunately, I think most adult patients realize that this is life. There isn't much escape from the diagnosis, once you have it and it's confirmed. My diagnosis has been confirmed by 3 doctors. The pulmonologist who initially gave me the diagnosis, a Fellow of the Royal Society (the first allergist accepted) who started me on IVIG, and the current group of doctors, lead by the former head of allergic disease at NIH. It's not going away anytime soon.
The folks most vehement about BMT being relevant/good for CVID patients were parents. Some of them have kids with more severe immunodeficiencies who were cured by BMT. Good for them. It's not going to cure the CVID kids and they wouldn't even have the chance unless they have something else requiring BMT. At least that's what the current medical literature states. Maybe there are other doctors who want to risk a patient who could live well for 90 years on a treatment that may kill them. I doubt these are the kinds of physicians you want to see anyway. I guess hope springs eternal, but I hate to see these people get their hopes up about something like this when I know it doesn't work this way.
One mother has a kid who has selective IgA deficiency. Talking about whether her daughter would be a candidate. NO! That's a TERRIBLE idea. All she has to do is take prophylactic antibiotics! I would never dream of risking her life on an experimental BMT. That seems absurd. Gene therapy? Maybe. BMT? Yeah, that's nuts. I can't even begin to tell you what a bad idea I believe that is. Maybe I've missed something, but the people I've talked to are all in agreement: no BMT for adult CVID and 90% sure none for CVID kids, either.
Now, there is GREAT evidence that SCID and several other PIDDs, CVID is just not one of them. Maybe it will be someday, and I'll be the first to get excited about the medical literature that comes out about it.
You also don't win friends with the truth.
Tuesday, August 30, 2011
The Vanity Line
Where do you draw the line to say something is vanity rather than a real concern?
I ask this because I'm pretty sure the drug I've been taking to quell my autoimmune problems is making me lose my hair.
I realize that this is a small issue, but I'm supposed to be on this drug forever. It isn't like a temporary chemo-related hair loss. I'm not exactly sure how to deal. I'm going to have other things checked to eliminate all other possibilities, but I really feel in my heart of hearts that I shouldn't be taking the plaquenil anymore.
This leads to additional questions, not the least of which is "If not plaquenil, what?" because, jeez o pete, I just don't know. Maybe something will be revealed by compliment and other testing this month at the immuno so we can determine a new plan of attack.
Just when I thought things were getting better...
I ask this because I'm pretty sure the drug I've been taking to quell my autoimmune problems is making me lose my hair.
I realize that this is a small issue, but I'm supposed to be on this drug forever. It isn't like a temporary chemo-related hair loss. I'm not exactly sure how to deal. I'm going to have other things checked to eliminate all other possibilities, but I really feel in my heart of hearts that I shouldn't be taking the plaquenil anymore.
This leads to additional questions, not the least of which is "If not plaquenil, what?" because, jeez o pete, I just don't know. Maybe something will be revealed by compliment and other testing this month at the immuno so we can determine a new plan of attack.
Just when I thought things were getting better...
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