Neti Pot Amoeba

I keep seeing various articles like this one about people contracting the Naegleria fowleri amoeba via a neti pot.

First of all, you should never use tap water in a neti pot. I find them to be a high risk item anyway, because they must be properly sterilized to prevent infections of a number of baddies, so I prefer to use a can of sterile saline to ensure my safety. My doctor has said numerous times that neti pots and saline irrigation systems must be sterilized often and you must use distilled water. The packaging also says this.

Second, this amoeba must travel through the sinus cavities. This means it could have been contracted in the shower, bathtub, any number of ways. Usually reports of the amoeba surface after swimming in a freshwater lake.

Finally, how bad is this water treatment facility? The amoeba "isn't supposed to survive" the treatment process. Sure, you can't get it from drinking it, but people regularly interact with water via their nasal passages without any neti usage. I think that the use of a neti pot or similar system is far too beneficial to dismiss or stop doing because of these incidents. If people follow manufacturer (and their physician's) instructions, they wouldn't be using tap water in these things to begin with.

A Central Line

There has been some chatter lately among my PIDD friends about central lines, the pros and cons, etc. A healthy friend saw some of it in the news feed, particularly an xray with a port shown, and sent me a Facebook PM.

"what's that thing in that girls chest? does she have what you have? will you have to get one of those sometime?"

I answered that it is a central line aka a port, and while I don't need one currently, the plan is not to give me one anytime in the forseeable future, but if I need chemo or something, I will probably have one installed. This is true, but it isn't the whole truth.

The whole truth is that I will very likely need one of these, or a picc line at the very least, at some point in my lifetime. A high number of chronically ill people do. With the odds of my getting some form of cancer being pretty good, a port is not far outside the realm of possibility. It scares me, so I don't like to talk about it or think about it. A port means critical illness to me, and I really don't want to have to go there.

Unfortunately, I have some friends who don't have a choice right now and that's really challenging me.

Concern

I know a lot of people with immune deficiencies. They are a constant source of love and support, especially lately. Knowing these people the way I do, I carry a lot of worry for them.

Two people who are very dear to me are quite ill. They each have very unique and scary situations. Things that I am not entirely sure I could deal with. They both need extra prayers to whatever deity suits your beliefs best.

One is having surgery at 11am, so whatever you've got, she could use it.

A Note to Smokers

I'm not going to sit here and lie to you. Yes, I have smoked cigarettes socially in the past. Could I dream of doing that now? No way. The nature of my disease progression has been into asthma and autoimmune lung problems, so smoking would not only be a bad idea, but a costly one to me in terms of overall well-being almost instantly.

That being said, I have friends who are smokers. Sometimes they complain about the lack of places for them to smoke or about people who ask them to not smoke in a certain area. I've been known to speak up to someone who is smoking where they lack rights to, because it actually matters to me. Most of the time, I suck it up and walk through or behind or whatever, but when someone is smoking in a nonsmoking area, it really bugs me. You make a personal choice to smoke. I didn't make the choice to have CVID and some sort of amorphous autoimmune issue. When you smoke in inappropriate places, places where my lungs should be getting a break, it causes me physical pain.

What do I mean by physical pain? I feel as though that cigarette has morphed into a torch, which has been inconveniently shoved into my lungs. It costs me enormous piles of spoons that I would much rather spend elsewhere.

I would like to make a request of my smoking friends. Please be polite about smoking and don't smoke where you aren't supposed to. It really can become a matter of life or death on a bad asthma day. I may look great, sound great, seem fine, but I could have had an asthma attack a couple hours prior. I'm not asking that you quit smoking or not smoke in public. On the contrary, I'm asking you to abide by the social covenant and laws set out by various jurisdictions. Don't smoke where you aren't supposed to. Is that so difficult?

"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.

Aww, Mom

This made my day better:

How are you feeling? Please take it easy if you can and rest. Have been thinking about you and how proud I am of you! You are able to get thru things that would put most people under. They would give up. I love you because you are my daughter. I respect you because because of the woman you have become. luv u mom

I love my mom.

Shutting down the whiners...

I find inspiration and advice for life in all sorts of places, many times those places are blogs about chronic illness. Sometimes, these posts hit home in a way that's hard to explain.

This post from Dear Thyroid is no exception. I really get the feeling a lot of times that my empathy is broken. I just can't get my knickers in a twist about your bad haircut. You're fortunate to know that your hair will grow back.

I especially get this way when average people complain about being sick. I work through things that would put most people on their ass for multiple weeks. Perhaps I shouldn't, but there are certain economic realities that encourage me to do so.

I've been a little quiet lately because this is one of those times that I'm struggling to keep my head above water in a lot of ways. I'm really sick. I tried taking a course of augmentin, that didn't work, so now I am on biaxin. I think I'm back on the upswing, but I know that if I don't take a break, I will be totally shafted and may get worse. I'm sure most people don't wake up in tears due to fear that this will be the time I end up hospitalized again or that this may be the infection that kills me. I'm going to go out on a limb and guess that most of the population hasn't felt that way.

I feel that way, at least to some degree, every. single. time I get sick. Every time. It's almost paralyzing, which is one reason I sort of just keep going. I figure if I ignore what is happening, perhaps it will get better on its own and I won't have to worry about it, so long as I take my medicine. Most of the time this is the case, but there will be a time when it's not. I just hope I'm prepared for that time.

In the meantime, and to get back to my point, I really feel like people take a lot of their blessedness for granted. I know I'm guilty of this too, but before you post that whiny facebook status about how you're out with a cold, just remember that there are people who get things much worse than colds far more often than you get a cold that are unable to take the time off for it they should, even if their employers offer sick leave. Also remember that your "tragic" events should be kept in perspective. There are usually worse things.

Junk Science

Lately, in my facebook group, there's been a lot of discussion of "miracle cures" which amount to nothing but junk science. I find this kind of post one of the most insidious and, frankly, mean ways people can post on the boards.

Why do I say that? Where do I get off saying something is junk science? Well, if it has existed for 50+ years and doctors aren't doing it for their patients, there aren't any really solid evidence-based studies... it qualifies, in my book. Especially if there's no logical reason it SHOULD work and do what it says. For example, Ozone Therapy. It's been around for ages and it supposedly super oxygenates your blood and kills all sort of viruses in the body. Ozone does that outside the body, but it certainly seems like a stretch that the ozination of your blood would be particularly helpful.

Here's why, from what I've read: Basically, your blood can carry so much oxygen. The de-oxygenated blood goes to the lungs for a "refill" and is gets something like 95% saturated. If extraordinarily oxygenated blood goes in, it just stays saturated, your body doesn't add more oxygen to it; the lungs don't work that way. So it doesn't go into the veins, it goes into the arteries. So you want to put little airbubbles in your blood stream? The kind of thing that causes those IV pumps to go crazy because it's risky? Yes. That's what these people do. They put thousands of tiny airbubbles straight into your arteries. These bubbles can remain for 30 minutes or more and cause an increased risk for such sunny things as pulmonary embolism.

I understand that people think that the FDA is a racket. In some ways, I agree, but I can get into that more later. The ozone process is not a single manufacturer that is being blocked by big money, though. It's a process. A process which the FDA says is dangerous, and I tend to agree.

There has also been this issue raised of GC-Maf, which is some sort of macrophage activation drug. This I believe that this is a possibility, but the fact that it's obscure and not really active since 2008 tells me it must not have been that productive a breakthrough. I'm sure there will be developments in this area, but right now, I'm not seeing anything particularly convincing. Besides that, my macrophages are fine and having more active ones don't seem like they'd help me all that much. Who knows, though? It remains to be seen.

Missing Out

I know I sometimes rush to judgement about certain things, but I have a hard time not doing that, especially when it comes to kids with PIDD.

As an adult with CVID, I take a lot of calculated risks in order to live a full life and do the things I want to do. It fills me with sadness when parents of CVID kids limit the fairly innocuous things they do. For example, someone in an online support forum decided not to let their child in the bouncy house because she didn't know how often they clean it. The jumpy house isn't the problem, in my mind, the other kids inside would be. With handwashing and everything the kid will be fine. I understand that parents feel the need to be protective, but to shut your child inside and not allow them to experience the world is a huge mistake.

In some respects, knowing my mom, I'm glad I wasn't diagnosed until later. Mom still has never tried to dissuade me from doing the things I want to do. I missed my grandmother's last 2 Christmases because of different active germs in my parents' house, so it's not like I'm cavalier about the risks. I didn't want C-Diff or Shingles because those can last a very long time in patients like me and have devastating consequences. If there was some sort of MRSA scare or something, maybe I'd be understanding, but to just tell a child "you have this thing, therefore you can't have fun" is just cruel to me. I believe it will lead to further resentment both of the illness and the parents, and will not teach the child that it's ok to live.

They don't keep CVID patients in a bubble and never have. I don't think there's a real reward to living that way, especially for children. Just teach them to wash their hands and to not touch their faces in public, and let the Ig replacement do its work.

Vaccine Infographic

I think the above portion (click to enlarge) of the infographic found here is rather telling. Parents who vaccinate feel that parents who don't are depending on them for the health of their child. Nonvaccinators also don't see the risks in not vaccinating their kids, which is frightening to me, especially given the rise in preventable death from things like whooping cough, measles, etc. I get vaccines even though they are totally useless for me. Vaccines are safe and are definitely safer than measles, mumps, polio, whooping cough, tetanus, etc. If you can't fathom that a healthy child is more important than you having control, perhaps you should seek therapy.... after getting your child immunized. And yes, I judge you for not getting your child vaccinated.

Mortality

A very public advocate for PIDD issues passed away on October 6th. She had the same immune deficiency, diagnosed the exact same time age-wise (she's about 15ish years older), and seeing the same immunology practice.... We even have the same first name.

I don't know how she died, but at the end of June, at the IDF Conference, she looked robust and healthy and seemed full of passion about PIDD issues and the new Affordable Care Act provisions that would help us.

The news of this death hit me pretty hard. I see myself in her shoes and I wonder... is treatment much better for me than for her? What killed her? Will I die in a similar fashion? Who would I want to know? How would I tell them? Being that I live a good deal of my "disease life" online, I have to make sure that there's some way to let them all know when things happen to me. It's scary to think that so many of my friends exist primarily in my virtual world...

That's really beside the point of my post though. Seeing her obituary, seeing the things she's done, I'm proud to say I knew her and we were connected. I'm still frightened because I wonder if I will have a similar expiration date. I hope not.

One of the 99%

I am so glad the young lady who wrote the sign above has had so much good fortune in her life to be able to work a 30 hour a week job at minimum wage and graduate from college debt-free. Good for her. I went to a fancy private school on a full ride that took care of everything. Books, room, board, etc. Something happened while I was in college, though. I was diagnosed with a Primary Immune Deficiency Disease... and nearly every major life choice I've made since has revolved around that illness.

It is good that there are people who are responsible with their money, but to imply that the argument of the 99%ers is just about their being irresponsible is silly. I can be responsible all day and all night, but yet there are unexpected expenses that just come up. I bet this young lady doesn't have to worry about several hundred dollars' worth of medication just for one bout of infection... which will happen several times this winter. Meanwhile, I am on a medication that is causing me to gain weight because it is the best option. So I am going to have to buy new clothes to make sure I can still look professional at my job. I am doing what I can to try not to gain weight, but there really isn't much I CAN do. I save as much as possible, but it's absurd to me that someone would think my financial situation had something to do with my character. It really doesn't.

This woman will never have to take a lower paying job to keep health insurance. Heck, her first job can even not have insurance and she can stay on her parents' policy. I had to take my first job when I did or risk losing insurance entirely... and with a $10k per month plasma habit... that is SO not happening. Oh, and what if you have to pay for one of THOSE out of pocket? Could she do that? I'm going to guess that's a negatory.

So before you judge people for their financially-strapped situation, perhaps you should consider that not everyone has the opportunity to choose the bed they're lying in.

"Not that I'm a doctor or anything..."

That phrase sums up my experience with many doctors, especially young ones. They have this medical-school-induced hubris that gets really annoying... especially when they try and out-jargon you. If a friend reaches out for medical advice for an ailment I know about or have experienced, I will often reply with what works for me. Inevitably, there's a post somewhere in the thread from a friend who is a doctor or a nurse who says essentially the same thing I said, but using medical terminology. I know those words, too, but they aren't particularly helpful in trying to explain to someone how to soothe their bronchitis.

I think I get most annoyed because I probably read a lot more medical literature than many doctors do, especially when it comes to immune system, asthma, and allergies. I read this stuff not only because I'm curious, but also because my life could someday depend on my knowing that a certain drug may induce a certain response in someone like me. It's the most time consuming "hobby" I've ever had.

Every doctor should realize that there are patients out there who do have knowledge and who are very well-versed in their diseases. I've finally got a team of doctors who realizes that I do read the literature and do understand it. It would just be nice if every doctor in every medical advice forum would actually consider that other people have good answers, too. But that would require people to read the whole thread before they comment... and who does THAT?

Deadly Vitamins!

I really wish the scientific community would come out with more concrete information when publishing statistical studies. I want even more for the media who publish such studies to include questioning information.

A great example is this study which links vitamin supplements with an increased mortality rate. There are several problems I have with this. First, I think it's a little muddy to be announcing supplements as dangerous. Many people supplement because they've seen a doctor who has told them to. Perhaps these people have more health problems, which lead them to take the supplements, which could also explain the increase in the mortality rate. I don't think I'm entirely going out on a limb to say I find the correlation to be dubious at best.

I feel as though I am constantly telling people that correlation is not causation, especially when it comes to medical information. Just because it happens to be that way doesn't mean the vitamins are the cause. We just don't know enough yet to scare people out of taking supplements that their doctors have approved or requested them to take. Besides, a 2.4% risk is pretty low anyway.

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.

Trying.

I am turning a new leaf in med compliance today. I filled the pill minder. I will be better.

Tick... Tick... Boom!

Sometimes I feel my life is a ticking time bomb, especially this time of year. I managed to make it through the summer relatively unscathed, which is unusual for me, so I am certain it is merely a matter of time before I head down the path of cold-sinus infection-bronchitis that is so familar.

I feel myself flinch every time someone around me coughs or sneezes. Every single time. Probably because I know those could be the germs that make me sick this time. Heaven only knows what those germs <i>are</i>. I, for one, don't want to be the one to find out.

I am thankful for this stretch of fairly good health. I don't think I've been on an antibiotic since July, which is really rare, as the summer months tend to be the worst for me. Now, as the weather turns colder and folks debate the flu shot and cold prevention, I become restless. I want to do things, to see people, but I don't want to be sick. It's a difficult position to be in, since I can't really control whether other people disclose their health status to me.

If you are sick, please stay home. I really don't want to get sick.

Back to School...

It seems to me that there are an increasing number of vaccine stories during the back to school season. I suppose that is when they are most relevant, but the fact that we're seeing record numbers of children go unvaccinated is stunning to me. I know parents are trying to do whats right for their children, but absent a reaction or religious reason, every child should be vaccinated as much as possible.


This article has a lot of good (and scary) information on lapsing vaccines and touches a big reason to get your kids vaccinated....

The increasing number of kindergartners entering school without immunizations poses a risk to others, especially children who have legitimate medical exemptions that prevent them from getting their shots, said Linda Davis-Alldritt, a school nurse consultant at the California Department of Education.

"Disease prevention is really a very important thing," she said. "These are diseases that can be very serious, and it can cause death and it can cause long lasting illnesses."

I really can't help but feel like I'm at high risk. I guess it's a risk I must take to be part of the functioning world, but it upsets me that I might be the victim of someone else's negligent behavior.

Agoraphobia

I realize that, as I grow older, I am becoming agoraphobic. I don't like the feeling of being surrounded by people and feel the need to escape. I feel almost panicked. It's really strange and only happens in certain situations. If I'm in a crowd that's supposed to be moving but isn't, that's a big trigger for me.

I also find that I don't really like going out if there are going to be a lot of people around. I mean, I do have some legitimate reasons to not really want to go out during cold and flu season, but I feel much more inhibited by my own hangups than by any perceived danger, at least most of the time.

Today, though, I had a dude sneeze on my foot. He turned away from one group of people to sneeze on another. I mean, I guess it's good that he didn't sneeze on those other people, but still...

I see people who look really rough on the metro, too, and I can't help wanting to use copious amounts of hand sanitizer when I exit. I've been trying to remember to wash my hands before I begin working, but I'm concerned that's not enough. I do touch my face a lot, so I'm pretty sure the metro is going to be the death of me...

Is 28 too young to become a total recluse?

Marie Claire Article

Marie Claire decided to say the unthinkable.

Though breast cancer researchers and advocates perpetually plead for more money, the disease is, in fact, awash in it. Last year, the National Institutes of Health, the nation's top agency for health-related research, allocated $763 million to the study of breast cancer, more than double what it committed to any other cancer. The Department of Defense also funds breast cancer research ($150 million this year), as do several states, most notably Texas and California. All that is in addition to the money raised by the roughly 1,400 IRS-recognized, tax-exempt charities in this country devoted to breast cancer. They operate in every state and in just about every major city. The largest of them, Dallas-based Susan G. Komen for the Cure, grossed $420 million last year alone. All told, an estimated $6 billion is raised every year in the name of breast cancer. And the money keeps pouring in....

Yet what many in the breast cancer community are loathe to admit, despite all these lifesaving developments, is that, in fact, we are really no closer to a cure today than we were two decades ago. In 1991, 119 women in the U.S. died of breast cancer every day. Today, that figure is 110 — a victory no one is bragging about. Breast cancer remains the leading cancer killer among women ages 20 to 59; more than 1.4 million new cases are diagnosed annually worldwide. Roughly 5 percent, or 70,000, breast cancer patients are diagnosed at a late stage, after the cancer has metastasized — that rate hasn't budged since 1975, despite all the medical advances and awareness campaigns. For these women, the prognosis remains grim: Only 1 in 5 will survive five years out. Fundamental questions still elude researchers: Why do a third of all women considered cured by their doctors suffer recurrences? Why are breast cancer rates rising in Asia, where they've been historically low? Is it even possible to prevent breast cancer, and if so, how?

Seriously, read the whole article: Breast Cancer Society Scam - Breast Cancer Industry Scams - Marie Claire

Confessions: Follow Up

One of the reasons I think I'm finding it difficult to blog lately is that I'm wrestling with a lot of things that I just don't know how to express effectively. The medical stuff I've been dealing with has been a little distracting and disheartening, but I think that will pass.

Earlier, I confessed how difficult it has been for me to be medically compliant and deal with things as they are happening. I was putting things off and being bad about things instead of acknowledging the problem and addressing it head on. So now I have done just that. I went to see the eye doctor. My eyes are apparently just fine. She was not concerned by the degradation of my vision, as it seems to be within normal bounds of what people experience year over year. So that's a relief. She said some of the other problems could be dryness in my eyes and that an OTC eye drop may help. That is a major load off.

The other thing is the matter of the plaquenil in general. I have established through my own, though admittedly non-scientific, methods that it was making me gain weight and lose hair. I'm having my immunologist run some thyrod tests along with the compliment testing we decided to do. He thinks the compliment testing will be negative, was curious about the TSH tests, and decided to add some vitamin D testing to the mix. Now we wait to see if there's anything going on or whether it is the plaquenil, which I've started taking again. Aren't you just so proud? You should be.

I am trying to frame things in a different manner. I am starting to feel like I am finally coming into my own as a person, and I am determined to treat myself better. If the plaquenil is going to make me gain weight, then I had better get my buns to the gym. I was discussing alternative treatments with my immuno and we agreed that the weight gain is a lesser of evils compared to what may happen on other drugs. We'll ride this out until December (if possible) and see where we are when I return to the rheumatologist.

I've Got Good News

I got some exceptional news! I have been contacting my congressman's office constantly about the Medicare IVIG Access Act and I found out that Rep. Moran has decided to sign on as a cosponsor!

Dear Ms. Miller: Thank you for taking the time to contact me regarding the Medicare IVIG Access Act (H.R. 1845). I appreciate learning of your interest in this legislation.

As you know, this legislation would establish a Medicare demonstration project to evaluate the benefits of providing intravenous immune globin (IVIG) in the home. This proposal has the potential to save billions of dollars for Medicare. I am pleased to tell you that I have added my name to the list of cosponsors to H.R. 1845.

Thank you again for contacting me.

Sincerely, James P. Moran

Huzzah! This makes me feel like I actually matter, at least a little bit, in the whole grand scheme of how things work in Washington. Maybe, just maybe, little people can sometimes have a voice.

Diverticulosis

Diets that exclude certain things entirely have always seemed to good to be true, to me. I did South Beach once and did lose weight, but I gained it back once I went back to regular eating habits. I have always believed that lifestyle change and portion control is much more important than any sort of gimmicky diet plan.

A friend of mine is talking about eliminating gluten from her diet. Gluten is not the bad guy. I have many strong feelings about this. I think that people doing this are just a slave to the latest fad. I know folks who have to eliminate gluten due to allergy or sensitivity and I understand that it is very difficult for them. Gluten is everywhere in the food you eat, the medicine you take, even some bath products can have gluten or wheat in them.

 I take special exception to the faddish elimination of gluten in no small part because my mother has diverticulosis. You know what they think causes diverticulosis? Low carb diets and processed foods. Shocking. So you mean we should eat more whole grains,  fruits, and veggies? Not rely on processed-to-the-point-of-being-unrecognizable foods? No. Way. Because that might actually make a difference in our overall health and well being.

Yet Another Breast Cancer Post

Andrea Mitchell has announced publicly that she has breast cancer. She was kind of flippant about the whole thing, which you can read more about here. The dismissive nature of her message was very odd to me. I certainly hope she's not in some kind of denial about the routine nature of breast cancer (1 in 8 women get it, according to what she said, and then went on to make it sound instantly curable). I think it's dismissive of the pain that women feel when they go through this disease and can make it a more lonely feeling.

I feel very strongly that the conversation on health in this country is so backward. We attach ourselves so closely to outcomes that we can't have the requisite compassion for people. Yes, breast cancer is very curable, but that curative process is very difficult both physically and emotionally. I wish more people recognized that about all illnesses, not just breast cancer.

SPOILER ALERT!

After my previous rant on the subject, I thought for sure I wouldn't get these:

Ok ladies, it's that time of year again, in support of breast cancer awareness!! So we all remember last years game of writing your bra color as your status?.....or the way we like to have our handbag handy? Remember last year so many people took part that it made national news and, the constant updating of status reminded everyone why we're doing this and helped raise awareness!! Do NOT tell any males what the status' mean, keep them guessing!! And please copy and paste (in a message )this to all your female friends to see if we can make a bigger fuss this year than last year!!! I did my part... now YOUR turn ! Go on ladies...and let's have all the males guessing! .. It's time to confuse the men again (not that its really that hard to do :)) Everyone knows it makes their brains work wonders on what we're talkin about!! The idea is to choose the month you were born and the day you were born. Pass this on to the girls only and lets see how far it reaches around. The last one about the bra went round all over the world. So you'll write... I'm (your birth month) weeks and I'm craving (your birth date)!!! as your status. Example: Feb 14th= I'm 2 weeks and craving Choclolate mints!January-1week Febuary-2weeks March-3weeks April4 weeks May-6weeks June-8weeks July-10weeks August-12weeks September-13weeks October-14weeks November-16weeks December-18weeks ..Days of the month: 1-Skittles 2-Starburst 3-Kit-Kat 4-M&M's 5-Galaxy 6-Crunchie 7-Dairy Milk 8-Lollipop 9-Peanut Butter Cups 10-Meat Balls 11-Twizzlers 12-Bubble Gum 13-Hershey's Kisses 14-Chocolate Mints 15-Twix 16-Resse's Fastbreak 17-Fudge 18-Cherry Jello 19-Milkyway 20-Pickels 21-Creme Eggs 22-Skittles 23-Gummy 24-Gummy Worms 25-Strawberry Pop Tarts 26-Starburst 27-Mini Eggs 28-Kit-Kat Chunkie 29-Double Chocolate Chip Chrunchy 30 Rocky Rd 31 Milk Duds

Awww. Did I ruin the "game" and your "fun?" Perhaps you shouldn't send me these things. How about it? Sounds good to me. Yes, I realize I'm a grump. I have my reasons. See post linked above.

Confessions

Like most people, I worry about the future. My worries are a little different than those of most people, at least the ones who don't have an illness like mine...

I worry about so many things about the future. Will I be able to keep working? How long until I have to go on disability? Will anyone be around to take care of me or will they care to? If I think too much about these things, I'm pretty certain my head would just explode. Most of the time I feel pretty confident that things will work out and that there is a greater plan at work, but sometimes, it's really difficult, especially on those days when getting out of bed feels like too much.

Recently, I've noticed some visual acuity changes. I went to a shopping center ophthalmologist. He said I need to see a better one with better machines. My eyesight has deteriorated a quarter over the past year. Prior to that, it hadn't changed in over 6 years. It frightens me because plaquenil can cause these changes. So, in addition to the hair stuff (which I mentioned the other day), I am really worried about my eye sight, but I really don't want to see a doctor about it. Funny how that works, huh?

I really feel like there's a lot going on with me in an emotional sense. The thoughts of what will happen next if the plaquenil has to be stopped are frequent and scary and bring me back to those worried I mentioned earlier. What if I have to go on chemo for the autoimmune stuff? What if I have to get IVIG more frequently? Both of these are possible, maybe not probable, but I really won't know until I see an eye doctor and call my rheumatologist. I promise I will, it's just a difficult call to make. It makes me really sad.

Thankfulness

As much as I complain I realize I do have many things to be thankful for, even with a chronic illness. I recently turned 28, and part of my birthday practice every year is to reflect on the most important and precious things in my life. Sometimes these things change, but some never do. I figured I should share these things with you...

I am thankful....

... that I have a loving and supportive family. We have our eccentricities, but I know they love me and I hope they know I love them. I try to show that to them as much as I can. We fight, we cry, but we're very functional.

... for my grandmother's life. She passed away between birthdays 27 and 28 and I couldn't be more grateful to have known her.

... for friends that actually care and take an interest. I have cultivated friendships with people that feel mutual and not like I'm using them or vice versa.

... for a stable, adult relationship that doesn't cause me more drama or create negative situations for me.

... that I seem to have finally wrapped my head around some parts of my illness. It's challenging and complicated, but I'm really glad I have a smart enough brain to think it out.

... that my sinuses don't get badly infected and that PO antibiotics still work well for me.

... that my insurance pays for my IVIG treatment.

... for my mobility, even if it isn't perfect all the time, it's nice to be able to walk around and go the places I want to without too much assistance.

... for unrestricted food. Many of my friends have to eat limited diets because of their disease progression and manifestations. Boy am I glad I have food freedom!

... for music.

... that there is real beauty to experience in the world.

... for last November's trip to Hawaii. I didn't know if I would ever get to go there, but I can tell you it's a magnificent experience. You should definitely do it.

... that I have people I can call if I get into trouble and need help.

This is the short list. There are more things, obviously, but these are the most important to me right now.

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...

Diagnoses

Sometimes, I just want to scream at people...

YOUR DIAGNOSIS IS NOT WHAT YOU THINK IT IS OR WHAT YOU KEEP CALLING IT!

Yeah, so, hypogammaglobulinemia is NOT a disease. It's a symptom. Just because it has an ICD-9 code doesn't make it a diagnosis. If you have low IgG and still respond to titers, you have selective IgG deficiency. If you have low in several immunoglobulin categories (which is the definition of hypogamma) you have CVID. Furthermore, it's hypogammaglobulinemia NOT hypogammaglobulin anemia. GAH!

I know this kind of precision doesn't matter to most people, but I think it is critical that we all at least speak the same language. If your physician has diagnosed you with this, perhaps it's time to ask some questions. If you look at the IDF website that lists every recognized primary immune deficiency, you will not see hypogammaglobulinemia on the list. It's time for more questions, people, because what you're talking about is a symptom, not the disease. This leads to confusion, etc.

Most of you that read this have noooo idea what I'm talking about. Some of you do, and some of you will differ with me. I'm talking medical literature. Your doctor could, in this case, be wrong.

The Cost of Noncompliance

Let's just say that sometimes, I'm an idiot. I know this about myself, it is truly not news. Sometimes, though, I am in utter awe of my stupidity and the silly things I do. My recent less-than-compliant behavior is a great example.

Apparently, I decided that being on vacation meant I didn't have to take my medication. I'll let that sink in for a minute. Lalala no pills 2x per day lalala. I got out of the habit of taking my plaquenil. I was already not great about it... Then vacation came and I lost all will to take my medicine as directed. It's really not that much to take in the grand scheme of things, but I wanted to be like the other kids, dammit. Or, more accurately, I just didn't want to have to think about it.

I have to think about it now, that's for certain. Why did I do this to myself? I know if I am noncompliant bad things happen. I start to feel more fatigued than ever... I was about to fall asleep at my desk last week... And still I didn't start being fully compliant until... YESTERDAY!?!?!? I have had bad pain and fatigue and everything else, but I was like LALALA NO PILLS! Look where that has landed me... At least it was only for a week or so, but still. I am a loser for not taking my meds like I should. I know this is a problem that many people struggle with, so I've decided to be completely honest here...

I am in pain (albeit less pain now than, say, Sunday) but every moment of it is my own darn fault.

WMATA's Disability Application

I was thinking this morning about disabilities. If you've read many of my posts, you know I contemplate this on a pretty regular basis. This time, I was sparked by the large signage about disability fares on WMATA. I have an ADA Disability that requires accommodation, so I should qualify... should being the operative word.

I went to WMATA's website to further investigate their policies. There are some things I am beginning to feel I should take care of before I something happens, as a kind of insurance policy against the future, and this is one of those things. Especially since the disability office has the worst hours known to man. Why are they only open 8:30 AM to 4:30 PM on some days and 8:30 AM to 2:30 PM on others? Because they're WMATA.

Anyway, I found their policies on the application but I found it to be a little quizzical. I expected that my doctor would be required to fill out the form, that's fine, but the categories they have are interesting and as follows:

1. NON-AMBULATORY: An individual is unable to walk and requires the use of a wheelchair or other mobility device.

2. SEMI-AMBULATORY: An individual has a chronic condition, which substantially limits the ability to walk, or is unable to walk without the use of a caliper leg brace, walker or crutches.

3. AMPUTATION: An individual has an amputation of one or both hands, arms, feet, or legs.

4. STROKE: An individual has substantial functional motor deficits in any of two extremities, loss of balance and/or cognitive impairments three months post stroke.

5. NEUROLOGICAL CONDITIONS OTHER THAN STROKE: An individual has difficulty with coordination, communication, social interaction and/ or perception from a brain, spinal or peripheral nerve injury or illness, has functional motor deficits, or suffers manifestations that significantly reduce mobility. A specific diagnosis is required.

6. PULMONARY OR CARDIAC CONDITIONS: An individual has a pulmonary or cardiac condition resulting in marked limitation of physical functioning and dyspnea during activities such as climbing steps and/or walking a short distance. * If diagnosis is asthma, please state whether: a) Individual has been on systemic medication for the immediate past six months, OR b) Individual has been required to use fast acting inhaler for three or more episodes per week for the immediate past six months. A specific diagnosis is required.

7. BLIND OR LOW VISION: An individual is legally blind, whose visual acuity in the better eye, with correction, is 20/200 or less, or who has tunnel vision to 10 degrees or less from a point of fixation or so the widest diameter subtends an angle no greater than 20 degrees. An individual has low vision, and whose visual acuity is
in the range of 20/70 to 20/200 with best correction.

8. DEAF OR HARD OF HEARING: An individual with a pure tone average greater than 70 dB in both ears, regardless of use of hearing aids.

9. EPILEPSY: An individual has had at least one tonic-clonic seizure within the past
four months.

10. DEVELOPMENTAL OR LEARNING DISABILITIES: An individual has a significant learning, perceptual and/ or cognitive disability. Some conditions are excluded from eligibility such as attention deficit disorder (ADD). A specific diagnosis is required.

11. MENTAL ILLNESS: An individual whose mental illness includes a substantial disorder of thought, perception, orientation, or memory that impairs judgment and behavior. A specific diagnosis is required.

12. CHRONIC PROGRESSIVE DEBILITATING CONDITIONS: An individual who experiences debilitating diseases, autoimmune deficiencies, or progressive and uncontrollable malignancies, any of which are characterized by fatigue, weakness, pain and/or changes in mental status that impair mobility. A specific diagnosis is required.

Ok, so most of this seems reasonable to me... until you get to #12. Autoimmune deficiencies? Do you mean Autoimmune Diseases and Immune Deficiencies? I mean, my illness is characterized by joint pain and fatigue, so I'm pretty sure my doctor will sign off, but I really wish it was more clear. I also fear that, since I don't have a listed diagnosis, approval will be challenging. I am hopeful it won't, but I don't think I have the courage or desire to start the process now. Maybe by the time my next treatment comes around, I'll be ready.

UPDATE: This little gem is also a little troubling to me... Granted, none of my things are contageous, but things like TB and HIV are...

Who is not eligible: People whose sole incapacity is pregnancy, obesity, acute or chronic alcoholism or drug addiction, or have a contagious disease. Financial need is NOT a consideration.

Whooping Cough Vaccination

Sorry for the hiatus from posting. I took a little vacation.... but worry not! Back to business as usual.

The Virginia Department of Health is urging parents to have their children receive the Tdap vaccine for tetanus, diphtheria and pertussis (aka whooping cough) in no small part because there has been a 217% increase in whooping cough cases in Virginia in the first 6 months of this year. (article
here) Whooping cough is scary, especially since it is so communicable. So next time you're due for your tetanus vaccine, ask specifically for tdap. It could save your life or the life of someone you love.

Read a little more about pertussis and get vaccinated!

SCID Newborn Screening: One Year Later

The Health Resources and Services Administration has released the report it submitted to the Secretary of Health and Human Services Kathleen Sebelius one year after adding SCID screening Recommended Uniform Screening Panel. Some of the highlights:

*To date, 961,925 newborns have been screened and 60 infants, or approximately 1 in 16,032, have been identified with some form of immune deficiency.

*Fourteen infants with SCID (~1 in 68,000) have been diagnosed and received treatment.

*No missed cases of SCID have come to the attention of the newborn screening programs conducting the pilots.

*Emerging findings from the pilots are advancing understanding of SCID and triggering new research efforts.



(All items taken from the report which can be downloaded in PDF format here.)

This is amazing! I am a little stunned by the numbers they mention in the report. I know I had heard about increased incidence among hispanic and closed populations, but I was really surprised by the Puerto Rico findings. I'm hopeful that perhaps this report can help encourage other states to go forward with screening efforts. Let's increase this from covering 25% of US births to 100%!

Doctor Patient Relationships

My medical team is awesome. We've discussed this before. I'm starting to form my own methods and opinions for how I've developed rapport with doctors. It's something that has taken a significant investment on my part, and some willingness to listen on theirs.

I think the difference for me has been becoming insistent. I had some of the same symptoms for a long time before I started trying to address them with my doctors. Sometimes, they were dismissive or wanted me to wait. It was when I decided to take more ownership and drive my own care that things began to change for me and I began to feel better about my level of care.

I didn't change doctors to accomplish this. I started doing research and asking better questions. I started connecting my symptoms and being more willing to discuss them in detail, making notes to ensure I didn't leave things out. I really wanted to understand what was going on with my care and wanted my doctors to understand how these things were making me feel physically. It has gotten to the point where my doctors now even ask about my emotional health. This is a major change for me.

I won't pretend that any of this was easy. It has taken years to get to this point, but now I go in to my appointments with more confidence. I discuss with my doctors the treatment plan, look at various options, and make sure we're all on the same page. It feels wonderful to be able to do that. My caveat would be that you must approach them in a professional, collegial manner. That's been the big key for me. I have utmost respect for my physicians, but I no longer fear them or just sit quietly while they dictate the treatment plan. Not every doctor will engage like this, but I think a lot more of them will than you might first think.

I know a lot of people struggle with this balance of asking questions but not seeming to be too aggressive. It takes time to learn and I think it's important to try. Small steps at first, like discussing all the available medications for a specific symptom, or even figuring out what OTC cold medications are right for you. These little things can begin to change the way you see your doctors and vice versa. You may also learn that things you've "always done" aren't necessarily the best or most advantageous for your body. Don't be afraid. Odds are your doctor wants you to be as healthy as possible, just like you do.

No, Your Immune System is NOT Horrible

My poor facebook friends become blog fodder sometimes. Sorry in advance, guys.

It kind of annoys me when people rant about their "horrible" immune systems. I know I should be used to such things, but it's still annoying to hear a healthy person say they have a "horrible" immune system. Now I understand that people use this as a hyperbolic statement when discussing why they get sick, but it strikes me as strange. I'm pretty sure I get sick more often than most people, but I still wouldn't say I have a "horrible" immune system, at least not most of the time. It's just not there.

Most immune competent people don't realize how insensitive it sounds when they talk to someone who is so fundamentally broken in this important way. It's like "yeah, my immune system is bad, too." You may think you've got a bad immune system, but I'd give anything I could to have the chance to have what you do, but that will never be possible for me. No one has perfect health, and if you're exposed to new bugs, you've got a decent chance of getting sick. The difference is, you'll be well in a few days with the right care. Even with the right care, a stray bug could kill me.

I guess it's all about perspective.

Breast Cancer "Awareness"

As I am wont to do, I got in a discussion on Facebook about these silly, cryptic status messages. I am of the opinion that telling your shoe size in inches with a frowny face doesn't actually do anything to promote awareness or show support for breast cancer patients. In fact, I think the whole thing cheapens the experience of cancer.

Cancer sucks, I'm sure. I've never had it, but I have been very sick and have a chronic illness, so I can pretty well imagine that it would suck pretty hard. I've seen people go through chemo and it seems rough. It's not glamorous, it's not easy. I think it would be much better if people took the time to go to a chemo ward and sit with these women going through the worst experience of their lives. Hold their hands, bring them a sandwich. Actually do something meaningful.

It was pointed out to me that some people hide their cancer. I think this is well within their rights. I hid my PIDD for a long time and was reluctant to talk about the serious nature of my illness. I get that people don't want to make people feel sorry for them. That all makes sense to me, especially after having to have a needle biopsy for a lump. I verbally threatened everyone who knew that if I had cancer, they were to never ever lay hands or give to me anything with a pink ribbon. The pink ribbon movement was borne out of a sister's grief, but has taken things to a ridiculous and disrespectful level. You can now get "breast cancer awareness" themed grocery items, kitchen wares, NFL hats, and yes, even fast food. But what does buying these things do for the actual patients? They keep trying to find a cure for cancer.

You know what? I'm going to be really bold here. There is no cure for cancer. There. I said it. Cancer is the overgrowth of your body's own cells. That's what makes it so challenging to treat in the first place. It isn't some sort of virus we just need to figure out. The only way to "cure" it entirely would be to either teach the body to fight these types of cells or to stop cell growth. When your cells stop growing, you die. So I would rather we have a conversation about better, more effective, less invasive treatments for cancer, instead of this constant drumbeat about awareness and a "cure."

There Are Some Things I Will Never Understand

I've been trying to recruit people to sign and pass along this petition about infant screening for SCID in Florida. It takes about 2 seconds to fill out and pass along. Or just fill out.

It has 324 signatures.

I received a notice about a friend supporting another petition. This one was about animal cruelty, specifically wanting someone to get the full punishment for hurting a dog. It's a negative petition rather than a positive one, in my opinion. It has signatures in the tens of thousands.

Apparently it is more appealing to punish someone who hurts a dog than it is to save dying babies. I don't think I will ever understand that. The kid who killed the dog will be punished in some way, maybe not the maximum sentence, but he will be punished.

Without public support, 8-10 SCID babies will die in Florida this year. The first one already died, the second is currently fighting for life.

I guess I shouldn't be too surprised. There was, after all, an animal welfare charity before there was ever one to protect children.

Inability

Inability is wicked.

There are tons of things I probably shouldn't do with a PIDD. There are things that are counterindicated and blah blah blah. I know I take a lot of "risks," but often these things are rewarding and/or necessary to continue to live a normal life.

Then comes the inability. Oh man. Today, guys, for serious, I could not for the life of me open my soda bottle. I struggled for several minutes to open the thing. Now, I'm pretty sure they haven't made these things locked down like Fort Knox, but that's how I felt. I did finally get it open, but left indentations on my hands to be able to grip it tight enough to open it. Seriously. From a soda bottle. Yeah. I'm that special.

It's stuff like this that makes me consider the big picture fears I have about career, family, etc. These fears, coupled with my overarching feeling of inability, can really impact the choices I make. Why am I doing my current job? Health insurance. Is that a good reason to take employment? Absolutely not. Did I have a choice? Not really.

There's this pervasive message in the US that you can do anything you set your mind to, be anything, anywhere. For some people, that's not the case. For me, that's not the case. I am generally an optimistic person and will still try to do those things I shouldn't or can't do, but that doesn't make it any easier. In fact, sometimes, it makes it more difficult to come to terms with the reality of inability.

Hey! It's Me!

A few months ago, I went to tell my story at a briefing for interested congressional staff. The event was sponsored by the Plasma Protein Therapeutics Association. I am reminded because the briefing was written up in their summer newsletter called The Source. (Check the section called Inside PPTA)

In case you're curious, here's my story, as shared that day in March.

Good Afternoon.

First, I’d like to thank the IDF and the PPTA for allowing me to be with you today to share my story and I’d like to thank all of you for taking the time out of your schedules to listen.

I look like an average 20-something DC professional. I have the suit, the shoes, the hectic schedule, but a big part of that schedule is my Primary Immune Deficiency. I was diagnosed with Common Variable Immune Deficiency (CVID) just after my sophomore year in college. I receive IVIG treatment once a month at my immunologists’ office in Chevy Chase. IVIG is a miracle of modern medicine. At any given time I have the antibodies of 10,000 plasma donors protecting me. These donors give me an incredibly wonderful gift and I am incredibly grateful for their gift.

Looking back, there were signs when I was a child. I was always small for my age, was a failure to thrive as an infant, and had some really strange infections, including a big skin thing on my forehead... in the 5th grade. Totally tragic for me, at the time. Overall, though, I was a pretty normal kid and nothing seemed to be amiss until my freshman year of college.

I went off to Washington and Lee University, looking forward to a bright future and a great education. About midway through the winter semester, I felt terrible. I figured it was just the “winter term blahs” as we called them and that I’d be fine. I just needed to work hard and finish the semester. Spring term would be easier.

Spring term was easier, because I could sleep all the time between classes. My friends were outside, enjoying the beautiful weather, and I made my dorm room into a cave for constant napping. I finished the year and packed my bags to work at a Christian Appalachian Project camp in rural Kentucky. I just assumed I was stressed out and that’s what was making me so tired. I went to the family doctor before I left, because I felt I had a sinus infection. The doctor gave me antibiotics and sent me on my way.

I felt good for the first 2 weeks of camp. At the beginning of the 3rd week, I became delirious with fever and landed in the ER, 45 minutes away from camp. From there, the doctor admitted me. A few days later I was transferred to the ICU at the University of Kentucky where I stayed for 11 days while they ran tests and tried to get me well again, at least well enough to leave the hospital and go home to Virginia. They put me on high-powered antibiotics that finally kicked the infection and I was released without any answers about why my pneumonia was so severe.

I began seeing a pulmonologist back near home at UVa who wanted to understand my pneumonia and why it was so intense, given my limited history of illness. He kept me on antibiotics for the start of the fall semester, until a few weeks before my winter break. I got pneumonia a week after I stopped antibiotics. After that, I underwent extensive testing that included a PET scan, bronchoscopy, thoracentesis to test the fluid around my lungs, and bi-monthly CAT scans to monitor a spot on one lung. That lung was later biopsied and it was found to have a massive pocket of infection the antibiotics couldn’t touch. Yes, I was still taking classes in spite of everything. My life consisted of eat, sleep, class, doctor’s appointments, and rounds of medical testing.

My doctor at the time reached his wit’s end. He’d never before seen any case like mine. He presented my case to colleagues and got no answers. As a last resort, he ran a fairly simple blood test on my antibodies and we found our answer. In his 30 years as a physician, he’d never had a patient with a Primary Immune Deficiency.

I was diagnosed with CVID and, after my fourth pneumonia in a year, I was admitted to the hospital to begin IVIG treatments. That first treatment was horrible. I had an anaphylactic reaction to the product, due to the lack of Immunoglobulin A in my system. Since then, we’ve learned to mitigate the reactions through a cocktail of pre-medication.

Thanks to IVIG, I have only been hospitalized once since I started in the summer of 2003. I was able to return to and complete college in 4 years, graduating with distinction in 2005. I have had a full-time job and an active social life. The only difference between me and most of you is that I miss work once a month to receive IVIG, am on a first-name basis with my pharmacist, and get call-ahead service in the Emergency Room.

I arrive at my doctor’s office at 7:00 AM on treatment day because I could be there for 3 hours or 8, depending on which product I receive. Right now, I’m on a product that infuses in 3-4 hours and this product makes me feel like I could take on the world. My friends lovingly refer to it as my “go juice.”

Prior to last month, I’d been on another product that isn’t as effective for me and does not keep me healthy. It also causes flu-like symptoms in me – massive headache, body aches, malaise. It’s very much like getting hit by a truck once a month and having to go to work the next day. Unfortunately, that’s all my insurance would cover at the time. I was still getting sick, but that did not influence the decisions from the insurance company, regardless of my physician’s recommendations. Fortunately, I became a permanent employee in November and have managed, 3 months of struggling later, to finally work out getting my preferred product.

Why does the product matter? Because I still get terrible infections on some brands of IVIG and I miss out on a lot of things when I don’t feel well. Because of the ineffectiveness of the last product for me, I’ve missed almost 3 weeks of work since September. I almost missed my sister’s wedding. Luckily, my fever broke sometime during the night before the ceremony and I was able to participate fully.

Getting that better-for-me product was quite a challenge and involved two and a half hours on the phone with the specialty pharmacy, insurance company, and my doctor’s office to ensure that my product would be covered, and that I would have it in time. Now, I make 3-4 phone calls a month to make sure that everything will go smoothly on treatment day. A call to the specialty pharmacy to initiate the refill and pay my copay, a call to the doctor’s office so they can initiate delivery, a call to the doctor to confirm delivery date, and a call to confirm the actual delivery of product the week before treatment. There is a lot of juggling and scheduling that goes along with having a chronic illness. It has made me a lot more calendar aware and organized. I have to plan my life around treatments. I try and plan trips to be right after treatment, so I can better ensure I’ll be healthy through the whole trip.

Without IVIG, I would spend much more time in the hospital. I wouldn’t be able to work and there’s a very good chance I wouldn’t be sitting in front of you today. IVIG makes me just shy of normal. I still get fatigued, I still get infections, but I can pretty much lead a normal life. I am very fortunate to be receiving the product I want in the setting I want. Most patients don’t have this luxury, and some can’t even get IVIG at all. For some, it’s a month-to-month insurance battle. For some, they’ve had to call state regulators and their congressional representation to get their IVIG authorized. I have friends who wound up in an ICU because their insurance decided they needed to do a trial off of IVIG for 3 months, despite the objections of their physicians. I recently learned another friend became septic due to infection while unable to receive IVIG. It’s scary to know that I could lose access in the way that I’ve seen others lose it, and even scarier to know that it would kill me. I am extremely lucky, but I wish I didn’t have to be.

It was quite a rewarding experience and I would definitely recommend this kind of advocacy to others.

"Friends"

I'm connected to several different people in a variety of disease communities. It's helpful to be able to think that other people have weird things going on and can offer advice on different subjects.

I have found myself very much enjoying the tweets of @Steph_in_NC. She's snarky and honest. I admire her sense of humor and the way she is able to express her frustrations. One of her tweets today struck a particular nerve with me. Why is it that people seem to care so much more about some stupid and frivolous things? I'm not nearly as tolerant of cause marketing for diseases as Steph is, but man oh man. Who does this sort of thing?

My feeling is that this is someone who can't actually face the big scary things, so she focuses on the small, trivial things. Really? Someone can't get their excess stomach skin fixed right now? It will eventually go back. Steph's lupus isn't going away, neither is my PIDD. Is it really so hard for people to think about someone else's actual, real problems? I guess it is more attractive to deal with the manufactured ones. Sigh.

Migranes? Really?

I will say it right now. I don't agree with much that Michele Bachmann stands for, especially with her tendency to rewrite painful parts of American history.

Now that's out of the way, I want to talk about this whole migraine business. She has been known to suffer from severe migraines for several years. I know first-hand that these can be entirely debilitating and difficult to manage. She says she is managing them with medication. That should be the end of the story, but somehow, it's not.

First off, I don't understand why this is a story. She should be free to manage her medical conditions as she and her physician see fit.

This is troubling on other levels. What if I were to want to run for political office? Sure, I don't get sick that often and am usually still functional, but how would the media react to someone like me running for office?

I think they'd be completely and totally understanding. YEAH RIGHT. They would tear me and my physicians apart, questioning every procedure I've ever had. They would not see me as a person, but as someone who has a "debilitating" condition. Are we not all more than the sum of our parts?

I recall the West Wing episodes where it was revealed the fictional president had MS. I believe now, more than ever, Jed Bartlet got off easy in those scenes, tense though they were.

I feel certain that FDR would never be elected in the internet age, or even in the television age. Presidential aptitude is judged on so many more criteria now, more than ever before. You have to be healthy, good looking, and have some gravitas (hopefully). You can't be seen as weak in any way. Where would I fit into this structure where physical weakness is an automatic non-starter? What if I wanted to be in our political system? I can't very well do a lot of shaking hands and kissing babies (especially recently-vaccinated ones).

Our representative government doesn't seem so representative of me or my needs today.

Better know a Cytokine: IL-25 (formerly IL-17E)

First things first. IL = Interleukin.

Now that the housekeeping is out of the way, we can get down to business. I figured it would be best to start with the cytokine that really got me into this line of thinking about the fundamental components of the immune system that we don't really know or understand well yet. I was reading a recent article from the Journal of Allergy and Clinical Immunology, like I have been known to do, and it hit me. This IL-25 thing may be a huge key to a lot of things.

How did I get there? Well, the article in question is related to non-IgE mechanics for allergic inflammation and airway reactions. I was curious about this particular phenomenon because I have many friends who produce no IgE but yet manage to have severe allergic reactions to varied things. I decided to read the article, then got hooked. I read further articles and there are some interesting things that make IL-25 unique.

IL-25 was once thought to be in the IL-17 family of cytokines because of the way it has been demonstrated to differentiate the T helper 2 lymphocytes into different immunoglobulins. It has a distinct role from IL-17A and IL-17F which eventually led to a different taxonomy. Basically, it impacts a whole different cell set. The 17 family seems to generate a distinct type of T helper cell (Th17), while IL-25 doesn't.

IL-25 does seem to have something to do with the daily problems I experience, however, because of its relation to the inflammatory processes of the body. It causes inflammation in the skin tissues as well as in the airways of the lungs. It can exacerbate asthma symptoms. In my disease experience, the inflammation of my airways has been pretty inexplicable. The antibodies aren't there to produce the problems... perhaps it is my cytokines like IL-25 instead? Hard to say.

I discussed this with my doctor yesterday and he agreed that there is a strong possibility that the allergies shown in people with immune deficiencies could well be something related to IL-25. We already know that hyper IgE syndrome (another PIDD) is a cytokine-mediated problem. Basically, the cytokines don't regulate the IgE properly. We don't really understand much of this yet, but it's super fascinating.

Happy 8th Anniversary

I realized yesterday I've been on IVIG for 8 years.

The math associated works out to over 100 IVIG treatments.

For the sake of argument we'll go with 1000 donors per treatment.

That means I am sitting here typing today because of the generosity of over 100,000 healthy individuals. If you take the higher donors-per-dosage estimates, it could be as many as a million. It's staggering to think that my life is so dependent on so many people and I could not be more grateful for everything they've enabled me to do.

So, happy anniversary, plasma donors! I sincerely couldn't have done it without every single one of you.

Sharing is Caring

I am waxing a little philosophical today because it is just after my 8th anniversary of diagnosis and IVIG treatment beginning. My oh my how the world has changed since then.

I can remember googling about immunoglobulins after I was first told I was low in all the types. I really didn't feel like I had leukemia, but that was pretty much the only thing I could find other than medical articles I didn't really understand. Then the doctor told me the terms "hypogammaglobulinema"and "Common Variable Immune Deficiency" and my life changed. Still not much came up on my google searches, but I had a much more specific set of terms to wrap my mind around. Social media was still in its infant stages when I was diagnosed, so I really felt like I was a freak of nature because I was convinced that everything was on the internet.

I was wrong.

Now, when you seek information about primary immune deficiencies, you find the website of the Immune Deficiency Foundation, along with some medical websites, like those from the Mayo clinic. It wasn't until 2008, on an additional search, that I found the IDF and found that there were more people like me. I was flabbergasted at the community and information available. I was ready to take ownership of my disease and was finally able to access the information to do so.

Fast forward to this past weekend. I got a tweet from someone who'd seen an exchange I had with @QueenofSpain about IVIG. I was giving her tips on mitigating side effects, etc, as she has started high dose IVIG therapy for her lupus. The woman who tweeted me somehow found her needle in the haystack. She'd been looking for a real, live person who could talk to her about PIDDs. This, kids, is why I write this blog (even if no one reads it), why I help administer a group on Facebook, and why I tweet openly about my disease. My experience can actually help other people. It's an amazing feeling that I'm able to make people feel far less isolated than I did.

It's been an amazing journey for me to get to this point, and I am hopeful that there will be a continued growth in our online community and continued sharing of information so that we become a more empowered patient group. That's my end goal, really, so we can all get the best care possible. Maybe, someday, there will even be better and more treatment options. A girl can dream.

Medical Teams

I am interrupting my posts on nerdily educational things to talk about something that is crucial for folks who have chronic illnesses: doctors. Medical professionals can make a huge impact on the trajectory of your disease and your prognosis. In my experience, if you like your doctor, you will comply more readily with their orders. Or if you think they are competent. This is not the case with all doctors, even well-respected doctors at well-respected clinics. They can sometimes think they're so good and always right it can be difficult for patients to get a word in edgewise.

I am very very fortunate among those in the PIDD community. I have a great team of doctors that work together for my health. They listen to me and to one another. The ENT calls the immuno, the immuno talks to the rheumatologist, the GI, and so on, and they all send results to my primary doctor. This, unfortunately, is a dream for most patients.

Complicated patients need a sophisticated team that can communicate. If this team doesn't work together, there's a chance something will be missed or major complications could arise. It's a very scary prospect. It's painful to see friends know they need help and not be able to find it. It's scary that they know more about what to do than their doctors and the doctors won't listen or decide not to intervene. It's a little shocking how close-minded some physicians are and how much hubris they can posses. Inevitably, the chronically-ill patient will know more about their body than you do, doctor, so take a moment to listen to them. It may save their life.

Cytokines

Once a month, I receive an email with the latest and greatest from the Journal of Allergy and Clinical Immunology. I like to keep up on developments and the articles usually have some useful information or send me down a useful path of research to increase my understanding of PIDDs, CIVD, and the immune system in general.

This month's journal included some research published about the cytokine now known as IL-25 (formerly IL-17E). Basically, it was found that the body produced this particular cytokine as a reaction to allergens, so it plays a role in allergic inflammation. Interesting. I hadn't previously read a great deal about cytokines, so down the rabbit hole I went.

I read several abstracts and settled on a few articles I thought were interesting, mainly focusing my work on things related to IL-25. I quickly saw that this was a broader and more interesting topic, so I expanded my search. I found this article and have been engrossed in the knowledge of each cytokine. This has expanded somewhat since 2009 when the article was published, but I'm finding it to be an interesting read.

Here are the basics of what I've learned about cytokines so far:
-Cytokines seem to be the immunological equivalent of hormones in the endocrine system.
-Researchers don't actually know much that is conclusive about these molecules.
-There are several that are responsible for B cell maturation and plasma cell differentiation.
-There are several cytokines that underlie allergic reactions and conditions - it's not just IgE, which I'd previously been lead to believe and is, seemingly, a pretty common misconception among physicians.
-Cytokines seem to be responsible at some base level for every function in the immune system.

There are several cytokines I want to talk about individually, but this is a good place to start. I will be focusing on the cytokines relevant to immunoglobulin production and autoimmune disease functions.

On Grocery Shopping

I love grocery shopping. I love the reading the labels, doing the math to comparison shop, finding the best products at reasonable prices, smelling the produce... I love it all. Truth be told, it exhausts me thoroughly. I must plan an entire evening (or afternoon on the weekend) to do simple weekly-type grocery shopping.

Wow, writing that down makes it sound pitiful.

I do it because I like to think about what I eat and what fuels my body every day. Also, because I haven't really figured out a way to avoid it and still get the products I want and need. I always have a list in hand (I love the meal planning and list generation on the Allrecipes Menu Planner.) and try to shop in a strategic way by grouping items by general location in the store, with dairy and frozen items going last if possible.

This obviously mitigates some of the exhaustion of having to walk back and forth up and down trying to find things. I also find it important to think logically about where things might be before I go on a massive aisle-by-aisle search for that one missing item. I've also learned to just give up. Scouring the 3 different possible locations for ricotta cheese sometimes just isn't worth it.

I think some of my attachment to grocery shopping came from watching Supermarket Sweep. I seriously love it as an activity, but it's one of the saddest for me because it is so tiring.

New IG Product Seeking FDA License

Baxter, makers of my beloved gammagard, are applying to bring their new SubQ preparation to market. It's called HyQ and you can read the full press release here.

Basically, this product is meant to infuse your entire month's worth of IG replacement subcutaneously. This is a fast-absorbing product using hyaluronidase to make the tissue more receptive to absorbing the product. The lack of good information on the effects of hyaluronidase, especially in this sort of long-term usage, concerns me. I don't know that I would want to switch to this product, especially before we get more in-patient, widespread information. I see the problems people are having with the change from Vivaglobin to Hizentra, and I do not want to be on the vanguard with this product.

I wonder if anyone has actually put thought into the fact that this would amount to not only an experiment on the value of subcutaneous preparations in monthly dosing but also on the long term impacts of putting this kind of tissue-loosening agent into your skin. I would imagine it would reduce some of the scar tissue problems I know patients experience with current preparations, but at what cost? I suppose we'll see if/when the FDA decides on the license.

Sick People Don't Pay Well

Over the past several days, I've realized that I really enjoy helping other patients navigate complicated situations, managing their care, and finding solutions to insurance difficulties. I enjoy this because I know personally how valuable this is. I would love to do this professionally, but the problem is: sick people often can't afford their medication, how would they afford someone to help them manage their situations? They really can't.

I think medical social work would be the way to approach this problem and be in a situation where I could reach and assist more people. I am not in a situation to go back to school. As mopey as I know that sounds, it's just a fact of life. I am not independently wealthy and must work to provide myself the insurance I need. Many school policies absolutely suck, so going on one of those really isn't a good option for me. Some would suggest I work and go to school. These people are nuts and do not live with chronic illness. I can barely buy groceries for myself and make dinner in the same night after work. I really don't think I can manage the added stress and scheduling difficulties that going to school while working would require.

So my desire to do these things plays out in online support groups. I do research, I offer advice, and give what assistance I can. It is fulfilling in a lot of ways, but it can also be very difficult. Sometimes people hit a nerve with me that makes it challenging to maintain my own sense of calm and decorum. Sometimes, they hit nerves that strike at things that hurt me outside the illness realm. It's hard to remember sometimes and create reasonable boundaries for myself. It's especially difficult when you understand what people are going through, but can't appropriately express it. It's even worse when you feel like you've become the bad guy in the situation. I know it may not make much sense, but sometimes it is easy to find fault within yourself for the way you've handled a situation. We all do the best we can.

Kudos Connecticut!

Yay for Connecticut deciding that it is good for workers to be able to take paid sick leave when they need to! Hopefully, this will mean fewer sick people come in to work unnecessarily, at least in theory.

Connecticut is the first state to mandate sick leave for all workers. Granted, it is only 1 hour of paid leave for every 40 hours worked, but it is a start. It is kind of stunning to me that this kind of thing needs to be legislated, but it definitely does. I see people on the metro all the time who are tremendously ill and dressed for their jobs in service professions. Think for a moment about the ramifications of that. These people are cleaning your offices, preparing your food, caring for the (also) sick... To think that they do these things while ill would make them less effective. Business just cares that the work gets done, not about the people who do it.

I am also saddened that it has taken this long and that business leaders are worried that it will make Connecticut less competitive. I'm sorry business leaders, but this is a matter of public health. One very sick person on public transit or in an office building or sneezing on salads could potentially sicken hundreds. What is so bad about making the employers that gain from their work also protect these workers (along with other employees)? Paid sick leave should not be treated as a fringe benefit, especially among those who are most impacted by this legislation.

Theories!

Ok, so yeah, I'm a little given to reading information about medical stuff. A friend mentioned she's having some compliment testing to track some autoimmune problems and I decided to do some research.

Compliment testing seems like it would make sense for me, since I'm having some autoimmune problems myself. After doing a little reading, a compliment problem may make sense for why I experience some of the things I do.

One of the grosser and more embarrassing things I deal with more than most are fungal skin infections. I have an active one currently, in fact. EEEW GROSS! Yeah, I know. It's unpleasant, but at least you aren't the one dealing with it.

Maybe this all ties in to the regular PIDD stuff, but maybe I should have some more testing done... Throw in some compliment testing with my T Cell testing? Hmmm?

Side Effects

Wow. So I started a new drug I've never had before last night. Um. Yeah. These side effects are not fun.

Side effects are not usually a picnic, but I will troop right on through them. I'll do that with these, but yikes. Maybe they'll subside after taking the medicine for a couple days, but the label indicates that symptoms could last for a month after ceasing the drug.

There are lots of things that people with chronic illness put up with, and medication side effects are a big one. This med makes you sleepy, this one keeps you awake, that one makes you fat, that one makes you want to tear your eyes out, this one will keep you in the bathroom until the rapture. Every medication is different and they all seem to have their own attendant problems. Some more than others, I'll admit, but sometimes you're on enough of them to make it seem like it would be better to just be sick. Luckily, I'm not yet to that point.

Most healthy people just switch medications when there are unpleasant side effects. I usually just suffer through, stiff upper lip style, because I know there are limited options and that the options grow increasingly limited as bacteria builds resistance.

I just wish I didn't feel so awful right now.

I don't know how you do it...

This blog post served as an inspiration and rallying call for me on what's been a tough morning in a series of tough mornings.

The IDF Conference tends to make me quite introspective and thoughtful about my (this is the first time I've actually typed these words and I just might cry) disability. Yes, it's a disability. Yes, it's difficult. It can be overwhelming and lonely, but I try my best to not let either thing get me down.

It IS a lonely disorder, because even at a conference full of people who have problems like yours, no one's problems are the same. Some are better, some are worse. While people are around that "speak your language," it's hard because you're still the only one fluent in "you." I know that is a difficult thing for most people to understand, and it's a difficult thing to explain. There's a desolation in knowing that you're the only one.

A dear friend has a colon issue and has yet to find anyone in the world with the same dysfunction. Expert physicians have heard of it, but no one can say they've actually seen a patient with the problem. That is lonely. Not a single person. This is not just presentation, this is entire disease diagnosis that she can't find anyone to share with. That's even more lonely.

But what does all this mean for daily living?

With rare orphan diseases, people are often sympathetic to what you're going through. I often hear "Well, I shouldn't be complaining to YOU about xyz illness thing I have going on. You have enough stuff to deal with." I do, but it's nice to know that other people get sick too. Granted, it's on a whole different scale, but sometimes it's nice to know that other people feel like crap.

I like to know that my experiences can help someone else, so I am happy to offer advice on OTC cold medication or explain the possible side effects of antibiotics. It makes me feel more like a whole person and makes my experience a lot less lonely. I may never get the sense that I feel normal or like everyone else, but, then again, who feels that way?

I am happier now than I've ever been, even with the struggles and challenges of illness. It's not easy, but there is a lot to be said for getting through. I do it because I still can, gosh darn it. I keep putting one foot in front of the other because it feels good to have a victory, however small it might be.

Numbers

As I mentioned, I went to several sessions at the IDF conference related to SCID and the recent initiatives to make SCID testing a regular part of infant heel prick screening. In these sessions, I learned about outcomes and prognosis for children who get diagnosed early. Those outcomes are great. There is a lot that can be repaired with Bone Marrow Transplant.

Then you look at older children at the time of transplantation and the outcomes are rather more bleak. This is sad to me because it would take only a little to get these children tested and treated early.

I read today about this little boy in India. His parents are struggling to get him a transplant at the age of 2.

I really hope he is able to get the transplant from his sister and beat the overwhelming odds against him.

Genetics, Confusion, and Me

File this under TIL at the conference...

I went to several sessions discussing both Severe Combined Immune Deficiency and genetics but I really didn't think I would have the same kind of revelations I have had.

At the last conference, I learned that my having a primary immune deficiency isn't quite as random as I'd been led to believe. My grandfather passed away from complications resulting from Scleroderma, an autoimmune disease. My father has B12 neuropathy, more than likely related to an autoimmune dysfunction. These are connected in an interesting way. They all are connected via the T-helper cells and are basically sides of the same coin. It was as though a light went on in my head and I finally understood.

I found out through further research that my grandfather's family had a history of such diseases. My great grandmother wrote of symptoms that sounded like autoimmune problems in her letters to my grandfather. Also, such autoimmune conditions are more common among the Amish. My grandfather left the Amish and took the gene malfunctions with him.

Sitting in several genetics lessons, I had been told several times that CVID is not really connected to any particular gene. There are several that are related, the TACI gene being the most recently discovered one, but there is no clear path for genetic testing.

Then I went to a genetic counseling session. One of the mothers in the session pulled me aside after to tell me that the RAG dysfunction genes run in the Amish and have been shown to relate to B Cell disregulation, similar to what happened in my family with the autoimmune problems and my PIDD. It has specifically tied to SCID.

Uh. What? The next step will be to discuss this stuff with my immunologist at my next appointment. I am not terribly sure about how I feel with this stuff. I am going to have my T cell function checked. There is a distinct possibility that I could actually have a SCID variant rather than CVID. Wouldn't that be interesting?

Importantly, though, is the information this gives me and the genetic testing possibilities for my siblings and cousins. There is the chance that they could pass such a dysfunction on to their children. We'll have to trace it up and out to figure that out, but it certainly concerns me for my future children. It's a tough thing to learn, but would be a good thing to know.

Indigestion

I thought I would post more about the conference and the wonderful things I learned. Well, I had the best intentions, but it just didn't work out that way and I wondered if there was some greater reason.

I was hoping it may have something to do with the fact that I've been stressed by external issues, and I really wish I could blame that. I feel strongly that there's something deeper at work. I just feel like I have a form of brain indigestion.

Uh. What?

Well, I think there has just been too much information uploaded to really be able to distill the valuable things yet. I am trying not to be too hard on myself, but that is a really challenging thing to do. It is difficult to explain properly. My mind feels like a brita pitcher. the top is full, and is slowly dripping down to give me the information that is most useful to me. I am working on it and will post about things I learned, but right now, it's just a little overwhelming. I know I learned things that were a relief as well as things that are a little scary. Eventually, I will share them with you. I promise.