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?