I Dreamed a Dream

I had a dream about a PI friend last night. He has had a long struggle trying to maintain some semblance of normalcy. We hadn't heard from him in some time, so a friend messaged his family to see if there were any developments in his condition. In my dream, we learned of his passing and it was all very sad. We sent flowers to the funeral and organized fundraisers in his memory. It was very vivid to me and left me feeling very uneasy about his condition in reality.

As it turns out, those fears have some basis. My friend heard from him today and found out he's been diagnosed with IPEX Syndrome. IPEX syndrome is often found much younger, so I'm very concerned for his prognosis. It is a defect in the T cells that leads to total chaos in the immune system. Immune deficiencies, autoimmune problems, ridiculous allergic reactions to almost every food. It makes sense based on his experience, but it's shocking to me that it wasn't figured out sooner.

So now my friend will prepare for the first line treatment for IPEX... a bone marrow transplant. He's very fortunate to have 2 matched donors and will undergo the treatment this fall. I'm thankful at this point for the developments in the transplants and the fact that even a partially successful graft will make a huge difference in his life. I just hope the rest of my dream does not become reality.

Reality Bites

I spent the past weekend in Baltimore at the IDF Conference (which I will get into in more detail later) and it was a good time to catch up with friends... at least for the most part. It always leaves me pretty run down, but this time it was an extra special dose of exhaustion.

On Saturday night, for the first night in about a week, I took my sleeping medication. I had a blissful night of sleep, about 10 hours. I woke up feeling pretty good... until I looked at my phone. Three missed calls from people who never call me (they usually text) and a flurry of texts. Uh oh. Come to find out, a friend had to go to the ER on the last evening of the conference. I'm actually surprised more folks don't have to go in, but she was the second (and I think last) that had to go, at least of the deficients I know. 

I get up at around 8:30, see all this stuff, hop in the shower, throw my things into my suitcase, then hurry over to the University of Maryland Emergency Department, where they were keeping my friend for observation. I'm really thankful I got over there when I did to help her explain things to doctors, etc, because it's really difficult to explain when benadryl has rendered you nearly unconscious and makes it impossible to focus. I was also able to use some of my lung knowledge gained by looking at scan slides to help explain to the docs what they were seeing with N's scans. That was good. 

I was certainly glad to be there to help her out, but it also set off some personal alarms with me. Perhaps my own fears of traveling alone are more warranted than I had previously thought. It's a little scary to see someone so close to you in age be in such need of assistance. I mean, we were absolutely 100% there to help her, but I wonder what would have happened if it had been me, alone, several hundred miles from home. As much as I would like to think I'm not limited by CVID, I really am in some very important ways. This demonstrates more than ever that I need a travel buddy always. I'm grateful that B was there on the other end to help me get my things out of the hotel and checked out and all of that, but I'm really trying to evaluate the plans I have in place and the ease with which my doctors can be contacted. It matters more than I would like.

Oh Crap. Alzheimers.

Let's start off by saying I do not begrudge other disease groups access to treatment. I don't wish for people to suffer.

Now that's out of the way, I am really frightened by the prospect of Alzheimer's patients receiving IVIG as a treatment. The therapeutic dosage I've seen online is something like twice as much as the PIDD dose every two weeks, instead of monthly. There are already rumors of shortages and talk that we may see the worst plasma situation since 2005. 2005 was scary. People couldn't get treatment. Doctors could not locate product. Not a good thing. This shortage scare is happening now with (I would approximate) 1 million people using the product, all of them rare diseases. This shortage is happening in an economic slump when there have historically been more plasma donations because people are strapped for cash.

Why else does this concern me? Alzheimer's is a "fashionable" disease. It has name recognition, celebrity boosters, etc. Any development with Alzheimer's is huge news around the world. There are also 5.4 million people with Alzheimer's in the United States. Using two-four times as much product. It is worrisome that the Alzheimer's lobby might get legal protections for their use of IVIG, which would trump mine. We have very few advocates, Alzheimer's has many very powerful ones.

My best hope is that they would develop a monoclonal antibody for Alzheimer's that would target the beta ameloid protein, but that is years of very challenging science down the road. 

IVIG is the only treatment for CVID. There is nothing else even in the pipeline to treat me.

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.

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.

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.

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.

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.