A Long Slow Slide

Well, for the last 6 months I have been more sick than I have ever been in my whole life. I began IVIG treatments here, in New York, in early February. Since then, I have had 5 colds and 4 bouts of stomach virus/food poisoning. Right now, my colon is twisting itself in knots…I have horrendous gastrointestinal issues. The immunoglobulin doesn’t seem to be doing much good. Granted, everyone else I know has been quite ill this winter/spring and they all have healthy immune systems. But I feel like my GI tract is really malfunctioning.

Now I don’t produce any IgA, the most common protective antibody for the colon, so I guess this is to be expected…but it really sucks.

My health really seems to be in a serious state of decline. I have an appointment scheduled this week with my immunologist to discuss diagnostics and next steps. I will let you know what she says. I’m afraid I may have colitis.

 I am having a hard time dealing with this decline. I have an image of myself as a rugged outdoorsman, backpacker, camper, swimmer, etc. Now I feel like that life is permanently over. I don’t ever forsee being healthy enough to even go to the gym. I’m even thinking about quiting my job, I just don’t have the energy anymore.

I watch my 15 month-old daughter scamper around the house and I doubt that I will ever be able to coach her softball team, teach her to ride a bike, show her how to pitch a tent.

I’m only 44 but I sit here with no answers, with no success, waiting out a long slow slide.

Into what?

There is nothing attractive about that future.

Ch Ch Ch Changes…

Well, I made it to New York. Traded in my 1200 sq. ft. apartment on the west coast for a 600 sq. ft. hatbox in Brooklyn. Lots of changes. It has been four months since my last IVIG. I had to find a new immunologist; it took a long time to get approval from my new insurance company; I was given a crappy project at my new job that required working 14 hour days and weekends for 3 months and it just ended… I finally got a really bad cold and norovirus all at the same time.

My wife tied me to the bed and told me I wasn’t leaving the house until I was 100% better and had an infusion appointment. I go in Thursday, I hope they have wireless internet…

The Four Types of Normal Immune Responses

this video susinctly shows the four types of immune responses. I would love to see a similar animation demonstrating autoimmune responses.

Healthy Antibodies at Work

I just found this spectacular animation that shows how the immune system should ideally work. It’s short and follows an antigen from its introduction into the blood stream to its final demise.

When does Idiopathic = Autoimmune

From a recent comment:

“I was diagnosed with ITP – Ideopathic Thrombocytopenia Purpura in December 2005 and then CVID in October 2006. I’ve been on IVIG for 12 months (started out 5 days per month – now it is down to 3). My trouble is the ITP which causes the platelets to destroy themselves – and in between IVIG I must still take steroids. My hope is to get rid of the ITP and of course if I didn’t have CVID they would have removed my spleen.”

It seems that a number of conditions that are labeled “idiopathic” are actually thought to be related to autoimmunity. It seems likely that the guest above has antibodies to her platelets. The antibodies wrongly destroy the platelets and thus make it difficult for her body to stop bleeding. My immunologist is reluctant to make a diagnosis of autoimmune urticarial (hives). This is frustrating because I have be getting them every day now for over a year. I get the sense that a diagnosis of autoimmunity is considered by her to be a diagnosis of exclusion. And there does seem to be a test to determine if I have an autoimmune response. I’m not sure why all the reticence to declare a condition autoimmune. Perhaps the tests are of questionable validity, perhaps the options for treatments are relatively ineffective or maybe doctors want to look at every other possible option first.

The question is how many of us who are diagnosed with idiopathic conditions actually have wonky immune systems.

Teenage Immuno Ninja Turtles

Check out this hysterical video that used real life versions of the Teenage Mutant Ninja Turtles to describe immunodeficiency. It takes a couple of minutes to get to the good parts but it’s worth it.

Visiting the Ass Man

For those of you who don’t understand the Seinfeld reference in this posting’s title, I visited the gastroenterologist today. I was referred by my immunologist. I had had three successful IVIG infusions and it was time to figure out why I was still experiencing all these crazy symptoms. The best place to start, she asserted, was with the colon. People with CVID often have issues with gastrointestinal infection, inflammation, etc. because they often don’t produce IgA (I know I sure don’t). IgA are the antibodies that primarily protect the colon from infections. She believes that something may be going on in my colon that is stressing my immune system.

So, off I went to see if we could start to determine why I have chronic hives and now arthritis. The doctor said that a CT scan showed that I had enlarged lymph nodes in the fat around my colon and it looked like this tissue was inflamed. He doesn’t think that he will find anything related to my hives but is still going to do a colonoscopy of my small intestine to make sure. I asked if there might be any infections that would cause hives and he was doubtful.

Once again, I have no real answers about the cause of my hives but I will keep trying. I don’t really have a choice.

The Idiopath abides…

How to Live your Life with CVID

For the past 6 months I have been freaked out about my diagnosis. I was really sick in March; I came down with a really bad lung infection secondary to a cold that I had caught from some family members. I developed swollen, painful nodes, my hives went into overdrive, I was dizzy all the time and my knees, elbows and hip joints began to swell and get painful. Additionally, I developed acute pain under my left rib cage. I felt like my body had just decided to fall apart.

In the meantime, I was getting bored with my job. I had held the same position for almost four years. I was feeling unchallenged, under appreciated, micromanaged and generally unhappy. My wife implored me to find something new. “Quit and I’ll be your sugar mama until you find a new job,” she would regularly say.

But I had great insurance and was not in the greatest health. I had just begun successful IVIG treatments, at $10,000 a pop that stuff ain’t cheap. Also, I was having CAT scans, expensive blood tests, regular visits to the emergency room. I felt like my health was a huge question mark and I didn’t want a change in insurance to suddenly cost me exorbitant amounts out of pocket. Additionally, I had great long term disability insurance. If I need to be out for extended periods receiving chemo for lymphoma or colon cancer or whatever, I would be well taken care of.

But as the docs look at me (and keep looking) they don’t find anything worrisome about my condition. I have no signs of lymphoma. I have no symptoms that could be construed as some sort of serious autoimmune disorder like Sjogren’s or Lupus or MS. I have your basic run-of-the-mill CVID issues…

and I have always had a dream — to live and work in New York City.

The doctors are still trying to find out why my hives are so bad and why my gut hurts so much (they think the two symptoms may be related) and there is some streaking in the fat that surrounds my colon. I am scheduled for a colonoscopy later next week to see if I have any serious gastrointestinal issues.

I just gave notice today at work, I will be starting a new job, a more interesting job in a great area, with really interesting people in 4 weeks. The insurance isn’t quite as good: there is a bigger copay on drugs, I have to pay more for my wife and kid. But it’s in SoHo.

I don’t know if something is seriously wrong with me, it seems always right around the corner. But when I walk down the street today I keep my eyes straight ahead. There is plenty to see in Manhattan right in front of you.

Clinical Trial for New CVID Drug

There is an ongoing phase II drug trial for people with CVID who have bowel issues. It sounds like it may be promising for the treatment of other autoimmune diseases. Check it out and see if it is right for you. Let’s hope it is effective.

Infusion-related Vein Collapse

Now that I know I am signing up for the IVIG long-haul, I have been thinking about the inevitability of vein collapse. Up until my CVID diagnosis last winter, I had always though that collapsed veins were limited to junkies and chemotherapy patients. They occasionally made it difficult for the executioner to find a good place to inject the condemned. They happened to others.

My sister was diagnosed with breast cancer about four years ago and was put on an aggressive and lengthy chemo regimen. Her veins collapsed one by one and she had to get an IV port implanted. The port allowed drugs to be infused close to her heart. The very idea gave me the creeps. I couldn’t imagine having a permanent opening into one of my veins. I had nightmares that someone would try to inject me with Pepsi or whiskey or Drano while I was sleeping. Of course if they were going to do that, a port wouldn’t really make it any easier than doing it with a plain old syringe! It just seemed weird and somehow dangerous.

Now that I have two uneventful infusions under my belt, it looks like I will need one every month for the rest of my life. Over time, losing the use of veins in my arms and legs is no longer a possibility, it is a virtual certainty. Certainly the primary immunodeficiency forums are replete with reports of this problem.

There are several options available to me:

• Subcutaneous immunoglobulin infusions
• A standard IV port
• A port-a-cath

I will weigh the pros and cons over the next couple of years. Hopefully, I won’t need to make a decision for a long time.