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…

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.

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.

My CT Scan Results or Son of The Blob

I had an abdominal CT scan on Tuesday and the results just came back. I have a “borderline spleen” according to my immunologist. No, it isn’t a DSM III diagnosis, it means that my spleen is larger than normal but not large enough that it needs to be removed or treated. I am concerned because this began around the same time that I started IV immunoglobulin treatments. I am wondering if there is a causal relationship. My doctor says she has not heard any reports of IVIG being related to splenomegaly but I have read forum posts of patients who have experienced the same thing after their infusions.

I was also told that I have enlarged nodes near my colon.

My doctor says that there is nothing to worry about, there is no sign of lymphoma or tumors. We will need to monitor my spleen and the colon nodes over the next few months. Meanwhile the pressure under my ribcage increases, no sit ups, no running, no interactive sports…I worry.

Visual Representation of the Immune System

I found this very useful diagram that outlines the immunoglobulin producing immune system in humans and discusses what intravenous immunoglobulin is.

Possible Medicine to Treat Severe Autoimmune Attacks in People with CVID?

I have been wondering for a while whether rituximab, a drug that is approved for the treatment of rheumatoid arthritis would work to suppress other severe forms of autoimmune disease in people with CVID. Rituxumab works by essentially scrubbing the blood clean of malfunctioning B cells that create autoantibodies, the stuff in your blood that attacks your body in an autoimmune response. New, normally functioning B cells are produced by the bone marrow and repopulate the blood, thus reducing autoimmune attacks. It’s kind of like pressing reset on a certain part of your immune system. This treatment has been tried sporadically with very ill patients who experience poor responses to more standard treatments like antihistamines, IVIG and steroids. One study shows that it worked well for a patient with a severe autoimmune response to his blood platelets. Another study found that patients with autoimmune responses to their own red blood cells had good outcomes from rituximab treatment.

A couple of words of caution in interpreting these studies:

1. These are case studies and their findings have not necessarily been corroborated by other broader studies or clinical trials. These findings could be flukes.

2. Rituxumab suppresses the immune system. Obviously, taking it may be risky for people with  already compromised immune systems. Of course, it only suppresses B cells which don’t work very well in people with CVID anyway.

None-the-less, I wonder if this could be a possible future treatment for CVID patients with a variety of stubborn chronic autoimmune issues.

My First IVIG Experience

It was really a very common procedure; they had never had a problem in the past. My doctors assured me that they would start the infusion slowly to see how my body responded, then they would up the drip rate until it was at full throttle. The first time would take 6 or 7 hours but they would probably be able to get it down to 3 or 4 in a few months. I got to the infusion center 45 minutes early, my Eddie Bauer backpack filled with books, magazines, sudoku, writing materials. I had pushed aside my nervousness and tried to imagine I was at a spa, time to myself with no interuptions…granted, I would have a huge tube feeding into my arm. I was told there was no wireless internet and there were cute little hand-written signs all over the place letting me know that I wasn’t to use my trusty Blackberry (As though cell phones were some newfangled inventions and its capacity for annoyance had just been discovered.) I was to be completely incommunicado from work. that freaked me out a bit, to be going through withdrawal while having my infusion. Everyone at work knew that I had mysteriously annouced several weeks earlier that I would be out today for “personal reasons.” People asked me if everything was alright. I dismissed them with vague references to matters that I had to attend to, rolling my eyes as though I were mediating a battle between quarrelsome relatives. 

I sat in the waiting room for two hours. It was clear that as a newcomer I was at the bottom of the liquid medicine dispensary totem pole. People who arrived after me and who had appointments after me we escorted in with first name greeting and hugs. The nurses all know their stories. Granted most of them had no hair and probably had less that 6 moths to live but an appointment is an appointment damn it. If they want to let the hospice folks in first then don’t invite the primary immunodeficiency newbies in until after noon. The really sick wore their colors as hats or scarves on their hairless heads. These colors like those of gang members at the local nightclub gaining them entree into vip rooms and past the scrub green bouncers. I awaited, hirsuit, with a less than impressive diagnosis credentials.

 My IVIG infusion began uneventfully and I hunkered in to my adjustable hospital bed, completely absorbing myself in back issues of Bon Appetit…

20 minutes into it:

My lungs start to get tight, my respiration becomes shallow and rapid, maybe I just have a touch of asthma, maybe I am just tense, what did I have for breakfast, my head is feeling hot, flush, I can’t really breath…

“Um, excuse me, I think I am having a reaction.”

My head feels like it is being crushed in a vice, “your face is really red”, I can’t breath at all now,

Cut the IV, flush, benadryl 50mg, 100mg Prednisone, call Dr. _______, he’s accutely hypertensive, wait, not hypo, no HYPER,

Lungs begin to release, slowly, more air, panic subsides, doctors hand rubbing my foot, comforting, I am feeling better, the pressure in my head goes away, that was freaky.

They must have the AC on,  get under the blankets, still too cold, I’m feeling nauseaus, excuse me, can I get an extra blanket and something to puke in, shiver, shiver, shiver, shake, uncontrolable shaking, jaws clenched, I’m in a meet freezer…

Page Dr. _______, he has the rigors, do you feel like you have the rigors, whhhhaaaat the hehehehehell is thththtatttt? 50mg Demerol, you are going to get very sleepy…warm, sleepy…

I walked out 2 hours later after sleeping off the Demerol. I had only received 20 mg of the 500mg I was supposed to get. The doctor would  call me about next steps.