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.

Getting Worse

So, I have been experiencing a number of symptoms lately related to my chronic urticaria, I believe, and they seem to be getting worse. Here is a list:

a) I have been taking Zyrtec for my hives. Up until last month, I only needed to take 10 mg. once every 2-3 days to control outbreaks. Now, I am taking the maximum dosage, 10 mg. every 12 hours and I am still having pretty serious outbreaks. Nothing that has necessitated the use of my epi pen yet, but the trajectory is toward less Zyrtec effectiveness. I now supplement with 25 mg. Benadryl and occasionally a Singulair or two.

b) I have several lymph nodes in my underarm, near my sternum and across my chest that are enlarged and painful.

c) I have periodic drenching night sweats

d) My hip joints are inflamed and it has become painful to walk.

e) I have increasing pressure under my lower left ribcage. I am afraid that my spleen is taking over my abdominal cavity.

 I think my immune system is in hyper-drive. I’m not sure if this is related to my recent IVIG infusions. There seems to be a rough correlation between when the infusions started and when these symptoms began. I am wondering if the residual IgA in the Gammagard SD that may be increasing the amount of anti-IgA antibodies in my system. I have a doctor appointment on Thursday. We’ll see what she says.

Using the Internet for Medical Research, Part 1

A reader writes:

“The biggest thing I can stress to you is to educate yourself wisely about this illness – DO NOT SPEND TOO MUCH TIME ON THE INTERNET. IT WILL SCARE YOU TO DEATH AND ADD TO YOUR STRESS LEVEL.”

One of the biggest questions I ask myself on a daily basis is “how useful is the Internet for medical research?” I use the Internet almost every day to do research on Common Variable Immunodeficiency (CVID) and Chronic Urticaria. But a number of issues arise when one uses this fabulous global repository of information for research on idiopathic conditions. I will attempt to address some of these issues over the next week.

Issue #1: Does it really help to know everything you possibly can about your condition?

I tend to perseverate on the absolutely worst possible things that can happen to me with respect to any disease or symptom I have:  fevers often become West Nile Virus, a rash is Lyme disease, headaches… brain tumors. I know that there is always a broad variety of symptoms and disease trajectories for every possible illness, but I always assume the worst. I guess I figure that I can only be happily surprised if the worst doesn’t happen. And if the worst does happen? Hey, I can at least be smug in my hospice. 

Unfortunately yet inevitably, you will run across the most heinous potential outcomes for your particular issue. For CVID, I have a 50-fold increased risk of colorectal cancer (CRC) and 23-fold increased chance of lymphoma over the population as a whole. However, my doctor says that of the 75-100 patients with CVID that she has treated, none has had CRC and only a couple have progressed to lymphoma. Also, the literature is replete with references to a ghastly condition called splenomegaly, a condition where your spleen essentially turns into The Blob and begins to take over your entire body. There is absolutely nothing I can do to prevent this condition, so the benefit of losing nights of sleep worrying about possibly waking up someday with a giant spleen filling my gut is questionable. Certainly, focusing on the array of possible outcomes for a syndrome like CVID is not the healthiest thing for me right now.

I do focus on the broad range of potential treatments. I have been investigating enzyme therapy to assist my colon in digesting proteins better before they enter my bloodstream, reducing the amount of histamines in my diet, and even taking broad spectrum antibiotics to suppress possible h. pylori infection. Searching for a treatment gives me a purpose; it makes me feel like I am accomplishing something.

The vast and easily available information on the web fuels me every day to continue my journey down the idiopath. I try not to let it paralyze me with fear or dishearten me.  I look on the web because no one has yet provided me with any information that helps alleviate my symptoms. No one, no doctor, naturopath, parent, friend, acquaintance, or stranger. And since I am not willing to accept that I have to live with these accursed hives the rest of my life, I will use the Internet as my main source of  information until I have an answer. But the reader’s point is well taken: be selective in what you look for, be selective in your sources, and only search for what will help you.

The complexity of the body

In reading about chronic hives over the past couple of weeks, I discovered that somewhere between 25-50% of all incidents are related to autoimmune responses. The body actually attacks itself. What baffled me was that I have a genetic immunodeficiency; how do I produce an unchecked immune response against myself if my body has a hard time suiting up against a bacterium?

The Primary Immunodeficiency Foundation  has produced a pamphlet that describes how this is possible. Essentially, the immune system is made up of multiple layers of defenses. I don’t produce any significant amounts of immunoglobulin (antibodies), but my immune system also consists of T-cells and B-cells that fight potentially dangerous invaders like bacteria and viruses. Apparently, these cells tend to proliferate when antibodies are in short supply. Some times, too many are produced and they can go a little haywire becoming sensitive to substances that they would normally ignore. In my case, it may be that my B and T cells may be attacking my skin cells. Autoimmune responses like this are not uncommon. Diseases such as MS, lupus and rheumatoid arthritis are all believed to be autoimmune responses.  When the body’s immune system is out of balance, it can overcompensate with the resources it has.

I may or may not have autoimmune hives (urticaria) but this example illustrates the complexity of the body. The search for answers to chronic, serious and idiopathic symptoms must always account for this complexity. Any symptom or set of symptoms can be the result of any number of competing or cooperating underlying causes. Symptoms may also be the result of biological processes that seem on the surface contradictory or logically unrelated to other diagnoses.

Recent studies have found that some autoimmune diseases may be the result of concurrent mutations in as many as 30 genes. Keep this in mind as you seek simple answers to your medical issues.  The idiopath is paved in shades of grey.

What the hell do I have now…

“… it’s quite rare, actually. It’s a condition that affects only about one in 50,000 people in the United States.” I was dizzy, trying to focus on my bloated midday email in-box, putting the finishing touches on a PowerPoint presentation, stuffing forkfuls of chow mein into my mouth and listening to my allergist suddenly inform me that I was afflicted with an incurable syndrome. “It’s called Common Variable Immunodeficiency(CVID). Basically, your body doesn’t produce immunoglobulin…at all.” I was stunned; I had weaseled my way into one of the top allergy clinics on the West Coast two weeks earlier because I had been having recurrent unexplainable hives . I thought I was allergic to shellfish or grapes or maybe dreaded sulfites. I wanted to get one of those magic skin tests where they prick your back about a thousand times and watch welts appear. “You have hypersensitivities to day boat scallops, red snapper and white zinfandel,” I expected her to say. Instead, she calmly, clinically informed me that it was actually impossible for me to have a true allergic reaction because I didn’t produce the necessary antibodies to begin histamine release. Instead of giving me prescriptions for some really great antihistamines and telling me to stay away from tree nuts and dairy, my doctor was giving me the news that I was born without half of my immune system.

It’s hard to describe how I felt, as my specialist listed the common symptoms and the diseases associated with CVID. I was somewhat relieved; finally, I had an explanation for almost every serious illness I had ever had, recurrent herpes, shingle, bacterial and mycoplasma pneumonia, chronic bronchitis, sinusitis, chronic irritable bowels, and swollen nodes in my neck. It was a nice, neat, objective, empirical diagnosis rubber stamped by the medical profession, it was a wrapper I could tie around the past 42 unhealthy years of my life and call my own. I felt a certain sense of serenity.  I was also freaked out. Was this a death sentence? Did I need to “get my affairs in order?” Did I need to take some sort of energy-sapping drug cocktail? Or was I destined to become a bubble man, living in a hermetically sealed environment the rest of my life?

And there was another nagging question, “why the hell do I have these hives?!” I mean, if I can’t be allergic to anything then why am I experiencing this insanely itchy rash over my entire body?

“It could be any number of things.” Ironically, according to my doctor, people with CVID also tend to have autoimmune disorders. The body actually attacks its own tissue. Or it could be some sort of pseudo-allergen that I have become sensitive to, like sulfites or MSG.

“We don’t really know; we will have to do some more investigating. Right now, you have what we call Chronic Idiopathic Urticaria.  Basically, this means I have unexplained hives. Thanks for the insightful diagnosis doc.

And thus begins my journey; I have been diagnosed with CVID for just over four weeks. I have had chronic, unrelenting hives over most of my body almost all the time for four months. I take every possible over the counter allergy medication at several times their recommended dosages. I itch like crazy, always. My pregnant wife, the saint that she is, cares for me the best that she can at four in the morning with my eyelids puffed almost completely shut and my incessant scratching shaking the bed for hours at a time. There may be answers out there but no one has them right now and no one cares about this as much as I do. I am my primary caregiver; I search day and night on the Internet, call my doctors and friends to solicit feedback about half-baked medical theories and to discuss obscure immunology studies. I live this, I dream this, I am obsessed.

But it’s the journey that my wife says is therapeutic and I think she is getting sick of my chronic worrying. If I didn’t keep searching for answers, I think I would go insane. So I will continue to look, no matter how long it takes, until I find some peace…

The Idiopath abides.