Herpes-related Colitis in Person with CVID

The association between herpes infection and complications in CVID has been a subject of study for some time. Here is an interesting abstract the found high levels of the common herpes virus in the colon of a woman with CVID and chronic colitis. Valaciclovir was given to her and her colitis cleared up.

Just one more sliver of hope for someone out there with serious bowel issues.

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.

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.

One Potential Genetic Cause of CVID

In 2005, researches in Boston found that one gene, TACI, may cause 15-20% of all cases of CVID. A National Institutes of Health press release states that this is a dominant gene and those who carry it may have a 50% chance of passing it to their children.

Will My Kids Get CVID?

If you have concerns about whether you will pass CVID to your children, read this great, easy-to-read primer on genetics and CVID inheritance.

 Key Points:

• Only 10-20% of people with CVID have genes that have been identified by researchers as contributing to the condition.
• Most people with CVID probably have multiple genetic defects which contribute to their symptoms and the probability of inheritance in difficult to predict.
• CVID may be related to multiple environmental factors as well.

According to the author, CVID is a “multifactorial” condition that may often involve multiple, interacting genetic and environmental factors. Thus, determining whether CVID will be inherited is very difficult.

Why Don’t Spleens Actually Have Vents?

It would really be helpful for those with the miserable condition known as splenomegaly, a condition in which one’s spleen dramatically increases in size. I have increasing pressure under my left ribcage, soreness in my sternum and left shoulder and suspect that I may be experiencing its initial symptoms.

What happens?
Basically, your spleen is a fist-sized organ under your left ribcage that processes blood. It removes impurities from your blood and creates white blood cells to help fight infections. When it malfunctions, it can do a couple of things. First, it can cause anemia by destroying too many red blood cells. Second, it can product too many white blood cells. These white infection-fighting cells either accumulate too quickly or don’t die when they are supposed to and keep accumulating in the spleen, thus greatly increasing its size. It can eventually press on neighboring organs, like the stomach and lungs. It can cause malaise, pain, a perpetual lack of hunger and if it really gets bad…it can burst. Bursting is really dangerous because you can develop internal bleeding that can kill you very quickly. If your spleen bursts, call 911 immediately!

Luckily, that rarely happens but you should take it seriously. And as my doctor says, “be aware but don’t worry until I tell you to worry.”

What does it mean?
Well, according to the literature I have read, approximately 30-45% of people with CVID have chronic splenomegaly. It’s fairly common. Most of the time it seems to be relatively benign…if you consider having The Blob living inside your abdomen benign. When it does have an identifiable cause, i.e., is not idiopathic, it is usually caused by an infection, viral, bacterial, fungal, whatever. If you can catch it, it can cause your immune system to freak out. A number of people are looking at various herpes viruses that seem to be strongly correlated with it. It can also be related to a systemic autoimmune response: if your body becomes allergic to itself, your spleen can produce the cells that attack you. Finally, it can mean you have a blood-based cancer, like lymphoma.

What can they do?
You only really have a few options. Generally, your doctor should try to determine if there is an underlying infection. If not, really bad cases may require the removal of the spleen. This is generally a last resort for people with CVID. The spleen is an integral part of the immune system and we are already have part of our immune system missing. Finally, if this condition is related to a blood-based cancer, they may choose to irradiate the spleen to shrink it and kill the cancerous cells.

I have a CT scan scheduled in two weeks. I’ll let you all know the verdict.

CVID-Related Clinical Trials

For those of you who are interested in advancing the pursuit of a cure for CVID, try going to clinicaltrials.gov. This is the governmental clearinghouse for all US clinical trials. Currently, the list of CVID-related trials includes the following:

• “Immune System and Gut Abnormalities in Patients With Common Variable Immunodeficiency With and Without Gastrointestinal Symptoms“
• “Immune Regulation in Patients With Common Variable Immunodeficiency and Related Syndromes“
• “Efficacy and Safety of Vivaglobin® in Previously Untreated Patients With Primary Immunodeficiency“
• “Molecular and Clinical Studies of Primary Immunodeficiency Diseases“

Slim pickings, I know, but the list changes all the time so check the site regularly. There is still a lot of basic science that needs to be done before we understand exactly why the immune system malfunctions in people with CVID, so if you have the inclination please participate.