Archive for August, 2007

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.

August 30, 2007 at 8:50 pm 1 comment

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:

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.

August 29, 2007 at 8:28 pm Leave a comment

Orphan Drug or Ineffective Molecule?

That is the big question. Does anyone know what happened to B Lymphocyte Stimulator (BLyS)? It seemed to be a promising treatment for CVID and other immunodeficiencies but then disappeared after phase 2 trials a couple of years ago. Was it not effective, did it have horrible side effects, was it not profitable or was it simply dropped so that Human Genome Sciences could spend valuable resources elsewhere? It’s frustrating to know that a treatment for my affliction is out there somewhere and I can’t get it.

August 28, 2007 at 8:38 pm 1 comment

What is IgA and Why Don’t I Like It?

So, I have mentioned below that my body is intolerant of IgA. IgA is an antibody that protects the mucous membranes in the nose, mouth and digestive tract. Apparently, a small subset of CVID patients have this condition. It is characterized by a complete lack of IgA production by the immune system and concurent production of large amounts of anti-IgA antibodies. Yes, that is right folks, I have been endowed with the ability to create antibodies to my antibodies. The anti-IgA actually destroys any IgA that enters my body. Therefore, when I had my first IVIG infusion, my body attacked the small amount of IgA that was present in the Ig solution (most of it is IgG) and created the unpleasant reaction I recount below. Who knew this could happen? I guess it makes sense since antibodies are proteins and you can theoretically be allergic to any protein.

Fortunately, there are IgA depleted IVIG solutions and I was able to find one that didn’t plunge me into anaphylaxis.

August 27, 2007 at 8:23 pm 1 comment

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.

August 26, 2007 at 7:56 pm 1 comment

Other CVID Bloggers

Check out this post from a woman living with CVID in Missouri.

August 10, 2007 at 1:01 pm Leave a comment

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.

August 10, 2007 at 11:35 am 2 comments


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