Bring on the X-Wing Fighter - Attacking the Lymphocytes

As I wrote about in my last post, while, initially, we thought my thymus was the problem. (Sam says my thymus is like the emperor from Star Wars. The supreme ruler of the most powerful tyrannical regime the galaxy had ever witnessed.)

We now think my lymphocytes (a type of white blood cell responsible for defending the body) are confused and have started attacking my normal, healthy infection-fighting white blood cells (my neutrophils & monocytes). We are calling the attacking lymphocytes the Death Star.

So, how do we defend against the Death Star? Rebel Alliance spy, Dr. Lopez, believes something called immunomodulating treatments will neutralize the attacking space station. We're hoping these treatments will knock down my lymphocytes so my neutrophils and monocytes can grow.

Before they start the immunomodulating treatment, the anti-lymphocyte program, they want to do another bone marrow biopsy so they have a baseline and can make sure there are "no funny cells." (I'm not sure when they will do the bone marrow biopsy. He usually does them on Fridays so it could be as early as next Friday.)

As part of the treatment, I will receive an injection of some type of steroid. (We've already tried quite a bit of Prednisone and while the doctor noted that I responded to it somewhat, he said, I didn't respond well and my response wasn't permanent.) They want to use something more profound (his words).

A few days after they have been able to review the results of the upcoming bone marrow biopsy, they want to give me an infusion (an introduction of a solution into the body through a vein for therapeutic purposes) of something called ATG (antithymocyte globulin) for a few days.

I will also take a pill of something called Cyclosporin. I don't know much about these specific drugs or any potential side effects. Dr. Lopez says they have had good results using immunomodulating treatments. He said it usually really works for patients like me.

He said there are three possible outcomes. One is that everything will be better, meaning that after the treatment, I will have normal numbers. The second possible outcome is that things will be "better enough," meaning I won't have the numbers I had before I became ill but that they will be better than they are now. The third possibility is that the treatment does not change anything. He said having no response is the least common outcome.

Sam said the treatment was like Luke Skywalker in his X-Wing Fighter. With the aid of Han Solo, the spirit of Obi-Wan Kenobi, and the power of the Force, Luke Skywalker was able to successfully hit the small target, and triggered the Death Star's demise.

The Death Star - Defending Against My Lymphocytes

Yesterday, I had my second appointment with my third hematologist. Dr. Alfredo Lopez is a really likable guy. However, I have to admit that during the two months since my first appointment with him, there were times I was perplexed, annoyed, concerned and even angry with him.

Dr. Lopez kept telling me to just keep doing what I had been doing, from a treatment perspective. This didn't make sense to me since I was moderately to severely Neutropenic almost the entire time. (Neutropenia is what it is called when you don't have enough infection-fighting cells.)

I wanted to believe that it was alright not to worry, since he didn't appear worried, but I was afraid he just wasn't paying attention. I was afraid I would get sick with an infection I could not fight, become seriously ill and possibly even die because that's was what I kept reading (and have been told) could happen to someone who is severely Neutropenic.

Yesterday, Dr. Lopez explained that he and a team of hematological experts have reviewed my bone marrow. Apparently, all of them are in agreement about what it looks like. He said it looks like I have an immune mediated condition.

What does that mean? I'm still learning but according to Wikipedia, Immune-mediated diseases are conditions which result from abnormal activity of the body's immune system. The immune system may over-react or start attacking the body. Autoimmune diseases diseases are a subset of immune-mediated diseases.

(The idea that I might have an autoimmune disease has come up a few times. Autoimmunity is the failure of an organism to recognize its own constituent parts as self, which allows an immune response against its own cells and tissues.)

While I had been discouraged by my number of Neutrophils (a specific type of infection-fighting white blood cell), Dr. Lopez was encouraged by my total white blood cell count. He said it was super.

He said my counts are at a functional level and he believes this is because I have had a nice response to Neupogen (the medication that was supposed to help me make white blood cells).

He's also very encouraged that my condition has stabilized. (I was sick before. I haven't been sick lately.) This has been encouraging to me too.

However, Dr. Lopez says there is something going after my infection-fighting cells and knocking them down. He said this thing thinks there is something wrong with my infection-fighting cells (which is not the case. I just have less of them than I should.)

He stressed this point, that there is nothing funny, sick, weird or abnormal about my infection-fighting my cells, several times, saying it was key and really important.

Even though the quality and function of my infection-fighting cells is good, he said there is a mother cell driving them out. When I was explaining this to Cam & Sam last night, Cam said it was like the Death Star was attacking me. Death Star: an instrument of terror posing threat of annihilation...

So where is my Death Star? Dr. Lopez thinks it is my lymphocytes. Lymphocytes are a type of white blood cell that plays a large role in defending the body. They make antibodies that attack bacteria and toxins or even attack body cells directly when they have been taken over by viruses or have become cancerous.

Dr. Lopez thinks mine are confused and attacking my Neutrophils. So what do we do about it?

Health Crisis Recap

As most of you know, I have been being treated for a problem with my immune system since last fall. I wrote about the early stages of my illness and treatment on this blog:

Chapter 1: What's The Deal?
Chapter 2: Blue-Green Algae/Night Chills
Chapter 3: Music, New Antibiotic, First Blood Work
Chapter 4: Trip to the ER - First Hospital Stay

To summarize, in August of 2009, I had a bacterial infection that wouldn't get better. In September, my blood work showed that I had almost no infection fighting cells. I was told to go to the emergency room. Initially, the doctors thought I had Leukemia. They did a bone marrow biopsy but did not find Leukemia. They kept searching for the cause of the problem.

A CT Scan revealed that my thymus gland was larger than it should be. They were fairly confident that this thymoma was causing the problem. Four different doctors said it needed to be removed.

In October, I underwent a fairly major surgery called a mini-sternotomy where they cut through my breastbone to get to my thymus (located under the sternum, near my heart and lungs). It was removed. (They call this a thymectomy.)

I had been told recovery from the surgery would take some time. (They said I would have pain for about six weeks. I ended up having pretty major pain, requiring pain medication, for four months. Physically, I have felt much better since mid-February. I do still have a bit of pain for which I take Motrin daily.)

After the surgery, I was told not to push, pull or lift more than five pounds for at least three months. At my three month post-op appointment, they said I should wait another three months before doing any weigh-bearing activity. Because our insurance changed, I haven't had my six month post-op appointment yet but was told yesterday that I could resume normal activity, within reason. If it hurts, don't do it.

Back in October, I was also told the problem with my immune system would likely be resolved fairly soon. They said my numbers would likely be better within 2-3 days after the surgery. They weren't.

For the past six months, I've been on various medications to help me produce infection-fighting cells. Sometimes, I had enough. A few times, I had way more than I needed. But, disappointingly, I haven't been able to maintain an adequate number of infection-fighting cells consistently with or without medication.

Here's something I wrote in November about one of the first times my numbers dropped. I wrote this update about my treatment, in February. In March, my numbers were worse than they'd been before the surgery. I wrote, The Bogeyman is back.

Yesterday, I had my second appointment with my third hematologist, Dr. Alfredo Lopez. He outlined a new plan. I'll write about it in another post. As always, thanks for caring.