Goodbye?
So, since last post I've gone through the high-dose cyclophosphamide protocol at Johns Hopkins with Dr. Brodsky, an oncologist who pioneered the treatment for SAA (severe aplastic anemia, medical term for bone marrow failure often caused by autoimmune disorders).
You can read about the treatment here or here. It's a promising new protocol for all sorts of autoimmune disorders and has been wonderful for SAA. Googling the term brings up hopeful articles that suggest it as treatment for SLE, Multiple Myeloma, and even MS. It consists for 4 days of transplant doses of IV cytoxan (cyclophosphamide) to kill the immune system, followed by a slew of antibiotics and antifungals to protect the weakened body and then, daily shots of growth hormone (nupogen, GCSF) to regrow the immune system. I am the first patient with Wegener's Granulomatosis to complete the protocol at Hopkins. I lost all my hair.
I don't think it worked. I have interventional bronchoscopies every couple weeks, where my surgeon goes down into my airways and tries to stretch open my inflamed and narrow bronchus and trachea so air can pass through. During the past few months the disease has progressed from my left lung to the entrance of my right lung and up into my trachea. I am not elligible for a lung transplant or even a heart lung transplant anymore because they sew on the new organs to the trachea, and my trachea has been irreversible damaged by disease.
Not only is my upper left lobe damaged and the exoskeleton of my trachea irreversibly destroyed, even worse, my Wegener's is still active. Still red, inflamed, angry, and destructive.
I had a very upsetting meeting with my doctors this morning. I suppose I shouldn't be shocked or surprised, but I am-- It was sobering. Basically my rheumatologist said, "Sarah, when we met you a year ago I wasn't ready to give up on a 16-year old. I told you I had more tricks in the bag. You know I am always an optimist and when you were down in the ICU and half of the doctors were saying you would be ventilator dependant for life, I was sure you would come through it. I have to tell you there are no more tricks in the bag. You've spent probably more than half, maybe two-thirds of the last year in the hospital. You need to decide now if you want to keep fighting or if you want to do all that you can at home. We can get into heroics-- but you have to understand we do those things without any expectations. I think there is absolutely no chance that you will have a long life. There is absolutely no chance to cure your disease. Your disease is in the end-stage. You're 18 now, you have to decide if you want to go out fighting."
Hard to hear when you've just turned 18, right? My surgeon already explained he was looking into several brand new stents for the trachea, but if he put them in, there is a likelihood they will become clogged with mucus and that will be an acute situation and I will die. I don't think he thinks I will survive the year.
Not only does my rheumatologist say he has nothing left except corticosteroids to halt the progression of my disease, but my surgeon says even if it were stopped that my airways are irreversibly destroyed.
Here's the thing... I don't believe in god... I don't believe in heaven. I really wish I did and I want to with all my heart but for me it's like standing in a corner and trying not to think of a white elephant, or wishing I had a pony or something dumb like that. Like, it just seems impossible but I don't want to die, I really don't. And it's not fair that my life is ending. My disease is in the end stage!! How come people who smoke and take drugs are still alive? Murderers and madmen? I never did anything like that. All I want is to live a little longer.
Whatever. I've cried enough today.
Anybody catch House on Tuesday? They thought the patient had Wegener's! Of course... you could tell he didn't have it. Who ever heard of Wegeners presenting in the liver?! Still, it was pretty cool. Third time they've mentioned it on the show. Turned out the patient had swallowed a toothpick!! I told my surgeon to look for a toothpick but... no go. Wish that's what I had, too.
PS: if I am going to die soon... another thought occurs: I should probably spend all my money, shouldn't I? Buy something really nice? That could be fun.