I had the big appointment with my Scleroderma Specialist from UCLA today and it was everything I had hoped for! After being told my tests results were normal by almost every doctor on my current team I finally have the validation of a physician that my disease is in fact progressing. My specialist took one look at my tests results over the past few months and could immediately point out the obvious advancement of my illness. He was very concerned by the sudden drop in lung functions, and by the recent Vasculitis diagnosis which he believes could be contributing to this recent drop in health status. He was also unhappy with the continuing downward succession of my GI tract, and my failing autonomic nervous system. One of the first new treatment options we decided on was to start weekly Saline Infusions to assist my body absorb electrolytes it desperately needs. This will be an intermediate treatment (that could be subject to change) just to keep me afloat until next month when I see my new Dysautonomia Specialist.
After reviewing my records together we did a physical examination where he also observed the development of further skin tightening. Scleroderma patients do what is called a Rodnan Skin Score which asses the thickening of the skin. Within the three months since I had seen this physician last my score jumped from a 6 to a 10, with tightening now in areas of the body such as the chest where it hadn't previously been. We spoke about the limited options Scleroderma patients have in regards to treatments that will slow, and sometimes even halt progression of disease. Each major intervention that is used to fight progression of this illness I had already been on, leaving one option open to us: Cytoxan. Cytoxan is a harsh chemotherapy drug used as the very last ditch effort in severe autoimmune disease. It is a Nitrogen Mustard class of Alkylating agent which directly damages the DNA. This drug is the typical "chemo" you think of when you hear the word. It sterilizes you, causes you to vomit, and of course makes your hair fall out. I will be starting once a month treatments that will last a year in duration. The doses will start low to prepare my body before we hit it hard on the third/fourth month of treatment. By December, if my body can handle it, I should be up to the full dosing with all the wonderful accompanying side effects. I have basically been told the next year of my life will be hell, but the long term benefits for my disease will be (hopefully) unimaginable! I am going to be honest, there are many opportunities for problems to arise during this treatment besides just the typical chemo side effects. This drug can actually itself cause Cancer due to the harsh damaging effects it has on the body, especially to the bladder and bone marrow. There is also the real possibility of becoming severely leukopenic which can be very dangerous if not taken care of immediately. My blood work will have to be carefully managed through out the next year. While this treatment will be cruel and jarring to my already weak body, it could save my life... Chemo is a bittersweet victory. At this point I am welcoming the opportunity to move forward with this option and be treated for the aggressive disease I have. This physician is not afraid to pull out the big guns and fight for my life... which I greatly appreciate!
This Friday is my Right Heart Catheterization. This is a high risk procedure, but one that is entirely necessary with my recent decline in DLCO. Originally we thought the cardiologist would go through my groin up to the heart, but recently I found out we will be going through the jugular instead. I am not often nervous for procedures, but I must admit I get a bit fluttery when I think of this one! I will more than likely not be up to posting until next week as this procedure will have me down and out for a few days. Hopefully all will go well!
After I escaped from the hospital this weekend my husband drove us up to the North Cascades to visit Lake Diablo. The water is such a surreal blue! I don't often get moments as incredibly serene as this, honestly I don't get many moments where I leave the house! lol So I just thought I would share it with you all! Thank you so much for your continued support and love! I wish I could have my impending chemotherapy infusions here... The outdoors are the best medicine!