I confess that the Italy post were a distraction, a little smoke and mirrors on my part to avoid discussing more difficult realities. Today, for whatever reason, I feel like I can write a medical update.
The Good News
I've gained weight. My bras actually fit again and, for a few of them, my cup runneth over. The weight gain is most noticeable in my upper body, especially my arms so I no longer look like a walking stick figure. The TPN is definitely doing it's thing. I try not to get furious when I consider the fact that I wanted to go directly to TPN ("Go directly to TPN. Do not stop for a G/J tube, or a second G/J tube. Do not owe thousands of dollars"). When the anger wells in me I try to meditate instead. I haven't hit nirvana yet, but I'll keep you posted.
Yesterday we spent an hour on the phone with my darling rheumatologist, Rick, in Charleston. The call was in lieu of the visit I was supposed to have yesterday but felt too exhausted to make. Someday I'll write more about this wonderful man because he is one of the few physicians I have met along the way that I not only consider open minded and intelligent but also warm and caring, a real mensch.
We discussed my current physical status at length and the options before me. We developed a plan of attack, and Bill and Rick divvied up tasks to investigate some newer drugs that have antifibrotic qualities. But I had an ulterior motive for the call.
When I was a young girl and began reading novels, I would always read the ending part way into the book. If I did not like the ending, I would sometimes stop reading the book. Sometimes I would still read it but keep my emotional distance from characters that I knew would fare poorly. I haven't done that in a very long time. I happen to be reading a mediocre book by John Grisham right now. It's very light and easy and takes places in Italy so it suits me for now. I skipped ahead yesterday to uncover the denouement and sort of chuckled at the 10-year old me resurfacing again.
Once she was there, she was not going back. So, during the call with Rick, I finally asked how it ended for pulmonary fibrosis patients. I needed to skip ahead for a few minutes so I could brace myself for what's coming. Rick, to his credit, answered calmly and honestly. Eventualy my heart, which is already working so hard beating 100-120 a minute at rest, will start to give out. Pumping against the increasing pressure in my lungs will prove to be too much and I will, most likely, die from cardiac failure. Unless of course I get another air embolism or sepsis or something fun like that.
A little anatomy lesson: The blood without oxygen (deoxygenated) enters our right atrium through the superior and inferior vena cavas. The blood enters the right ventricle from there and then enters the lungs through the pulmonary artery. In the lungs in becomes oxygenated via the capillaries the cover the alveoli (air sacs). The blood then returns to the left side of the heart which pumps it out to the rest of the body.
The pulmonary fibrosis has left me with insufficient surface area to oxygenate my blood and I cannot take very big breaths either. So my lungs and hert work overtime just to keep all the cells in my body supplied witht he oxygen that they need. So far they are managing. I had an echocardiogram in July that showed a completely normal looking heart, normal size and normal pressures. My heart, physically, is holding up. The question is for how long. I suppose the good thing is that we will have advanced warning. I will develop signs of right side heart failure and the echocardiogram will show an enlarged heart. At that point we will know I am nearing the end.
I suppose I'm glad I peeked ahead. The problem is that I cannot put this book down. I can't walk away this time because I'm not the reader, I am the author and a powerless one at that.
A week ago we decided to start a new drug tht is being used in Phase II trials in scleroderma. It is called Gleevec and is largely used for stomach cancer. There has been a case report of a patient with recalcitrant scleroderma improving on the drug. Bill really wanted me to give it a try so I agreed. Within three days I felt horrible. I developed edema (fluid) everywhere and horrible muscle pain. I even had edema in my gums that was making it impossible to sleep. So I am off that drug now. We will try it again in a few weeks at a lower dose and see if I can avoid the side effects this time.
When the interventional radiologist put in the first G/J tube, they had to move my stomach to tack it up against my abdominal wall. Something about that procedure seems to have irrevocably changed the amount of food that I can consume. The tube was removed three months ago and I can still only eat about 3/4 c worth of food at any one sitting. I don't think it's ever going to go back to normal, which means I will likely be TPN dependent for life. I cannot tell you how much this disappoints my inner glutton. It is good news for my dinner companions, however. We went out on a double date last night with our friends Courtney and George (yes, I left the house with make up on and everything!) at my favorite vegetarian restaurant. They have the most amazing gnocchi I have eaten in the United States. I managed to eat maybe 9 of them so everyone else devoured the remainder. At least it doesn't go to waste ;)