I had my visit with the specialist from the Interstitial Lung Disease Network a week ago Friday. It took me a bit to process all we talked about; a bit longer to convince myself the glass was "half full" instead of the other way around, and then I had my 5th round of chemo on Thursday. Dottie was off Thursday for that and Friday because she worked last weekend to cover someone else, so we managed to get to the nursing home to see my Mom and take her her Mother's Day and lunch as Dottie is working this weekend.
The specialist wanted his own set of Pulmonary Function tests, so I was in early in the booth for those. The results pretty much matched the first set I had done in January-I'm at 54% lung function. The rest of the numbers I don't truly understand, though I'll get a good explanation in a couple of weeks when I see my regular pulmonary medicine doctor.
The drug Pirfenidone, legal in 32 other countries but not here, is the magic bullet I was hoping for when I went for this visit. It turns out even if I didn't have the cancer making me ineligible for any trials of any sort, my lung function is also lower than the baseline they would accept into a program. The drug is legal in Canada, but the specialist said everyone he's known to try and obtain it has had a phone call or visit from the F.B.I., and to obtain it legally there I'd need to be a citizen.
It is in it's 3rd trial here, ordered by the F.D.A. and he said that will close in January, they will gather their results and present them and perhaps by sometime in the fall of 2014 there will be a vote on whether to approve the drug in the U.S. or not.
The normal rate of decline due to IPF is about 7% a year; Pirfenidone in most cases negates that exact amount. The decline rate however, in practice is not a steady glidepath through the years, but a stairstep one where it drops then catches up with the curve. Still, any cessation would be a boost!
There was also a supplement that works for some people he wanted me to start called N-Acetyl-L-Cysteine. It was originally made for those who had liver damage from acetaminophen, but has been found to knock 3-4% a year off the decline from pulmonary fibrosis as well. I've been on it a week now, and it seems to thin the mucus I cough up a lot, making it less paste like and much easier to get rid of. It does have a noxious, sulfurous after-effect, but so far I've managed to keep that mostly to myself...
The other change suggested was a blood pressure medicine that has also shown a 3-4% reduction in symptoms for those it works for, so yesterday after having checked with the oncologist and heart doctors, I switched my Cardura for Losartan. I went on the minimum dose of Cardura after my heart attack two months ago because they started me on Sotalol to control the arrhythmia, so I've traded to the minimum of Losartan and am monitoring my BP every few hours-so far so good.
I wasn't smart enough to ask about the minimum lung function for survival while I was there, I was so caught up in all the information I was getting, but came home and looked it up and it seems that 20% is about as low as you can go before they put you in the hospital to await a transplant. I've been told I can't do that because of the cancer, though the specialist said if I can go 5 years cancer-free there might be a chance.
He also said that he firmly believes that my fibrosis may be work-related to all the solvents and chemicals I was exposed to through the years, and therefore I may not progress as "typically" as a normal patient; that if I stay away from chemical fumes and don't do other things to aggravate my lungs, I may have a better chance at a longer survival rate.
We did discuss "end of life" things, because I've been terrified for years now of ending up on a ventilator like my Dad did 30 years ago and not being able to get off it. He said with my lungs like they are, there is a very good chance that would happen, and even be worsened because a vent tends to inflate the lungs, and positive pressure would cause even more damage and lessen my chances of getting off it. He said if it came down to trying to get through a pneumonia or something, that if I was on one at 2 weeks someone would need to extubate me and I probably wouldn't survive it. We also discussed my CPAP I already sleep with, and BIPAP as other options.
When we talked about my fight against the cancer, he said if it comes down to it and the chemo isn't finishing the job, to go ahead and do targeted radiation, though there will be some collateral damage; he said the cancer needs to be beaten to give me a chance to fight the fibrosis.
So that's where all that's at for now...
___________________________________
The rest of life seems to be going a bit better! Stronger this week, though sleeping a lot still. I'm down to the last of my stack of library books; still as enamored of Irene Nemirovsky or even more so than I was in the beginning. When I return these I'm going to start on things that have gathered around me here for the last few months. I've felt too guilty about not reading the library books to pick up some here that I really want to read; hopefully I'll finish those between now and when we return from vacation in a few months and then take my next stack of notes to the library (I've got about 14 things on a list right now, though most are shorter poetry books, some new and some to re-read, like some early Anna Akhmatova and Marina Tsvetaeva. I have one of Harold Nicholson's on the list, and more of Nemirovsky as well).
Dottie will be home in a bit and I'll have Mother's Day dinner ready for her when she gets out of the shower, along with her present from me. It's a book I had picked out to get her for Christmas until she decided she wanted us to get a joint present (a stereo that can transcribe my old albums to disc, among other things). A history of St. Johnsbury, Vermont, the book is supposed to have a chapter on "meet the Fairbanks family", her maternal family, with images from the houses we've never seen. I'm hoping she has a lovely evening looking through them!
I have Bill and Laura's dog Obie here with me now as well, as they are buying a house. The one they are living in is being shown and they didn't want to crate or kennel him for the next few weeks. He is very well-trained and a joy to have around, though he doesn't much like some of the commotion of the kids playing in the street or the shadows of my old tulip-tree playing across the front windows of the house! He does seem to feel sorry for me though, as I wander through the house and he watches my oxygen line snag under a door or on the corner of the stove and jerk my head sideways. He cocks his head and looks like he wants to tell me "he knows exactly how that feels"!
Now that I have a bit more computer under my fingers I'm going to try and start updating that old Flickr feed to the right, so if any of you have interest, there may be a few smiles or head-scratchers showing up there soon!
May the week be kind to each of you!
alan
