So, it was about one year ago that the doctor sat me down and explained that I am alpha-1 antitrypsin deficient with the PiZZ genes. I went on the computer for about 2-3 hours a day for the first month just trying to get more information. I joined a chronic obstructive pulmonary disease (COPD) support group because my illness fits under the COPD umbrella. I have learned a lot about this genetic disorder. I have passed on one of my Z genes to each of my children. I hate that one. And life has changed. There are many "new normals" to my days. I have to say that I'm not really happy about any of them, but then honestly I'm not really unhappy about most of them either.

I've been thinking more about what it's like to live with a terminal disease lately. I think the shock is beginning to wear off and life is somewhat stable. If I die from alpha-1 deficiency it will probably be a long time from now. Unless something gets more complicated I'm guessing this will be a very slow deterioration of my lungs. That's a good thing. For all the rest of my life though I won't be able to run around and dance like I used to. And I feel sad about that. It's much scarier and harder for me to go anywhere. And I feel sad about not being able to just jump on a plane to go visit Mom or jump in the car to go visit Joni or Brad or Wendy. Some of that is my being scared of getting sick and landing in the hospital with no insurance. Some of it is understanding my limitations. I get short of breath walking outside to take the trash out. I have to stop and rest when taking an armload of laundry to the garage. When I do dishes I lean against the counter the whole time. These are pieces of my new normal. Not horrible, but not fun. At first doing these things felt like a slap in the face each time and a big reminder that I am sick. Now they feel like a little sting, and oh yeah, I have to rest and catch my breath after I do that.

Life in the Sierra foothills is so pleasant, I can't be too sad. I walk out on the deck and look at the valley and the Coast Range and it is beautiful. And everytime I see my husband's smile, well, see, life is good. If I talk to the kids or the grandkids that is an extra special bonus to my day. And I love talking to Mom. I am happy.

Back on infusions

It took one month, but we finally got all our i's dotted and t's crossed, and I had an infusion of Aralast last week with another one scheduled for tomorrow. It looks like we'll be okay with Baxter's help and generosity until 2014. Baxter is the pharmaceutical company. The Affordable Care Act sounds as if it will help me continue to get my infusions. We shall see.

So, do the infusions make a difference? I think so. They are supposed to slow down the degradation of the lungs. The infusions do not make me better as in restoring my lung function. That is gone. We are attempting to keep my lungs where they are for as long as we can. My pulmonologist feels like I'm doing pretty good. Oh, so that's what this is. He says things like "At least you're not on oxygen," and "you still get around on your own." When he says these things I feel like I need to be stalwart and strong, but I don't always feel that way. And he has seen others who are in worse health than me and I totally realize that. And I am happy for the health that I have, and especially I am happy for the health that I have had for my whole life. He only has one other patient with the same exact disease, but lung disease is not good no matter what the etiology is.

My weeks off the medication were not great, but I couldn't tell for sure if I was losing more lung function. The infusion did brighten my mood for sure. I don't think that was an effect of the drug but more a feeling of hope that I'm not going to slip away too fast.

This disease is quite troubling to me. I don't know how much to push myself. I guess I could just keep going until I can't breathe anymore and then stop for awhile and catch my breath. Actually that pretty much happens every day anyway. And yes, it does suck. Yesterday I was saddened by the fact that talking to my sister on the phone for 45 minutes, while sitting on the couch, left me completely exhausted. Arrrrgh. 

That's enough of that. My personal pity party is officially over this morning.

Yay for summer! We set up our above ground pool and go out and dip in it pretty much every day. It gets hot in these here foothills.

Elliot turns 5 in June. We are having a party for him at the lake. I am really excited about it! He wants an Angry Birds theme. And definitely a pinnee-yata with lots of candy inside it Gotu. (Gotu is my grandma name = goddess of the universe). Of course Elliot, anything you want. Haha. Those grandkids put a smile on my face just thinking about them.

P.S. Wow I wrote this a while ago and never published it. Before Elliot's party, which was a big ole blast!