Three More

Three more alpha-1 antitrypsin infusions since my last blog. I got number 13 Saturday. I really did not want to get my infusion this weekend. The last 2 were no big deal. Just set up, needle in the vein, sit for an hour, done. But Saturday I just didn't want to do it. I didn't realize it was my 13th time until I counted them up for this blog. I do not like the number 13. Maybe my apprehension was my subconscious letting me know it was the 13th time.

In attempting to find my vein with the needle and get the IV dripping into my body, Bob had to give it about 4 tries this time. "I don't know how heroin addicts do it." That made me laugh. After my infusion Saturday morning Bob and I watched TV for a while, then I took a nap. When I woke up I had a lot of difficulty breathing. Then I got a major headache. I'm telling you, it's that 13 thing. :( 

Today is Monday and I'm feeling much better except that I haven't been sleeping well.That's pretty much okay since I can rest as much as I need to throughout the day. I'm thinking the not-sleeping-so-great is probably because it's December, not my favorite time of the year. Too many things haunt me this month. Some days it's all I can do to stay just this side of sane. Some moments I cross the line, but I have crossed that line enough times to quickly recognize where I'm headed, and I drag myself back to sanity, though admittedly not always willingly. And there will be times this month that I will give into it and cry and sob and miss my Amy and attack myself for not being able to save her. But not today I think. Today I feel better just writing about it. 

As a fellow COPD blogger put it "depression can and will slip in without notice, and without caring what it does to you." And I just want to add to that very insightful comment "or what it does to your loved ones." I try to hold the darkness at bay. I have learned many tricks over the years to keep it away, though at times I hear it knocking. Ugh, go away. GO AWAY! And sometimes when it gets in anyway it ends up rocking me while I cry and then sleep, and it slips out quietly in the night. Depression has not taken me over completely for a long time and I intend to make sure that it never happens again. 

This is a long and wandering blog. Thanks for letting me share with you.

Thanksgiving Infusion of Love and Antitrypsin

So my blog is up-to-date to the Saturday before Thanksgiving, November 17th, 2012. Infusion of antitrypsin on that day went fine. As long as we find a vein and the medicine drips into my body I think we did a great job. So far no scary reactions, although we do have 2 syringes full of epinephrine (like the one in Pulp Fiction) just in case. Bob always asks if he gets to (I mean has to) stab me 3 times if something goes wrong. Hahahaha, no. And where is the Sharpie for drawing the circle to stab inside of?

On Monday, November 19th, Brad, Jeffer, Joni and grandkids came to rock our world for 2 days. It was wonderful! Mickey waffles plus yummy omelets for breakfast. Bob's famous ribs for lunch. Lots of fun and lots of love. Four-year-old Elliot and I were outside playing. He was showing me how he walks really fast. "Everyone needs to walk really fast," he explained. I said, "I can't walk really fast anymore." He came back to where I was and grabbed my hand and said "Everyone but you Gotu. You can walk however fast you want."

Thursday Frank came up and brought Thanksgiving dinner for the 3 of us. The kids were at their dad's that day. It was yummy and very nice. After Frank left I felt completely exhausted. I don't cry too often, but I laid on the bed and wept. We still had one more family party - Friday morning brunch. I just kept on crying. Friday morning came and I decided I wanted to go to the brunch. I was still pretty tired but figured I would just sit home and be sad if we didn't go, so we went to Merced and met the West Coast Hutchings for a delicious brunch at Bella Luna. It was so great to see and hug everyone. Another roomful of love and fun and delicious food.

Home again by 2 p.m. Worn out but very happy. I slept for about 12 hours Friday night. Nice. 

 

Alpha-1 Antitrypsin Augmentation 8 & 9

I guess it's only week 9 of the infusions. Then it's probably okay that I am still getting used to this way of life. Pretty much every night I dream that I am running somewhere. Last night it was to Luther Burbank Elementary School. :) Then I wake up.

I want to write today to just put my feelings and insecurities outside myself.

Sometimes I am very scared. What is this disease doing to me? I wish I could see inside my lungs every day so I would know. Is it continuing to eat up my lungs? Are the IV infusions slowing down (maybe even stopping) the progress of the elastase? I go in January for more tests, so I will have to wait until then to find out the scientific answer. Until then I will continue to do what I think I can. My inability to breathe will let me know when to stop.

I have joined an online group on Daily Strength. It is a bunch of people with COPD and emphysema who talk (type) about what they're going through. We support each other and answer each other's questions and listen to each other's frustrations. It is interesting to me that people I have never met face-to-face, and probably never will meet, have become such a part of my life. The fact that I do not know the parts of their life that aren't shared online, and that they don't know mine, is part of what makes this feel like a safe place to me. We are respectful of each other. We don't talk politics or religion. It definitely helps me.

Sometimes it helps me because my questions about the disease are answered. Not the answers you can look up on COPD.com, but the answers about how it feels. The anger, the self-blame (mostly all previous smokers), the love for life, the pain of the disease. We have lost 3 members to this deteriorating disease since I joined (about 4 months ago). It is sad. 

Back to why I even introduced my group. One of the members wanted to know what would happen if they just kept on going, doing what they wanted/needed to do, as in running, or vacuuming, or cleaning the garage, or hiking in the mountains, or swimming in the ocean. Would one just keel over and die right there? The real answer is, you can't breathe. You have to stop. But I liked the question. Because that is what I dream about. Running and running and never stopping. 

Infusion #7

Well, we're definitely learning as we go. It doesn't seem like the IVs are a great big huge deal anymore, but they're still a big deal.

We bought a set of plastic drawers to keep all our medical supplies in. Bob is so organized. He labeled the fronts of them with the stuff that's in each drawer. It makes it easier to know when we're running out of stuff and how much more to order. A very good idea. A very smart husband.

Sometimes Bob finds that vein the 1st time, and that is really nice. This week it took about 8 tries. Ouchy. We bought Mickey Mouse bandaids so it wouldn't hurt so much. They really work!

Haha, remember I was going to keep track of how I feel and stuff after my antitrypsin infusions? Well, that isn't happening. I made a chart and filled it in for 2 days in a row. That was about 2 weeks ago. I just don't like doing that. I'm not a charter. Even when I was getting paid to chart stuff, ugh, I hated it. 

I do think the antitrypsin is helping me. It seems like Mondays through Thursdays are higher energy days. See, this is why I need to chart. Then I would know. We do the infusions on Saturdays. Sundays are football days, so they don't really count. Unless I get super excited about one of the games, Sundays are not high energy days. Monday, Tuesday, Wednesday, Thursday usually are good days. Sometimes I even climb on my treadmill and go for a while. That helps. By Friday I am getting tired. I don't know if it's psychological or physical. I'm going with physical.  

We'll keep this going. I'm glad that my husband is the one that puts the IV in me. I totally love that man.

Infusion #6

This week the IV of antitrypsin went well. It was Bob and I. It was pretty quiet and it took about an hour and a half to get everything set up and drip the medicine into me. Drip, drip, drip. 

Feeling better. Breathing better. I guess the antitrypsin is working. I still can't get too carried away. But losing my breath is one way to know that it's time to stop, since that's all I am capable of doing. Stop, breathe. Okay, move again, just not so fast this time.

This breathing better is helping me to feel happier.

Infusion #5

Phew, glad that one is over. Frank (father-in-law) was here. He brought me a very cool homemade armboard to help keep my hand still. That way the needle will stay in the vein instead of poking through it. Bob took 4 tries to find my vein. It hurt a little. But other than that all went smoothly. I think it's getting a little bit easier. Plus Frank brought us yummy sandwiches.

I am going to start keeping a journal about how I feel and how much energy I have each day. I'll keep it simple. I'm curious to know if the alpha-1 antitrypsin is helping me feel better. I know I'm not feeling worse, but I think it's time to record it.

Sunday we went to Yosemite Lake and played with the kids and grandkids and Aunt Jilly and Grampa Frank. It was a beautiful day, and we had a wonderful time. It was also Amy's birthday. Happy birthday baby. I love you. Her Pooh bear from Disneyland still sings, 20+ years later. That is so comforting to me.

 

Infusion #4

Friday was my 4th infusion of the alpha-1 antitrypsin. The nurse came for the last time. She basically watched while Bob set it all up and gave me the infusion. She had a couple of hints, but not too many. Except that this time there was a slight problem. The needle in my vein poked through my vein wall and allowed the saline to leak outside my blood vessel and into my tissues. Infiltration they call it. Luckily we were still setting up and it was saline and not the medication. Not sure if the antitrypsin would hurt me if it infiltrated, but it is way expensive. So I got stabbed like 4 times this week trying to find a good vein. But it doesn't really hurt. Plus I think it bothers Bob more than me, so no whining necessary. =)

We went to the pulmonologist Thursday, the day before my infusion. He was very encouraged. He said my lungs sound clearer. That was good news. He also teased Bob about having a "very special" wife, as I am a rare case in this disease. I have the ZZ genotype which is the worst. We will go back in January and do the pulmonary function tests again. These tests include blowing into a spirometer to see how well your lungs function (hence the name of the tests). Those tests were what showed that my lung function is at about 30%. They also were part of the reason for my doctor to look for something else causing my lung disease.

I have another infusion this Saturday. The nurse won't be here, but my father-in-law will be. So, it's Bob and Frank and me this time. I will be holding my hand with the IV needle in it very very still. Frank made me an arm board with velcro and everything to help keep my hand still. I will let you know next blog how that works out.

Shout out to blood donors everywhere. Thank you sooooo much.

Infusion #3

This time Bob did pretty much the whole infusion of the alpha-1 antitrypsin. The nurse only had to remind him of a couple of things and make 2 or 3 suggestions to make it easier on him and/or me. It went well, but it still freaks me out. 

My job is pulling the plunger back in the syringe to make sure my blood isn't clotting once the needle is in my vein but before we add the medicine. If my blood clotted, we would have to find a different vein to poke. It takes quite a while to mix up the medicine and get everything ready. So, I pull back on the plunger, watch the plastic tube fill with my blood, push down on the plunger, watch my blood go back into my body, along with a little saline. This just fascinates me. I could play with this the whole time Bob is working. Except then I remember it's my blood and well, maybe it's not so much fun. 

While Bob and the nurse put everything together I hold my hand with the needle in it very still. The nurse tells me I can move it around a bit like a regular hand, but I don't want to. I don't like having a sharp needle in my vein that could puncture another spot in my vein and allow the blood to escape into my tissues. So I keep my hand as motionless as possible. The nurse says I'll get used to it. She said that one of her clients had to go pick up her son at school and just threw the IV bag over her visor in her car and went to get her son. The police of course stopped her with all the tubing hanging down from her visor and out of her hand. What kind of new drug and delivery system was this? She just laughed and explained it, and they let her go. Well, anyway, I won't be driving around with all that hanging off me. Eeeew, scary.  

Glad week 3 is done.

Infusion #2

My second infusion was pretty much just as weird as my first one. We got everything out and ready for the nurse to come. Bob wanted to line everything up in order of use. That didn't really happen though. The nurse is really competent and confident, and she just grabs what she needs from the pile of stuff on the table. She kinda just dumps everything out and searches for what she needs. It is a pretty big pile of supplies. Needles of different sizes, gauze, tape, alcohol wipes, boxes of medicine, bag to hang on IV rack, tubing, syringes with saline, empty syringes, filters, gloves, and everything is all wrapped up in its own packages. We create quite a pile of trash.

The nurse was really aware that Bob was learning how to start an IV. She had him put on the tourniquet and feel my veins. That was pretty cool. My veins kinda popped up and you could definitely feel the difference. Bob did a lot of the preparation. Unbelievable how many alcohol wipes we go through. The nurse started the IV and while the medicine was going into my body she had Bob start a pretend IV. He jabbed that little needle right into my vein. He did a really good job first try. It didn't hurt too much. The needle has clear tubing attached to it. So he taped the needle and the tube onto my hand. Then he attached a syringe with saline solution to the tube. You have to pull back on the syringe to make sure you're in the vein. So when my blood comes out through the needle and into the clear tube hooked onto the needle you know you're in. Then you push the plunger of the syringe and my blood goes back into my vein along with a bit of saline. Pretty crazy.

The medicine dripped into my arm for about an hour. Ugh. Still not used to it. After Frank (father-in-law) and the nurse left, I cried again. Just kinda tears rolling down the cheek crying, not sobbing out loud or anything.

Yay, done for another week.

P.S. The nurse told me to be sure to be super nice to my husband since he will be jabbing needles into me from now on. :)

Infusion #1

Well, it took a while to get it all organized, but I had my 1st infusion of alpha-1 antitrypsin last week. 

The nurse was great. She is teaching my husband how to give me the infusions. We don't have insurance, so it will help to not have to pay for the nurse's time.

So the infusions are intravenous (IV) and they happen in our home. The medication and all the supplies to make this happen come to our doorstep. The medicine has to be reconstituted. It is quite elaborate with all the alcohol wipes and the needles and bottles of medicine and bandages and such. At least it seems to be now. The nurse says it will get easier and quicker.

Side effects of the infusion were minimal. I was somewhat nauseated for 2 days. I also felt on the verge of tears for pretty much the whole week. I think that's because it is so weird to sit there while stuff is going into you through your blood, knowing that you have to do this every week for the rest of your life. A friend suggested that I think about how the medicine is going into me and fighting off the bad stuff in my lungs. Yah, good idea, but it didn't really work. I think I was in shock most of the 2 hours that the whole process took. It was all kinda overwhelming. I cried for a little while after the nurse and my father-in-law left.

My father-in-law is a retired physical therapist. He came to make sure the nurse knew what she was doing. He did a good job of it too. He stood right behind her the whole time. He asked her a bunch of smart-sounding questions using all the right 25-cent words. She was up for it. It didn't rattle her at all. 

I haven't really felt much difference in my breathing. I'm not sure what to expect. The alpha-1 antitrypsin is supposed to slow down the degradation process, so maybe I'm not supposed to feel different. Maybe I'm just not going to get worse. We'll see how it goes.

To Don's and Naida's descendants - Getting the Word Out

Since I am PiZZ for alpha-1 antitrypsin deficiency I will definitely pass the bad gene (Z) onto each one of my children. This means they have a higher risk of having problems that relate to alpha-1 antitrypsin deficiency. 

Because I have two bad genes that means that my mom has at least one Z gene, and my dad had at least one Z gene. So, each one of my brothers and sisters has a 25% chance of having ZZ, which is the worst. But even having only one Z and one normal gene can cause problems.

This is called a punnett square. Genetics 101. Let's say that Don has an M (normal) gene and a Z gene, and Naida has an M (normal) gene and a Z gene. These are the possibilities of the genes their kids would inherit. I am kid 4 in this square.

	Naida M	Naida Z
Don M	Kid 1 MM	Kid 2 MZ
Don Z	Kid 3 MZ	Kid 4 ZZ

Reality is that every single one of us 8 kids could be Kid 4. Probability is not so much.

Why is this so important to me to explain to you? This inherited condition can cause or help cause disease. The more they research it, the more the research shows that alpha-1 antitrypsin deficiency may be present in (and at least partially the cause of) several diseases. These include emphysema, fibromyalgia, systemic vasculitis, panniculitis, and bronchial asthma. 

With this being an inherited deficiency I just wanted to help you understand that it could affect you, your kids, your grandkids, and on and on. 

There is a simple blood test to find out if you are alpha-1 antitrypsin deficient. If you are interested just leave me a message (my blood relatives, including cousins) on facebook. I can help you get hooked up. If you get tested without insurance, it could be considered a pre-existing condition, so just know that.

So, now that they found out I have this they are doing something about it. They are giving me infusions of plasma with the alpha-1 antitrypsin protein in it. This is supposed to stop or slow the progression of my disease - emphysema. We just started the protocol last week, so we'll know better in about a month I think.

Genetic Aspect of my disease

The doctor (well, nurse) called us back 2 weeks earlier than our next appointment. I was pretty sure that the results were back regarding my alpha-1 antitrypsin deficiency test.

In the meantime, I had been reading a lot about emphysema and COPD. I was feeling quite familiar with that mess. I came across an awesome support group online and read and read and read. So, what I was learning was that emphysema is a disease that progresses, but you can slow it down if you stop smoking, eat right, exercise, stay away from other sources of smoke, etc. I was feeling pretty confident about all that. I love to work out, but my shortness of breath had been making that difficult. I decided I would do what I could and not try to do what I have been able to in the past. Eating right is not hard for me. I don't care what I eat as long as I don't get a headache. Quitting smoking was way hard. That was a challenge. 

So, I'm feeling better about it all, and in I go to the doctor. He brought me in his office and sat down right across from me, knees almost touching. He very gently explained that my test was positive. I have alpha-1 antitrypsin deficiency. It is genetic. I got 2 bad genes, one from my mom and one from my dad. So my genotype is ZZ. That's as bad as it gets.

The pulmonologist explained that there are things we can do to help slow down the progress. First let me tell you what is happening to my lungs.

I got an infection (sometime in the past) and the white blood cells went to my lungs to try to get rid of the infection. In trying to make me better the neutrophils (one type of white blood cell) started releasing elastase. This is an enzyme that breaks down the elastic fibers (mostly) in your lungs. "Normal" people then produce a protein in their liver called alpha-1 antitrypsin. This protein travels to the lungs and stops the elastase from breaking down the elastic. 

I have very little alpha-1 antitrypsin, because my genes don't tell my body to make it. Therefore, my elastic fibers are getting destroyed with very little to stop this.

At first when I was researching this disease I read that if you don't smoke and you don't work with asbestos and you're not around a lot of smoke (like from fires and stuff) this deficiency won't necessarily show up. But, as I have researched it more, it does show up. And it does destroy lungs. There are people in my group who have never smoked and it is destroying their lungs. I have read stories about 26 year olds who are dealing with this. It is scary and life-changing. 

I will write more about how to slow this down next time.

Here is a link if you're interested in learning more

http://www.alpha-1foundation.org/

Finding out about Alpha-1 antitrypsin deficiency

August 27th, 2012

Well, this is my first time with blogging. So, here we go.

I was getting really short of breath a lot. I didn't want to go to the doctor about it. I was quite sure they would tell me to quit smoking. I did so love to smoke. My shortness of breath kept getting worse. To the point that I had to stop halfway down the hall to catch my breath. Ugh. Finally Bob said we needed to know what was going on. He went with me (practically dragging me) to see my primary doctor. She did a few tests, added some more junk for me to take (Spiriva, on top of current medicines Advair and Albuterol) and set me up with a pulmonologist. So, a month later (June 26th) we go off to see the pulmonary specialist. I had very little clue about what to expect. I figured he would give me the pulmonary function tests, which he did. And I filled out a questionnaire regarding smoking and family history and other medical issues. I have to say that I really like this doctor's office. It's a one-doctor, one-nurse office. They are both personable people and put you at ease to start. Even in the waiting room the nurse, was joking with people and had us all laughing.

The pulmonologist looked me over - listened to my heart and lungs, felt my thyroid, checked my throat. Then I went into a room where the x-ray I had brought with me was hanging up. The letter that came with the x-ray said my lungs were hyperinflated, indicating some sort of issue. The pulomonologist said that my lungs did not look very hyperinflated to him. But his nurse would run some tests.

So the nurse came in and had me blow into a spirometer that was hooked up to a computer. The monitor had several candles on the screen and I was supposed to blow them out. Fun visual. And boy did I try to blow them out. Twice I tried. Then she had me inhale some Albuterol and try again. I was able to blow out a few more candles with the Albuterol. This indicated I have an asthma component to my shortness of breath.

Back in came the doctor. He showed me a chart of COPD life expectancies. When we followed the trajectory I was on it showed I have 5 years. He explained that he was quite surprised that I was so far along in the disease. He said I didn't smoke that much (about 8 cigs a day) and I started smoking at 32 years old, so hadn't smoked my whole life. He wanted to test me for Alpha-1 antitrypsin deficiency. This is an hereditary (never know whether that's an or a) component of emphysema. So the nurse came back in and poked me with a needle and put some blood from my finger on a card and sent it off. They would know in about 2 weeks. Prior to this I had never heard of alpha-1 antitrypsin deficiency. When I got home I began researching it. More about what it is in later posts.

We set up an appointment for a month later. He changed my Advair to Dulera and gave me a month's worth of samples. Without insurance (we don't have insurance) this visit cost $250, plus the $50 to my primary healthcare provider. The medicine is outrageous. Advair is around $250/month, as is Spiriva. Don't know what Albuterol costs.

We went home to await results and the next visit.