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.