August 2 2015

So the last few days may have been my worst in 3 years. Between rampaging fevers, coughing fits, low oxygen saturation and being unable to lay down for the most part. My wife, bless every part of her soul, has been there at every moment encouraging me, comforting me, helping with the medicines and keeping track of them. All this while looking after our kids with barely any sleep.

At 4am this morning she awoke to me coughing violently and barely able to breathe. I was in real trouble. While using the nebulizer and trying to catch a breath she was right there rubbing my back. That's when it happened....the back rubbing was really distracting and I snapped at her to stop. I felt awful instantly and I know it hurt her feelings.

Sometimes when we are super stressed we say things so quickly and without thought. I never meant to snap. I am really disappointed with myself. She is a rock for our family and this cannot be easy to deal with.

We talked about it this morning and she was very understanding.... of course she was. That's the woman she has become.

June 22 2015

Today was the first day of my infusion therapy for the MAC Lung Disease. The infectious diseases specialist, Dr Smiley, told me 2 weeks ago that this was the next course of action given that my current cocktail of antibiotics is not yet having an effect on this disease.

My treatment will consist of 1-3 infusions per week for at least the next 3 months. Given my hectic work schedule, this will take some reorganization of my normal weekly travel plans. 

For the last few days I have been quite nervous about starting this new therapy. So many questions have been buzzing around my mind.... how much will it hurt? Will I feel sick during or after each treatment? What will the facility be like? How will the staff be? And then I wondered about more abstract stuff like will they have good Wi-Fi? Will it be cold? Should I bring a sweater? Will they have snacks or should I bring them? Weird how your brain works....

I am very happy to report that the staff at the facility were completely lovely, professional and skilled. One nurse brought me a warm blanket right away, the clinic was spotlessly clean, I was offered refreshments and yes....the Wi-Fi was good! 

Of course having an IV put in is not the most pleasant feeling. It’s not something you put on your Christmas list or request for your birthday. It burns. If the person doing it is any good then the pain doesn’t last long.

The entire process was to take about an hour and a half. Unfortunately as this was my first visit there was some hurdles to get over. We were all set up and ready to go when the pharmacist refused to release the medication until speaking directly with Dr Smiley. After that they requested that my blood be taken to check the state of my kidneys. The machine in the pharmacy broke down and my blood had to be rushed to the hospital for analysis.

The whole process ended up taking two hours before I could actually have the treatment administered. The infusion itself took another one hour and then a saline flush and removal of the IV took another half hour. Evidently my future visits will be much quicker and the process much smoother. 

Sitting here typing this I realize that I am much less stressed about the treatment after today’s experience. I’m sure I will have good and bad days with the IV insertion and removal but I am confident in the skills of the people I met today. 

June 14 2015

Just going to skip back to the present for this post....

Staples.....not the office supply store and not the ones you have in your drawer that you can never seem to find when your stapler runs out. No I’m talking about surgical staples. 

When I had my lung surgery back in 1995 (a more detailed account is soon to follow I promise), they used a bunch of these little staples to seal the lung before closing me back up. And there they stayed.....well kind of.

A couple of weeks ago while vacationing at the beach, I was enjoying a Pina Colada and a snack on the balcony overlooking the ocean one evening. I felt something very hard in my mouth. I pulled it out and lo and behold it was a little titanium staple just like the ones in the diagram above....the closed ones. Like a tiny little shiny pair of glasses.

I must have been shocked right? Well not really. It has been a couple of years but this has happened before. The first time I was eating a cookie and could have sworn the staple came from it or the packet it came from. Then I really looked at it and it didn’t seem like a normal staple. A few days later I felt two more in my mouth. This continued until I had a nice little collection of about eight. 

On my next appointment with Dr Busy I dropped the little baggie in front of him. He looked at me questioningly. I explained how I came to have these little shiny relics of the past. He was speechless. He was also non committal and did not offer any type of explanation or opinion. It was like he just saw a UFO and then ignored the fact so he wouldn’t have to think about it. Plus he probably had somewhere to be....

So I was left to my own devices and you know what that means....internet research! At the time I was able to find one other documented case from the UK where someone had experienced the identical thing. A quick check today revealed a few more cases. Should that ease my concerns? I’m not actually sure but no one seems to have had anything bad happen as a result.

June 8 2015

At some point during the first few months I decide to try and retrieve my medical records from Australia. Perhaps they will be of some use in relation to my current circumstances or at least provide some background for Dr Busy to work with.

In 1995 I had surgery to remove part of my left lung but more about that in another post. I manage to make contact with the right department back home and miraculously my records are accessed from a storage center and copied. I pay a small fee and a short time later they are sent to me here in the USA. 

For anyone who has had to deal with bureaucracy in Australia you will realize immediately what a small miracle this really is. To get something from 15 years earlier copied and mailed to you overseas, let alone found in the first place, goes beyond any realistic expectation. I have Australian friends who will read this and I guarantee that a breathless “Bull....Shit” will escape from their lips...

At my next appointment I hand over the thick file to Dr Busy to research. He seems pleasantly surprised that I have been able to pull this off.... I’m guessing doctors are no strangers to bureaucracy either. He skims over a few pages, promises to get back to me, and then scurries off to whatever the hell is so important as usual..... perhaps there’s donuts for morning tea!

On my next visit Dr Busy says he found one tiny hand written entry in my hospital file that he wants to follow up with. I get an extra blood draw as my prize for this discovery and he, once again, dashes off to save the world.

This tiny piece of information turns out to be a vital cog in the wheel of mystery that has been my condition so far. The extra blood draw was for a specific test for Alpha-1 Antitrypsin Deficiency or A1AD. Ever heard of it? Me neither...... but it turns out I have had it my whole life. It’s genetic so one of my parents unwittingly passed it on to me. 

I am classified as an MZ. That means I do not have full blown Alpha-1 but it does mean that I am a carrier and that one of my parents is also. In medical terms, Alpha-1 is a fairly new discovery (In 1963 by Carl-Bertil Laurell at the University Of Lund, Sweden) so the likelihood of them being tested for any reason still remains very low. Neither have a bad lung or liver history so there has never been a reason to test for it. 

So now I know I have this thing.... what does that mean? I have a Pulmonary Function Test and although my numbers are lower than average they are still well above needing to have the specific treatment (infusion therapy) so for now it’s just a part of who I am. Dr Busy is not showing any signs of extra concern but seems very pleased that his hunch was right. 

I’m still coughing though....

If you are interested in finding more about Alpha-1 please check out the following links;

Alpha-1 Foundation

National Institute of Health (NIH)