Happily Breathing

I was diagnosed with Alpha-1 Antitrypsin Deficiency in 2010. I'm an MZ carrier but not a full blown Alpha. This genetic disorder left me susceptible to MAC Lung Disease which I was diagnosed with in 2012. I have been treated ever since and now have COPD...

[iamhappilybreathing]

Thanks for letting me reblog pretty much everything on your page. I think I love glasses as much as you.

😊

[iamhappilybreathing]

Lung Evaluations

Over the last 2 weeks I have been experiencing what my new pulmonologist, Dr Concerned, calls a “flare up”. And yes, in case you are wondering I fired my previous pulmonologist, Dr Busy. It seemed like he had all but given up trying to find ways to get me more comfortable. I believe he was great at solving the riddle of my lung issues but once diagnosed, I found him to be way less interested in my ongoing welfare.

Back to the “flare up”....I had begun to go downhill again and 2 weeks ago my wife and I had serious discussions about checking me back into the hospital. I held out an extra day as I already had an appointment scheduled with Dr Concerned. I’m glad I waited.... he immediately prescribed me some antibiotics that I had not used before and boosted my Prednisone over a 10 day period. It took a couple of days to take effect but I now feel a whole lot better. In fact, the best I’ve felt in the last 3 months. My resting heart rate is the lowest it had been since leaving the hospital the very first time.

The other big news of the last couple of weeks is that my insurance company let us know that I would not be covered to have an evaluation or transplant at Duke Hospital in Durham NC....the closest and one of the best lung transplant hospitals on the East Coast. Even though it is “in network” they said I had a “Narrow Policy” which I purchased through the exchange which did not cover a transplant anywhere in our state. The options given were to go to Vanderbilt in Nashville or Charlottesville Virginia. The reality is that would mean moving our whole family to a place where we would have no network of help or assistance. That simple fact would probably rule me out for a transplant due to insufficient back up.

This situation triggered my wife to a level of “determined” that I have never seen or experienced before. She launched a social media campaign of staggering proportions aimed squarely at the insurance company and the lack of options on their own website that would have drawn our attention to an optional plan. By the way the plan we should have got (with the extra transplant coverage at Duke) has exactly the same name and details so there is no obvious way to tell the difference and you must buy it direct from them and not through the exchange. There is clearly no option to do that through the exchange website so us and many others have been caught out by this anomaly.

The campaign evidently triggered something in many of our close friends and even their own friends. Phone calls were made, emails sent, the insurance company’s Facebook page was overloaded with posts about our story, TV stations and other media were contacted, and local political identities were drawn into the fray. It was a pretty impressive display of the little guys being heard.

Finally, after a week or so of lobbying on many fronts, the insurance company succumbed to all the pressure and granted us a “gap exception” so that I can have my lung transplant assessment at Duke Hospital and be covered by my insurance. I still have to change my insurance to the same policy with the transplant stipulation included on Jan 1st. Duke have already contacted us and I am scheduled to be assessed over a week of testing in November.

In the meantime we have also heard from National Jewish Hospital in Denver who we are trying to schedule a visit with to completely assess my MAC Lung Disease and Alpha 1. At this point we are in the process of getting all the doctors to send the relevant information over to them so that they will know which particular tests they will need to perform during my time there. They are saying the trip could be anywhere from 5-10 days depending on my testing requirements. 

At this point we really don’t know if the trip will ultimately be necessary given that I am seeing Duke in the very short term. If I get on the transplant list then the need to go to Denver may diminish. It also depends on the length of time I’m likely to be on the list. If the doctors at National Jewish find a way to make me more comfortable during the wait for a transplant then it would totally be worth the effort to get out there.

The other issue is physically making the trip. I am so up and down at the moment that it’s difficult to predict how I will feel on any given day. My wife will not be able to come with me as we have a young family so luckily one of my closest friends has stepped into the breach to accompany me there and back and stay with me should I need to go. It’s such a kind and unexpected offer and a sign that your closest friends will always have your back when you need them. In fact this month we have seen many of these signs in the struggle to get me back to being healthy again.

One step at a time.....

[iamhappilybreathing]

Hospital

October 6 2015

I have been out of hospital for two weeks now without being readmitted. I was there three times for a total of 25 days during August/September. Each visit invoked more and more tests. In fact, I think I have had every test that a lung patient could possibly experience. 

It started just before I was due to see my Infectious Diseases doctor for a scheduled appointment. I had been slowly getting worse over the week prior. By the time I got to his office I was a mess. I was coughing and desperately trying to catch a breath. My fevers were also spiraling out of control. He admitted me immediately to the hospital with Acute Respiratory Failure. 

Over the next 9 days I was pumped full of broad spectrum antibiotics and gradually I got better. They said I had a secondary infection, some kind of superinfection on top of my MAC that was causing all the trouble. Slowly my fevers went away and I felt good enough to go home. I was discharged the day before my birthday.

Hospital was much better than I remembered from a few years ago. Firstly the food was much better and I managed to gain weight during my visit. The nurses were outstanding and overall I felt a lot safer while I was there. It had been a very stressful few weeks leading up to this and my poor wife had been getting little sleep and was watching me sink deeper with each day.

The first day home seemed fine but the next morning I awoke with the dreaded fever. I spent my birthday in and out of bed and felt mostly fragile for the day. The next day was my wife’s birthday and the fevers struck again. This time it was an all day event. The kids were really excited for our birthdays but we were both super stressed the entire time so they were not ones to remember with any joy. The next day I started out fine but by lunch time I could not breathe again. Back to the hospital.....

I was admitted with Hospital Induced Pneumonia and this time the stay was longer and I underwent a lot more tests including a VQ Scan and a Bronchoscopy. Again I was placed on broad spectrum antibiotics which were starting to give my veins a really hard time. They managed to give my lungs a good clean out during the Bronchoscopy which gave me some temporary relief. The focus of testing seemed to be more fungal than viral this time also. A few days into the stay I agreed to have a PICC Line placed in my upper arm as my veins were becoming less cooperative. By the tenth day I was feeling better and once again was ready to go home. 

Once home we had to maintain the PICC Line and I was sent home with a couple of days worth of infusions of the antibiotics. This involved a home nurse coming to teach us what to do and supplies to be delivered to the house. We managed the few days but it was stressful and the PICC Line was annoying. I had it removed the following week. 

My mother in law visited and we all went to the beach. Previously the beach had always been a soft remedy for my breathing issues. The fresh sea air had always provided a calm for me and the kids never stopped enjoying the sand and waves so it had always been a great family trip. Unfortunately I barely enjoyed it this time. My energy levels were drastically down and I was still having coughing fits so I spent most of the time inside. 

After returning from the beach I attempted to start up my regular infusions of Amikacin and went to a scheduled session as planned. Once there, the nurses took one look at me, took my vitals, called my doctor and then advised me to go to the emergency room again. 

So once again I was admitted to hospital. Since the last visit the different doctors had all discussed my case in some detail. I was given a different mix of broad spectrum antibiotics and the overall discussions regarding my ongoing health were now well and truly directed towards firstly getting me to National Jewish Hospital in Denver for a better assessment of my MAC and secondly getting all my results over to Duke to see if there is a chance I can get onto the transplant list. 

Wait......transplant? Honestly I had never thought deeply about a lung transplant before because I always assumed my MAC would be cured at some point and I would be able to preserve and work with the lung function I had left. The last few weeks have shown me how quickly I can decline and 25 days in hospital gives you a lot of time to reflect and decide what is important. I know I cannot continue to live like this. I want to live. I want to enjoy life. I want to walk. Run. Hike. Swim. Work. I want to chase my kids around and watch them grow. I want to walk down the beach with my wife. 

I’ll do anything now to be better.... 

[iamhappilybreathing]

Reliance

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.

I couldn't do this without her....

[iamhappilybreathing]

Infusion Time

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. 

Fingers crossed that I get no side effects from the medication.... this particular drug has disturbing side effects including renal failure, tinnitus and permanent deafness. I have been lucky over the last three years with the ever changing cocktail of antibiotics, having rarely experienced any major side effects except for the recent neuropathy which will probably never go away.....hey I’m still breathing!

[iamhappilybreathing]

Staples....

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.

The phenomenon is called metalloptysis and it’s quite rare as it turns out. It appears to be restricted to incidences of partial lung removal and seems to occur, in the cases documented, between 1 year and 10 years after the initial surgery. Mine was actually about 16 years later and now another one 19 years later.....I’m now waiting for the encore of a few more. I’ll keep you all posted.

[iamhappilybreathing]

A Vital Discovery

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)

University Of Utah

[iamhappilybreathing]

Now For A Second Opinion

June 7 2015

So after the episode in Atlanta, I get home and immediately see my GP who orders another CT. This time I have to tell them that I am allergic to the contrast dye which I’m told makes the image more difficult to read.... even so, the results confirm the hospital’s findings that I have nodules on my right lung. I’m referred to a pulmonary specialist.

My first visit to Dr Busy involves the usual blood draw, pissing in a cup and x-rays. I’m getting used to this already which is strange because I really have never liked needles.... I guess nobody actually “likes” them. Anyway they gather all the information they need and a couple of weeks later I return for a consult.

Dr Busy tells me that the x-rays back up the CT findings and the plan is to monitor these nodules to see if they grow any larger. I’m put on a schedule of monthly visits for x-rays and blood draws. 

There is no discussion about what the nodules could be. Dr Busy is all business. He’s short, to the point, answers only what he deems worthy and gives me the impression that we are not yet at the point of having that discussion. His demeanor is so emphatic that I dare not ask “stupid” questions. I always feel like he’s on his way to something quite pressing that’s definitely more important than giving any oxygen to my poorly researched babbling. 

This bedside manner continues to be a feature of our appointments. I’m torn between knowing everything that could be a possibility or closing my eyes until I really need to know what’s going on and dealing with it then. Dr Busy is showing no obvious signs of concern. Somehow that’s comforting....

The main focus over the first few months is to try and get my cough under control. It has been bad for a while and actually turns out to be the reason I had the chest pains back in Atlanta. The nodules are not growing, thank heavens, but I’m starting to stay up late to indulge in the foolishness of conducting my own “internet medical research” which is doing nothing to reduce any stress I might already have. 

These months mostly consist of an ever changing cocktail of prescription painkillers, antacids, antihistamines, lozenges, cough syrups and tablets in an effort to curtail my ever worsening cough. For a person who has never had any allergies, hay fever, acid reflux issues or long term pain medication, this is all quite overwhelming. Add to this the monthly terror of waiting for my x-ray results and you might get an inkling of how nervous, frustrated and mostly short tempered I have become. I am a bear to be around.....

[iamhappilybreathing]

When It All Began...

June 6 2015

Back in 2010 I was working in Atlanta and late one night I began to have quite severe chest pains. Having lived there for a time before I knew that the emergency room at St Josephs was very close. I tried not to panic as I made my way there as fast as I could, trying to put the thought of an imminent heart attack out of my mind.

Upon arrival they gave me an EKG which evidently they were not happy with and they admitted me to the ER for an all night battery of tests. I had x-rays, did a stress test on the treadmill, had blood drawn, pissed in a cup and finally had a CT scan which revealed 2 things..... firstly that I am allergic to the contrast dye. I developed hives within minutes which 2 quick injections of steroids fixed but either they knocked me out or I passed out....take your pick.

The second thing the CT uncovered was that I had several nodules on my right lung that they seemed very concerned about. I was discharged in the mid morning and told to follow up with my GP and get a referral to see a pulmonary specialist as soon as possible......worrying stuff. 

Oh by the way....my heart was fine as it turned out. My entire stay was overseen by a middle aged South African doctor. Once he realized I was an Aussie I became his favorite source of entertainment for the night. When I was running on the treadmill he was saying things like “I thought you blokes were tough?” and “I didn’t realize Aussies were so crap at running hard”....inspiring me to really try to stick it to him. His constant jibes actually made a fairly stressful situation for me a bit more easy to deal with. I still called him a “rhino fucker” at some point during the stress test though.....

[iamhappilybreathing]

Lungs767 by Bronia Sawyer

I wish my lungs were full of flowers....

[iamhappilybreathing]

I Don’t Want To Die

June 5 2015

That’s what Heather (wife/mum/BFF) said I yelled out before I awoke sobbing last night. I went back to sleep but it happened again not long after evidently. I can usually remember dreams fairly clearly, just moments after waking up, but this time I only remember being in a hospital and maybe an IV. I’ve also never woken in such a way before.

I’m sure it’s some combination of my current circumstances, the endless pills I have to take and my subconscious playing with my mind. In essence it’s true though......I don’t want to die!