It's been a while..

So I thought I'd better write an update as to my condition.
I'd gotten quite run down and fatigue was a major issue a few weeks back. The slightest exertion would have me puffing and panting and my heart racing. (it still idles at around 115bpm)

Then one Monday about three weeks ago I just seemed to spark-up and each day since then has seen a small improvement in my stamina and energy levels. The Erythromycin certainly seems to be working in allowing me to use a lower dose of prednisone than would otherwise be required to keep my BOOP in check. I would say that I am certainly on the mend from apparent relapse earlier in the year that caused a steady decline in my health from December till mid April.

I had a meeting with my respiratory Doctor last Thursday afternoon. It was the shortest medical appointment I've ever had. I told him what dose I was administering, for how long, and what my view was on the efficacy of the Erythromycin treatment. I had originally been told that they wanted me to be down to 20mg of Pred as of two weeks ago. I disagreed with this and stayed on a higher level until I was satisfied my body had adjusted. I'm only now just reaching the 22.5mg level.

I asked him if he could see any flaws in my reasoning. He said he agreed with what I was doing. He wrote down what I had said, the dosages of the Erythromycin that I had settled on through my own research, closed his folder. Said that they were all watching my case with great interest, shook my hand and we departed. The whole meeting took only a few minutes.

One bit of bad news was my haemoglobin count. Still waaaaay down at 88, it was 96 last time I checked it. I bet if it were in the normal range above 135 I'd be bouncing off the walls!
Have no idea why it's so low, but I'm hoping that it will improve as the chronic BOOP also dissipates.

I'm still being driven nuts by the constant itch. Life would be great if only I could stop scratching! I've tried herbal teas, lotions, potions and pills. Nothing works. I have some spray I've ordered off the Internet arriving next week. I live in hope!

Sarah and I went on a holiday to Takaka last week. We had a great time, it's a lovely place, however the trip was ruined somewhat by my constant need to scratch.

Wainui Falls, Takaka

Fifty !

Last thursday 14th April I turned 50.
Having battled for nearly six years to get to fifty, I must say it's a pretty sweet achievement. My goal is now to make sixty. :)

My actual birth day was not really celebrated, I worked a pretty hard day in the workshop building a complex front exhaust pipe for a turbo-charged Mazda RX7. My blood counts are still off the map and te resulting fatigue means I can only work hard for about twenty seconds before I need to rest for around the same amount of time. Hack-sawing through 3" pipe took forever.

I also found that my welding skills have taken a tumble due to the prednisone making my hands shake so badly. In the end I found that if I fired up my new Tig welder and used two hands to hold the torch I could go over the top of the ugly welds and smooth them out.
Where there's a will, there's a way!
The customer was very pleased with the result so that was good.

The other job I had in was V8 powered Ford Escort that I have had a lot to do with since it was first built in the late 1980's. The owner wants to get the car to a point that it can be used on the road and my job was to try and silence the extremely loud exhaust enough that it would pass the 95db noise limit.

This is what I came up with..



 I went for a sort of "Maserati" look by bringing the twin pipes together in the middle rather than out by the rear guards. The sound is awesome! The little SVO Ford V8 just sings at the slightest touch of the throttle. I can't wait to see it back on the race track and road again.



Anyway I digress..  So my 50th birthday was fairly much an average day but I did get a lot of pleasure out of still being able to design and fabricate this type of thing even though my body is completely wrung out. As I say to the customers... "it's better than sitting, watching Daytime TV on the couch"!
At the end of the day I am stuck with what I've been dealt.. lung disease, macrocytic anemia, a steroid trashed body, resting heart-rate of 110+ and no muscle tone at all, it all having wasted away due to the steroid increase. But I actually feel well so that means I tend to just plough on and do stuff regardless.

My 50th Birthday Party however was  a blast!. We had a good turn-out despite the weather and it rocked on from it's start at 4pm until the last bod fell out the door at around midnight. At one point there was around eight people in the spa pool, one or two having just stripped to their underwear and jumped in after rising to the dare!
About 40-50 people dropped in throughout the evening. It was really great to see everyone and I must make a special mention of the effort my buddy Murray O'Neil made in providing a good quantity of champagne and becoming the impromptu toast-master to see the occasion off on the right foot.

Healthwise my haemoglobin count is still stuck at 95, so no energy and no improvement there. The doctors have gone with the "Scanlan protocol" and I am now taking 800mg of Erythromycin (I chose the dosage) daily and so far it seems to be working. However it won't be until I lower the prednisone dose to around 20mg daily that we see if the BOOP is able to be held in check. I'll reach the 20mg target in two weeks. From there I think we should stay at that level for quite some time just to make sure we don't move too quickly and blow the whole thing with a relapse.
Apparently they had a meeting to discuss my treatment idea, could see no reason not to go with it and are very keen to see if it works as no other studies have been done since the first and only Japanese trial in 1995, which is of course the one I used to justify my treatment plan.

I'm now officially a Type 2 diabetic, another side effect of prednisone. I have a little electronic meter that tests and records my blood sugar levels. I have a pen that pricks my finger and then the meter reads the level from the blood sample. I do this about six times a day. I'll do it less as I see patterns appearing and as I get use to modifying my eating habits (less sugar and carbs) to keep the reading under ten. My highest reading so far has been 23.3! and that's with medication. So who knows how high it's been when I've been on 60mg of pred!. 

I've set myself a new goal.. Get my health level back to where it was six months ago. To think that back then I thought I was in bad shape! To be able to get to the top of boulder hill again in 30 minutes with only a little bit of puffing would be great! At the moment I can just manage to walk to the top of our drive. Tim my step-son has modified my mountain bike to fit on a stand so I can gently build up some leg strength. I'll also do some work with some very light weights too to build up my arms.

I'll let you know how I get on...  would be much easier if I had some red blood cells. I wonder if wishful thinking will grow some.?

Catch up...

My health has been a real problem the last few months. Energy levels have ranged between extreme fatigue and just plain exhausted. The blood transfusion I had three weeks ago has done it's dash and my levels are now almost as low as they were. But I'm not the sort of person to let that affect my day to day life so I have just plodded on with my work and taken things as they come.. if not a fair bit slower than usual.

I diagnosed relapsed BOOP a couple of months back and wanted to go for the full 60mg for four weeks treatment but the doctors didn't like that idea (even though it's the gold standard for relapsed BOOP).
They wanted me to reduce down to 20mg by next week when I see the respiratory clinic (another new doctor). I never made it past 30mg.

I've been running a fever morning and night for the last week and have now developed a cough in the last couple of days. My heart rate is still very high and I get dizzy if I stand up too fast.

In my last post I mentioned that I planned to ask the respiratory doctor if I could try the antibiotic Erythromycin as a means of treating my BOOP and weening off prednisone. My haematologist has no objections and has left it up to the respiratory doctor to give the OK. I'll find out next Thursday when I see him.

Now interesting thing... back in September last year I developed a cough and was put on an antibiotic for a week for fear that it could develop into pneumonia with my weakened immune system. The cough cleared up overnight and yet the lab test results showed no infection in my sputum. My GP was at a loss to explain how an antibiotic would have resolved the problem if there were no nasties there in the first place. It sure confused me too!  I was reading back through my health notes this morning and guess what that antibiotic was? You guessed it Erythromycin!

So armed with that bit of knowledge I managed to grab five minutes with my #2GP Kyra late on Friday afternoon and she prescribed me a seven day course.  Standard dose is 1600mg a day for infection. For BOOP it's 10mg per Kg of body weight. So I'll be cutting the pills in half and taking around 600-800mg a day until next week and monitoring the results.

Because I've been running a fever for the last week it will become immediately obvious if it's working or not. If the fever doesn't go away then I can crank up the dose and treat it as an infection.
(I don't want to start on a high dose because that may affect the efficacy of the drug at the lower dose.)

On March 20th the Wellington Car Club held it's annual Hillclimb on Alexander road. It's one of my favorite events and I hold the record for the fastest car to the top of the hill. On the Saturday night before hand I was woken by my heart pounding erratically in my chest. I was having my first ever episode of atrial fibrillation (erratic heart rhythm). I put on my sports heart-rate monitor and it was unable to get a fixed reading, it swung wildly from 150-226 beats per minute and by the pounding in my chest it probably wasn't doing too bad a job at getting the readings right.

I had two options... wait and see if it settled, or call an ambulance. I decided to wait. Within half an hour I'd drifted off to sleep again and awoke at 7am still alive and ready to race.
Once we got to Mount Victoria and unpacked all the gear ready to race I introduced myself to the ambulance officer from St Johns, who generously offer their services at all our events, and he suggested that he hook me up and perform an ECG on me to be safe.

My resting heart-rate was 156 but it had a normal "sinus rhythm" so we decided to keep an eye on it through the day and if any problems arose he would take me to hospital. Interestingly my heart rate never moved once I was in the car. It was only walking around that increased it. I guess I was more relaxed behind the wheel  :)  Had it increased significantly while racing I would have retired for the day for safety's sake.
Below is video of one of my runs. It's looks very smooth from this perspective. Top speed would have been around 170kph as it was a marshalls run (where we take the helpers for a run up the hill) and I was driving slightly slower than I would during competition.




I won the event, which was good. My time was .2 second slower than my best time, but that's OK it's still a fairly consistent time and shows I'm not getting any slower. However the competition is getting faster, with some competitors postings some incredibly fast times. I said at prize giving that they were certainly snapping at my heals. It might be time to retire the Evo and get back into the middle of the feild where all the fun and true competition lays.

To that end I'm pouring my efforts into getting my old AE86 Corolla race car (above) put back together.. sort of a 50th birthday present to myself.

I'll post an update on my health in a couple of days... right now my fatigue levels are huge.. I can't even walk around the supermarket without having to rest all the time... eventually going back to the car and laying down.

I've got two big exhaust jobs on this week.. it's going to be interesting getting them done with no energy!.

...Ron

Why I hate needles.. Not a good day.

It's not that I've developed a phobia or that I reel back in terror when I see one, but after five years of blood tests, cannula insertions and PIC line disasters, let's say I've developed a rather well earned disdain for them.

There was the central line insertion into my neck a few years back that went so horribly wrong that it was only on the third attempt by another doctor that happened to passing the theatre, that the hard plastic tube was finally inserted into a vein. That episode left me unable to move my neck for several days and with massive bruising that lasted weeks. The hospital even had an internal inquiry that resulted in changes to the way the operation was performed.

Then there was PIC line they tried to insert into my bicep in Germany. The 12" long wire went straight through my vein at the elbow and along my bicep under the skin. It was supposed to be inside the vein.
They injected saline into the line to check it and my bicep grew! fail again. Remove line and give up.

When I was younger the veins on my arms used to stand out like a relief map of the Nile Delta.
These days they cower and hide, tiny thin lines barely visible under the skin. Blame that one on countless chemotherapy sessions.

Many people have failed to get a line or even a needle into my veins. Sometime a simple blood test can become an exercise in mental anguish both for me and the person trying to obtain a sample.
I can steel myself for one failure. Two at a pinch but after that I loose my metal and although I just sit and bare it, Internally I'm over it and stressed to the max.

However some really good nurses have done a brilliant job and with some direction from me as to a suitable site have gotten a needle in first time with no bruising or fuss. However during a blood test two weeks ago the nurse dug the needle straight through the vein and then proceeded to push it backwards and forwards about six times hoping for something to start flowing. It didn't and I had a huge black bruise on my arms for a week.

The excellent nurse at Wellington hospital blood test clinic told me this is called "Tissuing" and is the sign of a person that is not very good at what they do. She then took a blood test and hit a vein first pop, leaving just a tiny red speck where the needle entered. As it should be.

Lets move forward to last Thursday and my blood transfusion.
I got to the Cancer day ward at 10am and I figured I'd be there around 2-3 hours.
I knew that time frame was probably not going to accurate when it took nearly an hour before I was finally hooked up to a machine and a cannual inserted into the back of my hand.

It had taken the nurse two goes at putting the line in , but I figured that might be the case and had mentally braced myself for the procedure. I asked the nurse how long it would take to transfuse the two units of red blood cells. The answer was four hours. Bugger, it would be well after three before I got away. I reached for my mobile phone and started to dial my first customer. A little image of a battery flashed on the screen for a second and then it went black. Bugger! I had no way of informing my customers that I would not be able to make it that afternoon. Could the day get worse?

A couple of hours pass by and I need to use the toilet... I unplug the pump unit from the wall and it automatically switches over to battery power. The unit hangs on a wheeled coat-rack looking thing along with the blood bag. I wheel it trough the day ward and into a vacant toilet. As I'm about to leave the cubicle a felt a familiar but uncomfortable sensation in the back of my hand and I panic! It's the horrible feeling of that long cannula needle sliding out of the vein in the back of my wrist. I look down and blood is hosing out of both my hand and the plastic line that now hangs down by the floor.

Blood covers the floor and is pumping from my hand. I reach round for something to stem the flow, a paper towel does the trick. I stick my head out the toilet door look pathetically at the nearest nurse and say "umm" . She escorts me back to my seat and tells me not to worry.
The others patients around me have that look on their faces, like.. "hey wasn't he all hooked up to a machine when he left?". I sit down and wait to be attended to.

The nurse once again attempts to insert a new cannula needle and fails. Then she looks at the needle and says "It's a good thing it didn't go it" It's the wrong type!" Oh great, that really makes me feel better!
She the turns to my other hand and has a poke around there with the correct type of needle. Again she fails and she's been "tissuing" with the needle too.. straight through the vein. It's hurts like a bee sting deep in the under the skin. Mentally I'm a mess, but I put on a brave face.

She returns back to my right hand, digs some more and fails. Then back to the left hand and puts on a tourniquet tightly and starts looking for vein brave enough to pop up. Then one does. massively! The vein she just tissued hasn't sealed yet and the pressure of the tourniquet causes it to blow out. A marble sized blue lump appears on the back of my left hand.  She quickly removes the elastic strap and apologises.

At this point I'm almost ready to run screaming from the room, but big boys don't do that. all I can do is scream inside, grit my teeth and sweat profusely. She returns to a spot on my other wrist, up the arm from where the last line was successfully inserted and she finds a vein. The blood flows out into the plastic tube and we both sigh. She puts on extra tape to prevent a repeat of my earlier disaster. I slump down in the lazy-boy chair and try to sleep to the rhythmic whirr of the blood pump. I fail.

By 4:30pm it was all over. The line is flushed and removed and I headed off home. Six and a half hours of my life I'd rather have not had to deal with.

Last night I had two very large handles of Jim Beam Bonded Bourbon and Coke. It's been over five years since the cap came off that bottle. Now I must say that tasted great!
Yesterday would go down as probably one of the least enjoyable days of my life.
But hey that Bourbon was pure magic! 

The new blood seems to be helping with the energy levels too. I've spent most of today getting the Evo ready for Mount Vic Hillclimb next Sunday  :)

Feeling pretty darn proud of myself...

My energy levels are at an all time low. I'm back on a high dose on prednisone because of a possible (but mild) relapse of BOOP. (However never one to sit around, I'm still working flat stick and every day is booked with various tasks)

Basically my immune system is in over-drive and there is an inflammatory response in my lungs that causes the minute air passages to block up. By treating with a corticosteroid such as prednisone, my immune system is suppressed and the inflammation reduced.

The steroids have massive side effects, both short and long term. Thinning of the skin, fat build-up in the face and stomach. higher risk of glaucoma and diabetes, bone density loss and possible bone necrosis in the hips etc over a long period of use.

So as you can imagine the doctors want to get me off prednisone if possible. Trouble is that it would appear that every time I go below 20mg a day I go into BOOP relapse. So last month we tried Azathioprine, an immunosuppressive drug often used to avoid rejection in transplants. The problem was that this and other similar drugs are also myelosuppressive ie they damage bone marrow (where blood is made) , something I very little off to start with.

So after my blood counts plummeted following a week long trial of the drug it was decided I would have to stay on prednisone indefinitely as I wouldn't survive very long with no marrow!

Not one to give in lightly, That got me on a hunt for a drug/ treatment regime that was not going to wipe out my bone marrow. I found a medical paper that mentioned a study in 1995 by a group of Japanese doctors who had successfully treated six BOOP patients with a low dose of the antibiotic Erythromycin.

Apparently there is an anti-inflammatory side effect to the drug even in relatively low doses.
I printed off just one paragraph of the study, just enough to get the haematologist interested enough to look into it. He accepted it just might work and has promised to discuss it with his respiratory colleagues and phone me back.

If they accept my treatment plan, and so far I can't see what we have to loose with no other options, I will be over the moon!

Imagine that. Interrupting the senior haematologist as he tells you there are no drugs that will treat your disease without killing you, and I say while passing him my fndings "have you considered Erythromycin ?" and he pauses, reads my document and say .."that might just work".  Priceless!!.

Fingers crossed for  the go ahead and positive outcome.  I'm feeling pretty darn proud of myself right now. So much so that I'm about to celebrate with a Bourbon and Coke. Something I haven't had in a number of years. Tomorrow I'm in for a blood transfusion anyway so it's not like I can do any permanent harm!  :)

Cheers!   ...Ron