Epside 3: Evil Phil

The cells that make up our bodies are just like us – they grow up, get jobs, have kids. The jobs vary from making hair or moving muscle to oxygen transport. Unlike us, they follow in their parent’s footsteps – if your parent was in the toenail manufacturing business then you will be too. As the generations pass, and as we get older, slight mishaps occur. A cell might still make hair, but it won’t be a nice shiny black hair but a bit grey. Some of these mishaps can have nasty consequences.

Cancer is one such consequence. The cell cycle goes wrong. Cells divide, but don’t get jobs or do their jobs properly. And they divide again and again and again.

With Chronic Myloid Leukaemia (CML), the cells that should be growing up into the myloid variety of white blood cells don’t. Instead they divide lots and your white blood cell count goes up. It takes a bit of energy to make so many cells so you feel tired. Your body tries to get rid of them and your spleen, whose job it is to remove trash from the blood, gets enlarged.

The actual CML mishap usually relates to a particular chromosome mutation creating the “Philadelphia Chromosome” – Evil Phil – formed when part of chromosome 9 (the abl gene) breaks off and attaches itself to part of chromosome 22 (the bcr gene).

I have Evil Phil in my body. Evil Phil’s signature bcr-abl (bee-sea-are-able) gene was measured at over 200 when I was first diagnosed. I’m not sure what it was 200 of, but it was not a good number.  It is now down to under 0.5 and has been bouncing around below one since February.  The aim is to get it to al level where it is undetectable.

Every month or so I travel down to hospital for another blood test to measure Evil Phil and a few other markers of my progress. Occasionally it is a bone marrow sample and I am soon going to celebrate one year since my treatment began with my third bone marrow biopsy.

The hardest part isn’t the needles extracting blood, or the big needle extracting bone marrow (that process is simple – I get zombied out and then it’s all over), it’s worrying about what this month’s number will be.

I have had the numbers go the wrong way. It was quite a shock. I was beginning to believe that all was well, the treatment was doing all it was meant to and the danger was over. Then the result came in with a bounce. Evil Phil was fighting back, maybe throwing another mutation into the mix? An analysis showed that it wasn’t a new mutation; just the drug losing effectiveness. Because I am on a trial, I was able to change medication and my numbers started dropping again.

Although my last test showed a small bounce – from 0.16 to 0.43 – my haematologist is happy with my progress so I wait for the next test and the next set of numbers to tell me what Evil Phil is up to.  

An electrospectromicrograph of the Philadelphia Chromosome, or Evil Phil.

Episode 2: Into the black world

Hospitals are big places. Rooms and corridors and “Follow the blue line from the elevator on level 3”. And you don’t want to go inside but you know that you have to.

Australian Olympian Raelene Boyle recently described her ordeal with breast cancer as like stepping into a black world and not knowing where she might come out. It is an apt description for taking those first steps into the hospital. Despite the bright flouro lights it feels bleak, I felt bleak, worrying about what was happening and what could happen, all the unknown possibilities.

The first thing I learnt was that I had to surrender. I had picked up from the doctors and the net that there was a wonder drug called Glivec that would probably cure me, sort of.  I wanted it now. Please, can we start treatment now? I read in forums about people going on to the drug pretty much the day they were diagnosed, so why not me? However, there were further tests needed to make sure I had the Philadelphia Chromosome. The doctor would decide what direction I would proceed in and it wasn’t Glivec; not just yet. Hydroxyurea first.

I had the script and went to the hospital pharmacy to have it filled. The pharmacist was reluctant. Had the specialist told me such-and-such, or arranged for a whatsit procedure? He had written the script, hadn’t he? He was a doctor and that’s what doctors are trained to do. Surely he had ticked all the boxes. And how would I know if he hadn’t?  I was stuck, unable to answer the pharmacists questions and wanting to scream at him “Ask him your bloody self!”

The following weeks include a bone marrow biopsy. Deep breathing and meditation were needed as the needle went into the back of my hand. Trust the registrar; it was all I could do. There was no other option.

The worst was lying in bed one night, discussing the disease and the treatment with my wife. “We were meant to grow old together”, she said. Those words cut deeper than anything else I have experienced with this disease before or since. I suddenly realised how all the plans we make can be so easily lost and that we have no power whatsoever to do anything about it.

I did have a future planned and it did include growing old with my wife. It included watching my children growing up. It included a whole range of dreams that I didn’t even know about. It still does.  I am not going to let them be snuffed out into a black world.

Episode 1: Discovery of the mutant horde

It was April last year. I had started collapsing on my bed in the afternoons for a nanna nap  and thought that, at fifty, I must be getting old. Bugger! Maybe a course of multivitamins would help so I started taking a range of coloured pills to fix what I thought was ailing me. My sense of smell was a bit weird (although things still tasted okay).  I also found my fingers going numb, particularly walking to work on the cold mornings. That worried me as numb fingers make playing guitar a bit difficult. A couple of bruises on my leg didn’t really attract my attention.

I had a routine health check-up for work. The doctor feeling my abdomen declared that I must be constipated as he could feel a bit of a lump. He wrote me a letter to take to my family doctor, but I ignored his diagnosis – I knew I wasn’t constipated. The letter sat on my desk for about a month.

I had a cough that wouldn’t go away.  A raspy little cough that I put up with. It was only when colleagues started asking me to “Die quietly” that I thought that I should see someone about it. My doctor examined me and couldn’t find anything.  As an afterthought, I handed over the letter which I luckily remembered to take with me. My doctor read the letter, had a feel of my guts and suggested I have a blood test – preferably that afternoon – just to rule out a few possibilities.

A few days later I received a call asking me to come down the surgery to discuss the results of the blood test – now! My white cell count was 224 – about twenty times what it should be. A few other things in my blood film pointed to chronic myeloid leukaemia (CML) aka chronic myelogenous leukaemia. The doctor told me a heap of other stuff but not much of it sank in: he had never treated anyone with CML before but apparently the survival rate has improved a lot and is now quite good, although he added he didn’t know what ‘quite good’ meant. He made me an appointment at the hospital for the next day and I would learn more from an expert in the field. He suggested I drink lots of water because with a white cell count that high my blood would be quite thick and stodgy and could just clog up in my brain and that wasn’t a good thing to have happen. He also suggested that, if possible, I shouldn’t Google leukaemia just yet.

I didn’t get much sleep that night.  My wife and I sat the kids down and explained what we knew and that the doctor was positive I would do okay. And we cried a lot.

The hospital the next day was a tad surreal. My referral was written on a  scrap of paper that the nurse on the desk didn’t seem willing to accept. I told her that I was only diagnosed yesterday and an appointment had been made and I had just driven two hours to be there and wasn’t going away. Eventually I saw the haematologist and had a big discussion about CML and treatments. More blood was taken for further testing. At the end of a long day I had a prescription for a course of hydroxyurea (which the pharmacist seemed reluctant to fill at first but eventually handed over along with some dire warnings about taking the drug), had signed up for a clinical trial and made some more appointments for stuff. And more information that I didn’t quite know what to do with, and lots of questions that I could ask but didn’t because I wasn’t sure I’d really understand or absorb the answers, like just what has Philadelphia got to do with anything?

The start of an adventure – whether I want one or not.