Blasts, Buddha and a bucket full of chemo – Part 1

I must apologise for my radio silence. I signed off my last blog entry ‘to be continued...’ Well, sorry guys, I didn’t think it would take me 10 months to get back to you. You see life has been a bit busy of late. From a blog that started out documenting a new drug trial, it quickly became clear that a MINI Bone Marrow Transplant (just chemo, no radiation) would be on the horizon. I had prepared myself for that and was ready to hit CML head on so I could move on with my life. Well what happened next really took the biscuit... (I would really like to swear as the word ‘biscuit’ just doesn’t seem to cover it.) so I will update my blog over the next few weeks with what has happened these past months.

Let me rewind to the very next day after my last blog entry, 10 months ago, Friday 29^th April 2011, the second consecutive 4 day weekend to celebrate the wedding of Prince William and Kate Middleton.

My Granddad John had been taken into hospital. He had been fighting lung cancer and was admitted with a possible infection after his chemo and radiation. My family and I were straight in the car to see him on Friday. He was on a general ward looking strong and ready to get out of there. He was on oxygen to help him breathe, but overall he saw this as a blip in his recovery and was fighting it all the way. He was off his food but we went to get him a milkshake from McDonalds to get the calories inside him. He lapped it up. We were joking around that we would be running a marathon together next year. It was very hard for me to see my Granddad in a hospital bed, but after speaking to the doctors they reassured us that he was a strong man and that he was being treated for all possibilities, (likely pneumonia or a blood clot) until the tests came back to confirm what they were dealing with. Early evening we all hugged goodbye and promised to visit again soon.

It had been a long day, and I had also managed a 3 mile run that morning, so as we drove home I turned my phone on silent and had a snooze in the car. As my parents pulled up to my house to drop me off I noticed I had a missed call from an ‘unknown number’. Before getting out the car I listened to my voicemail, it was Sophie, the on-call Heamatology Doctor, asking me to return her call. Now, I was a little perplexed to say the least, I was only at the hospital the day before, it was 8pm on a Friday night and someone from the hospital was calling me. What did they want to talk to me about? She even signed off her voice message asking me if I had enjoyed the Royal Wedding. I played the message to my parents and Mum was trying to reassure me all was OK, saying “Why would Sophie mention the wedding if it was something serious?” But deep down I knew something wasn’t right. Chris, my husband, was at a BBQ round our neighbours, so I asked my parents in to hang around until we got to the bottom of the call. I rang back and spoke to Sophie, she explained that there were some problems with yesterdays blood results and asked if I could return tomorrow to be bled again.

Now I like to think of myself as an informed CML patient. CML has 3 phases of the disease, Chronic, Accelerated and Blast Crisis. Each of these phases are defined by the number of blast cells that are in your blood/bone marrow and follow on from each other as the disease progresses. The more blasts present the worse more serious the treatment and prognosis. The idea is for the TKI drugs to keep you in a permanent safe Chronic phase, much like diabetes.

Still on the phone, Sophie was reluctant to give me details of the problems but I pushed her. “So you’re telling my there is an abnormality in my blood”

“Yes.”

“Are we talking about blast cells?”

“Yes.”

“What is the number?”

“Why don’t we discuss this at the hospital tomorrow?”

“WHAT IS THE NUMBER?”

“Michelle, you have 100% blast cells in your blood.”

Silence.......“Oh shit, I’m in blast crisis aren’t I?”

“I’m sorry to say, yes.”

That night was the longest night of my life, I was hysterical, scared, anxious and inconsolable. My husband and family were telling me that I could get through this but I needed to speak to someone that had knowledge of the path I was about to follow. I turned to two people, Cathy, a CML friend who had recently had a mini transplant and Elizabeth, a lady who ran our CML support group, she had had a transplant for 2 types of Leukaemia 6 years prior. Both Cathy and Elizabeth were there for me throughout and mean the world to me.

I have no idea how my family felt. I didn’t have the capacity to even think about it. Helpless and in despair I suppose, my mum just wanted it to be her in this situation.

 “No way!” I joked through the tears “You wouldn’t be able to handle it like me!”

With only a couple of weeks to live, I was at the hospital the following day for tests and admitted as an in-patient on Sunday for more tests and on Monday my best friend Kim, another rock to me, was cutting my hair short in anticipation of treatment to follow.

To give me the best chances of cure I would need chemotherapy to put me back into Chronic Phase CML (2^nd remission) before I moved forward to have my Bone Marrow Transplant.

Unfortunately, my Granddad John ended up in Intensive care over the same weekend. He deteriorated fast. He had pneumonia, blood clots AND the lung cancer had gotten bigger. It was too much for even the strongest person to overcome. On Wednesday 4^th May 2011 @ 16:50 my Granddad passed away with his family by his side and his favourite music playing – by 19:30 I was receiving my first bag of chemotherapy in a sterile hospital room with my husband holding my hand...

From that moment on I was doing this for my Granddad. Statistically we couldn’t both live through such serious conditions, I believe he passed away to let me live, he knew what had happened and even though he could not speak towards the end, my mum told me you could see the heartbreak in his eyes when they spoke about me. His strength, courage and belief was now with me and all I wanted was to get through this for him.

Love you Granddad.

x

CTRL, ALT, DELETE

As I sat down to write today’s blog entry my laptop froze before I had a chance to get past my log-in page.  As any IT expert would tell you, you administer the trusty CTRL, ALT, DELETE restart manoeuvre and Bob’s your uncle, your computer behaves again.

If only things to do with CML were that simple! Where are my restart buttons? Why can’t it be that easy...

...To say I have had a bumpy couple of weeks is an understatement. I went back to Hammersmith Hospital last Thursday expecting a bone-marrow biopsy in my left hip, what I ACTUALLY received was a bone-marrow aspiration in my sternum. With only a local anaesthetic!

Now, to be fair, the actual process of getting marrow from my sternum wasn’t too bad; I think the anticipation of such an invasive procedure being so close to your face is worse than the actual event. It was slightly more intense than the hip biopsy but over more quickly and we got lots of marrow.  The icing on the cake is that you get way more sympathy with a big white plaster on your chest that looks like you could have a 3^rd nipple hiding underneath. Chris came in with me for support, he held my hand, reassured me and had a good boy-gawp at what was going on.  I would certainly reassure anyone who has to go through this that it isn’t that bad. We were confident that we had enough marrow to get a result and get going on the trial.

Fast forward to today. I was going back to Hammersmith with every intention of collecting the Ponatinib pellets to start the trial.  What I ACTUALLY got was Prof. Jane Apperley telling me that they have picked up an additional translocation in my chromosomes.

A human body cell, such as those that make up skin, kidney tissue, heart tissue and blood vessels, all contain 46 chromosomes. The chromosomes are arranged in pairs, so that each cell has 23 pairs. My CML is caused by pieces of the 9 and 22 chromosome pairs breaking off and swapping round. (Written as:  t(9;22)). In addition, I now also have a small percentage of cells which are showing the same thing happening between my chromosomes 3 and 21. (Shown as t(3;21)).

We are not sure of the true meaning of this additional translocation, but we all feel that it would be better to treat immediately rather than wait for 3 months to see if a new drug MIGHT work against it. I am trying to keep the odds in my favour, so the decision has been made that I am to head for a bone-marrow transplant in the next 4-6 weeks.

So there is it is, I am heading for not just a restart of the machine, but for a full system wipe and reboot. (Let’s hope I get upgraded to the new XP version with added anti-virus technology.)

I will never become a true lab rat. Sorry folks. 

At the moment I am surprisingly calm about what’s going on, I think I am ready to get rid of this disease squatting in my body.  It has truly outstayed its welcome, like a long lost relative that comes to stay and never leaves. It’s time to say goodbye for good and change the locks.

The next step is to contact my bone-marrow donor in the USA (who from here on in will be referred to as Brad Pitt) and find out when he has a blank date in his diary to donate some of his good marrow. Brad, if you are reading this, I am forever grateful, I hope we can meet one day.

I mentioned earlier in this blog that the past 2 weeks have been a rollercoaster. So far I have told you the lows, but in-between I have had some great highs. Being off my drugs has allowed me to revert back to my pre-diagnosis days.  I have been spending some fantastic time with family and best friends, burning the candle at both ends. Nights out in London, lunches out, BBQs and lying in the summer sun. Absolutely perfect. 

The support network around me makes me feel like I can conquer the world; my husband, my family, friends, neighbours and work colleagues. I have such strong foundations and amazing people at my side, how can I lose when I have already won?

Bring it on.

To be continued...

x

Dem Bones Dem Bones Dem Dry Bones

Last night I was very excited at the thought of going back to Hammersmith Hospital today to pick up my new rat-pellets to start on ARIAD. So why am I sitting here writing this blog instead of sitting at the hospital downing my drugs…      

To qualify for the ARIAD phase 2 trial I have to pass certain criteria. Now, I have always found passing tests to be relatively easy, so as you can imagine having NO control over my CML results can be quite frustrating to say the least, (especially when you are constantly failing CML class).

The main qualifying criteria for ARIAD is to have failed at least two out of three current TKI treatments – Imatinib, Dasatinib or Nilotinib. BINGO! I have finally passed something (ironically by failing twice!) I have failed Imatinib and Dasatinib so I qualify right? Well yes, nearly…

You need a bone marrow biopsy result to confirm this and show that you still have a good number of leukemic cells. The result is also used as a baseline to monitor your progress throughout the trial.

Last weeks bone marrow biopsy = FAIL!

My biopsy result has come back as ‘inconclusive’ not because I don’t have leukemic cells, but because they couldn’t get any bone marrow out of my bones to get any result at all. AKA my bone marrow is as dry as a popcorn fart.

Initially, ‘MOST’ CML patients have BMBs (bone marrow biopsies) every three months until they are shown to be responding well to a drug and then increasingly, BMBs fade away into the distance and are replaced with PCR blood tests, which are more accurate as the levels of bad cells reduce. For me, I have consistently been having biopsies every three months since my diagnosis a year and a half ago.

Biopsies have become a thing of dread for me. For all you non CMLers, if you’re interested to see a biopsy CLICK HERE there are loads on YouTube. For everyone else, I won’t make you relive the experience; you all know how it feels.

Six times I have some confident cocky trainee trying to poke around in my hips and get to my ever-so-scarce and valuable marrow without anything other than a local anaesthetic.  My gorgeous mum holds my hand and looks on with dread (she is scared of blood and needles) as they poke around in my hip looking for the amber nectar. As time has progressed, my marrow has become as endangered as the Madagascan Aye-aye. Ever seen one of those? Nope, nor have I.

Generally my biopsies follow the same routine – the local anaesthetic goes in, I yelp and ask for more anaesthetic, they administer another anaesthetic shot for luck (I am very persuasive), the marrow needle goes in, they poke around, they panic, look at my mum, whisper, poke around some more, wipe some blood on some slides, realise they have no marrow, cautiously ask if they can go in again, I whimper… (go to the top and repeat x 5), then we give up.

Not so confident or cocky now are you Dr. Trainee…? As I hobble out the room I mostly mutter under my breath, “I wish I could say it had been a pleasure…” and “I hope we don’t meet again too soon”.

So there it is, I was sitting on the sofa last night when I got a call from Prof. Jane Apperley (I love Jane, I quite literally owe her my life. Love you too Mum!) telling me we have another ‘inconclusive’ BMB result and it would be a wasted trip popping by to pick up my drugs tomorrow as I can’t start them yet until we get a result. The plan of action is to wait a week, stay off my Dasatinib and try again on the left side of my hip – we usually go to the right. If that doesn’t work, don’t worry she says, there is always the sternum to try after that . . . (I REALLY hope going left side works!) I will then have to wait another week for results, so I have no hope of starting the trial for another fortnight.

I am frustrated to say the least.

In the meantime I am going to enjoy a drug-free couple of weeks, a rare treat. I am out on the town Saturday – at least I won’t have a TKI hangover on top of a self-induced hangover.

x

The story so far...

I was diagnosed with Chronic Myeloid Leukaemia (CML) on the 09/09/09 after an initial diagnosis of shingles, a spleen the size of an unborn baby, a swollen stomach and a subsequent blood test showing a white blood count of 245 (a normal persons WBC is between 4-11).

Leukaemia might kill me; then again, it might not. But it’s certainly not the kind of prospect I thought I would be pondering in my early 30s.

Until my diagnosis, I hadn’t really thought of death. Well at 29, why would you? I was at an age where I thought I was getting somewhere, I was creating my future. Death was something that happened to older people or sick people on the news. I exercise, I eat well. This death malarkey won’t be relevant to me for a very long time, so let’s deal with that then. Let’s get on with life.

Then BAM!, overnight I became one of those sick people.

My life will never be what it was before. I guess that’s a really important thing that people have to deal with when they find out they have cancer – that no matter what happens, they can’t go back to the carefree feeling they had before. I have a new intimidating reality that involves measuring the amount of mutant BCR ABL Philadelphia Chromosone (CML) genes that threaten my body. But as people with CML say, life becomes a ‘new normal’. I still do and enjoy all the things I did before, I work, I go out with friends, I play netball, damn, I have even completed in a triathlon since my diagnosis. But life has got a bit busier, I also now attend hospital appointments for regular check-ups, I have become obsessed with blood levels, scared of bone marrow biopsies, I have become good at listening to my body, resting when I need to, recognising the signs of Neutropenia and injecting myself with GCSF growth factor. But most importantly I have become a good listener and made some very dear CML friends.

As they say in Thailand: same same, but different.

Now my response to treatment thus far hasn’t been ‘textbook’ to say the least. I failed the 1st line maintenance therapy drug (TKI) Imatinib, with a bone marrow biopsy showing 100% cells leukemic after a considerable period on the drug. (The aim is to get to 0% on a biopsy.) I then moved onto a stronger 2nd generation drug, Dasatinib, again my biopsy showed 95% of my cells as leukemic. I just couldn’t seem to shake this CML. In addition, I am not known to have any other mutation that would render the drugs useless.
I figured that that this CML was created by me, I am as stubborn as mule, so why wouldn’t my CML show the same traits. Trying to turn this light switch off with a finger wasn’t working, so now let’s try with a sledgehammer...

In December 2010, the decision was made to move to a reduced intensity allograft bone marrow transplant. I have a 10 out of 10 non-related match identified in the USA and we were moving forward with a mini transplant for April 2011.

To add to my ever increasing list of things you shouldn’t have to do before your 30, because the transplant will most likely render me infertile, my husband Chris and I decided to undergo the first half of the IVF procedure. We moved forward with the treatment and dropped off 12 embryos at the ice rink. We will pick them up one day, we promise lads. Well actually, on second thoughts, maybe not all of you...

However, there is another twist in this tale, a new 3rd generation TKI has come to the UK in phase 2 trials. The ARIAD trial: AP-24534. Ponatinib. And I have had the chance to be one of the first people in the UK to try the drug. It’s shown great promise in Phase 1 trials in the USA and I am eager to see what all the fuss is about. I figure that at its worst this drug keeps me out of hospital to enjoy the summer before a transplant in Autumn, but at it’s best I will finally get to see some reduction in my bad genes and pave the way for others in this new pioneering treatment.

So as all good lab rats discover, documenting the journey is all part of the trial. I hope this diary will help others dealing with CML. So, join this lab rat on her journey into unknown and fingers crossed I don’t grow a third ear on my back...