The Not-So-Gentle Answer: 2. Chronic Lymphocytic Leukaemia

My last “how are you?” blog was well received, I thought I’d continue with another episode of not-so-gentle answers.

The disease I am being treated for is called Chronic Lymphocytic Leukaemia, a cancer of the B-cell white blood cells.  It is the most common type of leukaemia, usually diagnosed in patients over 50 (but not always), and usually in males (but not always).  Mine was found when I was 46 by the Red Cross Blood Service, but please don’t let that stop you donating.

There are some common chromosome markers for CLL, however it is not inheritable or congenital. One of these markers is tested for with a ZAP-70 test.  If it is negative, you will likely have a natural lifespan without needing treatment. My ZAP-70 was positive, making the more aggressive form of the disease the most likely.  However, it would be another four years before it was treatable.

The usual treatment for CLL these days is known as CFR (fludarabine, cyclophosphamide, and rituximab), but in 2010, only “CF” was on the PBS and “R” was not.  Fludarabine and cyclophosphamide operate by disrupting cell mitosis, the process by which cells reproduce.  For example, cyclophosphamide forms chemical cross-links between DNA strands; this is irreversible and leads to cell death during mitosis.  Here is a very informative talk and video about cell mitosis, note how many fragile things have to all go “just right” for cells to divide successfully.  The genetic markers, mentioned earlier, are places in the human chromosome where errors of reproduction (a form of mutation) are more likely.

The problem with messing with cell mitosis is that there is a staggeringly high rate of “friendly fire“, and the most visible symptoms of this are hair loss, mouth ulcers and nausea: all of these processes involve cells that must reproduce often.  To minimise this friendly fire, we wait until the cancer cells are the fastest reproducing cells in the body.  This is why I had to wait until 2010 before the disease could be treated.

My chemotherapy in 2010 started in February.   After four cycles the blood counts looked good, but a CAT scan revealed that the lymph nodes in my abdomen were not getting any smaller.  Lymph nodes are where many of the useless leukaemia cells hang out.  So, out of my own pocket, I paid for MabThera (a brand name of Rituximab) at $5000 per dose, for four doses. Our savings were already wiped out (I’d been unable to work for over 12 months by then), so our mortgage got bigger, but the lymph nodes got smaller, so that was good.

MabThera operates in a completely different manner than the other two drugs.  It is an antibody that binds to a protein on the surface of B cell lymphocytes, destroying the cells, and is therefore used to treat diseases that result in too many lymphocytes.  This still has “friendly fire” consequences for good lymphocytes, and also it only attacks mature cells, and does not target the underlying defective progenitor cells.

Speaking of hair loss, for people with straight hair like mine, when you get your head hair back after chemotherapy, it is almost always curly for the first few months before eventually straightening out again, this is called “chemo curls” (it has something to do with the shape of the hair follicles).  This was my one and only ever chance to have curly hair.  (My dad had wavy hair, and he lied when he said all I needed to do was eat my crusts.)  But my hair didn’t fall out, not a one.  So, I’ve been gypped, ripped of, short changed, grrr.  MT was looking forward to hugging a man without 3cm of fur in the way; that didn’t happen, either.  And, yes, I know folks with curly hair often beg for theirs to be straight, but I would have liked the chance to find out why, hmm?  Ahem… where was I?

Treatment finished September 2010, with an optimistic estimate of 5 years, maybe 10, before I would need chemotherapy again. But it wasn’t all smooth sailing, and 20/20 hindsight tells us the leukaemia was still actively messing with bone marrow and my blood and my immune system.  My neutrophils never recovered to normal levels, and neither did my platelets. I was hospitalised early in 2011, then over-extended myself training, and camping, and travelling overseas. More and progressively worse infections followed (pneumonia for five months) requiring more hospital time in 2012. Through it all the lymphocyte count was rising much faster than the “five to ten” estimate. One of the bright spots in this time was that MabThera was added to the PBS 1-Dec-2011, woo hoo, because we couldn’t work out how we could afford it this time. Another hospitalisation and the lymphocytes went crazy, so my reward for recovering from pneumonia was immediate chemotherapy.  You can’t have an infection and chemotherapy, the drugs will kill you.

In 2010 we were able to start chemotherapy exactly on time, but in 2012 we were months late in starting, due to the infections, and the CLL was way more advanced.  In April we had an appointment with the oncologist, and she had The Death Talk with us, and it wasn’t easy for anyone in the room.  If I don’t have chemotherapy I will die (not eventually, either, but within six months) and if I do have chemotherapy I may still die.  To make the situation more complicated, it may be that my bone marrow has been “merely” overwhelmed by the cancer factory (in which case my blood will recover), or it may be that all the good cells in my bone marrow are all dead (in which case my blood will never recover).  We can’t tell which is the case until the chemotherapy starts to make some progress, in maybe 3 or 4 months.  If my bone marrow is dead, even if the chemotherapy works, I will die unless I receive a bone marrow transplant, but even if I receive a bone marrow transplant I may still die.

This was hard to process.  It was hard to take in.  It was scary as… as… there is no comparison.  Even my “as real as possible” self defence training doesn’t compare. Where is the gift in that news?  How do I accept that news?  Soon after I was hospitalised again, and, let me tell you, the hospital social worker got an ear full.  I had to say some of this stuff out loud, and I didn’t want to add to MT’s burdens.  I had to say “I could be dead soon” out loud, to a person.  If you have CLL, or any cancer, I recommend you get a psychologist (if you don’t trust the first one, ask your GP for anther referral, until you do).  I have one, have had for a few years, now.  You don’t have to be nuts or depressed to consult a psychologist. Instead, they are an uninvolved observer who can help you with techniques and strategies to cope with all the crap, to help you avoid depression and hopelessness.  I saw my psychologist asap after I got out.  It’s taken a while, over six weeks, and my reasons will not be your reasons, but I have come to accept this gift, this sharp spiky morning star of a gift.  Every now and then, one of the spikes gets me, and I’m left weeping.  It isn’t easy.

The first cycle was in May.  Each cycle was supposed to be like last time, a week of treatment, followed by three weeks to recover.  Except this time my blood isn’t recovering as well or as fast, and we can’t start the next cycle until my neutrophils rise to safer levels (but still below normal), so we wait.  And that brings us up-to-date.  Next week, I’ll cover some other aspects of my blood and their implications.

I have confidence in my oncologist, and I have confidence in my treatment.  I accept this gift.

You may be interested in reading the other Not-So-Gentle episodes: (1) Introduction, (2) CLL, (3) Neutropenia, (4) Platelets, (5) Commensals, (6) Infections, (7) Migraine.