Waiting

...and so the condemned man waits for the punishment to begin. Because WM is such a sluggish disease (I believe indolent is the technical term) this march towards another set of treatment seems interminable. Dates have been set and diagnostic tests and scans arranged, I've had a leaving lunch with my friends from work and a good luck curry with my friends from St Albans; all the various bits and pieces have slotted into place, and still I really don't feel ill in the slightest. So all there is to do now is to wait for it to begin. And doesn't time run ever so slowly when you're waiting, even bizarrely when you don't want the thing for which you're waiting to arrive?

For the CHOP

There's been an inexorable slide towards this moment. I knew it was going to happen but somehow that doesn't make it any easier to take. I've got to endure another 6 months of chemotherapy and this time I'm for the CHOP, or to be more specific R-CHOP. Last time around I only had to take the one drug, Fludarabine in tablet form. This time it's the full- go to hospital and have the treatment through a needle (oh yes one of those again...) - deal. And it's not just the one drug this time but an exquisite combination of nasties.

C - Cyclophoshamide (causes hair loss and infertility)
H - Hydroxydoxorubicin (hair loss and heart toxicity)
O - Oncovin (aka Vincristine...I'm not having this because it causes peripheral neuropathy)
P - Prednisone ( A steroid which can cause mood swings, water retention and kill bones)
R - Rituximab (imunotherapeutic agent; not chemo. Can kill during first infusion.)

lovely. I can't wait. It's a three week cycle with the CH and R going in on day 1 and the P in tablet form for the next week and we then start the whole thing again on day 21. They want to do between 6 and 8 of these cycles depending on how it goes. The most difficult thing for me to get my head round is the fact that I don't feel at all ill. The only symptoms I have are slight breathlessness when going up stairs and dizzyness when I get up too quickly out of a chair, both of these due to anaemia. I chart this cancer through numbers and graphs on a sheet of paper, and the numbers say start treatment. So in November I say goodbye to my colleagues at work for another 6 months and prepare to feel like shit. It had better be worth it.

I Don't Believe it!

So after all the stress, the worry, the not knowing why I had to see the doctor so far ahead of my scheduled appointment I finally got the answer today.

Administrative cock up.

Someone (unknown apparently) cancelled my November appointment and re-scheduled for today. My IGM is holding steady but it is still on the high side. No alarms. No need to go immediately onto chemo.

AAAAAAAAAAAAAAAAAAAAAAAAAAAAAAAAAAAAAAAAAAAAAAAAAGH!

Thank you NHS.

Worry

At my last appointment with my specialist I was told that my IGM levels were going up and that there was a possibility that I may have to have some more treatment in the Autumn. We scheduled another appointment for mid-September when we would discuss the results of the blood tests that were run that day.And then today...through the post I received a new appointment card (with my name and hospital number) on which someone had written the date and time of an appointmet next week; August 8th to be exact.

I am, to put it mildly, perplexed and not a little pissed off. No letter from the consultant explaining why there is a need to bring my appointment forward, no phone call from the secretary asking if this date (at such short notice) is convenient, (it isn't.) no common bloody courtesy whatsoever. Let's be blunt about this...it is not going to be good news. Doctors and hospitals do not move at such giddy speed for a routine cataract operation or to tell you that all is well and there is no need to worry. No. This can only mean that the blood results were not good and we need to get on with some treatment pdq. Christ on a bike, the cancer might have turned from the benign one I was diagnosed with into something altogether more malicious, (like multiple myeloma for example) my IGM might have spiked to levels where urgent action is required or they might simply want to recommend that we start the treatment we were discussing a little bit earlier. The point is I DON'T KNOW.

I touched on this area in an earlier post. The medical profession seems to me to be divorcing itself from one of the founding principles of the NHS, patient welfare. Communication with cancer patients is absolutely crucial as our lives are quite literally at stake. Yet we are told of our illness with clinical efficiency and sent appointments through the post without any explanation. The effect of this is to cause patients a degree of worry and stress that they could simply do without. Does no one actually think about what getting an unexpected appointment through the post could do to a patient's state of mind? The simple fact is that some basic priciples of patient care are being neglected.I shall go to my appointment (having taken a day off work) and for the first time ever I shall let the consultant know how I feel in no uncertain terms. It's not right to treat people like this.

Going Up!

I mentioned in an earlier post I think how we WM sufferers obssess about our IGM levels, and with good reason as they are a pretty reliable indicator of how the disease is progressing. My figures have been pretty good for two years now but the last couple of readings have shown a small but distinct upward trend. I had hoped that this might be a "blip" and that they would go down again but it seems that this is not to be.

I'm not quite sure what I think about this yet. I've pretty much managed to put all thoughts of disease and cancer to the back of my mind and get on with life again, and this hasn't been that difficult as I have been feeling very well. Now I face the prospect of another round of chemotherapy in the Autumn with all that that entails; time off work, long visits to hospital, needles (yuk), feeling like shit, losing my appetite, losing weight. Actually the last bit is no bad thing as I have packed on the pounds recently, unfortunately it is a rather drastic kind of diet. During the last round of chemo I remember my wife heading off to work with the words, "don't forget to eat" as I could easily go through the whole day without feeling the need or the desire to feed myself. Anyway, there's no getting away from the fact that I face the prospect of a fairly crappy few months, a prospect that I suspect I haven't fully come to terms with yet.

I think the technical, doctor-speak, explanation for what's happening to me is that I've "relapsed." I have several problems with this, not least of which is that it makes me sound like some young thug who has reneged on his asbo agreement. Relapse has the implication that the patient has somehow not been taking their medicine, or not looking after themselves properly and that this is why the disease has started to rear it's ugly head again. It's also a label that attaches itself to the long-term cancer patient and not the newly diagnosed, and so I have to re-adjust the way I view and describe myself. I am now one of the "old-lags," the old geezer in the cell down the wing who knows the ropes and can show the newbies the ropes. "Ah, Fludarbine is it? Oh yes I've had that, I've had 'em all mate. Have you tried that Rituximab yet? Oh and by the way, don't turn your back on Dr Smith if you know what I mean!"

The other thought that crosses my mind at this point is that I shall be given a different drug or set of drugs to the one I started on. There are only a certain number of drugs that are efficacious in WM and the list dwindles as you get through them. I've heard them called "silver bullets" and we don't have that many in the armoury. I've used up one and by the end of the year will probably use two more. There is a chance that some of these treatments can be used more than once, but when the disease starts to get resistant (or refractory in doctor-speak) to them you can cross them off the list for good. Let's hope that all those good people working in silver bullet factories around the world can come up with one that will actually slay the beast rather than just wound it!

Irritating Gargantuan Molecule

Yes it's our old friend IGM. What is it? "IgM is by far the physically largest antibody in the circulation" says Wikipedia. "The class of antibodies found in circulating body fluids and the first antibodies to appear in response to an initial exposure to an antigen" according to yourdictionary. "IgM is a large pentameric structure, whose higher molecular weight tends to confine it within blood vessels" says gpnotebook.

Immunoglobulin M (to give it it's full name) is often held up to be the villain of the piece to those of us who suffer from Waldenstroms. WM affects plasma cells, which develop from white blood cells called B-lymphocytes, or B cells. Some of these B cells become plasma cells, which make, store, and release antibodies.In WM abnormal plasma cells multiply out of control. They invade the bone marrow, lymph nodes, and spleen and produce excessive amounts of IgM. As we've seen IGM is a huge molecule, the "buster blood vessel" of the antibody world, and it's therefore not advisable to have too much of it. As the plasma cells pump out this stuff your blood begins to get thicker and thicker; it's called hyperviscosity and it's not much fun.

"Confusion and mental status changes result from the increased viscosity of the blood and decreased cerebral blood flow. This sludging leads to segmental dilatation of retinal veins and retinal hemorrhages. Mucosal bleeding may occur from prolonged bleeding time caused by myeloma proteins interfering with platelet function.
Cardiopulmonary symptoms such as shortness of breath, hypoxemia, acute respiratory failure, and hypotension also result from this sludging of blood and decreased microvascular circulation." (eMedicine)

Before I knew I had WM my IGM soared unchecked and my blood started to turn to treacle. I was sluggish and lethargic; I woke up everyday with a headache that I just couldn't seem to shake off; the slightest bit of physical exertion left me short of breath and dizzy. I was unaware that I was terribly ill. One of the abiding memories I have of that time is trying to play a game of football with my daughter on the beach and having to stop not only for a breather but a full-scale lie down after only a couple of minutes. The treatment for acute hyperviscosity is called plasmapheresis. I've described in an earlier post how this involved the insertion of an unfeasibly large needle into my left arm through which my gloop filled blood was passed to a large centrifuge. I felt instantly better, clear-headed and much more alert than previously. My joy at the instant success of this treatment was tempered rather when it was explained to me that this was only treating the by-product of my cancer and not the cancer itself; that of course would need chemotherpay. Bollocks. I'd thought I might get away without needing it. No such luck.

I and all other WM sufferers obssess about our IGM levels. We have regular blood tests to monitor what it's doing. WM is a disease that presents differently in every individual it affects so that we all become symptomatic at different levels of IGM in our system. For some it's very low, others can seem to get on quite happily at astonishingly high levels. For all of us it's the way we trace the progress of our disease and the effectiveness of the treatments we take for it. After my 6 months of chemotherpay I have watched happily as my IGM levels slowly but steadily fell month on month. It had to happen I suppose but now, just over 3 years down the line with a nice partial remission going on, it's started to creep up again. It's just one reading, and it might be a "blip." Watch this space.

Jan the Man!

Words cannot express the debt of gratitiude we owe this man. He is of course Dr Jan Waldenstrom, without whom the rare blood cancer from which I and thousands of others suffer would be known as lymphoplasmacytic lymphoma. Thanks to him it rejoices in the rather more splendid name of Waldenstrom's Macroglobulinemia or WM for short. The good doctor was a Swedish physician who was born just after the turn of the last century and almost saw it out, dying in 1996. He made the discovery that brought him into my sphere of reference as recently as 1944 when he described patients suffering from a disease characterised by "hyperviscosity syndrome" in which abnormal lymphocytes crowd out the bone marrow, leading to anemia and an enlarged liver and spleen. Research has come on only sporadically since then and there is still no cure for the condition, nor indeed a prescribed course of treatment. Because WM is so rare, not enough research has been done on it specifically. Instead, doctors tend to look at what works in closely related diseases such as Multiple Myeloma and Chronic Lymphocytic Leukemia, and then try to use similar approaches on WM.

So if Jan Waldenstrom were around today do you think that he would be happy at this state of affairs? I doubt it very much, and it's a shame that the great man is not here to drive forward and provide a focus for the latest research work. Anyway....before this blog starts to sound too much like a medical textbook I shall wrap up this post by saying why not read Jan Waldenstrom's obituary here and adding that I thought that a blog that had his name at the top ought to feature his picture too. So there you are!

RIP My Career

Cancer claims many victims and we mourn them all. But we can't count it's cost in lives alone, as I've learned. Five years ago I was getting along rather well at work. I've never been what you would call an ambitious person, I don't get on too well with greasy poles. (The climbing sort you understand.) Nevertheless after a period of unashamed idleness on my part (which lasted for most of my thirties) I had decided that for the sake of my wallet, and possibly my sanity I ought to show an interest in promotion. In my department career development operates on a "dead man's shoes" basis, so the opportunity for advancement is rare. It was with some excitement therefore that we greeted the news that our immediate boss was retiring and that he was going to be replaced with not one but two people.

I wont bore you with the ins and outs of the selection process and the various hoops through which I was obliged to jump. I didn't get either job and I took it rather badly. Perhaps I had been too complacent going into the interviews, been too certain that my years with the department and my popularity with my colleagues would carry me through. It was not to be and I slumped into a malaise that lasted far far longer than it reasonably should have. I now know of course why that was. Anyway, at this point feeling snubbed and a little bruised I started my campaign to leave my departmental home of many years and try and get work elsewhere, and began with an attachment which was designed to fill out my CV and give me a broader range of experience. It was hard work but thoroughly rewarding and I began to see that there was a working life beyond my current boundaries. Moreover the contacts that I had made gave me the confidence to believe that should I put in an application for a full time post it would not be dismissed out of hand. All through this period I felt desperately and wretchedly tired. I put it down to the stresses and strains of working in unfamiliar surroundings and very long hours, and it was with some relief that I set off to Portugal for a family holiday in the sun. Things got no better there, however, and I felt so utterly drained of energy and devoid of any desire to do anything at all that I made that now fateful appointment to see the GP on my return.

And so the landscape has changed beyond recognition. Those tentative efforts to spread my wings have been well and truly scuppered. I find myself back in the same old job in a department that has been phenomenally generous to me (in terms of time off and working arrangements) and here I shall probably have to stay. What way out is there for me? I am in remission now but there's no way of knowing for how long it will last. Would the extra effort required to find and get a new job actually make me ill again? And if it didn't for how long could I find that extra energy? And let's be honest, what prospective employer is going to take on someone who is likely to have extended periods of time off for treatment and the possibility of having to give it all up anway because of ill health? It's unsettling to think that at 42 I might have achieved all that I ever will in my working life. Is my new "career" to make sure that I stay healthy?

Red sinks

"In Waldenstrom's macroglobulinemia, abnormal plasma cells multiply out of control. They invade the bone marrow, lymph nodes, and spleen and produce excessive amounts of an antibody called IgM. Excess IgM in the blood causes hyperviscosity (thickening) of the blood."

Ever wondered what it might be like to have blood the consistency of strawberry jam? I exaggerate (obviously) but that is roughly the effect of having loads of IgM coursing through your veins. We all have it...it's part of the body's defence mechanism. Ig stands for Immunoglobulin (These types of cell are also known as antibodies) and there are many of them: IgA, IgD, IgE, IgG and IgM, with 4 IgG and 2 IgA subtypes. IgM just happens to be one whopping big molecule, by far the biggest of the antibodies, and when you have too much it starts clogging up the system. If left untreated it can cause heart failure and strokes. Just before I was diagnoised, when my IgM level was at it's highest I felt constantly sluggish and thick-headed. Every morning I woke up feeling hung over (even on days when I hadn't been drinking....honest) and went through my days with that "one step removed from reality" feeling you get when you've got a head cold...as if you're viewing the world through a fish tank.

Now here I am on the other side of plasmapheresis treatment (BIG needles aaarrgh) and my head is clear, like I've had a steam inhalation of peppermint and eucalyptus. But my blood is still thicker than it should be and that for me has one minor but irritating and unpleasant side effect. As the thick IgM molecules push their corpulent way through my smaller veins and capillaries they put them under enormous strain. The veins bulge and stretch and in some cases burst - either spontaneously or when agitated by something....like a toothbrush. Even with the gentlest of brushing, when I rinse and spit there's always a reddish brown tinge to the water. The red trickles between my teeth start almost immediately the brush has done it's first scrub and a smile at the mirror reveals a mouth that Count Dracula would be proud of. It's a daily reminder of the cancer within and an embarrassment that leads me to make sure no one's around when I'm cleaning my teeth. To have blood seeping from your gums is not a great way to start the day, and certainly slows you down as you wait for the flow to stop before you leave the bathroom. Originally I thought I must have gingivitis....how I wish it was.

I'm one of the lucky ones. For some this bleeding happens in their eyes and impairs their vision. For others it happens in the brain. What do I see looking into a red sink? If I'm feeling morbid (which is rare), my demise, a visual sign of the excess IgM that may ultimately get me. If I'm feeling cheerful (which is mostly) relief that so far hyperviscosity has been no more than an inconvenience.

Needles

When I was at university I gained a reputation for being somewhat lazy, and I have to say that it was fully deserved. Never at my best in the morning I would regularly stagger bleary-eyed from my room at around midday to the jeers of those others who had already attended several lectures or been rowing on the river and who were just about to head off to college for lunch. On one famous occasion I rose as the classified football scores were being delivered on Grandstand. Nine o'clock lectures were a physical impossibility...eleven was pushing it. You can well imagine then the mirth with which the news that my first job would entail getting up regularly at half past five in the morning was received. The general view was that this was in some way "pay-back" for my years of idleness and sloth.

This jaunt into my past is occasioned by the fact that I believe the same forces are at work when in comes to my cancer. I have always been terribly squeamish; the slightest hint of the red stuff and I would collapse into a dead faint. I even passed out once at school during the screening of an educational film about cataract operations in Africa. Needles terrified me. Every blood test was an effort of will on my part first to turn up at all and then to remain conscious during the procedure. Most embarrassingly I remember waking up on the floor of my local health centre a couple of years ago with two worried nurses staring at me, probably wondering why such a big strong boy was actually a quivering wimp.

And so it was that I got a lymphoma. Someone or something has obviously got a sense of humour. The tumour (such as it is) is located in my bone marrow which required the use of a spectacularly long needle to pierce my hip bone and remove some of the marrow so that the docs could have a look. They then broke it to me that one of the consequences of this cancer is that your blood becomes extraordinarily thick and that mine was probably about the consistency of treacle. So it was that I was sent for plasmapheresis where they hook you up to a large machine and run your blood through a sort of centrifuge to remove the gloop. In order to do this they had to insert a needle so large into my left arm that it required a local anaesthetic in order to get it in. I thought during all this, and as I contemplated the years of frequent needle-based tests, that the process must be similar to putting an arachnophobe into a room full of tarantulas. I'm making progress - I don't pass out any more and I enjoy Tony Hancock's "The Blood Donor" even more than I used to:

"Nurse: needles don't bother you then?
Hancock: Me? No. I've had too many of 'em my dear. I've had the lot; got arms like pin cushions. Yes I reckon I've had a syringe full of everything that's going in my time. Needles the size of drainpipes some of 'em! You name it I've had it."

Quite.(Watch it here. )

So, what goes around comes around. Is there some kind of cosmic order? Are these things ordained? Or is it just sod's law? I could ask "why me" but I'd have to get up bloody early to find an answer.

On diagnosis 2

I just want say a few things about the breaking of bad news. Now obviously I'm coming at this from one side of the equation and receiving a diagnosis of a life threatening illness is never really going to be taken with equanimity, but in my experience (and those I've met in a similar position) it could be done a hell of a lot better. One blogger, who is a doctor, puts it rather well and like this...

"The first time a doctor breaks bad news to a patient, or tells a family about the death of their loved one, it's an emotionally difficult thing to do. The subsequent times are difficult too. Every patient is different, and the doctor relaying the news has to be prepared to deal with the fears, emotions, questions and comments of the person or people to whom he relays this news. Breaking bad news is never easy. But (and I'm almost ashamed to admit this) it gets easier."

The doctor who told me I had an incurable cancer must have delivered this kind of bombshell hundreds of times before me and doubtless many times more since. It's his job after all. For me it's a one off. And yes I'm sure that he must have either got used to it over the years or distanced himself emotionally in some way but the result was that I was told the most awful news in manner resembling an accountant discussing an overdue tax return - Yes it's crap but not worth getting flustered about. He was calm, precise, thorough...and completely and utterly devoid of any kind of humanity whatsoever. Not a spark. Not a glimmer. Even when my tear-soaked and sobbing wife asked him if he could offer any hope he singularly failed to connect with us on a basic human level. To make a bad situation worse the room in which this dreadful business was transacted was not a place set aside for the purpose but a grotty, airless little cubby hole where various supplies for the ward were kept. Thus it was when we were given some time to be alone we were constantly interrupted by people looking for pots for urine samples.

Yes I appreciate that breaking bad news is a shitty thing to do and that to stop yourself becoming clinically depressed you have to remove yourself in some way from the reality of what's going on....but do doctors really have to do this at the expense of their compassion and humanity?

On diagnosis

Oh deary me where do we start? Where to begin to catalogue the events that so dramatically changed your life? For instance, where exactly is the beginning? It could be the creeping lethargy, the inabalility to get going in the mornings, the reluctance to play with the children because of the energy involved. It could be the call from the GP asking if I could come in immediately to discuss my blood tests. Would it be when the consultant uttered the dread word cancer? Or perhaps it begins when the first tendrils of chemotherapy snake themselves into your bones to destroy everything in their path? Whichever one you choose they're all pretty grim.

The official start , however is the diagnosis. This is when the wheels of the NHS start to turn in your direction and when you realise that when it works it's a pretty amazing thing. It begins with a vial of blood, taken at the request of the GP and dispatched to the local path lab for analysis. Routine. Or so I thought. The phone rings. It's Wednesday afternoon. It's my GP. Could I come in for a chat about the results? Now. The GP spoke softly, mentioned "anomilies," indicated further tests would be needed, "to be sure." But she was pretty sure it was something serious. Within hours I was admitted to the local hospital because they too thought it was something serious. The consultant who'd first spotted the problem arrived with a very long needle which she informed me she intended to stick into my hip bone to perform a bone marrow biopsy. This would confirm what the something was and just how serious. It was left to consultant number two to fill in the details. The something was cancer. A lymphoma. Waldenstrom's Macroglobulinaemia.

GP consultation - Monday morning Diagnosis - Friday afternoon. Five days and your life's in freefall.

Fit the First

I've been what's known as a lurker for the past few months, reading the wealth of material to be found on all the myriad blogs that there are out there. (Bit pejorative that term lurker don't you think?) Anyway I've decided to join the fray and contribute some of my own thoughts and experiences on life with a rare blood disorder. Waldenstrom's Macroglobulinaemia. That's what it's called. That's what I've got. And they can't cure it.

It's a NHL or Non Hodgkins Lymphoma which is cancer of the lymphatic system. There'll be more on what having it actually involves in subsequent posts. Suffice to say that I've had the chemotherapy - with more in the pipeline - and the possibility of a bone marrow transplant (or autologous stem-cell transplant to be precise) to look forward to; so there's plenty to talk about and plenty of treatment related issues to get stuck into. Should be fun!