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!