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.