On coping with the Camel in the living room
Jude W. Vaz – Bombay, 2008
There seemed something cruel about the abruptness of it all.
After having spent a lovely vacation in Lucknow with the family we took a long train journey back to Mumbai. I had contracted a rather nasty respiratory infection which was exacerbated by the prevailing cold weather in the north. I was unable to get rid of the infection even after two courses of rather potent antibiotics, and was still troubled by a hacking cough some weeks later. Finally I decided to have a routine check-up and ran some blood tests. The resultant CBC report was the stuff of which shock and awe is made of: my white cell count had crossed 130,000 per micro litre. Moreover, the blood test was suggestive of chronic myeloid leukaemia. I was admitted into a hospital and a bone marrow aspiration was done which confirmed the dreaded diagnosis of chronic myelogenous leukaemia (CML). I guess I was overwhelmed but not shaken by the gravity of this personal catastrophe. Faced with a diagnosis of a deadly form of blood cancer I figured that I had only a few months to live and I had visions of being violently ill.
A dear friend and fellow pathologist steered me in the direction of a physician, a medical oncologist who was well-versed in the treatment of leukaemia. He was a genial man with a kindly smile and a charming Assamese accent who offered advice and support for dealing with the condition. He seemed quite sanguine in his approach to this disease, and when I asked him about the prognosis with a tentative query, “How long do I have to live?” he answered, with a twinkle in his eye, “Wait and see. This is a disorder which continues to offer hope and pose challenges. If the cytogenetic analysis shows that you are Philadelphia positive, I have something up my sleeve!” He assumed that being a pathologist I was cognizant of the Philadelphia chromosome, which of course I was. That evening I retreated to a nearby seaside promenade to be alone, to absorb the enormity of the diagnosis, and to watch the sunset. It was spectacular – clouds of salmon pink and ecclesiastical purple with a myriad shades of interspersed crimson made the sky appear like a surrealist canvas as the golden orb descended towards the waiting horizon. It felt great to be alive and experience the salt spray of the sea breeze in my face. Strangely in the face of this monstrous calamity that had struck me like a bolt from the blue I realized that I had come to personify Albert Camus’ quote “ In the depths of winter I finally learned there was in me an invincible summer.”
So much to do, so little done!
Strange thoughts raced through the troubled recesses of my mind. Being the same age as Tony Blair, Orhan Pamuk, Vladimir Putin and Mel Gibson (give or take a few weeks) places unwarranted demands on one’s ordinary existence. Churned by my recent indisposition the ghosts had returned to haunt me. Why had I not achieved any significant accolade yet? I always believed that somehow or other I would be famous, and now, with the finality of a sledge hammer’s stroke I realized that well ……this was it : the last act of a mundane life and then it was curtains for me. I would not be achieving any special honour after all. Like that memorable line from an old John Lennon song “Life is what happens to you while you’re busy making other plans”. It dawned on me then that most people die with much of their music still trapped inside them. I thought about all the places I wished to travel to but would never visit : the commanding Andean heights of Machu Picchu in distant Peru , the unblemished Wordsworthian countryside of England’s Lake District, Sikkim, that Shangri-La in the eastern Himalayas, and Egypt, once more the eternal encore . I thought of the books I always intended to read but hadn’t yet : “The Catcher in the Rye”, “To Kill a Mockingbird”, Emily Brontë’s “Wuthering Heights”, Amitav Ghosh’s “The Calcutta Chromosome”, Somerset Maugham’s “Of Human Bondage”, Thomas Hardy, Oscar Wilde, Katherine Mansfield ….. The unread list went on and on.
If God puts you to it, He sees you through it.
The Camel (“CML”) had strode into my well-ordered life and I had resolved to do battle with “the slings and arrows of outrageous fortune”. If CML needed a sobriquet what could be more appropriate than the Camel?! This great, prodigious unwieldy beast had cantered into the living room of my existence, uninvited, like a biblical mirage portending rough sailing ahead. The challenge lay in the fact that I had to rise above it, which I did, notwithstanding the fact that the camel is a tall animal! The signs of chronic myeloid leukaemia are sometimes discovered unintentionally during a blood test for some unrelated condition. My diagnosis had been incidental, and I was not gravely ill, but there were many decisions that had to be taken. My initial reaction of disbelief was now replaced by restrained acceptance. My wife had been informed and she handled it with fortitude, brushing away her tears and trying not to show me her sadness as she put on a brave front, but how would we explain to our two children who were young and still schooling, about the serious nature of my condition? In fact, would it not be wise to keep them in the dark for the time being? With regard to relatives we decided to inform only a few comprising the inner circle of our kith and kin , as we were not feeling up to dealing with the myriad phone calls and visits from concerned relatives that would ensue. So I dashed off a letter to a favourite nephew who was given the onerous task of informing the select few, being confident he would handle it well. The way in which things and events unfold does not always coincide with our expectations. The older you get, the more scary life seems to get. Here I was at the crossroads of fear and bewilderment but terror did not take possession of me. Confronted by questioning moments of negative thinking I had to pull myself together and resort to positive thinking. I remember reading that modern behavioural scientists have shown that positive thoughts release endorphins which promote a sound mind in a healthier body. It seemed only rational to choose this path in order to deal with the Camel successfully.
The detailed bone marrow report took more than a week to arrive. I had anticipated a long wait as it involved culturing the bone marrow cells and then having the chromosomes stained, photographed and viewed. I had to wait patiently for the report and that’s when I delved into studying about the cytogenetics of chronic myeloid leukaemia. It appears that a reciprocal translocation between the long arms of chromosomes 9 and 22 results in a shorter chromosome 22 known as the Philadelphia chromosome which is an acquired mutation. The Philadelphia chromosome bears the hybrid fusion gene BCR-ABL which produces the abnormal fusion protein Bcr-Abl with increased tyrosine kinase activity which activates signal transduction pathways that lead to uncontrolled cell growth of myeloid stem cells – an over-production of leukocytes, manifest as leukaemia. When my cytogenetic analysis report announced that I was Ph’ positive it was good news. At the beginning of the new millennium an innovative new drug called imatinib mesylate (Glivec) had revolutionized the management of CML, and it worked only if the patient was positive for the Philadelphia chromosme. Imatinib is a tyrosine kinase inhibitor that blocks the signal that causes the increased production of leukaemia cells. Moreover it combats the cancer by targeting only the errant diseased cells with uncanny precision, leaving the normal cells in the circulation. When it first came on the scene it was hailed as a breakthrough in the war against cancer. Before the advent of imatinib the only feasible treatment was interferon, an immune system booster that often didn’t work and had horrendous side effects. Bone marrow transplant is an option that is offered to younger patients and I did not qualify. The hydroxyurea I was initially put on was replaced by a daily dose of 400 mg Glivec, and within weeks my blood counts were back to normal. It was almost magical. Glivec is an astoundingly expensive drug which I could ill afford but once again the problem was surmounted. My physician put me on a programme run by the pharmaceutical company that manufactures Glivec that provides the drug free of cost to more than 33,000 patients in 80 countries across the globe. This patient assistance programme is managed by the Max Foundation, a non-profit organization established to honour the memory of a 17-year old Argentinian lad Max Rivarola, who fought CML with astonishing courage and grace before he succumbed in 1991. In acquiring Glivec from the foundation I had the pleasure of meeting with this amazing lady who manages the programme and support groups in India. Her warm approach and indefatigable drive have certainly infused courage in the 10,000 odd Indians afflicted with CML who are benefiting from this magnanimous programme.
God works in mysterious ways and I know that Glivec helped me reclaim my life.
In a way it made me a psalmist impelled toward praise and thanksgiving. It is unusual to make new friends late in one’s life, but I am grateful for the friends I have made in the wake of being diagnosed with leukaemia. Besides the phenomenal lady who manages the programme, I had the good fortune of meeting with a fresh-faced Peruvian who arrived out of nowhere and distracted me with his wonderful friendship that kept me from brooding about the disease I was suffering from and brought back laughter into my sombre existence. In a strange way he nudged me into spirituality as well. These new friends were life’s surprises – the kind that sneak up on you and grab hold of you.
God dispatches guardian angels in times of crisis!
Its been four years now since that fateful day in January 2004 when I was first visited by the Camel , but thanks to the magic bullet called Glivec I have been in remission ever since. It’s been such smooth sailing that I have not yet got down to writing my will! However, being a sufferer I can still say that in spite of having this marvellous remedy the Camel always hangs over your life like a Damoclean sword, as your white cells see-saw in an exquisite ballet causing a delicate balance between errant cells and normal ones and you are often left wondering about how much longer you have. Had imatinib not appeared fortuitously on the scene when it did I would probably be history by now, cold in my grave.
CML has taught me to count my blessings.
I shall die soon, I know.
This thing is in my blood.
It will not let me go.
It saps my cells for food.
It soaks my nights in sweat
And breaks my days in pain.
No hand or drug can treat
These limbs for love or gain.
– Vikram Seth’s “Soon”,
From: “All You Who Sleep Tonight”.
I had learned about the three phases that CML typically progresses through, and I had constructed a sustained metaphor to encompass the whimsical natural history of the affliction :
(i) the Chronic Phase : this is the initial stage when the Camel grazes unruffled in the oasis of one’s domestic domain. This is when the patient bonds with the Camel and learns to respect his disease. The Bedouin Arab I am told , respects the gangly camel who he believes is a superior animal because it knows the unrevealed hundredth name of Allah ; mankind, according to Arabian folklore knows only ninety-nine !
(ii) the Accelerated Phase : the advanced stage when the Ship of the Desert gets unruly and prances around the drawing room, upsetting the furniture and sometimes causing the patient to dismount.
(iii) the Blast Crisis : or terminal stage when the Camel, possessed by unbridled madness, goes wild and gallops uncontrollably out of one’s living room into the stark desert sunset . I reckon every CML-afflicted person stands in utter dread of this last overwhelming phase and prays for its deferment. The Camel is an unpredictable creature and even though currently quiescent, may yet accelerate and blast !
You live and learn – and then you die and forget it all .