Battling microscopic foes: the IS or Immune System connection
Note: Let the reader be advised that I am neither physician nor nutritionist nor scientist. The thoughts expressed in this narrative are strictly for the purpose of recounting my early experience with a certain cancer and it's apparent spawn of para-neoplastic pemphigus (PNP). My grim Non-Hodgkins Lymphoma cum PNP experience should not to be viewed as templative for another similiarly afflicted patient but rather as how it directly applied to me. It is hoped that the descriptives, including examples of self-reliance in dealing with the consequences of immune system failures and physician failures, will serve to encourage and illuminate.
Reality check
It is not rare to contract cancer. It is rare however to contract pemphigus. It is extremely rare to be afflicted with both these fearful diseases simultaneously. An IS inherently fails when cancer establishes itself in a human body. An IS fails in another instance when its components initiate defensive measures attacking innocent body tissue 'perceived' to be alien to its host body. In my case my IS failed in both type situations to genuinely 'protect' me within the performance parameters of its innate biological purpose. One cannot separate oneself from ones IS during its times of failure. But when addressing such issues one can at least exercise control of resultant medical care to the extent of being proactive and have the small satisfaction of jettisoning any or all physicians along the way who fail to provide related quality care!
The big C and me
My cancer time-line officially began in June 1999 with the diagnosis of Chronic Lymphocytic Leukemia (CLL) via bone marrow aspiration. At that significant point in time I had asked my then oncologist if there could be added diagnostic value in doing a CAT scan. None was ordered for me. That physicians decision and my laypersons acceptance of it continues to haunt me. There is a saying that an ounce of prevention is worth a pound of cure. Early detection of my ensuing lymphoma would have been to my benefit.
CLL is an 'indolent' or slowly progressing form of leukemia - much too many cells of a kind - involving B-lymphocyte cells. B-cells (B for bone origin) are generally destined to journey through the circulatory and the lymphatic systems of the ones body. They normally adhere to a strict biological life span. In CLL, because of unknown precipitating mechanism(s) a single B-cell, somewhere, while in marrow or blood or lymph or tissue, becomes cancerous in behavior. It ignores genetic blueprints of timed death. Its self-replicating descendant generations of B-cells via its own clonal behavior - follow suit and accelerate in numbers. This phenomenons resultant leukemic 'overflow' progressively elevates the population of abnormal B-lymphocytes in blood plasma and lymph fluids. With bone marrow infiltration, the capacity to produce other blood components such as platelets, red cells etc may be severely compromised - crowed out. Other organs and tissues are equally susceptible to colonization by many such clonal B-cells and or tumorization from a single clonal B-cell.
'Watch and wait' - with routine physical and blood tests - in theory, when properly applied, is the prescribed general medical approach in still dealing with applicable CLL patients. In April 2000, following a demonstrably failed application of the 'watch and wait' and a no CAT scan approach, a needle biopsy which I had selected from personally researched options - over a planned surgical biopsy procedure under general anesthesia ordered by my then oncologist - led to a diagnosis of Non-Hodgkins Lymphoma (NHL), an extension of my CLL. My NHL were the end result of a prolonged and clinically undetected buildup of B-lymphocytes in a number of lymph nodes in my abdomen. One such lymph node had expanded into a lymphoma 17cm in length and was directly responsible for 75% permanent loss of function of my right kidney and the cascading effect of permanent damage to the retina of both eyes because of a hypertensive crisis - 'the' precipitating event which required ultrasound and CAT scan investigations. Lymphomas associated with NHL/CLL and of 17cm in length do not 'grow' overnight hence a CAT scan would have been revealing 10 months earlier.
Front line chemotherapy for NHL/CLL began in September 2000 under a new oncologist and with a drug named Fludarabine. Rituxan, a newer mono-clonal chemotherapy agent, was also administered to me during April and May of 2001 as a pre-planned 'mop up' drug. The apparent benefits of my chemotherapy were to be: remission of my NHL/CLL to date - there was and is no cure and lymphoma albeit diminished vexingly remain; remission to date as well from the visible affliction of para-neoplastic pemphigus (PNP). Conversely the dis-benefits during my chemotherapy gauntlet were the establishment of atrial fibrillation from a support drug called Numega, which promotes blood platelet production, together with a further pile on by the later development of para-neoplastic pemphigus (PNP). What caused my PNP? PNP is linked to neoplasms, as in NHL/CLL. Not every NHLr gets PNP however. To add mystery in that regard to my medical case, one interesting result from an internet search on NHL & PNP yielded the following: reported from the Department of Dermatology, The Oxford Radcliffe Hospital, Oxford, UK. (June 2001), it was described that 3 adult men treated with a drug called Fludarabine for Chronic Lymphocytic Leukemia (CLL) thereafter presented with confirmed Paraneoplastic Pemphigus (PNP). Hmmmmmmm. Coincidence or correlation? Neoplasm or Fludarabine as my genuine PNP cause?
The big P and me
My pemphigus time-line unofficially began in Summer 2000 with mouth sores. Diagnoses were first herpes simplex, then stomatitis; nothing prescribed, worked. I was at one point seen by a dermatologist who taught at Yale University medical. Chemotherapy for my NHL/CLL commenced in September 2000. Soon after, red spots began to appear on my skin; an accurate diagnosis was that of Lichen Planus; its cause - perhaps IS related, perhaps from chemotherapy, perhaps from 'support' drugs - was not pursued as my cancer was focal of concern.
Chemotherapy proceeded otherwise, where noted to the contrary, expectantly as to side effects. Bone marrow are always impacted significantly by the drug Fludarabine; I required several postponements from the customary pattern of administering the Fludara while cell counts recovered sufficiently to continue its use. Exercised caution, notwithstanding Procrit and Leukine support drugs used to promote red and white blood cell productions respectively.
In February 2001 it was on a treatment day of my last scheduled cycle week of the Fludarabine infusion for my CLL/NHL when I noticed a single clear blister the size of half a pea on my right forearm - my mouth (mucosa) was still an unhealed bloodied mealy mess from earlier onset. Within a day larger wrinkled blisters began to appear on my inner thighs and would slough off at the slightest rubbing effect leaving raw flesh in its wake. More blisters followed on my torso and limbs with identical vulnerabilities to touch. These curious developments were reported promptly to both my oncologist and to my then second putative dermatologist. My oncologist was mystified as to my new skin problem - I was taking Acyclovir (anti-viral), Septra DS (to prevent septicemia or blood poisoning), Allopurinol (to protect kidney function); he deferred to my newest dermatologist to diagnose the cause of my blistering.
This nameless dermatologist demonstrated, in short order, practiced unprofessionalism. Three biopsies taken of my skin including my lips were stretched over several weeks. While waiting for time consuming lab results, blistering continued on my body and began about the eye lids. My prescribed remedies were the use of topical cortisone, antibacterial creams and baby shampoo with Q-tips - three brilliant prescriptions for me. In hindsight, I shake my head at the laid-back effort of that derm given that there were volumes on the subject of skin disorders, with photos, to research and colleagues to plumb knowledge from and yet the application of none of those resources was evidenced on my behalf. At a point of exasperation I turned to my internist for help. My internist acted, unhesitatingly, by prescribing short term oral prednisone use at a formulation of 60mgs/day to begin and for several days thereafter; to be followed by successive reductions of 10mgs with each new multi-day cycle until down to zero - that was the plan. That initial 60 mgs/day began to visibly suppress new blistering/lesions from occurring - the miracle of Prednisone, when timely and applicably used. By this time however my body was a patchwork of cotton - basted with Bactroban (a bactericide) cream - which had been applied to raw flesh. And the cotton adhered. And I chose to allow the cotton to remain in place as a sealant; in time as new skin developed beneath, the cotton would easily separate, exposing a 'skin' glazed-like in appearance. This 'method' of dealing with absent skin had been an improvisation. Band-aids were found to be useless - their stickiness all too often helped create fresh wounds when removed from seemingly safe skin bordering affected skin: as example a nurse ignorant of pemphigus and meaning once well peeled off a bandage and exclaimed " ooh, it came off " referring to skin attached to its adhesive. Vaseline pads were messy/slippery. And for psychological reasons, reduction of caregiver time spent in addressing sores was deemed important.
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Ultimately, each pemphigus patient will have researched and experimented in search of their own solutions as to techniques they would rely upon for treating the damaged skin/mucosa on/in their bodies; ideally a favorable ratio of maximum comfort and protection of skin/mucosa to minimum expenditures of time and emotion.
A giant step backwards
Abruptly, as my blistering was finally submitting to a measure of control - the occurrence of only a few new blisters - my then dermatologist intervened when he became aware of my internists prescription and instructed me to drop to 40mgs/day prednisone in divided doses. My internist deferred to him. I did not question as a layperson the derms decision. By inference together with being unaware that 40mg/day can be significantly less effective than 60mgs/day in the treatment of Pemphigus, I surmised that I was receiving 'appropriate' care given my internists reticence. I simply believed my dosage was being fine-tuned.
It was unbelievable to experience first-hand what the potential of this disease is without, or with insufficient, appropriate medication early on. Prednisone is 'the' established first line of defense for virtually most pemphigus patients - empirical knowledge from reference books decades old. It certainly was having positive effect on my condition at 60mgs/day. But with a newly sliced daily directed dosage per my derm, my condition worsened, and quickly over a weekend period. When I had achieved blister 'bags' the size of spent quart-sized IV bags under each armpit - seemingly out of nowhere - I took it upon myself to elevate my prednisone dosage back to 60mgs and immediately. Infuriated, and after the initial shock wore off, I made the quickest plans of disassociating myself from the incompetent I had entrusted my pemphigus treatment to. It was a crucial decision on my part, otherwise I have no doubt I would have soon required recovery hospitalization. My decisions, led to a regaining of acceptable control of my pemphigus after a long week while clinging to a fixed 60mgs/day of prednisone. The damage that had been done though when I was short-changed on prednisone took many months to heal. I can safely say that I can easily empathize with what a burn victim must endure when their skin is lost.
Moving ahead again
A new dermatologist - different, as day is from night - who also lectured at Columbia University in NY - helped pin down my type of pemphigus to para-neoplastic pemphigus (PNP) via ordered blood tests at Johns Hopkins University together with initiated communications to its renowned pemphigus expert Dr. Grant Anhalt. I was thereafter prescribed effective dosages of prednisone with a tapering plan consistent with visible healing progress. How relatively simple treatment can be for a pemphigus patient when they're in the right hands. I was relieved, my oncologist was relieved, my internist, well, ...I let his subordinating his correct instincts of care for me to a demonstrably lesser physician - slide.
Convergence
So then, at a point in mid-February 2001, my affliction with cancer was now readily entwined with the affliction of pemphigus for which prednisone was my only defensive medication. Naturally, when one is on prednisone, the predominant general medical preference is to taper off expeditiously given its notorious side effects. But that inclination can be a disservice to a pemphigus sufferer: a slow schedule of prednisone reduction has been empirically proven to be of most benefit to a pemphigus patient once effective control has been achieved. In support of the goal of 'weaning' from prednisone dependence, a selection of what may be referred to as 'helper' immunosuppressant drugs is available from which physicians may choose - many being anti-cancer agents. In my circumstance it was concluded by my oncologist and white-hat dermatologist that 'immunosuppressants' such as Cellcept, Imuran or Cytoxan etc were unsuitable given the chemotherapy treatments I had received adversely impacting my bone marrow. The mono-clonal chemotherapy drug Rituxan which was pre-planned for use after Fludarabine treatments would now essentially serve as a 'two birds with one stone' B-lymphocyte killing agent without detriment to bone marrow. Rituxan is a seek and destroy designer drug by virtue of its effect of turning the tables on targeted B-lymphocyte cells by 'marking' them as pseudo antigens which other components of an IS would recognize to be alien, converge upon and purge. A rather cool concept. In all pemphigus cases, mucosa and skin components will be attacked in varying degrees by mistakenly being 'perceived' by ones IS as antigen substance. The root cause of my pemphigus was tied to my NHL/CLL comprised of malignant B_cells and B-cells, cancerous or not, predominant as the natural biological 'factories' which spawn anti_bodies against antigens; destroy those factories producing any type of antibodies and whalla!!
Remission
Chemotherapy for my NHL/CLL cum PNP was successfully concluded with an uneventful 4-week standard course of Rituxan treatment in May of 2001. Gradually, over the course of the following Summer, my skin healed as I continued a slow tapering of prednisone use together with presumptively Rituxans' extended terminator effect on targeted B-cells. As of this writing I have been visibly free of pemphigus for approximately 3 years. My NHL/CLL is similarly in remission for the equal amount of time yet I have remaining vexing lymphoma one of which for example encases a segment of my aorta. I remain on 4mg EOD of prednisone as certain periodic itching spells would seem to indicate some type of underlying skin 'irritant'.
Perspectives
No one elects to have cancer. No one would elect to be tormented with Pemphigus. No one wants diabetes, a heart attack, stroke, to become paralyzed ...to die; I have a reference book listing 600+ 'known' diseases a human being may contract during their finite lives. This book evinces the fragility and by extension the potential for accelerated mortality of each and everyone. In the final analysis, in each patient-specific medical case the degree of success in overcoming a bewildering potentially terminal disease to a point of remission or cure is dependent upon the
ongoing synchronous harmony of a host of factors, some of which for me were to be of greater significance: ones familial and extra-familial relationships in terms of support structure realization; insurance coverage and access to chosen medical resources; the psychological anchors one has adopted - including ones spiritual beliefs - to help usher them through lifes more unpleasant experiences; the expectation and realization of professionalism from ones physician(s) - or else hasta la vista baby; ones level of general health; ones instinct to be proactive in combating their ailment(s); ones receiving appropriate medication(s) and dosages in a timely manner; generous supplements of vitamins and minerals together with high protein intake daily for body repair. These considerations were all strategic to keeping my head above water. Plus I should not neglect to mention my frequent visitor shoulder companions and their shadowy metaphysical whispers offering advice.
Lastly, I was dispelled of any misconceptions of implied guarantees when in the care of an individual whose name is followed with the letters MD. One must, repeat must, be aroused to be proactive. No one hold ones life more dear than they themselves. So work at it. Especially by holding to the fire ones physicians feet.
And so it goes.
Email: esknbabecurtny@worldnet.att.net
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