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R. Mark Bickerton 21 August 2014 Online Submission to the Senate Legal & Constitutional Affairs Legislation Committee Medic

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R. Mark Bickerton 21 August 2014 Online Submission to the Senate Legal & Constitutional Affairs Legislation Committee Medic R. Mark Bickerton 21 August 2014 Online Submission To the Senate Legal & Constitutional Affairs Legislation Committee Medical Services (Dying with Dignity) Exposure Draft Bill Dear Senators Rare Cancers Such As Mycosis Fungoides At the outset I would like to thank the Senate for considering this Bill, and offer my profound support of it. Please consider my submission as a public document, if warranted. In 2009, at age 50, I was diagnosed with a rare cancer that is known as mycosis fungoides. I have used the abbreviation “MF” extensively in this submission. I am at a very early stage of MF and am not presently in any pain. I thought it worthwhile to make a submission for the following reasons: Rare cancers can progress in unusual ways, and some of these cancers are not necessarily rapidly terminal – death may take many years to occur. Often death may instead come from infections, and a prediction of ‘terminal illness’ may not be easy for a specialist to make. The late Janet Mills, who was the second person to use the Northern Territory’s Rights of the Terminally Ill Act 1995, had an advanced stage of MF. I expect that Janet Mills’ case may be mentioned in other submissions, and as I have some knowledge of this rare cancer, I thought that my observations, and the attached medical literature, may be of assistance. To convey my thoughts on how the Bill, if enacted, may affect my situation. What is Mycosis Fungoides (MF)? The following explanation from Wikipedia explains why the name mycosis fungoides is a misnomer http://en.wikipedia.org/wiki/Mycosis_fungoides: Mycosis fungoides was first described in 1806 by French dermatologist Jean-Louis- Marc Alibert.[2][3] The name mycosis fungoides is very misleading—it loosely means "mushroom-like fungal disease". The disease, however, is not a fungal infection but rather a type of non-Hodgkin's lymphoma. It was so named because Alibert described the skin tumors of a severe case as having a mushroom-like appearance.[4] MF is one of a group of lymphomas known as Cutaneous Lymphomas. More specifically MF is a Cutaneous T-Cell Lymphoma. T-Cells are “white blood cells” and are therefore part of the blood and lymphatic system, and accordingly MF can also be described as a cancer of the blood and immune system. MF is quite rare, with an incidence of about 0.3 persons per 100,000 persons per annum. As a consequence of this rarity, the average GP, with 2,000 patients “on the books”, would expect to see a patient with MF about once about every 150 years. In early stages of MF, discoloured areas of skin appear – these are called patches. Patients with less than 10% of the skin area covered in patches are at Stage 1A. Persons with greater than 10% are Stage 1B. Patches can progress to plaques, which are raised patches, perhaps 1cm high. Plaques can progress to tumours on the skin, and in later stages within the body. Involvement in lymph nodes and the blood can occur. Three articles of medical literature are appended to this submission. The first of these appendices is from the American Society of Hematology and is titled ‘How I treat mycosis fungoides and sezary syndrome’ authored by three eminent specialists. The remaining two others are clinical practice guidelines. These appendices provide further details of MF, and other forms of Cutaneous Lymphoma. Each form has unique pathways of progression, and some are more aggressive than others. The “mechanics” of MF are worth explaining. Any person who has cut themselves and bled during shaving will appreciate that a thin layer of skin encapsulates tissue containing blood. In MF, a certain type of T-Cell becomes malignant. In my case, T-helper cells, known as CD4 lymphocytes periodically become lymphoprofilerative – which is to say that they multiply in an uncontrolled manner. The malignant CD4 lymphocytes proliferate and do not die – instead they migrate from the blood and lymphatic system, to the surface of the skin. In my case the malignant CD4 cells tend to migrate to areas of the skin that do not receive direct sunlight, such as my buttocks, and lower legs. Treatment for Stage 1A MF can involve exposure to Ultra Violet light and steroid cream to induce apoptosis in malignant CD4 lymphocytes. Treatments for more advanced stages include a barrage of chemotherapeutic drugs, radiotherapy and novel new medicines and clinical trials. Progression of MF The early stages of MF are characteristically indolent. In later stages the cancer may be much more aggressive. A dermatologist treats my early stage MF, and he has mentioned that he had another patient with more aggressive MF. This patient had large MF tumours on the soles of his feet such that he could not stand up. He also had large tumours on his buttocks such that he could not sit down. This other patient is now deceased. Causes of Death Even in advanced stages, MF does not necessarily cause death. The body can bear a great tumour burden without killing the patient outright. This prospect terrifies me. However, plaques and tumours on the surface of the skin often lead to patients dying of hospital acquired infections, and sepsis - bacterial infections. Diagnosis of early stage MF is not a terminal disease. If the disease progresses, as it does in about 20% of patients, progression to advanced stages can lead to a miserable, disfiguring, painful, and incredibly itchy existence, which may not be quickly and directly terminal. Relevant Specialists MF, and other forms of Cutaneous Lymphoma, are usually treated by dermatologists, haematologists, radiologists, and oncologists. In the event my disease progresses I have a file open at Sir Charles Gairdner Hospital's Haematology Department. The rarity of MF, and other Cutaneous Lymphmas, is such that there are few medical specialists capable of making a reliable prognosis. My Personal Views Thankfully, in the 5 years since diagnosis, my MF has not progressed. It is merely a periodic nuisance. I had concluded that if my MF progressed, and became refractory (which is to say resistant to standard treatments), that I would consider suicide. My personal preference for self-termination using Nembutal. However, if my disease were to progress I may not be able to personally make a trip to Mexico to procure such. In that event, one would rely on another family member making the trip, which would cause moral dilemmas and potentially expose a family member to committing a criminal act. If the Bill were to be enacted in its present form, it would affect me as follows: 1. I would have the comfort of knowing that in a worst-case situation, I could apply to die with dignity, at a time of my choosing, having said goodbye close to those close to me. 2. I would also tend to "hang on" for as long as I could. 3. If the Bill were not enacted, and my MF starts to progress, then I would start to make arrangements to obtain the necessary drugs for self-termination at the earliest opportunity. 4. I would also be concerned at the Bill's present definition of 'terminal illness', as MF does not always directly progress to death. One could literally become a walking tumour for some considerable time, until a hospital acquired infection carried me away. Yours sincerely Richard Mark bickerton From bloodjournal.hematologylibrary.org by guest on February 19, 2014. For personal use only. 2009 114: 4337-4353 Prepublished online August 20, 2009; doi:10.1182/blood-2009-07-202895 How I treat mycosis fungoides and Sézary syndrome H. Miles Prince, Sean Whittaker and Richard T. Hoppe Updated information and services can be found at: http://bloodjournal.hematologylibrary.org/content/114/20/4337.full.html Articles on similar topics can be found in the following Blood collections Free Research Articles (2202 articles) How I Treat (121 articles) Lymphoid Neoplasia (1612 articles) Information about reproducing this article in parts or in its entirety may be found online at: http://bloodjournal.hematologylibrary.org/site/misc/rights.xhtml#repub_requests Information about ordering reprints may be found online at: http://bloodjournal.hematologylibrary.org/site/misc/rights.xhtml#reprints Information about subscriptions and ASH membership may be found online at: http://bloodjournal.hematologylibrary.org/site/subscriptions/index.xhtml Blood (print ISSN 0006-4971, online ISSN 1528-0020), is published weekly by the American Society of Hematology, 2021 L St, NW, Suite 900, Washington DC 20036. Copyright 2011 by The American Society of Hematology; all rights reserved. From bloodjournal.hematologylibrary.org by guest on February 19, 2014. For personal use only. How I treat How I treat mycosis fungoides and Se´zary syndrome H. Miles Prince,1 Sean Whittaker,2 and Richard T. Hoppe3 1Division of Haematology and Medical Oncology, Peter MacCallum Cancer Centre and University of Melbourne, Victoria, Australia; 2St John’s Institute of Dermatology, Guy’s and St Thomas’ NHS Foundation Trust and Division of Genetics and Molecular Medicine, King’s College London, London, United Kingdom; and 3Department of Radiation Oncology, Stanford University, CA The most common subtypes of primary roids, phototherapy (psoralen plus ultra- topheresis. Examples of drugs under ac- cutaneous T-cell lymphomas are mycosis violet A radiation or ultraviolet B radia- tive investigation include new histone fungoides (MF) and Se´zary syndrome tion), topical chemotherapy, topical or deacetylase inhibitors, forodesine, mono- (SS). The majority of patients have indo- systemic bexarotene, and radiotherapy. clonal antibodies, proteasome inhibitors, lent disease; and given the incurable na- Systemic approaches are used for recalci- and immunomodulatory agents, such as ture of MF/SS, management should focus trant early-stage disease, advanced-stage lenalidomide.
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