Now I Am The Voice: <i>“a journey of self‐discovery through the eyes of a Thalassemic”</i>
Authors: Maria Hadjidemetriou
Journal: American Journal of Hematology (2020)

At 2 ½ years old, I was diagnosed with Thalassemia, a rare genetic blood disorder that primarily affected those of Mediterranean descent. My parents are both Greek Cypriot and at that time, one in 200 infants born were diagnosed with Beta-Thalassemia on our beautiful, ancient island of Cyprus. My mother only recently told me the story of when I was first informally diagnosed by a pediatrician when I was barely 3 years old. He said to her “just by looking at Maria I can tell you she has Thalassemia”. My face was jaundiced; my eyes were hollow, and my lips a pale yellow. I had the classic Thalassemia overbite, bossing of the forehead, high cheekbones and was missing my toddler milestones. I was formally diagnosed at New York Presbyterian Weill Cornell Medical Center (Formerly New York Hospital). The pediatrician at that time gave hope to my parents that I would live a beautiful life and to not limit me from any experiences. She reiterated to my parents “Maria will have a full life”. Unfortunately, that came to an end when I turned 8 years old when a new hematologist replaced my beloved doctor who was filled with positivity and love. My new hematologist now painted a bleak future for me that I would not achieve much in life. She limited all of my dreams. She told my mother I could not participate in any physical activities during my gym classes because my bones were too weak and can easily be fractured. The years from primary school into high school were spent watching from the bench other kids play basketball, volleyball, tag, duck-duck goose, musical chairs, gymnastics….hating myself, hating Thalassemia. I started day dreaming that it was ME throwing the ball and winning for the team and my friends would all clap, cheering for me “hooray Maria” and that put a smile on my face. It fulfilled me so much that I became a professional daydreamer and created in my mind heroic images of myself succeeding to suppress the inner emotional pain. As a child it is okay to get hurt; it is part of growing up. It is okay to scrape my knees and get bruises, as that is part of childhood. The oppression of childhood experiences did not stop as my hematologist continued to take dreams away from me into my adolescence. My formative years had been plagued with her negative words that lead me not believing in myself. The words of my hematologist were always “Maria, alls you have to do in life is take care of your disease. Remember you have a disease”. Remember, I have a disease…YES it's something I can never forget because I live with my disease. It is in my body; it is my heart that hurts because my body is so depleted of healthy red blood cells - the cells that carry the oxygen. I am the one that gets stuck with a butterfly needle three to four times each time I go for transfusion, because my veins are so scarred from being stuck so much every fourteen days to receive my two pints of life saving blood. I'm the one that sticks a 1″ butterfly needle in my stomach every night for ten hours a night, five nights a week, to chelate the toxic overload of iron out of my liver, causing abscesses. Please doctors, do not ever tell your patients “REMEMBER YOU HAVE A DISEASE”; don't ever disrespect your patients or speak to them in such a condescending manner just to get your way. It is as if people with disease and/or disabilities should not be allowed to dream, should not be allowed to have personal or professional goals and yet how relevant this still echoes in today's society. At that age I didn't have the courage to speak up. I had no voice at all, instead I had fear because she would remind me that she was the only thalassemia doctor in New York and no one else would take care of me. Where would I go? I kept quiet….just for a little while until I found the courage to start speaking up and that came once I started learning more about my disease. I had finally found my voice. Knowledge Builds Confidence: I found myself at age sixteen making my way to our local public library in Astoria, Queens, a heavily populated Greek community. I asked the librarian “I would like please to gather information for a specific disease”. The librarian hands me an index card and asks me to write down the disease. My Greek roots kicked in, thank God my parents made me go to Greek school when I was young as much as I disliked it at that time. I wrote: T H A L A S S A which means sea in Greek and E M I A coming form the Greek word EMA which means blood. THALASSAEMIA - I handed her the index card. She returns with a stack of American journals and carried them for me setting them down on the table. She touched my arm as if she knew I was a Thalassemic. Of course she knew because I had all the typical facial features of Thalassemia. The very unique facial features that took me time to fall in love with and accept, that this is my face. And, as I looked in the mirror I would sing to my reflection that beautiful song from the classical film Funny Face, with Fred Astaire and Audrey Hepburn “I LOVE YOUR FUNNY FACE; YOUR SUNNY FUNNY FACE”. The song became my anthem, and these very facial features scientists, clinicians and hematologists described as a “rodent”, a “chipmunk”, and a “mongoloid” with “growth retardation”. How disheartened I felt that moment as a sixteen-year-old finding ways to accept the rarities of my disease. How disheartening for the medical community to think of us in such a degrading way. I continued reading, as much as it hurt to read the morbidity of Thalassemia. I read, I learned and I built a stronger Maria. The journey started in believing in myself and I implemented something doctors never ever taught….SELF CARE. Self Care: In Googling “what is self-care” the response is: “the practice of taking action to preserve or improve one's own health; the practice of taking on active role in protecting one's own well-being and happiness”. First and foremost - I will never take away the importance of the work a hematologist does for Thalassemia. I respect all outstanding doctors who connect with their patients and the dedication they give to their profession. But it's time for doctors to treat disease in a holistic ways as the world implies - from the Greek word “holos” meaning “whole”. At age eleven I made a decision to forgo ALL JUNK FOOD. After surgery for removing over thirty gallstones, my surgeon asked me, “Maria what is your diet like”? I did grow up in a traditional Greek home eating my mother's homemade delicious Greek food every day, but the junk food was there every single day, and my mom did not know how bad I was. I knew I had to change my way and so I did. I got rid of all junk food, sodas, trans-fats, fast-food and consumed a very healthy diet. As I became older, I implemented a regimen of the most sophisticated antioxidants, supplements, and vitamins. I explored alternative ways of healing and keeping strong despite the disapproval of my hematologist who called my nutritionist/scientist at Rockefeller University the “vitamin lady”. I remember the words from my hematologist as if it were yesterday “you are still seeing that vitamin lady? You cannot have both of us in your life so you choose either her or me”? I was stunned at these words and never told her anything my nutritionist taught me after that meeting. I didn't have the energy to fight my doctor, because I was fighting to stay strong for my life and that was more important than her ego. Did doctors forget “Let food be thy medicine and medicine be thy food”? Please note that I am not taking anything away from outstanding doctors who listen and connect with their patients. Please work as a team with nutritionists. I also started meditating every day connecting to my pain and taking a moment to thank God for giving me this body and this disease. I embraced Thalassemia and the positive energy exuded outwards with my smile. The spiritual connection I have with God has become so profound in my life. It is He that gives me the strength, the wisdom and the patience to go on everyday living with chronic pain and taking me through the deepest of pains. But as you get older Thalassemia gives you a handful of secondary diseases and for me that was Osteoporosis. Diagnosed at age 30 with a DEXA score already at −1.5 into the −2's I went on typical drugs like alendronic acid, risedronate, and zoledronic acid. None of the bisphosphonate drugs worked for me. I came off of these drugs and did what I know best….heal thyself through weight training. In sitting with my orthopedic doctor about four years ago with a DEXA score that was now −3.4 to −3.8, he genuinely was sad and said “I just don't know what to do with you anymore”? I was aghast with the words he chose so I said “well I'm going to take it into my own hands”. “And how do you suppose you do that” he said angrily. “Well doctor I will weight-train every day because I have choices in life, right? I can either do nothing about it and end up in a wheelchair, or do something about it and keep myself as strong as I can, so I choose to do something about it”. When you train with weights you are making osteoblasts, and it is exactly what the bones need. My 2019 bone density results showed a 4% improvement. I weight train five times a week at the gym doing yoga and Pilates as well, and it's not just the body I exercise, but the mind. I train the mind with motivational and spiritual techniques from leaders such as Tony Robbins, Napolean Hill, Eckhart Tolle, Robert Green….how do you take yourself from the negative to the positive and stay in the positive state of mind especially when the future looks like I need to climb Mount Everest (on a hemaglobin of 10). Remember you want to heal your whole self, and with a negative mind you are not completely healing yourself…a negative mind will never allow you to heal. I also took myself from the word “diet” to the word “lifestyle”. I'm not vegetarian or a vegan nor do I follow an alkaline diet or the keto diet - I eat clean, wholesome foods - organic and non-GMO with a plethora of plant protein from legumes as that is a huge part of what we eat in Cyprus. I later read a study on wheatgrass and how in India it has raised the hemoglobin of 120 Thalassemia patients. Since starting wheatgrass in June of 2016 my hemoglobin has gone from the 10's to the 11's taking me from a transfusion regiment of every 14 days to every 21 days for the most part of the calendar year. People ask me all the time “aren't you tired of being so healthy”? No, I can never tire of respecting my body; I can never tire in respecting my mind, in learning more about living a healthier lifestyle. It is for me SELF LOVE. Self Love: As a mother who conceived my daughter Julia naturally I raise her with two words: responsibility + respect. These two words play a role in every aspect of our lives - our colleagues; our teachers; neighbors; family; our partners and our bodies. It is your responsibility to keep yourself healthy, not your doctors. Even with disease it is no excuse to be gluttonous with your food and to be irresponsible with your choices. Remember we have choices in life. Respect your body; cherish it and give it self-love because your body and your mind deserve it. It is an extraordinary path to a greater healing. Healing is not just the treatments that we need to undertake, but also the self-care and the self-love. We are at a time where society (and doctors) needs to look at disease as strong and not weak, beautiful and not ugly and yes even a little sexy. It may have taken me way into my university years to find my voice, but alas, now I am the voice. Maria Hadjidemetriou. @downtownmomnyc. Maria is a resident of downtown Manhattan where she lives with her 11 year-old daughter Julia. She is a real estate professional and a free-lance writer. She has written and produced a short documentary “Thalassemia: Life Without Boundaries” and has published previously in American Journal of Hematology “Thalassemia: Yesterday, Today, Tomorrow”.1 She is an Executive Board Member and Officer for the Cooley's Anemia Foundation and an Expert Patient Advisor for the Thalassemia International Federation headquartered in Cyprus. She speaks globally on living a positive life with Thalassemia.