View from inside
I do not recall the exact moment I was told that my kidneys were failing. Perhaps because I was slowly drip fed the news over the course of 5 years. As soon as each outpatient appointment at the Charles Dent unit in London was over, I'd begin reviewing and researching the latest my consultant shared GFR, creatinine clearance, ultrasound findings and anything else I was quick enough to mentally take note of. The tests would reveal poor kidney function my body had finally succumbed to my mismanagement of glycogen storage disease type 1A. My body was overwhelmed and failing in basic self sustenance. I work in a clinical environment I understand standard lab panel data, the Krebs cycle, G6P gene mutation, KaplanMeier curves, but when it came to seeing myself clinically, I could not fit myself into any of the models or articles that the internet would throw at me. I hoped I could draw upon my science background but the GSD paired with renal failure just did not quite make sense. Meticulously reading journal papers, scouring the internet and social media, I kept trying to find the one piece of information that would tell me that renal failure in those with a rare metabolic disease could be reversed. I'd make detailed notes, action plans and conscientious decisions to implement x, y and z changes and by the end of the day was too exhausted to do any of it. Physically, mentally and emotionally just drained. I realised quickly that facing my present had changed both nothing and everything. Before kidney failure, I spent a lot of time in and out of hospital. After kidney failure, I would spend a lot of time in and out of hospital. So the problem wasn't really that Bmy life has changed^ So I wondered why, in the waiting room waiting for my first dialysis session, was I so grief stricken? Time began to feel less like the ticking clock and more like a state of being. Stillness settled in but my heart beat frantically. I knew all the motivational concepts Beverything happens for a reason^ Bthis too shall pass^ Bborn survivor^ Bhard wired for struggle^ But in that waiting room, I disregarded them as baseless delusions. I eventually settled for pondering upon how I got to this point in life in the first place. This point, where I'd finally succumbed to my lifelong passenger, one that I had named Defeat. The certainty of the past was easier than this capricious present and this simple act of reflecting was almost comforting. Like everyone, throughout my 30 years of life I'd followed the question of what makes human life meaningful, but in this moment, Defeat had won and here I was even further away from answering this question. My childhood with GSD was very much controlled and dictated by a number the time on the clock, centimetres of an NG tube, scoops of Caloreen and later starch. Alongside this, I was also wrapped up tight and securely by those around me so no external factors could harm me. My mother's classic line was Bmy daughter can't take part in school assembly because she has a liver problem^ Soon that word Bassembly^ started being replaced with other significant milestones in any child's life nursery, swimming, playing in the sun, camping, dancing, jumping in puddles, and going on holidays anything you did, I did not. I felt left out and wanting to break free. Then there was my father who generally never used the Bliver problem^ card. Until one day, he came to know about the benefits of a disabled parking badge. I was angry. If there was a reason I was handicapped it was because mywings were tied, not because I did not have any. On reflection, both my parents had seen me at my worst childhood seizures, epistaxis and emergency trips to the hospital for vomiting events so severe and constant that not even a molecule of Caloreen would stay inside me. So to keep me as healthy as possible, they cocooned me, secured me and protected me by implementing these added Jyoti Halai
Journal of Inherited Metabolic Disease