Breathless By Larry Martel A true story of one man’s struggle to survive the horror of IPF (Idiopathic Pulmonary Fibrosis) Breathless By Larry Martel No part of this book may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, electronic, mechanical, photocopying, recording, or otherwise, without prior permission of the copyright owner. Copyright © 2017 Larry Martel All Rights Reserved Concord NH USA Dedication To my wife Ewa, a kind and gentle person, who came into my life during my darkest moments and made me whole again. Our years together gives meaning to the words ‘In Love’. You are my lover, my best friend and my confidant - the sunshine of my life – the only person I can count on with 100% certainty. When I wake up next to you each morning and see your beautiful smile, I know I am the luckiest man on earth. I will always love you no matter where I am. Index Chapter 1 . The Call . Page 1 Chapter 2. Searching . Page 9 Chapter 3. Ewa and Larry . Page 18 Chapter 4. Hope . Page 26 Chapter 5 . The Decision . Page 33 Chapter 6. Reality .. Page 42 Chapter 7. Easing The Burden . Page 49 Chapter 8. Looking For Answers . Page 56 Chapter 9 . The $96,000 Question . Page 65 Chapter 10 . Uncertainties . Page 80 Chapter 11 . Walking A Careful Line . Page 93 Chapter 12 . The Lost Daughter . Page 111 Chapter 13. Disaster Strikes . Page 116 Chapter 14. Recovering . Page 128 Chapter 15. Disappointment . Page 136 Chapter 16. Getting Real . Page 145 Introduction This is a true story about a man and his struggle with Idiopathic Pulmonary Fibrosis. Idiopathic Pulmonary Fibrosis (idiopathic meaning no known cause) is part of a group of lung diseases known as Interstitial Lung Disease of which there are a variety of different illnesses with diverse causes, treatments, and prognosis. IPF as it is called, is a horrific disease that few people have heard about, yet it takes the lives of over forty thousand Americans each year. The same number that die of breast cancer each year. As of this writing, in late 2017, there is no cure for IPF and treatment options are limited. It can progress at varying rates - it may progress slowly, it may progress rapidly, or it may even stabilize for weeks or months. But the disease is relentless and will keep progressing over time until there is nothing left of a patient’s life. The average life expectancy of patients with IPF is currently 2.5 - 5 years. Larry, who lives with his wife Ewa, developed IPF when he was 64 years old. They live in a small single family home in a suburban neighborhood. Larry has two grown children from a previous marriage and one grown step-son from Ewa’s previous marriage. They met by chance when they were in their mid-fifties - he after losing his spouse of 25 years through cancer and she after divorcing from her spouse of 18 years. Their lives were truly blessed as they went about their daily lives travelling, biking, walking, shopping, yard work, etc. -- you get the picture – everything in their lives was done together - as a couple. They were madly in love and felt grateful that they were given one last opportunity at happiness so late in their lives. The news of Larry’s diagnosis was very hard on both of them and as the disease progressed they were forced to accept that they couldn’t do things together as they used to. Larry became more and more dependent and had to rely on Ewa even to do simple daily task. However, their love and devotion to one another kept them strong as they faced each day’s trails. Every person represented in this book is real. However, as they say, “Some of the names (non family characters) have been changed to protect the Innocent .” As a caveat, all medical terms and/or explanations of medical situations are portrayed as accurately as possible, but may not always be 100 percent scientifically correct. They were written as the author understood them at the time of his writing. Every person’s wants, needs and expectations are different. The decisions, experiences, and results of anything conveyed in this book was written solely as it pertains to the author’s own experiences. It does not mean that anyone else should make those same decisions, nor does it mean the results will be the same. Each and every person must choose what is right for themselves and each and every person must depend on their own wants and needs to make those choices. This book takes you through six plus years of the most important, and not so important, events in one man’s life living with Idiopathic Pulmonary Fibrosis. The author sincerely hopes it can serve some purpose in the life of someone facing the same challenges. Chapter 1 – The Call “Hello” “Hi is Larry there?” asked the voice on the other end of the line. “Yes, speaking.” “This is Jan, Dr. Dowd’s nursing assistant. We have your results back from your CT scan from the other day. It shows that there is some possible signs of Interstitial Lung Disease. Dr. Dowd suggest you follow up by making an appointment with a pulmonologist.” “Interstitial Lung Disease? What is that? Can you tell me how serious it is? Is it progressive?” “I can’t answer those questions. As I said, Dr. Dowd would like you to make an appointment with a pulmonologist. A pulmonologist will be able to help you answer those questions,” replied Jan. “You can’t tell me anything? Jan repeated herself. “Sorry you’ll have to talk to Dr. Dowd or make an appointment with a pulmonologist for more information.” “Okay, thank you. Goodbye.” Larry didn’t know it when he hung up the phone on that beautiful day in May of 2011, but his life would become an end point journey. He was feeling that this mysterious discussion that the nurse didn’t want to get ~ 1 ~ into was just the beginning - but the beginning of what? What is the end point? He had no idea what the nurse was talking about. “Interstitial Lung Disease – what in the world is that?” he thought. Larry was always more than just curious about the human body - researching anything and everything from vitamins to human anatomy and everything in between. This served him well over the years, allowing him to intelligently discuss, ask questions, or debate treatment plans and test results. He never took what a doctor had to say as ‘gospel’ - it was his body and he needed to know what was going on with it. “If you don’t advocate for yourself – no one else will,” Larry would tell friends. At twenty-eight, Larry gave up smoking cigarettes - a terrible habit he had started when he was fourteen years old. He decided and made a commitment that he would change his way of life. Family history told him he was genetically predisposed to heart disease and diabetes - he knew by living a healthy lifestyle he could avoid those debilitating medical issues. At the very least, he felt his plan would delay their grasp on him well into the future, and not allow him to succumb to them in his mid-fifties or early sixties like his parents, grandparents, and aunts and uncles had done for generations. And for the most part, he stayed true to his word by staying fit and trim into his mid sixties. He lifted weights (body building), and ran endless miles competing in road races into his fifties before switching over to bicycling thousands of miles each year. His food selection was just as structured as his exercise regimen - eating to stay healthy never for pleasure. His plan worked - his lab tests were great and his friends ~ 2 ~ admired how he kept in shape - he was a picture of health. One day in late March of 2011, during a routine physical exam, Larry complained of pain in the back of his throat when he would inhale deeply. After asking many questions and palpating Larry’s neck area Dr. Dowd decided that an x-ray of the neck area would be in order. The results of the x-ray came back negative and did not show any abnormalities. To continue her investigation, Dr. Dowd recommended that Larry do a Pulmonary Function Test. This simple non-invasive test measures various aspects of how a person’s lungs function by breathing into a machine. Various criteria are used to assess how much air the patient’s lungs can hold, how forceful the patient can expel the air, and how well gasses are exchanged within the lungs to name a few of the items tested. At the end of the test Larry was given albuterol to inhale to see if he might have COPD. If he did have COPD inhaling albuterol would relax the muscles in his airways and allow his lungs to open up for more air. The albuterol had no effect on Larry’s breathing and for the most part eliminated COPD as a diagnosis. All the other lung functions that were tested were determined to be within normal limits of a predicted value. This means that for Larry’s height and weight he fell within the acceptable parameters of the general population for any given test. These types of results were not new to Larry. He had always pursued health issues with zeal. Unfortunately, it was once again looking like Larry was being overly sensitive in his concerns over this matter.