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. Over the years, he told many people that if something bothered him health wise he would get answers. Larry would say, “I’m not like most men who ignore their body’s warning sign(s) only to end up dying needlessly. I would rather have a doctor tell me I came to see him for
~ 3 ~ nothing then to be told that if I had just come by six months earlier he could have helped me.”
After the Pulmonary Function Test, Dr. Dowd sat down with Larry to go over the results. “Everything is normal. There doesn’t appear to be any issues with your neck or your lungs,” she said. “Let me listen to your heart and lungs again before you go.” Bringing the stethoscope down to the lower portion of his back she instructed him to breathe deeply in and out . . . . again, and again, and again, and again. “I can barely hear some crackling on your lower lungs. You really have to listen intently otherwise you would miss it. It’s like it’s there and not there.” So after further discussion Dr. Dowd agreed that a definitive answer should be pursued. She felt that a HRCT (High-resolution computed tomography) should be ordered and the question would be put to rest - the phone call from nurse Jan was the result of that HRCT from May 2011.
After getting off the phone, Larry’s wife Ewa asked, ”Who called?”
“It was the doctor’s office,” Larry replied. “It was about my CT scan.”
“What did they say - is everything okay?”
“I’m not sure. The nurse said something about Interstitial Lung Disease, but I have no idea what she was talking about, and she wouldn’t answer any of my questions. I’ll have to look it up. Can’t be too serious as the doctor herself would have called me to explain it. Oh, the nurse did say I should make an appointment with a pulmonologist, which doesn’t sound very good.”
~ 4 ~ “Oh, it’s probably nothing,” Ewa responded in her usual upbeat tone.
Larry went directly to his laptop to search for this new medical term. He quickly typed in Intirstatial Lung Disease but got no results. “Hmmm must be spelled wrong,” he quipped. Looking at his computer more closely he saw Google asking him, ”Did you mean interstitial?”
“Yes, of course that is what meant,” as he smiled to himself. “What else would I have meant?”
Larry types in the correct spelling and the truth pages appear – each one appearing to be more ominous than the one before.
“Interstitial (in-tur-STISH-ul) lung disease describes a large group of disorders, most of which cause progressive scarring of lung tissue. The scarring associated with interstitial lung disease eventually affects your ability to breathe and get enough oxygen into your bloodstream.
Interstitial lung disease can be caused by long-term exposure to hazardous materials, such as asbestos. Some types of autoimmune diseases, such as rheumatoid arthritis, also can cause interstitial lung disease. In some cases, however, the causes remain unknown.
Once lung scarring occurs, it's generally irreversible. Medications may slow the damage of interstitial lung disease, but many people never regain full use of their lungs. Lung transplant is an option for some people who have interstitial lung disease.”
~ 5 ~ Interstitial lung disease seems to occur when an injury to your lungs triggers an abnormal healing response. Ordinarily, your body generates just the right amount of tissue to repair damage. But in interstitial lung disease, the repair process goes awry and the tissue around the air sacs (alveoli) becomes scarred and thickened. This makes it more difficult for oxygen to pass into your bloodstream.
Interstitial lung disease can be triggered by many different things — including airborne toxins in the workplace, drugs and some types of medical treatments. In most cases, the causes are unknown. (article quoted from Mayo Clinic website)
“Holy shit. This does not sound good. Life expectancy on average can be as low as two to five years depending on the type of Interstitial Lung disease a person has of which there are more than two hundred varieties. I wonder what type I have? This could be serious stuff. Why in hell didn’t Dr. Dowd call me herself to talk to me about this?” Larry’s thoughts were going from one thing to another. Then he thought how ironic to get something that can’t be cured or, for the most part, even treated when his whole life has been devoted to making sure to get diagnosed before it was too late to make a difference.
Larry felt he had done enough research on the subject to get an overview of this nasty disease. It was time to call Dr. Dowd’s office for answers. Of course, as is usually the case in these situations, when answers are needed to be had there’s no one there to answer them. It was after 4:00 and the office would be closed. He decided to call anyway and the answering machine said in the friendliest of voices possible, “Please leave a message.” Larry was not in a very friendly mood at that
~ 6 ~ moment and left what could only be described as a very terse clenched jaw message. “Dr. Dowd after having been a patient for many years how could you possibly have had nurse Jan call me with such dreadful news about a potentially deadly disease. I would have thought that you could have at least given me the courtesy of a call.”
The next day Larry received a call back from Dr. Dowd - she was very apologetic and asked him to come in for an office visit the next day, so they could talk more about the issue. Unfortunately, the appointment didn’t amount to – as the old saying goes – a hill of beans. Dr. Dowd, a very experienced physician, was a PCP (primary care physician) who didn’t really know much about Interstitial Lung Disease, or at the very least, didn’t want to delve into a subject she had seen infrequently in her practice.
“Your CT scan shows that they suspect or that you might possibly have the beginning of Interstitial Lung Disease,” she said in her calmest voice.
“Wait did I just hear suspect, possible - maybe I don’t really have this disease after all?” Larry thought to himself.
Dr. Dowd continued, “I would recommend strongly that you see a pulmonologist and I will put in a referral after our visit.”
So Larry left disappointed and confused - and except for apologizing several more times about the lack of courtesy for not calling personally, Dr. Dowd didn’t offer anything to ease his concerns or increase his knowledge about his condition.
~ 7 ~ “Why did she have me come to the office for nothing?” he asked Ewa.
“Her many apologies could just as easily have been done over the phone instead of dragging me here for a visit. This day was all for nothing,” he thought.
~ 8 ~ Chapter 2 – Searching
It seems like there’s a million physicians out there but when you need one there’s none to be found. At least that’s what Larry was thinking when he was told his first scheduled visit with a pulmonologist would take place in about two months – July 2011. Dr. Dowd’s referral was sent to a local pulmonologist group who in turn assigned Larry to a Dr. Ipwich. The nurse who called Larry to give him the appointment date also told him that Dr. Ipwich had ordered another pulmonary function test to be done the day before his scheduled appointment.
“Why do I have to repeat test that were just done a few weeks ago?” Larry asked the scheduling nurse.
“Dr. Ipwich feels more comfortable reviewing data from his own staff’s testing rather than from a PCP’s office,” was her reply.
Being the quintessential cynic that he is, Larry thought that duplicating a test that was just done five or six weeks prior was a good way to bill the insurance company for more money. Normally, he would have questioned this but decided that he wanted answers more than arguing with a new physician, so he just bit his lip and went with the flow on this one. The pulmonologist also ordered additional blood tests to rule out other diseases, toxins, or irritants that could cause Interstitial Lung Disease.
Dr. Ipwich was a short stout kind of individual with a calmness about him and a sincerity in his voice – good bed side manners. As he walked into the room he
~ 9 ~ extended his hand. “My name is Dr. Ipwich, how are you doing today?” he asked.
“I’m doing fine,” Larry responded.
“I’ve been looking at your file and test results. Are you in any pain or distress today?”
“No, everything is okay.”
“Your Pulmonary Function Test results are within the range of normal. Your blood test are all normal and the questionnaire that you answered doesn’t indicate that you have been or are exposed to anything at the present time to precipitate an issue with Interstitial Lung Disease. But your CT scan does shows signs of scarring which may be from a previous injury to your lungs or the very beginning of Interstitial Lung Disease. We can’t know for sure at this time and we will have to wait and see if the scarring progresses. You also have five nodules on the left side of your lungs, which may be nothing but we will need to keep an eye on them.”
“Nodules?” Larry asked. “What is that all about?”
“More than likely calcium deposits but it could also be tumors - benign or possibly malignant.”
“You mean cancer? Oh great,” Larry thought. “Now we’re throwing the ‘C’ word around.”
“We’ll follow up with another CT scan in December to see if there is any change in their size.”
“What about the possibility of Interstitial Lung Disease? That’s why I thought I had come here for – to discuss that issue.”
~ 10 ~ “Right now your Pulmonary Function Tests are normal and the scarring is very very limited on your lower lungs. As I said, the scarring could be from an assault to your lungs in which your lungs repaired themselves. We’ll have to wait and see. It’s nothing I can treat you for at the moment until we determine if it’s progressing or not.”
“If I have this disease how serious is it? What are the treatment options? How fast does it progress? Why can’t I get something to treat it now instead of waiting for it to progress?” Larry was determined not to leave without getting some answers.
Dr. Ipwich replied in his most assuring voice. “If you have Interstitial Lung Disease, and we’re not sure that you do, the treatment options are limited. The reason we don’t start any kind of treatment prior to being symptomatic is that these drugs can have severe side effects. Interstitial Lung Disease is progressive and develops differently for different people - most are progressive but some can be treated. But right now you are healthy and we can talk more about your condition after your next CT scan.”
As a side note – Esbriet (Pirfenindone) and Ofev (nintedanib) – the current acceptable treatment for IPF (Idiopathic Pulmonary Fibrosis) - were not FDA approved in 2011 and consequently not available as a means of treatment.
“Doctor, seven months ago I had this terrible chest cold that lasted several weeks. I had a terrible relentless deep hacking cough with heavy mucous. Once I started coughing it was impossible to stop to the point that I couldn’t catch my breath and often became dizzy. It almost made me vomit. Probably the worse cold of
~ 11 ~ my life bar none. Could this cold have been the beginning of this issue or at the very least the scarring on my lungs are the result of the healing process from that cold? Maybe that’s what is showing up on the CT images.”
“It’s hard to say. Most often we cannot determine the root cause of how Interstitial Lung Disease begins. Let’s schedule you for a CT scan in December and we can talk more then.”
As always, Ewa was with Larry for the visit as they always attended each other’s medical appointments. They felt comfort in knowing the other was there for mental support, as well as, being informed of the other’s medical condition from a care taker’s standpoint, and if need be, to ask questions that the other might forget to ask during the visit. As they were exiting the examining room Ewa commented, “Seems like a good guy. He’s friendly, encouraging, and seems to listen to what you have to say.”
“Yea, I guess so.” Larry responded. “But I think I want a second opinion. I don’t know much more than I did before except now I have nodules on my lungs. I’m going to call Dr. Dowd in the morning and see if she can refer me to another pulmonologist.”
Shaken by the day’s events, Larry had this need to prove to himself that he still had it – “it” being physical stamina that came from lung capacity and function. Upon returning home from the office visit with Dr. Ipwich, he immediately went to the stairwell that led to his basement. Up and down, up and down, up and down – twenty-five times, “See I can still run the stairs like the best of them,” he thought to himself.
~ 12 ~ “Ewa I just went up and down the stairs 25 times. My legs are a little tired but I have no breathing issues.”
“That’s great. You’re worried for nothing. You’ll see everything will be alright,” Ewa smiled as she voiced her encouragement.
The next day, as soon as her office opened, Larry was on the phone calling Dr. Dowd to request a referral for a second opinion. The referral went through very quickly as the very next day Larry received a phone call from the office of Dr. Delrio. It was scheduled for August – only four weeks away, and he was happy and relieved that he would not have to endure waiting eight weeks like he did for his appointment to see Dr. Ipwich.
“I wonder what she has for credentials?” Larry thought to himself. “I’ll Google her to see what I can find out.”
“That’s strange,” he thought. “I don’t see a Dr. Delrio listed at the clinic that Dr. Dowd referred me to. Wait a minute, here she is, it’s not Dr. Delrio but Nurse Practitioner Delrio. She was not a pulmonologists but a nurse practitioner who specializes in pulmonary medicine. Nurse practitioner? Is she going to have enough knowledge to help me?”
Except for the information he had read over the internet, which was informative but scary, Larry still did not know just how serious Interstitial Lung Disease could be as it pertained to his personal well being. No one was giving him any answers. He felt that at least someone else was going to review his file and his test results. Maybe Nurse Practitioner Delrio can come up with some information and tell him what is going on. He felt he was at zero at the moment and anything is better
~ 13 ~ than nothing. So he decided that he would see Nurse Practitioner Delrio and see what she had to offer.
Nurse Practitioner Delrio was not as Larry had pictured in his mind. She was young, maybe early to mid thirties, who had completed specialized training under various pulmonologists at the same pulmonary association that Larry had visited the month before. Her practice was fairly new having set it up less than a year ago. She attempted to come across as being sure of herself, yet displayed a confidence level that aired of uncertainty rather than one who really believed in herself. Nurse Practitioner Delrio started the visit by telling her assistant that she wanted Larry to climb a flight of stairs using an oximeter (a hand held device that measures a person’s oxygen level). Her assistant led him out the door and up the stairwell. At first the device, that the assistant had placed on his left index finger, read ninety-eight percent oxygen saturation - this was good – very good. A healthy person’s oxygen level should be between ninety-five and one hundred percent. But by the time Larry reached the top of the stairs, he looked down to see his saturation level had dropped to ninety-two percent – that was not so good. However just as quickly, within a dozen steps or so on level ground, it was back to ninety-eight percent.
“No big deal,” Larry thought. “It came right back to normal.”
What Larry didn’t realize is that it was a big deal. Nurse Practitioner Delrio told Larry that it was a sign of impaired oxygen intake – that his lungs were not absorbing the oxygen that he needed to keep his body healthy.
~ 14 ~ “But I immediately came back up to normal,” Larry protested.
“But that’s not normal. A healthy normal lung would have seen no drop in oxygen levels or at the most a drop of one percent,” responded Nurse Practitioner Delrio. “I would say, looking at your CT scan that this is the beginning of Interstitial Lung Disease.”
“So where do we go from here?” Larry asked.
“We’re going to run a series of test to rule out various possibilities and to see if we can pinpoint what type of the disease you might have. If we can’t find a specific cause it’s called idiopathic. The term generally used is IPF – Idiopathic Pulmonary Fibrosis. I’ll set up the lab tests at the local hospital right after our visit.”
“What is the long term prognosis and what can be done?”
“There is no cure for this disease. The best we can offer is offer palliative care.”
This statement took Larry back a bit.
“Palliative care – what is she talking about? We don’t even have any results from the test and she’s already giving up on me,” Larry thought.
It was disconcerting and not an expected answer. After the visit, he went to the hospital as directed and did most of the test that Nurse Practitioner Delrio had ordered. The only test that wasn’t done that day was a blood gas test (ABG) in which a needle is inserted deeply into the wrist so as to remove an arterial sample of blood. This test was to be done at a later date. At
~ 15 ~ this point Larry started questioning Nurse Practitioner Delrio methods and asked her assistant about the need for the blood gas test.
“Why, with the accuracy of today’s oximeters was it necessary to perform such a painful test?”
“She needs to establish a medical baseline in the event that you are eventually hospitalized,” responded the assistant.
“Heck, if I wind up in the hospital in a year or so just treat me for the issues that brought me there. You don’t need to know where I was,” he told the assistant.
“Too many test,” he thought. “She testing me to death?”
“I think I will pass on the blood gas test. Except for collecting more data, I don’t see it helping me in the future,” he said to the assistant.
“So you’re refusing to take this test.”
“That’s correct. I’m out of here.”
So Larry came to the conclusion that he was right about his first impression of Nurse Practitioner Delrio. She did not have confidence in herself - she was not the old fashion touchy feely type of medical professional who treat patients based on both medical data and personal rapport. She was pretty much just a data person. He felt that given enough tests data almost anyone can come up with a diagnosis. This was not the type of person he wanted evaluating, treating, and caring for his life. Ewa agreed fully with Larry’s assessment of
~ 16 ~ Nurse Practitioner Delrio – not someone you would want to place your trust and your life.
~ 17 ~ Chapter 3 – Ewa and Larry
Larry and Ewa shared “date” nights four to five times a week. It usually consisted of a glass of wine and a good (sometimes not so good) movie. On occasion when they wanted to relax and just enjoy each other’s company more fully and without distractions, they would go into their sunroom, dim the lights, and open a bottle of Champagne. Larry thought that tonight might be a good time to share one of those evenings. He suggested to Ewa that although this was certainly not a celebratory occasion, they should share a bottle of Champagne and talk about what they knew thus far and what Larry had learned from his research on the internet.
“I’m ready Honey,” Ewa called out from the sunroom.
“I’m getting the Champagne. I’ll be right there. Do you have the glasses?”
“No I forgot. Can you bring them?”
The sunroom was one of the first major projects they had tackled when they first became a couple. It extended out from the house into the backyard some twenty feet and created an air of openness with its twenty foot ceiling and huge glass windows on all sides. As he walked into the sunroom, he could see that the sun had already set behind the trees and the remaining light in the early night sky was glowing with a faint pinkish color. “September is such a lovely time of the year,” he thought. To his left he saw his beautiful wife sitting on the sofa – his presence immediately drew a radiant smile on her face. She has always been such a
~ 18 ~ happy person – she loves life and everything about it. People who meet her for the first time walk away thinking how joyful and so full of life she emits from her person. It was this “joie de vivre” that Larry first noticed on that walking trail some four years ago.
At first they just passed each other from time to time on the path politely saying hello – Ewa with her enormous Great Dane (Brutus) and Larry with his Labrador Retriever (Sully). Then one day around 8:00 a.m., Larry pulled into the parking lot and saw this lady standing by the entrance to trail.
“Hmmm. I wonder why she is just standing there. Must be waiting for a friend,” Larry thought.
He got out the car and walked across the parking lot to the entrance.
“Hi my name is Ewa Wszolkowska,” she said extending her hand for a handshake.
“Hello I’m Larry.”
“Can we walk together? It’s early in the morning and no one is on the trail yet. I really don’t want to walk alone.”
Unknowingly, the seed of romance had just been planted - but neither one of them knew or even had the slightest idea that this was happening. Ewa had just gone through divorce from an eighteen year marriage. She was not thinking of connecting with anyone anytime soon – especially romantically. For her, it was all about having someone to walk with on the trail which was pretty secluded in spots. Larry was a widower who had lost his wife of twenty-five years to cancer three years earlier. Time, the great healer, had not
~ 19 ~ worked its magic – the scars of his loss were still very much with him. If walking with this lady made her feel more comfortable then he was okay with that but certainly nothing else entered his mind.
“Sure why not.” Larry replied.
They walked together with their dogs off and on for months – never setting up a definite time but most often haphazardly being there to enjoy each other’s company. After a while they pretty much knew each other’s schedule and showed up at approximately the same time each day. Then came the anticipation of seeing the other person upon arriving at the park, yet they never really connected and remained distant to one another – for the most part Ewa talked while Larry pretty much just nodded his head between sentences. When he did speak he spoke of the past as if it were the present. He still wore his wedding ring and spoke of his deceased wife in the present tense. In fact, Ewa was pretty sure that he was married as he had never given her any indication to the contrary.
One day as they were walking Ewa thought, “What a sad grumpy man. He never smiles. I’m not sure I want to keep coming here and walking with him. He’s not friendly but he’s not unfriendly either. It’s like I’m walking alone.”
Just as those thoughts were passing through her mind Sully walked up to Larry with a treasure. It was a tennis ball he had found in the woods. Larry immediately had a big smile on his face and bent down on one knee showering Sully with praise for his find and for bringing it to him. He was like a different person with a personality that Ewa had not previously experienced in him.
~ 20 ~ “The man can smile after all,” thought Ewa. “And I can see he has a good heart in the way he loves his dog.”
So they kept walking day after day and week after week, but they never reached out to one another except to share a beautiful trail along the river. Then one day about six months after they met, Ewa decided to reach out - not romantically but as a friend.
“Would you like to come over and have lunch with me some day?” she asked.
“Yes, I would like that,” Larry replied.
“How about tomorrow at 1:00 o’clock.”
“That’s fine. See you then.”
“She’s a very nice lady and I enjoy her company,” Larry thought as he was walking back to his car. “So why not - it’s just lunch - nothing more,” attempting to convince himself that he had made the right decision.
The next day, as Larry approached Ewa’s door for their planned lunch, he could see very quickly that he had the right address. Standing behind the screen door was Brutus – all 180 pounds of him. Brutus never had any issues with Larry on the trail but here he is in his own domain blocking his entry. Ewa always told Larry that Brutus was very protective of her and during several walks had narrated stories of Brutus’ protection efforts. Larry was never afraid of dogs as his parents raised and showed dogs when he was growing up - so he was always around them. But he also knew that when a dog is in his domain you need to be cautious – respect their space.
~ 21 ~ “I wonder if Ewa is close by. Well, let’s see what happens as I approach the door.”
Dogs always give away what they plan on doing prior to their action - you just have to know how to read the way they wag their tail, how they point their ears, what kind of bark or growl they project. However, Brutus wasn’t giving anything away. He just stood there - majestic and staunch like a knight guarding his queen - no bark, no growl, no sounds - staring without so much of a twitch in his entire body.
“Hmmm, I’m not going to press my luck. Ewa are you in there?”
Ewa came to the door and let Larry in. Once Larry was in the door with Ewa’s permission Brutus could have cared less. Larry walked in and saw that the dining table was beautifully set with salmon being the order of the day. As the meal progressed Larry saw Ewa for the first time as they sat face to face and talked.
“She is really a lovely person,” he thought. “She smiles with her eyes. It comes from within – an inner beauty you very seldom see in a person.”
Ewa asked Larry if he might be interested in going to a singles dance with her - not as a date but as friends. She had been to a singles dance several months earlier and really enjoyed the good times and the attention she got from all the men who wanted to dance with her.
“I don’t think so,” Larry quickly replied.
“Why not? It will be fun.”
~ 22 ~ Larry was lost for words. Was it because he didn’t want to be bothered going to a silly dance, or perhaps because he wasn’t ready to date or see other people?
“Why not? Well that’s a good question?” he responded not knowing what else to say and stalling for time. “I guess I don’t want to see you dancing with a whole bunch of guys,” he blurted out.
“WHAT? What did I just say? Where did that come from?” he thought.
So after six months of casual friendship their romance came forward like a firecracker exploding in the night sky. And with each passing day thereafter, Larry’s sadness began to lift as he and Ewa started to share a life together. He would often tell people, “Ewa came into my life during my deepest sorrow and despair and through her love and understanding lifted me up and made me whole again.”
Tonight, four years later, Larry was especially thankful that this wonderful human being whom he loved so very much was sitting there waiting for him. “What a warm beautiful smile – her eyes glowing with love from within,” he thought.
“The Champagne is just the right temperature ...... ‘POP’, let’s drink a toast to our future. It’s only just begun.”
“What have you found out about Interstitial Lung Disease?” Ewa ask.
“Well, I know there are more than two hundred varieties of this disease. It can be caused by toxins, medical conditions, medications, dusts - a whole bunch of
~ 23 ~ things. When as in my case they haven’t been able to determine a cause it’s called Idiopathic Pulmonary Fibrosis – IPF. I learned that Interstitial Lung Disease can be very aggressive with patients dying within six months, others can live fifteen years or so. The average life span is two to five years especially in patients with IPF. Essentially the disease starts when the lungs are assaulted and injured. Normally the body repairs itself and stops after the repairs are completed. But in this disease, the body keeps trying to do repairs that are not needed causing scar tissue to accumulate. This ultimately makes the lungs stiff and unable to absorb sufficient oxygen to survive. There is nothing positive about this disease – if you have it, it will eventually kill you. Finally, the worse news is that there is no treatment for most of the types of this disease – especially IPF. The doctors treat the symptoms but it doesn’t affect survival time nor the final outcome. Unlike cancer there is no sending it into remission, although it can run in spurts seesawing back and forth between being stable with little development to progressing in severity over short periods of time.” He spitted it out all at once not wanting to have his train of thought interrupted. When he was done a stillness filled the room with neither one of them knowing what to say.
“But we’ve only just started our lives,” Ewa’s said in a quivering voice.
Larry looked at Ewa’s usually smiling eyes and saw a tear running down her cheek. It brought a deep puncturing sadness to him that became almost overwhelming. They cried a little, they talked a little, but mostly they were just together - holding each other and wondering what lie ahead. The only thing they knew for
~ 24 ~ sure - is that their support for each other was unwavering - at that moment they were as one.
~ 25 ~ Chapter 4 – Hope
Larry’s daughter Karen lived in Syracuse New York. She was an ER physician and he had been in touch with her to see what she knew about Interstitial Lung Disease. Unfortunately, just as with his PCP - Dr. Dowd, she had general knowledge of medicine but was not trained in the discipline of pulmonary diseases.
“It all depends on the type of disease that you have dad,” she answered to one of his questions. “Your HRTC report does not give much information except to say ‘they suspect’ you might have something. There is scarring, but at this time, they don’t know how serious it might be or if it is progressive - some can be treated, others like fibrosis is very serious.”
“We are thinking of going out to visit you guys next week. Do you know of anyone at your hospital who can read my HRCT images and give me another opinion? I have them on disk,” Larry asked.
“Yes, of course. Bring the disk with you and I will see what I can do.”
Larry and Ewa took the three hundred mile journey to Syracuse just before Labor Day. The trip was uneventful and the three day visit with family was wonderful. Karen has six children but is still able to maintain a professional career, spend time with her family, and keep her tall slender figure in shape. She is her father’s daughter in every way – intelligent, strong willed, resolved, and stubborn as can be. Her children are her life, and she loves them deeply - a person can easily see this as it emanates from her very being when
~ 26 ~ she is with them. Unfortunately, Karen did not have time off from work during Larry and Ewa’s visit but spent as much time as she could with them after work. On the second day of the visit, Karen came home from work with some wonderful news. The radiologist at the hospital read Larry’s CT scans and said Larry had nothing to worry about. Yes, his lungs were damaged, and yes, when he ran or did hard exercise he would notice a decreased level of stamina, but he didn’t see signs of inflammation so the disease was not active and was not progressing. Their hearts were just bursting with joy at this wonderful news – all their worries were for nothing. Ewa and Larry could go on with their lives.
Several days later after they returned home, and Larry being the cynic that he is, started thinking about the reevaluation of his HRCT readings by the radiologist in Syracuse.
“This is just a snap shot in time,” he thought. “How would the radiologist in Syracuse know what is going to happen to my lungs in the future? How does he know with certainty that the disease is not progressive? Why would he say not to worry when the information is not definitive?”
Larry called his daughter and explained his thoughts and concerns.
“He sounded pretty sure about his diagnosis,” she said. “But I can see where you’re coming from – he is reading the scan at that moment in time.”
“WOW, in big capital letters. So it’s not over. This thing is still hanging over my head. I guess I will just have to wait and see after my tests are completed in December,” Larry thought.
~ 27 ~ What Larry didn’t know at the time, but the radiologist should have known, is that IPF is not an inflammatory disease like most of the other Interstitial Lung Diseases. That is why he didn’t see any inflammation on the scans. It is also why IPF is no longer treated with prednisone which reduces inflammation.
Larry attempted to resume his normal life during the fall months of September, October and November waiting for December to come so he could finally get some answers. It was a difficult time - a seed was planted and now the fruit of that seed had its tentacles clutching Larry’s thought process.
“How to purge these negative thoughts out of my mind?” Larry thought.
Almost everyone has had a catchy song or a jingle that was heard on the radio or TV that played over and over again in their head. Larry learned that endless loops of the mind actually had a name. It’s called ‘earworm’, or more scientifically, ‘stuck thought anxiety symptom’. Although not completely understood, the theory is that stress increases the brains electrical activity which in turn increases thought generation. These excessive thoughts cause one to replay that thought over and over again. The mind cannot rid itself of that thought whether good or bad.
Logically, Larry fully understood that if indeed, the scarring was so small as to label it ‘suspect’ or ‘possible’ that it would be impossible to diagnose with certainty at this early stage - but nonetheless it weighed heavily on him and he couldn’t shake it off. He knew he couldn’t change the path he was on - the ‘why me’ thing
~ 28 ~ that people often delve into – sometimes called a ‘pity party’ was never in his vocabulary. Ewa once asked Larry, ”Do you ever ask yourself ‘why me’?”
“Never,” Larry responded. “When you asked yourself ‘why me’ it almost conotates ‘why me and not someone else’. It’s like a person wishes it had happened to someone else instead of them. I don’t think that’s a nice thought. Or it could simply be taken as ‘why me’ as in - why was I so unlucky as to have this happen to me. Either way – it’s a pity party.”
Unfortunately, Larry was not a religious man, and sometimes he regretted that. He often admired people who took solace in praying to a higher being asking to be relieved of their pain and suffering. It allows the faithful to find peace and comfort. Larry feels that a higher being, if one exist, doesn’t take sides, nor favors one individual over another. That isn’t to say he doesn’t believe in spiritual beings, or that each life has a human spirit – it’s just that he doesn’t believe a higher being chooses who is more important – this individual or perhaps that individual. If as they say, “We are all children of God,” then all his children are equal. “We all have free wills and independent lives,” he would say. “To be contrary to this would just make us robots to a higher being.”
So he and Ewa spent those fall months going on walks, enjoying time at the beach, and riding their bicycles - whatever they could do to set aside any unpleasant thoughts for the time being and get the most out of their life together.
“I don’t want to tell anyone about this issue,” Larry told Ewa. “Especially the children.” “Why not? The kids should know”
~ 29 ~ “That’s my point. We don’t know anything yet. Let’s wait until we find out if this is something real or just needless worry. There’s no point having them concerned for nothing.”
“Karen knows. She’ll probably tell the other children”
“I don’t think so,” Larry replied. “She was pretty happy with what the radiologist had to say at her hospital in Syracuse and I think she believes it’s a non issue. So let’s just wait. Besides I don’t want people asking me about this issue every time I see someone. If there is anything to it then it will all come out in time.”
The days quickly passed and the upcoming HRCT scan was scheduled for the second week of December – about a week before the follow up visit with the pulmonologist. During his last visit, Dr. Ipwich told Larry he would wait a year from the date of his last PFT (pulmonary function test) test before repeating it so that test would not be done at this time.
“So how are you doing today Mr. Martel,” Dr. Ipwich said upon entering the examining room.”
“I’m fine no changes.”
“Any discomfort or pain?”
“No.”
Dr. Ipwich walks over to the computer screen and brings the images of Larry’s HRTC up for viewing. “I don’t see any changes to the nodules that we are following. They are stable and have not grown. We will do another scan in six months to make certain that there is no growth.”
~ 30 ~ “Yes, I saw that from the radiologist report. What about the scarring? I see the radiologist didn’t address that issue.”
“No, I guess he focused on the nodules.”
“But I thought this visit would be more about the scarring and the Interstitial Lung Disease. That’s what I’m interested in finding out about. I’m not nearly as concerned about the nodules as I am about the progression of the scarring.”
Dr. Ipwich begins to scan through some of the images on the computer screen. “I don’t see any difference from the last scan. It looks stable.” Adjusting his stethoscope Dr. Ipwich continues, “As I said, we will follow up with another scan in six months. Sometimes these things take time to resolve and will have to be followed over long periods. Let me listen to your heart and lungs before you go.”
Larry straightens himself up in the chair as Dr, Ipwich places the stethoscope on his chest and moves the probe around. As Dr. Ipwich moves to his backside he instructs Larry to take deep breaths stopping for a particularly long time on his lower back.
“What are you hearing?”
“There’s a little crackling in the lower lobes but very faint. I could just barely hear it. It doesn’t seem to have changed since your last visit. As I mentioned, it could simply be scarring from a previous injury to your lungs. We may have to follow this for years to see if it is progressing.”
“Years?”
~ 31 ~ “Yes, we’ll do another scan in six months, if nothing develops a year later, then eighteen months, and maybe two years until we are sure it hasn’t progressed.”
“And if it starts progressing?” Then what?” Larry asked.
“We will tackle that when and if it comes. In the mean time you are asymptomatic and healthy. Enjoy your life and stay positive.”
~ 32 ~ Chapter 5 – The Decision
A week later, Larry and Ewa celebrated Christmas with all the joy and happiness of Christmases past – at least on the surface. Larry’s nagging concern of what was happening to his body dampened his feelings of the true spirit of peace and joy of the season. He did his best to shield Ewa from his mental woes trying his best to not let it show. After all, how could he spoil Ewa’s holiday season in any way.
They picked out a beautiful tree at the local tree farm and spent several hours decorating it. Ewa so loved to brighten the house with Christmas cheer. Larry on the other hand was not really a scrooge but he could have done with less fanfare for the occasion. Nonetheless, the tree and all the decorations that went along with it made Ewa happy and that’s what it was all about for Larry – seeing his bride happy. Of course, the tree took center spot in their sunroom. The lights reflected off the windows creating an incredible storybook like fantasy with a dozen images projecting on all sides as you walked into the room. Christmas Eve was spent with friends at a neighborhood get together. A yearly gathering that was organized not only to enjoy the holiday spirit but also to collect food donations for the city’s less fortunate. It was such a happy affair and Larry and Ewa looked forward to seeing old friends and neighbors. The event was also a welcome respite from the norms of everyday living by sharing stories and jokes, or just getting caught up with friends that pass you by each day. It was a beautiful opportunity to lift one’s spirit.
~ 33 ~ Christmas day was spent with just the two of them together. The children always came to their house for Thanksgiving festivities so Christmas for them was spent with their other half’s families. It was kind of an arrangement that had been formulated several years ago and became a tradition. Great at Thanksgiving with lots of love and joy – but sort of empty on Christmas day. However, Larry and Ewa made the best of it with a quiet dinner and talk of the coming year.
The snowstorms of 2012 came and went and Larry shoveled his eighty-five foot long driveway along with a four foot wide path completely around the house by hand as he had in years past. His breathing was normal and there were no issues with lack of oxygen - perceived or otherwise. He felt good. “How can there be anything wrong with my lungs? I must have shoveled two tons of snow today,” Larry thought after one snowstorm. The problem is . . . . as hard as he might try to convince himself the disease doesn’t exist, he was not fooling himself. Larry only relies on empirical data. Anecdotal stories and events, such as him shoveling snow, serve no purpose. He will never get this disease out of his mind until he sees data - black and white data - that shows him proof positive that it’s not there.
In March, Larry’s yearly physical exam and lab test were all excellent. Dr. Dowd did emphasize the need to get both available pneumonia shots now that his lungs were compromised.
“But we don’t know for sure if I have Interstitial Lung Disease.”
~ 34 ~ “Yes, but your lungs are damaged from the scarring and you are at that age where your immune system is not as good as it used to be,” replied Dr. Dowd.
“By all means go ahead. How good are these vaccines?”
“They won’t stop every pneumonia but they will stop more than forty types. First, I will give you the PCV13 than next year we’ll give you the PPSV23. I’ll have my nurse come in after our visit to give you the first shot. And don’t forget your flu shot each year. As I mentioned, your immune system is not what it used to be so make sure you follow up with a new vaccine each fall,” Dr. Dowd said in her most no nonsense voice.
“I always get the flu shot,” Larry exclaimed. I haven’t missed a year since I don’t know when – probably back in the eighties.”
“Let’s have a listen to your heart and lungs before you go,”
“Do you hear the crackling?” asked Larry.
“Yes, but it’s very faint – it hasn’t changed.”
“That’s great that’s exactly what I wanted to hear,” Larry said with a smile.
“We’ll see you in three months for your blood pressure check.”
“Thank you doctor I hope you have a great day.”
His six month CT scan was fast approaching and over the winter months Larry had given his medical condition
~ 35 ~ much thought. “Ewa, I think I’m going to cancel my next CT scan along with the pulmonary function test,” he declared one morning at breakfast. “I just don’t see how those tests are going to do me much good except to weigh down my mind with more negative thoughts.”
“Do you think that’s a good idea.”
“I don’t see how it’s going to make a difference one way or another. If I have this disease they can’t do anything about it anyway. So what’s the point – just so they can have more data for their records?”
“Well, it’s up to you. If it were me, I probably wouldn’t want to know either and you can always go back for more tests later if you change your mind.”
“I’ll call Dr. Ipwich’s office later this morning and tell them what I’ve decided.”
Later that morning Larry did call the doctor’s office and cancelled both tests as he had discussed with Ewa. The nurse was concerned that he might be making the wrong decision and told Larry she would pass the information on to Dr. Ipwich. About a week later Larry received a call from Dr. Ipwich.
“Hello Mr. Martel, Dr. Ipwich calling.”
“Yes, Doctor.”
“I understand you do not want to have your CT scan or pulmonary function test done.”
“That is correct. Except for gathering data exactly, what are these test going to do for me? If you find I have Interstitial Lung Disease there’s no cure and there’s no
~ 36 ~ treatment at this time except to help calm the symptoms. If you can’t fix or ameliorate the condition then ‘it is what it is’ – it’s as simple as that.”
“We need to check on the nodules. I would hate to have to tell you at some later date that you don’t have Interstitial Lung Disease but your lung cancer has grown and spread because we didn’t follow up on it.”
“I am not concerned about lung cancer. As I said, ‘it is what it is’ and I probably wouldn’t want to be treated for it anyway. Statistically, and I know that you know this, for all types of lung cancers the five year survival rate is only twenty-five percent. And what about the quality of life during those five years or even worse what about the quality of life for the seventy-five percent who don’t make it five years. The surgeries, the chemo, the x- rays – for what? Only to have a few crappy months or years of pain and suffering that allows one to ‘exist’ – not live, but exist - from day to day.”
“Well, Mr. Martel, I think you’re making a big mistake.”
“I mean no disrespect doctor, but I think you have to say that to me and I appreciate your concern but no more testing. I would, however, ask you if you would continue to see me on a yearly basis to follow up with my condition. I know you wouldn’t be able to diagnose me when you have no data available, but I would be most interested in a yearly informal assessment of my condition.”
“Yes, I would be willing to do that. If you change your mind about the CT scan or the pulmonary function test please call my office. Otherwise, I’ll have my nurse set up an appointment for December.”
~ 37 ~ Larry and Ewa enjoyed spending time at the ocean. They would drive the sixty miles or so every couple of weeks - year round - to walk on the beach, ride their bicycles along the shore, or visit local restaurants. They had a favorite ice cream stand where Ewa would always order ‘death by chocolate’ and ‘moose track’. Larry kept it simple with his vanilla soft serve cone. They would then take their delectable treat and walk across the street to sit on the sea wall overlooking the waves as they came in endlessly to shore. Who could ask for more – warm sunshine, a gentle ocean breeze, and the smell of salt water, while filling the palate with such a delectable creamy sweetness? One day as they were sitting on the sea wall Larry turned to Ewa, “We like it here so much, what would you think of renting a place?”
“I would love that. What did you have in mind?”
“I don’t know off hand – I was just thinking out loud. Let’s look around and see what’s available.”
Much to their surprise and delight they quickly found an ocean front condo on Hampton Beach – 5th floor with incredible views of the ocean and surrounding areas. At high tide the water was no more than a hundred feet from their balcony. They elected to rent for seven weeks in late spring (May/June) and seven weeks in early fall (September/October). As much as they loved the ocean, summer was not a favorite time to be there. “Too hot, much too hot,” Ewa would say. So the weeks that were available suited them perfectly.
Route 1A runs along the coast of the ocean – it is incredibly flat and has miles of beautiful ocean views absolutely suited for bicycle riding. Larry usually took the lead on their excursions and Ewa felt comfortable
~ 38 ~ doing it that way. With Larry in the lead, she didn’t feel the pressure of having to go faster than she was comfortable with as she often did when Larry was behind her. Larry always kept an eye on Ewa in his rear view mirror and if she got a little behind he would slow down until she caught up. Their normal routine ride was twenty - twenty-five miles round trip of which Larry had no lung issues, except for the one and only hill on the route that made him a little winded. He wasn’t sure if being winded was from his pushing the hill or from his lungs not keeping up, but he paid it no little mind.
Later that fall after returning home, Larry started his gym routine again. He noticed that pushing heavy weights on the chest press, leg press, or even arm curls caused a shortness of breath he hadn’t experienced a few months back in the late spring. His mind immediately recalled the stair climb during Nurse Delrio’s appointment in which his oxygen saturation had dropped into the low nineties. It concerned him a little that this was happening but it didn’t stop him. Larry knew the old adage ‘no pain no gain’ was a fallacy, after all he was a certified personal fitness trainer, and would never utter those words to any of his clients, but on ‘his’ personal level, the motto made perfect sense. “You have to feel it to make gains, you have to push beyond,” he would say to himself. And so he continued to push through it.
The holidays were fast approaching and so was Larry’s visit to Dr. Ipwich. Nothing much had changed since he saw him last December so he wasn’t sure what this appointment would accomplish in terms of information. However, Larry’s reasoning for having these appointments was that if this potential medical issue did rear its ugly head in the coming months, he wanted an
~ 39 ~ available physician who was familiar with his case. He certainly did not want to start searching for a new doctor nor start from scratch going over all the details with someone new. So there was a purpose to these visits - opening a line of communications with this doctor, as well as, getting to know each other in the event that medical issues developed in time.
“Good afternoon Mr. Martel, how have you been since the last time I saw you.”
“Hello doctor, I don’t think anything has really changed in the last year. I can still go up five flights of stairs without much trouble – maybe some breathlessness at the end but I’m not huffing and puffing.”
“Five flights of stairs? That’s good,” Dr. Ipwich said as he seated himself on the stool in front of Larry. “You may be a little deconditioned which is why you have some breathlessness at the end. I don’t have any lung problems and I get a little winded after going up five flights of stairs. Let’s have a listen to your heart and lungs.”
Larry turned a bit in the chair he was sitting in so Dr. Ipwich would have better access to his back and chest.
“Now deep breaths – again – again – again -- again.”
Completing his evaluation with the stethoscope, Dr. Ipwich returned to his former position on his stool. “I don’t hear anything unusual or different then I did last time you were here. There’s some distant faint crackling but it hasn’t gotten worse. As I mentioned in the past, it could just be a past injury to your lungs that left scarring in the healing process. That’s about the most I can say because you won’t let me do a CT scan.
~ 40 ~ But from what you describe, and from what I hear you seem to continue to be asymptomatic.”
“That’s fine, I understand that I have limited you by not allowing you to do any testing but as I said in the past, ‘it is what it is’ and thank you for seeing me. Barring any unforeseen circumstances, I’ll see you next year.”
And so the visit went smoothly and lasted all of fifteen minutes.
~ 41 ~ Chapter 6 – Reality
During mid-winter 2013, Larry started to notice that snow shoveling was becoming more difficult and he would often have to stop to catch his breath. “Maybe it’s heavier than normal, or maybe I’m trying to go too fast,” he thought. But the reality is that the progression of the disease had been weighing on his mind more and more. “Stay positive and don’t let it get to you.” Try as he might to stop bad thoughts from engulfing his mind, the image of the disease was planted many months ago and was flourishing. “Of all the stupid things that could go wrong with me, it’s my lungs. My lungs - that which I’ve relied on so heavily all these years.” Larry’s family had a history of heart disease and diabetes. That was the primary reason why, for last thirty-five years, he had concentrated so hard on a healthy life style through nutrition, aerobic training, and muscle building. That is also why he wasn’t worried about the nodules in his lungs. Not a single case of cancer was ever reported in his family. Heart disease and the inability to control one’s sugar was always the downfall of his ancestors – on both sides of the family. He knew his gene pool was not with him on these issues, and he was determined to be the first in his family to not follow in his ancestor’s path of destruction. “But the lungs? Who would have thought?” expressing internally his dismay that this was even possible. Yes, Larry was a former smoker - but that was some thirty- five years ago before he did a reality check of where his health was headed. “Actually lung cancer would make more sense,” he thought. But with the lack of a family history of cancer, he was confident that the nodules were benign and never gave them any serious thought.
~ 42 ~ Spring brought his annual physical exam. It seems Larry’s blood pressure had up ticked a bit so Dr. Dowd increased one of his blood pressure medications by a couple of milligrams. Larry explained to Dr. Dowd that he was stressed because his internet business was taking up a lot of his time.
“Maybe you should give it up?” said Dr. Dowd.
“Being retired, the business gives me something to do with my time so I don’t want to give it up. Besides it’s growing like crazy. It’s a challenge to see how far I can take it.”
“You need to find a way to relax and get away from the business once in a while,” she said. “You mentioned that you used to meditate - that would help a great deal.” After checking his heart and lungs Dr. Dowd reported that she was not hearing anything different in his lungs then what she had heard during his last visit. This was good news and made Larry very happy.
One quarter of the year had passed them by and it was now early May. Larry and Ewa were packing up to go to the beach condo once again. Having totally enjoyed the condo last September and October they were eagerly anticipating their second go round and looking forward to continuing where they left off last October. They were not disappointed. Except for the continued stress of the business which they ran from the condo, it was a great seven weeks of fun and relaxation. Their daily five mile walks or twenty plus mile bike rides were always the order of the day. “That was a good walk. A hair over one and a quarter hours for five miles,” Larry exclaimed to Ewa. “That’s about four miles an hour – fifteen minute miles.”
~ 43 ~ “Not bad,” Ewa returned with a smile. “But I’m tired – a little too fast for me.”
“Yeah, I guessed I pushed it a bit. Sorry. I guess I’m feeling a bit tired myself.”
Larry didn’t say anything to Ewa, but on this occasion he had deliberately walked fast in an attempt to prove to himself that nothing was wrong with his lungs. He knew she could keep up with him as Ewa was a true walker. In fact, she loved to walked more than anything - almost addicted to it. She felt like something was missing on days she had to forego her daily excursions. Larry felt her pain on those days. He used to feel that way about running and to this day identifies himself as a ‘runner’. His gravestone, which was purchased several years ago when his wife died, was already inscribed with an etching of a runner. This is who he was and he wanted everyone to know. While at the beach he made several attempts to start running again but his efforts were futile - less than a half mile and he was gasping for air. He thought, “It’s been a long time maybe too fast? Maybe if I slowed to a ten minute mile – maybe an eleven minute mile – maybe a twelve minute mile? Maybe if I run a few hundred feet, then stop to recuperate, then start up again - over and over until I get enough stamina to run steadily?” Nothing worked - after three weeks he realized that it was not getting any easier and that each run was probably desaturating his oxygen to unsafe levels. He would just have to continue walking, “UGH”, he thought. As a runner he hated walking. ”I would rather keel over then walk,” he would say in his glory days. But when it came down to it, he knew that walking and biking with Ewa was much more fun than running by himself.
~ 44 ~ Larry often wondered just how low his oxygen dipped during walks and bike rides so he decided to purchase an oximeter on the internet. Once he received it, he used it for several days monitoring his oxygen levels while walking outside, bike riding, walking around the house, or just sitting doing nothing at all. He discovered that at rest his oxygen saturation level maintained a steady ninety-eight percent – sometimes ninety-nine percent. However, almost any hill or incline he encountered, whether he was walking or biking, would plummet his oxygen numbers into the mid to upper eighties. Ninety percent is considered minimum by most in the medical field. However, insurances and Medicare won’t pay for oxygen supplementation until levels drop below eighty-eight percent upon exertion. Whenever blood oxygen levels fall into the eighties for more than a short period of time, organs and tissues don’t get the oxygen they need to function properly. Over time this could lead to serious health consequences.
“I do recover back into the nineties quite quickly,” Larry thought. “So I’m not going to carry this thing around with me all the time. I’ll just do what I have to do.” So the oximeter was relegated to the top left hand drawer of his desk – ‘out of sight, out of mind’.
Larry and Ewa returned home from the beach at the end of June. The remaining months of the summer, their stay at the condo in September and October, and the Thanksgiving holidays all seemed to pass by with the snap of the fingers. “Look we’re into December again,” Ewa said to Larry. “We were just at the beach enjoying our summer. How can life go by so fast?”
“Do you remember when you were a child waiting for something to happen - like Christmas or your birthday?
~ 45 ~ It seemed like each passing day was forever - the event was a hundred years away and would never seem to come. Now? Well, what can we do about it? Oh, speaking of events I have my pulmonologist scheduled for December 18th. I know you put things on your calendar but just wanted to remind you.”
Larry had been doing a lot of research on the internet regarding Interstitial Lung Disease. He ran across hundreds of articles about miracle herbs, medicines, and concoctions to stop and cure fibrosis – the scarring that occurs with this disease. One particular article caught his eye. “InterMune’s pirfenidone approved in Europe for IPF. The article was written in the PharmaTimes and dated March 4, 2011. It went on to say that, here in the US, the FDA had rejected the drug nine to three and would require further testing if pirfenidone were to be sold in the US. “Well, I guess that’s out,” he thought. However, upon further research Larry discovered that pirfenidone was already approved in Japan (2008), India (2010), China (2011), and Canada (2012). “What to hell? What’s wrong with the FDA? Five countries approve of this drug and they want us in the US to wait for further testing?”
“Ewa look at what I found. There is a drug that can be used in the treatment of Interstitial Lung Disease – specifically Idiopathic Pulmonary Fibrosis.”
“How do you know you have Idiopathic Pulmonary Fibrosis?”
“Well, I don’t. I mean, I don’t even know if I have a real lung issue at this point. But if I do, I believe they would call it Idiopathic Pulmonary Fibrosis - IPF, since the blood work and questionnaire didn’t point to any one cause.”
~ 46 ~ “Really?”
“Yes, they haven’t found a reason or a cause that precipitated why this disease would have occurred. That’s the definition of IPF – no known cause. If I knew there was something out there that could help me, if I do have this disease, I would certainly allow the additional CT scans to be done - but not before. I will bring this information with me to my visit on the 18th and discuss it with the doctor.”
Dr. Ipwich’s office visit was pretty much the same as prior visits. The doctor said the crackling sounds in his lungs sounded as faint as before. However, this time Larry told Dr. Ipwich that going up four or five flights of stairs was very difficult and exhausting. This newest revelation did not seem to concern Dr. Ipwich who said, “Five flights of stairs even with difficulty is still at lot.”
“What do you know about pirfenidone?”
“It’s not an effective treatment and it hasn’t been approved by the FDA,” replied Dr. Ipwich.
“So why has it been approved as a treatment for IPF in five other countries. I saw in my research that Japan approved of the drug back in 2008.”
“It’s not a useful drug and I wouldn’t be able to prescribed it anyway – I would lose my license.”
“How can you say, ‘it’s not a useful drug’. I don’t get it. These are not voodoo doctors we’re talking about. The most renowned scientist and researchers in the world come from these countries.”
“It doesn’t matter, I can’t and won’t prescribe it.”
~ 47 ~ So Larry left the office kind of downtrodden. He was thinking that he had stumbled across some valuable information but it was quickly dismissed by Dr. Ipwich. “He didn’t even want to discuss it,” Larry said to Ewa. “I can understand why he didn’t want to treat me with the current protocol for IPF, which is prednisone, especially not knowing whether I have IPF for certain. That would be a harsh treatment with many nasty side effects which were discussed during our first visit. But this new drug – pirfenidone – has been specially formulated to treat this one disease - IPF. It’s the first available treatment and hope for IPF sufferers. I was hoping that he would let me take this pirfenidone as a preemptive drug just in case. But without a prescription there’s not much I can do and God only knows if and when the FDA will approve it.” And with those words he laid the issue of pirfenidone to rest.
~ 48 ~ Chapter 7 – Easing The Burden
“It seems like the driveway is getting longer with each snowstorm,” Larry exclaimed as he came in through the garage door.
“Do you need some help – I can help you?” said Ewa as she was walking out of the kitchen to greet him.
“No thank you, I’m pretty much done but there’s such a noticeable difference from last year. Maybe it’s just my imagination? I think I’ll get the electric snow blower down from the overhead storage in the garage. We’ll see if maybe that can help me so that I’m not so short of breath.”
“Maybe we should buy a real snow blower. The electric one is not very wide and you have to go back several times if the snow is too deep.”
“It’ll be fine for now. Besides it’s passed mid-winter and I’ve come this far. We’ll talk about it later in the year.”
As it turned out, the winter of 2014 was one of those winters that just seemed to go on and on like the Energizer Bunny. But Larry managed to get through it, albeit, the driveway clearing was a little narrower and the path around the house didn’t get cleaned up for the last couple of storms.
Larry was well aware of the symptoms of IPF – shortness of breath, dry hacking cough, rapid shallow breathing, weight loss, fatigue - except for heavy exertion he didn’t have any of them. Physically, he was still feeling quite normal. Mentally, the possibility of
~ 49 ~ having IPF was like an anchor pulling him down to the depths of the ocean. He could never shake it - it was always in the back of his mind from the time he woke up until the time he lost consciousness to the wonderful world of sleep. “But do I really want to know?” a questioned he wrestled with each and every day. “Even if I knew, what purpose would it serve? They can’t do anything about it anyway. No, you made the right decision – it is what it is. Besides if I knew for certain that I have IPF I might be worst off - mentally. At least now with the uncertainty, I still have hope that I don’t have this horrid disease and that, in some strange way, comforts me.” Fortunately, up to this point, Larry never allowed this ‘mind burden’ to bring about complete despair. Yes, he would be happier if by some strange event those awful thoughts were erased from his mind - but that was not going to happen. “You have to play the cards you’ve been dealt.”
Larry took Dr. Dowd’s suggestion and started meditating again. He tried several of the popular techniques like focusing on a word or number, focusing on individual body parts, or picturing the flow of energy entering his body to make it whole again. “The mind is such an amazing entity,” he would say. “The body is controlled by the mind. What the mind believes the body will become. It can make you ill, or it can restore itself to perfection as it was meant to be. The problem is we clutter our minds and don’t allow it to do its work. I believe this with my whole being. Unfortunately, like everyone else I clutter my mind and confuse it from doing its work properly.”
On occasion during meditation Larry would experience a blue color floating against a black background – small and cloudlike in appearance with shifting intensities and shades which often faded in and out. At first it was just
~ 50 ~ something that was just there - he couldn’t demand its appearance, nor control its movement once it appeared. He realized that when it was there he was more relaxed and more at ease. His session would end with a calmness that he didn’t experience when the blue cloud was missing. He wondered whether it was his energy from within, a subconscious part of himself. That part of why, where, or how the blue cloud came about was never resolved. He just knew it was good and its appearance made him feel good.
“How’s your meditation going?” Ewa asked.
“Well, today it was so so. I had trouble focusing, Too much on my mind. It didn’t happen today but you know sometimes I have this blue cloud that appears when I meditate. I don’t know what to make of it and I’m not sure what it is, but I’d like to think it’s my energy from within.”
“Maybe it is – you believe in that sort of thing don’t you?”
“Yes I do. I’m not sure if it means anything except that it makes me feel good when it appears. I don’t have much else to say about - I just wanted to share my experience with you.”
As late spring approached Larry was concerned about how he would mow his lawn this year. The house sat on roughly two-thirds acre of lawn that needed to be mowed every week to ten days. He remembered last year that he was starting to struggle with his lawn mower. It was gas powered but was not self driven. In fact, when he had bought the house some twelve years ago, a fairly new riding lawn mower was park in the garage and was purchased as part of the cost of the
~ 51 ~ house. After mowing the lawn a couple of times, Larry felt guilty about sitting on his butt and decided to give the riding mower to his son in-law in exchange for the push mower he currently had. Although he hated cutting the grass, he used it as an opportunity to get a bit more exercise by pushing the mower around for two and a half hours or so. “How do you feel about getting a lawn tractor? I’m not sure I can push that old lawn mower around again this year.”
“I told you we should get one – I think two years ago.” Ewa replied.
“You’re right you did. But I just didn’t feel I was ready until this year. It will be so much easier especially with the dozens of hours we spend with fall clean up each year.”
And so that was Larry’s first concession to his decreasing ability to keep up his normal life.
It was mid-May and Larry and Ewa were at the beach again. It didn’t take long for Larry to notice that their bike rides were becoming more cumbersome. He was continually out of breath. Except for one hill in their twenty plus mile excursions, the shore road was as flat as a pancake. “I’m starting to have a hard time pacing myself,” Larry thought as they were riding along.
On their next ride, Larry felt he would be better off if he became a follower.
“Ewa, why don’t you take the lead today,” Larry asked.
“I can – it’s just that you know I feel more comfortable with you in front.”
~ 52 ~ “I know. But I’m starting to have a hard time. Maybe with you leading us I’ll have a different perspective – besides I love riding with a beautiful woman in front of me.”
Ewa took the lead at her usual pace with Larry following shortly behind. It was good at first but on their way back – somewhere around mile fifteen, “Thirteen . . . . thirteen and a half miles per hour – good pace but I can barely keep up,” Larry thought. “I’m glad we only have five miles to go.”
When they finished Larry did not want to say anything to Ewa about his struggle.
“Good ride and thank you for leading. Can you continue to take the lead from now on? It was easier for me and I really appreciate it.”
As the season progressed Larry’s bike rides became more and more difficult. It would be the last summer he would ever bike again. His total mileage for the season was only ninety-one and a half miles.
The lawn tractor was working out really well. Even while they were at the beach Larry would return home every ten days to mow the lawn.
“I love the tractor. It’s a lot faster than the two and a half hours it used to take me to do the lawn and I’m not exhausted when I’m done. Maybe I should give in and buy a snow blower as well. Last year was very tiring and stressful and I don’t want to go through that again,” Larry said after returning from mowing the lawn at home.
“Maybe we should hire someone to plow the snow?”
~ 53 ~ “No, I don’t think that would be a good idea. You can see how they dig up the lawn at Sheila’s house. It would just be more work for me to repair all the damage each spring.”
“You’re right. I was just thinking it would be easier on you.”
“When we return home why don’t we take a look at what’s available. I’m not sure what it cost to get a good machine.”
The snow blower would be another concession to Larry’s health issues but a needed one. Despite Ewa’s assistance last year in clearing the snow from the decks and portions of the backyard, Larry knew there was no way he could shovel the snow by hand this winter - even with the help of the little electric snow machine.
His visit to Dr. Ipwich that December was non descript with the usual questions about how he was feeling and a heart and lung check. Larry did not address his latest concerns about being tired all the time or how he was getting more and more out of breath with mild exertion. Without data through CT scans or pulmonary function testing, he knew he was limiting Dr. Ipwich’s ability to diagnose and treat him. He saw no point in theoretical discussions. During late summer Larry had come to realize that he really did have a medical issue with his lungs. Dr. Dowd had ordered an echocardiogram during his last yearly physical exam in the spring and the results from that procedure were normal – so he knew it wasn’t his heart that was giving up on him. He knew that it must be his lungs not absorbing the oxygen he needed. Whatever it was, it was progressing slowly but steadily. After three years his worst nightmare was
~ 54 ~ coming to fruition and he didn’t need a doctor to tell him he was in some serious trouble.
~ 55 ~ Chapter 8 – Looking For Answers
The new snow blower was a blessing sort of. It was a powerful machine, commercial grade, cleared the deepest snow and ate up the plowed compacted snow at the end of the driveway with ease. There was nothing that got in its way – it was truly worth the money. However, with all these compliments comes its drawbacks. It was also a monster of a machine which Larry found very difficult to maneuver due to his current medical condition. Going in a straight line was not a problem with the impeller sucking in great swaths of snow and spitting it out through the chute. But manhandling the machine on turns or physically adjusting its path while in reverse was a trying and exhausting experience. “Wow what have I gotten myself into?” Larry thought. “This was supposed to make my life easier. I’m totally out of breath.” As demanding as it was the machine did save a bunch of time but it was way too heavy and taxed his oxygen levels to the max.
“How do you like your new snow blower?” Ewa asked as he came into house.
“I should have listened to you and bought a machine that is more standard and not so commercial. It is way too heavy. But the upside is that it does a terrific job – and it’s fast.”
Unfortunately, the new snow blower did not make Ewa’s life any easier as she still had to manually shovel the snow off the decks and along portions of the backyard. Larry did try to help shovel alongside of Ewa several times, but it only exhausted him to a point where he had
~ 56 ~ to stop and sit down to recuperate from lack of oxygen. “I told you I would do it.” Ewa would say in the background. “Why are you jeopardizing yourself. I really don’t mind. Please just let me do it.”
As much as Larry feared being put on oxygen, he decided he would talk to Dr. Dowd about getting tested for supplemental oxygen. He explained to her that even walking at a reasonable three miles per hour (twenty minutes a mile) was difficult. “I can push myself but when I get done I’m huffing and puffing and can’t catch my breath – it seems that there is no air to breath.” Because oxygen is a prescribed medical item, Dr. Dowd told Larry that he would have to walk around the clinic accompanied by her assistant with an oximeter attached to his right index finger to see if he qualified. In order to be qualified for insurance coverage, a patient’s oxygen saturation must fall below the critical level of eighty-eight percent during exertion. On the first loop around the office area, and at a very brisk pace, Larry’s oxygen level dropped to ninety-one percent. As he continued on and nearing the completion of the second loop he saw that the oximeter’s reading was dropping quickly . . . ninety percent . . . eighty-nine percent . . . eighty-eight percent . . . eighty-seven percent. “Stop,” the assistant commanded. “Let’s try it again with some oxygen.” The assistant rolled an oxygen tank, which she had been pulling behind her, to Larry’s side. She then attached a cannula around his neck and inserted the rubber prongs into his nose. After adjusting the valve on the side of the tank to read 2 lpm (liters per minute) she said, “Go ahead. Start walking again.” Larry resumed his walk and completed two loops this time before his level eventually dropped again to below eighty-eight percent. “Stop”. The assistant adjusted the black knob on the oxygen tank and had Larry start
~ 57 ~ up again. This time he maintained a level above eighty- eight percent. Larry returned to the examining room where Dr. Dowd was waiting. “It looks like you need 3 lpm of oxygen to maintain a saturation level above what is considered safe,” she explained to Larry. “I’ll write you a prescription and you should hear from the medical supply company in a day or two.”
A couple of days later the medical supply company called Larry asking him when the oxygen machines could be delivered. They explained that he would be getting a stationary floor unit which provides a steady stream of oxygen, a portable unit that puts out pulses of oxygen coordinated as each breath is taken, and an emergency cylinder that was to be used in the event there was a loss of power. “I’ll pick them up myself,” Larry said responding to their original question about delivery. He still had not told anyone of his condition and he didn’t want a medical supply van parked in his driveway for all to see.
When he picked up the units the next day, he attempted to tell the representative, who was assisting him, that he didn’t need the stationary concentrator as walking around the house was not a problem. The representative told Larry that medically and for the safety of their patients they could not just provide a portable battery operated unit without providing a full time plug in unit. Larry thought about it for a second and said, “That makes sense.” However, he did pass on the large emergency cylinder as it did not fit in his car. He told the representative that he would call when he wanted it delivered - he never did.
Larry fiddled around with the portable unit a bit, exercising with it once or twice but could not get his head around walking in public with a hose up his nose.
~ 58 ~ Being an athlete all his life created a mental wall that would not allow him to be seen in public in such a diminished state. “You really shouldn’t let it bother you,” Ewa said. “It’s hardly noticeable and it will help you exercise better.”
“I’m just not ready. People that are handicapped are looked upon differently. I don’t care how opened minded someone is. That’s pretty much a fact. It may be my pride but I can’t deal with that.” He ended up putting the portable unit under his desk and did not use it at all.
Sleep apnea had been brought up at various doctor’s visits. If his saturation level was low during exertion was he getting enough oxygen while he slept? Larry had always diverted this type of discussion by asking Ewa if she had ever noticed that he stopped breathing while he slept. “Never,” she would reply. He would then turn the discussion into another subject. However, in the back of his mind he wondered whether the doctors may be on to something. During one of his research days at the computer, Larry came across a wrist oximeter that could be worn overnight. The machine collected O2 data at one second intervals throughout the night which could then be downloaded to a computer for printing various reports and graphs. Larry knew that during sleep a person’s breathing becomes slower and shallower which in his case could lower his O2 values to unacceptable levels. “Hmmm, not a bad price,” he thought. “Even if I don’t have sleep apnea it will let me know if my numbers stay above eighty-eight percent.”
Upon receiving the new wrist oximeter, Larry did his first sleep study. He found the device to be much like a watch. It was strapped to the wrist with a five inch wire
~ 59 ~ extending from the unit to a probe that was placed on a finger. Larry found the device to be very comfortable to wear, and discovered that the display would disappear after fifteen seconds so the brightness of the light wouldn’t affect a person’s sleep. The next day, Larry downloaded the results and found that there were three times during the course of the night that his numbers dipped to eighty-eight percent; the effort of getting into bed, his nightly trip to the bathroom, and getting out of bed the next morning. It also showed that he quickly recovered in less than a minute. Overall, the data showed he was above ninety-five percent ninety-eight point seven percent of the time. “Hey Hon, it looks like I don’t need oxygen at night.”
“That’s great. Can I see the results?”
“Sure take a look. I am really happy about that but I will repeat this test every three or four months to make sure things don’t change.”
Larry spent hundreds of hours on the internet researching medical websites, forums, trials, natural remedies, and any and all documents related to IPF. He learned that if he did have IPF the prognosis was less than promising – sooner or later it will get you. Worthwhile trials, that is trials to cure or at the very least restore/repair scar tissue are in their infancy. The ones that looked promising are still in phase 1 or phase 2 – nothing close that would help today’s sufferers. “Crap, with the way the FDA operates in this country this generation of IPF patients and even the next generation or two will die before something is found to be useful. The researchers are still trying to understand the basic processes of this disease. They have no clue in which direction to go,” Larry contemplated as he was reviewing some articles. “How can this be? Forty
~ 60 ~ thousand people die from this disease each year – that’s as many as breast cancer and we’re nowhere with the research.”
One site that Larry found interesting was a forum sponsored by the American Lung Association. It is called Team Inspire and is specific to people with fibrosis. “Ewa look at this site. People post their views, experiences, and opinions while offering their total and full support to their fellow IPF sufferers.”
“Hmmm interesting. Are you going to join?”
“I did join. Each day you receive an email with new posted comments and questions along with updated responses from previous days. I don’t want to actually participate but I think it is worthwhile to get this information each day from people like myself who are living through this. Occasionally someone will post a worthwhile website on the latest research or trials but in general it’s a support group. It’s also sad because people are afraid. Sometimes, it’s apparent in their post but most often you have to read between the lines. They’re looking for help and assurance that everything is going to be okay. Many of the members have their own anecdotal stories of a remedy, herb, or vitamin that helps them or a loved one – others talk about the difficulties they’ve gone through with the disease, still others post success stories to help lift the spirits of others. It’s all good – it’s a safe place for people to interact without being ridiculed for what they want to get off their chest.”
“Can I join? I want to learn as much as I can to try to understand what you are going through.”
~ 61 ~ “Sure, I’ll sign you up. You should start receiving emails tomorrow.”
Larry came across many scam sites that prey on people’s fears. Miracle cures for fibrosis – restore your lungs to when you were young, repair the scarring – just send your hard earned money to us and you will be as good as new. “These sites should be shut down. There’s nothing there that can support any of the things they claim. I guess there are just too many of them for the government to investigate them all.” Larry fully understood why people would try different remedies or concoctions - after all what have they got to lose. “If it doesn’t work, you’re no worse off than the loss of some hard earned bucks.” However, it bothered Larry that many people - especially those living off social security, had no means of researching their own information, or even more importantly, evaluating the credibility of the information they found, were being taken by these scammers. “It’s like gambling,” he said to Ewa. “If you can afford it – go for it. If not, leave it alone. On second thought, I guess that was a faux pas, we’re talking about life and death here so I guess that was a lousy analogy. Anyway they’re selling hope and they can’t deliver results with these products.”
Ewa was in complete agreement, “Yes, it’s like my dad always said, ‘It’s always about the money’.”
“WHAT !” Larry shouted out in a loud voice.
“Is there something wrong?” Ewa shot back.
“Yes and no. The FDA approved pirfenidone over a year ago. I can’t believe I haven’t run across this before today. I mean a year ago.”
~ 62 ~ “Why didn’t Dr. Ipwich notify you of that?” asked Ewa
“That’s not his fault. I wouldn’t allow him to test me so he has no idea what condition my lungs are in and no reason to call me. But now that there is something out there I will certainly call his office tomorrow and ask him to order whatever tests he needs to get me started on this drug. The tests will coincide well with my next scheduled appointment in 2 weeks.”
Dr. Ipwich ordered a HRCT (High Resolution Computed Tomography) and a PFT (pulmonary function test) for the following week. The HRCT went smoothly – but the PFT was extremely difficult for Larry. The technician had to interrupt testing several times as Larry coughed uncontrollably and couldn’t catch his breath as he attempted to inhale and exhale fully as instructed. “Are you okay?” he heard the technician say.
“Yes, I’m fine, I just need a few seconds.”
“Take your time. Let me know when you’re ready.”
Finally, after several more cycles of tests, Larry heard the words he had been waiting for.
“We’re done,” said the technician as he opened the door to the test booth.
“Great I’m out of here.”
And so what seemed like an endurance trial from hell that would never end, it was finally over. Because Larry’s response to the test was so severe - one or two of the tests that were supposed to be repeated three times were stopped after two cycles. The technician told Larry that the data was sufficiently consistent with
~ 63 ~ the two tests that had been completed that a third cycle was not needed. At the bottom of the PFT report the technician wrote, “Patient became extremely light headed and dizzy requiring long breaks between test. Patient also showed signs of being diaphoretic (heavy sweating).”
“I could never imagine in my wildest dreams that such a test could be so devastating,” Larry said to Ewa as they were leaving the test area.
“You scared me. I know you said you were sick and getting worse but in my mind I didn’t believe you - I didn’t want to believe you. It’s not possible. How could my strong Larry who can ride a bike a hundred miles with ease or lift ridiculous amounts of weights be so frail? I couldn’t and still can’t bring myself to thinking this is true. But seeing you like that today - how is this possible – how can it be?”
Larry turned to Ewa, “It’s okay, we’ll work through this.” He put his arms around her and said. “I love you,” as he kissed the top of her head.”
“I love you too.”
~ 64 ~ Chapter 9 – The $96,000 Question
It was the first week of December and it was time to start thinking about another winter. New Hampshire often had snow on the ground by now so Larry didn’t want to press his luck. Today was the day he needed to take out his snow blower to change the oil and fill the tank with fresh gas. He walked around the house to the shed, which had been converted from Sully’s old dog pen on the back of the house, to get the machine out of storage. Because he had emptied the gas tank at the end of last winter, the tank needed to be filled before he could do anything else. Once filled, he set the lever to the choke position - pushed the rubber primer bulb four or five times - then gave the cord a quick pull - the machine immediately came to life. Engaging the drive gear, Larry started to drive the machine around the house. After just a dozen steps, he noticed his breathing was becoming more and more labored, and he was pretty much out of breath upon reaching the garage. “Boy, that wasn’t easy,” he thought. “How in hell am I going to be able to work this machine when there is snow on the ground. After changing the oil and cleaning up the blower Larry went looking for Ewa. “I don’t think I can heft that snow blower around this year. It’s just going to be too much,” Larry said as he walked into the kitchen where Ewa was cooking.
“I tried moving it last winter and it certainly was too heavy for me,” Ewa exclaimed. “Maybe we should sell it?”
~ 65 ~ “We’ll take a beating. I mean it’s like new and I only used it for – I think ten or twelve times last year - but it’s a used unit.”
“How much do you think we can get for it,” asked Ewa.
“Fifteen or sixteen hundred if we’re lucky.”
“But we paid over twenty-nine hundred dollars for it,” Ewa said in dismay.
“Like I said, no matter how you look at it, it’s a used machine. It’s unfortunate because neither one of us can handle it, which makes it zero value to us. We can either sell it or let it sit there. If we sell it, I’ll ask Tom across the street if he would be willing to snow blow our driveway for us. He works from home and has to do his own driveway anyway. I know business is a little slow for him during the winter and this will give him some extra money. This would be ideal, and the best part of it is, if the driveway is done with a snow blower the grass won’t get ruined like what a plow would do. Tom’s a good guy I’m sure he will help us out. So what do you think - sell?”
“Yes, go ahead.”
“I’ll put an ad on Craig’s list this evening.”
And so within four days another chapter closes in Larry’s life. The machine sold for fourteen hundred ninety-nine dollars - about half of what Larry and Ewa had paid for it.
After what had happened during the PFT’s Larry was very anxious about his appointment with Dr. Ipwich.
~ 66 ~ He had known for some time now – at least in his mind – that he had something going on with his lungs. He thought it was probably IPF and he knew it was progressing. Now, as he and Ewa sat in the examining room waiting for Dr. Ipwich to enter, he realized the . . . . ‘hoping I don’t have it mentality’ would be over. After four and a half years there will be a label placed on his condition – for good or for bad. He was thinking about Ewa and how she would be impacted as his health continued to decline. “She has so much love, I hope I can continue to be a good husband.” He had no regrets that he had postponed this moment for so long. After all, any testing prior to this would not have helped him - it would have been done just for data collecting. “Maybe I don’t have IPF. Maybe things aren’t as bad as they seem, maybe ......
Dr. Ipwich enters the room with his familiar, “How are you doing today?”
“Well, I guess you need to tell me that today,” Larry responded.
“Your tests show a significant decline in lung function from four years ago. The nodules are still the same size so there is no cancer. The scarring on your lower lobes indicate that you do have IPF. It must be treated - you will have to start taking medication as soon as possible.”
“You mean pirfenidone? That’s the drug I talked to you about three years ago that you said had no value. I believe the brand name in the US is Esbriet.”
“There’s also another drug that was approved for IPF called Ofev,” responded Dr. Ipwich completely ignoring Larry’s comment about pirfenidone.
~ 67 ~ “Yes, I’ve read about Ofev (nintedanib). Do you feel one is better than the other?”
“Based on the trials that were conducted, they both have about the same effectiveness.”
“One of the things that troubles me about Ofev is that their literature is concerned about heart issues. This is clearly posted on their website. I know if only one person in a trial has an issue they have to report it. But I do have heart issues in my family and I don’t want to have this potential risk hanging over me. Plus, I know there is no generic version of Ofev at the moment and with the cost of either one of these drugs being around ninety-six thousand dollars a year for the brand name, I want to proceed with the generic version of pirfenidone.” “Costs is not a factor,” replied Dr. Ipwich. “I have patients who pay zero dollars for this medication. Let me call the representative from Esbriet who will give you a presentation about the drug, and how you can get it for free.”
“I have no objections about talking with the rep, but like I said, I would like to go with the generic version. I can get it much cheaper in Canada.”
“If you want to pursue the generic after you hear what the drug representative has to ‘say we can do that. She has a lot of information to offer and they are very supportive. But it’s up to you.”
“That’s fine the more information I have the better.”
I’ll have my nurse set up a meeting with the representative and she will get in touch with you in a couple of days.”
~ 68 ~ The meeting with Esbriet’s representative took place at the pulmonology center where Dr. Ipwich worked. As usual, Larry had done oodles of research on Esbriet and pirfenidone and there wasn’t much new information the representative was able to pass on to him regarding the drug itself, or IPF for that matter. He did learn a lot about the Esbriet company and all the support they gave to their patients. He found out that a patient couldn’t buy Esbriet at a regular pharmacy and that only certain specialized pharmacies provided the drug – mainly because of the cost. The representative explained that getting the medication for free was ‘as simple as applying for various grants’ that were given out by compassionate companies, who recognize that most people can’t afford the ninety-six thousand dollar price tag to stay alive. She also said Esbriet would pick any remaining portion that could not be attained. Earlier in the week, Larry had contacted his insurance company and found out the drug was classified as a ‘specialty drug’ and they would cover twenty-five thousand dollars towards his costs. “That’s not very much,” he thought. “I would still need to raise a whole bunch of money.” Now talking with the representative, he was very skeptical about getting funding sources and free drugs. “If it sounds too good to be true,” he thought. The representative went on to explain that if he signed on with Esbriet, he would get a thirty day supply for free to get him started on the medication. This would allow him time to get his funding sources in line. “What have we got to lose?” he told Ewa who was sitting next to him. If it doesn’t work out I can always order the generic version.”
The next day Larry started calling the list of grant sources that had been given to him by Esbriet’s representative. “Hello, I’m calling about grant money
~ 69 ~ that might be available to qualifying individuals for purchasing Esbriet.”
After answering many questions – mostly financial – the person on the other end told Larry they could help him. “You qualify for seventy-five hundred dollars. That’s pending receipt of a copy of your income tax returns. We’ll need that each year for you to stay qualified. Forms for you to fill out will be sent first thing in the morning.”
“Thank you. I hope you have a great day and thank you for your assistance.”
Well, Larry went through that process several times – six times to be exact - and by early afternoon he had accumulated about thirty-eight thousand dollars in grants. “This is going to take forever,” Larry thought. “Plus I still have forms to fill out and tax returns to copy. Forget it, I will just go with the generic and pay for it myself.”
Larry informed Ewa that he was not going to spend all his time sourcing funds to buy Esbriet. The generic version was approximately thirty-eight hundred dollars a year and if she agreed he would go that route. He would call the doctor tomorrow and let him know his decision.
“Hello this is Larry Martel. Can I speak with Dr. Ipwich’s nurse please . . . . . thank you, I’ll hold.”
“Hello this is Dr. Ipwich nurse. Can I help you?”
“Yes, please tell Dr. Ipwich that I’ve decided to go with the generic version of pirfenidone. If he could write a script for me I will be glad to pick it up this afternoon.”
~ 70 ~ “Dr. Ipwich is not here today. Is there a reason why you don’t want the Esbriet?”
“I just think I don’t want to go through the hassle of spending hours filling out forms and being on the phone soliciting money. Besides I can afford to pay for the generic version and people who are in more need than me should get the funding for Esbriet.”
“”I don’t think Dr. Ipwich will agree with you. He had the representative come in so that you would go on Esbriet.”
“Yes, I understand. But Dr. Ipwich also said to me that if I ultimately chose the generic version he would be okay with that. Can you please call me to let me know when the script will be ready?”
“I’ll pass the message along to him.”
Well, Larry doesn’t know what kind of message the nurse passed on to Dr. Ipwich but when he called the next day he was practically breathing fire through the phone.
“Larry this is Dr. Ipwich.”
“Hello Doctor.”
“You cannot talk to my nurse that way or give orders to her.”
“I not sure what you are talking about. I simply asked her to tell you that when you had time to please write a script for the generic version of Esbriet. I asked her to call me when it was ready.”
~ 71 ~ “That’s not the way it’s done. I wrote a prescription for Esbriet because you told the Esbriet representative that you wanted to give that a try.”
“Yes, that’s correct. But now after consideration I wish to go generic. At my last office visit you said we could go that route if things didn’t work out with Esbriet. I have spent hours calling various funding sources and I don’t want to continue doing that. It would be much easier for me to just buy the generic version outright. And that’s what I’m prepared to do. I have picked out a licensed pharmacy in Canada – a brick and mortar situated in a medical clinic in Vancouver Canada – who I wish to deal with.”
“I won’t write a prescription to Canada. I don’t have confidence in the products coming from there. How do you know you would be getting the real drug? It’s not FDA controlled.”
“Come on - Canada’s not a third world country. They have their own rules and regulations to oversee pharmacies and the drugs they dispense. Besides the pharmaceuticals are in bed with the FDA in this country anyway. Like I said, ‘this is not an internet fake pharmacy’. It’s a licensed Canadian pharmacy who happens to do business on the internet as well as their usual retail business. You told me you would allow me to go generic. Won’t you at least look at their website before saying no?”
“There is no need for me to look at the website.”
“You won’t be writing a script to Canada. Just give me the script and I will take care of it. Look, if you don’t write the script I could die sooner than I should. If you do write the script and the generics I receive are fake I
~ 72 ~ will die sooner also. The former makes you responsible for my early demise, and the latter makes the pharmacy responsible for my early demise. Don’t you see, the former is a certain early death, and the latter is an uncertainty, which based on the high level of Canadian medicine has little chance of happening. Canada is not a third world country. So please allow me to make this choice. And what about your promise that if I listened to what the representative had to say I could decide later?”
“I wrote a prescription for Esbriet because you told the representative that you wanted to sign on with them. That’s the only one I will write.”
“Are you in with the drug companies? You had the representative come in special to ‘talk to me and now you’re upset that I won’t buy their drugs. How much of a cut are you getting from them.”
“That’s ridiculous. They’re not paying me anything.”
“Well sure looks like that to me. Good bye and have a nice life.”
Larry sat there silently after hanging up the phone. “Now what?” asked Ewa. She had been sitting on the other side of the sofa listening to the conversation “How can he deny a prescription that will help you with this disease? You did everything he asked and he did say he was okay prescribing the generic if you decided to go that way. How can he do that to you?”
“I don’t know,” Larry replied in a low depressed voice. He was stunned at how the conversation went. He had spent four years cultivating a relationship with the guy. “I can’t believe he was so offended and defensive.
~ 73 ~ Although I said it out of anger, I now wonder if he does have some stake in prescribing this medication? I’m just in a state of disbelief right now.”
“Maybe we can go to Canada and find a doctor up there that will help you?”
“There’s got to be another way. No matter whether I stay here or go to Canada it will take weeks to establish myself with a new pulmonologist. I wonder if Karen can help? I’ll text her.”
Larry was reluctant to contact his daughter as he was concerned that prescribing medication to a direct family member, especially out of state, might not be in her best interest. She responded immediately and told her dad that she certainly would do whatever was necessary to make sure he received what was needed for treatment. “Send me all the information you can about pirfenidone along with any information you have about the pharmacy you chose in Canada. I want to make sure that this is right for you before giving you a prescription,” she texted back to him.
“Ewa, Karen wrote back and said she would take care of it.”
“That’s great,” Ewa responded smiling from ear to ear.
“Yes, but I’m not happy about getting her involved with this. I will, if necessary, but as my daughter she should be my daughter not my doctor. She doesn’t need this on her plate. I’m going to call Dr. Dowd’s office and make an appointment with her. Maybe she would be willing to help.”
~ 74 ~ A few days later Larry was in Dr. Dowd’s office explaining the whole situation to her. “I have no problem giving you a prescription for drugs in Canada,” said Dr. Dowd. “If you can help me by giving me what you have on pirfenidone and the Canadian pharmacy you want to buy from I will review it. You know there are a lot of scam pharmacies on the internet.”
“Yes I know, but I’ve checked this pharmacy out fully - even going to the Canadian ministry’s website to make sure their license was valid,” Larry explained.
“As I’ve mentioned before, I am not qualified to act as your pulmonologist, so you’ll have to work with me on getting someone to take over your treatment for this disease. I’ll make a referral and you must follow up on it. Also, from what I can quickly see by flipping the pages of the documents you just handed me, this drug requires continuous liver testing so you’ll have to make sure your consistent with that also. There may be other requirements, I’ll let you know after I review and research.”
“No problem, I don’t want other issues cropping up like liver disease.”
Larry was totally delighted that Dr. Dowd was so cooperative in working with him on getting the pirfenidone. It seemed like a thousand pounds had just been lifted off his shoulders.
“I’ll have to review this information in the evening after office hours so it may take a few days. I’ll have my nurse call you when I’ve finished.”
A couple of days later Larry received a call saying the prescription for pirfenidone was ready for pick up and
~ 75 ~ that Dr. Dowd had set up orders for liver testing on a monthly basis – at least for the first three or four months.
Larry picked up the script that day and immediately called the Canadian pharmacy. He learned that pirfenidone was manufactured in India by Cipla under the trade name Pirfenex. It would cost about Nine Hundred Fifty dollars USD for a three month supply and would take about four to five weeks to arrive. This costs would have to be absorb as part of Larry and Ewa’s household expenses as the insurance company would not cover drugs purchased outside of the United States. Being on a fixed income, this additional expense would definitely put a dent in their life style. As he was talking to the pharmacist, Larry remembered that he would still be receiving a thirty day supply of Esbriet, and asked if starting on Esbriet, and switching to Pirfenex would be an problem. The pharmacist told him that although they are the same exact drug he couldn’t guarantee there would be no issues because Esbriet is a capsule and Pirfenex is a tablet. “The absorption rate may be different but this should be only a short term issue, if at all.”
Being totally unfamiliar with Cipla, Larry decided he would need to make peace within his mind that the drug he just ordered was on the up and up. Specifically, he wanted to know – who the hell is Cipla? “Let’s take a look at the ‘all knowing’ Google god and see what we can find out. Cipla, incorporated in 1935 – headquartered in Mumbai India with presence in over one hundred seventy countries – employs twenty-three thousand people – FDA approved in 1984 – there is a US subsidiary in Miami Florida – started manufacturing Pirfenex (pirfenidone) in 2010. “Hmm, seems like all is good with Cipla,” Larry sighed with relief at what he had
~ 76 ~ found. “I can’t believe Dr. Ipwich would not give me a script for generic pirfenidone. It even says Cipla has FDA approval. I really wonder what kind of relationship he has with the manufacture of Esbriet?”
The thirty day trial supply of Esbriet arrived by mid- January but Larry held off a couple of weeks to allow the ‘just ordered’ Pirfenex time to be on route. Because the dosage requirements called for a slow increase in medication over a two week period to allow the body to adjust to the pirfenidone, he didn’t want to run out of Esbriet before the pills from Canada arrived. Running out of Esbriet would force him to have to start the slow increase all over again. He marked the calendar – START ESBRIET - February 1, 2016. This would be the big day.
On his appointed day, Larry took his first dose of Esbriet. He apprehensively waited for something to happen from the long list of side effects printed on the brochure that came with the pills - nothing happened. He continued upping the dosage as prescribed until he was at full dosage. His planning was perfect as he had just reached his full dosage requirement when the generic pirfenidone arrived. It was going smoothly, almost too smoothly, as Larry was tolerating the medication well. There was serious nausea, vomiting, heartburn, stomach pain, sensitivity to the sun, or any other of the many side effects that was on the list of warnings.
One thing that puzzled Larry was that both the manufacturers of Esbriet and Pirfenex recommended that food be consumed prior to taking the pills in order to lessen side effects. However, there were no recommendations as to what type of food (fats, protein or carbohydrates) nor the quantity of food that should
~ 77 ~ be consumed to avoid the pitfalls of side effects. It seemed that by eating something with or just before taking the medication, altered the amount and rate of absorption of the drug. “But surly if not the kind of food then the amount of food should be a big deal,” Larry thought. “If you eat too much you’ll slow down the absorption of the drug and it may not be as effective – if you don’t eat enough then the drug will get into the blood stream too fast – presumably causing side effects.” However, despite much research effort, he could not find a written statement with food recommendations anywhere on the internet including those of the manufacturers.
Larry had asked Dr. Dowd to prescribe an anti-nausea medication in the event that he would need it to counter the effects of the pirfenidone. So he was prepared for the worse. But the worse never happened and the anti- nausea medication was unnecessary. Except for a touch of very very mild nausea which occurred about two hours after taking his morning dosage, and lasting about two or three hours, he never felt any serious adverse effects. His transition to the generic was also smooth and caused him no undue stress. Larry tried a multitude of different food choices ranging from half to quarter sandwich with meat, cheese and veggies, fruit (banana), combination of fruit and meat, even chicken McNuggets to name a few but the mild nausea stayed with him for several weeks - then one day it had pretty much disappeared.
During the first week of February, Larry received a call from a nurse at one of Northern New England’s largest medical facilities. It seems that Dr. Dowd had made a referral to one of their doctors. “Hello Larry we received a referral from Dr. Dowd for you to see Dr. Gilson.
~ 78 ~ We’ve set up the appointment for March 23rd at 2:30. Is this a good time for you?”
“Is that the earliest date you have? That’s over six weeks from now.”
“Yes, it is. Actually we’re scheduling three months out for new patients but because Dr. Dowd is from one of our affiliate clinics I was able to squeeze you in sooner. If you like, we can schedule this in and also put you on our cancellation list in the event an earlier date opens up.”
“Yes, great. That works for me.”
Unfortunately, there were no cancellations as Larry never got a call to move the appointment up to an earlier date.
~ 79 ~ Chapter 10 – Uncertainties
The IPF seemed to have stabilized which is often the case with this disease. It never really goes into remission but it can go weeks or even years without much progression. Larry started thinking about how he felt a year ago, or even six months ago, but it was difficult to assess and compare time frames. “I had a hard time with the stairs today,” he thought. “Is this worse than it was in October after we left the beach condo? The changes are so subtle that I can’t be sure. Besides I think it would be just wishful thinking if I thought that my condition was the same.” These self evaluations of his current medical condition versus some past time frame were quite frequent. Larry was trying to make himself feel better. Yet, he knew the comparisons were useless and he always came back with the same response, “Can’t know for sure and wishful thinking.”
The March 23rd appointment with Dr. Gilson was fast approaching and Larry did his usual research to review his credentials. He saw that Dr. Gilson was a clinician, professor of medicine in microbiology and immunology, and vice-chair of research affairs. Clinically, he specialized in lung disease, particularly the idiopathic interstitial pneumonias. His laboratory consist of two junior faculty members, three postdoctoral fellows, one graduate student, and two research assists. Dr. Gilson also has twenty years experience participating in clinical trials – specifically idiopathic pulmonary fibrosis. “Holy shit. Ewa come see this.” Larry shouted out almost scaring Ewa who was in the next room. I can’t believe I was referred to this guy. Look at his credentials.”
~ 80 ~ “What is it? Are you okay? Ewa sensing urgency in Larry’s voice.
“I’m okay. Just come and look at this.”
Ewa hastily walked over to where Larry was sitting with the computer and looked over his shoulder to read Dr. Gilson’s profile. “That’s amazing. It looks like Dr. Dowd referred you to one of the best.”
“Look at this. He also has awards for being one of America’s Top Doctors going back to 2002. But the best part is that IPF is his thing. He not just a general pulmonologist. I mean how many doctors specialize in one disease?”
“You’ll certainly have to call Dr. Dowd and thank her for the referral.”
“I will.”
A few days later Larry and Ewa were sitting in the waiting room precisely on time waiting to see Dr. Gilson. A half hour went by - nothing. An hour went by - nothing. “Excuse me, I had an appointment with Dr. Gilson at 2:30 - am I getting close to being seen?” Larry asked the receptionist.
“Yes, Dr. Gilson is running a bit behind but it shouldn’t be long now,” replied the young lady behind the counter.
Larry went back to his chair and continued fiddling with his tablet to pass the time away. Another forty-five minutes went by and suddenly, “Mr. Martel,” cried out a voice from across the room.
~ 81 ~ “Here”
Larry, with Ewa next to him, was led into a waiting room. The usual vitals – weight, blood pressure, temperature, etc., were taken and the nurse said Dr. Gilson would be right in. Well sort of - another fifteen minutes transpired before he made his appearance.
“I am very sorry for making you wait so long but things have been really hectic today.” Said Dr. Gilson as he entered the room.
Larry was already feeling good about Dr. Gilson. He has always felt that it really doesn’t matter if someone makes you wait – they just have to acknowledge it – it just courtesy and it validates the patient as a person. This was a good start.
“What can I do for you.”
Larry explained the situation with Dr. Ipwich, the pills from Canada, and Dr. Dowd’s referral because she felt uncomfortable treating him for a disease she knew little about.
Dr. Gilson was soft spoken and answered in detail all of Larry’s questions.
“Can you tell me about pirfenidone? How good is it.” Do you have any issue with getting drugs from Canada?” the questions were endless.
Dr. Gilson explained that he certainly had no problems with Canadian drugs. However, “our knowledge of pirfenidone is very limited,” he explained. “We don’t know if the dosage is optimal, or if the drug really works. The disease progresses at varying rates
~ 82 ~ therefore if there is a slowing of this progression is it because of the pirfenidone or would it have slowed anyway? The trials were stopped in phase 3 because it looked promising and it was the only thing out there that had possibilities. But there are so many unanswered questions. We don’t even know the mechanism by which the drug effects the disease.”
“What about food? I’ve been trying to find out what and how much to eat before taking the pills. If I take too much food, I may be affecting the rate of absorption to the point of not getting enough of the drug in my system. Does carbs, protein, or fats affect the optimal use of this drug?”
“It’s hard to say. Not knowing the mechanism of how it works gives us little insight in how foods affect its potency. Even the dosage is not etched in stone. The dosage you are taking is the maximum safe level that was determined by the trials. We don’t know if taking less would be just as effective.”
Dr. Gilson was not hurried and took all the time in the world attempting to answer Larry’s questions. But for the most part there were no hard cold answers. At the end of the visit Larry discouragingly thought, “How little is really known about IPF”. Doctors still don’t know what triggers it or the process that keeps it going.
“What about a lung transplant? Have you thought about it?” asked Dr. Gilson.
“I’m not interested. The survival rate is small, there’s endless testing, and then there is the pain and suffering that goes along with the procedure. I’ve heard it described as exchanging one disease for another. I just want to live my life as natural as can be. That is
~ 83 ~ why I never got tested for four years prior to the approval of pirfenidone. I don’t want to spend my remaining days in and out of hospitals.”
“You’d be a good candidate. But many people feel as you do. I just thought I would ask.”
Larry asked about trials but was told by Dr. Gilson that he had no open trials available at the moment. He did say that he would keep Larry in mind as new trials might be opening later in the fall. “I’m not interested in phase 1 or 2 – it has to be phase 3 or 4,” commented Larry.
“Do I really need to see you on an ongoing basis? Dr. Dowd said she didn’t want to treat me because of her lack of medical knowledge on this disease. But it doesn’t sound like there’s much you can do either. I mean, I don’t want to get into the system with endless tests, I just want to live my life,”
“No you can see me whenever you like and Dr. Dowd can keep an eye on you just as well. I’ll let her know. If anything comes up, I would be glad to consult with Dr. Dowd or for that matter we have the patient portal and you can write to me with questions or concerns – I will be glad to help you personally.”
“I’ll set up an appointment in six months. I do want to stay in touch if that’s okay?”
“Yes, six months would be fine. You can do that on your way out,” said Dr. Gilson
As they were walking out of the examining room Larry and Ewa noticed most of the lights had been turned off in the offices, and reception areas, and there was
~ 84 ~ nobody present on the floor. “What time is it?” Ewa asked Larry.
“It’s a little after 6:00. We were in there for over one hour and forty-five minutes. That’s almost exactly the amount of time we had to wait in the waiting room. I can see why he gets behind. I guess I will have to wait until tomorrow and call to make my next appointment. There’s nobody at the appointment desk.”
“Well he certainly didn’t rush us. I like him a lot. I guess we were lucky.”
On two different occasions shortly after his visit, Larry had questions for Dr. Gilson and wrote to him via the patient portal. Sure enough, as promised, Dr. Gilson responded within a couple of days. “He answered both times - personally,” he told Ewa. “Not through his nurse like most doctors do. I really like this guy.”
The pirfenidone was going well - sort of. Larry noticed that his food taste were changing and he was losing his appetite. Nothing appealed to him anymore. Even when he was hungry, he would feel full after only a few bites. Foods that he used to crave were no longer on his menu and one particular pleasure completely disappeared. For thirty-five years Larry would have a glass of whiskey each night at 5:00 o’clock – actually CC and ginger ale. He never missed a night – just the one drink but he so enjoyed it. Much to his chagrin, he no longer enjoyed the taste of whiskey and stopped having his afternoon delight. “Aren’t you having you’re 5:00 o’clock drink anymore?” Ewa asked one afternoon.
“No, I stopped a couple of days ago. I used to really enjoy the taste of whiskey but now it’s almost tasteless. There’s no point in drinking for the sake of drinking.”
~ 85 ~ Meals became a long drawn scene each day. Ewa would asked Larry what he wanted to eat and he had to mentally list all the different types of food in his mind to see what would be the most palatable and tolerable – something he thought he could get down without upsetting his stomach. This was particularly difficult for Ewa. She knew the difficulties Larry was going through and bought dozens of varieties of food to help him eat nutritionally. However, he often would tell her that there was nothing in the house to eat. “I don’t know what to do anymore? Whatever I offer you, you don’t like,” lamented Ewa.
“I’m sorry honey but I’m trying. It’s just sometimes nothing seems appealing to me – especially when I’m not hungry to begin with.”
“But you have to eat. You’ve got to maintain your strength.”
He reached a point where he could eat an English muffin in the morning and go most of the day without anything else. However, on the plus side, he was overweight by some twenty - twenty-five pounds, so he wasn’t concerned about losing a few pounds. Larry also noticed that for some reason water had become a very real problem. Even though nausea was no longer plaguing him in general, drinking water - specifically plain water - did make him feel nauseous. This was causing him to have difficulty keeping hydrated. Larry eventually found out that if he flavored the water with tea or juice it helped curbed the nausea tremendously. Overall, none of these issues discouraged Larry and he kept a positive attitude towards pirfenidone. He felt he just had to endure these minor setbacks for the time being, and that eventually his body would get used to the pirfenidone and his appetite would return.
~ 86 ~ At the end of May, Larry drove home from the condo at Hampton Beach to mow his lawn. A beautiful day, not particularly hot, but the sun shone brilliantly in the cloudless sky. The tractor made quick work of the mowing and Larry finished the entire lot in about an hour. It was soon thereafter that he learned that the pirfenidone warning label regarding sun exposure was there for a reason. Although bright, the May sun was not particularly strong, yet he could feel his face and arms glowing with warmth as he was returning to the condo. “Ewa does my face look red to you?” Larry said as he entered the condo.
“Yes, I think you got burnt today. You should have applied the 50 spf and used a hat like they recommend. You shouldn’t put yourself in jeopardy like that. What if you have to reduce your medication or stop it all together waiting for your skin to heal? That wouldn’t be good.”
Ewa was right and Larry knew it. As it turns out the sunburn was fairly mild and not very painful. He did not have to change or stop taking pirfenidone as no other skin rashes develop from this exposure. But Larry learned an important lesson – either stay out of the sun or use sunscreen and protective clothing.
The May/June time period at the condo was very difficult. Larry began coughing off and on which was consistent with the symptoms of IPF. It would come in spurts – a cough here and there. However, there were times that the cough persisted and was quite brutal for several minutes. Overall, he felt fortunate as the coughing didn’t appear to be as severe as some IPF sufferers from the forums had posted. “Do you have a cold?” Ewa would asked occasionally.
~ 87 ~ “No,” Larry would reply. “It’s just the disease manifesting itself.”
Larry brought the portable oxygen concentrator with him on this stay at the condo. His purpose was to use it for some aerobic exercising. However, he was still ego bound and became conscious of the hose stuck up his nose. He couldn’t bring himself to use the concentrator more than a couple of times. Instead, he tried slowing down his routine but was unsuccessful as his oxygen numbers still fell to unsafe levels.
“Why don’t you go out for a walk,” implored Ewa. “Anything is better than nothing. Just go a few blocks at a slow speed – I’ll go with you.”
They did walk a few times – not much, maybe a half mile back and forth, but it was so difficult. His oximeter told him his oxygen levels were in the mid eighties -- he would come back exhausted.
“This isn’t working out,” he told Ewa.
“Stop being so stubborn and use your portable machine. It will help you.”
Larry gave in and used the portable concentrator at a setting of 6. Being a pulse machine the settings do not equate to the numbers of a constant flow machine. A pulse machine sends a puff of oxygen – called a bolus – when it perceives negative air pressure i.e., when the patient inhales. The theory behind a pulse oxygen concentrator is that a patient is not using all the oxygen a constant flow machine produces, because when the patient exhales the oxygen going into his nose is wasted. The pulse eliminates that waste by providing oxygen only on the inhalation portion of breathing.
~ 88 ~ Unfortunately, portable pulse machines are not as strong as constant flow machines. As an example, Larry’s machine delivers 180 ml/min per number setting – so a setting of 6 produces a little over 1 lpm equivalent to a constant flow. Plus 180 ml/min per setting is the most it can deliver – breathing faster will not produce more oxygen. Whereas, a constant flow oxygen concentrator allows a patient to breath as fast as needed in order to receive as much oxygen as he requires at the moment. Larry found that his pulse concentrator – one of the higher generating pulse machines on the market was just barely providing the oxygen he needed to stay above ninety percent. “Look at my oximeter,” Larry said to Ewa. “We just bought this machine a couple of months ago and it can hardly keep me above ninety percent. We’re certainly not creating any speed records in our walks. I wonder how long it will be before it’s of no use to me. Then what? I won’t be lugging tanks around.”
“Do you have it all the way up?” asked Ewa.
“Yes, it’s at 6. I’m thinking a thirty-five hundred dollar mistake.”
“Did you have any other choices besides the tanks?”
“No, the pulse concentrator I purchased was one of the strongest I could find on the market.”
“So just use it for now and see what happens.”
Larry’s daughter broke the news to him that she was getting married in the fall. It seems that her boyfriend - now fiancé - had pop the question during one of their vacations. She was very happy and excited yet there was this air of apprehension. Karen has six children
~ 89 ~ from her previous marriage and thoughts of acceptance – both from him to the children and vice-versa were weighing on her mind. “That’s wonderful, I like Joe a lot and from what I can see when you visit us, he loves the children and is very good to them. I know he certainly loves you and that’s what is most important. If a man loves a woman unconditionally all else will follow.”
“He’s kind of old fashion and tried calling you several times to ask for your blessing. He said he couldn’t reach you. I told him I would try - I didn’t want someone else telling you - I wanted to make sure you heard it from one of us,” replied Karen.
“Really, nobody does that today. Cell reception is terrible here it’s no wonder he couldn’t reach me.”
“How are you feeling dad?”
“I’m doing fine.”
“You always tell me your doing good. You promise you’re doing good.”
“Yes, stop worrying. I’m okay - really.”
“Good. Joe and I want you to marry us. Are you still a Justice of the Peace?”
“No, I gave up my appointment several years ago.”
“I thought so. Can you get it back? I really want you to do this.”
“Probably not. Besides my JP appointment wouldn’t allow me to officiate in New York. If it’s that important to you, I will look into how to make this happen. Maybe
~ 90 ~ they have a onetime license to marry – not sure just thinking out loud. I will look into it and get back to you. Love you and say Hi to Joe and the kids for us.”
Ewa was sitting on the sofa during the entire conversation and after hearing bits and pieces was curious about what was going on.
“So what’s new with Karen?” asked Ewa.
“Karen and Joe are getting married and she asked me to perform the ceremony.”
“That’s wonderful. When?”
“In the fall - I’m sure she will call you and tell you all about the girl things,” Larry said with a big grin on his face. “I need to research how I can get authority to perform the ceremony in New York.”
Larry had mixed feelings about officiating at his daughter’s wedding. He was honored beyond words that she would want him to do this for her. It was something he never expected. But he was also thinking ‘as a dad’, who just wanted to go to his daughter’s wedding as ‘her father’. If he officiated, he wouldn’t be giving her away - but he gave her away at her first wedding so he didn’t think that issue should tilt the scale. Then he thought about his IPF, “Will I be physically able to perform the ceremony. I don’t want to cough all through the ceremony. I certainly - under no circumstances – want to have a tube up my nose – that would distract from the couple getting married. I would just the soon not do it then have that happen,” Larry thought.
~ 91 ~ He found out that it would be next to impossible to get a onetime license to officiate a wedding in the state of New York. However, he did come across an organization that ordained ministers. All one had to do was apply and wait for approval. It was called Universal Life Church and was located in California. Apparently the church believes that a person’s calling should come from one’s inner self and that neither the church nor any government entity should stand in the way of that calling. Larry soon discovered that it was not just an internet organization calling itself a church – it actually has a building in Modesto California and performs religious ceremonies each Sunday. Upon further research, he found out how our Constitution protects the rights of religious freedom in the US. Through those laws Larry discovered that the government has no right to dictate how a church ordains its ministers. Universal Life Church (ULC) decided years ago that they would receive applications through the internet, and upon review of the applicant, ordain them as a minister of their church – for free. There are absolutely no charges. This policy has been tested in the courts several times with the outcome always siding with the rights of the church. Subsequently any person ordained by the ULC directors shall have the right to officiate a marriage ceremony in all states of the Union. “I think I have found something here,” Larry thought. I will look up the laws of New York to see if it concurs with what I’ve just read. Maybe I will call the town clerk to see if I have to register as a visiting minister.” So Larry filled out the application and waited several days. He received an email about a week later saying that he had been approved and would be receiving his ministerial credential in the mail in about a week or so. “I guess I will be officiating the wedding after all. I better start putting a ceremony together.”
~ 92 ~ Chapter 11 – Walking a Careful Line
“It was good to be home from the condo,” Larry thought as he was entering the front door to his house. The physical limitations that were placed on him through his disease made what was the most enjoyable time of the year turn into a slow day to day journey. The excitement of the daily walks on the beach, or to the boardwalk and arcades, the bike rides to get ice cream or the twice weekly day trips to various parks, scenic areas, or local restaurants became almost nonexistent. He was starting to feel trapped in the one room condo. Sure the ocean was beautiful as ever, and the sunrise was so spectacular each morning. But the condo was just so confining. At least, when he was home, he could enjoy his property and the beautiful flower gardens Ewa had planted. He could walk from room to room and look out at the turkeys as they paid their daily visit or curse the woodchuck that was chewing on the flowers in the back garden. “I even miss the skunk that’s residing under our house. It’s just nice to be home again.” While they were at the condo Larry continually encouraged Ewa to get out of the room and enjoy herself. “Go for a bike ride or a walk. Don’t just stay here with me.”
“But we used to do everything together - I feel so guilty,” Ewa would reply.
“Don’t be. I’m happy when I see you enjoying yourself. Really - go - it’s okay. You need to get away from me once in a while.”
So Ewa would reluctantly go for her bike rides and come back telling Larry how much she felt alone on her
~ 93 ~ rides. “As much as I enjoy riding my bike, it’s just not the same,” she would say each time she came back.
“Why don’t you take a day a week and get away - leave in the morning - and take the whole day for yourself. Go shopping, spend money, stop by one of the other beaches, anything you want. It’ll do you good.”
As much as Ewa was feeling guilty about leaving Larry alone, Larry was feeling the same about Ewa being stuck with him. It wasn’t fair for her to be confined day in and day out. She eventually agreed that she needed alone time and Larry felt good that she would have her time to herself.
About midsummer he started feeling another side effect from the pirfenidone – stomachs aches – actually stomach pain. It happened each day about 1:00 o’clock – a general dull ache that went from his lower rib cage, side to side across his body, and traversed just past his belly button. It didn’t matter whether he had eaten or not eaten. If he ate he found that the quantity of food or the type of food had no effect - the pain was there constant and unrelenting. It usually lasted until late afternoon or early evening and quietly disappeared only to appear again the next day. “It’s almost 2:30. What can I get you for lunch?” Ewa called out.
“Nothing right now, I’m not hungry but thanks anyway.”
“Is your stomach hurting again? You’ve got to eat something before taking your pills. You can’t take them on an empty stomach.”
“Do we have any donuts left? I’ll have half of one if we do.”
~ 94 ~ “That’s not good.”
“It’s the best I can do right now.”
Larry did much research on natural medicine that might help with the IPF. Rather than relying on anecdotal information which are prevalent with scam sites, he looked for studies and trials that had some scientific value – he found that many of these studies were conducted by scientist and doctors in Europe and Asia. N-acytycysteine (NAC) looked to be very promising and was in a phase 3 study with varying results. Larry text Dr. Gilson to see what he thought of the medication and its value in helping patients with IPF. Dr. Gilson did not comment either way on the value of NAC, but did tell Larry he had no objections if he wanted to pursue it for himself. So Larry went ahead and started taking the medication in early May, but decided to stop it in mid August as he saw no changes in his condition one way or another.
Next on the list of medications that looked promising was cordycepts (fungus/mushroom), astaxanthin (carotenoid found in shrimp, lobster, crab, and salmon, and curcumin (substance found in turmeric). All had been observed in studies and trials. Unfortunately, Larry was disappointed with these as well. “Are you still taking the three special medications that you researched several months ago,” Ewa asked as Larry was taking his morning medications.
“Yes, but I’m near the end of the bottles. I was hoping that a combination of the three medications would help me, but I don’t see that they’ve produced any results. I probably will not continue.”
So that was the end of Larry’s experimental drugs.
~ 95 ~ The summer of 2016 flew by as usual bringing Larry and Ewa back to the condo in early September. Larry’s capacity to do things, even simple things, was continuing to diminish due to his continuing decline in breathing capacity. He occupied much of his time writing the ceremony for his daughter’s wedding which was coming up in about four weeks.
About ten days before his daughter’s wedding Larry started to cough like never before. The coughing must have irritated his vocal cords as he now coughed when he would talk in a voice any louder than normal conversation. This became of great concern as it impeded Larry from practicing the words to the ceremony in a loud voice to simulate performing before an audience. He had previously gone through periods whereas coughing became a real nuisance. It would usually last a week or two - but this time it was real bad. “How in hell am I going to perform this ceremony if I can’t speak loud enough for people to hear me?” Larry said angrily to himself. In the back of his mind he also knew that coughing may be a sign of disease progression. In the past, these coughing periods precipitated a downward turn in his disease. “This can’t be good. I wonder how long this coughing will last this time? I’ll call Karen and make sure she gets a sound system in place so I don’t have to strain my voice and start coughing during the ceremony.”
Upon arriving at the wedding, Larry walked slowly from the car to where the wedding was taking place so he wouldn’t become hypoxic (low blood oxygen). “Be careful,” said a concerned Ewa. “Are you feeling okay?’
“Yes, I’m fine,” Larry replied.
“But you look like you’re out of breath.”
~ 96 ~ “Doesn’t matter - I will not have a hose in my nose for this wedding.”
“You should have at least packed the portable oxygen machine in case you need it,” Ewa exclaimed. “What are your numbers?”
“Why? I wouldn’t use it. I didn’t bring the oximeter either so I have no idea what my numbers are.”
This was his daughter’s wedding and he wasn’t going to remind her throughout the events just how sick he had become. He wanted Karen to just focus on ‘her’ day.
The outdoor wedding, which took place in the late afternoon, was a beautiful event and went off without a hitch. Earlier in the week Larry surmised that the coughing was mostly due to congestion - clearing his lungs might reduce the coughing spells. He decided that he would take Mucinex to loosen the mucus along with some coughs drops to soothe his irritated throat. It was a successful strategy as he didn’t cough once during the entire ceremony.
After the ceremony, the guests were treated to a boat ride along the Erie Canal and later a beautiful reception was enjoyed by all. As with all happy occasions it passed by very quickly. The only hitch that occurred was when his seven year old granddaughter ran up and asked him to dance with her - a fast one. Larry tried to limit the dancing to a lot of arm swinging but even so, he could feel his oxygen numbers plummet and he became a little dizzy. “You shouldn’t push yourself like that,” Ewa said after she saw him out on the dance floor.
~ 97 ~ “It’s our granddaughter. What do you want me to do? Tell her that her grandfather doesn’t want to dance with her. She would never understand and I would end up hurting her feelings. Besides I’m fine now so don’t worry. It’s getting late anyway. I think we should leave as we have a long ride ahead of us tomorrow.”
Larry was scheduled for a follow up visit with Dr. Gilson a week after the wedding. The waiting room scenario played out as before – one and a half hours wait – one and a half hours actually visiting with him. Larry felt if Dr. Gilson gave all his patients whatever time they needed then it was no big deal for him to wait his turn. He was confident that Dr. Gilson would not rush him, and would in turn give him the time he needed to answer all his questions. The visit itself was rather routine – lots of questions but nothing specific to add to the equation. His PFT’s were declining, but Larry didn’t need any tests to tell him this as his daily life experiences easily confirmed the results – FVC (volume) was fifty-five percent, which is like having one lung, and his DLCO (ability of lung to absorb oxygen) was forty-three percent, which is in the range of moderate loss. Larry’s main concern was the DLCO test. “It doesn’t matter how much capacity you have - if you don’t absorb the oxygen, you’re not going to survive for long,” Larry thought. He asked Dr. Gilson about trials but there were still none available that he qualified for at the moment. He would return in March of 2017 for another follow up with Dr. Gilson.
Larry was not exercising during this fall stay at the condo and Ewa was able to get Larry out only a couple of times for walks. The portable oxygen concentrator was no longer sufficient to keep up with his oxygen needs. This was the start of his complete and total deconditioning of his body. “You’ve got to keep
~ 98 ~ moving,” Ewa would implore. “You can’t just sit around all day. You’ve been an athlete all your life you know what it’s doing to your body.”
“Yes, I know. But what good is it doing me when my saturation levels drop into the low to mid eighties. I’ll see what happens when we get home from the beach.” But Larry knew he was just putting her off as his mind was telling him to stay in the comfort zone.
About a week after returning home from the beach Larry and Ewa received a letter from Dr. Dowd. She was leaving her practice for parts unknown. “Great look at this Ewa. Dr. Dowd is leaving her practice. We’ll have to find another PCP.”
“WHAT!” exclaimed Ewa in disbelief.
“Yes, Dr. Dowd is leaving. After 10 years, I can’t believe we have to start all over with someone new. Do you remember the last time we were there? I asked her if she had any plans on retiring?”
”I remember – she said no.”
“Well she didn’t lie. She still practicing but not with the public – she’s giving up her private practice to become a physician in an institution. Here’s a list of physicians from the clinic that are taking on new patients. I’ll start researching tomorrow to see if there is anyone we want to sign up with.”
The remainder of the fall months were non eventful for Larry. He was still able to ambulate around the house slowly without having to use oxygen. However, the basement was now a definite no no. However, there were times Larry would sneak down to the basement –
~ 99 ~ all his man stuff (tools, manuals, glues, tapes, etc.) were down there. Of course, Ewa would catch him every time. “What are you doing down there?” she would yell out.
“It’s okay. I’ll take the stairs very slowly – one or two steps then rest for a bit.”
One time Ewa found an old pocket knife amongst Larry’s things on his tool bench. “Look what I found?” she exclaimed to Larry as she was coming up the basement stairs. “It’s beautiful. How old is it?”
“I completely forgot about that knife. I used to have it in my tool box in my younger days.”
“Look how well it was made. It must be very old. A little polishing and cleaning and it will be like new. Can I have it?”
“Of course. It’s yours.”
A few days later Ewa went out to do some errands. “Now’s the time to tackle that old knife,” Larry thought. So he went down into the basement and cleaned and polished the knife with his bench polisher and then by hand. A couple of drops of light oil and presto a knife to behold. When Ewa came home Larry said, “I have a surprise for you.”
“What is it?” Ewa asked in anticipation.
“Promise you’ll bite your tongue if I show you,” Larry said with a big grin on his face.
“What is it?”
~ 100 ~ “Promise me first.”
“Okay.” Ewa said reluctantly.
Larry reached into his pocket and pulled out the knife. Ewa’s face immediately lit up. “It’s beautiful, I love it. How did you do it?”
“Don’t ask.”
“You went downstairs again didn’t you?”
“Easy, you promised.” Larry responded with a big grin on his face.
So Ewa held back from scolding him and Larry was happy that he was able to do something that pleased his wife. All in all he felt it was a pretty good deal.
Larry finished researching the list of physicians that was sent by Dr. Dowd’s office to replace her. “There’s not very much on this list. Most of them are new to the clinic. The ones we’ve seen in the past as fill ins, when Dr. Dowd was not available, are not taking on new patients. Here’s the profiles I printed up for you to review.” Larry said as they were sitting down for lunch. “I guess I would go with doctor Roker. He’s only been a physician for a few years but he looks promising and has a good background.”
Ewa perused the papers for several minutes. “Yes, go ahead and call the clinic for both of us. We can see what he’s like during our first visit.”
Dr. Roker was a mild mannered person with curly hair and well proportioned body. He greeted them almost shyly but was very friendly. It was Larry’s appointment
~ 101 ~ for a blood pressure follow-up and as usual Ewa was there for support as well as assessing this new doctor.
“I see your oxygen level was ninety-four percent when the nurse took your vitals,” said Dr. Roker looking at his paperwork. “Is this normally what you have?”
“Yeah, pretty much without supplemental oxygen. As you can see I have IPF.”
“Do you exercise? Even with lung disease that is important.”
“Yes, off and on.” Larry stretching the truth a bit. “Even with oxygen I have a hard time getting my pulse up above a hundred and five.”
“You need to get your heart pumping for maximum benefit.”
For some reason that comment hit Larry the wrong way. “I don’t need a lecture. Fitness has been my gig for thirty-five years. I was a certified fitness trainer so I know quite a bit about what I should be doing.”
Dr. Roker look stunned at Larry’s response but decided to move on.
“I see you have your liver checked every few months because you are taking pirfenidone. We’ll need to follow up on that.”
“My prescription for pirfenidone runs out in January. Will you write another script for the pirfenidone? Dr. Dowd took care of that for me.”
~ 102 ~ “I don’t know anything about this drug. That prescription should be written by your pulmonologist.”
“But Dr. Dowd had no qualms filling it for me.”
“Dr. Dowd did some things that some of us wouldn’t do. I really don’t have time to research it to be comfortable enough to write the prescription.”
And so Larry’s visit with Dr. Roker got off to a rough start. “What do you think?” Larry asked Ewa as they were walking to the car.
“I’m not sure. You were kind of rough on him. He probably thought you were some sixty-nine year old man who knew nothing about heath and exercise.”
“Yes, I know. I shouldn’t have bit his head off like that. It’s obvious he’s only going to act as a gatekeeper which is what most PCP’s do today. He won’t get involve with a particular issues like Dr. Dowd used to do but send you to a specialist. You have an appointment next week. Why don’t we wait and see how things go with you? If he doesn’t work out we can always look around some more. Let’s just see what happens?”
The stomach aches are now a daily occurrence. There was no nausea associated with it just this strong dull pain across his abdomen. Larry was hoping that just as the nausea had passed over time so would this side effect of the pirfenidone subside. He was fairly sedentary at this point, so even though he was eating less and less his weight hadn’t fluctuated at all. Ewa would implore him each day to eat enough to keep up his health but this met with a lot of resistance. “You know you have to eat something. You can’t keep taking these pills and not get something in your stomach.”
~ 103 ~ “I do eat. Maybe not much, but I am eating. Besides I haven’t loss any weight and I have a good twenty-five pounds to lose so I’m not worried.”
“How have you been sleeping?”
“Not that great – you know toss and turn. But it’s not because of the disease I’m just restless.”
“Do you think about your disease? Is that keeping you awake?”
“Well, I do think about it - it’s hard not to think about it - but that’s not what’s keeping me awake. You know a few night ago I did sleep almost the entire night undisturbed - a once in a blue moon event. It was such a deep sleep. When I woke up and looked at the time I realized how deep I must have been as this hardly ever happens. The first thought that came into my mind was, ‘This isn’t so bad’. If I had died it just would have been a forever sleep. I never would have known any different.”
“Don’t scare me. You’re not going to die. What would I do without you?”
“You know about once or twice a month I have this recurring dream. I’m running – sometimes up a hill, sometimes up a mountain, sometimes just endless flights of stairs. While I’m running I’m thinking to myself, ‘This is really good and it’s not that difficult. I have plenty of oxygen and I’m not out of breath. Why am I worried about my health all the time?’ Then I awake and face the reality that I was only dreaming and a deep sadness covers me like a shroud. How can my mind be so cruel to me?”
~ 104 ~ “I’m so sorry honey. I wish there was something I could do to make you better.”
“Thank you. I just wanted to share that with you.”
Christmas was just around the corner and Larry and Ewa knew that going to the tree farm to pick and cut a nine foot tree was not going to happen this year. Larry would not have the stamina to trounce around the fields to find the perfect tree, cut it down, load it onto the car, trim it to fit into the tree holder and carry it into the sunroom. “What if we get a table top tree this year - say a four or five footer? We can still go to the tree farm. It would be easier to pick out, cut, and bring home,” Larry asked Ewa
“Yes, I’m okay with that. It won’t be as big as we’re used to but we can still make it beautiful.”
Larry was able to plod along through the winter months without much perceived change either to his health or to his daily life. There were fifteen plowable storms that required the driveway and path around the house to be cleared of snow that winter. A task that would have been impossible for Larry to accomplish on his own. Tom, the neighbor across the street, whom Larry had planned on asking after selling the snow blower, did agree to clear the snow for Larry and Ewa. It was such a mental and physical relief for Larry to not have to think about snow removal. Tom was exceptionally dependable and was right there immediately after every storm. “Tom did such a great job this winter,” Larry said to Ewa. “I hope he will do it next year as well.”
The appointment with Dr. Gilson was coming up at the end of March and he was hoping that his disease had stabilized enough to reflect that on his PFT tests. In
~ 105 ~ addition to the PFT’s, Dr. Gilson also ordered an echocardiogram, a non invasive test in which a technician uses a probe to scan the heart with ultrasound waves producing images for diagnostic purposes. IPF is a restrictive disease that causes the lungs to be less pliable, and along with the scarring, decreases the lung’s ability to absorb and convert air into oxygen. This in turn can cause the heart to work much harder to push the blood through the lungs in order to get enough oxygen throughout the body. The end result is an enlarged heart that becomes less efficient possibly to the point of heart failure and increased pressure in the pulmonary arteries (pulmonary hypertension).
The visit to Dr. Gilson followed pretty much the same pattern. He was late as usual, apologetic, and gave Larry as much time as he needed.
“The results of your PFT’s show a slight decline in both your FVC and DLCO,” Dr. Gilson said after taking a moment to review the tests on his computer.
“What is a slight decline?” questioned Larry.
“Your FVC is fifty percent and your DLCO is thirty-eight percent. Both are down about five percent.”
Larry knew that anything below forty percent on most DLCO charts was considered severe loss.
“Your echocardiogram is pretty much like it was a year ago so that’s good news. Just make sure to keep your oxygen saturation up with no long term levels below eighty-eight.”
~ 106 ~ “I’ve been feeling quite a bit of reflux lately. Not so much when I’m in bed but during the day.”
“Reflux is dangerous for IPF patients because the acids can be aspirated into the lungs causing the IPF to get worse,” said Dr. Gilson
“Yes, I know. I’ve also read that reflux can be a side effect of the pirfenidone.”
“Try putting a block of wood – like a two by four – under the feet of the headboard. This will raise the bed and should help. I am also going to prescribe Omeprazole to help minimize your acid production.”
“We’ve never done a sleep study of you have we?”
“No. But I do have a new oximeter that records data that is downloadable to a computer. I did my own sleep study a few weeks back and I recorded an oxygen level above ninety-five percent for ninety-eight point nine percent of the night. It showed I had three dips in my saturation level. One when I got into bed, one when I got up to go to the bathroom, and one when I got out of bed – during those three events I slipped down to eighty-seven to eighty-eight percent. My recovery according to the data was less than a minute. I’ll do a study a week or so before I come in for my next visit and bring in the data.”
Larry asked Dr. Gilson if he could write a new prescription for his oxygen requirements. The original prescription called out for a concentrator capable of putting out a constant 5 lpm of oxygen. He explained he wanted to start exercising again and 5 lpm was insufficient to meet his oxygen requirements. “I want to do some aerobics and probably need a machine
~ 107 ~ capable of going up to 10 lpm continuous flow,” he explained.
Dr. Gilson said he would schedule a walk test to ascertain his exercise requirements of oxygen and submit them to Medicare for approval. “When you get the machine just turn it up to where you have sufficient oxygen saturation. You can’t really get too much oxygen.”
The rest of the visit was routine. Larry found out that no new trials were available to him. His next scheduled appointment would be in September.
“I think it’s time we tell the children,” Larry said as they were eating breakfast one bright warm late April day.
“Karen already knows and I’ve already mentioned it to Peter. It’s just Wayne that needs to be told.”
Peter is Ewa’s son from a previous marriage who lives in New York City.
“Honey, I asked you not to tell the kids,” said Larry
“Well Peter was asking about you and I just told him a few things.”
“Like what?”
“That you had some lung issues - I didn’t really expand on it. I just felt I should say something to him. He is family.”
“I know he’s family but I just wanted to tell people on my own terms.”
~ 108 ~ Wayne and Karen are both Larry’s children from his first marriage. Wayne owns a metal fabrication business located about eight miles from Larry’s home. Larry decided to visit him there so he could talk to him about the situation with his disease.
“Hi Wayne. Do you have a minute for us to talk?”
“Sure.”
“About five years ago, I developed a disease called IPF. The disease ...... ”
Wayne is a man of few words. He listened intently at what his dad was telling him but had few comments or questions. Larry was particularly sensitive to the fact that he hadn’t told Wayne until now - five years later and this needed to be addressed.
“I didn’t want to cause you any undue worry or stress,” Larry explained to Wayne. “Karen knew about it because I had asked her to have one of the doctors at her hospital give me a second opinion on the results of my first CT scan. I just didn’t feel it would serve any purpose to worry you - especially since I wasn’t even sure I had this disease until a year ago. Plus, as long as I took things slow, I was feeling pretty good until recently. That’s why I decided it was time to share. I’m sorry I left you out, but like I said, ‘I didn’t feel it would have served any purpose’.”
Wayne said he understood and accepted his dad’s explanation.
“Ewa and I are thinking of selling the house. It’s just getting to be too much – I just can’t handle everything that needs to be done and it’s not fair to Ewa. She is
~ 109 ~ getting stuck with everything. We’re thinking of going with a condo. No work just close and lock the door when you leave and that’s it.”
“You don’t have to do that,” Wayne quickly answered. “Laura and I can come over when you have a list of things to do and we can help with whatever you need.”
“Really? Thank you so much for your offer. I will talk to Ewa about it. You know I really appreciate that you would do that for us. I’m happy but not surprised – you’ve always been there for me when I needed help.”
After Larry returned home, he spoke to Ewa about what had happened at Wayne’s place along with the offer of help. They decided that they would take Wayne up on his offer. “This is such a beautiful home,” Ewa said. “I don’t want to leave it.”
“I’ve never lived in anything but a single family residence.” Larry responded. “I can’t imagine anything but green grass and flowers around me.” Larry was thinking about the condo he and Ewa had visited with a realtor this past week. It was nice enough inside, but when he looked out the living room window to the parking lot below, he knew that this was not for him - asphalt and parked cars - UGH! But if he had to he would adjust to this life style, as he has done to so many other things over the last few years. But thanks to his son, he knew he would be staying in his home for now.
~ 110 ~ Chapter 12 – The Lost Daughter
“We haven’t talked about Tracy,” Ewa commented at breakfast the following day.
Tracy is Larry’s step-daughter - Kris’s daughter - who was raised by Larry as his own from the time she was three years old. In fact, two months after they became a family, Tracy whispered into her mother’s ear. Kris turned to Larry and said, “Tracy wants to know if it’s okay to call you dad?” Tracy’s biological dad was still in the picture and pick her up occasionally for visitation. This request was totally unexpected and Larry was overwhelmed with happiness knowing that this little girl thought so much of him to want to call him ‘dad’. They were very close through the years and Tracy knew that she could count on Larry when anything went wrong with her life. She knew he would always be there for her.
“What about Tracy?” Larry said in a surprised voice.
“Are you going to tell her about your illness?”
“I thought you said you had mentioned my illness to her a couple of years ago?”
“Yes, I ran into her one day and told her that you had serious lung issues. Her response was that everyone gets sick eventually. I can’t believe she’s treated you this way after all you did for her in raising her. It’s because of you that she’s had the successful life that she’s had. I remember many times you wouldn’t even let people call her your step-daughter. I heard you
~ 111 ~ correct them by telling them that you consider Tracy to be your daughter.”
“She’s the greatest disappointment of my life. We used to be so close even after her mom passed away. Because she lived with Kris and I, she got more of me than even my own biological children, who I used to see two or three times a week - she basically got one hundred percent of my time. To be rejected like this really hurts. I remember about a week after her mom passed away, she was with Mark (her future husband) and I and she started crying. ‘I’m not sure I want to get married right now’, she blurted out, ‘I still need my dad’. Then she gave me a big hug and held on so tight. I can remember it vividly – it was right in front of the house. Now she’s not even talking to me.”
“Well, I see on Facebook that she talks a lot about what her mother and she did. She never mentions that you were in the picture,” said Ewa.
“Yes, I have seen some of those Facebook comments. Most are just imaginings of hers. She hardly ever did anything with her mom because she was always too busy with her friends to bother. Did you see the post about the 4th of July outing with her mom? And how her mom rode all the side streets to avoid traffic, and how they brought blankets and picnicked at the park before the fireworks? That’s all fabrication. That never happened. First of all, there were many 4th of July outings and we always did them as a family – always. It was like you and I are today – if you saw Kris you saw me. She’s just trying to get the ‘poor girl’ sympathy from all her followers on Facebook. Do you remember I responded to that post by writing my own post telling her that her mom was my world, and how we did everything together as a family, including the July 4th
~ 112 ~ outing that she was referring to? I also posted that I tried to do my very best at raising her. It was sincere and I was hoping I would hit a soft spot maybe even a breakthrough. The only thing it got me was Mark ‘unfriended’ me and blocked me within thirty seconds of my post. Now I can’t even view their Facebook postings or photos of the children.”
“How could this have started over such a small thing?” Ewa asked.
The small thing, and it was small, that Ewa was referring to was an incident that happened in January of 2013. One day after the grandchildren (ages 7 and 10) went home from being looked after by Ewa, Larry noticed a red marker line on the sofa. It was probably one and a half to two inches in length. “Ewa did you see this red marker on the arm of the sofa?” Larry yelled out to Ewa who was in the kitchen.
“The kids were doing their homework on the sofa. More than likely one of them had a mishap.”
“I’m going to email Mark and Tracy and asked them to ask the kids about it.”
“I wouldn’t bother,” replied Ewa. It’s such a small thing.”
“No, I certainly don’t want them to get into trouble over it, or get punished or anything like that, but I feel children should at least be aware of what they did so they can be a little bit more careful the next time.”
So Larry sent an email requesting that Mark and/or Tracy talk to the children.
Larry:
~ 113 ~ “Can you guys talk to Courtney and Christopher about the red marker line on the sofa - photo enclosed? Maybe Christopher would know more about it as he was sitting near the arm of the sofa.”
Mark immediately writes back: “Are you accusing Christopher of deliberately marking your sofa.”
Larry: “No, I am not accusing anyone of anything and I certainly don’t think they did it on purpose. I just thought if you asked them about it they would be more aware and also know that grampy noticed it.”
Mark: “My children would not do anything like that.”
Larry: “Look Mark, I love the grandchildren and it’s no big deal but children are children and they do make mistakes. Your children are no different than any other children. You sound like a parent who doesn’t think his kids can do any wrong.”
And so it went downhill from there. Tracy and Mark stopped having anything to do with Larry and Ewa after that. The hardest part is that they live just up the street from Larry. In the same house that Tracy grew up in – the same house Larry sold to them three months after Kris died – and at a price that was well well below market value.
“Ewa, getting back to your original question. I don’t feel there is a need to tell Tracy. The whole neighborhood knows how sick I am and I see her talking to them all the time. I am sure Tracy knew about it a long time ago
~ 114 ~ and she has never attempted to see me or find out how I’m doing. I think most daughters would be worried sick if their dad contacted a terminal disease - that’s what hurts the most - she doesn’t care.”
~ 115 ~ Chapter 13 – Disaster Strikes
Larry and Ewa decided that they would cut their time at Hampton beach to five weeks this year. As much as Larry enjoyed being there it was very confining as he had all but stopped going out for walks. “I love being here,” he told Ewa. “But after a couple of weeks of sitting on the balcony it starts getting pretty boring. Yes, the view is beautiful but I feel so isolated. I’ve never been a couch potato but it seems all I can do here is watch TV.”
“And I still feel so guilty every time I go for a bike ride or a walk,” Ewa responded. “It’s so lonely – we used to do everything together. It’s not the same.”
“No absolutely not. Never feel guilty. You need to get away from me and have your free time. We’ve had many a discussion on this. If you don’t go out, it just makes me feel guilty that you’re stuck here. Look you love bike riding and you love the ocean – it’s why we come here so go out and enjoy it. I’m okay - really - don’t give it another thought. I want you to do this”
“Would you try and go for a walk with me today? Even a short one - it’ll get you out of here and we can hold hands and feel the breeze on our face.”
“Sure why not. But I will have to walk real slow – I’ll hold you up. Plus it won’t be enough for you.”
“I can walk more later if I want to. What’s important now is that we do something together.”
~ 116 ~ They did go out for maybe three quarters of a mile total. Despite having his portable oxygen concentrator all the way up to number 6 position Larry had to stop several times as his oxygen saturation kept dropping to below eighty-eight percent.
“Do you know what next Friday is?” asked Ewa.
“Yes, it’s the second of June.”
“You know that’s not what I meant. It’s your seventieth birthday. Can you imagine? Seventy years old. We can go to the Old Salt for diner and come back and open a bottle of Champagne. Or we can do nothing. It’s up to you.”
“Yes, I would like to go out to eat. That would be good.”
Fortunately, Bret’s stomach pain was on the mild side that day. They spent his birthday together quietly - watching a Netflix movie - eating a wonderful gourmet dinner at The Old Salt – and sipping Champagne throughout the evening. And for Larry, who doesn’t like a lot of fanfare around birthdays and holidays, it was the perfect day. “Thank you Ewa - it was a great birthday. Peaceful and quiet. And I got to spend it with my best friend ...... I love you.”
“How much do you love me?”
“I love you immensely.”
“And I love you with all my heart – happy birthday.”
And so the rest of the condo stay went smoothly – well almost. I heard you coughing last night. Are you
~ 117 ~ getting a cold?” Larry asked Ewa as they were waking up on that last Monday morning of their stay.
“I think my sinuses are bothering me again, or maybe it’s allergies.”
“Sounded like a cold to me. The cough was pretty deep.”
“I hope not you certainly don’t need that.”
“I coughed a little bit more than usual last night myself. If you have a cold I think I have it too - and don’t worry I didn’t get it from you. It’s too close together. Incubation of a cold is about two or three days - we got it from the same source.”
“How is this possible? We wash our hands frequently, we stay out of crowds, and ask friends and relatives to be careful if they have a cold so that we are exposed as little as possible.”
“You know as well as I do that it only takes one touch of something that was touch by someone who was sick. Yes, we do our best but sometimes shit happens.”
By the end of the day it was obvious that Ewa did indeed have a cold and Larry was not far behind. They both went to bed that night hacking and coughing. By the next day the severity of the cold had become quite severe for Larry. “I’m just going to hang around today – I feel like crap. How are you doing?”
“Not great but I’m going to get up and get going.” Ewa responded.
~ 118 ~ Larry ended up by staying in bed most of the week. He was chilly one minute and hot the next. His energy level was minus five on a scale of one to ten, yet his temperature was only ninety-nine point five - just barely a fever – low grade. His cough was very very deep sort of croupish with ugly greenish stuff coming up. “Do you think you should call the doctor?” Ewa ask.
“No I don’t think so. What are they going to do? It’s only been four days and they’re not going to want to give me antibiotics this soon. Do you remember last spring when we went to Dr. Roker for my cold? He said he wasn’t sure if it was viral or bacterial so he didn’t want to prescribe anything. He did end up giving me antibiotics but told me to hold off a day or two to make sure – I ended up not taking them and the cold went away.”
By Saturday Ewa had shaken off most of what the cold had to offer and was on her way to recovery. Larry, on the other hand, was still wrapped up in bed, hacking and coughing, and according to his oximeter, was needing supplemental oxygen to stay above ninety percent. “This cold is really getting to me and I’m so tired. I could sleep 24/7,” Larry told Ewa about mid- morning. “I’m so short of breath. It was fortunate that we brought the portable oxygen unit with us. It’s helping to keep my saturation up.”
The next day was check out day - Larry could just barely stand and had a difficult time just getting dressed to go to the car. When he asked Ewa to drive home she knew that he was very sick - Larry never has Ewa drive. The one hour drive seemed like it took forever. When they got home Larry went straight to bed. He woke up in the middle of the night and felt like he was dying – his cough was unrelenting. He realized it was
~ 119 ~ time to call the doctor and would do so as soon as the office opened in the morning.
Dr. Roker was not available to see him as his schedule was full for that day. However, he was able to see Dr. Volstead at 11:00 o’clock that morning. Upon entering the examining room the nurse took his temperature and attempted to take his blood pressure. “You temperature is ninety-nine point nine,” she said. “I am having a hard time reading your blood pressure.” After three tries she said, “the best I can get is seventy-eight over forty-nine. “Are you dizzy?”
“A little,” Larry responded.
“Just sit tight. The doctor will be right in,” she said as she was leaving the room.
Dr. Volstead entered the room and immediately took a blood pressure reading of her own – actually she did it three or four times – pumping a little and allowing it to fall before pumping it again until she was satisfied with her readings. She then sat down on a stool in front of Larry. “You are a very sick man.”
“I’m not exactly feeling like running a marathon right now. Can you give me some antibiotics and I’ll be on my way.” Larry responded.
“I can’t treat you - not here. You need to be in a hospital.”
“I just want to go home with some medication and rest. I’ll be okay.”
~ 120 ~ “No you won’t be okay and I can’t and won’t treat you. It would be malpractice for me to treat you. As I said you need to be in a hospital.”
“Really – no choice?”
“I can call across the street to the hospital and have you admitted immediately. You just have to give me your permission.”
“I would rather go to the hospital where my pulmonologists works. He’s the one who knows my lungs the best and I trust him.”
“Either one but it has to happen.”
Larry sits there for a minute starring – trying to get his head around what is happening. He didn’t expect that this would be so complex when he came in. “The hospital across the street, which is a good hospital, or Dr. Gilson’s hospital which is an hour and a half away? I really feel like shit – I’ve got to lie down. I don’t think I can stand the long ride to Dr. Gilson’s hospital,” were thoughts going through Larry’s mind.
“What do you think Ewa?”
“It’s up to you,” she replied. “Are you strong enough to travel over one and a half hours?”
“I’ll go across the street. I just want to get this over with,” replied Larry.
So Dr. Volstead called the hospital across the street and by the time Larry arrived at the emergency room they were waiting for him and he was admitted very quickly. Ewa had text Larry’s son and he quickly left
~ 121 ~ work and appeared in the emergency room to offer his support.
By Tuesday, Larry woke up in the hospital to find himself feeling one hundred percent better than when he was admitted. Apparently, the antibiotics plus the fluids they were giving him were doing what they were supposed to be doing. However, his coughing had not subsided in the least. Sometimes, he would cough so violently that the air in his lungs would be repeatedly expelled none stop disallowing his body to intake new air. It was like out, out, out, out – but not a single intake. It was scary not only for Larry but for Ewa as well. He also found out that in addition to the lung infection (pneumonia) his kidneys were in acute distress due to dehydration so they were dripping all kinds of saline solution into his body. But overall, Larry was relaxed and happy - he felt that he was well on his way to recovery.
Occasionally, mother nature can play cruel tricks on you and she was about to bestow a particularly nasty one on Larry. As Wednesday progressed, he felt different - something wasn’t quite right. By evening, Larry’s O2 requirements became more and more demanding. “It feels like I can do less and less without having to raise my level of oxygen. It’s getting worse hour by hour, I can feel the difference,” he told Ewa. Larry, whose oxygen level had been set to 2 lpm, while at rest, and 6 lpm when he ambulated to the bathroom and back, was allowed by the hospital staff to adjust his own oxygen settings. He found that he now had to adjust the level to 2½ lpm, then 3 lpm, and now 3½ lpm at rest. He reported this to the nurse on duty who noted it in his chart. By Thursday, he was quite in distress and a CT scan was ordered to see what was going on. Larry was becoming more and more in panic mode as his oxygen
~ 122 ~ requirements were now at 5 lpm at rest. When he moved from the bed to the transport gurney for the CT scan his oxygen level dipped to seventy-six percent. “Breathe deeply and slowing,” the nurse said. “In through your nose and out through your mouth with pursed lips - slow, relax.”
Shortly after the CT scan was done the physician on duty came to visit with Larry and Ewa. “We have reviewed you CT scans and we don’t think it’s the pneumonia that’s causing your distress. It’s more than likely the rapid progression of your IPF.” Larry knew through his research about so-called exacerbation, a sudden and swift acceleration of the disease, which can come out of nowhere or be triggered by something like pneumonia. He knew it could be fatal with some studies stating that forty to forty-five percent of patients with acute exacerbation die with three to thirteen days. “There’s not much we can do,” he continued on. “We will ask for a consult from our pulmonary department.”
Ewa’s face turned ashen white and she started to cry. “Nothing - there must be something?”
“I’m sorry, but I’m afraid not,” the doctor replied.
Larry was trying to be stoic for Ewa’s sake by not showing any emotion. He laid there without saying anything at first then tried to calm Ewa. “I know what exacerbation is,” she said. “I’ve read about it on the forums.”
They were both stunned and remained silent for quite some time. Larry was deep in thought thinking, “This can’t be the end. There’s has to be something more that can be done.” After several more minutes Larry
~ 123 ~ suddenly blurted out, “I’m going to call Dr. Gilson. I don’t know why I hadn’t thought of that before.”
“Yes, that’s a great idea,” exclaimed Ewa.
He quickly pulled out his phone and made the call. Unfortunately, he reached Dr. Gilson’s answering service and had to leave a message. “This isn’t good. I sure hope he gets his messages often during the course of the day.”
About 10 minutes later just as the consult from the hospital pulmonology department was walking through the door, Dr. Gilson was returning Larry’s call. Larry answered the phone and explained the situation to Dr. Gilson, then handed the phone to the hospital pulmonologist. After several minutes of discussion, the hospital pulmonologist hung up the phone, and explained to Larry that Dr. Gilson would consult with her - they would be working on a new course of treatment. There might be some hope after all.
After the pulmonologist left the room Larry and Ewa felt some relief. “I can’t believe he called back within ten minutes,” Larry said to Ewa. “I sure am glad I came up with the idea of calling Dr. Gilson instead of leaving it up to the hospital staff. At least with his expertise of twenty years with IPF patients I feel that if there is anything that can be done Dr. Gilson will know about it.”
“Yes, he’s a good caring doctor. How many doctors would call you back almost immediately? We are so lucky to have him.” Ewa said in agreement.
Larry had no idea what the hospital pulmonologist and Dr. Gilson came up with for a new treatment plan, but within an hour from the phone call, new meds were
~ 124 ~ being administered through his IV port. The next day Larry woke up feeling quite different – he was starting to feel pretty good again. In fact, he felt he was almost at the same point of recovery as he was on Tuesday, the day after he was admitted - and that was good. He got stronger and stronger throughout the day and his oxygen needs were reduced until he was back to 2 lpm. By Saturday, the hospital doctor came in and declared he was now on his way to recovery and he could be discharged the next day.
“You know I think Dr. Gilson saved my life.” Larry said to Ewa after the attending physician left the room. I was going downhill so fast I don’t think I would have made it without Dr. Gilson’s input.”
“Yes, definitely. They basically gave up on you.”
On Sunday, the attending came in and told Larry he would be going home.
“But I don’t feel I’m ready to go home. Hell, I was just on death’s door two days ago.” Larry argued with the doctor.
“Everything checks out okay and you can continue treatment with medications at home. The standard protocol for pneumonia is a one week hospital stay,” replied the hospital doctor.
“You mean you don’t want to write up a justification report to Medicare for me to stay here. ”
“I’ll write up the discharge papers and as soon as they’re done you’ll be able to go home.”
~ 125 ~ Well just like that Larry was being kicked out of the hospital.
“It’s all about the money,” Ewa said. “It’s always about the money.”
“But we have good supplemental insurance in addition to Medicare. I can’t believe they’re tossing me.”
“Maybe, this is a good thing. You’re on the pulmonology floor and there are all kinds of patients with all kinds of problems. Maybe you should leave before you pick up something else. You know people often pick up bacteria from hospitals and get sick or die from something completely different then what they were admitted for,” said Ewa.
“You’re right. It’s probably for the best. I could end up worse off than I am. Besides it will be nice to sleep together in our own bed again. I miss being next to you. You must be all cramped up from sleeping on that window seat all week. It must have been really uncomfortable. I want you to know that it was comforting for me to wake up knowing that you were there. I love you ...... immensely.”
“I love you too with all my heart.”
Larry’s discharge orders were quite simple:
80 mg prednisone daily for two weeks then 60 mg until follow up with pulmonologist in four to six weeks.
Bactrim (antibiotic) 500 mg - three times per week.
~ 126 ~ Oxygen 24/7 – setting of 2 lpm until pulmonologist follow up.
Follow up with primary care physician in two weeks.
Normal diet.
Larry questioned the need for prednisone as it was not the standard protocol for IPF. He was told that it helps reduce inflammation due to the infection he had in his lungs and would help him to recover faster. He would be weaned off of it over a long period of time depending on the preferences of his pulmonologist.
After he got dressed, the discharge nurse came in to assist him out of the hospital. She wanted to have him sit in a wheelchair so she could wheel him to the exit door. Larry would have no part of that. “I came in on my own two legs and I’m leaving on my own two legs.”
The nurse started to insist that he get into the wheelchair, “It’s hospital policy.”
But Larry was as stubborn as usual, “If I’m healthy enough to leave, I’m healthy enough to walk out.” And that’s exactly what he did.
~ 127 ~ Chapter 14 – Recovering
It was Monday morning, the first day back from the hospital. Larry was just getting out of bed when the phone rang. Ewa answered the phone, so he waited a moment before leaving the room to see if it might be someone wanting to talk to him as a follow up to his hospital stay. “It’s someone from the VNA,” Ewa said as she cupped the phone in her hand.
Larry started gesturing and waving his hand frantically and responded in a low voice, “Tell them I’m not interested in whatever they have to offer – not interested.”
Ewa held the phone back up to her ear and told the other party that she didn’t think her husband would be interested in their services. After a few more moments of discussion Ewa cupped the phone again, “You really should talk to her. It might be helpful assistance to you and for us,” and then handed Larry the phone.
“Hello this is Larry.”
“Larry this is Tessa from the Visiting Nurses Association. You’ve been referred to us by the hospital doctor to follow up on your recent hospital stay. I would like to come to your home to make certain everything is going well and to make sure that you don’t have a relapse and have to go back there again.”
“Does Medicare pay for these visits.”
“Yes, one hundred percent it won’t cost you anything.”
~ 128 ~ Larry started thinking about his concern that he had left the hospital too early.
“Okay, let’s give it a try.”
“I can be there at 11:00 if that’s good for you.”
“Sure, I’ll see you then.”
After hanging up the phone, Larry told Ewa that he still felt that he was released from the hospital too soon after his recent downfall. Having a visiting nurse come in was possibly a good alternative - he would be professionally monitored for any issues that might arise without being subjected to hospital germs.
Eleven o’clock chimed on Larry’s grandfather clock and before long there was a knock on the front door. Larry arose from his recliner and opened the door. He was greeted by a young lady (everyone is young at 70) with a casual demeanor and warm smile, “Hi Larry, I’m Tessa from the VNA. How are you doing today?” He knew immediately that he was going to like this person.
Tessa explained that the purpose of her visits was to act as an intermediary between Larry and his doctors. “Studies have shown that a nurse monitoring patients just out of the hospital will often circumvent issues that might otherwise cause the patient to relapse and be readmitted,” explained Tessa. Depending on Larry’s progress she would visit him three times a week to start and taper down to every other week until he was discharged.
Tessa was very thorough in questioning Larry about his health. He found her to be incredibly knowledgeable and showed a genuine interest in Larry as a person.
~ 129 ~ On her first visit she discovered that Larry had developed a case of thrush in his mouth (a fungus) more than likely from the high doses of antibiotics and prednisone he was taking. Tessa also was concerned that Larry’s oxygen concentrator was not humidified and would eventually create issues with the dry oxygen flowing through his nose. A quick call to Larry’s pulmonologist and prescriptions were issued to take care of both matters. As the weeks went by, Larry found Tessa to be a caring person from within - it was her nature - her outside personal life with foster care, animals, and community projects were as compassionate as her professional life. Larry and Ewa were uplifted each time she visited. “I like her so much,” Ewa said to Larry as Tessa was getting into her car to leave after one of her visits.
“Yes, we are lucky to have her. I can’t believe I almost passed up the opportunity to have these visits. But she’s been a godsend.”
The hospital stay had set Larry back a little but surprisingly his oxygen numbers were not that far off from when he had gone in. He experimented with removing the supplemental oxygen and found he could still maintain a level of ninety-three to ninety-four percent saturation while at rest. When walking around the house, a level of 2 lpm was sufficient for him to stay above ninety percent oxygen saturation. This was good, as everyone told him it would take four to six weeks to fully heal and recover from a bout with pneumonia, so he was expecting to drop the full time supplemental oxygen very soon.
He was still coughing off and on but the intensity and length off the coughing spells was greatly diminished and it was getting better almost on a daily basis.
~ 130 ~ Larry discovered that four events were the main cause of his plummeting oxygen levels on a day to day basis.
Getting in and out of bed. Getting in and out of a chair – particularly a soft chair or sofa. Bending at the waist to pick up something off the floor. Taking a shower.
He never realized doing any of the above would be such an oxygen grabber. Those simple task were always taken for granted. His saturation level would go from ninety-eight percent to as low as eighty-two percent by doing any one of them even with supplemental oxygen. “How can the simple act of getting into bed rob me of all my oxygen?” he would ask himself. He would take a deep breath or several deep breaths before and during any of those four oxygen stealers – it didn’t matter the results were always the same. Nor could he get a satisfactory answer from his doctors. However he did take solace in that every doctor told him that dips of that nature are not harmful. “As long as you recover in a minute or so, it won’t hurt you. It’s the long consistent low oxygen levels that will damage your organs, heart and brain.” Larry decided that taking a shower was a long enough event so that he raised his supplemental oxygen to 6 lpm while doing this activity. The other three events were too short and too frequent to raise the level of his oxygen each time one occurred.
The follow up visit with Dr. Roker was quite short. Larry’s blood pressure was in the mid one hundred thirties systolic, and a bit higher than what he would like to see. He asked Dr. Roker to put him back on two of the blood pressure medications that were stopped in
~ 131 ~ the hospital. He explained to Dr. Roker that he had a tense discussion with the hospital doctor after he was taken off the blood pressure medications. “Your systolic pressure usually runs between one hundred thirty-eight and one hundred forty-three and that’s fine and accepted for someone your age.” Larry recalled the discussion with the hospital doctor.
“Yes, that’s fine for someone who has no known medical issues. But I have a history of heart disease in my family and my ascending aorta is slightly enlarged. The standard for someone at risk is one hundred thirty maximum. I should be in the one twenties or even teens,” Larry shot back at the hospital doctor. Dr. Roker had no comments on the reasoning behind the hospital doctor’s assessment but he did agree to reintroduced the discontinued medications - albeit on a lower level. Blood test indicated Larry’s kidneys were still not functioning fully as some of his electrolytes were still off a bit. “This will take time,” Dr. Roker explained. “Your extreme dehydration did cause damage that will heal but it will take time. I will have you do blood work weekly to make sure your numbers are continuing to go in the right direction.”
He was still on sixty milligrams of prednisone and the effects were really taking a toll on Larry’s physical strength and appearance. His hands were shaking so that he couldn’t write a legible sentence – either in cursive or printed in block letters. When he tried to go up a step or two his legs wobbled and one time when he tried to rise from a kneeling position he fell over because his legs gave out. “What the hell? What is going on with me?” Larry thought as he looked into the mirror. “I’m like a monster.” His face had bloated up like an overblown balloon waiting to burst. Several days ago he had noticed his legs, feet, and stomach
~ 132 ~ were swollen but this new facial discovery really got to him. “I’ve got to get off of this crap as soon as possible.” Larry called Dr. Gilson’s office and left a message asking if he could start tapering down his current sixty milligram dose by ten milligrams a week. A day later, he received a call from Dr. Gilson’s nurse okaying him to do this.
Despite the horrible issues that prednisone created there were some positive elements of this drug - not that the positive outweighed the negative but they were positive nonetheless. Before the hospital stay, Larry used to fall asleep frequently during the day - so much so that he was never able to finish a television program. Now with the prednisone in his system, he was awake and clear headed from the time he woke up to the time he went to bed. His stomach aches that occurred each day around 2 o’clock were completely gone. In addition, his appetite was back and it was veracious – food was tasting good again. Larry was eating anything and everything and soon gained back fifteen pounds of the twenty pounds he had loss. Chocolate bars was his new favorite food. “Ewa do you mind getting three of those mini Kit Kats and Reese’s out of the fridge for me?”
“How many is that today?”
“Oh, not many. Besides they’re small – you know portion control. Isn’t that why you buy them?” Larry responded with a big grin on his face. . Larry’s follow up visit to his pulmonologist was scheduled for August 9th – about six weeks after being released from the hospital. It was the first appointment after lunch so the wait time was fairly short - about a half hour. As Dr. Gilson entered the examining room
~ 133 ~ Ewa couldn’t wait to get the first words out, “Thank you doctor for saving his life. They had given up on him until you got involved.”
Larry followed that with, “Yes, and thank you for the incredibly quick call back. I mean you returned my call within 10 minutes. I really appreciate that you did that for me.”
Dr. Gilson responded in his usual humble manner, “Your welcome. I always try to do the best for my patients.” He then started to review Larry’s CT scans from the hospital on his computer. After a couple of minutes, he turned the computer around to show Larry this big white blotch on his upper left lung. “This was the problem,” Dr. Gilson said as he pointed to the blotch. “Could have been pneumonia or some other infection. We really don’t know.”
Dr. Gilson went on to explain that there are always certain types of bacteria residing in the lungs that may be harmful. “As long as your immune system is healthy and strong there are no worries. But the cold that you contracted more than likely lowered your immunity to a level that allowed this bacteria to flourish. This will take 10-12 weeks to completely heal and resolve itself. It’s too early for PFT’s. When is your next scheduled appointment?”
Larry’s ears perked up. Since this is only week six, maybe he would be able to get off full time oxygen supplementation once the healing process is complete. “About six weeks from now - September 15th, he responded.
“That should be good. We’ll do the PFT’s then.”
~ 134 ~ Dr. Gilson asked Larry again whether anyone had done a sleep study on him. Larry remembered that the last time this was brought up, he had promised Dr. Gilson that he would do his own sleep study with his data recordable oximeter. He promised that on his September 15th visit, he would bring in his overnight data for review.
Larry asked Dr. Gilson to look in his mouth as he still felt tingles of thrush, “I’ve been taking this ‘swish and swallow’ stuff since I’ve gotten out of the hospital – it keeps coming back.”
“That drug is not really strong enough for someone like yourself taking antibiotics and prednisone. You’ll never get rid of it as long as you are on them. I’ll prescribe fluconazole (Diflucan). It’s a daily tablet that gets into your system. It will better control the thrush.”
As usual, Larry asked about available trials and Dr. Gilson said there was one but it carried a lot of risks which under the circumstances of Larry’s recent health scare, he was not willing to have Larry participate in at the moment. “There is a second one that might be open later this fall. I will keep you in mind.”
~ 135 ~ Chapter 15 – Disappointment
“How do you feel about going back to the condo in September?” Larry asked Ewa.
“I would like to go but it’s really up to you and how you feel.”
“The biggest concern I have is with the oxygen concentrator. It is so loud and it puts out so much heat that I don’t see how we could comfortably live in that tiny condo with it running all the time. If there was a separate room we could put the unit into that would be different. Also from my perspective, I’m thinking, ‘What am I going to do there for seven weeks’. I can’t walk with the portable unit anymore which just leaves me sitting on the sofa watching television or on the balcony watching people walk by.”
“Yes, I understand. I am going to miss being there. You know I can’t get enough of the ocean.”
“I really am sorry. I could probably stand it for a couple of weeks but beyond that I would get stir crazy. However, that doesn’t solve the issues with the concentrator.”
“Can we put it out on the balcony?” Ewa asked.
“No, not really. It’s not made for outdoor use and certainly not for an ocean environment with salt air.”
“I guess we will have to cancel then,” Ewa said with sadness.
~ 136 ~ “Let’s take a ride to Hampton tomorrow. We’ve been going there for five years now and they’ve been good to us. I don’t want to do this over the phone - I want to talk to them in person.”
“I agree that’s the way it should be done.”
After being out of the hospital for some eight weeks now Larry was getting antsy and wanted to start doing aerobic/weight exercises. Besides, he was being prodded by Tessa, and especially Ewa, that he would recover faster he got off his butt. “You know you can’t just keep sitting around all day?” Ewa would say. He knew that – but sometimes you just need to hear it from someone else.
Larry set the oxygen concentrator to level 6 lpm and began his routine. The work out, with a mixture of short aerobic and dumbbell exercises, went smoothly. He was able to maintain a saturation level above eighty- eight percent for the entire forty minutes of the program. Larry planned on doing this routine four days a week along with a bit of walking on his off days. Since his portable unit was insufficient for him to go outdoors to walk, he knew he would have to do something that allowed to use the more powerful steady flow unit inside. Larry measured the distance of his house from end to end – one hundred ten feet round trip. So back and forth he went, “Almost as boring as watching paint dry,” Larry thought. “Holy crap, I will need seventy-two round trips to achieve one and a half miles. That’s not much, but it’s a start after being sedentary for two months”.
About a week later, he noticed that something was notably different in his oxygen requirements. His ‘at rest’ oxygen saturation without supplemental oxygen,
~ 137 ~ which is something he experimented with at times for twenty to thirty minutes to see how his lungs were working, was now dipping to eight-nine to ninety percent. It used to be ninety-three to ninety-four percent when he first came out of the hospital. Also, at level 6 lpm while exercising, he was now having difficulty maintaining oxygen levels above eighty-eight percent - even while walking. Larry remembered that this happened pretty much in sync with his prednisone dosage being reduced from forty milligrams to thirty milligrams. “Could the prednisone have made a difference he thought?” He decided to text a note to Dr. Gilson to see if this was possible. The next day Dr. Gilson called Larry. He explained that the prednisone should have no bearing on his oxygen levels at this point in time. It was prescribed to reduce the inflammation caused by the pneumonia. “IPF is not an inflammatory disease that’s why prednisone is no longer used to treat IPF. If what you’re saying is true, we will have to take a closer look at what is going on,” explained Dr. Gilson.
“I realize that the numbers I gave you are anecdotal - it could be just a coincidence that my numbers dropped in the same time frame that I reduced my prednisone dosage,” Larry replied.
“Why don’t you go back up to 40 mg for a week? See what happens then send me a text message about how you are feeling,” instructed Dr. Gilson.
As much as Larry wanted to get off the ‘drug from hell’, he was willing to up the dosage if it might possibly help him get back to where he was with his oxygen saturation levels. After a week, he could see it was all for not. Nothing changed. He text Dr. Gilson the results of his experiment. The next day, Larry adjusted
~ 138 ~ his dosage back down to thirty milligrams, and continued the tapering process at ten milligrams per week.
Larry begrudgingly adjusted his ‘at rest’ level of oxygen from 2 lpm to 3½ lpm. He found that his current state of being did not allow him to even get up to get a glass of water without his oxygen level desaturating to eighty- eight percent or lower. The extra 1½ lpm helped him to stay above ninety percent for those short jaunts across the house. Now the realization of his never getting off of oxygen came into full view. “I will always be on this leash (hose). My oxygen levels were better when I first got out of the hospital then I am now.”
On September 15, 2017 Larry and Ewa made the two hour trip to see Dr. Gilson for his regular check up. They arrived about forty-five minutes early so the PFT lab could perform the test that Dr. Gilson had ordered. Larry expanded his lungs and blew as fast and as hard as he could – forty-five percent of predicted value. This was five percent lower than six months ago. “Let’s try it again,” said the technician operating the machine. For the next two attempts Larry did his very best to up his numbers even a bit but to no avail. Forty-five percent was the best he was able to muster. “Now we will do the DLCO test. As soon as the machine clears itself we’ll be ready to start. Just relax for a moment.”
Larry turned around in his chair and looked at Ewa. She returned his gaze with a smile. It was not her usual glowing smile, it was a ‘I am kind of concerned’ sort of smile. “Are you ready to get started?” asked the technician. Larry placed his mouth around the rubber mouth piece – blew all the air out of his lungs – then took a deep breath. “Hold it, hold it, hold it,” instructed the technician. “Now blow, blow, blow, blow . . . . “
~ 139 ~ Larry was feeling slightly dizzy from the sudden inhale exhale but it quickly passed. “What does it look like,” Larry asked. The technician flipped his monitor around to show the results – it read twenty-two percent. “ Twenty-two percent,” Larry blurted out. “Holy shit. How can this be?”
“Take a break. We’ll do it again in a moment.” said the technician. Larry looked over at Ewa again only to see her smile had disappeared. The only thing he could focus on was the deep sadness in her face - her lips were quivering and her eyes were watering up.
“Okay,” Larry thought. “You have one more shot at this. Make it a good one.” As hard as he tried the results were exactly the same as the first one – twenty- two percent.
“Now we’ll do a walk test,” said the technician. He asked Larry what his normal level of oxygen would be when he walked. Setting the dial at 6 lpm they strolled out the door and into the hallways of the clinic. At three hundred fifty feet his saturation level dropped to eighty- seven percent. “Stop,” said the technician. “I’m going to bump you up to 7 lpm.” After a quick click of the dial they were on their way and Larry was able to finish the walk at that level.
Upon completion of the testing, the technician brought Larry and Ewa into the examining room where they waited for Dr. Gilson to arrive. Again, this must have been Dr. Gilson’s first appointment after lunch because just like last month, he was only about thirty-five minutes late. He reviewed the PFT’s and told Larry the FVC results (45 percent) was a better predictor of his disease then the DLCO. He explained that many factors enter into the DLCO and he felt Larry’s numbers
~ 140 ~ were probably not a reflection of what is really happening. “After all you went through in the hospital I thought your numbers would be lower,” he explained.
Larry asked Dr. Gilson if would use his twenty years of experience with IPF to predict what was happening – that is, his projected outcome, but Dr. Gilson sidestepped the question. Larry kind of expected that he would but he felt it was worth a shot. “You’ll never find out anything if you don’t ask,” he thought.
The sleep study that Larry had performed two weeks earlier at home using his recordable data oximeter was reviewed by Dr. Gilson. He saw that Larry’s saturation level stayed at ninety-two percent, or greater, ninety- nine point one percent of the time at a level 2½ lpm. Dr. Gilson seemed satisfied with the results and made no recommendations.
Larry asked about his swelling of the feet, stomach, and face. After listening to his heart and lungs Dr. Gilson commented that Larry might also have some water in his lungs. “It’s hard to discern whether the crackles are from the IPF or from water. “If there is water it could impede your oxygen absorption. I’m going to start you on furosemide (Lasix – a diuretic) to see if that helps. You’ll have to contact your PCP and get blood work done every couple of weeks to make sure your electrolytes stay within range. I also want you to have an echocardiogram. This will help determine if your heart is working too hard in an effort to supply oxygen to your body and also if you have signs of pulmonary hypertension.” j Larry asked some questions about “end of life” care. He was mainly concerned about passing in a hospital or
~ 141 ~ hospice. “I want to die at home,” he explained. Ewa concurring fully.
“That’s not a problem. There’s nothing we can’t control in the hospital that we can’t control in a home environment. You won’t suffer at all. If that’s what you want we can certainly make that happen. Please make your next appointment in about three months along with PFT’s. And continue exercising – that’s important.”
“Are you feeling better now?” Larry asked Ewa as they were walking down the hallway.
“Yes, Dr. Gilson’s positive attitude is uplifting. He doesn’t sugar coat but he also doesn’t bring you down. He said you weren’t as bad as he was expecting.”
“And what do you think?”
“I’m okay with that. Like he said it could have been worse.”
A couple weeks later Larry was lying on a gurney in the cardiology department of the local clinic having his echocardiogram performed. It had been two weeks since starting the Lasix, so after the echo, he walked over to the lab and had his blood drawn to test his electrolytes. The results of both test were posted several days later on the clinic’s website. “I’m not a doctor,” Larry said to Ewa “But I think everything looks to be okay. I only see the words slightly or mildly associated with various areas of my heart and the blood work is all within normal range.”
“That’s great news. So basically you’re healthy except for your lungs.”
~ 142 ~ “Looks like - but that fact doesn’t give me much consolation at this point.”
Larry was now completely off his prednisone regimen and things were getting back to normal. The edema (swelling) in his face had subsided, his hands were more steady, his muscle weakness was getting better, and the swelling in his feet was almost non-existent. He wasn’t sure if his returning to normal was the result of taking Lasix, which was pulling water out of his system, or from the discontinuance of prednisone, but he wanted to find out. “Next time I text or talk to Dr. Gilson I’ll ask him if I can get away from the Lasix,” he thought.
The Lasix was a ‘bear’. Although Dr. Gilson had prescribed it as an ‘every other day’ dosage and the effects didn’t last long, it was very disruptive on the day he took it. From about a half hour after taking the medication to six or seven hours thereafter, he would have to urinate every twenty to thirty minutes. This meant that Larry and Ewa had to forego any day trips planned on dosage day. “Can we take a ride to the mountains on Thursday?” asked Ewa.
“Let me see . . . . . sorry hon. that’s a pee day.”
The cessation of the prednisone was not all fun and games. Larry began to fall asleep again while watching television. In fact, if he sat for too long without stirring, he would doze off. Food became an issue as his appetite gradually diminished and his taste buds became desensitized. He was back to imagining various foods, and what they tasted like, to see what was the least objectionable for a particular meal. “What do you feel like having for dinner honey?” asked Ewa.
~ 143 ~ “Hmmm, pasta – nah, ground beef – nah, soup and sandwich – nah, chicken salad – that might be okay,” Larry thought.
“Honey, did you hear me?
“Yes, I was just going through the possibilities that might be palatable. How about chicken salad?”
Ewa tried her best to offer Larry lots of food varieties, but meals were chosen based on what he could tolerate and consume. It’s not that a wrong choice made him nauseous, it just that a wrong choice was unpalatable – something that couldn’t be eaten. Unfortunately, even a good selection most often lead Larry to feel full after a half dozen bites. Things got so bad - and this is really sad - that even his favorite treats – Kit Kats and Reese’s were no longer palatable. The stomach pain was also back. It came on gradually. One day he felt twinges of pain - barely noticeable, “I think I feel the pain I used to feel before the prednisone,” he told Ewa. Within the next couple of weeks the intensity increased to where it used to be.
“I don’t know what’s worse. The prednisone or the stomach pain,” he told Ewa one afternoon after stopping at a pizzeria. “The prednisone was probably doing a lot of damage to my body in general, but this pain in my gut every afternoon is relentless. I never had it when I was on the prednisone.” “Sorry baby. I wish I could do something to help you.”
~ 144 ~ Chapter 16 – Getting Real
The disease was mentally starting to bother Larry more and more. He was depressed and was well aware of it - although he wouldn’t fully admit it to others. He still enjoyed life, yet he found it hard to look at the long term. “What’s the point of planting spring bulbs when I may not be here to see them when they flower,” he would ask himself. Priorities took on a whole new meaning and living for the moment became a mantra. It was also difficult for Larry to observe and appreciate things of beauty that surrounded him such as a colorful sunset or the bright colors of autumn leaves. “I see a family of deer in the meadow and marvel at nature’s offerings. But what does that really matter? For once I’m gone, it really makes no difference whether I saw that picturesque scene or not?” I mean I’m not going to remember it after I pass so obviously I won’t feel deprived. It won’t make me a better person for having seen it nor will it give me peace and happiness for all eternity.”
“So what are you saying?” asked Ewa.
“I guess what I’m saying is I don’t have a bucket list. What’s the point.”
“You should enjoy your life as much as you can.”
“Don’t get me wrong I’m still happy with my life. It’s been a good life. And I still have hope. You have to have hope. I believe life is about hope. If someone doesn’t have hope then they are on their way to suicide – and I would never ever contemplate taking my own
~ 145 ~ life. You know the old saying, ‘Tomorrow is another day’ – so simple and so true.”
“You have me in your life and I love you with all my heart,” Ewa said with a smile.
“I know dear. I love you too. I wake up each morning to your beautiful smile and it jump starts my day. I am so lucky to have you in my life.”
Larry knew that he was very fortunate to have Ewa in his life. She has unwavering support and was always there for him – not just now that he is ill - but always. Unfortunately, his personality had changed over the last several months. He was often curt and sharp tongued at the least little thing Ewa would say. It was like he was always looking to pick a fight.
“Why are you so nasty all the time? I’m only trying to help you,” Ewa asked.
“I don’t know. I just spit things out then feel remorse that I said what I said. And once I start it’s like someone else is speaking for me.”
“Is it the disease? Are you depressed? Maybe you need to talk to the doctor about ‘happy pills’.”
“I don’t want ‘happy pills’. I should be able to control myself and my emotions.”
“The only thing I know is that I love you. You need to stop treating me like I’m your enemy.”
Larry knew she was right of course. He loved Ewa immensely and he would do anything for her - and she knew that too - which is why she sticks by him. “Your
~ 146 ~ sickness could have been my sickness and I know you would be there for me. We’re in this together. There’s just the two of us,” Ewa told Larry.
Larry was always critical of other people, including himself, which often made it hard to relate to people and their shortcomings. However, Ewa wasn’t ‘other people’. During his pensive moments, he realized he was going overboard with the one and only person who truly loved him, and who would be there for him during this horrible ordeal. Time and time again after each moment of nastiness, he would be sorry and remorseful but ultimately that was not good enough. He had to stop hurting Ewa with his unkind words.
“This is real,” Larry thought while lying in bed at 3:00 am one morning. Even though his illness had been a constant companion for the last six plus years, he very seldom dwelled on what was actually happening to him. “This is never going to go away. Things will never get better. If I’m lucky it could stabilize for a while but ultimately . . . . .” His mind began to wonder back in time – to the good things in his life, to what he had accomplished, to the people he loved, and to the people he had hurt with words that never should have been spoken. He thought of Kris, his love of 25 years, who had passed at just 48 years of age – her joy for life, her suffering though her illness, and how they had made a good life together. He was worried about Ewa -not that she wouldn’t survive, she is strong and resilient, but worried that she would be alone. Larry knew what feeling alone was all about. He was so alone before he met Ewa – so downtrodden after losing Kris – he didn’t want Ewa to feel that way. “Maybe she’ll meet someone new? Someone who will be good to her. Someone who will make her happy again. Lord knows she deserves happiness after what I’ve put her
~ 147 ~ through.” Financially, he knew she would be good. He had carefully checked every asset, every account, and every legal paper to make sure the transition would be as easy for her as possible. He just wished he could do the same for her in the ‘grief’ department. “She is too good of a person to suffer and be sad.”
“I have to stop thinking about this. It’ll drive me nuts. I realize it’s happening but I just can’t get my head around it. I have to just let it happen.” And so Larry drifted off to sleep.
Nurse Tessa from the VNA arrived for her weekly visit that morning. Larry told her that he had blood drawn for a comprehensive metabolic panel several days ago, due to his taking Lasix and the results were good. He also told her that he had reduced the dosage of the Lasix from forty milligrams to twenty milligrams every other day and was thinking of stopping it altogether. Larry had text Dr. Gilson the day after Tessa left from her weekly visit last week and he okayed discontinuing the Lasix altogether. Tessa felt that his lung sounds were better while he was taking the Lasix, and although she didn’t say it in so many words, gave Larry the impression that he should continue. “It’s too bad you don’t have a visit with Dr. Gilson for two more months as I think he should hear your lungs,” commented Tessa.
“I’ll continue the twenty milligrams for a while longer,” Larry replied. “It’s just that the forty milligrams was so uncomfortable with me going every twenty minutes or so but I can handle the twenty milligrams.”
“Are you sleeping well?”
~ 148 ~ “Not really. It’s like I can’t get comfortable lying down. I have these aches and pains throughout my body. I used to take Excedrin back and body medication but I’m taking so much crap now that I am trying to stay away from adding more junk to my body.”
“How would you describe your mood? Are you depressed?”
“Well, I’m not jumping for joy every day but on the other hand, I don’t mind getting out of bed in the morning to face the day. I still like my life and look forward to each and every day. Overall, I would say I’m mildly depressed most of the time and moderate on some days - I’m not afraid to admit it”
Tessa went on to explain that there is a class of antidepressant called SSRI (selective serotonin reuptake inhibitor). Basically it makes more serotonin available to the brain by stopping it from absorbing excess serotonin. The brain uses this serotonin for neurotransmission of brain waves. It is not addictive. “I used it for several months when I was going through some personal difficulties. It does help.”
“I would be open to trying that – did you say SSRI?” asked Larry.
“Yes, the most common one is Zooloft – the generic is sertraline.”
“Okay, I just don’t want anything that will mess up my brain chemically. If this drug just helps my brain to work more efficiently I’m good with that. Can you call my PCP and help me get a prescription for it?”
“Yes, I’ll take care of that for you.”
~ 149 ~ “What do you know about hospice care?” asked Larry
Tessa went on to explain that hospice care involved a doctor(s) certifying that the expectation of a patient living more than six months is improbable. Under hospice, Medicare covers more items, like hospital beds for example, but there is no treatment plan. The object for hospice is to make the patient as comfortable as possible during the remaining time that is left. It can be carried out at home or in a hospice center. Palliative care, on the other hand, can be used by anyone with a serious illness. Medicare will pay for the same items you are currently eligible. The goal, like hospice, is to make the patient as comfortable as possible, but it also includes treatment plans, if available, to promote health and to allow the patient to get better. Home treatment, as well as care facilities are also available with palliative care.
“Interesting, I know your busy today so maybe we can talk about it more next week?”
______
Larry’s footsteps here on earth (November 2017) could go on for three months or three years – it’s all dependent on whether the disease stabilizes. His oxygen requirements at rest are currently 3 ¾ lpm and he needs 8 lpm just to maintain an oxygen saturation of eighty-eight percent while walking slowly in the house. When he was discharged from the hospital four months ago, he only needed 2 lpm at rest and 6 lpm for exercising – and that was a more rigorous exercise then he is currently doing with his walk. So his disease is progressing. Larry told Ewa that he was probably not
~ 150 ~ going to do the PFT’s on his next visit to Dr. Gilson. “No reason,” he said. “It just for data. I thought I would get into a trial, but now even if one is available I’m too far gone. I wouldn’t be accepted.”
And so Larry continues to go on with his life - it just getting harder and harder with each passing week. Last spring, realizing his future had limited expectations, he started making short term goals for survival – his birthday (made it), their wedding anniversary (made it), Christmas (next), Ewa’s birthday (he so wants to make that one – she will be seventy) and Easter.
This is the last public chapter of Larry’s story. He has decided that there is no more that he wants to tell. After all, the last chapter would literally be the ‘last chapter’, and he doesn’t want to be on his death bed figuring out how to end his story (dark humor). No, whatever life has to offer, it will be shared only with Ewa and his family.
When all is said and done – IT IS, WHAT IT IS.
~ 151 ~