This week, the world lost a champion. On June 3, 2019, Kim Fredrickson, a stoic and heroic inspiration to so many people living with pulmonary fibrosis (PF), died after nearly four months in the intensive care unit at UCSF Medical Center, where she was awaiting a life-saving lung transplant.
When events like these happen in our lives, they have the potential to shake us to our core, devastating us and shifting the ground on which we stand, as individuals as well as collectively as groups, such as those people living with a chronic respiratory illness like PF.
We all have that little voice inside our heads (and our hearts) that is hoping for that fairy-tale ending in which the hero receives new lungs and lives happily ever after. Unfortunately, life is not a Disney movie, and sometimes it is not fair and that happy ending never comes.
In times like these, it is important for us to recognize the good in the situation, and in the case of Kim Fredrickson, there is so much good we can look to and be inspired by. Kim is someone for whom I had immense admiration as I know so many did in the respiratory community. While she never got her new lungs, she fought tooth and nail for every breath, each and every day, right up until the last day, for herself, for her loved ones, and for those she cared about — and that includes you.
What I hope is that you will not see Kim’s loss as a failure. Instead, I would encourage you to see Kim’s life as an inspiration and use her fight, her struggle, and her courageous and triumphant life, which was a gift to so, so, so many people, as motivation not to quit, not to give up, and not to take the position that all is lost. Rather, use Kim as inspiration to say that in spite of this and in light of this, there is now more of a reason for us to dig down and push harder and to ask ourselves what we can do with our time on this earth, regardless of our medical condition or any other challenges we may be facing in our own lives.
Even though Kim had her back to the wall, she never gave up, she never stopped trying, she never lost hope, she never lost faith in her God, and she never lost the belief that she would get lungs. And despite all of that, Kim was an incredibly selfless individual, who was able to step outside herself and still put others first, and think about what she can do for the world and what she can do for each and every one of us.
I have always believed the best way to honor someone we have lost is to keep pushing, to keep living your best life, and to keep carrying that flag for those who are no longer with us. Kim fought an incredibly epic battle with tremendous courage and grace, right up until the last day. Despite her own struggles, Kim was somebody I saw over and over again take the time to pass on a compliment, to pass on a word of encouragement to someone else in the struggle, and someone who was able and willing to step out of her own situation and ask what good can come from this incredibly challenging situation. I encourage you to do the same.
A few weeks ago, Kim and I met in San Francisco, and we agreed to collaborate on a new book that would be a blend between her book, Pulmonary Fibrosis Journey, and mine, Ultimate Pulmonary Wellness. The title we agreed upon is Pulmonary Fibrosis: Yes, We Can. That book will go forward, and it will come out, so even after her death, Kim will be influencing and inspiring many, many others.
Again, to Kim’s family, I want to say I am so sorry for your loss. As you know, you had a warrior in your camp, and I know you know that. To the pulmonary community, I encourage you to keep your head up, people, and don’t get stuck. Keep moving forward. We still have more to do.
May God bless Kim Fredrickson and her family.
Finally, during our last conversation on May 28, Kim and I agreed we would publish one chapter at a time on Pulmonary Fibrosis News as each becomes available so that people can read for free and benefit from the material before the final version is published. I present the first look of that project here.
Why This Book?
Four years ago, I was diagnosed with pulmonary fibrosis as a rare complication from the chemotherapy and radiation treatment I received for breast cancer. When I was first diagnosed, I found so much information on the internet that was either inaccurate, confusing, or shared in truly terrifying ways. I also noticed there were very few resources available to help patients deal with the emotional turmoil of being diagnosed with a life-changing illness. It was overwhelming dealing with this new uninvited and unwanted reality, the painful emotions associated with being diagnosed with a chronic disease, and figuring out what to do from there on in.
I’ve learned a lot from my own experiences as well as from fellow patients. I hope it will be helpful for you to have practical information from a patient’s and counselor’s perspective, in addition to the knowledge and experience of a specialist in cardiovascular and pulmonary wellness and rehabilitation, all in one place. While doctors are our lifeline, and most of us would be lost without them, what many doctors may not know is what it’s like to actually be the patient, feeling alone, devastated, confused, and in the dark as we face a life-threatening or life-limiting illness.
I know your diagnosis turned your world upside down. Let us now walk this path together. Allow me to be your guide, resource, and friend, and I’ll help you become a good friend to yourself along the way. I will be real with you and talk about how to live your life to the fullest, as well as cover some tough topics we all find difficult to discuss. You’ll find answers to your questions, which I hope will be more meaningful because they come from someone in the trenches with you who truly understands what you’re going through. My hope is that your fears will be reduced, and you will find comfort in knowing you’re not alone.
What I share is grounded in my faith and my belief that God is good no matter what, and that He will guide us each and every step of the way in ways we can’t imagine. We’ll be real with the hard stuff while grabbing hold of the life we still have to live. Let’s walk together as we treat ourselves with compassion and kindness for the road ahead. I’m so glad we’re in this together.
As you read this book, you will notice we refer to “pulmonary fibrosis” or “PF.” Please note that we are also referring to all interstitial lung diseases, including idiopathic pulmonary fibrosis (IPF), nonspecific interstitial pneumonitis (NSIP), hypersensitivity pneumonitis (HP), and pulmonary fibrosis due to autoimmune or connective tissue disease (CTD-ILD), among many others.
There are few sensations in life that are as absolutely terrifying as not being able to catch your breath. In fact, most of us will do absolutely everything in our power to avoid that sensation at all costs, even at the expense of things we love, such as going to the theater, visiting with friends and family, even everyday activities such as taking a shower, grocery shopping, or even having sex. Shortness of breath can deliver a tremendous blow to our self-esteem as well as our overall quality of life.
My mission in joining with Kim to write this book is to teach you that for the overwhelming majority of people, being diagnosed with a chronic respiratory disease is not a death sentence and your situation does not have to be hopeless, nor should it be. In fact, there are many things you can do to minimize your shortness of breath as well as maximize your overall health and quality of life.
Our mission in writing this book is not to sell you a bill of goods promising a quick fix or cure for your disease or an overnight solution to your shortness of breath. You didn’t get here overnight. You’re not getting out overnight either. We are also not suggesting that managing your disease will be easy. It will not. I repeat, managing your pulmonary disease will not be easy. But if you follow the suggestions in this book, even some of the suggestions, some of the time, I promise you that you will begin to experience small (and in many cases, not so small) but noticeable improvements in your daily life, whether it be less shortness of breath, the ability to do more of the things you love, increased energy levels, or an improvement in your overall sense of well-being.
This is not something I think; this is something I know from firsthand experience because I have seen it work time and time again over the course of my 27-year career as a cardiopulmonary physical therapist, taking care of patients just like you or someone you love.
Many of the suggestions in this book will work for many, if not most, people reading it. However, it is important to understand that when it comes to patient care and people in general, all of us are different and, therefore, need to be treated as individuals. As such, there will always be exceptions to the rule that will require some adjustments or “tinkering” with the methodology.
Shortness of breath can range in severity from barely noticeable to all-encompassing. At times, you may not even be aware of your symptoms, while at others, they may stop you dead (or at least, completely breathless) in your tracks. Depending on where you are, what you happen to be doing, even whom you happen to be with at the time, you may attempt to minimize or make light of your symptoms. You may even tell yourself (and others) little white lies such as: “I’m getting old” or “I’m out of shape” or “it’s not a big deal.” But you know darn well it is. When I hear people use phrases like “it’s not a big deal,” I am reminded of that famous river in northeast Africa called “the Nile.” Denial, get it?
Our purpose in writing this book is not to give you a complex list of instructions or fancy protocols that are difficult to understand and virtually impossible to follow. Instead, my goal is to present you with a wealth of information my patients and I have found to be both successful and practical over the past 27 years.
Again, each person is different, and every situation is unique. Therefore, not all things will work for all people. However, my hope is that this information will help guide you and your healthcare team in determining which tools and techniques will work best for you and which ones won’t be as helpful, or not helpful at all.
As I mentioned earlier, there are few greater teachers than firsthand experience. I am proud to say that the overwhelming majority of what I have learned about pulmonary disease (and its most effective management) has come from my patients. I have been extremely fortunate to be exposed to great instructors and brilliant mentors throughout both my formal education and my professional career, but by far, it has been my patients who have been my most instrumental teachers.
What is pulmonary fibrosis?
Pulmonary fibrosis (PF) falls under a category of respiratory disease called interstitial lung disease (ILD). There are more than 200 different types of ILDs, of which PF is a primary characteristic. The most common type of ILD is idiopathic pulmonary fibrosis (IPF). The word idiopathic means that the cause is unknown. Approximately 30,000–40,000 new cases of IPF are diagnosed each year. Most IPF patients first start noticing symptoms between the ages of 50 and 70 years old, although some patients can be diagnosed much earlier.
PF can sometimes be caused by exposure to hazardous materials, such as breathing in asbestos, silica, or bird or animal droppings. Radiation treatments and certain types of medications can also cause PF. Sometimes PF can have a genetic component and runs in families, and other times it can be caused by autoimmune diseases such as rheumatoid arthritis, Sjogren’s syndrome, or scleroderma.
At its most basic level, the word pulmonary refers to the lungs and the respiratory system. The word fibrosis means scarring. Therefore, PF means scarring of the lungs, particularly the lung tissue itself, as opposed to obstructive diseases such as asthma or emphysema, which primarily affect the airways. PF is a restrictive disease, in which scar tissue can cause the lungs to become stiff, making it hard for you to take a breath and preventing oxygen from entering your bloodstream. Low oxygen levels (and the scar tissue itself) can cause you to feel short of breath, particularly during exercise or exertion such as climbing stairs or walking uphill (or sometimes, even on level surfaces), among other things.
Interstitial lung disease
The subject of interstitial lung disease is an enormously complex topic, even for clinicians and physicians, and even for many pulmonary specialists. So, it is no surprise that it is equally, if not infinitely more, difficult for patients and other laypeople.
When it comes to ILDs, there is so, so, so much confusion, misunderstanding, and misinformation out there, including online, in print, and in everyday life; so much so that before we go any further, I want to highlight three key points right here from the get-go, because failing to appreciate these concepts can literally mean the difference between life and death.
I will try to break it down as simply as possible, providing you only with the most important and relevant information and leaving out the things that you don’t really need to know or that will have little impact on your lives.
So, please, if you take nothing else away from or don’t understand anything else in this book, understand the following three points:
- Getting a definitive (correct) diagnosis is absolutely critical to your care. One of the reasons for this is that some of the treatments used to treat some ILDs have not only been proven ineffective, but they can actually be harmful in other ILDs, as in they make the disease worse.
- Early diagnosis and treatment are also critical, especially since some ILDs have a life expectancy of three to five years from time of diagnosis.
- Having the right team, especially a pulmonologist who is an expert in ILDs is also critical. These diseases are serious and cannot afford to be handled by amateurs. I would say “as serious as cancer,” but in many cases, an ILD can actually be worse. As such, only the best will do, and if you do not have access to physicians and other healthcare professionals that specialize in ILDs, then you need to travel to find one. Again, this can literally be the difference between life and death.
Please understand that I am not telling you these things to scare the bejesus out of you. Chances are, that’s already been done. I am telling you these things to impress upon you that ILD is nothing to play with, nothing to sleep on, and nothing to dabble with. You cannot afford not to take this seriously.
So, let’s begin.
And if you choose to stop reading here, “get thee to an ILD specialist, go.” – William Shakespeare
Being diagnosed with PF (or any chronic illness) is profoundly difficult on so many levels and in so many different ways. Being told you have a life-threatening or life-limiting illness leads to feelings of fear, confusion, grief, and a whole host of other difficult emotions. These are all in addition to the physical challenges of getting to doctors’ appointments, going through the various testing and other medical procedures, medications (and their side effects), adapting to worsening symptoms, and dealing with supplemental oxygen.
Our uncertain future causes anxiety, and we may struggle with varying degrees of support from family members, friends, and healthcare professionals. Getting solid information, taking care of yourself, and garnering the support you need will help make this journey a little easier. We will be covering all these topics in this book, and I pray they will be a big help to you.
Nothing in the world gives me greater satisfaction than helping people breathe better, to feel well instead of sick, and to truly live their lives instead of watching it pass them by from the sidelines. A big part of that involves clearing up confusion about their disease and dispelling myths and misinformation (of which there is a lot), an unfortunate but not uncommon byproduct of today’s fast-paced healthcare environment.
As Kim alluded to earlier, the internet can be one of the best sources of medical information, but in others, it can also be one of the most detrimental, not to mention, terrifying. For one thing, information found online is not always completely accurate or current. Often, it’s not even remotely accurate, and sometimes people discover far more than they ever wanted to know, often completely out of context and without a proper filter or guide to help them separate fact from fiction.
That’s what I’m here for. Kim and I are writing this book to help remedy that precise situation and help clear up any confusion you may have regarding your disease (or at least point you in the right direction). Yes, We Can is meant to be an infinitely and intimately useful guidebook covering the medical, behavioral, and lifestyle issues of not just living, but living well and thriving, despite your pulmonary condition.
I’m so thankful you have decided to read this book and to take the first steps in making your life better. This means more than simply not being short of breath or learning how to live with your disease and its associated symptoms. Instead, it means setting out on a path to truly becoming your best self and to achieving your own state of Ultimate Pulmonary Wellness.
Yes, We Can!
Next month: Getting the Right Diagnosis
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