I HAVE JUST BEEN DIAGNOSED WITH COPD – NOW WHAT?? By Uncle Jim & Aunt Mary

I am not a physician. Not even close. What I am is a former lung disease patient. I can say “former”, because on December 12, 2011, I was blessed with the miracle of a double lung transplant. At age 71, I received the lungs of a 32-year-old marathon runner! I was incredibly lucky to receive lungs that were a perfect match for my body, and I have had virtually no complications. I take about 30 pills a day, and my immune system is compromised due to the anti-rejection drugs, but my life is as normal as can be.

I was born a month early, I grew up enveloped in my dad’s cigarette smoke, worked with agricultural chemicals, and began smoking myself at age 15. I quit at 35, but the damage had begun. I was diagnosed with severe COPD (Chronic Obstructive Pulmonary Disease) at age 55. My tests on that day showed that I only had about 33% of the lung function that I should have had, given my age, weight, etc. Since then, Mary, my life, my love, and my caregiver and I have worked with the American Lung Association, the COPD Foundation, EFFORTS, the international online support group, with several Facebook support groups, and with Pulmonary Horizons, an organization that holds annual conferences of some of the best minds in the pulmonary treatment field. We have taught classes and lectured support groups on living well with COPD. We have written many articles for the various organizations, and have been featured in several magazine articles. We are known as Uncle Jim and Aunt Mary. We have spoken in San Diego, Washington, Miami, Chicago, and London, England. We have participated in a workshop in Bethesda, MD at the National Institute of Health to help develop a master plan for the treatment of lung disease.

Just so you know that I am not your average yahoo…. Now let’s talk about you…

You’ve gotten used to the respiratory infections. They have been going as long as you can remember. They were periodically laying you low early on, even before you started smoking in High School. Maybe you were born a few weeks early, before your lungs had a chance to really develop, to be able to resist the insults of the outside world.

Did your folks smoke? If so, don’t blame them! They grew up in a world where the vast majority of people smoked something, usually cigarettes. There were ads everywhere featuring guys in white doctor’s coats with a cigarette in their hand, extolling the virtues of Camels or Chesterfields, or, if your throat was a little raw, Kool’s Menthol. Then, later, the era of Virginia Slims created a whole new demographic of smokers. Women were gaining equality in many ways, including the right to smoke.

If the smoke that was inhaled and blown back out was so harmless that such luminaries as Ronald Reagan advertised them, that smoke shouldn’t be harmful to others, the kids and such who were wandering around, should it? The term “second-hand smoke” had yet to be invented, and “third-hand smoke” was far in the future. Even when the terrible toll brought on by smoking finally came to light, it was still some time before the realization that inhaling the smoke from the mouths and ashtrays of smokers was nearly as deadly. Then, the concept of third-hand smoke, the toxic chemicals that permeate the carpet, the couch, the curtains, the car upholstery and the clothing and hair of the smoker hit the smoking community hard. The realization that the grandchildren are sitting on a chair that is soaked in toxins is a difficult pill to swallow.

There are many excuses for lungs that are sensitive to infections. When we are young and otherwise healthy, the illnesses come and go within a few days or a week, but they leave us just a little bit weaker, just a little bit more susceptible to the next attack. There are usually readily available excuses for weak lungs. Premature birth, second and third-hand smoke, agricultural and industrial chemicals, and, of course, personal smoking. The majority of people who develop lung problems in later life have a history of smoking for a few years, or for most of their life. However, about 1 in 5 adults with lung disease never smoked! In such cases, they may have a genetic disorder called Alpha-One Antitrypsin Deficiency, or simply Alpha-1. The genetic lack leaves the patient with a severe susceptibility to lung disease. It is important for lung disease patients to undergo a simple blood test for Alpha-1.

So, here you are. As the years went by, you found that you were coughing more, that your lungs seemed to be producing excess mucus, that you were experiencing more shortness of breath when you exerted yourself. Trouble was, it happened so very slowly, so gradually that you really did not notice the difference. Sure, you knew that you were slowing down, that you were seeking activities that required a little less effort, but after all, you were getting older, right? Plus, you were a little out of shape, and you had gained a few pounds! In addition, you were still getting the old familiar chest infections, except that they seemed to be getting a little more severe as the years went by.

A fairly sudden onset of an infection, the sudden worsening of a condition such as a breathing disorder, is known as an “exacerbation” or a “flare-up”. The severity of the hit, the condition of the patient going into the episode, and the care received and compliance of the patient in following doctor’s orders, will all help to determine the length and severity of the exacerbation. However, and this is a really big however, each time we are knocked down by one of these periods of nastiness, the harder it is to regain our former level of strength, of stamina. The strength and resilience of youth will carry us along for quite a few years, will allow us to recover to nearly the condition we were in prior to the flare-up.

Then, as we progress through the years, we tend to lose that resilience. The usual scenario involves heavy coughing, severe breathlessness, and chest pain that finally drives us to the Emergency Room. After the initial diagnosis of pneumonia, the next step is normally a pulmonary function test. Blowing into the tube as hard and fast as you can will give the doctor a score that is known as FEV1, or Forced Expiratory Volume in one second. There are many other tests that may be administered, but the FEV1 is paramount in the diagnosis of COPD, or Chronic Obstructive Pulmonary Disease. The lower the score, the more severe the diagnosis.

COPD is most commonly a combination of emphysema and chronic bronchitis. It may be primarily one or the other, but it is normally both. Emphysema effects the microscopic air sacs where the oxygen is absorbed into the bloodstream and the CO2 (carbon dioxide) is transferred to the lungs to be exhaled. In healthy lungs, the air sacs, called alveoli, are like tiny balloons. They inflate when you inhale, and then the elasticity pushes the waste air back out. Consequently, with good lungs, the work of breathing is inhaling. Once the lungs are full, we are able to relax and let the alveoli expel the air. When emphysema strikes, the alveoli begin to break down. The sacs develop holes in their walls, which enlarge as time goes on. In addition, they lose their elasticity. Thus, there is less “wall space” available for the absorption of oxygen and the elimination of CO2, and the lack of elasticity makes it much more difficult to get the “bad air” out of the lungs.

Chronic bronchitis effects the tubes that carry air from the nose or mouth to the microscopic vessels that feed the alveoli. Obviously, anything that blocks or impedes that air flow is going to affect the quantity of air that can be inhaled or exhaled. That blockage may take the form of mucus, inflammation, or swelling. The more advanced the bronchitis, the greater the blockage, and the less air that is available to the alveoli. Advanced bronchitis, in combination with severe emphysema, quite obviously makes it very hard to breathe.

That’s the bad news. Well, that’s not totally true. If you indeed are diagnosed with COPD, you must come to the realization that both types of lung disorder that we have been discussing are chronic and progressive. Chronic means that it is not going to go away. Progressive means exactly that…it will get worse as time passes. There! That is the really bad news. Sorry to lay that on you, but you deserve to know what you are facing.

Ready for some good news? There is a LOT that you can do to treat the chronic bronchitis. There are inhalers and nebulizers to relax and open the bronchial tubes, to loosen and thin the mucus. There is also available treatment for emphysema, but it involves supplemental oxygen, another whole subject that we will discuss in a bit.

More good news! There is also a lot that you can do to delay the progression of COPD. One of the advantages, if you can bring yourself to call it that, of a diagnosis if COPD is that the progression is normally very, very slow. That is one of the problems with the disease, and is the reason that the diagnosis so often comes late in life. It takes so many years for the disease to drive the average patient to that ER and the diagnosis that it is normally in its late stages and more difficult to treat. As we mentioned before, it is very easy to blame age, weight, or deconditioning for the fatigue, the shortness of breath. Sadly, many General Practitioners, ER Docs, and support personnel are not nearly as familiar with COPD, or the testing for it, as they should be. The spirometer, the instrument that you blow into to determine your diagnosis and the stage of your disease, is sorely lacking in most medical offices. You may be prescribed an inhaler to open your bronchial tubes, but you will likely learn nothing about the underlying ailments.

We spoke of exacerbations, the periods of nastiness that drive you to your recliner, your bed, or the ER. The worse the flare-up, the longer it lasts, the further it will knock you down. Once you are down there, you will find that it is nearly impossible to regain your former strength and endurance. It is possible, it has been done, but few have the sheer determination to do it. So, in most cases, your conditioning, your strength, drops a step. It might be becoming obvious to you by this time, but each of those steps lead downwards. The more exacerbations, the more steps, and the quicker you will lose ground. The older you are, the harder it is to recoup your former muscle tone, your previous endurance.

So, it actually comes down to a matter of choice on your part. When you get your diagnosis, you can choose to go home, sit down, do some serious whining, and wait to die. That is a choice. Or, you can set out to learn as much as you can about the disease, seek expert advice from whatever medical types that you can find, and do whatever is necessary to keep yourself in the best possible shape. That involves diet, lifestyle, and above all else, exercise! By making the effort to stay strong, you will maintain the resilience necessary to resist the infections, the pneumonias, the exacerbations. I cannot emphasize enough that those efforts will keep the downward steps far apart. The further apart the steps, the longer it will take for you to lose the ability to live a life of good quality, to remain active. It will allow you to live longer.

Just to prove the point, one of the first things that I did after my diagnosis was to order my first treadmill. I put a lot of miles on that thing, but it and the other exercise equipment that I acquired along the way allowed me to stay at that 33% lung capacity for almost 10 years! It was exercise that kept me strong enough to truly enjoy that 10 years, to maintain a great quality of life. It was exercise that allowed me to make it through the next 5 years, although my lungs were deteriorating. It was exercise that helped me to qualify for the transplant, to make it through the operation in great shape, and to recover quickly. It is exercise that now allows us to travel and speak and help others to live well.

Some days, it is easy. Some days it is not. But, it is worth it. Had I not spent all of those hours, walked all of those miles, I am quite sure that I would be dead now.

Again, it is your choice…I made mine many years ago, and it has made all the difference.

Sometimes, during an exacerbation, you will be prescribed supplemental oxygen. As we mentioned, oxygen is the only available treatment for emphysema. Admittedly, no one that I have ever met enjoys wearing an oxygen cannula. There is the embarrassment, the feelings of shame, the bruising of one’s ego, not to mention the logistical nightmare of stocking, gathering, carrying, and keeping track of all of the stuff involved in the transferring of oxygen from a bottle or a concentrator to your poor, belaboured alveoli. Thing is, supplemental oxygen is in fact the only effective treatment for emphysema. Once you have reached that stage, the only way that your poor, tattered alveoli can absorb enough oxygen to keep you healthy is with the oxygen equipment.

There is a small device called a pulse oximeter that fits on your fingertip and measures not only your pulse rate but also the percentage of oxygen saturation in your blood. It is commonly recommended to keep that saturation above 90%. The higher, the better. If you are on oxygen, check with your medical team to see if they will approve turning the flow up so that you can maintain at least that level under all circumstances. A level of 90 is good. A level in the high 90s is better.

Usually, the use of supplemental oxygen begins with a period, perhaps a long period, of using it while sleeping and while exerting yourself through exercise or other activity. It is certainly possible to get along without it, to just stop and rest when you run short of breath, but that independence comes with a cost. Again over time, a lack of proper oxygen saturation in the blood can result in damage to the right side of the heart, the side that pumps blood into the lungs. The harder it has to work, the more it tends to enlarge and weaken. Eventually, it can result in congestive heart failure, swelling of extremities, and death.

In addition, low oxygen levels destroy brain cells! If you don’t mind a gradual loss of brain function, forgetting how to use the TV remote or your grandchildren’s names, then go right ahead and let yourself run low on oxygen. However, if you want to retain your ability to reason, to remember, then do whatever is necessary to keep a proper level of oxygen in your blood.

If you fear that your family or friends will look upon you askance if you appear in public wearing an oxygen cannula, I have a handy method of enlightening them. Ask them to take a deep breath and hold it in. Then, while they are holding that breath, ask them to try to inhale. They will likely say something like, “There isn’t room to breathe!” You can then explain to them that the feeling they have just experienced is very like what you go through. Another method is to have them hold their nose shut and breathe through a straw while trying to walk in place. If they don’t understand after that, there is no hope for them.

Another result of refusing to wear your oxygen equipment outside of your home is the harm that you are doing to the entire COPD community. There are over 15 million Americans who have been diagnosed with COPD. It is estimated that, because of the slow progression of the disease, there are at least another 15 million or so who have the disease but who have yet to be diagnosed. Given those numbers, you would assume that at least several million of us would have been prescribed supplemental oxygen. However, it is actually somewhat unusual to see anyone out shopping, eating, or whatever while wearing a cannula. One of the symptoms of COPD is actually social isolation! We tend to hide in our homes, to become invisible to the rest of the world, rather than endure the occasional stare from some random stranger.

So, by hiding, by struggling to breathe if we do go out rather than doing what we should, we are not only harming our own bodies and brains, we are giving the totally wrong impression to the rest of the world, especially people like Congresspersons who make decisions about such things as funding for COPD research. Compared to such things as HIV/AIDS, the funding dollars for COPD research is absolutely pathetic! Why? Awareness! We have talked to many members of Congress or their aides over the past few years, and it is rare to find anyone who knows anything about our disease unless they happen to have a family member with the disease. So, hide if you will, but be aware that you are hurting yourself and many others.

I sincerely urge you to put on the cannula, gather the tanks or portable concentrator, and get out of the house! I assure you that about one person out of a hundred will even notice that you are wearing it! People have their own problems, their own issues. People will open doors for you, offer to carry stuff for you! If some idiot does stare at you, just look them in the eye and give them your biggest smile! If it seems appropriate, use it as a teaching experience, especially with young people. Just might keep them from smoking…

A last bit of advice… There are surgical procedures that can, in some cases, help those with COPD. If a patient’s emphysema is confined to one lobe of a lung, LVRS (Lung Volume Reduction Surgery) can remove the damaged portion and leave more room for the healthy tissue to process oxygen. Lung transplantation, either single or double, is major surgery and requires a great deal of testing to qualify, but it is the only real “cure” for COPD. It has been described as trading one disease for another, in that the anti-rejection drugs compromise the immune system. The immune system helps to ward off such things as skin cancer and lymphoma, as well as germ-related illnesses, so there is a real-trade-off. However, it has earned me almost 5 additional years of life, so I am a fan.

There are trials going on that involve the implantation of such things as one-way valves that help to reduce trapped air in the lungs, again giving the patient more room to breathe. Several institutions are involved in research in the field of stem-cell therapy, the utilization of stem cells removed from the patient’s body to regenerate lung tissue. In talking to physicians and technicians at several of these research facilities, I found that the consensus is that they are at least 10 to 15 years away from human trials.

You have likely seen advertisements for clinics that are selling “stem cell therapy”. They claim that they can perform the stem cell transplantation that the experts say is far in the future. They charge several thousand dollars for their treatments, they are not approved by the FDA, and despite the testimonials that they publish, such organizations as the American Lung Association and the COPD Foundation have issued statements denouncing their services.

So, what have we learned? Take care of yourself! Eat right, avoid anyone who smokes, take your meds when you should, and exercise! You are the only one who can do it! I am living proof that the dreaded exacerbations can be, if not eliminated, at least delayed. Remember, we need to keep those steps downward as far apart as we can.

This has been a brief brain-dump of some of the things that Mary and I have tried to impress upon patients and caregivers over the years of classes and lectures. We have been told by those wonderful people that we have changed, or in a couple of cases, saved their lives. We have been called inspirational! That’s pretty cool!

We have learned a lot over the years, but we certainly don’t know everything! I hope that this article has answered some of your questions, but I urge you to find a good doctor, preferably a pulmonologist, and pick their brains. Since the time of a physician is limited, please learn as much as you can about your disease. The doctor will appreciate it, the two of you will be able to communicate a lot better, and you will understand his explanations on a much greater level.

All the very best,
Uncle Jim & Aunt Mary