Prolog: Joann’s Disease—The Reason for This Blog

Let me introduce you to Joann Gottlund, my wife. This blog exists because she is terminally ill and dying. Many people associate terminal illness with such diseases as cancer, heart disease, AIDS, Alzheimer’s and simple old age. These are obvious diseases because they are physically recognizable as, or immediately associated with the eventual end of life. What though, about the person who appears healthy yet may also be on the road to death? By the time anyone takes notice (including the sick one) or symptoms begin to present themselves, that person may already be beyond meaningful help, other than to cope with a constantly deteriorating "quality of life" and eventual incapacitation, then death.

Joann has emphysema, one of the less obvious terminal diseases, at least until you become really ill with it. Here is how WebMD describes emphysema:

Emphysema is a degenerative disease that usually develops after many years of assault on lung tissues from cigarette smoke or other toxins that pollute the air. These toxins destroy the small air sacs in the lungs, called alveoli, that stretch as they transport oxygen from the air to the blood and then shrink as they force out carbon dioxide. As a result, the lungs lose their elasticity, and exhaling becomes difficult as the damaged lungs trap air and cannot effectively exchange it with fresh air. As the damage progresses, the effort needed to breathe increases and, ultimately, each breath becomes labored.

Emphysema is one of a group of lung diseases referred to as chronic obstructive pulmonary disease (COPD) that can interfere with normal breathing. Other diseases that come under COPD include asthma and chronic bronchitis. According to the American Lung Association (ALA), nearly 16 million Americans are estimated to suffer from some form of COPD, and COPD is the fourth-ranking cause of death just behind heart ailments, cancers, and stroke.

—From "Every Breath You Take," WebMD.com, 2006.

Joann wasn’t a smoker, except for one year in her thirties. Her emphysema resulted from a history of childhood pneumonia, pneumonia so long in duration and frequency of attacks, that Joann would be in the progressive stages of her emphysema by the time she turned twenty-one. Luckily for her, Joann was otherwise healthy and because of her health wouldn’t notice the effects of her disease until she turned fifty. She might have felt more tired than usual in her forties, but she could attribute that to getting older. The real symptom came in the form of a heart attack when she was fifty. Emphysema, as it progresses, overworks your heart, placing strains on it that would otherwise not be there.

Subsequent testing revealed that she was also very anemic, had high cholesterol requiring an arterial stent and a severe case of gastroesophageal reflux disease (GERD). Over the next year Joann would continue to deteriorate, eventually to the point where she qualified for full disability, Medicare and Medical to pay her medical expenses. When she was fifty-one Joann had a second heart attack and four additional stents were implanted.

That was four years ago. Joann was awarded her Social Security Disability because the examining doctors knew she wouldn’t get any better. Joann was already unable to work as a registered nurse and things didn’t look as though they were going to get much better.

On Februaty 14^th of this year, Joann’s battle with taking a breath is ending. After several visits to the hospital over the last three years, Joann was finally sent home from Community Hospital of the Monterey Penninsula (CHOMP) with the updated diagnosis of "terminal stage" emphysema and COPD. The doctors said that there simply wasn’t anything they could do for her anymore. With only a couple of months left for her, Joann was admitted into the Visiting Nurses Association "Home Hospice" program. Joann’s simple request was that she be allowed to die at home, not in some strange place amongst people she didn’t know.

For myself, having Joann back at home wasn’t a big stretch. Physically, I would have some problems if she fell, I wouldn’t be able to pick her up, but I run the motel we live in so I’m always handy and can see to it she gets her medication. When I’m not handy, our good friend and fellow roommate Bill Walker oversees the cooking and most of the shopping. Joann came home to a nice, safe environment in an apartment the motel owners just finished building for us. (More on this stuff later in upcoming prologs.)

Joann’s disease is complicated by several things, mainly her anemia and heart condition. The sum of these disorders is considered Chronic Obstructive Pulmonary Disease or COPD. The National Emphysema Foundation (NEF) provides this overview:

Chronic Obstructive Pulmonary Disease (COPD) is a term that refers to a large group of lung diseases that can interfere with normal breathing. It is estimated that at least 11% of the U.S. population has COPD, and incidence is increasing.

Other factors have the potential of playing a role in the development of COPD. They are air pollution, occupational exposure to dust and chemicals, repeated lower respiratory tract infections, maternal smoking, poor nutrition, lower socioeconomic status, and prolonged untreated asthma.

—From "COPD - What is it?" The National Emphysema Foundation, emphysemafoundation.org, 2006.

In Joann’s case, her COPD is primarily the result of emphysema, though she does have other complications, such as chronic bronchitis. The NEF has this to say about the interaction of these two conditions and COPD itself:

Most patients with Chronic Bronchitis do not develop obstruction (COPD). The 15% that do will eventually develop thickened bronchial walls, increased secretions, cough and eventually cyanosis (lips and skin appear blue), swelling of the feet, and heart failure.

Emphysema is a chronic lung disease that affects the alveoli (air sacs) and/or the ends of the smallest bronchi (breathing tubes). The lung loses its elasticity (similar to an overused rubber band), and therefore these areas of the lungs become enlarged. These enlarged areas trap "stale" air and do not effectively exchange with fresh air. This results in difficulty with breathing and may result in insufficient oxygen being delivered to the blood. In some cases, the patients also have difficulty getting rid of a waste gas called carbon dioxide. The predominant symptom in patients with emphysema is shortness of breath.

NOTE: While some patients with COPD have only chronic bronchitis or emphysema, most have some combination of both.

The causes of COPD are not fully understood. It is generally agreed that the most important cause of chronic bronchitis and emphysema is cigarette smoking. (Keeping in mind that Joann was not a smoker and her disease originated from repeated bouts of pneumonia.)

Symptoms (What the patients feel)

Unfortunately, patients may be symptom-free until they have lost 50% of their lung function! The first sign of COPD may be shortness of breath on vigorous exercise that may be ignored as an expected result of smoking. Patients with predominantly chronic bronchitis usually have cough and sputum for many years before they develop shortness of breath. Patients with predominantly emphysema usually have shortness of breath and develop cough and sputum during respiratory infection or later stages of the illness.

Signs (What the Doctor finds on examination)

Chronic Bronchitis: Initially the patient looks normal; however, as time passes the lips and skin may appear blue (cyanosis). There may be abnormal lung sounds, swelling of the feet, and heart failure.

Emphysema: Initially the patients do not appear blue at rest but eventually appear underweight and visibly short of breath. The chest may increase in size from front to back (the so-called barrel chest), and lung sounds may be diminished. Weight loss occurs in more severe cases. Swelling of the lower extremities is a sign of heart failure, also found in later stages of COPD

—From "COPD - What is it?" The National Emphysema Foundation, emphysemafoundation.org, 2006.

As of this date, Joann exhibits all the signs of "final-stage" emphysema with bouts of chronic bronchitis. Her feet are starting to swell because of reduced cardiac function, she weighs ninety pounds, she must constantly use about 3.5 times the normal amount of oxygen, she has great pain because the muscles in her diaphragm and chest are so overworked from labored breathing. All of her medications were changed from "recovery" to "comfort." Morphine is her main medication now, with large amounts of stool softeners. In a way, the new regimen is easier to deal with, she has gone from 21 medications to about seven, though I have to keep a log and administer the liquid morphine because she gets confused from all the drugs. My mindset has had to change also, but that’s another blog entry.

There is no way of knowing how much time she has left. The criterion for hospice treatment is that the patient has less than six months to live. It’s been three weeks since Joann came home and she is already a lot weaker. Yet, her spirits remain high and she assures me that its probably worse for those of us around her than it is for her.

I hope that’s true.