This "sheet" prevents enough oxygen from entering the lungs when
doing everyday tasks, no matter how mindless or routine.
Undertakings such as bathing and drying off, dressing or taking the
trash out become arduous, she says.
"The smallest amount of physical exertion tires you out like
you're hiking," she said.
Even eating carbohydrates may produce too much carbon dioxide for
a COPD sufferer to blow off, said physician Bill Chang, a pulmonary
critical care fellow at both Jerry L. Pettis Memorial Veterans
Medical Center in Loma Linda and Loma Linda University Medical
Center.
And in what seemed like an endless list: Hair spray, spices,
cleaning products, dust, et cetera, anything you can smell is a
potential irritant, Anderson said.
Anderson wakes up around 5 a.m. to prepare for school since she
just can't rush, period.
COPD is the fourth leading cause of death in the United States
and is a primary reason people go on disability.
But Anderson's been teaching nearly 36 years, 23 at Alta Loma,
and it provides her a necessary diversion from fear.
The basics
COPD is an umbrella term for lung diseases associated with
airflow obstruction, usually chronic bronchitis, chronic
irreversible asthma or emphysema.
COPD International says there's 16 million people living with the
disease now, and an estimated 14 million people who haven't been
diagnosed due to few or no symptoms.
"While the mortality rates of some other diseases have improved
over the years, Chronic Obstructive Pulmonary Disease's rate has
risen or hasn't really changed. We've slowed the COPD down, but not
controlled it," Chang said.
Despite COPD's critical nature, only 23 states have adopted
November as COPD Awareness Month, and California's not one of them.
The largest risk factor for the development of COPD is smoking,
but Jill Anderson's never lit up.
Anderson's COPD is caused by a hereditary condition in which the
body circulates a decreased amount of the serum protein Alpha-1
Antitrypsin due to the production of mutated Alpha-1 Antitrypsin,
Chang said.
In a healthy body, AAT, also called alpha-1 proteinase inhibitor,
circulates in the blood, protecting the lungs and body tissues from
dangerous enzymes in white blood cells.
AAT, synthesized in the liver, counters these enzymes and is
capable of increases in concentration in blood and tissues when
temporary infections occur.
When abnormal Alpha-1 Antitrypsin is produced, the liver can't
secrete it. The accumulating bad AAT can lead to liver and skin
disorders.
In the lungs, said Chang, 32, low levels of circulating AAT allow
the harmful enzymes to break down the alveolar region of the lungs
where oxygen and carbon monoxide are exchanged, leading to
emphysema.
At the time of her diagnosis in fall 2003, Anderson's inherited
Alpha-1 Antitrypsin Deficiency was causing her lung functioning to
break down irreversibly at a rate of 2 percent per month.
Early diagnosis key
When Anderson sees someone smoking in a car with a child in the
back seat, it drives her crazy.
"If I had ever smoked, I'd already be dead, so it's awful to see
someone subjecting their child to that, knowing (the child) hasn't
been tested for Alpha-1," Anderson said.
In her early 50s, she started noticing and going to the doctor
for symptoms: breathlessness, or having trouble getting a good
breath, unexplained coughing and a cough in the fall that wouldn't
go away.
Trouble is, early 50s is late.
Looking back 30 years, there may have been warning signs Anderson
interpreted then as just being out shape. She remembers a time she
was winded following her son, then 3, around with a video camera.
"You could hear me breathing and huffing on the video as I
attempted to narrate," Anderson said.
Her childhood asthma and bouts with bronchitis may have been
indicative of Alpha-1 Antitrypsin Deficiency, "but Alpha-1 usually
affects the liver first," Chang said.
And the lungs later.
By the time serious symptoms develop, lung capacity is already
reduced by 50 percent in some patients, according to Emphysema
Foundation For Our Right To Survive, or EFFORTS.
In August 2000, at her first spirometry check -- a simple breath
test used for the early detection of many lung disorders --
Anderson's lung capacity was at 47 percent, 18 percent higher than
her lung capacity now.
More than three years later, after more spirometry tests, chest
X-rays and being told her COPD was due to breathing in smog or too
much exposure to her father's smoking, Anderson's primary care
physician did a simple Alpha-1 Antitrypsin blood test. Normal is
150, but 50 or above is OK, too. Anderson's was 22.
Staying alive
"I wake up a lot to make sure I'm still here."
Anderson hasn't slept through the night more than three times
since being diagnosed.
She abandoned her bedroom a long time ago for the greater comfort
of her fluffy, L-shaped couch. The furniture's arms and pillows
support her breathing efforts better than a bed does. At best,
Anderson sleeps six hours. Oxygen levels decrease in a COPD or
Alpha-1 patient's sleep; this reduction can cause damage to the
brain and other organs if not corrected.
She uses a pulse oximeter, a small device that measures her
blood-oxygen level. When her numbers dip, she hooks up to a
supplemental oxygen cylinder by Apria that's delivered to her home
and refilled regularly.
In addition to daily inhalers and the supplemental oxygen,
Anderson has augmentation therapy, weekly infusions of Prolastin,
Alpha-1 Proteinase Inhibitor (Human), to increase the AAT protein
levels circulating in her blood.
For a year and a half, every Wednesday for about an hour,
Prolastin -- prepared from pooled human plasma of normal donors --
is administered to Anderson intravenously and cost-free.
"Right now my insurance covers everything, but I worry about when
Medicare kicks in at 65, if they'll cover it," Anderson said.
She's still weighing the cost and benefit of another option, a
lung transplant, and she's not on the waiting list yet.
COPD awareness
Quickly after Anderson's diagnosis, Chaffey Joint Union High
School District purchased an audio enhancer for Anderson. Worn
around her neck, it projects Anderson's unraised voice out of four
speakers into her classroom.
Riley Bendel, 15, of the Alta Loma area of Rancho Cucamonga, can
hear her with the enhancer on.
"Mrs. Anderson told the whole class on the first day that she has
a problem with her lungs that will make her out of breath, that she
inherited it from her genes and not from smoking," Riley said.
She teaches her class "To Kill a Mockingbird" . . . and to just
say no.
Nov. 18 was World COPD Day 2005 organized around the theme,
Breathless Not Helpless!, and as established by President Bush in
2001, this is COPD Awareness Month.
"It's crucial to bring attention to this, and encourage people to
get spirometry tests now, before they have symptoms," Anderson said.
EFFORTS is urging medical professionals to include a spirometry
check in annual physicals and "if you've been diagnosed with early
onset emphysema and you're under 45, it's a good idea to get an
Alpha-1 Antitrypsin Deficiency test and stop smoking," Chang said.
He also recommends that couples considering pregnancy get
screened for the Alpha-1 Antitrypsin Deficiency genes.
And the Alpha-1 Foundation along with the Medical University of
South Carolina sponsors a free and confidential ATT Deficiency test
with a kit that can be sent to the home.
"The biggest enemy is a lack of information," Anderson said.
RESPIRATORY SYMPTOMS - COPD,
ALPHA-1 ANTITRYPSIN DEFICIENCY
Shortness of breath while at rest or with exercise
Wheezing, persistent cough
Recurrent lung infections
Persistent sputum (or phlegm) production
History of suspected allergies and/or asthma
Sinus infections
Source: Alpha-1 Foundation
FOR MORE INFORMATION
Alpha-1 Foundation: www.alphaone.org
Emphysema Foundation For Our Right To Survive:
www.emphysema.net
Global Initiative for Chronic Obstructive Lung Disease:
www.goldcopd.org
To become an organ donor: www.shareyourlife.org,
donatelifecalifornia.org
Mari Nicholson may be reached at mari.nicholson@sbsun.com, (909)
483-8549 or in care of The Sun, 2239 Gannett Parkway, San
Bernardino, CA 92407.
