An Alpha-1 COPD Love Story

Sally Everett recounts struggle with Alpha-1 COPD in new book

Published: Wednesday, January 25, 2012, 6:07 AM

 By Christine White 

Photo by Cory WhiteSally Everett at her home in Longmeadow. Everett has written a book about her battle with chronic, genetic lung disease.

LONGMEADOW - In 1993, Sally Everett was 50, a single mother, living in Nyack, N.Y., and working as regional director of the Medicaid fraud unit in the attorney general’s office, when a bout of pneumonia led to a devastating diagnosis. 

Doctors told her she had Alpha-1 COPD, a genetic pulmonary disease. They gave her 10 years, at best, to live. 

Nineteen years later, and now living at Glenmeadow with her husband, Dr. Gordon L. Snider, Everett has written a memoir - “An Alpha-1 COPD Love Story,” published by Small Batch Books. It is about her journey from health to illness, the love she encountered along the way, and her fight to raise money and awareness to battle this deadly disease. 

“COPD (Chronic obstructive pulmonary disease) is the third greatest cause of death in the U.S., after heart disease and cancer,” Everett said recently, “and yet the Centers for Disease Control doesn’t have a budget for it. Every four minutes someone dies of COPD.” 

Everett is working to change that, but she didn’t start out embracing her illness or championing its cause. 

“After my diagnosis, I gave myself one week to feel sorry for myself, and then I just got on with life,” she said - with one exception. “I threw caution to the wind. I gave myself permission to be extravagant for one whole year.” 

But when the year ended, it was time to confront her condition. She joined a church and its support group for those with challenging illnesses. 

“It enabled me to open up,” she said. 

She was also ready to meet and embrace others with Alpha-1 COPD and in 1994 joined their association. 

“I found strong people there, with a good attitude,” she explained. “Many of them are still good friends.” 

Then in the late 1990s, she was elected to the board of the Alpha-1 Foundation. Unlike the Alpha-1 Association, which serves as a support group and information distributor, the foundation is designed to raise money for research. 

Also elected to the board that year, as science adviser, was Dr. Snider, a renowned pulmonologist. They became good friends. Everett didn’t realize how good. 

In 2001, he called her from his home in Boston and told her that he loved her and wanted to start a relationship. 

“You could have picked me up off the floor,” Everett said. The only response she could think to give him was, ‘Can I get back to you in a couple of weeks?’” 

A year later, she moved to Boston to be with him and in 2006, they married. That same year, they moved to Longmeadow. Oddly enough, their roles have in some ways reversed. She was the one with a critical diagnosis when they met. Since then, Snider has suffered a stroke and has battled lymphoma. But they have a philosophy they live by: “Life’s still worth living if it beats the alternative.” 

Alpha-1 is a deficiency in a protein, manufactured primarily in the liver, that protects the lungs. It can affect both the liver and lungs, leading to COPD in adults and liver disease in newborns, children and adults. 

COPD is often called “Smokers Disease,” but new research points to a genetic predisposition in those diagnosed with it. That’s why only about 20 percent of smokers get it. According to Everett, awareness of this condition needs to be raised, since even many primary care physicians are unfamiliar with its genetic aspect. 

“Both liver doctors and pulmonologists know about Alpha-1 and know to test right away for the genetic component when they encounter liver disease or COPD, but among family doctors and internists, it’s not so well known.” she said. 

Clinical trials are now under way in New York to test all newborns for the genetic disorder. While there’s nothing that can be done to cure the problem, short of a transplant, anyone with a predisposition would be warned to protect themselves from risk factors such as smoking, and environmental and industrial pollutants. 

It took Everett 2 1/2 years to write and publish the book. 

“I started out writing the book for my grandchildren,” she said. “I wanted them to know they always have choices. It may not be the choices you wish you had, but you always have choices. Then I realized that I could help others, as well. Stuff happens in life that we can’t predict. It’s what we do when it does that defines us.” 

The book is available at www.amazon.com or www.barnesandnoble.com. Half the profits from the sale of the book will support research into Alpha-1 and COPD. 

© 2012 masslive.com. All rights reserved.

ANOTHER ALPHA ANGEL

                                  “FOREVER ON THE UPSWING”

My friend Greg Hules died this morning. For those Alphas (persons with Alpha-1 antitrypsin deficiency) lucky enough to have known him, he was a one-of-a-kind.  For those who weren’t so lucky, Greg figured prominently in the last chapter my book, An Alpha-1 COPD Love Story. Here’s part of what I wrote about him.

            By 1988, Alpha-1 emphysema had severely compromised Greg, then age 38.  As a result, he joined a pulmonary rehabilitation course and then one thing led to another.  By 1990, he had formed an Alpha-1 support group in San Francisco and then his group joined with another in Minnesota to form the Alpha-1 National Association.  As sick as he was, Greg agreed to serve on the Executive Committee of the Association and later became its President.

             Greg continued to serve the Association right up to his double lung transplant in 1997.  Following his transplant, Greg first re-established himself as an entrepreneur.  Later, he joined the board of the Alpha-1 Foundation and, from 2003 to 2004, served as its Chair.  He finally rotated off in June 2011, after nine more years of service to the Alpha-1 community.

            Greg wrote a piece for the Foundation’s Ten Year Retrospective.  Its ending gives you the flavor of Greg better than I ever could.  “My story began in the late ‘80’s.  It is filled with memories of people who have made a difference . . . And tomorrow’s promise makes me believe our story has just begun.  To those who have served in the past, to those that serve today, and to those who will join with us to make a difference tomorrow, I thank you.  I wouldn’t have missed it for the world!”

Greg was a Giant within our Alpha-1 community. We will all miss his can-do attitude and warm smile. Greg was also a wonderful husband to Sandi and a devoted father to their two boys. I will always remember Greg, however, as “forever on the upswing.”

Greg’s health began to seriously decline over the last couple of years. But from talking to Greg, you never would have known it.  Whenever I asked Greg how he was feeling, he’d invariably reply, “Well, I’ve just been through a little rough patch, but now I’m on the upswing.” Whenever I begin to feel sorry for myself now, all I have to do is think of Greg. Then, as if by magic, a smile creeps back onto my face.

We have a wonderful tradition within our Alpha-1 community of honoring all those who have lost their battle with Alpha-1. We call them our “Alpha Angels.” Now Greg is one of our Alpha Angels. I know he is watching over all of us who loved him. 

The Real Silent Killer

 I have Alpha-1 COPD.  You don’t know what that is?  It’s chronic obstructive pulmonary disease caused by Alpha-1 antitrypsin deficiency.  That didn’t help?  I’m not surprised.  Even though Alpha-1, the major genetic risk factor for COPD, is more common than cystic fibrosis, most people have never heard of it.  Likewise, COPD is now the third greatest cause of death in this country, but we seldom read anything about it.  Search the New York Times website like I did and you won’t find a single story focusing on COPD since November 2007.  Just this past year, however, you’ll find plenty of stories focusing on Parkinson’s disease, Breast Cancer, Prostate Cancer, Alzheimer’s, Diabetes, and HIV even though COPD kills far more people in this country than any of these other diseases. 

Federal funding for COPD research is likewise disproportionate to its impact. In fact, the Centers For Disease Control doesn’t even have a budget for COPD.  As for the National Institute’s for Health (NIH), the Fair Allocations in Research Foundation (FAIR) takes NIH’s annual estimate of its support by disease and divides that by the number of deaths from each such disease to calculate its support per death.  By FAIR’s calculations, in 2012 NIH plans to spend only $8 per death for COPD, as against $42 per death for diabetes, $86 per death for Alzheimer’s, $157 for Parkinson’s disease, $177 for Prostate Cancer, $3,047 for HIV/AIDS and $3,721 for Breast Cancer.

Goggle “silent killer” and you will come up with heart disease, diabetes, and hypertension, but not COPD.  That’s not surprising.  The Real Silent Killer is the one you never read about.  A recent Canadian study estimated that one in four people over age 35 would likely develop COPD.  I’d say that was big news.  But it wasn’t reported in the New York Times.  Or how about the recent news that COPD is now the third greatest killer in the United States?  That wasn’t reported in the Times either.  I don’t mean to pick on the Times.  I’m sure the reporting on COPD is no better in any other major newspaper in this country.  But the Times is my newspaper of choice.  I depend on it.  So the question is why haven’t there been more stories about COPD? 

Perhaps it’s the outdated stigma that people with COPD are smokers.  In other words, we did it to ourselves. Yet according to the National Cancer Institute, of the 440,000 premature, preventable deaths each year in this country from exposure to cigarette smoke, 75 percent are from cancer, heart disease and stroke and only 25 percent are from COPD. Perhaps, instead, the mere image of those of us who must carry oxygen everywhere we go makes readers uncomfortable.  Alternatively, perhaps it’s the sound of our portable oxygen equipment that disturbs them. 

Ironically, the Times did carry one COPD-related story this past year written by one of its music critics.  Instead of reporting on the concert he’d just attended, however, James Oestriech focused instead on the “relentless rhythm” of a portable oxygen device that, he said, had thoroughly spoiled his enjoyment of the music.  He then expounded on whether the rights of persons with medical devices trumped his rights as a concertgoer and critic.  Finally, he advised readers who might find themselves in a similar situation that, if they complained, an usher would move the offender and his device.  To its credit, the Times then published a rebuttal from John Walsh, President of the COPD Foundation.

Current estimates are that there are twenty-four million of us with COPD in this country. Tragically, only twelve million of us have been diagnosed to date.  If and when the rest of us are, it could be too late.  COPD is preventable and treatable, especially if diagnosed early enough.  To their credit, the COPD Foundation and the National Heart and Blood Institute have each launched awareness campaigns.  Recently pharmaceutical companies have also subjected those of us already diagnosed with COPD to an onslaught of TV ads.  But we need a national conversation about COPD focusing on the major risk factors and the importance of early testing.  Who better to initiate this conversation than my favorite newspaper, the New York Times? 

Why Cure COPD?

Hello.  My name is Sally Everett and I have Alpha-1 COPD.  That’s chronic obstructive pulmonary disease (COPD) caused by a genetic abnormality called Alpha-1 antitrypsin deficiency (Alpha-1).  I was diagnosed nineteen years ago.  Today I’m on oxygen 24/7.  But, as my husband would say, life’s definitely still worth living because it beats the alternative hands down.

When I was diagnosed, I was a single parent and a successful attorney.  I was also healthy, or so I thought.  I ate right, I didn’t smoke, I was almost never sick and I exercised regularly.  But a simple cold turned into pneumonia and I had to be hospitalized.  X-rays revealed previously undiagnosed COPD. A subsequent blood test confirmed the diagnosis of Alpha-1 antitrypsin deficiency. Needless to say, I was devastated.

One year later, however, I got back to the business of living. Not long thereafter, I met another Alpha (person with Alpha-1) named John Walsh. Before I knew it, he had convinced me to join the board of the Alpha-1 Foundation, one of the best decisions of my life. Not only did it shift my focus to the search for a cure for Alpha-1, but also one of my fellow board members became my current husband.

Three years ago, I decided to write a “little book” for my grandchildren. I wanted to share my story with them.  I wanted to tell them that, no matter how bad things might seem, they would always have choices. Two and a half years and two hundred pages later, my little book was actually published. I called it “An Alpha-1 COPD Love Story”, and dedicated it to my husband, Dr. Gordon l. Snider.

Along the way, I discovered that not only did I enjoy writing, but also there were some who actually thought I might have some talent. The next question was where to apply it. The answer was simple. I’d become an advocate for a cure for COPD. The next question was how. I wrote an Op-Ed piece for the New York Times. It wasn’t accepted. Then my daughter suggested a blog.

Current estimates are that there are twenty-four million of us in the United States today that have COPD. But more frightening is the estimate that twelve million of us don’t know it. Likewise, current estimates are that there at least 100,000 Alphas in the United States today and less than ten thousand of us know it. For those of you who don't know it, Alpha-1 is the principal genetic cause of COPD. 

In sum, my first ojective in creating this blog is to advocate for a cure for COPD. But both Alpha-1 and COPD are treatable, especially if diagnosed early enough. That's my second objective, to advocate for early diagnosis for both Alpha-1 and COPD. My final objective is to continue my advocacy for a cure for Alpha-1. 

Over the next day or two, I will post my unpublished Op-Ed piece. In the meantime, I’d love to hear from you. What to you think of my mission?  Will you join me?