My Sister's Lungs, Part One

By Nancy Wurtzel

Editor’s Note: In the first of a five-part series, thirdAGE contributor Nancy Wurtzel (at left in the above photo) shares the ordeal her sister Barbara (at right in the above photo) is undergoing as she waits for a match for a lung transplant. Barbara’s situation is complicated by the fact that her husband, Jim, has Alzheimer’s. Additionally, the sisters’ mother is suffering from dementia as well. Here is the story of a family on a frightening, grueling healthcare journey of the kind that will be all too familiar to millions of Americans. For an article by Nancy on the transplant issue overall, click here.

By Nancy Wurtzel

My older sister, Barbara, and I are having dinner at a local restaurant when her cell phone rings.  Barbara doesn’t recognize the number, and her hands shake as she answers.  I hold my breath, but exhale when I realize the call isn’t the one we’ve been anticipating.

That call, the one we’d hoped for, would have been from a lung transplant coordinator at the University of Minnesota, and the caller would have said the six words that would change our lives, “We have a match for you.”

Since the right match could take days, weeks or even months, we wait, practice patience, and reflect on my sister’s healthcare journey.

The story of my sister’s lungs began decades ago, but for most of those years, Barbara wasn’t even aware she had a serious illness, only knowing that when she got a cold it always affected her lungs.

By the time she was in her late 40s, however, Barbara knew something was very wrong.  Her lung infections had become more intense, frequent and prolonged.  Sometimes she had a new infection before she had even fully recovered from the last.

Over the years, Barbara had been seen by several pulmonologists, yet her condition remained a mystery.

One day a friend told her that the Mayo Clinic would evaluate walk-in patients seeking a diagnosis, even a person without a referral or appointment (a policy that has since changed).  A short time later, armed with her already-heavy medical record, my sister drove three hours south to Rochester, Minnesota, hopeful she might finally get some answers, but equally reticent about what she might learn.

The Mayo Clinic excels at solving medical puzzles.  Within a few days, Barbara had undergone dozens of tests and been seen by a team of health care experts.

The diagnosis was sobering: Interstitial Lung Disease, caused initially by an underlying condition called hypersensitivity pneumonitis (HP), a toxic lung inflammation brought about by breathing in a foreign substance such as certain types of dust, fungus or mold.

If HP is not detected and treated promptly, it can become a chronic condition and eventually morph into another, even more serious disease, which is exactly what happened to Barbara.

Doctors have determined Barbara now has pulmonary fibrosis, which has caused her deep lung tissue to become inflamed, scarred and stiff.  As the condition worsens, her lungs produce thick, sticky mucus which can trigger recurring infections.  Additionally, the lungs stop producing enough oxygen into the bloodstream.

Her physicians wanted to know: What had she inhaled?  When did it happen?  How did she contract this awful disease?

Barbara had no answers, but the questions kept her awake at night.  It would be years before she would know the root cause of her disease.

At the time of her diagnosis, Barbara learned her condition was incurable and irreversible.  While it would progress slowly, the disease would eventually cut short her life.

I remember the phone call after her meeting with the Mayo doctors.  Barbara told me, “I think I’m in shock, but now I know what I have.  It was awful not knowing and thinking I perhaps had only a short time left to live.”

Her first task was to begin eliminating the mucus build-up in her airways and lungs.  At least once a day, Barbara mpw uses a bronchodilator to induce coughing.  This “clearing” process takes anywhere from 30 to 60 minutes, and it leaves her sweaty, light-headed, out of breath and exhausted.  Sometimes the coughing becomes so intense she vomits.

Treatment also includes an array of powerful drugs, including Prednisone, a steroid that helps Barbara immensely, but has many side effects, including significant weight gain.

Overall, Barbara’s doctors were right.  Other than yearly testing at the Mayo Clinic, her decline was indeed slow, and she was able to live a relatively normal life.

In fact, if you saw my sister during those years, you might never have guessed she was battling a terrible disease.

Barbara continued her work, designing jewelry and running a successful business selling her products across the country.  Along with Jim, her husband of 26 years, she traveled extensively both for business and pleasure.  Eventually, they bought a home in Florida and spent the winter months in the Sunshine State, enjoying the balmy winter weather, and the rest of the year in Minnesota, our home state.

Then, in 2012, life changed dramatically.

I’d been living in California for many years, but decided to move back to Minnesota to take care of our aging mother who had Alzheimer’s disease.

As usual, Barbara and her husband arrived home later that spring to spend the summer months.

I was immediately startled by my sister’s condition.  She appeared pale and fragile, and she admitted her health had worsened.  Jim’s health was also a concern.  His behavior seemed odd and he often appeared confused.  Since our parents had both died from Alzheimer’s disease we knew the warning signs.

Later that year, Jim was diagnosed as being in the early stages of Alzheimer’s disease.

“Even though I was expecting it, I still felt sick hearing the neurologist say the words,” Barbara said.

Could their situation get any worse?  With both of them facing major health challenges, they decided to sell their Florida home and live permanently in Minnesota.

It was a dark time, and I remember sitting up late at night talking to her about Jim’s cognitive condition and her own health battles.

“Sometimes I think I just can’t stand it,” she said.  “I feel like my life is over.”

However, my sister rarely indulges in self-pity for longer than a few minutes, a trait that has served her well, especially during the past few years.

While Jim’s memory loss has progressed slowly, Barbara’s lung disease has worsened quickly.   In the space of six months, she has been hospitalized three times for pneumonia, and she’s never fully recovered from those illnesses.

Today, her health continues on a downward spiral.  She now requires a lot of sleep, increased medications and oxygen full-time.  Activities must be planned carefully and spaced out.  Simply going to the grocery store has become a challenge.

Without a transplant, doctors have told Barbara that she has only a few years to live.

Initially, she resisted the idea of a transplant.

“A transplant isn’t a cure, and I saw it as trading my current, known condition for the totally unknown,” said Barbara.  “It took me a long time to wrap my head around it.  However, if I could have another decade or more to live, then I want that chance.”

After exhaustive transplant evaluations – first at the Mayo Clinic and later at the University of Minnesota – Barbara was assigned a high score, placing her near the top of the lung transplant list in our upper Midwest region.

At night when I’m trying to sleep, I often go over-and-over my sister’s health journey.  There is so much that we’ve been through and so much unknown about what is ahead.  When I consider the whole picture, my throat starts to close up.

It’s easier for me to recount the facts of her disease than it is to talk about my feelings and fears.

Instead, I focus on the next appointment, test or support group meeting.  As my sister’s primary caregiver, my job is to help keep her life moving forward.  We’ve adopted a ‘one foot in front of the other’ coping strategy.

Last night, Barbara’s cell phone rang.  It was almost 10 p.m.

Was it the call?  No. The man on the line was from her local cable company reminding her about some equipment that needed to be returned.

But the next call, we tell ourselves, could be the one.

Look for installments in this story every Monday. Next week: Someone Must Die So My Sister Can Live

Nancy Wurtzel, a frequent thirdAGE contributor, is editor of the blog www.datingdementia.com.