OneThousandOne represents the amount of time, verbally, that it takes to count to one second. In this one second of time, a great thing happened at Nebraska Medicine. In fact, several great things probably happened. A patient was cured, a researcher found the missing link, a nurse treated an injury, a doctor comforted a family or maybe a child just smiled.
Fall | Winter 2011

A Deep Breath

For as long as Karen Behrens can remember, she has caught a cold or suffered from bronchitis nearly every month of her life. The colds would often linger longer than normal and sometimes they would progress to pneumonia.

An occasional cold now and then would be understandable — but every month for most of her life?

She never understood why she fell ill so often. And neither did her doctors. So she just learned to deal with it. Until several years ago.

Her health seemed to be getting worse. “I felt like I couldn’t get enough oxygen,” recalls Behrens, who was 43 at the time. “I used to run, but I had to give it up because I was too short of breath.”

Little by little, Behrens began giving up other things, too — her daughter’s play, a softball game, a track meet. “I just didn’t have the energy,” recalls Behrens. “There were days when I didn’t even want to get up and cook a meal.”

She pushed her doctor for an answer. She eventually saw two specialists and was referred to the adult program of the Nebraska Regional Cystic Fibrosis (CF) Center located at The Nebraska Medical Center, the only comprehensive program of its type in the region.

“When Karen came to us, her lung capacity was at 35 percent of predicted,” says Peter Murphy, MD, pulmonologist and director of the adult program of the Nebraska Regional Cystic Fibrosis Center. “At 30 percent, we start talking about lung transplant. She also had MRSA and pseudomonas lung infections. She had been chronically tired, run down, pale and had no energy. She was very ill.”

Dr. Murphy immediately suspected CF. A sweat test confirmed his suspicion. CF is an inherited condition that affects the lungs and digestive system of about 30,000 people in the United States. A genetic defect causes the body to produce mucus that is thick and sticky rather than loose and watery. Instead of sweeping out bacteria from the lungs, it clogs the lungs and leads to lung-damaging infections and inflammation. The mucus also blocks the pancreas, preventing natural enzymes from breaking down and absorbing food.

Behrens’ condition was serious. She was admitted to The Nebraska Medical Center for 12 days where she was put on oxygen, an aggressive regimen of CF therapies and airway clearance techniques. “Behrens’ condition would have continued to decline to a critical range had she not come in to see us when she did,” says Dr. Murphy. “For every week or month that she didn’t come in, she was losing lung function that she would not be able to get back.”

When Behrens finally left to go home, she was sent with detailed instructions for a daily therapy regimen that included several oral medications, two inhalers and mucus-clearing treatments.

But Behrens didn’t mind. In fact, she was happier than she had been in a long time. She finally had a diagnosis and a reason for her repeated illnesses. She could also breathe again. “I left feeling so much better,” says Behrens. “It’s a totally wonderful feeling to go from not being able to breathe to being able to take a deep breath again.”

Four years have passed since Behrens’ diagnosis and she is doing significantly better. Her lung capacity has peaked as high as 68 percent (a lung capacity of 80 percent or more is considered normal). She is sick far less than she ever has been and she is living an active life again.

As Behrens’ health improved, she eventually took a full-time job at Rockwell City School’s early learning program in her hometown of Lytton, Iowa. She also returned to walking and exercising regularly and is keeping up with her girls’ activities again.

“Unlike many CF patients, her lung chart function shows a continual incline rather than a decline,” says Dr. Murphy. “It is testimony to her enthusiasm, hard work and desire to get better and live life to the fullest. She is easily one of our hardest workers.”

Managing CF is hard work. The goal of treatment is to reduce damage to the lungs by regularly clearing the thick CF mucus from the airways. This helps reduce inflammation, the severity and frequency of lung infections and subsequent lung damage. Poor management of symptoms can lead to recurring illnesses and infections and progressive damage to the lungs.

"Unlike many CF patients, her lung chart function shows a continual incline rather than a decline. It is testimony to her enthusiasm, hard work and desire to get better and live life to the fullest."
Peter Murphy, MD

Treatment involves several strategies. The most time-consuming of these is daily airway clearance techniques that loosen and clear mucus from the lungs. Behrens uses a mechanical vest for about 30 minutes two to three times a day, which shakes the mucus loose to help clear the airways.

This is combined with other therapies such as inhaled medications, which allow drugs to reach the airways more quickly. These may include several types: medications that thin the mucus, antibiotics to treat infection, and saline that provides the airways with more water to help cough out mucus. Attention to nutrition is also vital to disease management and directly impacts lung function.

Behrens says she is very committed to her treatment regimen. “I’m not going to let this disease beat me,” she says. “I have a family who needs me. I have too many things to live for.”

Peter Murphy, MD
Peter Murphy, MD

It wasn’t that long ago that individuals diagnosed with CF were lucky to live through their teenage years. New medical treatments and aggressive disease management introduced over the last 30 years, however, have greatly improved survival rates for people with CF with many people living well into their 30s, 40s and beyond. “In the 1950s, many children died before they reached elementary school,” notes Dr. Murphy. “Recently, our oldest patient just turned 70. With proper treatment, most people with CF can live a fairly normal life.”

“Karen is very committed to her treatment,” says Jill Fliege, MS, APRN, nurse practitioner and coordinator for the adult program of the Nebraska Regional Cystic Fibrosis Center. “She works outside the home, has two daughters and a very supportive husband who keep her motivated. Usually the patients who have a reason to get up in the morning are more motivated and do the best.

“If she keeps doing what she’s doing, she can prolong her life for many years. She is quick to call when she is not feeling well, which is key. The earlier you can get started on treatment, the better your chances are of preventing more serious infection and damage to the lungs.”

New drugs are now under study that offer hope of changing the basic defect in CF that causes thick mucus, notes Dr. Murphy. In 1989, the gene responsible for CF was identified. This gene produces a protein whose function is to regulate the production of mucus. A compound that changes the mutant protein is proving to be effective and may be released in a year or two. “Unfortunately, the drug targets a protein that affects only about 3 percent of CF patients,” notes Dr. Murphy. “But it provides hope that other drugs may be produced that can help CF patients on a larger scale.”

“Managing cystic fibrosis requires daily care and treatment at home, so providing patients with proper education and instruction is critical for successful management of the disease.”
Peter Murphy, MD

Proper treatment by specialists trained in the treatment and management of CF is critical to managing CF successfully. “Cystic fibrosis is a very challenging and complex disease and requires proper care,” says Dr. Murphy. “Studies show that patients who are treated at a dedicated CF center are able to manage their disease with the greatest success and have the longest survival rates. Getting early and proper care is essential to achieve the best outcomes.”

Unfortunately, there are still quite a few patients who are living with the disease who are either misdiagnosed or undiagnosed. Many patients, like Behrens, have mild symptoms of CF, which can make the disease difficult to diagnose. Even in milder forms of the disease, CF still progresses and causes lung damage. Awareness of CF among the medical community has also been low. But that is changing.

A newborn screening that is required by most states is helping identify people with CF at birth. Once diagnosed, patients are started on a rigorous course of therapy that helps slow the progression of the disease and manage its symptoms.

Awareness among doctors is also increasing. “Just by having an adult center here at The Nebraska Medical Center, more and more medical trainees are exposed to the disease and its symptoms,” says Dr. Murphy. “I’ve received many referrals from across the region from doctors who trained here.”

The CF program at The Nebraska Medical Center includes both pediatric and adult teams that use a multidisciplinary approach to care. Staff includes dedicated physicians, nurses, respiratory therapists, dietitians, social workers and research coordinators. In addition to Dr. Murphy and Fliege, other members of the adult CF team at The Nebraska Medical Center include: Austin Thompson, MD, pulmonologist, associate director; Jennifer Rounds, RN; Jane Matsui, respiratory therapist; Brigid Mordeson, dietitian; Laura Romero, social worker; and Shandalle Fertig, clinical certified research coordinator.

The team partners closely with endocrinologists for the management of diabetes, which is a common problem among CF patients. “We see our patients about every three months and provide a lot of teaching at each visit,” says Dr. Murphy. “Managing cystic fibrosis requires daily care and treatment at home, so providing patients with proper education and instruction is critical for successful management of the disease.

“We have adopted a successful model of team care and multidisciplinary management of the disease that is very personal and has produced some of the best outcomes in the country. In 2007, our center was identified as one of eight benchmark centers in a study sponsored by the national Cystic Fibrosis Foundation (CFF). In addition, last year our center was awarded the CFF Quality Care Award in recognition of our quality improvement work.”

A key to the program’s success is the longevity of the staff. “All of the current team members were the first people to fill their positions since the adult program’s inception in August 2000,” says Dr. Murphy. “Another reason for our patients’ excellent outcomes is the outstanding care our patients receive by the dedicated staff of the pediatric program of the Nebraska Regional CF Center.”

Karen Behrens with her daughters and grandson
Karen Behrens with her daughters
Nicole, Left, Christine, Right,
and her grandson, Kenray

The pediatric CF program, which sees patients both at The Nebraska Medical Center and Children’s Hospital & Medical Center, is under the direction of John Colombo, MD, pediatric pulmonologist. The clinic typically transitions patients from the pediatric program to the adult program by age 21.

“Something that our CF providers share is a personal commitment and investment in our patients,” says Dr. Murphy. “Unlike other specialties, we are not treating just a single organ, we are treating the whole person so we take a more holistic approach to care. We really rejoice in our patients’ successes.”

One of the most satisfying and defining moments for Dr. Murphy was when he came to Behrens’ hospital room to visit. “Her daughter, who was also in the room said, ‘We have our mom back again,’” says Dr. Murphy. “That was extremely gratifying.”

Behrens makes the two and one-half hour trip to The Nebraska Medical Center once every three months for tests and reevaluation. “It’s worth it,” she says. “I couldn’t be in better hands. I wouldn’t go anywhere else. I trust them dearly.”

Behrens says she doesn’t push for answers anymore. She pushes for life.

Next article in the Fall | Winter 2011 issue of One Thousand And One:
Limitless Possibilities