On a chilly Kansas City afternoon, we embarked on an adventure to KU Medical Center. We met with Adrienne Liebergen, ARNP. As a nurse practitioner who has specialized in cystic fibrosis for the past 37 years, she was the ideal person to answer our plethora of questions about this disease. As we began our conversation and got to know her, we were curious as to how she began her career in this field. After having a child, she sought a part-time nurse practitioner position. As history would have it, the cystic fibrosis department was hiring. She even admitted that she was only vaguely familiar with the disease at the time and had to seek additional information from the library prior to beginning. After working in the field for 30+ years, she is "married to it", but each day presents new challenges which keeps her coming back for more. She explained, "It's different everyday...different problems come up everyday...laws change...Medicaid changes..."
As we began the interview portion, Mrs. Liebergen clarified the details of cystic fibrosis. She explained that cystic fibrosis is a genetically caused progressive disease. It is a recessive disease, which requires the cystic fibrosis genes from both parents. Infants are commonly diagnosed with the disease just after birth. A newborn screen is blood testing that is done within 24 hours of birth and tests for 30+ diseases and conditions. As of 2009, the state of Kansas requires that cystic fibrosis is one of the diseases that newborns are screened for. If there are any abnormalities, the parents and their newborn are referred to a specialist. This is where Mrs. Liebergen comes into the process. To test for cystic fibrosis in particular, a sweat test is performed. It is a noninvasive test in which electrodes are placed on the arm to stimulate the sweat glands. This process measures chloride concentration in the sweat. If a patient has cystic fibrosis, the concentration of chloride will be elevated. A test with a measurement over 60 indicates a positive test. This simple process allows parents to know the results within about 30 minutes and before leaving her office.
When asked about additional symptoms of cystic fibrosis, she responded that there are multiple common and evident signs of this disease. Pneumonia at a young age (under 6 months), asthma, bronchitis, poor weight gain, salty skin, and greasy, poorly formed stools are all symptoms. If a pancreas is blocked by mucus, then the digestive enzymes cannot reach the intestines. This leads to greasy stools.
Mrs. Liebergen stated that she sees 4-6 patients per week with cystic fibrosis on a repeating basis. It is recommended by the Cystic Fibrosis Foundation that patients have a checkup with a specialist quarterly after being diagnosed. It is essential to ensure that patients, especially young children, are at an adequate height and weight for their age. Although the disease can be crippling, growth stunts are preventable. A unique aspect regarding treatment of this disease is the work of an entire care team. On her team in particular, there are physicians, a nurse practitioner, a clinic nurse, a respiratory therapist, a psychologist, a dietitian, and a social worker. They are all present on clinic days while seeing patients and all play a vital role in caring for each patient who is battling this disease.
When asked to elaborate on influential stories that have resonated with her, she recalled a story of one patient in particular who has a severe case of CF. Despite recommendations from her care team of when to take medications and how to perform at-home treatments, the patient has failed to follow through. This has led to multiple hospital visits, and eventually a chest tube was temporarily put in due to a partial lung collapse. Although this story is a tragic one, it is an influential reminder of how essential it is for patients to incorporate at-home treatments into their daily routines. There are various at-home therapies such as vests that the patient wears for a set period of time. These vests emit a high frequency chest wall oscillation which sloughs off excess mucus from airways. It allows the patient to cough up (via a "huff cough") and expel the excess, making it easier to breathe.
Although there is no current cure for cystic fibrosis, science is coming close. Mrs. Liebergen elaborated on various drugs that are in the research phase. To learn more about research development, visit the "Drug Development Pipeline" link at http://www.cff.org/research/DrugDevelopmentPipeline/. Most of the drugs that have been approved by the FDA already are used to prolong life after diagnosis, but
Kalydeco is different. This drug is the first one that targets the gene mutation (G551D mutation) that causes the disease. There are also inhalational drugs that are effective at reaching the airways quickly and easily. Patients with cystic fibrosis have more treatment options than ever before, but the medications do come with a price. Mrs. Liebergen admitted that a 30 day supply of some medications can be as expensive as $5000. Factoring in the price of an at-home airway clearance vest can be upwards of $15000-$20000. Although insurance does help, battling this disease does become a significant expense.
We are so thankful to have had the pleasure of learning more about this disease from Mrs. Liebergen. She gave informative insight that eluded a more personable element through her firsthand experiences. Her extensive knowledge helped to clarify the misconceptions of cystic fibrosis. Although the fight against cystic fibrosis is stressful and expensive, there is hope. Medical breakthroughs are happening everyday. Our group is so grateful to have had this opportunity to have had the chance to sit down and speak with Mrs. Liebergen and know that there is special care for patients with cystic fibrosis at KU Medical Center.
Interviewing Mrs. Liebergen was very interesting and enjoyable. She gave incredible insight to this devastating disease, which helped us have a better understanding of cystic fibrosis. She explained not only the medical side of cystic fibrosis, but also the personal and mental aspect of the disease. It was personally shocking for us to learn that there is such a large number of cystic fibrosis cases, and they're all different from one another. It was really eye-opening to learn about the extensive lifetime care that one has to follow if diagnosed with this disease. It is not something that you can take multiple medications, for and it is not something relatively inexpensive. You have to be dedicated to your health at all times, and it's very sad and almost disturbing to realize how much the disease can consume you. Every decision that you make has to be based on your condition. Apart from the ongoing care and lifestyle changes, there are also emotional issues that you must face. This is a problem that is often overlooked a lot by people. Living with cystic fibrosis can cause fear, anxiety, depression, stress and much more. This condition is not physically draining but also emotionally draining. Through this interview, we were able to understand the struggles one faces with cystic fibrosis--at least as much as a third party can understand. Mrs. Liebergen was truly knowledgeable about cystic fibrosis, and she showed us how she is in fact "married" to this disease. This interview gave us a new outlook on cystic fibrosis, and we truly appreciate Mrs. Liebergen for making time for us to talk to her.
"For to be poised against fatality, to meet adverse conditions gracefully, is more than simple endurance; it is an act of aggression, a positive triumph." - Thomas Mann
