It was natural that Amy McKeen should choose pulmonary diseases as her specialty when she became a nurse 20 years ago. “I lost a cousin to Cystic Fibrosis (CF) when he was four, and I’m a carrier, although my children don’t have it,” said McKeen, RN, BSN.
Cystic Fibrosis is a life-threatening disease that causes the body to produce unusually thick, sticky mucus that clogs the lungs and can lead to serious lung infections. It also obstructs the pancreas and stops natural enzymes from helping the body break down food and absorb vital nutrients.
“CF is one of the most common genetically- inherited diseases and an illness that people associate with early death, but that’s not the case anymore. We have better drugs, better screening and more knowledge. Many of our patients are now living long and fruitful lives.”
McKeen knows because she’s a part of her patients’ lives. As a nurse clinician for pediatric pulmonology and coordinator for the Cystic Fibrosis Center at the Children’s Hospital of Georgia (in Augusta), she serves as case manager for its 100 patients.
“I was asked to prom by one patient, and went,” she said. She is also invited to birthday parties and high-school graduations. Some of her former patients have become lawyers, nurses, policemen, teachers and mothers. She’s proud to have helped them beat the odds.
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“There is no cure for CF, but we have better treatments and promising drugs in the pipeline,” she said. “If you have to be born with CF, this is a good time, because things have changed so much.”
Still, it’s hard discussing a CF diagnosis with new parents. “Since Georgia and South Carolina now have newborn screening, we can catch our patients before the illness starts and be proactive about it,” she said. “But it is devastating news to parents who thought they had a Gerber baby.”
It’s an emotional experience for everyone that first year. Families have to visit the clinic at least once a month for patient evaluation, treatments and education. Some families must travel five to six hours for those appointments.
They learn how to clear airways, break up mucus by beating on the chest, how to fight infections and to administer enzymes and vitamin supplements long before a child would normally take solid foods. “There’s a lot of crying,” she said.
“Then there are all the emotional and financial questions. Should the mom go back to work? The drugs are expensive and can cost $3000 to $10,000 a month. Even families with insurance struggle with co-pays and other expenses,” she said. Making sure families have the resources and emotional support they need requires a team to serve as clinicians, educators, cheerleaders, counselors and social workers.
Reaching that first birthday is a milestone, but the hurdles don’t stop. “Families go through seasons when things go well, and seasons when they don’t. We go through those stages with them. When moms and dads are in trouble, I know it,” she said. “I’m a positive person and an honest one. I tell them that I’ll let them know if they need to worry.”
Always searching for new ways to help families, McKeen participates in research studies with the Cystic Fibrosis Foundation and in its Quality Improvement Initiatives. She’s developed resource books for parents of newly diagnosed patients, adolescents who struggle with being different and no longer want to comply with their regimens, and patients who are transitioning to adult care. The center produces a quarterly newsletter and hosts an annual education day for families where clinicians share the latest findings from medical conferences. McKeen and her family often participate in CF fund-raising events around the state.
“I keep a pen and pad by the bed, because some of my best ideas of things we should do come in the middle of the night,” she said. At present, she’s seeking a grant to add a pediatric palliative care physician to the team to assist with symptom management. “I’m excited, because I know it will help,” she said.
Her favorite part of the job is talking to patients as they transition into adult care. “Knowing that we’ve taken care of them, that they’ve successfully made it through and are about to enter the second phase of their life is a wonderful feeling,” she said.
In May, McKeen was named Nurse of the Year at the Spirit of Nursing Showcase hosted by the CSRA Chapter of the Georgia Nurses Association in May. “This award should go to our entire team. We have developed a lot of tools to help families,” said McKeen. “I’m blessed to be a part of that.”