Summer 2007 - Arkansas Children's Hospital

Cystic Fibrosis Team isHelping Children andFamilies Breathe EasierHOSPITAL PROGRAMSCameron and CarterWilkins have bothbeen CF patientsalmost since birth.Their father, Thomas,is very active in theACH CF FamilyAdvisory Board.■ Kila OwensAs a parent, being told your child has a life-threatening illness canbe heart-breaking. Imagine having to hear it twice.Thomas and Karena Wilkins have two sons, 4-year-old Cameronand 1-year-old Carter, and both have been diagnosed with cystic fibrosis(CF).In CF patients, the cells that line airway tubes and other passagewaysin the body do not secrete normal, free-flowing fluid. Instead, adefective gene causes the body to produce abnormally thick, stickysecretions that block airway tubes and other passageways, particularlyin the lungs and intestines, interfering with vital functions such asbreathing and digestion.Cystic fibrosis is a genetic or inherited disease, beginning at conceptionfrom a defect or gene abnormality.Cameron and Carter have been visiting the ACH Cystic FibrosisCenter almost since birth. The CF Center meets the many needs of CFpatients across the region, from initial diagnosis to ongoing treatment.The CF program’s staff is an interdisciplinary team of individuals,including representatives from the full spectrum of health care —physicians, specialty nurses, social workers, respiratory therapists, nutritionists,child life specialists and chaplains, among others.“It takes a multidisciplinary team to care for patients with CF,” saysLauren Willis, respiratory therapist. “We really have a fantastic group offolks who meet regularly, work very well together and are committedto providing the highest quality of care.”The CF Center is always working to improve the level of care providedto patients. Thanks to the team’s hard work, many great thingshave been achieved recently. One such accomplishment is the creationof a CF Center Family Advisory Board (FAB). The board, comprisingparents of CF children, was spearheaded by social worker Mary JoChambers.“Since CF patients are not allowed to socialize together, due to theneed to avoid infections, parents and families felt isolated,” Chamberssays. “We wanted to bring these parents together to discuss manyissues — how they felt, how they thought we were doing, how wecould improve.”The program has been a great success. Communication betweenparents and the CF team has been greatly enhanced with FAB-driven projects,such as a quarterly newsletter and online bulletin board. The programis being used as an example for other ACH departments, and Chamberswas asked to present about the FAB at the North American CFFoundation conference. In fact, because of the team’s successes this pastyear, several members were asked to present information at the conference.Another new development is the recent commitment by the ArkansasDepartment of Health to screen newborns for CF (and a number of otherdiseases) beginning in July 2008. According to Dr. Dennis Schellhase*,director of the ACH CF Center, implementing newborn CF screening willbe a challenge, but the benefits will be substantial.“The earlier you can make a diagnosis, the more opportunities you haveto begin early treatment of the child’s nutritional problems and lung disease,”says Schellhase.The outlook for young CF patients is bright, due to many advances inmedicine. Twenty years ago, the life expectancy of a CF patient was barelyout of adolescence. Today, the median life expectancy is 37 years andincreasing.Members of the ACH CF team feel a family-centered approach is integralto providing quality care.“We develop very close relationships with our patient families,”Schellhase says. “We see the kids so often, it’s like we become a member oftheir family.”Wilkins is thrilled with the care his children receive from the ACH CFteam. It is hard having one child with CF, much less two, but he says thesupport from the team is unparalleled.“They have great respect and love for their patients and their families,”he says. “The support they offered to us when our children were diagnosedwas amazing. They gave us information about the disease and let us knowwe were going to fight it together.”*Dennis Schellhase, M.D., is director of the Cystic Fibrosis Center at ACHand associate professor in the department of pediatrics, UAMS College ofMedicine.3