Cystic Fibrosis Guidelines for the NICU

This Doctor of Nursing Practice scholarly project consists of developing diagnostic guidelines for Cystic Fibrosis in the Neonatal Intensive Care Unit. This guideline is based on current literature and consultation with experts in Cystic Fibrosis, nutrition, and neonatology. Cystic Fibrosis (CF) is the second most common life-threatening inherited disorder occurring in childhood in the United States. CF affects 1 in 3500 births, with one in every 31 Americans being carriers of a defective CF gene. Approximately 20% of infants with CF will have a meconium ileus, but 95% of infants born with a meconium ileus will have CF. Newborn and prenatal screening for CF presents the opportunity to diagnose the disease and start treatment early, thus maintaining health rather than treating the disease processes. By starting treatment early, complications such as failure to thrive, malnutrition, and decreased pulmonary function can be minimized. Initiating early treatment will not only improve patient outcomes, but will also decrease overall health costs by maintaining health, rather than treating the disease. The objectives of this scholarly project were to develop CF clinical guidelines for newborns in the NICU.  Determine current practices for screening and diagnosing Cystic Fibrosis in Utah.  Determine evidenced based practices and develop clinical guidelines for diagnosing and initiating treatment of newborns with a meconium ileus.  Prepare a poster of Cystic Fibrosis guidelines in the NICU for submission to a conference. CF is currently a terminal disease, but outcomes have improved dramatically over the last thirty years, with a current median age of survival of 36 years old. Prior to newborn and prenatal screening of CF, individuals were diagnosed after presenting with significant symptoms such as meconium ileus, respiratory symptoms, or failure to thrive. In the NICU, infants are generally diagnosed with CF after presenting with a meconium ileus. Early diagnosis and treatment has shown improvements in nutrition and lung function, but further improvement is needed. Implementation and evaluation of the first object was met by establishing an easy to use algorithm for the NICU. Implementation and evaluation of the second objective was achieved when the clinical guidelines were reviewed by the project chair and content experts, and approved for dissemination. The second objective was successful when the guidelines were submitted to the Division of Neonatology for approval to integrate into practice guidelines in the NICU. Implementation and evaluation of the third objective will be met through disseminating the guidelines to a wider audience. This objective will be met by submitting the poster to the NACFC in October.