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Objective: This study described the prevalence and duration of mothers' breastfeeding infants with phenylketonuria (PKU) and explored factors related to duration of breastfeeding as a surrogate for breastfeeding success. Subjects and Methods: Descriptive analysis as performed from an international Internet survey of mothers (n = 103) who met the inclusion criteria: (1) at least 21 years of age, (2) able to read and write in English, (3) child with PKU, and (4) living in the United States or Canada. Results: Of the 103 mothers, 89 (86%) initiated breastfeeding immediately following delivery, whereas 14 (14%) chose bottle feeding. In comparison to breastfeeding after delivery, significantly fewer mothers breastfed after diagnosis (McNemar's v 2 = 30.33, p < 0.001; n = 72 vs. n = 89). Breastfeeding duration ranged from less than 1 month to 24 months with one modal duration category (n = 20, 22%) at less than 1 month. The timing of the addition of commercial infant formula to supplement breastfeeding or expressed mothers' milk was associated with a shorter duration of breastfeeding among infants with PKU: v 2 (42, n = 73) = 88.13, p < 0.001. Conclusions: PKU is treated with phenylalanine (Phe) restriction. Breastfeeding infants with PKU is challenging in part because Phe intake is difficult to determine precisely. We studied breastfeeding duration in infants with PKU and factors associated with success. Further research should identify the unique needs of mothers' breastfeeding infants with PKU to guide the development of interventions specific to these mothers to support their efforts to continue breastfeeding after the diagnosis of PKU.

The skin serves many purposes, acting as a barrier to infection, protecting internal organs, contributing to temperature regulation, storing insulating fats, excreting electrolytes and water, and providing tactile sensory input. This article focuses on a review of normal skin structure and function and selected neonatal skin disorders. The disorders reviewed are Staphylococcal scalded skin syndrome, epidermolysis bullosa, and the ichthyoses. The basis for each skin disorder is presented. Nursing management and skin care are incorporated into the review of each selected disorder.

Findings demonstrate the importance mothers attribute to breastfeeding and their willingness to invest considerable effort to breastfeed. Health care providers working with these mothers should help them strategize for success.

With recent advances in laboratory technology with tandem mass spectrometry (MS/MS), the number of infants identified with a fatty acid oxidation disorder has increased dramatically. Disorders of fatty acid oxidation comprise one of the most rapidly growing groups within the field of errors of metabolism. This review will explore the recent developments in newborn screening related to the use of tandem mass spectrometry and disorders of fatty acid oxidation.

Breastfeeding duration for infants with phenylketonuria (PKU) is less than other full-term infants. However, no study has examined the challenges encountered by mothers' breastfeeding infants with PKU. In 75 mothers of a child with PKU, three categories of breastfeeding challenges were identified: common breastfeeding issues, breastfeeding and PKU, and no challenges. The common breastfeeding issues can be identified in the literature but for these mothers, the issues are heightened due to frequent phenylalanine (Phe) monitoring. Even so, many mothers adapt breastfeeding to maintain desired Phe levels. A few mothers had no issues and were the exception, not the norm.

The roles of clinical nurse specialist (CNS) neonatal nurse practitioner (NNP) have originated and evolved to meet specific needs in the nursing and medical community. The CNS role was developed in response to the perception that patient care needed to be improved; the NNP role resulted from an acute shortage of physicians to provide neonatal medical management. While on the surface these two roles may appear to be vastly different, many facets of each may be effectively interfaced. The NNP-CNS practice at Doernbecher Neonatal Care Center is an example of successful convergence of the two roles. The implementation of the combined practice is described in this article.

Since 1961, newborn screening for errors of metabolism (EM) has improved the diagnosis, treatment and outcome of newborns with an EM. Recently, advances in laboratory technology with tandem mass spectrometry (MS/MS) has increased the identification of newborns with an EM. With a single dried filter paper blood spot (Guthrie R, Susi A. A simple phenylalanine method for detecting PKU in large populations of newborn infants. Pediatrics. 1963;32:338-343), MS/MS can identify more than 30 disorders of metabolism. This review will explore MS/MS to provide a better understanding of the development and application of this technology to newborn screening for perinatal and neonatal nurses.