Achieving adequate growth in infants with congenital diaphragmatic hernia prior to discharge.

BACKGROUND/PURPOSE: To evaluate the enteral feeding requirements, including caloric provisions, of infants with CDH in relation to growth patterns. METHODS: A retrospective observational study was conducted on infants with CDH between August 2012 and March 2017. Electronic medical records were reviewed to extract detailed infant feeding data and anthropometric measurements at monthly intervals until discharge. Statistical methods of analysis included generalized linear models, Pearson correlation coefficient, Analysis of variance (ANOVA), Kruskal-Wallis, Wilcoxon rank sum, and Fisher's Exact tests. RESULTS: Among 149 infants with CDH, 45% (n = 67) met criteria for malnutrition at discharge. Maternal human milk (HM) was initiated in 95% of infants (n = 142) and continued in 79% of infants (n = 118) at discharge. Overall, 50% received fortification of feeds, including 60% (n = 89) of formula fed infants compared to only 21% (n = 31) of HM fed infants (p<0.001). Infants fed formula had lower weight-for-length z-scores at discharge compared to those fed HM. CONCLUSIONS: Infants receiving HM demonstrated improved growth compared to formula fed infants. However, higher calorie feeding regimens need to be initiated earlier to improve growth velocity. Prompt recognition of malnutrition and growth failure with aggressive supplementation may improve the overall growth of infants with CDH and has the potential to improve long term neurodevelopmental outcomes.

Congenital Infantile Fibrosarcoma in the Premature Infant.

BACKGROUND: Congenital infantile fibrosarcoma (CIF) is rare and represents less than 1% of all childhood cancers. It is a tumor that originates in the connective fibrous tissue found at the ends of long bones and then spreads to other surrounding soft tissues. These lesions are typically large, grow rapidly, and can often be mistaken for teratomas. Diagnosis is confirmed by pathology, where cellular proliferation of fibroblasts occurs. Imaging is an important part of the diagnosis, which includes the use of magnetic resonance imaging and/or computed tomography scan. Although surgical resection is the primary treatment, chemotherapeutic agents may be used as adjuvant therapy. PURPOSE: To describe modalities for accurate diagnosis and treatment of CIF. METHODS/SEARCH STRATEGY: PubMed was searched using terms "congenital infantile fibrosarcoma" and "infantile fibrosarcoma." Eleven relevant, English language articles were identified and utilized in the preparation of this case presentation. FINDINGS/RESULTS: Complications addressed in this case presentation are prenatal diagnostic challenges, pharmacologic interventions in the setting of prematurity, immunosuppression, and acute liver and renal failure. Pharmacologic treatments will include chemotherapy agents, antimicrobial agents, as well as granulocyte colony-stimulating factor for immunosuppression. Nursing challenges included positioning and integumentary disturbances. IMPLICATIONS FOR PRACTICE: Utilization of diagnostic imaging and pathology to accurately identify and diagnose CIF is essential. IMPLICATIONS FOR RESEARCH: Safety and efficacy of chemotherapeutic agents in premature infants with CIF need to be established.

A Continuous Quality Improvement Project to Implement Infant-Driven Feeding as a Standard of Practice in the Newborn/Infant Intensive Care Unit.

OBJECTIVE: To increase the number of neonates who were fed according to cues prior to discharge and potentially decrease length of stay. DESIGN: Continuous quality improvement. SETTING: Eighty-five bed level IV neonatal intensive care unit. PATIENTS: Surgical and nonsurgical neonates of all gestational ages. Neonates younger than 32 weeks gestation, who required intubation, continuous positive airway pressure (CPAP), high flow nasal cannula (HFNC), or did not have suck or gag reflexes were excluded as potential candidates for infant-driven feeding. INTERVENTION/MEASUREMENTS: The project was conducted over a 13-month period using the following methods: (a) baseline data collection, (b) designation of Infant Driven Feeding (IDF) Champions, (c) creation of a multidisciplinary team, (d) creation of electronic health record documentation, (e) initial staff education, (f) monthly team meetings, (g) reeducation throughout the duration of the project, and (h) patient-family education. RESULTS: Baseline data were collected on 20 neonates with a mean gestational age of 36 0/7(th) weeks and a mean total length of stay (LOS) of 43 days. Postimplementation data were collected on 150 neonates with a mean gestational age of 36 1/7(th) weeks and a mean total LOS of 36.4 days. A potential decrease in the mean total LOS of stay by 6.63 days was achieved during this continuous quality improvement (CQI) project. CONCLUSIONS: Neonates who are fed according to cues can become successful oral feeders and can be safely discharged home regardless of gestational age or diagnosis.

Congenital hyperinsulinism: exclusive human milk and breastfeeding.

Congenital hyperinsulinism is a genetic condition causing dysregulation of insulin and results in persistent hypoglycemia. The most common types are sulfonylurea receptor (SUR1), potassium inward rectifying channel (Kir6.2), glutamate dehydrogenase (GDH), and glucokinase (GK), with SUR1 and Kir6.2 being the most prevalent. It is imperative that these infants undergo diagnostic testing, which includes genetic, neonatal fasting study to induce hypoglycemia, glucagon stimulation, and imaging. Once a diagnosis has been made, surgical intervention may be needed to help regulate blood glucose levels. During this diagnostic process and as the infant is undergoing treatment, there may be little concern for the mother's feeding plan. Because human milk is the preferred form of nutrition for all infants, these mothers should receive prenatal counseling regarding the initiation and maintenance of milk supply. Parenteral nutrition may be necessary to maintain blood glucose to support human milk administration and breastfeeding.

Making the case for using donor human milk in vulnerable infants.

Vulnerable infants are at an increased risk for feeding intolerance due to immaturity or dysfunction (ie, congenital anomaly or obstruction) of the gastrointestinal system and/or hemodynamic instability. Symptoms of feeding intolerance include vomiting, water-loss stools, increased abdominal girth, and increased gastric residuals. It has been well documented that human milk provides optimal nutrition for infants and decreases the incidence of feeding intolerance. Donor human milk can be used for these at-risk infants to supplement the mother's own milk supply if insufficient or if the mother has decided not to or is unable to provide human milk for her infant. Establishing a donor human milk program within your institution will allow an opportunity for all vulnerable infants to receive an exclusive human milk diet.

An innovative model for achieving breast-feeding success in infants with complex surgical anomalies.

This manuscript describes an innovative nurse-driven continuous quality improvement project. Infants born with congenital surgical anomalies face significant challenges within the newborn period and human milk/breast-feeding may not be viewed as a priority. In many hospitals, nurses refer families to lactation consultants for all breast-feeding assistance and support. The Transition to Breast Pathway was developed on the basis of the evidence-based standards and protocols at The Children's Hospital of Philadelphia. The pathway consists of (1) initiation of pumping and maintenance of milk supply, (2) mouth care with human milk, (3) skin-to-skin care, (4) nonnutritive sucking at the breast, (5) transitioning to at breast feeds. A sample of 80 infants were enrolled in this project. Major results of the project are as follows: (1) mother's average milk supply was approximately 603 mL/d, (2) 71% (57/80) of the infants received mouth care with human milk, (3) 48% (38/80) mother/infant dyads performed skin-to-skin care, (4) 60% (35/58) of mother/infant dyads performed nonnutritive sucking at the breast, (5) 100% (58/58) of mother-infant dyads transitioned to breast prior to discharge. This continuous quality improvement project demonstrates that nurses can and should lead the process of transitioning infants to at breast feeds.