Patient-Reported Experience in the Pediatric Emergency Department: What Matters Most?

INTRODUCTION: Although the Child Hospital Consumer Assessment of Healthcare Providers and Systems is a validated tool for the inpatient experience, it may not address features unique to the pediatric emergency department (PED). There is currently no publicly available validated patient-reported experience survey for the PED, and what matters most in this setting remains unknown. METHODS: Twelve semistructured interviews were conducted with a convenience sample of parents of children younger than 14 years at a Canadian PED. Data analysis was performed using inductive thematic analysis to identify aspects of patient-reported experiences that matter most to parents in the PED. RESULTS: Five themes were identified: (1) making waiting a positive experience, (2) taking the time to provide care, (3) forging a positive partnership, (4) speak up for safe care, and (5) making the environment feel safer. Parents highlighted that while waiting for care is not desirable, it is made more acceptable through the communication of wait time estimates and the presence of child activities in the waiting room. Furthermore, although interactions with providers are brief, parents emphasized the importance of creating an environment of partnership with open communication, taking the time to examine their child, and actively demonstrating the provision of safe, quality care. CONCLUSIONS: Results from this study suggest that a patient-reported experience survey in the PED may need to embed elements not currently captured in Child Hospital Consumer Assessment of Healthcare Providers and Systems, such as waiting room experience, comprehensiveness of health assessments, and observations of safety measures. Future studies can use these findings to develop a patient-reported experience survey for use in the PED.

Incontinentia pigmenti in an XY boy: case report and review of the literature.

BACKGROUND: Incontinentia pigmenti (IP) is a rare genetic skin disorder with X-linked dominant inheritance and a characteristic sequence of cutaneous manifestations, which is regarded as lethal in XY males. OBJECTIVE: To report a case of a surviving XY male with the common IKBKG (NEMO) gene deletion confirming IP. METHODS AND RESULTS: A newborn XY male with suspected IP underwent a skin biopsy on affected tissue for histopathology. Molecular genetic testing was also performed on the specimen and revealed the common IKBKG gene deletion with a pattern suggestive of somatic mosaicism. Our findings are aligned with a PubMed literature review for XY males with IP and documented IKBKG mutation. We determined that only 10 such genetically proven cases have been reported, including our case. CONCLUSION: Although relatively rare, cases of IP in XY males with the common NEMO mutation have likely been underreported due to the unavailability of appropriate testing in the past. Karyotype and molecular testing should be considered when clinical suspicion of IP arises for a male patient.