Trying to Do What's Best: Maternal Perspectives About Toddler Sleep Health Among an Underresourced Sample of Mothers With Diverse Racial and Ethnic Identities.

INTRODUCTION: This study describes mothers' knowledge, attitudes, beliefs, and practices about their toddler's sleep health among an underresourced sample of mothers with diverse racial and ethnic identities. METHOD: This was a descriptive qualitative study with 16 mothers and their 12- to 36-month-old child. Mothers completed a semistructured, audio-recorded interview about their toddler's sleep health. Data were analyzed using inductive content analysis on the basis of established methods. RESULTS: Mothers self-identified as 18.8% Black, 43.8% White, 12.5% multiracial, 25.0% other race, and 37.5% Hispanic. Of the mothers, 80.0% reported a past year household income of ≤ $40,000. A core construct, "Trying to do What's Best," emerged from the interview data, and this construct included three domains: Getting Good Sleep, Getting Thrown Off, and Rolling With It. DISCUSSION: Findings support future strengths-based and multilevel sleep health-promoting interventions.

Molecular and functional correction of a deep intronic splicing mutation in CFTR by CRISPR-Cas9 gene editing.

Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the CFTR gene. The 10th most common mutation, c.3178-2477C>T (3849+10kb C>T), involves a cryptic, intronic splice site. This mutation was corrected in CF primary cells homozygous for this mutation by delivering pairs of guide RNAs (gRNAs) with Cas9 protein in ribonucleoprotein (RNP) complexes that introduce double-strand breaks to flanking sites to excise the 3849+10kb C>T mutation, followed by DNA repair by the non-homologous end-joining pathway, which functions in all cells of the airway epithelium. RNP complexes were delivered to CF basal epithelial cell by a non-viral, receptor-targeted nanocomplex comprising a formulation of targeting peptides and lipids. Canonical CFTR mRNA splicing was, thus, restored leading to the restoration of CFTR protein expression with concomitant restoration of electrophysiological function in airway epithelial air-liquid interface cultures. Off-target editing was not detected by Sanger sequencing of in silico-selected genomic sites with the highest sequence similarities to the gRNAs, although more sensitive unbiased whole genome sequencing methods would be required for possible translational developments. This approach could potentially be used to correct aberrant splicing signals in several other CF mutations and other genetic disorders where deep-intronic mutations are pathogenic.

Relationships Between Adolescents with Cancer and Healthy Peers: A Qualitative Study.

Purpose: Despite the developmental relevance and role in social support, research on relationships between adolescents with cancer and healthy peers is limited. To address this gap, we aimed to describe adolescents' perceptions of their friendships during the 1st year following a cancer diagnosis, including relationship changes, factors that promote/inhibit relationships, and definitions and experiences of peer support. Methods: Eligible adolescents were 12-20 years old, <1 year of a new cancer diagnosis, and English speaking. Participants completed 1:1 semistructured interviews that were analyzed using inductive content analysis. Results: Fourteen adolescents enrolled and completed interviews (mean [M]age = 14.8, standard deviation [SD] = 1.8; M = 6.3 months postdiagnosis, SD = 3.2 months). Domains included (1) shifting relationships, (2) staying connected, (3) making it hard to stay close, and (4) showing me they care. Relationship changes were positive and negative, and many described a process of recognizing true friends. Staying connected with peers through communication, technology, and feeling up to date promoted closeness, while distance, treatment-related restrictions, and friends' discomfort were hindrances. Adolescents defined supportive friends as those who were there for them, checked in often, and gave them gifts. Conclusion: Despite relationship changes, adolescents with cancer desire connection with peers during treatment and perceive that healthy peers provide valuable support. Supporting connectedness to healthy peers during treatment may be a promising future direction to mitigate social disruption and promote well-being.

Walking on Eggshells: Parents' First Year After Their Adolescent Completes Their Cancer Treatment [Formula: see text].

Background: Despite an awareness that parents experience distress and heightened uncertainty at the end of their adolescent's treatment for cancer, little is known about the specific challenges and concerns parents hold for their adolescent survivors or how they manage these challenges. This study described challenges parents experience in the first year after their adolescent's cancer treatment and what parents did to manage those challenges. Method: Single occasion semistructured interviews were conducted with parents and were transcribed verbatim. Data were coded using inductive content analysis methods adapted from grounded theory. Results: Participants were 30 parents of adolescent survivors (13-18 years) who were in the first year following treatment completion. The core construct, "Walking on Eggshells," explained the processes and experiences parents faced once treatment was complete. Two additional domains captured how parents managed posttreatment challenges ("Doing What We Can") and the ways parents helped their adolescents adjust ("Balancing"). Conclusion: Study results suggest that services and materials are needed to help parents know common struggles that arise after treatment and ways to mitigate the severity of distress parents experience.

New approaches to genetic therapies for cystic fibrosis.

Gene therapy offers great promise for cystic fibrosis which has never been quite fulfilled due to the challenges of delivering sufficient amounts of the CFTR gene and expression persistence for a sufficient period of time in the lungs to have any effect. Initial trials explored both viral and non-viral vectors but failed to achieve a significant breakthrough. However, in recent years, new opportunities have emerged that exploit our increased knowledge and understanding of the biology of CF and the airway epithelium. New technologies include new viral and non-viral vector approaches to delivery, but also alternative nucleic acid technologies including oligonucleotides and siRNA approaches for gene silencing and gene splicing, described in this review, as presented at the 2019 annual European CF Society Basic Science meeting (Dubrovnik, Croatia). We also briefly discuss other emerging technologies including mRNA and CRISPR gene editing that are advancing rapidly. The future prospects for genetic therapies for CF are now diverse and more promising probably than any time since the discovery of the CF gene.

Trying to Feel Normal Again: Early Survivorship for Adolescent Cancer Survivors.

BACKGROUND: Despite knowing the potential medical consequences of cancer treatment, little is known about how adolescents cognitively and emotionally frame, process, and manage in the early survivorship period. OBJECTIVE: The specific aims were to describe the worries, perceived challenges, and ways of dealing with these issues for adolescent cancer survivors in the early period of survivorship. METHODS: Twenty-nine adolescent survivors (12-18 years) completed a semistructured interview. Inductive coding methods adapted from grounded theory were used to analyze the data. RESULTS: Seven domains and 18 categories organized the adolescent's experience with early posttreatment survivorship. The domains included getting back to school; relationships with parents, siblings, friends; feeling changed by the experience; and concerns about relapse. CONCLUSIONS: This study contributes to our understanding of survivors' relationships with parents, siblings, and friends and survivors' models of the illness. Future studies are needed to understand how parents can help adolescents assume greater responsibility for their care, to understand what it is like for friends to have a peer with cancer and what behaviors by healthcare providers contribute to feelings of abandonment later in survivorship, and to better understand adolescent survivors' models of the illness and survivorship. IMPLICATIONS FOR PRACTICE: Study results suggest that nurses are in an ideal position to begin and to continue discussions with adolescent survivors about the adolescent's view of medical follow- up, its purpose and importance, and ways in which the adolescent can begin, early on, to engage in planning their own health during survivorship.

Being Fully Present: Gains Patients Attribute to a Telephone-Delivered Parenting Program for Child-Rearing Mothers With Cancer.

BACKGROUND: Oncology nurses can assist patients in gaining skills and confidence in multiple areas of illness self-management, including parenting skills. Child-rearing parents with cancer are a unique population because they must self-manage their illness and also help their child manage the intrusion of cancer on everyday life. The telephone offers an inexpensive channel for nurses to assist mothers in developing competencies to parent their child. The acceptability and attributed gains from such telephone services are unknown. OBJECTIVE: The aims of this study were to (1) describe the gains child-rearing mothers attribute to participation in a nurse-delivered telephone cancer parenting program and (2) assess mothers' evaluation of the telephone as a channel for delivering the program. METHODS: Study participants were child-rearing mothers diagnosed with cancer (N = 31) who had completed a manualized telephone-delivered cancer parenting program by a nurse. Mothers were interviewed 1 month after exiting the program by a specially trained interviewer masked on the content of the program. RESULTS: Most mothers were white (74%), highly educated, and had breast cancer (93.5%). Mothers attributed gains from the program in 3 areas: (1) being fully present for my child, (2) communicating in new ways, and (3) putting away my assumptions. CONCLUSIONS: Communication skills learned from nurses can assist mothers to self-manage the impact of the cancer on their own well-being and add to their parenting skills and competencies to help their children. IMPLICATIONS FOR PRACTICE: The telephone is an effective and indeed preferred channel for delivering services to child-rearing parents impacted by cancer.

Struggling in the Dark to Help My Child: Parents' Experience in Caring for a Young Child with Juvenile Idiopathic Arthritis.

PURPOSE: The purpose of this study is to describe parents' experiences in caring for 2-5-year-old children with juvenile idiopathic arthritis (JIA). DESIGN AND METHODS: A qualitative study using single-occasion in-depth interviews was conducted. Nine parents (eight mothers and one father) were interviewed in-person or via telephone. Data were analyzed using inductive content analysis. Methods used to protect the trustworthiness of study results included maintenance of an audit trail, peer debriefing, and member checks. RESULTS: The core construct Struggling in the Dark to Help My Child explained parents' experience in six domains: not knowing, trying to reach out in the dark, feeling my child's pain, working out the kinks to stay on top to manage, feeling drained by the whole process, and being hard on the entire household. Parents struggled with the unknown, searched for resources, witnessed their child's suffering without knowing how to help, and tried every possible way to stay on top of the child's illness and treatment, even when they felt drained physically and emotionally. JIA not only consumed their lives, but also affected the entire family, including the siblings and spouse, and the relationships among family members. CONCLUSION AND IMPLICATIONS: Findings highlight the day-to-day lived challenges parents face when caring for a young child with JIA. Healthcare providers including nurses need to assess the particular needs of an ill child and parents as well as the impact of the illness on the physical and psychosocial health of the entire family so that proper resources can be provided.

Using conceptual work products of health care to design health IT.

This paper introduces a new, model-based design method for interactive health information technology (IT) systems. This method extends workflow models with models of conceptual work products. When the health care work being modeled is substantially cognitive, tacit, and complex in nature, graphical workflow models can become too complex to be useful to designers. Conceptual models complement and simplify workflows by providing an explicit specification for the information product they must produce. We illustrate how conceptual work products can be modeled using standard software modeling language, which allows them to provide fundamental requirements for what the workflow must accomplish and the information that a new system should provide. Developers can use these specifications to envision how health IT could enable an effective cognitive strategy as a workflow with precise information requirements. We illustrate the new method with a study conducted in an outpatient multiple sclerosis (MS) clinic. This study shows specifically how the different phases of the method can be carried out, how the method allows for iteration across phases, and how the method generated a health IT design for case management of MS that is efficient and easy to use.

Behavioral and Educational Interventions to Support Family Caregivers in End-of-Life Care: A Systematic Review.

The demand for family caregivers steadily increases as the number of people receiving hospice and palliative care rises. Family caregivers play a significant role in supporting their loved ones in end-of-life care. However, there is limited evidence about the effectiveness of the interventions for supporting family caregivers. This article synthesizes behavioral and educational interventions that support family caregivers in end-of-life care. A systematic review was conducted and searched interventional studies published between 2004 and 2014 in PubMed, CINAHL, Embase, and The Cochrane Library electronic databases. Fourteen studies were identified and analyzed: 4 educational studies, 6 cognitive behavioral therapy studies, and 4 psychoeducational studies. All educational and behavioral interventions had developed structures and treatment manuals and improved family caregivers' outcomes. The cognitive behavioral therapy resulted in more positive outcomes than the other 2 interventions. More rigorous randomized controlled trials are needed to replicate current effective interventions with larger and diverse sample. Future studies need to develop tools for assessing family caregivers' needs, create consistent and specific tools to effectively measure family caregivers' outcomes, incorporate a cost-effectiveness analysis, and find the most efficient intervention format and method.

A review of pain measures for hospitalized children with cognitive impairment.

PURPOSE: The aims of this review were to examine pain measures for hospitalized children with cognitive impairment who are unable to self-report and to describe the best available evidence for their clinical utility in acute care settings. DESIGN AND METHODS: Electronic searches to identify published evidence were conducted and studies reviewed. Reported psychometrics and feasibility of the Non-Communicating Child's Pain Checklist-Postoperative Version, Individualized Numeric Rating scale, Pediatric Pain Profile, and revised Face, Leg, Activity, Cry, and Consolability scale were examined. CONCLUSIONS: These four measures have established validity and reliability. However, clinical utility findings varied. PRACTICE IMPLICATIONS: The revised Face, Leg, Activity, Cry, and Consolability scale has demonstrated feasibility in acute care settings related to ease of use, time requirements, and flexibility regarding caregiver input.

Nocturnal sleep-wake parameters of adolescents at home following cancer chemotherapy.

PURPOSE: The purpose of this descriptive, longitudinal study was to describe objective nocturnal sleep-wake parameters of adolescents at home after receiving chemotherapy in the hospital or outpatient clinic and explore differences in sleep variables by age, gender, and corticosteroid use. METHODS: We collected 7 days of wrist actigraphy and sleep diary data from 48 adolescents (10-19 years) who were receiving cancer chemotherapy for a primary or secondary cancer or a relapse. The actigraphic sleep variables included rest interval (i.e., time in bed), sleep onset, sleep offset, sleep duration, total sleep time (TST), wake after sleep onset (WASO), and %WASO. RESULTS: Of the 48 adolescents, 38 had at least five nights of scored actigraphy and were included in analyses. Older (13-18 years) adolescents went to bed later and had fewer minutes of TST than younger adolescents (10-12 years). Exploratory analyses revealed no differences between adolescents who were taking oral corticosteroids (i.e., prednisone, dexamethasone) and those who were not or between males and females. CONCLUSION: These adolescents had sleep durations that met or exceeded the recommended sleep duration for their age groups but experienced significant WASO. Further research is needed to estimate sleep needs of adolescents during chemotherapy and determine factors that contribute to nocturnal wake-time so that targeted interventions can be designed to improve sleep quality.

Sleep-wake patterns of school-age children and adolescents before diagnosis and during induction chemotherapy for acute lymphocytic leukemia.

Sleep disturbances are commonly reported by children receiving chemotherapy for leukemia. Sleep patterns before diagnosis and during induction chemotherapy were evaluated in 38 children (7 to 18 years old). Child Sleep Assessment (CSA) was used to evaluate sleep patterns prior to diagnosis. Sleep diaries and actigraphy were used during chemotherapy. Adolescents went to bed later and awakened later than school-age children before diagnosis and during chemotherapy. During chemotherapy, children averaged 60 minutes of nighttime wake time. The early recognition of sleep problems associated with disease, treatment, and age is important for school-age children and adolescents with leukemia.

Sleep quality and sleep hygiene behaviors of adolescents during chemotherapy.

STUDY OBJECTIVES: Describe the self-reported sleep quality and sleep hygiene behaviors of adolescents before diagnosis and during chemotherapy (CTX), compare their sleep quality and sleep hygiene behaviors with published normative data for healthy adolescents and adolescents with chronic pain, and explore factors that predict sleep quality. METHODS: Subjects were 51 adolescents (10 to 19 years) receiving CTX for cancer. A questionnaire was used to assess sleep patterns prior to the adolescent's cancer diagnosis, and a 7-day sleep diary was used to assess subjective sleep-wake activity during CTX. Sleep quality was assessed using the Adolescent Sleep Wake Scale, and sleep-facilitating and sleep-inhibiting behaviors using the Adolescent Sleep Hygiene Scale. RESULTS: Adolescents receiving CTX reported significantly worse sleep quality and sleep hygiene behaviors than healthy adolescents, and better sleep quality but similar sleep hygiene behaviors to adolescents with chronic pain. Significant interactions were found between bedtimes and wake-times on weekdays and weekends before diagnosis and during CTX. Sleep hygiene and demographic variables accounted for 24% of the variance in sleep quality. The cognitive and emotional subscales of the sleep hygiene scale and demographic variables accounted for 36% of the variance in sleep quality. CONCLUSIONS: Both the weekday and weekend sleep-wake patterns of adolescents receiving CTX resembled their weekend sleep patterns prior to diagnosis.

Differences in symptom occurrence, frequency, intensity, and distress in adolescents prior to and one week after the administration of chemotherapy.

PURPOSE/OBJECTIVES: The purpose of this study was to describe differences in occurrence, frequency, intensity, and distress of symptoms prior to (T1) and one week following (T2) the administration of intravenous chemotherapy. DESIGN: Longitudinal, descriptive. SETTINGS: Two regional children's cancer centers in the Pacific Northwest. SAMPLE: A total of 51 adolescents 10 to 19 years old receiving cancer chemotherapy. METHODS: Memorial Symptom Assessment Scale (MSAS 7-12). FINDINGS: No significant differences were found in symptom occurrence rates between T1 and T2. Fatigue was the only symptom with significantly greater frequency and intensity, and nausea was the only symptom with significantly greater distress at T2 compared with T1. CONCLUSIONS: Adolescents receiving chemotherapy experience multiple symptoms that persist over time. IMPLICATIONS FOR NURSING: In monitoring for symptoms, nurses need to be aware of the extent to which symptoms are present throughout treatment, as evidenced by these findings that symptom occurrence was significant even prior to receiving a subsequent course of chemotherapy.