The experience of adults with sickle cell disease and their HLA-matched adult sibling donors after allogeneic hematopoietic stem cell transplantation.

AIM: To provide a rich description and in-depth understanding of the recipient-donor allogeneic hematopoietic stem cell transplantation experience. BACKGROUND: A stem cell transplant has a high likelihood of improving symptoms of sickle cell disease in adults. While studies have reported the transplant experiences of recipients and donors with haematological malignancies, no published reports have examined the experience of both adult recipients with sickle cell disease and their donors. DESIGN: Exploratory qualitative descriptive analysis. METHODS: We conducted individual interviews with 13 recipients and donors (eight males, five females) representing five recipient-donor dyads and one recipient-donor triad from one Midwest transplant centre between August 2017-February 2018. Interviews were digitally audio-recorded, transcribed verbatim and analysed using conventional content analysis. FINDINGS: Five themes were identified: the downward spiral and a second chance; getting the monster off my back; difficult and manageable; it was worth it; and relating to the healthcare team. CONCLUSIONS: The results provide a description and insights into the complex nature of the stem cell transplant experience in sickle cell disease from the perspectives of both recipients and donors. IMPACT: Health provider awareness of recipient-donor experiences can contribute to family-centred care that supports the health and quality of life for both recipients and donors. This understanding promotes high quality clinical care and improved communications by taking into account the knowledge, values and informed preferences of recipients and donors and contributes to improved decision-making and clinical care. Future research can assess family experiences that support informed choice for potential transplant candidates.

Home-Based Telemedicine for Children with Medical Complexity.

Background: Children with medical complexity (CMC) are high utilizers of health care services. Telehealth encounters may provide a means to improve care outcomes for this population. Objective: To evaluate the feasibility, usability, and impact of an in-home telehealth device in the care of CMC. Methods: This single-center feasibility study employed a nonblinded randomized clinical trial design. English-speaking caregivers of children within a pediatric complex care program with home Wi-Fi were eligible for participation. Participants were randomized 1.5:1 with stratification based on tracheostomy status to a control group that received usual care or an intervention group that received a telehealth device for in-home use. Patients were followed up for 4 months. The primary outcome was successful device connectivity and data transmission. Data included clinician encounter device usability; caregiver satisfaction; and encounter type, purpose, and cost. Descriptive statistics, negative binomial regression, and Kaplan-Meier plot were used for analysis. Results: Twenty-four patients were enrolled (9 controls, 15 in the intervention group) in September 2016. The telehealth device was attempted in 73 encounters. Device connectivity was successful 96% of the time. Image and sound quality were acceptable in 98% of visits. Caregivers expressed their overall satisfaction with the device. The hospitalization rate was lower in the intervention group (0.77 vs. 1.14 intensive care unit days/patient-months), resulting in $9,425/USD per patient savings compared with the control group. Conclusion: Despite small sample size and short observation period, this study demonstrated that use of an in-home telehealth device is feasible, well received by caregivers, and can result in decreased hospitalizations when compared with usual care.

Health care professionals' views of sharing information with families who have a child with a genetic condition.

The purpose of this study was to examine health care professionals' views and strategies for individualizing information sharing in families who have a child with a genetic condition. The sample consisted of 37 health professionals from three clinical sites in the greater metropolitan area of a large Midwestern city. Qualitative content thematic analysis was used to analyze data from the health professionals' semi-structured interviews. Four themes captured how health care professionals work with families around information management: Sharing Information with Parents, Taking into Account Parental Preferences, Understanding of the Condition, and Helping Parents Inform Others. These findings contribute to understanding the processes that health professionals use in sharing information with parents who have children with a genetic condition, and they provide guidance for clinical practice, professional training, and future research.

Parental perceptions of the outcome and meaning of normalization.

The purpose of this secondary analysis was to identify the meaning of normalization for parents of a child with a chronic genetic condition. The sample was comprised of 28 families (48 parents), selected to reflect two groups: Normalization Present (NP) and Normalization Absent (NA). Constant comparison analysis was used to identify themes characterizing parents' perceptions of the meaning of normalization. The meanings parents attributed to normalization reflected their evaluation of condition management, parenting role, and condition impact, with parents in the NP and NA groups demonstrating distinct patterns of meaning. These meaning patterns are discussed as an outcome of normalization. Providers can play a pivotal role in helping families achieve normalization by providing guidance on how to balance condition management with normal family life.

Information management in families who have a child with a genetic condition.

In families with children with genetic conditions, information management may influence how parents view their child; their reproductive decision making; and how, when, what, and why they communicate about the condition to other family members. The purpose of this study was to identify unique patterns of information management and to explore the relationship between these patterns and individual and family characteristics and functioning. The sample was composed of 142 parents in 86 families in which a child had a single-gene condition. Four distinct information management patterns were identified that varied in the parents' understanding of the genetic aspects of the condition: accurate understanding-open (35%, n = 30), accurate understanding-selective (24%, n = 21), discrepant understanding (15%, n = 13), and confused understanding (26%, n = 22). In two patterns, accurate understanding-open and accurate understanding-selective (59%), parents had an accurate understanding of the genetic aspects of the condition, and the patterns were differentiated from one another by parents' views about sharing information. In the discrepant understanding pattern, which included only two-parent families, one parent had an accurate understanding and one parent a confused understanding of the genetic aspects of the condition. In the confused understanding pattern, parents had an inaccurate understanding of one or more of the genetic aspects of the condition. Statistically significant differences were found between information management patterns and parents' education, family annual income, and health insurance coverage. These information management patterns reflect how parents access, interpret, and convey genetic information and can influence the effectiveness of health care providers' interactions with these families.

Parents' concerns about issues related to their children's genetic conditions.

PURPOSE: The purpose of this analysis was to examine parents' (N = 142) concerns about issues (i.e., privacy of information, insurance, healthcare costs, employment, school) related to their children's genetic conditions. DESIGN AND METHODS: Using a series of matrices, thematic analysis was conducted focusing on parents' concerns. RESULTS: Parents were less concerned with privacy and more concerned with insurance, healthcare costs, employment, and school issues. PRACTICE IMPLICATIONS: Nurses and other healthcare professionals need to give parents the opportunity to discuss their concerns and to assist parents with strategies and resources to meet the needs of their children and families.

Outcomes of early nutrition support in extremely low-birth-weight infants.

BACKGROUND: Early nutrition intervention, both parenteral and enteral, is becoming a standard of care for the extremely low-birth-weight infant (ELBW; <1,000 g) in many neonatal intensive care units (NICU) across the United States. However, there are no published or widely accepted guidelines regarding nutrition support strategies for this population. Most NICU clinicians have developed their own guidelines, so nutrition practices vary widely. In an effort to standardize our practice, we implemented nutrition support guidelines for ELBW infants, initiating both parenteral nutrition (PN) and minimal enteral feedings (MEFs) within the first 24 hours of life, whenever possible. The purpose of this study was 2-fold: (1) to evaluate the adherence to the nutrition guidelines and (2) to compare pre- and postguideline outcomes such as time to regain birth weight, time to reach full enteral feedings, and average daily weight gains. METHODS: The study was conducted at a level III NICU from January 2002 until February 2003. Charts of 70 infants with a birth weight

Severe Iron Deficiency Anemia in Infants and Young Children, Requiring Hospital Admission.

Objective. This study evaluated patient characteristics, milk intake, and associated lab findings of children 6 months to 5 years old, admitted to a children's hospital with severe iron-deficiency anemia (IDA). Methods. A chart review was conducted on patients admitted with microcytic anemia (hemoglobin concentration less than 7 g/dL), accompanied by a low serum ferritin and/or low serum iron level between January 2000 and December 2006. Results. A total of 18 children with severe IDA were evaluated. Many had parents with private insurance and jobs. Almost all children drank >24 ounces of milk daily. Hemoglobin on admission was 3.8 g/dL, and the mean of the patients' mean corpuscular volume was 52.8 fL. Median iron levels were 4 µg/dL. Conclusions. Severe IDA is still prevalent in children, yet physicians may not perform necessary testing. The devastating long-term effects of severe IDA should prompt clinicians to screen for severe IDA in children regardless of absent risk factors.

Procalcitonin as a screening test for late-onset sepsis in preterm very low birth weight infants.

OBJECTIVE: To compare the utility of procalcitonin (PCT) vs C-reactive protein (CRP) as indicators of late-onset neonatal sepsis in very low birth weight (VLBW) infants. METHODS: PCT and CRP levels were measured in VLBW infants with suspected sepsis and controls. Comparisons were made between infected vs noninfected infants. Using cutoff values of 0.5 and 1.0 ng/ml for PCT and 0.8 mg/dl for CRP, sensitivity, specificity, positive and negative predictive values were calculated to evaluate these assays as potential predictors of late-onset sepsis. RESULTS: A total of 67 infants were evaluated. Mean PCT levels were significantly higher in the infected group (5.41 ng/ml) compared to the noninfected group (0.43 ng/ml) (p < 0.001). At a cut off value of 0.5 ng/ml, the sensitivity of PCT was 97%, whereas that of CRP was 73% in predicting late-onset sepsis. At a PCT cutoff of 1.0 ng/ml, sensitivities of PCT and CRP were similar (72% each). CONCLUSION: PCT (0.5 ng/ml) is more sensitive than CRP in predicting late-onset sepsis in VLBW infants.

Parents sharing information with their children about genetic conditions.

INTRODUCTION: Nurses are in an ideal situation to help families share information with their children about genetic conditions. While many factors contribute to this decision making, little is known about how parents decide when and how to convey this information to their children. The purpose of this qualitative analysis was to examine parents' beliefs and strategies related to sharing information about a genetic condition with their affected and unaffected children. METHOD: One hundred thirty-nine parents participated in this study. Narrative analysis and fundamental qualitative description were employed to identify approaches and strategies that parents used to share information with their children. RESULTS: Parents' information sharing approaches and strategies were grounded in the goal of promoting the child's adaptation to the genetic condition. Parents shared information based on their assessment of the child's developmental readiness and interest and described information sharing as an unfolding process that continued throughout childhood. DISCUSSION: The approaches and strategies contribute to understanding the processes associated with information sharing between parents and their children and between health care professionals and parents.

Does umbilical cord care in preterm infants influence cord bacterial colonization or detachment?

OBJECTIVE: The purpose of the present study was to compare alcohol versus natural drying for umbilical cord care in preterm infants and to examine its effects on bacterial colonization and cord detachment. STUDY DESIGN AND METHODS: Eligible infants <34 weeks gestation admitted to the NICU were randomized to receive either umbilical cleansing with 70% isopropyl alcohol at each diaper change or natural drying. Umbilical stump cultures were performed at 12 to 24 hours, 72 hours, 7 days, and 14 days of age. RESULTS: A total of 109 infants were enrolled; 102 completed the study. Analyses revealed that the median cord detachment time was significantly shorter in the natural drying group compared to the alcohol group (13.0 versus 16.0 days; p=0.003). There were no cases of local umbilical infection in either group. CONCLUSION: Based on the present study, it appears that natural drying is a safe and effective means of umbilical cord care in preterm infants.

Upregulation of neutrophil surface adhesion molecules in infants of pre-eclamptic women.

OBJECTIVE: To evaluate evidence of neutrophil activation in infants born to pre-eclamptic women and examine any association between degree of neutrophil activation and severity of pre-eclampsia. DESIGN: This study utilized quantitative flow cytometry to determine whether the expression of surface adhesion molecules: CD18, CD11a, CD11b, and CD11c on cord blood neutrophils using mean channel fluorescence values (MCF). A total of 20 infants of pre-eclamptic women were compared with a control group of 19 infants of normotensive women. RESULTS: MCF values were significantly higher in infants born to pre-eclamptic women vs controls: CD18 (432.0+/-236.3 vs 230.5+/-97.9; p=0.002), CD11a (552.9+/-272.4 vs 326.9+/-268.6; p=0.003), CD11b (937.2+/-521.9 vs 576.6+/-352.9; p=0.025), and CD11c (228.5+/-130.3 vs 133.0+/-77.1; p=0.006), respectively. The mean MCF values appeared higher in severe vs mild pre-eclampsia. CONCLUSIONS: This study revealed neutrophil activation in infants born to pre-eclamptic women. The relationship between neutrophil activation and severity of pre-eclampsia warrants further study.

Infection control knowledge, attitudes, and practices among cystic fibrosis patients and their families.

BACKGROUND: In 2003, the Cystic Fibrosis (CF) Foundation in the United States published evidence-based infection control guidelines and distributed these to CF care centers. However, it is unclear how well the guidelines have been disseminated to patients and families, how well patients and families understand the principles of infection control, and what barriers they experience implementing the guidelines. METHODS: We assessed infection control knowledge, attitudes, and practices among CF patients and their families at 17 randomly selected CF centers. Anonymous surveys were completed by CF patients (≥16 years old) or their family members (patients <16 years old). To adjust for similarities of patients within each center, generalized estimating equations regression was used. RESULTS: From January 2007 to May 2009, 1,399 respondents completed surveys of whom 38% were patients and 62% were family members (overall mean age of patients = 14 years). Overall, 65% of respondents were aware of the CF infection control guidelines, but only 30% had discussed them more than once with their CF care team. More than one discussion was associated with increased knowledge of infection control, including routes of pathogen transmission; the importance of avoiding close contact with other CF patients; increased confidence in practicing infection control; and increased belief in the health benefits of infection control. CONCLUSIONS: This study revealed that many CF patients and families are aware of the infection control guidelines, but that few had discussed them more than once with their CF teams. These findings underscore the importance of engaging patients and their families in regular discussions about infection control that address questions and concerns including the potential impact of infection control on health and well-being. Further strategies are needed to overcome barriers to implementing these guidelines.

Barriers to adherence to cystic fibrosis infection control guidelines.

BACKGROUND: In 2003, the American Cystic Fibrosis (CF) Foundation published revised, evidence-based guidelines for infection control. We sought to assess potential barriers to adherence to these guidelines experienced by health care professionals (HCPs) caring for CF patients. METHODS: From April 2004 to December 2005, a knowledge, attitude, and practice survey was administered to HCPs at randomly selected CF centers in the United States to explore potential barriers to adherence to selected guidelines: (1) obtaining quarterly cultures from CF patients, (2) discouraging socialization among CF patients during hospitalization, (3) educating patients and families about hand hygiene, (4) educating patients and families to clean and disinfect home nebulizers, and (5) cleaning the clinic exam rooms between CF patients. RESULTS: The survey was completed by 528 HCPs from 25 sites (5-50 respondents per site). Only 60% of respondents were aware of the guidelines, but despite awareness, 31-47% were unfamiliar with the specific guidelines. Self-reported adherence was low; only 23-63% of respondents reported practicing the selected guidelines >75% of the time/opportunities. Lack of self-efficacy, that is, confidence in adequately performing the guidelines, was commonly experienced by respondents. Access to a copy of the guidelines was associated with increased agreement with the recommendations and increased self-efficacy. CONCLUSIONS: Strategies to reduce barriers to adherence to CF infection control guidelines are needed. Strategies could include quality improvement initiatives with enhanced education and skills workshops, sharing successful interventions among CF centers, and linking adherence to improved patient outcomes.

Parents' perspectives on having their children interviewed for research.

We describe parents' perspectives on research interviews with their children with single gene conditions. One hundred forty-two parents were interviewed between 2002 and 2003 in a larger study using a qualitative descriptive design. Two questions from the semi-structured interview guide were used to identify parents' perspectives about future interviews. Almost all of the parents said they would allow an interview with their children, but some parents specified stipulations. These stipulations included: focusing on age-appropriate information, limiting information with child, considering input from parents, and providing a child-oriented environment. Knowing this information, researchers can prepare to work more collaboratively with parents and include them more fully in the research process.

Gaps in pediatric clinician communication and opportunities for improvement.

Teamwork and good communication are central to the provision of high-quality care. A standardized focus-group protocol was used. Analysis assessed emergent themes of patient safety-related effective and problematic clinician communication. Sixty-three focus groups were conducted with clinicians from five Chicago Pediatric Patient Safety Consortium hospitals. Effective and problematic clinician-to-clinician communication themes were described in all focus groups and at each participating hospital. Problematic communication contexts included the communication process for orders, consultations, acuity assessment, management of surgical and medical patients, and the discharge process. Organizational policies and systems leading to patient safety risk included a lack of clear responsibilities and expectations for clinicians and for clinical communication, as well as a lack of a clear chain of responsibility for communication when hierarchical communication barriers affected safe patient care. Results of this investigation highlighted gaps in pediatric clinician communication and opportunities for improvement.

Parents' perceptions of functioning in families having a child with a genetic condition.

In a study of families having a child with a genetic condition, patterns of family functioning were identified through cluster analysis of families with two spouses. Patterns were based on both parents' assessments of family satisfaction and hardiness, as measured respectively by the Family APGAR and Family Hardiness Index. The validity and clinical significance of the clusters were supported by demonstrating that cluster membership distinguished between parental reports of their own quality of life and their child's functional status, as measured by the Quality of Life Index and the Functional Status II, respectively. The clusters were non-categorical in the sense that they did not depend on the type of genetic condition. These findings point to the importance of addressing family functioning as part of genetic counseling.

Family patterns of decision-making in pediatric clinical trials.

The decision-making process related to a child's participation in clinical trials often involves multiple family members. The aim of this study was to compare family patterns of decision-making within and across family units in pediatric clinical trials. Participants for this secondary analysis included 14 families from a larger study of informed consent. Four distinct patterns of decision-making were identified: Exclusionary, informative, collaborative, and delegated. These patterns varied with regard to three dimensions of parents' decision-making goals, child level of involvement, and the parental role. These patterns of decision-making affect how parents and children communicate with health professionals and influence the effectiveness of health care providers interactions with the family related to the decision-making process.

Fluorescence in situ hybridization and chromosome studies after transfusion in newborns: is a waiting period necessary?

BACKGROUND: Delaying chromosome studies after transfusion is common practice in many neonatal intensive care units (NICUs). Yet, no evidence exists to support this practice. PURPOSE: To investigate the effects of filtration and irradiation on chromosome detection, and to evaluate donor chromosome interference after transfusion. METHODS: Packed red blood cells (PRBCs) were evaluated by fluorescence in situ hybridization (FISH) and chromosome analyses. To evaluate donor leukocyte survival, blood was collected from female neonates who received male-donated PRBCs. RESULTS: Irradiated, leukodepleted blood had no Y chromosome detection by FISH. Irradiated, microaggregate filtered blood had Y chromosome detection in all samples by FISH but no metaphase growth. No donor chromosomes were detected in neonates after transfusion. CONCLUSIONS: Delaying chromosome or FISH analysis in transfused neonates who have received irradiated blood is unnecessary.