Reproductive intentions in mothers of young children with sickle cell disease.

BACKGROUND: Sickle cell disease (SCD) is an autosomal recessive hemoglobinopathy associated with morbidity and mortality. We sought to understand family planning intentions of parents of young children with SCD including the awareness of three reproductive options (adoption, in vitro fertilization with egg/sperm donation [IVFD], in vitro fertilization [IVF] with preimplantation genetic testing [IVF/PGT]) to decrease the risk of having a child with SCD. PROCEDURE: Qualitative, semistructured, one-on-one interviews with 18 female parents of young children with SCD at an urban, tertiary care pediatric hospital. RESULTS: Half of the parents knew their hemoglobinopathy status or their partner's status before pregnancy. Eight parents chose to have no further children because of fear of SCD in another child. Awareness of reproductive options prior to study enrollment was limited. After brief introduction, 7 parents would consider adoption, 2 IVFD, and 10 IVF/PGT. Desire for a biological child, fear of parental jealousy, ethical or religious considerations, and cost affected the acceptability of these options. Participants universally wanted information about reproductive options available to others prior to pregnancy. CONCLUSIONS: There is limited awareness and variable acceptability of alternative reproductive options available to decrease the risk of a future child having SCD. Participants universally endorsed the need for education regarding hemoglobinopathy status, SCD, and reproductive options prior to pregnancy because for many participants having a child with SCD affected their reproductive intentions. Educational interventions to ensure informed reproductive decision making should be sensitive to desires for a biological child, and ethical and financial considerations.

Further Evidence of Inadequate Quality in Lateral Flow Devices Commercially Offered for the Diagnosis of Rabies.

As a neglected zoonotic disease, rabies causes approximately 5.9 × 104 human deaths annually, primarily affecting low- and middle-income countries in Asia and Africa. In those regions, insufficient surveillance is hampering adequate medical intervention and is driving the vicious cycle of neglect. Where resources to provide laboratory disease confirmation are limited, there is a need for user-friendly and low-cost reliable diagnostic tools that do not rely on specialized laboratory facilities. Lateral flow devices (LFD) offer an alternative to conventional diagnostic methods and may strengthen control efforts in low-resource settings. Five different commercially available LFDs were compared in a multi-centered study with respect to their diagnostic sensitivity and their agreement with standard rabies diagnostic techniques. Our evaluation was conducted by several international reference laboratories using a broad panel of samples. The overall sensitivities ranged from 0% up to 62%, depending on the LFD manufacturer, with substantial variation between the different laboratories. Samples with high antigen content and high relative viral load tended to test positive more often in the Anigen/Bionote test, the latter being the one with the best performance. Still, the overall unsatisfactory findings corroborate a previous study and indicate a persistent lack of appropriate test validation and quality control. At present, the tested kits are not suitable for in-field use for rabies diagnosis, especially not for suspect animals where human contact has been identified, as an incorrect negative diagnosis may result in human casualties. This study points out the discrepancy between the enormous need for such a diagnostic tool on the one hand, and on the other hand, a number of already existing tests that are not yet ready for use.

Parental Attitudes Towards Prenatal Genetic Testing For Sickle Cell Disease.

Uptake of prenatal genetic testing (PGT) is low among those with sickle cell disease (SCD). This study evaluated the association of knowledge and attitudes towards prenatal genetic counseling (PGC), awareness of posttesting intervention options and omission bias with attitudes towards PGT. In addition, we explored changes among knowledge, attitudes, and awareness of options following exposure to an educational, clinical vignette among parents of children with SCD. Parents (n=44) completed a questionnaire and an educational, clinical vignette presenting a detailed account of a pregnant woman with sickle cell trait seeking PGT and PGC was read to each participant. t Tests, Spearman correlations, multivariable regressions, and moderation/mediation analyses were used. More positive attitudes towards PGC (P=0.01), lesser tendency of omission bias (P<0.01) and private insurance (P=0.04) were significant correlates of more positive attitudes towards PGT. Omission bias mediated the relationship of attitudes towards PGC and attitudes towards PGT (95% confidence interval: 0.13, 3.03). Awareness of options (P=0.02), knowledge of PGC (P=0.01) and knowledge of PGT (P=0.01) significantly improved after exposure to the clinical vignette. Patients and families with SCD can benefit from education about the importance of prenatal diagnosis to improve attitudes, address omission bias and promote more informed decisions of PGT.

Acceptability of In Utero Hematopoietic Cell Transplantation for Sickle Cell Disease.

BACKGROUND: In utero hematopoietic cell transplantation (IUHCT) has curative potential for sickle cell disease (SCD) but carries a risk of fetal demise. METHODS: We assessed the conditions under which parents of children with SCD and young adults with SCD would consider IUHCT in a future pregnancy, given a 5% fixed risk of fetal demise. Participants were randomized to consider a hypothetical cure rate (20%, 40%, or 70%). Subsequently, cure rate was either increased or decreased depending on the previous answer to reveal the lowest acceptable rate. Participants also completed the Pediatric Research Participation Questionnaire (PRPQ) and an omission scale. RESULTS: Overall, 74 of 79 (94%) participants were willing to consider IUHCT, and 52 (66%) participants accepted IUHCT at a cure rate of 40%, the estimated rate of therapeutic mixed chimerism. Participants with higher scores on the PRPQ perceived benefits scale were more likely to participate at lower cure rates (OR 1.08, p=0.007) and participants with a greater degree of omission bias were less likely to participate at lower cure rates (OR 0.83, p=0.04). Demographics and SCD severity were not significantly associated with acceptability of IUHCT. CONCLUSION: This study suggests that the majority of parents >and young adults would consider IUHCT under expected therapeutic conditions.

Adherence to prompt fever evaluation in children with sickle cell disease and the health belief model.

BACKGROUND: Children with sickle cell disease (SCD) are at increased risk of death from invasive bacterial infections. Emergent evaluation of fever allows early treatment of potentially fatal infections. Limited data exist regarding caregiver adherence to physician recommendations of prompt medical evaluation of fever in children with SCD. Better understanding of parental behavior around fever management may inform improved models for support in families of children with SCD. PROCEDURE: Cross-sectional survey based on health belief domains, Wake Forest trust scales, and self-reported adherence among 163 caregivers of children with SCD during routine hematology visit. RESULTS: Fifty-five percent of caregivers were adherent to fever evaluation recommendations as defined by "always" seeking medical evaluation of fever in their child with SCD. Perceived susceptibility to fever/infection, benefits of prompt evaluation, and cues to action were significantly different between those who adhere to recommendations versus those who do not. Twenty-five percent believe their child does not need antibiotics with every fever whereas 17% believe their child does not need evaluation of fever after immunizations. Fifty-seven percent report their employer understands missing work whereas 25% report concern regarding cost of evaluation. Trust in their child's hematologist and medical profession was high (composite scores 23.4/25 and 21/25, respectively). CONCLUSION: Despite a high degree of agreement in importance of fever evaluation and high levels of trust, many caregivers do not consistently seek care when their child has a fever. Future studies should address additional barriers to seeking emergency care in children with SCD and fever.

NewYork-Presbyterian Hospital: translating innovation into practice.

BACKGROUND: NewYork-Presbyterian (NYP) Hospital, a 2,242-bed not-for-profit academic medical center, was formed by a merger of The New York Hospital and The Presbyterian Hospital in the City of New York. It is also the flagship for the NewYork-Presbyterian Healthcare System, with 37 acute care facilities and 18 others. OVERALL APPROACH TO QUALITY AND SAFETY: The hospital embeds safety in the culture through strategic initiatives and enhances service and efficiency using Six Sigma and other techniques to drive adoption of improvements. Goals are selected in alignment with the annual strategic initiatives, which are chosen on the basis of satisfaction surveys, patient and family complaints, community advisory groups, and performance measures, among other sources. USE OF INFORMATION TO SET AND EVALUATE QUALITY GOALS AND PRIORITIZE INITIATIVES: A new business intelligence system enables online, dynamic analysis of performance results, replacing static paper reports. Advanced features in the clinical information systems include computerized physician order entry; interactive clinical alerts for decision support; a real-time infection control tracking system; and a clinical data warehouse supporting data mining and analysis for quality improvement, decision making, and education. APPROACH TO ADDRESSING THE SIX IOM QUALITY AIMS: To achieve clinical, service, and operational excellence, NYP focuses on all Institute of Medicine quality aims.

Applied informatics for quality assessment and improvement.

In our hospital, transition planning for order entry and clinical documentation has presented an opportunity for process changes and the ability to capture quality initiatives into data warehouses, where they can be utilized for building evidence-based practice. For example, the order entry and clinical documentation system allows for data retrieval of performance measures set by organizations including the Joint Commission on Accreditation of Healthcare Organizations, Centers for Medicare & Medicaid Services, and National Quality Forum. These data will reside in one system where they can be mined and extracted for reporting. Details of this magnitude are crucial when developing a CPR that will serve as the primary data source of clinical information. As we continue to seek IT solutions to improve patient safety and provide quality care, the use of informatics as a foundation in quality programs will provide the structure and database needed to support evidence-based practice at the point-of-care and reduce potential for error.