Diet therapy in patients with rare diseases: a scoping review.

BACKGROUND: This scoping review presents existing research evidence regarding diet therapy in patients with rare diseases (RDs). METHODS: Using the five-stage scoping review framework proposed by Arksey, O'Malley and Levac, we searched the published literature in PubMed, Web of Science, Royal Society of Chemistry, China National Knowledge Infrastructure, VIP Database and Wan Fang Database from January 2010 to November 2022. We selected diet therapy studies on 121 RDs, as categorised by the National Health Commission of China in 2018. Charts for research analysis were developed and used to categorise the data. RESULTS: We ultimately included 34 diet therapy studies from 19 countries and territories for 10 RDs and 3 RD groups. RD diet therapy studies have mainly focused on inborn errors of metabolism (92.3%) and are common in Western countries. Most studies focused on diet therapy methods for RDs (44%). In addition, 29% of studies included diet therapy management, 15% included guidelines for diet therapy and 12% included the impact of diet therapy on patients. CONCLUSIONS: Current diet therapies for RDs lack specificity and present with limited characteristics. Therefore, it is necessary to expand the scope and depth of future research and explore evidence-based recommendations and new diet therapies focused on patient needs and family support to provide a reference for improving the efficacy and safety of diet therapies for RDs.

Experiences of COVID-19 patients in a Fangcang shelter hospital in China during the first wave of the COVID-19 pandemic: a qualitative descriptive study.

OBJECTIVES: This study aimed to examine COVID-19 patients' experiences in a Fangcang shelter hospital in China, to provide insights into the effectiveness of this centralised isolation strategy as a novel solution to patient management during emerging infectious disease outbreaks. DESIGN: This study adopted a qualitative descriptive design. Data were collected by individual semistructured interviews and analysed using thematic analysis. SETTING: This study was undertaken in 1 of the 16 Fangcang shelter hospitals in Wuhan, China between 28 February 2020 and 7 March 2020. Fangcang shelter hospitals were temporary healthcare facilities intended for large-scale centralised isolation, treatment and disease monitoring of mild-to-moderate COVID-19 cases. These hospitals were an essential component of China's response to the first wave of the COVID-19 pandemic. PARTICIPANTS: A total of 27 COVID-19 patients were recruited by purposive sampling. Eligible participants were (1) COVID-19 patients; (2) above 18 years of age and (3) able to communicate effectively. Exclusion criteria were (1) being clinically or emotionally unstable and (2) experiencing communication difficulties. RESULTS: Three themes and nine subthemes were identified. First, COVID-19 patients experienced a range of psychological reactions during hospitalisation, including fear, uncertainty, helplessness and concerns. Second, there were positive and negative experiences associated with communal living. While COVID-19 patients' evaluation of essential services in the hospital was overall positive, privacy and hygiene issues were highlighted as stressors during their hospital stay. Third, positive peer support and a trusting patient-healthcare professional relationship served as a birthplace for resilience, trust and gratitude in COVID-19 patients. CONCLUSIONS: Our findings suggest that, while sacrificing privacy, centralised isolation has the potential to mitigate negative psychological impacts of social isolation in COVID-19 patients by promoting meaningful peer connections, companionship and support within the shared living space. To our knowledge, this is the first study bringing patients' perspectives into healthcare service appraisal in emergency shelter hospitals.

Risk perception, knowledge, information sources and emotional states among COVID-19 patients in Wuhan, China.

BACKGROUND: The rapidly evolving COVID-19 pandemic has become a global health crisis. Several factors influencing risk perception have been identified, including knowledge of the disease, information sources, and emotional states. Prior studies on COVID-19-related risk perception primarily focused on the general public, with little data available on COVID-19 patients. PURPOSE: To investigate COVID-19 patients' risk perception, knowledge of the disease, information sources, and emotional states in the epicenter, Wuhan, during the COVID-19 outbreak in China. METHODS: Data were collected online using self-administered electronic questionnaire developed with reference to previous relevant studies and publications by the World Health Organization. FINDINGS: A higher level of perceived risk was found in relation to COVID-19 as compared to other potential health threats. Knowledge gaps existed regarding transmission and prevention of COVID-19. Additionally, risk perception was negatively related to knowledge and positively related to depressive states. Moreover, social media was a primary source for COVID-19 information, whereas the most trusted sources were health professionals. DISCUSSION: Realistic perception of risk should be encouraged considering both physical and mental health while developing relevant strategies. Furthermore, risk communication needs to be specifically tailored for various target groups, such as the elderly and mentally vulnerable individuals, with the adoption of popular media platforms.

The outbreak of coronavirus disease in China: Risk perceptions, knowledge, and information sources among prenatal and postnatal women.

BACKGROUND: The COVID-19 pandemic has created anxiety among members of the public, including all women over the childbirth continuum, who are considered to be at a greater risk of contracting most infectious diseases. Understanding the perspectives of health care consumers on COVID-19 will play a crucial role in the development of effective risk communication strategies. This study aimed to examine COVID-19-related risk perceptions, knowledge, and information sources among prenatal and postnatal Chinese women during the initial phase of the COVID-19 pandemic. METHODS: A cross-sectional survey design was adopted, and a four-section online questionnaire was used to collect data. Using a social media platform, the online survey was administered to 161 participants during the outbreak of COVID-19 in Nanjing, China, in February 2020. RESULTS: The participants perceived their risk of contracting and dying from COVID-19 to be lower than their risk of contracting influenza, however many of them were worried that they might contract COVID-19. The participants demonstrated adequate knowledge about COVID-19. The three major sources from which they obtained information about COVID-19 were doctors, nurses/midwives, and the television, and they placed a high level of confidence in these sources. There was no significant relationship between the perceived risk of contracting COVID-19 and knowledge about this disease. CONCLUSION: The present findings offer valuable insights to healthcare professionals, including midwives, who serve on the frontline and provide care to pregnant women. Although the participants were adequately knowledgeable about COVID-19, they had misunderstood some of the recommendations of the World Health Organisation.

Development of atraumatic heel-stick procedures by combined treatment with non-nutritive sucking, oral sucrose, and facilitated tucking: a randomised, controlled trial.

BACKGROUND: Preterm infants manifest pain and stress by behavioural agitation and state change. Few studies have explored the effects of combining nonpharmacological interventions, i.e. non-nutritive sucking, oral sucrose, and facilitated tucking, on infants' behaviours across painful procedures. OBJECTIVES: To explore the effects of combined use of three nonpharmacological interventions (non-nutritive sucking, oral sucrose, and facilitated tucking) on infants' pain- and stress-related behaviours during four assessment phases: baseline, intervention, heel stick, and recovery. DESIGN: Prospective, randomised controlled trial. SETTING: Level III neonatal intensive care unit in Taipei. METHOD: A convenience sample of 110 infants (gestational age 27-37 weeks) needing heel sticks was randomly assigned to five combinations of nonpharmacological treatments: (1) routine care, (2) non-nutritive sucking+facilitated tucking, (3) oral sucrose+facilitated tucking, (4) non-nutritive sucking+oral sucrose, and (5) non-nutritive sucking+oral sucrose+facilitated tucking. Outcomes were infants' withdrawal or stress (grimace, limb and trunk extension or squirming) and approach or self-soothing (sucking, sucking search, or mouthing; hand holding or grasping; and hand to mouth, face) behaviours. RESULTS: The frequency of infants' withdrawal behaviours decreased significantly when they received combinations of nonpharmacological interventions before heel stick. Specifically, grimace frequency decreased by 32.2%, 30.6%, 19.7%, and 13.8% in infants receiving oral sucrose+non-nutritive sucking+facilitated tucking, non-nutritive sucking+oral sucrose, oral sucrose+facilitated tucking, and non-nutritive sucking+facilitated tucking, respectively, compared to those receiving routine care across assessment phases. Furthermore, infants' frequency of limb and trunk extension or squirming decreased by 24.0% when they received non-nutritive sucking+oral sucrose+facilitated tucking compared to those receiving routine care. Infants' frequency of approach behaviours did not change significantly across all phases when they received non-nutritive sucking+oral sucrose+facilitated tucking, non-nutritive sucking+oral sucrose, and oral sucrose+facilitated tucking compared to those receiving routine care. CONCLUSIONS: The combined use of nonpharmacological interventions (non-nutritive sucking+oral sucrose+facilitated tucking) effectively reduced the frequencies of infants' withdrawal behaviours, i.e. grimace and limb and trunk extension or squirming. Our results provide evidence supporting clinicians' incorporation of the combined use of facilitated tucking, oral sucrose, and non-nutritive sucking into clinical practice during painful procedures. Heel-stick procedures can be atraumatic when conducted while infants are stable and quiet, appropriately positioned, and stabilised and by offering facilitated tucking, oral sucrose, and non-nutritive sucking before gently sticking the heel and squeezing blood.

A descriptive phenomenology study of newcomers' experience of maternity care services: Chinese women's perspectives.

BACKGROUND: Maternity health care available in Canada is based on the needs of women born in Canada and often lacks the flexibility to meet the needs of immigrant women. The purpose of this study was to explore immigrant Chinese women's experiences in accessing maternity care, the utilization of maternity health services, and the obstacles they perceived in Canada. METHODS: This descriptive phenomenology study used in-depth semi-structured interviews to examine immigrant Chinese women's experiences. Fifteen participants were recruited from the Chinese community in Toronto, Canada by using purposive sampling. The interviews were digitally recorded and transcribed verbatim into written Chinese. The transcripts were analyzed using Colaizzi's (1978) phenomenological method. RESULTS: Six themes were extracted from the interviews: (1) preference for linguistically and culturally competent healthcare providers, with obstetricians over midwives, (2) strategies to deal with the inconvenience of the Canadian healthcare system (3) multiple resources to obtain pregnancy information, (4) the merits of the Canadian healthcare system, (5) the need for culturally sensitive care, and (6) the emergence of alternative supports and the use of private services. CONCLUSIONS: The findings provide new knowledge and understanding of immigrant Chinese women's experiences in accessing maternity health services within a large metropolitan Canadian city. Participants described two unique experiences within the themes: preference for linguistically and culturally competent healthcare providers, with obstetricians over midwives, and the emergence of alternative supports and the use of private services. Few studies of immigrant maternity service access have identified these experiences which may be linked to cultural difference. Further investigation with women from different cultural backgrounds is needed to develop a comprehensive understanding of immigrant women's experiences with maternity care.

The lived experience of Taiwanese mothers of a child diagnosed with adrenoleukodystrophy.

This research was conducted to describe Taiwanese mothers' lived experience of caring for their child diagnosed with adrenoleukodystrophy. Analysis of eight interviews by Colaizzi's method revealed six themes: (1) difficulty confirming the diagnosis; (2) powerlessness toward unsatisfactory treatment; (3) struggles with decisions around carrier testing; (4) guilt about being a carrier; (5) support from family, other parents, and religion; and (6) lack of integrated resources and support. The results suggest the need to raise public and physician awareness about adrenoleukodystrophy via the media and medical education. A central organization to provide parents with comprehensive information is needed.

Psychometric analysis of a Taiwan-version pain assessment scale for preterm infants.

AIM: To determine the inter-rater reliability, internal consistency, construct and concurrent validity and feasibility of the pain assessment scale for preterm infants. BACKGROUND: Repeated exposure to painful procedures has an adverse impact on preterm infants' health outcomes. Although many scales are available for assessing these infants' pain, only a few reliably and validly assess pain and no gold standard has been established in clinical practice. DESIGN: Instrument development and psychometric analysis. METHODS: Preterm infants (n = 60) born 27·6-36·3 weeks gestational age were assessed for pain 3 minutes before (phase I), during (phase II), 3 minutes after (phase III) and the tenth minute after (phase IV) heel-stick procedures. Pain scores were independently coded from video recordings and observations by three trained nurses using our pain-assessment scale, premature infant pain profile and visual analogue scale. RESULTS: Scores on the pain assessment scale for preterm infants differed significantly across four phases of heel-stick procedures (F = 56·86, p < 0·0001). Internal consistency was 0·84 and inter-rater reliability was 0·88-0·93. Scores on our pain scale correlated well with scores on the premature infant pain profile (0·74-0·83) and visual analogue scale (0·72-0·81). CONCLUSIONS: The pain assessment scale for preterm infants integrates all possible pain indicators, with each item modified for clinicians' simple and easy pain measurement to potentially yield different information. Our scale is valid, reliable and feasible for preterm infants with gestational age > 27 weeks. Further examination of the scale's psychometrics is recommended with diverse samples of infants and different painful procedures. RELEVANCE TO CLINICAL PRACTICE: The pain assessment scale for preterm infants clearly defines item scoring and weighting, consistently discriminates different levels of pain and helps nurses to recognise infants' pain. Nurses can easily remember the definition of each item, allowing them to use our scale to evaluate preterm infants' pain at any time and to provide pain-relief interventions when needed.

Husbands' experiences before wives' hysterectomy.

After Caesarean section, hysterectomy ranks as the surgical procedure most frequently performed on women. To most women, the partner's support is vital. Currently, there is a lack of research on the experiences of husbands facing their wife's hysterectomy. The aim of this study was to understand husbands' experiences when their wives were to undergo a hysterectomy. The study used a phenomenological approach and purposive sampling; data were collected at three teaching hospitals in northern Taiwan. Twenty participants were interviewed and data were analyzed by Colaizzi's method. The findings demonstrated that, when their wives were to undergo a hysterectomy, six major themes emerged among the husbands, as follows: (1) limited knowledge about the surgical process; (2) considerations about and adjustments to changes in one's sex life; (3) support in making necessary decisions; (4) difficulty in acquiring information on uterine disorders; (5) ensuring the wife's safety during the operation; and (6) expecting full communication with medical personnel. The findings from this study should contribute significantly to advances in practice, education and research concerning Taiwanese males' experiences as they face their wife's hysterectomy. These findings highlight the significance of the husband's involvement in meeting his wife's needs as regards providing information and communicating. Implications for nursing practice and further research are discussed.

[The effectiveness of the video-based Lamaze method on prenatal mothers' knowledge, attitudes, and practice].

This study was conducted to explore the effectiveness of the video-based Lamaze method on pre-natal mothers' knowledge and attitudes, compared with that of traditional nursing guidelines. Using a quasi-experimental design, women in labor with gestations of 32 weeks or more were divided into four study groups, including two experimental groups (E(1): issued with traditional nursing guidelines and instructed in video-based Lamaze method; and E(2): instructed in video-based Lamaze method), and two control groups (C(1): issued with traditional nursing guidelines and C(2): issued with no guidelines). Before the intervention, E(1) had the highest score for knowledge. Scores for attitude showed no significant difference between the four groups. The experimental groups had higher scores than the control groups in the posttest. With the exception of the scores for attitude (E(1) > E(2)), knowledge (E(1): 13.09 +/- 1.40, E(2): 12.40 +/- 1.17) and management of the labor process (E(1): 119.00 +/- 10.91, E(2): 112.97 +/- 14.33) there were no significant differences between E(1) and E(2) in the posttest. Correlation analysis showed that the higher the scores for prenatal knowledge, the better the performance in prenatal practice, postnatal knowledge, attitude, and management of the labor process. The more positive the prenatal attitude, the better the performance in postnatal knowledge, attitude, and management of the labor process. The higher the scores in prenatal practice, the better the performance in postnatal knowledge, attitude, and management of the labor process. Positive associations were also found between postnatal knowledge, attitude, and management of the labor process. This study showed that the video-based Lamaze method is likely to promote more effectively than traditional guidelines the knowledge, attitudes, and practice of prenatal mothers in relation to giving birth. The use of this method in conjunction with traditional nursing guidelines may be even more effective in relation to maternal attitude.