Development of the parental needs scale for rare diseases: a tool for measuring the supportive care needs of parents caring for a child with a rare disease.

BACKGROUND: Children and families affected by rare diseases have received scant consideration from the medical, scientific, and political communities, with parents' needs especially having received little attention. Affected parents often have limited access to information and support and appropriate health care services. While scales to measure the needs of parents of children with chronic illnesses have been developed, there have been no previous attempts to develop a scale to assess the needs of parents of children with rare diseases. OBJECTIVE: To develop a scale for measuring the supportive care needs of parents of children with rare diseases. METHOD: A total of 301 responses to our Parental Needs Survey were randomly divided into two halves, one for exploratory factor analysis and the other for confirmatory factor analysis (CFA). After removing unsuitable items, exploratory factor analysis was undertaken to determine the factor structure of the data. CFA using structural equation modeling was then undertaken to confirm the factor structure. RESULTS: Seventy-two items were entered into the CFA, with a scree plot showing a likely four-factor solution. The results provided four independent subscales of parental needs: Understanding the disease (four items); Working with health professionals (four items); Emotional issues (three items); and Financial needs (three items). The structural equation modeling confirmed the suitability of the four-factor solution and demonstrated that the four subscales could be added to provide an overall scale of parental need. CONCLUSION: This is the first scale developed to measure the supportive care needs of parents of children with rare diseases. The scale is suitable for use in surveys to develop policy, in individual clinical assessments, and, potentially, for evaluating new programs. Measuring the supportive care needs of parents caring for a child with a rare disease will hopefully lead to better physical and psychological health outcomes for parents and their affected children.

The supportive care needs of parents with a child with a rare disease: results of an online survey.

BACKGROUND: Parents caring for a child affected by a rare disease have unmet needs, the origins of which are complex and varied. Our aim was to determine the supportive care needs of parents caring for a child with a rare disease. METHODS: An online survey was developed consisting of 45 questions (108 items) and separated into six domains. The survey included questions about perceived level of satisfaction with receiving care, experiences and needs of providing daily care, the impacts of disease on relationships, the emotional and psychological burdens of disease, and parents overall satisfaction with the support received. RESULTS: Three-hundred and one parents from Australia and New Zealand completed the survey; 91 % (n = 275/301) were mothers, with 132 distinct rare diseases being reported. Fifty-four percent (n = 140/259) of parents were dissatisfied with health professionals' level of knowledge and awareness of disease; 71 % (n = 130/183) of parents felt they received less support compared to other parents. Information regarding present (60 %, n = 146/240) and future services (72 %, n = 174/240) available for their child were considered important. Almost half of parents (45 %, n = 106/236) struggled financially, 38 % (n = 99/236) reduced their working hours and 34 % (n = 79/236) ceased paid employment. Forty-two percent (n = 99/223) of parents had no access to a disease specific support group, and 58 % (n = 134/230) stated that their number of friends had reduced since the birth of their child; 75 % (n = 173/230) had no contact with other parents with a child with a similar disease, and 46 % (n = 106/230) reported feeling socially isolated and desperately lonely. Most frequent emotions expressed by parents in the week prior to completing the survey were anxiety and fear (53 %, n = 119/223), anger and frustration (46 %, n = 103/223) and uncertainty (39 %, n = 88/223). CONCLUSION: This study is the first to develop an online survey specifically for use with parents to investigate their supportive care needs across a large and diverse group of rare diseases. The findings highlight that parents with a child with a rare disease have common unmet needs regardless of what disease their child has. Such information may allow health providers to improve child outcomes through improving parental supportive care.

The Supportive Care Needs of Parents With a Child With a Rare Disease: A Qualitative Descriptive Study.

There are few studies that exist which focus specifically on parents with a child with a rare disease. The purpose of this study was to better understand the lived experiences and supportive care needs (SCN) of parents caring for a child across a spectrum of rare diseases. A qualitative descriptive approach was used to guide the research, and four semi-structured focus group interviews were conducted with 23 parents (17 mothers and 6 fathers). Participants described 'feeling boxed-in outside the box' due to a number of limitations unique to their child's disease, daily practical challenges in providing care and the various relational impacts of caring for a child with a rare disease were discussed. The results from this study help to give clearer direction for health professionals on where to focus future efforts in better meeting the supportive care needs of parents and their child with a rare disease.

Factors associated with domperidone use as a galactogogue at an Australian tertiary teaching hospital.

BACKGROUND: Insufficient milk supply is 1 of the most commonly reported reasons for discontinuation of infant breastfeeding. Although domperidone is often used to improve milk supply, knowledge of factors associated with the use of domperidone in clinical practice is scarce. OBJECTIVE: This study aimed to examine factors associated with the use of domperidone as a galactogogue at the Women's and Children's Hospital (WCH), Adelaide. METHODS: A retrospective cohort study was conducted, involving women who delivered live-born singletons (N = 21 914) at the WCH between January 2004 and December 2008. Women dispensed domperidone were identified using WCH pharmacy dispensing records. Maternal and infant clinical data were obtained from the WCH Perinatal Statistics Collection. Relationships between maternal/infant demographic and clinical variables and the use of domperidone were examined through univariate and multivariate logistic regression analyses. RESULTS: Key factors associated with an increased likelihood of women receiving domperidone were increasing maternal age (adjusted odds ratio [aOR] = 1.04; 95% confidence interval [CI], 1.03-1.06), maternal obesity (aOR = 1.41; 95% CI, 1.16-1.77), primiparity (aOR = 1.94; 95% CI, 1.63-2.30), delivery by cesarean section (aOR = 1.31; 95% CI, 1.10-1.55), preterm birth (aOR = 3.54; 95% CI, 2.79-4.50), and neonatal hospitalization (aOR = 2.51; 95% CI, 2.01-3.14). In addition, statistically significant trends were observed between increasing socioeconomic status and year of delivery and an increased likelihood of women receiving domperidone (all Ps < .004). CONCLUSION: These findings are of clinical importance as they not only reinforce previous findings regarding risk factors for women experiencing lactation difficulties but also highlight the need for improved research regarding the rational and efficacious use of domperidone to improve breastfeeding outcomes.