Mothers' Experience of Breastfeeding Infants with Phenylketonuria (PKU)

描述（由申请人提供）：本研究的目的是了解母亲如何处理患有苯丙酮尿症（PKU）的母乳喂养婴儿。PKU是一种先天性代谢障碍。在美国，每年大约有160-400名新生儿患有PKU。将母乳喂养纳入PKU的管理尚未得到很好的研究。这导致缺乏针对这一人群母亲的具体母乳喂养指南。具体目标是：1)描述母亲如何在PKU的背景下管理母乳喂养，2)根据家庭管理风格框架（FMSF）确定母乳喂养管理模式。FMSF已被用于描述一些慢性病的家庭管理以及早产儿家庭的哺乳管理。第一个目标是确定母乳喂养并需要补充不含苯丙氨酸配方奶粉的PKU婴儿的母亲的日常管理经验。第二个目标是解决母亲如何同时管理母乳喂养和PKU治疗的责任和活动。这一目标将确定PKU人群中的母亲如何学习管理母乳喂养的模式，以及基于FMSF的PKU治疗的责任和活动。本研究主要采用定性方法来了解母亲喂养患有PKU的婴儿的经历。此外，为了更充分地理解这一主题并提供对家庭更全面的看法，将采用两项措施来收集关于养育子女压力和家庭功能的数据。定性访谈将是主要的数据收集技术。关于父母压力和家庭功能的标准化措施的数据将用于将母乳喂养管理置于整个家庭生活的更广泛背景下。数据将从25-40名母乳喂养患有PKU婴儿的母亲中收集。母亲们将从美国各地招募。本研究将为母乳喂养的PKU患儿的护理提供科学依据。LAY语言：本研究的目的是了解母亲如何处理患有苯丙酮尿症（PKU）的婴儿的母乳喂养。PKU是一种先天性代谢障碍。将母乳喂养纳入PKU的管理尚未得到很好的研究。本研究将确定并描述母乳喂养PKU婴儿的母亲的日常管理经验，并确定影响母亲在坚持PKU管理计划的情况下母乳喂养PKU婴儿成功经验的因素。

DESCRIPTION (provided by applicant): The purpose of this proposed study is to understand how mothers manage breastfeeding infants with the disorder of phenylketonuria (PKU). PKU is an inborn error of metabolism disorder. Approximately 160-400 infants per year are born with PKU in the United States. The incorporation of breastfeeding into the management of PKU has not been well studied. This results in a lack of specific breastfeeding guidelines for mothers in this population. Specific aims are: 1) describe how mothers manage breastfeeding in the context of PKU, and 2) identify patterns of management of breastfeeding based upon the Family Management Style Framework (FMSF). The FMSF has been used to describe family management of a number of chronic conditions as well as lactation management in families of premature infants. The first aim will identify the day-to-day management experiences of mothers' of infants with PKU who are breastfeeding and are required to supplement with phenylalanine-free formula. The second aim addresses how mothers simultaneously manage breastfeeding and the responsibilities and activities of PKU therapy. This aim will identify patterns of how mothers in the PKU population learn to manage breastfeeding and the responsibilities and activities of PKU therapy based upon the FMSF. The proposed study uses a predominantly qualitative approach to provide an understanding of mothers' experiences of breastfeeding infants with PKU. In addition, to more fully comprehend the subject matter and to provide a more comprehensive view of the family, two measures will be used to collect data on parenting stress and family functioning. Qualitative interviews will be the primary data collection technique. Data from the standardized measures on parental stress and family functioning will be used to situate breastfeeding management within the broader context of overall family life. Data will be collected from a sample of 25-40 mothers of breastfeeding infants with PKU. Mothers will be recruited from across the United States. This proposed study will contribute to establishing a scientific basis for the care of breastfeeding infants with PKU. LAY LANGUAGE: The purpose of this study is to understand how mothers manage breastfeeding an infant with the disorder of phenylketonuria (PKU). PKU is an inborn error of metabolism disorder. The incorporation of breastfeeding into the management of PKU has not been well studied. This study will identify and describe the day-to-day management experiences of mothers who are breastfeeding infants with PKU and identify the factors that contribute to the experiences of mothers' success in breastfeeding their infants with PKU while adhering to the PKU management plan.