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Factors affecting quality of life of caregivers of children with glycogen storage disease type 1

Carroll, Jennifer (2018) Factors affecting quality of life of caregivers of children with glycogen storage disease type 1. Master's Thesis, University of Pittsburgh. (Unpublished)

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Glycogen storage disease (GSD) is a family of inherited metabolic disease (IMD), of which the most common type is autosomally inherited GSD type 1 (GSD1). The symptoms of GSD1, which vary among patients, are primarily hepatic in nature, though a multitude of other body systems are involved to an extent which is dependent on level of metabolic control in the patient. Treatment of the disease is rigorous and involves frequent feeds to avoid life-threatening hypoglycemic episodes, avoidance of certain sugars, and in many, consumption of uncooked cornstarch between meals to help maintain near-normal blood glucose levels. While missing scheduled feeds can have dire consequences up to and including seizures, brain damage, and death, caregivers of children with GSD1 face risks of not only undertreatment, but also overtreatment of the disease. As a rare disease, GSD1 and its management have not been well-studied in terms of effect on the quality of life (QOL) of parents caring for affected children. We administered to GSD1 caregivers the Pediatric Inventory for Parents (PIP), PROMIS Emotional Distress-Anxiety Short Form 8a measure (PROMIS), and a novel survey of disease management, mental healthcare usage, and social media usage to add to the literature a more complete understanding of caregiver QOL. Caregivers had levels of distress higher than what has previously been published and were largely interested in seeking mental healthcare for discussion of caregiver-related challenges. Distress was driven mostly by the domain of Emotional Distress and less so by Medical Care as evidenced by the PIP and further supported by caregiver report of overall comfort with the dietary management of GSD1. Social media was reported as an overall positive support system and a resource for making medical decisions, though may be associated with increased anxiety in some caregivers. Increased support for caregiver QOL, especially in terms of emotional wellbeing, is indicated by the results of this study. The goals and results of this study demonstrate public health relevance by assessment of unique challenges facing the GSD1 caregiver population and contributing to the assurance that these caregivers are linked with necessary services and competent healthcare providers.


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Item Type: University of Pittsburgh ETD
Status: Unpublished
CreatorsEmailPitt UsernameORCID
Carroll, Jenniferjep155@pitt.edujep1550000-0002-1087-8664
ETD Committee:
TitleMemberEmail AddressPitt UsernameORCID
Thesis AdvisorWalsh Vockley,
Committee MemberGrubs,
Committee MemberEl-Gharbawy,
Committee MemberBear,
Date: 18 September 2018
Date Type: Publication
Defense Date: 12 June 2018
Approval Date: 18 September 2018
Submission Date: 1 June 2018
Access Restriction: 2 year -- Restrict access to University of Pittsburgh for a period of 2 years.
Number of Pages: 147
Institution: University of Pittsburgh
Schools and Programs: School of Public Health > Genetic Counseling
Degree: MS - Master of Science
Thesis Type: Master's Thesis
Refereed: Yes
Uncontrolled Keywords: glycogen storage disease; gsd; gsd1; von gierke disease; inherited metabolic disorders; imd; inborn errors of metabolism; iem; genetics; quality of life; caregiver
Date Deposited: 18 Sep 2018 13:05
Last Modified: 18 Sep 2022 05:15


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