Disordered Eating and Body Esteem Among Individuals with Glycogen Storage Disease

Abstract

Glycogen storage disease (GSD) is an inherited disorder that requires a complex medical regimen to maintain appropriate metabolic control. Previous research has suggested the disease is associated with decreased quality of life, and clinical experience suggests that patients are at risk for disordered eating behaviors that may significantly compromise their health. The current study assessed eating attitudes, eating disorder symptoms, and body image among 64 patients with GSD ranging from 7-52 years old (M ¼ 18.5 years old). About half the participants were male (n ¼ 33, 51.6%). Most participants were diagnosed with GSD Type I (n ¼ 52, 81.3%). Quantitative and qualitative analyses were utilized. Results indicated that 14.8% of children and 11.1% of adolescents/ adults with GSD met the clinical cutoff for dysfunctional attitudes toward eating, suggesting high likelihood for presence of an eating disorder. However, traditional eating disorder symptoms (e.g., binging, purging, fasting, etc.) were less prevalent in the GSD sample compared to population norms (t ¼ À6.45, p < 0.001). Body esteem was generally lower for both children and adolescents/ adults with GSD compared to population norms. These results were consistent with interview responses indicating that GSD patients experience negative feedback from peers regarding their bodies, especially during childhood and adolescence. However, they reported growing acceptance of their bodies with age and reported less negative attitudes and behaviors. Assessing mental health, including symptoms of disordered eating and low body esteem, among individuals with GSD should be an important component of clinical care.

Feeding Difficulties and Orofacial Myofunctional Disorder in Patients with Hepatic Glycogen Storage Diseases

“…Within this context, we identified that several participants in our sample did not eat meals as a family and found an association between GSD type I and feeding difficulty. These findings may be related to the high overall prevalence of feeding difficulty in the sample, as individuals with feeding problems often do not eat at the family table (Dunitz-Scheer et al 2009;Benjasuwantep et al 2013), as well as to the disease itself and its treatment, since patients with hepatic GSD need to eat at prescribed times, which may diverge from family mealtimes (Rake et al 2002;Weinstein and Wolfsdorf 2002;Flanagan et al 2015). In the case of GSD type I, dietary control is associated with even greater restrictions and need for even more frequent intake of uncooked cornstarch to maintain normoglycemia and prevent secondary metabolic disorders than in other GSD subtypes (Rake et al 2002;Flanagan et al 2015).…”

“…This population is particularly susceptible to FDs, as both the disease and its treatment are associated with risk factors for the development of feeding disorders. Poor acceptance of dietary plans by patients and families is also a concern (Correia et al 2008;Santos et al 2014;Flanagan et al 2015). Within this context, the present study aimed to investigate FDs, OMDs, and olfactory and gustatory perception in Brazilian patients with hepatic GSDs.…”

“…Treatment can include restricted intake of simple carbohydrates (fructose, maltose, glucose, lactose, galactose), administration of uncooked cornstarch several times a day (including overnight, as some patients do not tolerate fasting for more than 3 h), and management of clinical and laboratory parameters (Weinstein and Wolfsdorf 2002;Chen and Kishnani 2012;Derks and Smit 2015). Sometimes, due to the dietary restrictions and continuous feeding required, tube feeding is indicated to maintain normoglycemia and proper metabolic control (Rake et al 2002;Weinstein and Wolfsdorf 2002;Flanagan et al 2015).…”

Impact of glycogen storage disease type I on adult daily life

“…Flanagan et al studied eating attitudes, eating disorder symptoms, and body image among 64 patients with GSD ranging from 7-52 years and found a lower body esteem in children, adolescents and adults with GSD compared to population norms [14]. Interestingly, patients reported growing acceptance of their bodies with age associated with less negative attitudes and behaviours in adulthood.…”

“…Living with a chronic disease might not only impact QoL due to the disorder itself, but also due to the necessary treatment, which may be a major challenge. For patients with GSD I this includes frequent meals, strict planning of activities, loss of spontaneity as well as sleep disturbances due to night-time interruptions for nocturnal corn starch intake [14,18]. When asked for their opinion about the severity of GSD I, most patients ranked GSD I as a disease with moderate severity and disease burden, but rated the challenges of dietary treatment as rather high.…”