Sarah, a cheerful 12-year-old, lives with Prader-Willi syndrome (PWS). From infancy, she exhibited constant hunger, constantly seeking food beyond a reasonable amount. Despite frequent, filling meals, her stomach never seemed satisfied. This insatiable hunger, a hallmark of PWS, fueled childhood-onset obesity and constant frustration.
At diagnosis, Sarah’s ghrelin levels were measured, confirming “Ghrelin’s Paradox”: abnormally high levels, even after meals. This explained her relentless hunger despite physical fullness. Further investigations revealed the genetic deletion on chromosome 15 characteristic of PWS, along with blunted leptin signaling (another satiety hormone).
Sarah’s case presented a complex picture. Nutrition counselling aimed to structure her meals while managing her overwhelming hunger cues. Growth hormone therapy was recommended to improve metabolism and build lean muscle. Additionally, cognitive and behavioral therapy helped Sarah understand her PWS’s impact on her eating patterns and develop coping mechanisms to manage her overwhelming hunger urges.
The journey was challenging. Cravings were relentless, testing Sarah’s resolve and requiring constant support. Schoolyard taunts about her weight added to the emotional burden. Yet, Sarah’s determination and the team approach, including parents, therapists, and healthcare professionals, yielded gradual progress. Sarah learned to identify and manage her hunger cues, developing healthier eating habits. Growth hormone therapy improved her body composition, boosting her confidence.
Although Ghrelin’s Paradox remains a lifelong challenge, Sarah’s story showcases the multifaceted impact of PWS and the importance of comprehensive management. With unwavering support, individuals like Sarah can navigate the complexities of PWS and reclaim a sense of control over their lives.