Introduction

SINGAPORE – For growing teens, having a big appetite is typical, but for those with Prader-Willi Syndrome (PWS) like 17-year-old Teo Zi Yee and 13-year-old David Lucas Soo, hunger is an insatiable beast that can never be tamed. These children are faced with a unique and challenging neurodevelopmental condition where the brain's 'fullness switch' remains permanently off, leading to constant feelings of starvation and an overriding compulsion to eat.

Understanding Prader-Willi Syndrome

Prader-Willi Syndrome arises due to missing or malfunctioning genetic material inherited from the father. This condition is characterized by uncontrollable hunger, often resulting in severe overeating and, consequently, rapid weight gain. As noted by Associate Professor Angeline Lai, a senior consultant at KK Women's and Children's Hospital, this pattern frequently leads to morbid obesity, accompanied by serious complications including obstructive sleep apnea, diabetes, and hypertension.

"Controlling their food intake is critical," Prof Lai explains, noting that many parents resort to locking kitchen cabinets and pantries to safeguard against excessive eating. "Early diagnosis is crucial as it allows parents to foster healthy eating habits right from the start."

Broader Implications of PWS

However, PWS is not solely about food cravings; it encompasses a broad spectrum of physical, cognitive, and behavioral challenges. Children with PWS may experience developmental delays, sleep disorders, and a wide range of unique strengths and weaknesses. Their educational paths vary, with some attending mainstream schools while others require special education.

Prevalence and Treatment

Statistics reveal that PWS affects between one in 10,000 and one in 30,000 births globally, impacting individuals across all genders and ethnic backgrounds. In Singapore, KK Women’s and Children’s Hospital identifies one to two new cases of PWS each year and is equipped with the nation’s only facility offering genetic testing for this syndrome.

To manage their growth and health, children with PWS typically receive growth hormone treatment, which has the potential to increase height and lean body mass while reducing fat. Early intervention, alongside a well-managed diet, offers hope in mitigating obesity and enhancing physical and cognitive development.

Personal Stories

When Zi Yee was born, she displayed typical symptoms of PWS, being floppy and lethargic. Her parents, Lynn Er and Teo Wee Kiat, were initially anxious about her condition, leading them to conduct a DNA test. After learning of Zi Yee's diagnosis, they faced the daunting challenges ahead, including tube-feeding due to her inability to coordinate her suck and swallow reflex.

"As parents, our perspectives changed dramatically. We had to adjust our expectations and come to terms with our new reality," Lynn shared, reflecting on their journey. Zi Yee's condition has resulted in developmental delays, scoliosis, and the need for regular medical care, further complicating family life.

David's story is similarly poignant, diagnosed at nine months old after demonstrating low muscle tone and a voracious capacity for food. His grandmother, Linda, and mother, Nadja Schaumann, describe the ongoing challenge of managing his increasing appetite, especially as he navigates the changes of puberty. To counteract his relentless hunger, they promote active engagement through sports and other activities, emphasizing healthy meal choices.

Family Support and Future Perspectives

Despite their differences, both children exhibit a deep connection with their families who are dedicated to providing support while grappling with the realities of PWS. Prof Lai warns that adults with PWS often face serious health issues related to obesity, underscoring the urgency for continuous care and support.

In light of these challenges, the parents have established trust funds to secure their children's futures. Their journeys have sparked dialogue on necessary changes within society, such as job opportunities that do not exacerbate food-related impulses and accessible facilities for individuals with disabilities.

"Creating an all-inclusive environment requires not just understanding but actionable change. We need facilities that accommodate the needs of those who are disabled, particularly adults requiring personal assistance," Lynn emphasized.

Conclusion

With ongoing research and growing awareness, the hope for better management and societal integration of individuals with Prader-Willi Syndrome continues to gain momentum, illuminating paths toward understanding, acceptance, and better care for those caught in the web of insatiable hunger.