Bariatric surgery and semaglutide in a youth with juvenile Huntington disease and severe obesity: a case report.

BACKGROUND

Managing severe obesity in youth with chronic, progressive conditions, such as juvenile Huntington disease, presents unique challenges. Juvenile Huntington disease, is characterized by rapid neurodegeneration, with most patients experiencing a significant decline in motor and cognitive functions within 10-20 years of symptom onset, leading to reduced life expectancy. Obesity further complicates these conditions, reducing quality of life and exacerbating physical, cognitive, and metabolic dysfunctions. A multidisciplinary approach is essential to address these complex issues.

CASE PRESENTATION: A 17-year-old Hispanic male with juvenile Huntington disease and a body mass index of 44 kg/m2 (190% of the 95th percentile) presented for obesity management. He experienced extreme food cravings and difficulty with portion control. After thorough discussions with the patient and his family, and with ethics consultation, a treatment plan was developed that included pharmacological and surgical interventions. Semaglutide was initiated at 0.25 mg weekly, titrating up to 1 mg. After 3 months, his body mass index reduced by 7% (40 kg/m2). Despite this, he continued to experience obesity-related complications, including elevated liver enzymes and obstructive sleep apnea, and underwent laparoscopic sleeve gastrectomy at 18 years old. Six-months postoperatively, his body mass index decreased to 33 kg/m2, liver enzyme levels improved, and his sleep apnea resolved. Additionally, he reported improved physical activity and enhanced quality of life.

CONCLUSION

This case illustrates the ethical considerations and challenges of managing severe obesity in juvenile Huntington disease. The decision to proceed with bariatric surgery prioritized improving quality of life, given the patient's limited life expectancy. The use of a multimodal treatment approach, including semaglutide and laparoscopic sleeve gastrectomy, significantly improved both physical health and psychosocial well-being. This case highlights the importance of shared decision-making in managing complex obesity cases in pediatric populations with neurodegenerative diseases.