Behavior Modification Maintenance with Long-Term Blood Glucose and Weight Management in Prader-Willi Syndrome Complicated with Diabetes: Team Management Approach Combined with Pharmacological Treatment.

ABSTRACT

The patient was a 40-year-old woman, who had been diagnosed with Prader-Willi syndrome (PWS) at 1 year of age and type 2 diabetes at 27 years of age. At 34 years of age, she was hospitalized to start insulin therapy and receive guidance on treatment. During the next 6 months and through regular once-monthly outpatient clinic visits, the blood glucose level was relatively stabilized although body weight gradually increased. Two years following discharge, the blood glucose level became unstable, and she was hospitalized again to receive guidance on treatment. A team medicine-based approach was established upon hospitalization. The basic treatment was unchanged (insulin, diet, and exercise). The approach taken by the team included understanding the characteristics of PWS by all team members, clear definition of treatment goals, positive evaluation of the patient, and maintenance of the patient's motivation for treatment. Anxiety and stress related to mother's illness dampened motivation and adherence to treatment, but the addition of appropriate pharmacological treatment helped in rapid recovery of motivation to adhere to the treatment protocol. At 3 years after discharge, HbA1c is maintained at around 6%, and body weight continues to fall. Our protocol of the combination of a team medicine approach with appropriately timed pharmacological intervention could probably be applied to not only type 2 diabetes in PWS but also the management of patients with poorly controlled type 2 diabetes.