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.

Figure 1

The main portion of one meal.

Figure 2

Examples of actual daily schedule ((a) Japanese; (b) English) and time of exercise ((c) Japanese; (d) English) used to evaluate changes in the patient's daily lifestyle and treatment behavior following discharge. The patient submitted these at her monthly examinations, and information such as specific exercise time was used for team evaluation.

Figure 3

Energy intake recorded by the patient during 40 months after the second hospitalization for treatment guidance, plotted with the results of manual muscle testing (MMT). Changes in the home environment (mother illness) at 5 months after discharge were associated with increased daily energy intake and decrease in quadriceps femoris muscle MMT. Canagliflozin was subsequently started at 10 months after discharge in order to deal with these changes and correct blood glucose levels.

Figure 4

Changes in HbA1c and body weight during the 40 months after the second hospitalization, plotted for treatment guidance. Canagliflozin administration was started at 10 months after discharge in response to changes in energy intake.