Ora, a 22-year-old male, was admitted with acute respiratory failure secondary to other complications associated with Prader-willi Syndrome. This syndrome is characterized by hypotonia, growth retardation, and developmental delays. Behavior characteristics included food seeking, tantrums and non compliance during multidisciplinary treatments. When admitted, he presented challenges with behavior along with failure to wean, weight management, glucose stabilization and poor tolerance in therapy. He required maximum assistance with two people to roll left-to-right and sit up on the edge of the bed. Also with Ora’s weight at 366lbs and blood sugars ranging from 291-395 mg/dl, multidisciplinary goals were identified and implemented immediately.
Ora was followed closely by our Pulmonary Medicine Associates (PMA) group, physicians, nursing, respiratory therapy team, dietitian, rehab team, and case management. His progress during his stay varied greatly. Ora continued to have a difficult time weaning on the ventilator. However, he had enough strength to tolerate T-mist during the days in order to participate in therapy. Managing his weight and blood sugars was an ongoing process since elevated blood sugars were multifactorial. As time passed, Ora progressed in therapy and reached his physical therapy and occupational therapy goals. He was able to graduate to the Restorative Nursing Aid program where they focused on maintaining his skill and ambulation. His behavior was controlled by the help of family and staff by keeping Ora occupied with magazines, toys, and physical activity.
At the time of discharge, Ora continued to tolerate T-mist during the day and vent at night. He made significant progress with dietary. Ora was able to drop his weight down to 340 and stabilize his blood sugar around 167-230 mg/dl. He also could proudly transfer from his bed to a chair or toilet with Supervision. And began to participate in activity of daily living including dressing and grooming. He also enjoyed playing in the gym and climbing stairs.
Our case management team worked diligently to have Ora discharged to the appropriate level of
care with the ultimate goal of going back home to his family. We will miss Ora’s smile and carefree
personality. We wish him and his family the best!