Transition of care and adherence in patients with Wilson disease

Wilson disease (WD) is commonly diagnosed in childhood and adolescence and medical therapy is lifelong. As patients mature to adulthood, they are transitioned from the care of pediatric specialists into the care of specialists in adult disease. Transition is a process focusing on self-management. Adherence to medication, laboratory tests, diet, and medical follow-up is key to successful long-term outcomes and non-adherence often leads to significant morbidity and even mortality. Patients with WD have unique challenges, as some may have neurologic or psychiatric involvement leading to impaired executive functioning. In this chapter, we discuss the significance of transition, self-management, current transfer of care models, and assessment of adherence specifically in patients with WD.