Living with Cerebrotendinous Xanthomatosis: Patient, Caregiver, and Expert Perspectives.

In this article, patients with cerebrotendinous xanthomatosis (CTX) and caregivers detail their experience with lifelong symptoms, diagnosis, treatment and efficacy, and ongoing disease management. One patient and four caregivers describe the challenges associated with pursuing a correct diagnosis for years before testing confirmed a CTX diagnosis. They also detail their ongoing struggles and desire for greater access to physicians with CTX knowledge and to reliable online resources to continue their education about the disease and strategies for symptom management. The expert perspective is a direct response by three CTX researchers, including physicians who are treating patients with CTX in the United States and experts whose laboratories provide genetic and biochemical testing for CTX. They respond to many of the patient and caregiver concerns, including steps that are being taken to identify CTX earlier and provide access to confirmatory diagnostic testing sooner, and suggest the best online resources for CTX-related information and access to webinars and support groups. While the expert perspective is a direct response to the patient and caregiver authors' CTX journeys, it should be beneficial to any patient with CTX or their caregivers.

Intravascular thrombophlebitis related to the peripheral infusion of amiodarone and vancomycin.

Patients on a telemetry unit experienced an increase in thrombophlebitis in 2004. The purpose of this research was to determine if peripheral IV amiodarone and vancomycin influenced the incidence of thrombophlebitis in an adult cardiothoracic population. Amiodarone phlebitis rates range up to 27%. In December 2004, Pharmacy diluted the amiodarone concentration to 600 mg/500 ml. By 2005, data demonstrated a consistent decrease in the incidence of thrombophlebitis. However, related to institutional policies and patient safety concerns, the amiodarone infusion concentration was reversed back to 900 mg/500 ml in October 2005. Thrombophlebitis increased after the return to a more concentrated amiodarone IV solution. Vancomycin infusion administration did not change during this time period. A retrospective chart review and observational, before and after study, demonstrated a correlation between amiodarone concentration and the incidence of thrombophlebitis. Vancomycin infusions appeared to prevent peripheral thrombophlebitis in the study population. Data was compelling and resulted in the institution standardizing the more dilute amiodarone IV concentration.