Voice rehabilitation and quality of life in laryngectomized patients.

BACKGROUND: Total laryngectomy represents the surgical procedure necessary for the treatment of some advanced neoplasms of the hypopharyngeal-laryngeal district and involves strong functional, physical and emotional repercussions. This research investigated the way in which the rehabilitation methods, used to improve the communicative needs of laryngectomized patients, influence their perceived quality of life. METHOD: The questionnaires "V-RQoL" and "SECEL" were administered to 45 patients divided into four groups on the basis of the type of vicarious voice: group TE (27 patients), group E (7 patients), group EL (2 patients), group NV (9 patients). RESULTS: Patients using electrical or tracheo-esophageal prostheses reported a better quality of life than patients with an erythromophonic voice. Regarding postoperative satisfaction, the group with esophageal voice was the most satisfied. CONCLUSIONS: The results lead us to emphasize the importance of preoperative counseling to make the patient as aware as possible of his future condition. KEY WORDS: Cancer, Laryngectomy, Vicarious Voice, Voice Rehabilitation, Quality of Life.

Improvement of dysphagia in a child affected by Pompe disease treated with enzyme replacement therapy.

AIM: Dysphagia is a known complication in Pompe Disease (PD), a severe metabolic myopathy due to alpha-glucosidase deficiency. Enzyme replacement therapy (ERT) with alglucosidase alfa is the only approved therapy for PD. Presently no data are available on the effects of ERT on dysphagia in PD patients. The aim of this work is to evaluate the course of this complication in a 6 years old boy affected by PD after treatment with ERT. METHODS: Dysphagia was assessed by Videofluoroscopic Swallowing Study (VFSS) at baseline, before the start of ERT and after 36 months of therapy. We used the Dysphagia Severity Rating Scale (DSS) to define the severity grade of dysphagia. RESULTS: VFSS performed at baseline revealed complete incoordination of oral stage swallowing which was classified as a grade 1 dysphagia according to DSS. After 36 months of treatment VFSS revealed normal swallowing, classified as grade 0 by DSS. CONCLUSION: Our results suggest that ERT is effective in improving dysphagia. VFSS may be a useful tool to investigate and monitor swallowing disorders in patients affected by PD.