Quality of life after liver transplantation in patients with familial amyloidotic polyneuropathy

Liver transplantation in familial amyloidotic polyneuropathy is associated with considerable risks for the patient and produces only a slight improvement in neurological function. The aims of the study were to assess these patients' quality of life after transplantation and their satisfaction with the outcome of the operations and also to identify the physical and mental symptoms that had some impact on their quality of life. Twelve patients, who had undergone liver transplantation for familial amyloid polyneuropathy (Portuguese) answered a questionnaire including 61 questions on somatic and mental symptoms, social aspects, information, confidence and satisfaction. Eight of the patients (66%) were completely satisfied with the outcome of the transplantation. Furthermore, ten patients (83%) reported a distinct or considerable improvement in spite of persistent symptoms from their lower and upper extremities after the transplantation. Functional impairment of the upper extremities was more closely related to patients' quality of life than impairment of the lower extremities. Thus, the patients were quite satisfied with the outcome of the transplantation. The patients' assessment of their functional status after the transplantation did not correlate significantly with the physicians' pre-operative assessment; a better pre-transplantation assessment of the patients' functional status, especially hand function, is needed.