Early liver transplantation is essential for familial amyloidotic polyneuropathy patients' quality of life

Abstract

Nineteen patients, who had undergone liver transplantation for familial amyloidotic polyneuropathy, had answered a quality of life questionnaire including 61 questions on somatic and mental symptoms, social aspects of life, confidence and satisfaction before, one year, and two years after transplantation.

We found that patient satisfaction was generally good two years or more after the transplantation. Most of the patients were very or quite satisfied with the result. All of them had the drive to go on and felt hopeful about the future. However, on the second follow-up, 37% of the patients noted that they felt more insecure in their everyday life and there was a significant difference between the two assessments. The diarrhea score became worse between one and two years after the transplantation and was closely related to the duration of the gastrointestinal symptoms and to the duration of the disease before transplantation. The mental symptoms also increased significantly between the evaluations and this related to the severity of the somatic symptoms.

Our conclusion is that liver transplantation should be performed before advanced somatic symptoms start to develop in order to improve the patients' chances of a good quality of life following liver transplantation.

Key Words:

• Amyloidosis – hereditary
• inborn errors of metabolism
• quality of life
• transplantation – liver