The life expectancy of untreated patients with TTR-FAP averages 10 years from symptom onset, during which time there is a progressive decline in neurologic function.2,3,5 While initial symptoms may be mild, TTR-FAP progresses steadily, and symptoms become more severe over time.11
TTR-FAP leads to an irreversible and progressive deterioration in neurologic function16
During the early years of TTR-FAP, sensory, motor, and autonomic symptoms are typically mild in severity, with limited impact on Activities of Daily Living (ADL) and no impact on ambulation.11
As TTR-FAP progresses, the sensorimotor and autonomic symptoms become severe. ADL are significantly affected and patients eventually become wheelchair-bound or bedridden.11
During the final years, patients suffer from continuous diarrhea or fecal incontinence and eventually become severely malnourished.17 With worsening neuropathy, they eventually die from secondary infections, cachexia, or sudden death.11,12
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