OBJECTIVES: To determine the effects of inpatient intervention for people with Friedreich ataxia (FRDA), and to identify whether improvements gained were sustained postdischarge. DESIGN: This retrospective observational cohort study comprised people with FRDA admitted to inpatient rehabilitation. SETTING: All participants in the study were referred by a specialist multidisciplinary FRDA clinic to inpatient rehabilitation. PARTICIPANTS: From 2003 until 2010, people (N=29; men, n=17; women, n=12) with FRDA were admitted to rehabilitation, representing 42 admissions. On admission, 9 participants were ambulant and 33 participants were nonambulant. INTERVENTIONS: Each participant was prescribed goal-related therapy on an individual basis by the multidisciplinary team, and this consisted of a range of treatment approaches. MAIN OUTCOME MEASURE: The FIM was used to determine the efficacy of inpatient rehabilitation. RESULTS: Consistent with the progressive nature of the condition, FIM scores, as measured on an annual basis preintervention, declined over time. However, FIM scores increased by a mean of 8.5 points during periods of inpatient rehabilitation and continued to increase by a mean of 2.0 points during the period immediately after rehabilitation. Results demonstrate these increases during and immediately after inpatient rehabilitation were significant (P<.001). CONCLUSIONS: To the best of our knowledge, this study provides the first evidence that a period of inpatient rehabilitation reverses or halts the downward decline in function for people with FRDA. The benefits from this intervention continued during the period immediately after inpatient rehabilitation, indicating that these gains are more than just short-term achievements. Further exploration of intensity, type, and length of rehabilitation is required to ensure that the most appropriate rehabilitation is provided.
OBJECTIVES: To determine the effects of inpatient intervention for people with Friedreich ataxia (FRDA), and to identify whether improvements gained were sustained postdischarge. DESIGN: This retrospective observational cohort study comprised people with FRDA admitted to inpatient rehabilitation. SETTING: All participants in the study were referred by a specialist multidisciplinary FRDA clinic to inpatient rehabilitation. PARTICIPANTS: From 2003 until 2010, people (N=29; men, n=17; women, n=12) with FRDA were admitted to rehabilitation, representing 42 admissions. On admission, 9 participants were ambulant and 33 participants were nonambulant. INTERVENTIONS: Each participant was prescribed goal-related therapy on an individual basis by the multidisciplinary team, and this consisted of a range of treatment approaches. MAIN OUTCOME MEASURE: The FIM was used to determine the efficacy of inpatient rehabilitation. RESULTS: Consistent with the progressive nature of the condition, FIM scores, as measured on an annual basis preintervention, declined over time. However, FIM scores increased by a mean of 8.5 points during periods of inpatient rehabilitation and continued to increase by a mean of 2.0 points during the period immediately after rehabilitation. Results demonstrate these increases during and immediately after inpatient rehabilitation were significant (P<.001). CONCLUSIONS: To the best of our knowledge, this study provides the first evidence that a period of inpatient rehabilitation reverses or halts the downward decline in function for people with FRDA. The benefits from this intervention continued during the period immediately after inpatient rehabilitation, indicating that these gains are more than just short-term achievements. Further exploration of intensity, type, and length of rehabilitation is required to ensure that the most appropriate rehabilitation is provided.
Authors: Louise A Corben; David Lynch; Massimo Pandolfo; Jörg B Schulz; Martin B Delatycki Journal: Orphanet J Rare Dis Date: 2014-11-30 Impact factor: 4.123