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BRIEF REPORT

Retrospective Study of the Effects of Inpatient Rehabilitationon Improving and Maintaining Functional Independence inPeople With Friedreich AtaxiaSarah C. Milne, BPhysio, Emma J. Campagna, BSci, Louise A. Corben, PhD, Martin B. Delatycki, PhD,

Kwong Teo, FAFRM (RACP), Andrew J. Churchyard, PhD, Terry P. Haines, PhD

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ABSTRACT. Milne SC, Campagna EJ, Corben LA, Delaty-cki MB, Teo K, Churchyard AJ, Haines TP. Retrospectivestudy of the effects of inpatient rehabilitation on improving andmaintaining functional independence in people with Friedreichataxia. Arch Phys Med Rehabil 2012;93:1860-3.

Objectives: To determine the effects of inpatient interventionfor people with Friedreich ataxia (FRDA), and to identifywhether improvements gained were sustained postdischarge.

Design: This retrospective observational cohort study com-rised people with FRDA admitted to inpatient rehabilitation.

Setting: All participants in the study were referred by aspecialist multidisciplinary FRDA clinic to inpatient rehabili-tation.

Participants: From 2003 until 2010, people (N�29; men,n�17; women, n�12) with FRDA were admitted to rehabili-tation, representing 42 admissions. On admission, 9 partici-pants were ambulant and 33 participants were nonambulant.

Interventions: Each participant was prescribed goal-relatedherapy on an individual basis by the multidisciplinary team,nd this consisted of a range of treatment approaches.

Main Outcome Measure: The FIM was used to determine theefficacy of inpatient rehabilitation.

Results: Consistent with the progressive nature of the con-dition, FIM scores, as measured on an annual basis preinter-vention, declined over time. However, FIM scores increased bya mean of 8.5 points during periods of inpatient rehabilitationand continued to increase by a mean of 2.0 points during theperiod immediately after rehabilitation. Results demonstratethese increases during and immediately after inpatient rehabil-itation were significant (P�.001).

From the Departments of Physiotherapy (Milne), Rehabilitation Medicine (Teo),and Allied Health Research Unit (Haines), Kingston Centre, Southern Health, Chel-tenham; Departments of Physiotherapy (Campagna) and Monash Neurology (Church-yard), Monash Medical Centre, Southern Health, Clayton; Bruce Lefroy Centre forGenetic Health Research, Murdoch Childrens Research Institute, The Royal Chil-dren’s Hospital, Parkville (Corben, Delatycki); Department of Medicine, Universityof Melbourne, Austin Health, Heidelberg (Delatycki); Department of Clinical Genet-ics, Austin Health, Heidelberg (Delatycki); Department of Rehabilitation Medicine,Angliss and Yarra Ranges Health, Upper Ferntree Gully (Teo); Physiotherapy De-partment, Monash University, Frankston (Haines); and Department of Medicine,Monash University, Clayton (Teo), Australia.

Supported by the Friedreich Ataxia Research Association (Australasia) and theFriedreich Ataxia Research Alliance (United States). Three of the authors are sup-ported by the National Health and Medical Research Council (Australia), one as aPractitioner Fellow (grant no. 546452), one as an Early Career Fellow (grant no.1037002), and one by a Career Development Fellowship (grant no. 606732).

No commercial party having a direct financial interest in the results of the researchsupporting this article has or will confer a benefit on the authors or on any organi-zation with which the authors are associated.

Reprint requests to Sarah C. Milne, BPhysio, Physiotherapy Dept, Kingston Centre,Warrigal Rd, Cheltenham, Victoria, Australia 3192, e-mail: sarah.milne@southernhealth.org.au.

In-press corrected proof published online on May 11, 2012, at www.archives-pmr.org.

0003-9993/12/9310-01137$36.00/0http://dx.doi.org/10.1016/j.apmr.2012.03.026

Arch Phys Med Rehabil Vol 93, October 2012

Conclusions: To the best of our knowledge, this study pro-vides the first evidence that a period of inpatient rehabilitationreverses or halts the downward decline in function for peoplewith FRDA. The benefits from this intervention continuedduring the period immediately after inpatient rehabilitation,indicating that these gains are more than just short-termachievements. Further exploration of intensity, type, and lengthof rehabilitation is required to ensure that the most appropriaterehabilitation is provided.

Key Words: Activities of daily living; Friedreich ataxia;Inpatients; Rehabilitation; Treatment outcome.

© 2012 by the American Congress of RehabilitationMedicine

FRIEDREICH ATAXIA (FRDA) is the most common in-herited ataxia, affecting approximately 1 in 30,000 people.1

People with FRDA experience major symptoms of ataxia,spasticity, and weakness, with a mean age of onset between 10and 15 years.2 Because of the progressive nature of the disease,people are usually wheelchair bound 10 to 15 years afterdisease onset.3 There is currently no pharmacologic treatmentproven to slow disease progression.4

Rehabilitation has been used for people with FRDA tomanage the negative effects of the disease on function. There isa paucity of studies investigating the effects of multidisci-plinary rehabilitation for people with ataxia.5 In a prospectiveohort study,6 intensive coordinative training significantly im-

proved motor performance and symptoms of ataxia in personswith degenerative cerebellar and afferent ataxia. A systematicreview7 including 9 studies based on a range of patient popu-ations with ataxia indicated that rehabilitation can improvetaxia symptoms, but these studies did not focus on FRDA.

We aimed to examine the effects of inpatient allied healthntervention on the function of people with FRDA, as defined byheir ability to perform activities of daily living. We also sought todentify whether improvements gained through inpatient rehabil-tation were sustained after discharge from inpatient care.

METHODS

esignThis was a retrospective observational cohort study.

articipantsAll people who were homozygous for a GAA expansion in

ntron 1 of the frataxin gene, aged 17 years and older, anddmitted for inpatient rehabilitation at Kingston Centre from

List of Abbreviations

FRDA Friedreich ataxia

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2003 until May 2010 were included in the analysis. Data from29 people with FRDA (17 men, 12 women; mean age � SD onadmission, 34.77�10.71y), representing 42 admissions, werecollected (8 people had multiple admissions). Nine people wereambulant on admission and 33 were nonambulant. People wereadmitted after an average disease duration � SD of21.77�9.18 years.

All participants in the study were referred by a specialistmultidisciplinary FRDA clinic after identification of the re-quirement for intensive inpatient rehabilitation. Eight peoplewere admitted from an acute care facility after an unexpectedsignificant medical event, and 34 admissions were referreddirectly from an FRDA clinic review. Of those referred di-rectly, 8 were admitted after elective orthopedic surgery, 4 afterpharmacologic management of lower limb spasticity, and 22directly from the community. Reasons for admission weredeterioration in function, being at risk of losing capacity tomobilize or transfer independently, or requiring interventionfor lower limb spasticity or contracture to enable stable footcontact for mobility.

SettingThe FRDA rehabilitation program, based in an inpatient

rehabilitation ward, included a rehabilitation physician and ateam of allied health professionals (physiotherapist, exercisephysiologist, occupational therapist, speech pathologist, socialworker, nurse, clinical psychologist, dietician) specifically ded-icated to the rehabilitation of people with FRDA.

InterventionEach person with FRDA was assessed on admission and pre-

scribed individual goal-related therapy. Intervention comprisedstrength and stretching exercises, education, functional and bal-ance retraining, aquatic physiotherapy, and development of ahome or community program. Treatment considerations specificto people with FRDA were safety and risk management; hip,pelvic and spinal control; spasticity management; hand function;communication and swallowing; cardiac involvement and exer-cise; and specialized equipment prescription.

MeasuresThe FIM8 is a validated and reliable measure of functional

ndependence that is responsive to change as compared withther functional measures for people undergoing neurologicehabilitation.8,9 In this study, the FIM total score9 was used.

Table 1: Change in FIM Score

Specific Time Period

Between all assessmentsBetween assessments: without inpatient rehabilitation in betw

FRDA clinic reviews and admission scores)Between assessments: between FRDA clinic reviewsBetween assessments: between FRDA clinic review and inpati

(direct FRDA clinic referrals)Between assessments: between inpatient rehabilitation admisBetween assessments: immediately after inpatient rehabilitatioBetween assessments: immediately after inpatient rehabilitatio

discharge and FRDA clinic review only)Between assessments: without inpatient rehabilitation in betw

immediately after inpatient rehabilitation

NOTE. Values are mean � SD.

ProcedureRetrospective admission and discharge FIM scores were col-

lected for all people with FRDA admitted for rehabilitation duringthe study period. To establish the efficacy of rehabilitation, pre-intervention and postintervention scores were compared with FIMscores collected by the FRDA clinic from the preceding years.Thus, FIM scores from both the FRDA clinic and the FRDArehabilitation program comprised the final analysis.

This study received approval from the Southern Health Hu-man Research Ethics Committee.

AnalysisA generalized estimating equation approach was used to

investigate the effect of inpatient rehabilitation on functionalindependence.10 FIM data were entered as change scores fromhe previous assessment and used as the dependent variable. Aultiple regression model examined the association between

hange in FIM scores and 4 independent variables: (1) receiptf inpatient rehabilitation (intervention); (2) period immedi-tely after inpatient rehabilitation (sustain); (3) time since firstssessment (time since onset); and (4) time between assess-ents (time between assessments).A second multiple regression model including the same 4

ndependent variables with the addition of interaction termsetween “intervention” and “time since onset,” and “interven-ion” and “FIM score before” (the score at admission) exam-ned whether the effect of intervention varied depending onow functionally dependent the individual had been at admis-ion to inpatient rehabilitation.

RESULTSThere were a total of 211 assessments (inpatient rehabilita-

ion, 84; FRDA clinic, 127). The mean period between FRDAlinic reviews was 431.99�281.07 days, which is consistentith the service expectation of annual reviews (table 1). This

anged between 11 and 1652 days. The inpatient rehabilitationength of stay ranged from 2 to 166 days (mean, 37.71d). Themmediate period after rehabilitation also varied between par-icipants.

Participants who were transferred from an acute care facilityfter an unexpected medical event had an average acute careospital length of stay � SD of 14.13�21.04 days, while thoseequiring an elective acute care admission had an averageength of stay of 5.50�5.32 days.

Time Between Assessments

Change inFIM Scores No. of Days

–0.8�13.0 219.47�225.91including

�3.6�13.0 264.65�230.83�1.5�8.6 424.77�267.38

habilitation�12.3�8.4 297.74�287.55

nd discharge 8.5�7.9 37.71�28.322.0�11.1 261.67�270.55

etween3.8�10.3 277.72�289.41

nd not�6.0�12.4 265.29�222.45

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On inpatient rehabilitation admission, the mean � SD FIMscore was 89.1�17.1. This increased to 97.6�17.1 on dis-charge and to 102.0�15.7 after the period immediately follow-ing. Overall, FIM scores declined by a mean of 0.8 pointsbetween adjacent assessments (see table 1). This decline wasgreater over periods when inpatient rehabilitation had not beenprovided (decline of 3.6 points). However, mean � SD FIMscores increased (8.5�7.9) during periods of rehabilitation, andduring the period immediately after rehabilitation (2.0�11.1).The mean � SD change in FIM score of first rehabilitationadmission only was 7.3�7.3, whereas the mean FIM changescore of subsequent admissions was 11.2�7.3, indicating thatthe multiple admissions of the 8 participants did not overlyinfluence the results. FIM change scores were normally dis-tributed.

Results of the first multiple regression/generalized estimat-ing equation model (table 2) demonstrated that increases duringand immediately after inpatient rehabilitation were significant(P�.001). The FIM items that showed the greatest improve-ment during the intervention period were transfers toilet, trans-fers bed chair, transfers bath, bath/shower, dressing lowerbody, and toileting. The items that showed the greatest im-provement during the sustain period were toileting and trans-fers bed chair.

Neither interaction terms between “intervention” and “timesince onset,” and between “intervention” and “FIM score be-fore” were statistically significant when added to the firstmodel. This indicates that the effect of rehabilitation wasrelatively consistent regardless of the amount of time sinceonset or the level of functional dependency on admission.

DISCUSSIONThis study examined the effects of inpatient rehabilitation on

people with FRDA compared with the typical functional de-cline in this population.11 A period of inpatient rehabilitationappears to reverse or halt the downward decline in function forpeople with FRDA identified as requiring rehabilitation. Thebenefits continued immediately after inpatient rehabilitation,indicating that these gains are more than just short-termachievements. This corresponds with the anecdotal improve-ment in the functional status of these patients, particularly inthe effort and assistance required to transfer. Furthermore, thecombined mean FIM change scores, from the intervention andsustain periods, are in excess of the 10 points that reflect areduction by half in the time spent caring for a person post-stroke.12 This is likely indicative of a meaningful change foreople with FRDA, although specific studies of a minimallylinical important difference in the population have not beenonducted. To the best of our knowledge, this study is also the

Table 2: Multiple Generalized Estimating Equation RegressionModel Output Explaining Change in FIM Scores With Inclusion of

Intervention-by–“FIM Score Before” Interaction Term

Independent Variables CoefficientLower95% CI

Upper95% CI P

Intervention* 11.20 7.54 15.31 �.001Sustain† 7.58 4.07 15.36 .001Time since onset �0.002 �0.004 �0.001 .001Time between assessments �0.002 �0.008 0.003 .41

NOTE. Model Wald �24�60.89, model P�.001.

Abbreviation: CI, confidence interval.*Inpatient rehabilitation period.†Period immediately after inpatient rehabilitation period.

rst to identify that the effect of rehabilitation on functional

Arch Phys Med Rehabil Vol 93, October 2012

ndependence is likely to be consistent regardless of the level ofunctional dependency people with FRDA have on admission,nd expands on studies including only ambulant participants.6

People with FRDA do have significant cardiac comorbidity,2

which has a considerable impact on function.4 Rehabilitationmay have an indirect effect on cardiac function that maycontribute to the improved functional independence after reha-bilitation had ceased. Individuals may also have recognized thebenefit of the rehabilitation program they were participating inand continued with elements of these. Moreover, an importantcomponent of inpatient rehabilitation is establishing an outpa-tient or home rehabilitation program. Further research isneeded before we can better understand the reason for contin-ued improvement in functional independence during the periodafter inpatient rehabilitation.

Study LimitationsA limitation of this study was that the selection of partici-

pants requiring rehabilitation may have biased the outcome.People requiring inpatient rehabilitation are often admitteddirectly after an acute illness or surgery, which may cause asteeper decline in FIM scores just prior to admission, notreflecting their usual level of function. A randomized con-trolled trial would address this question; however, given theinfrequency of FRDA in our community, it would be difficultfor a single center to conduct such a trial.

CONCLUSIONSTo the best of our knowledge, the results of this study

provide the first evidence that a period of inpatient rehabilita-tion improves or halts the downward decline in function forpeople with FRDA. However, further exploration to determineappropriate criteria for inpatient rehabilitation would assist inidentifying those who would benefit from rehabilitation. Ac-curate documentation of intensity, type, and length of rehabil-itation would be advantageous in enabling reproducibility anddetermining length of stay. Moreover, given the unknowneffect of rehabilitation on quality of life and disease severity inFRDA, it is essential that future studies include clinical mea-sures of these outcomes.

Acknowledgments: We thank the Acute Rehabilitation Unit,Kingston Centre, Southern Health, and, in particular, the FRDA reha-bilitation team for their support, their expertise in this area, and theirpermission for allowing their work to be reviewed.

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