T - Huntington's Disease Society of America

T ' ' i t A T t 4 E r , l T
IVothing may be mzre chalkngtng tltan to design a plan of care tltat alloas maximal
independence uith minimalrisfr--rspedalfiforpatients it/t a disease tltat not only impairs
motor sfrills but ditninishes cognitiL,e abilities antl ahers personaliry.
ffiu,
By Suzonne lmbriglio, PT
Lrntin-eton's disease (FID) is
a progressive and degenerari'
- ^--^"i..^",1;.;^. ^f the
central nervous system that
is transmitted through ar-rtosomal
dominant inheritance. The incidence
of HD is one case per 10,000
people in the general population, and
the child who has a parent rvith HD
has a 50 percent chrnce ..-tf inheriring
rlrc geuc and consrqLrcrrtlr acquiring
the disease, lvhich alr'vays manifests
itself rvhen the individual lives long
enough (Huntington's Disease Socieiy*
of America 1991).
'l h" rr- oinn.et vrrics considerably;
rvith s\,mptorns generall,v appearing
bcnrccn 3U entl 50 vears; houever,
in sonre cases, svmptoms have been reporteLl
as eariy as 2 1'ears and as late as
701's2r-5 (Nlartin and Gr-rseila 1991).
The HD Triod
Huntingon's disease involves a
triad oIchnica] ferrurcs: cognitive deficit'.
motor impairnrcnts. rnd behavioral
changes. The time of onset for
svmptoms in each of these three areas
Varics ll'om licrson to person. as does
thc r',rtc oIdetcriorrtion. Irr adtlition,
s) mpton)i progrcss irrdepcn'.lcnrlr.
0ognitiue defiri ts. Although n-rovement
disorclers trpicall1'are the rnost
conspicuor.rs feature of HI), the cog-
,ritir e rhrrroes thet nlerrr i. th" prt]r'
stages are the major sollrce of disability
(N4ayeu.x et al. 1986). These
mental changes are more variable in
onset and manifcstation than are the
movenent disorders.
Cognitive deficits in HD may
range from forgeCulness and inability
to concentrate for long periods of time
to severe memory deficits. Early-stage
symptolns affect the persorJs ability to
manage personal finances and occupational
ski-Ils. As the disease progresses,
deficits in recent and remote memory
become increasinglv apparent. The
person ma,v not be able to recall the
nan.re of the \Ace President of the
United States, for example, but may
bc able to correctly answer a mu.Ltiplechr-'icc
qLrestion. Over timc, prompting
becomes a less and less effbctive
sfateg.y- in dealing with information
retrieval and other memorv deficits
(Caine et aL I977).*
Even in the most advanced stages
of HD, howeve5 the person may
remain cognizant of his or her surroundings.
Higher-level intellectual
fi-rnctions, such rs those involving
"Editor's notc: For an in-t/eptlt disctusion
af cognitk't r/efirit.r ttnrl their inpor'l
on plrysir.al tlterapl tre(ttmmt, .tee
"Bqonrl tlte Sensorimotor ^ltrip" 14t
M. I'l'oherv/orf, CN[, L'o/. ]2, i\io. 3,
IIaylJune 1992, pnges 63-69.
Clinical Nlanageme nt

uumne Inbrig/io rtttr/ /tet'.rtdf
ff
\fo//ot;'nan.r
potiutts tt'irfi HD
# rfiroug/tour thefinu/ stag(s. HI-,D
do tltq, /tandle t/te innitrrbilitl of tlteir
patients' deterio ration ? Alt/to ug/t r/tere
carrentb is nofornnl supp\rt grLup,
tlte treatntc.nf Iea?n meets 0n a quarter/y
bask aitlt rr consulting niltrzlogistl
psyc/titttrtsto discuss t/te nrl-stages of
HD, w/ten, says Intbriglio,"a discussion
offeelngs usualfi comes ilp. W nner
use the term 'burnouf.' W aclnoa/er/ge
t/tnt it's ohay to cry, tlmt oe should
feel attaclmt?nt.t t0 t/tese patienn. [;[lten
one 0f zur patient.r dies, use nre preparulfor
it, borlt in a professionalsense
ant/ in il Itersorut/ s(nse."
acquired knorvledge and insight, r-rsually
are rvell-preserved 1},'lcHugh
ancl Folstein 1975; Fisher et al. 1983).
A,[otor imlsoinnents. 1'he earliest
signs of movement disorder in HD
typicalll, appear as choretc mo'ements
in thc fingersl as a result, thc pelson
lvith HD may appear nervous or
"fidgety." As the disease progresses)
coordination deteriorates, resulting
in diFficLrltics with sclllcare rctirities.
Dystonic posturing, bradykinesia, or
rigidiw and postural instabilitv that
severely inrpairs walking also mr1
ocst. Problems with speech and sw'al-
Iolt'ing der clop in the later stagcs,
reqr-riring a high level ofclre ro n]cer
the person's daily needt.
Behauiorrt/ t hatgcl People with
HD typically hrvc a range oIpsychiatric
problems that may be manifested
as behavioral probiems, such as delusional
an.i paranoid behrvior, .lepression,
and other alfective disorders.
lbl. 12 No.5 September/October 199?
Epi:odcs ot-isolation mav occur, end
i mpulsc-control difliculties, aprthl,
rltd Itsel self-estecma1'exist.
These personality changes create a
tremendous bLrrden on the pcrsoris
social relationships.
Mani individLrals t-ith etrh-srage
svmptoms are able to live at home
with a supportive family; horvever,
because ofthe early onset ofpsychological
problems, manv people with HD
enter the mental health svstem at this
tinrc. Repeated ps1 ehiatric problems
and disruption of the familv social
strucfure rnav lerd to placement in a
psvchiatric f-aciliw that provides longterm
care. This rnav mean that b1'the
tirne motor zrnd cognitive problems
become apparent, the individual is 1iving
in a setting that is poorly eqLupped
to address these s,vmptoms. Even for
people r'vhose fanrilies are able to continue
providing care at hone during
the early stages, there may be a lack of
appropriate long-term care providers
to meet the needs of these individuals
rvhen thev enter the later stages of
the disease.
Teomwork, To Ensure o High Quolity of
Life in the Leost Reslrictive Setting
Stafrat the Christian Hill Rehabilitation
and Skilled Nursing Center
(CH) in Lowell, N'lassachusetts, prov-ide
rnedical management, skilled
nursing, subacute neurological and
ps,vchological treatment, and traditional
rehabilitation services for peoplc
in thc intermediate l"transitionai";
and adranced stages of HD. Patients
range in age from their early 20s to
late 60s and early 70s. In all cases,
treatment is based on a belief that people
with HD should maintain a matimal
level of independence for
as long as possible.
Patients mav be followed b,y the
treatment team thror,rghouthe final
&*
63m

Physical Therapy and HD:
- ' From A Social Worker's Perspective
-' I ;,:- eople with Hunting;ton's disease,4ro;e' u'hat's happeling to dretrl,"
- emphasizes
I -- 'social lvorker Paui F-erreira, NIS\V. "They mav still be anrbulator,r'i br-rtheir
,- coordination skills are decrcasing. l-hey have less endurance tl-ran theY trsed
to have, thel'hn1,e difficulq' transferrir.rg fiorn chair to bcd, and the,v rnay need safety
devices to pieventhem fronr falling or sustairrilg head injuries...Thel' 11gecl a lot r:f
ernotional supporto help thenr deal u'ith thcse losses in ph1'sical function. A cr-itical
fbctor in the tr-eatur.rent of these patients is the attitude of the phvsicai therapist, lvho
rnust have a positive-but realistic--outlook." Both ps)'chodreraPist and case matiageq
Ferreira coordinates the tleatment team tlrat lr,orks rvith patients rvidr HD at tbe
C|u-istian Hill Rcfiabilitatior.r and Skilled Nursing Center ir.r Lorvcll, N4assachr.rsetts.
"Ph,vsical
tlrerapy is a major compone nt of the trcatmcnt progfarn for pcople rvith
"TIre
rnid-stage FID," says Fcrreira. trich.part is that HD isrlt just a ph1'sical problem.
It's the ph1,sica1 therapist'sjob to clistinguish anrons changcs relatcd to ph1'sical deterioration,
mental detcrioration, and tllc patient's elt-totional state'"
As Suzanne Imbriglio, P! discusscs o1 drese pagcs, sornc patients t'ith HD 1.ray
"For
strongly resist physical therapl.: these patients, to participate in phl'sical therapv is
to aclilit that the1, are sick," cxirlains Fcrreira. "Some paticuts are t(xr deep in dcnial to
to do that. In otlter cases, the disease affccts the mind, rcsultin$ in a psych6{c thought
process. Son-re patients rnay be depresscd-r'vhicli tvpicall1'affects dreir urotivatiou aud
iself-startilg' 'pzrssinQ
abiliq'-or may leel guilq' for thc diseasc on' to drcir cfiildre1'
Sone studie suggest that the incidence c-,f suicidc tr.rav be l.righer among peoplc rvith
HD than among the general population. Ald then thcrc arc patients rvho u'ant to do
anlthingthcy can do to help themselves." Ferrcim adds th:rt a paticnt's preexisting
"If
persolaliq' 16its tnay be agrplifred by thc disease. tfie patient telded to be anrit'rus
tr negative befc,rc theonset of F{l), he or she will probably have a big problenr t'ith
an-xiety as the disease progfesses; if the patient tended to be optimistic, that will be a
big help as tiure goes ott."
Florv does the social rvot-lier help the patient accept physical therapvl
"Patients
leecl to ex;'rlc.rre their- feelings about IfD,. .Iltdrei/re ir.r deniai, for eriamprle,
1'ou cirtr't'force'them out of it. L-rstcad i'ou tDr to find some t'indolv of oppol'tunirv'
it's important to talk about the real-ities of da1'-16-6|n" 6le' 'FIou'' are l'ou doiugi Are you
talling dorvn a lot? A.r-e ),ou able to r.valk around as rnuch as you used tol'As the patient
'RetnemLler
becomes rnore comfortable talking about these problerns, )rotl can sa1"
phvsical therapi'f lVlaybs rve could tr.v drat. N'[at'be it could help'' "
When Ferreir-a sepses during a counseling sessiotr that a patient nral'be readl' f61'
pitysical therapii he lr-ra1'rvalk u,ith tl.re patient to the ph,vsical drerapy deparlrnento
h1d out rvhetfier a ph,vsical therapist can mect rvitlt the patient right au'a1i L]ntil the
patient becorr.res familiar rvitlt the ph1'sical tlierapist. F-erreiralso may "sit in" otr the
phi'sical thcrapy scssions.
"This "After open door policl'can be hard on the PG," he sa1's' all, tl"rey're very
busti In addition to 2J patiertts'w4lo ltave I-ID, diey also treat geriatric patients and
patients with subacute tratunatic brain iljury But because of the day-to-da-V variability
among patients rn'ith HD, dris is the method that lr,orks best." Ferreira pauses.
"I guess
you could say that PG havc to str-il;e ivlile the iron is hot."
Psycholropic Medicotions
In addition to lcrorving rvhether a syrxptom is related to thc tlisease Process or to
the patient's aruiiegi the physical therapist also ntust r.urclerstand the eflects ofpst'chotropic
r.nedications.
"There are basical\, fbur categories of drr-rgs r,rsed u'ith patients n'ith FID," explains
Ferreila,
"a1l of rvhich tnal' l1n1's an irnpact on ;'l.rrsical therapl'treatnrent' f'he first
includes the ner-rroleptics, suclr as Flalclol", ri'liich, in lou'dclsages-'l to 10 mgstages,
inciuding death and dying.
Some fanrilies believe tl-rat when the
paticnt has reached the last stage of
the disease, he or she should be transferred
to a nursing horne for medical
mallagement, whereas other families
believe that the patient should
remain in familrar surroundings.
T-\^, r^ l^, -.^'..-.nrent
udy-lw-ud) 1rrarraSLt and care
of the person rvith HD are delivered
bJ, u tet- of nembers from the follouing
disciplinet: nursing, restorative
sen'ices, health and special education
sen ices, and :ocial services.
h'ursittg carc.'Ihe nursing staffdeal
witl-r such issues as l) the monitoring
ofdietary intake because ofeating and
f'eecling problems that ma1 rcsult in
chokingl 2) skin management problems
resulting from excessive invoLuntarv
moverrents; 3) deficits in
activities-of-dailpfvltt* (ADL) skiJls,
rvhich may result from incoordination
and deteriorating psychomotor
functions; and 4) pharmacologicai
management.
Rrstotuti:ce serttins. These services
include speech therapy, occupational
therap1,, and physical therapir Physical
drerapy is beneficial in improving
or stabilizing motor abiliti! preveuting
contracrures, and adapting the
enr.ironmento foster independence
rvl'rile ensuring safety. Occupational
thcrapy sen'ices help tJre patient maximize
coordination abilittes as rvell as
ADL skills. \\-itlr speech therapy,
people witli HD may be able to improve
intelJigibility; sonre may benefit
frorn the use of augmentative speech
der,'ices. The speech therapist also is
responsible for evaluating dysphagia.
Healtlt and special education seraice-s.
Provided by health educators
and special education tcaclrers, these
servicef include cognitir.e retraining,
rnemor)/ and awareness training, and
"self-ir-nprovement" classes especiaily
designed to help people rvith HD
improve their self-esteem.
Soria/ srrt'ires. Peoplc u itli HD
require specialized intenrention strat-
64 Clinical N4anagement

egies to defiise or avoid potentiall,v
explosirc episodes. Because ofpoor
impulse control, patients rvith HD
rnay become easily frustrated rvhen
their needs are not quickly met. Thev
also may benefit from psychotherapv
that addresses their grief, fear, and
concerns about loss offirnction. Because
judgment may be impaired and
impulsivity may increase as the disease
progrcsses, thcsc paticnrs ma1 require
close nrorritoring and supervi=ion.
Maintainine rcgrrlar la mil1' contrct
and familv counseling also are social
services priorities (see "Physical Therapy
and F{D: From a Social \\brker's
Perspective," pages 6.1-65).
A Never-Ending Educotion Proces
Whether it's the education of a ner,v
ph1'sician on the medical staff or the
continuing education of direct-care
staff,, ongoing training is integral to
the success ofanv treatn-rent program
for people with HD-no mafter hor.v
big or small the facility. To treat the
whole person, everyone on staffmust
have an understanding ofthe disease
process. Classes mav be offcred for
both day and evening shifts on such
topics as "Wl.rat Is FIDi", "\Vhat the
Person witlr HD Can Do," and"Hor
to \\hlk with a Person with lID."
Ciasses, rvhich mav be scheduled on
a quarterly basis, for example, also
can be provided on bchai ior manegement,
restorative services, and feeding
protocols. Experienced members
of the treatment team may be the
best teachers.
Intoke ond Assessmenl
Upon admission to the facilirv,
each patient undergoes a colnpreher.rsiv'e
medical, neurological, and
psychologrcal asscssrncnt perlormed
by the attending physician and the
co nsulting neurologi st/ps1-chiatrist
and an intake process @gure 1) that
mav be facilitated by any mernber of
the treatment team.
\/ol. I I \o.5 Seprcmbcr/Oerubcr l,lQl
mav rcdnce chorcic movement. Llpon adnrission to our progranr, son-re people r,vith
FID are receiving too higli a dosage, and if you get too far outside tl're theraper-rtic
lvindou,-, tl.re medication actually rnal'make rnatten rvorse, resnlting in side efllcts sgch
a:- d1,516n1" or rigiditv and afrecting the abiliry* to walk. If a person is not realJy bathet"ul
by dre choreic lnovements, it ma1' 116g be necessary for tl.rem to take these drugs at all."
I,trreira sirys that among younger people widr HD, s).mptoms ma1. [s sirni]ar to those
ofParkinsorls disease, in u'hich rnovenent tends to be stiffrather than choreic.
Nledication for aruxiety, sr-tch as Ativan'n', mav be used in lolv doses with these patients
rvhen thev are prone to explosiv'e or ag'glessive behar"ior, notes Ferreira. Antidepressants
also rnav be helpfi.rl.
"lldivicluals lvith HD mav har.'e an underffig depression drat is not readil1' appryent,"
he eiaborates.
"Traditional sylllptorn such as a depressed rnood, sleepingdisorders,
and'"veight gain or loss may not be evident. In FIn, the patient's mood may be ilat,
irritable, or extremelv labile. Drugs such as Prozac@ and sorne of the tr-icyclics rnay
help." The fourd.r category of rnedication incl-rdes drugs such as ltgretoln! and lithium,
which rnav help people with HD rvho hare qytrerne mood sr,vilgs.
The Teom Efforl
Because of the physiological, cognitir,'e, ancl behavioral changes and the effects of
medications, it is especialll'- i-rn;lortant for the treatrnenteam to have r-egular communication.
An interclisciplinary'st;rffmeeting r,vith physical drerapists, occupational therapiss,
speech d.rempists, ntirsing stafi and social workers takes place weekly for the revierv of
cases' and qpicaily includes the aftending physicians and the consulting psvchiatrist.
Quarterly and annual tneetings are held fbr each patient, with special tearn meetings
calied as rleccssary
In adclition to team'"vork among stald savs Fbrreira, there is a kind of tearn'"vor-k
among patients.
"Patients
rvith Hl) don't need to be isolated from patients with other
qpes of disorden. lVe group patients according to linctional abilities. In social i:rteractions,
patie nts v"ithHD mtry be irnpulsive or aggressive. Holvever, il many instances, the
emotional problens of patients with HD and patients with brail injuy, for e:

T R T A T [ i t N T
f or nrany patients with Huntington's disease (HD), de-
F .t.ur.d pirysical activity may result in degeneration drat
I *.u^ too early to be attributed to the diseasc process
alone. This suggests that nraintetrance or
temporary irllfiIorr.rn.nt of fu nctional
abilities within the limitations of tire HDilvolved
"Among
the 40 potienh wilh whom I hove
centrai nervous svstem may be
workeduring the posl seven yeors, loss
possible in the early stages ffoung ii86). of $rength in lhe eorly sloges wos nol 0
A program ofactive exercise based on the
uatiends identified needs is recomrnend- significont foctor for lhose who remoined
.a 6Ioy Hicks, and Barraclough 1985; ortive. When lhere is o decreose in octivity
Hayden 1981).
os the diseose progresses, los of stren$h
The physical therapy evaluation may
moy be consideroble. Although weight lifting
moy increose intoordinslion ond mus-
indicate that the oatient needs to develop
an awzlreness of the differences benveen
muscle tension and muscle relaxation; cle tension, it moy be effective in building
learn controlled breathingte.ldq:.tl
musde strenglh for some potients."
matntaT or lmprove coorclmauon, rlqlbiliry
and balalce; and, when applicable,
-lnez Peocock
increase muscle stength (Pcacock i987).
Routine active exercise for neck, trunk,
and extrernities and firnctional activities forbalance and coordi-
;"; ; m;ftant cornponents of the treatrnent program;
wa$ing should be part of the daily regimen (Chiu 1989).
It is irnportanto begirl an ongoing exercise program early
in the course of the disease befotz many patients and fanillies
believe there is a need for it. At this point, tle patient still rnay
By Inez W. Peocock, PT
ffi,,
Durir-Lg the initial nvo rveeks, the
patient is given time to "get used" to
the new surroundings, and the treatment
tearn assesses motor skills and
coordinarion (IrigLrre 1 ). Phvsical
therapy evaluation documents range
of motion, muscle strengd-r, leg lengdr,
trurrk mohiliry standing enJ sining
balance, prosture, sensation levels, cardiopulmonary
status, the existence of
pain, the ability to rriake transfers, gait,
tlre need for special equipnrent, and
the need for special tests. Also assessed
by tJre team: spceclr, sw'allowirrg, anJ
.^o.ir,rze firnetinns
-'b^'-"
Figurcs 2, J, and -[ are examplcs
of some of the fbrrns used in rnotor
skill and cognitive assessment. These
particular examples are based on the
precision teaching method (N4c-
Greery 1984; Pennlpacke5 Koenig,
and Lindsley 1972), rn which tlie
therapist or teacher breaks dorvn each
task into separate movements withirr
1S-second intervals and records the
per-minute rate at which these movements
are con-rpleted. Both ability and
thefluency of movement are assessed,
with the goal of increasing the rate at
which a patient is able to perform a
task. IJsed prirnarilf in special education
settings, this method can be
useful widr patients rvith HD because
these patients have many of the sarne
.leficits tlrat people in spccial education
settings have. In assessing reading
skil1s (trigure 2), for example,
the tcster wants to detcrrnine not
onLy whether the patient understands
what he o[ she reads, but whether eye
movements already may have begun
to decrease because ofthe disease,
resuhing in what may appear to be
slorved comprehension.
When the various assessments are
brought together to provide a comprehensive
functional and clinical picfure
of the patient's status, the team formu-
Iates an individualized plan of care.
'Friendly
Persuosion"
Persons in de middle stages of the
disease nsually are all too aware of a
number of losses, both physical and
mental. They also may be aware of the
degcn erative and progrcssive nature
of dre disease. Thcy may feel helpless
Clinical Management

; I I A T I t I N T
ball. Music can accompany movement-pattern e

i F E t . T L l E t { T
ffi,'
i:{{
: .:t,, :': :/.1:'!
which in turn help to bolster selfesteem.
After meeting u'ith some level
of success, the paticnt may be more
open to discussing the aspects ofphysical
functioning with which he or she
is hai,ing the most dificulgv. The therapist
and patient together can adapt the
environment or provide a-ltematives to
conpensate for the loss of function.
A"lurd se//." The process described
above often is a slorv one. Bv the time
patients reach the middle stages of the
disease, thev already have had to deal
lvith a multitude of "failures," an6lbecause
of disease-related cognitive
losses and emotional imbalancetheir
abiiiq'to cope with these fai.lur-es
mav he sevcrclv comoromised.
'"-'-r-' At
FF nowting thnt I Tnould this point, many people rn'ith HD
isolate themselves. Horverter, a
to /mae at /east some conn-o/
patient mav seek out one person frorn
olu' ft'erj siuarion. tlre nrimarw caresivinrt staff and conpatient
J -*'-b.'^'-b
I try to a//oa: tlrc Patient sorn e/ement
0f cznrrzl in finding solutions.
linue to respond well to this person,
as in the case described below.
Tltis drffers somerCIhatfrom tlte
LH's Story
traditional role of t/tu'apkt as I'ae LH had had a series of near falls,
experienced it in t/te past. In many
traditional settings, tlte tlterutpist
and her therapist had begun to discuss
some safety guidelines rvith her.
LH became angry rvith her therapist
?nafres tnzst of tlte treannent decisions,
such as c/toosirtg t/te treatment
and stated that she would not see hirn
again for "arry kind of therapy." LFI
regxmen and settirtg tlte goak. But clearly was unable to cope ivith the
then, in nrun! troditiznol settings,
tlte patient is going to ger bettaror,
deterioration in her physical functioning
and furthermore associated her
at t/te oer! /east, /earn to com-
therapist rvidr this deterioration. LH
eventually turned her back on her
pensatefor a drsability and get on
speech therapist and occupational
airlt ltfe, and therefore night not therapist as u,ell, becoming reclusive
take lift-t/trcarening risrts. But rt;lten
lou frnout ysu're going to become
tnore and more i// ancl nentuafit
and withdrawn; holveveq she did
maintain a relationship with one of
the health education teachers. This
tearher hceemn rrerrr imrrnrferrt in
die frorn tlils debilitating disease,
the care and treatment of LH.
lou migltt thinh,'Wty ilot take I-FI's treatment plan r,vas revised
a @a/k u;it/tout rny /te/ma? So altat
if I fa// and get a ltematonm?'
to allow much of thc physical therapy,
occupational therapr5 and speech therapy
to be provided by the one person
It's aur clta//enge to conaince t/te
LH trusted. Although this stratcgy
patient tltat tltere arv altenutiDes."
n'as far from thc traditional delivery
-$v7sluts Intbriglio
oFserr-ice in many skilled nursing
centers, the outcome r /as successful:
LHt dignity was preserved because
she was provided with a way to continue
fi.rnctioning at her optimal level.
LFI now is much less reclusive
and allows her therapists to observe
her periodicalil so that her progress
can be charted and her program can
be updated.
lsues o{ So{ety ond Self-Determinotion
Because of the nature of the HD
process, faulqv judgment and impulsivity
may result in difficult or
unsafe situations.
For some patients, the fear of selfinjury
is enough to motivate them to
adapto their new limitations. These
patients may rnore readily accepthe
idea ofwearing knee and elbow pads
or a protective helmet, for example,
because using these aids means maintaining
the ability to walk without help
or supervision. Patients who deny
their limitations or who are not intimidated
by the possibility of self-injury
howeveq pose difficult chailenges to
the treatment team.
Nthough it is the responsibiliry of
all stafrto ensure the safety ofpatients,
the physical therapistypically is the
one r,r,ho assesses the condition of a
patient when physical functioning and
safety are in question. It may be very
difficult to convince the person whose
judgment has become faulty or whose
physical functioning has deteriorated
that he or she now needs assistance.
There is nothing more challenging,
horvever, than developing a plan of
care that allows maximal independence
with minimal risk to safety
and well-being.
There are no easy or set "cookbook"
answers to the problems of
HD. In developing approaches to
solve these problerns, it may help the
therapist to envision himself or herself
in the patient'situation. How
important is it for you to have control
over a given situation inyurltfel
Clinical \,lanagement

1 R i A T i l 1 T i . l I
The patient may go through a period
ofanger or depression or both as
changes occur in physical starus. \\tth
counseling and support, hor,vever,
these changes can be "turned around"
into something more positive. The
patiEnt may learn that he sti1l can go
out to the donut shop as long as he
wears a helmet or knee pads for protection
or that using a wheelchair
does not prevcnt him flrom going
out to the Monday night bingo
gamc. Helping the patient nraintain
a normal routine while adjusting to
physical changes is essential.
The Fomily os Future Potienl
Because of the genetic component
of HD, many family members of
patients are at risk for FID and
therefore may be patients themselves
in the furure.
tramily members must deal with
many emotions when placing a
patient in long-term care. When the
patient is a child ofan unaffected
parent, the parent's concerns primari-
Iy revoive around obtaining highquality
care for the chi1d, and the parent
feels some reliefr,vhen this care is
found. When the patient is a parent,
the children are themselves at risk for
HD and therefore have additional
concerns. The childrcn oIpatients
with HD require support and the
opportuniry to talk in-depth abour
the rype of care the patient wiil
receive, pardv because they know
they may need that care themselves
one day.
Family members who are themselves
at risk for HD rypicalJy have
been caregivers in the past and therefore
may have some very helpful ideas
about how to deal with the patient.
This information can be vital, particularly
upon admission. This transfer
of information also is a good way for
that family member to become accustomed
to the notion that the patient
rnill be cared for by someone else.
Very often the "at-risk" family mem-
Vol. 12 No.5 September/October 1992
ber is esneciallv interested
the
therapeutic interventions available to
th" LrrL ^.tio.r r.rl tl'"t C-ilr, dIIU tll4t lall/ll) -"-ht. rrrLlrluLr
PAUlllL\
sometinies looks to the therapist for
hope that treatment will delay or preverrt
the inevitable degeneration.
Working with families who are at
risk takes a great deal ofsensitivity
on the part ofthe therapist. In addition
to meeting the needs of tlie
patient, the therapist must provide
connseling, teaching, and support to
the family, making appropriate counseJing
referrais as needed.
It has been r,vell-documented that
thc qualiry of care given to patients
rvith HD-as perceived by the patients'
children-decply affects rhe
childlen's ability to face their orvn
illness. The perception of the quality
of care also influences their abilig
to make use of professional services
( Martindale 1987 ). A slogan commonly
used in the HD communiry
underscores this need For quality
care: "Until there's a cure) there's
only care." crt
Suzanne Imhriglio, PT, is Director
of Restoratice Seroica, C/tristittn Hill
Relnbilitation and S li/led l{ursittg
Ccnte6 19 y'arnurn St., Lor'le//, l,lA.
The author tlunls Jim Po/lard, Paul
Ferreira, and Tbn Imbrigliofor their
assistance in uriting rhts article, and tie
HD treatment te(tm at C/tristian Hill
for tlteir continu&/ roopff(ttizu.
For more informotion obout
Huntingfon's diseose, cqll the
Huntingfon's Diseose Sociefy:
of Americo, 212 / 242-1968.
REF'ERENCES
Caine ED, Ebert NIH, \\'eingartner
H. An outline for the analysis of
dementia: the memory disorder of
Huntington's Disease. Neurologt
1977;27:1087 -1092.
FisherJNl, KennedyJL, Caine ED,
Shoulson I. Dementia in Hunt-
"'b'"" inqton's " disease' a cross-seclional
analysis of intellectual decline.
Adv Neurol. 1983;38: 229-238.
Fluntingtorls Disease Sociery of
America. Facts at a G/ance. New
lbrk, NY: Huntingtoris Disease
uvLlLL/
q^^:^-. ^t Ur r
^ !rlr!r1L4)
^^:^^. Iggl.
Martin JB, Gusella JE Huntingtorls
Disease: pathogenesis and management.
N EngJ Med. I99l;
315:1267
-1276.
Martindale B. Huntington's chorea:
some psychodynamicseen in
those at risk and in the responses
of the helping professions . Britislt
Joumal of Pslchiatrl. 1987 ;
| 50319-323.
Mayeux R, Stern d Herman A, et
al. Correlates of early disability in
F{untingtods D rsease. Ann
Neurol. 19 B 6;20 :727-731.
Mccreery P Frequency and the
standard celeration chart: Necessary
components of precision
teaching. Joumal of Precision
Tiaclting. 198 4 ; 5 (2):28-36.
NlcHugh PR, Folstein Mtr Rychiatric
syndromes of Huntington's
chorea: A clinical and phenomenological
study. In: DF Benson,
D Blumer, eds. Pslcltiatric Aspec*
of Neurolctgic Disease. New York,
NY Crune and Stratton; 1975.
Pennlpacker HS, Koenig CII,
Lindsley OP.. Handbook of tlze
Standard Beltavior Chart. Kansas
City, KS: Precision Medta 1972.
SUGGESf'ED READINGS
Harper PS, ed. Huntington's Disease.
London, Engiand: W.B. Saunden
and Co.; 1991.
Shoulson I. Huntington's disease:
cognitive and psychiatric features.
Neurolog 1990 ;3:I 5 -21.
Young AB. A Neurologist Speaks
Alout Fluntingtoni Disease. Excetpt:
from Dr Young\ speech to tlte annual
workhop. NewYork, NY Huntington's
Disease Society of
America;1986.
w
6effi

Intoke: Regording your fomily membe/s core while home wiilr fomih-
1. Who assisted u'ith self-care?
\Vith u,hich tasks?
Bothing: Dressing: Grooming: Toileting:
Upper body- Upper bod-v- Brushing hair fusist with pants up/down -
Lower bodv- Lorver body- Brushing teeth - Help complete task
Hands/face -
Socks/shoes - Shaving - Commode was used
Underclothes only - Makeup - Bathroom was used
Nail care -
Which tasks were the most difficulti \\hvi
2. \Ahen did assistance wirh homemaking skills become necessary?
Which chores could your family member still perforn-r independentlyi
Which chores could he or she Derform oart of?
3. Sho assisted your family member with eating?
\Vere there anv "tricks" that made
this task easier? (such as using special cups or utensils, avoiding certain t-vpes of food, using special chairs or clothes)
4. Please describe the position used by your family member while he or she was eating or being fed:
Heod:
Trunk (chest) Hips: Legs ([eet):
Up - Forward - Back in chair - Flat on floor -
Down _ Backward _ To one side ieft/right - Resring on stool -
Foru,ard - Sidervays right/left - Fonvard in chair - In constant motion -
Straight ahead - Upright -
Under buttocks -
Othei (please describe): Orher (please describe): Other (please describe):
5. Did your family member have an,v difficulty sitting in a chair? Yes - No -
Which type of chairs did he or she use? Sling back chair - Straight backed chair -
Wheelchair - Lounge chair - Rocking chair - Recliner (e.g.,Lazy Boi€) - Didn t use a chair -
Did you use anything to help your family member stay in the chair?
A sheer tied around the chair - A tray placed over chair - Never used anything -
\\hen did you use something to help your family member stay in the chairi
During meals - In the morning - In the afternoon - In the evening - In the middle of the night -
6. Did you need to help -vour family member u'alk? Yes - No -
Did you provide a wheelchairl Yes - No -
How did you help,vour family member walk?
One person held onto belt - One person held onto hips - One person held onto arm -
'livo
Didn r let him/her rvalk - Tivo people on each arm - people held hands - Two people held onto belt -
7. Is there anyrhing yor.d used during your family member's stay at home that made his or her stay easier for you?-
ffiro
Designed by Linda Anderson, OTNL, 1990
Figure 1. Sample intahe form used hy rhe Huntington's disease treatment€am to he lp assess tlte patient's functional lroels--and os part of a
strateg to help ensurefamifinoolaement in the patient's care.
Clinical Management

Hunlinglon's Diseose AssessmenlJVlovemenls to Moniior
l. ADL Streening ond Asesment-ADL screening 0nd ossessment will be completed.
ll. "The Big Six'-Specific movements will be torgeted bosed on the informotion obtoined through these ossessments.
lll. Augmentotive Communicolion-Movements rlsed in communicotion will be osessed.
lV. Nutrition-Movements used in the wrilten ond verbol expression of nufillionol knowledge will be osessed.
ll. "The Big Six", Hond Usoge
Reach for 9" target
Reach for 6" target
Reach for 3" target
Pointo 9" target within 12" of target
Point to 6" targetrvithin 12" oftarget
Point to 3" target within 12" of target
Touch 9" target
Touch 6" target
lbucll 3" target
Grasp release ("Hook")
Crasp release ("C1'lindrical" )-lcft and righr
Grasp release ("Lateral";-hands for all
Grasp release ("Three Jaw Chuck")-grasps
Grasp release ("Pincer")
Grasp release ("f ip Pinch")
Aim place object onto 8"-by- 11
" target
Aim place object onto 6" by 8" target
Aim place object onto 4" bv 6" target
Thist doorknob
Twist 1/2" nut onto bolt
Grasp pull cards from holder
lll. Movements Used in Augmentotive
Communicotion (to prepore potient to use ommunicotion boord)
Imitate therapist as therapist reaches for target
Poinr to l" snr;:res leFto rioht-fnn tn hottnm
Point to 1" colored squares on 9"-X-11" grid
Point to requested picrure and to picture of the requested size
Point to letters in name
\\'rite words from word iist
Read rvords with picture
Read word list
Type name
lV. Movemenh Used in the Written ond Verbol Exoression of Nutrilion
Knowledge
Write list of high-calorie food
Say list of high-calorie food
Circle high-calorie food choices
Huntington's Diseos+-Worksheet for the Assessmenl of Moior Coordinoiion ond Hond Uscge
Rerord the number 0f ilmes potient c0n reoch within o l5-second time period
MovEMENTcY(LE Left
Reoch (Gros Motor)
Arm length +10'
Right
9" 6"
P0int,.................>
3" 9" 6" 3"
Arm length + 5'
9"
Touch ..................p
6" 3' 9' 6" 3"
(Fine Motor)
srlt GRAsPs
#l Hook
(block ond whife gripper)
#2 Cylindrirol
(sponges-specify no.)
#3 Lsterol
(cllck+op pen or click toy
olligotor)
#4 Three Jow Chuck
(spinner-pinwheel)
#5 Pincer
(clothespin)
#6 Tip Pinch
(pin or swob $ick wifi
tension
onend)
Figure 2. A form tltat ma! be used in assessing coardination and hand usage amang patients aith Huntington\ disease. Based on the prici
sion teaching method of monitoringfanuional performance (LIcGreny 1984; Pennyparher, Koenig, and LindslE 1972).
Left
Right
Vol. 12 No.5 September/Oc rol:er 1992
Tm gH

Purpose Assess Read-ng
StartDate_
Plonning Sheet
Behavior Label Proiect # 1
Cnmnrahen q j on StopDate
Ir{anager Adviser
Dole Situslion: Selfing
npIYRFormol,
fine
One-to-one
Quiet room
Patient seated
at table
7 days
Nlaterials:
.Sample worksheet
. Test Worksheet
.2 pencils
.Timer or watch
rv/second hand
.Standard Behavior
Chart (Dailv)
Events Before/Unlil/Dudng
Day 1
1) Give pacient a sanple worksheet,
and explain how it is complered.
2) Place test worksheet face dou'n
on table.
3) Directions to patient:
. Read and answer questions
by marking correct ones.
. Skip arw questions,vou don t knorv.
4) Flip paper.
.5)Ask patient to "Go."
6)Time for one minute.
7),$k patient to "Stop."
Days 2-7
1) Revierv directions.
2) Repear steps 2-7.
Movemenl
Cyrle(s)
See/read
and answer
questions
(by marking
correct
response).
Evenls After
Praise work.
Correct
worksheet
rvith patient.
Review skips
and errors.
Page #2
Peformonce
Slondord(s) AIM(S)
8-13 items
completed
per minute
@rh6
mqt use tlt sfarm in assessing t/te cognitioe shilk of patfunts wth Hnttingtons disease. In t/tis exttmp/e,
,"r'odirrg roorprehenii6n skilk arc to he tested. (Adapted aitlt permis.sion of Precision Teac/ing and Management Systems, Nar;ton, MA).
ffirt
To qssess le:ord the nunber of items lhe Gompleles pel minute,
Coin Combinofions
1. nickel, penny
2. dime, 2 per-rnies
3. dime, nickel
4. quarteq dime
( ^r tartnr nPnn\/
6. quarter, dime, nickel
7. 2 dimes,3 nickels
8 3quarters,2dimes
9. 1 quaner,2 dimcs,
3 nickels, 1 penny
10.3 dimes, 4 pennies
Bill (ombinotions
1. 3 one dollar bills
?.? fwe dollar bills
and 1 one dollar bill
3. 1 ten dollar bill,
1 five dollar bill
Pctienl ldentifies llen Costs {ou Poy with"
$ 1.89
ongwers.
'{ou Gel Chonge Bock"
; in assessing tlte paticnt's ability to use ntonqt-botlt in terms of functionalrnotor skills
oid in trrr,t of ngitite ahilin-roitlt a ga/ of rtelpirtg t/rc poticnt renmin as independent as possible'
b+.ti)
Db. /J
b /.95
$11.64
$18.65
Items Cost
4 for $1.00
3 for $ .99
5 for $1.00
6 for $1.50
3 for $1.00
4 for $1.00
5 for $1.00
3 for $ .99
$5.00 bill
$1.00 bill
$10.00 bill
$10.00 bill
$20.00 bill
$20.00 bill
$20.00 bill
'{ou Wonf'
I
1
1
1
I
3
2
2
'{ou Poy''
Clinical N4anagement