70 F I S I O T E R A P I E SEPTEMBER 1981 E tz el, F . (1980). T h e cost o f h a e m o p h ilia tre a tm e n t a n d th e cost o f lack o f tre a tm e n t. P re se n te d a t 1st In te r ­ n a tio n a l H a e m o p h ilia C o n fe re n c e , B o n n . F a n tl, P ., S aw ers, R . J. a n d M a rr, A. G . (1956). I n ­ v e stig a tio n o f a h a e m o rrh a g ic disease due to beta- p ro th ro m b in a s e defic ie n c y c o m p lic a te d by a specific in h ib ito r o f th r o m b o p la s tin f o rm a tio n . A u s tr a la s . A n n . M e d ., 5, 163 - 176. G ra h a m , J. B., B u c k w a lte r, J. A ., H a rtle y , L. J. a n d B rin k h o u s, K. M . (1949). C a n in e H e m o p h ilia . O b ­ se rv a tio n s on th6 c o u rse , th e c lo ttin g a n o m a ly and th e e ffe c t o f b lo o d tra n fu s io n . J. E xp . M e d , 90, 97 - 103. H o fm a n n , P ., S c h w u c h o w , E. P. a n d R o e ssle r, R . (1980). Jo in t-c o n s e rv in g o p e ra tio n s in h a e m o p h ilia c s. P re se n ­ ted a t 1st In te rn a tio n a l H a e m o p h ilia C o n fe re n c e , B onn. K a ra b u s , C . D . (1981). In h e rite d b lee d in g d iso rd e rs in S outh A frica : T h e SA H a e m o p h ilia R e g iste r. S u b ­ m itte d to SA H a e m o p h ilia C o n g re ss, B lo e m fo n te in . L evine, P . (1980). C o m p re h e n siv e c a re p ro g ra m m e fo r h a e m o p h ilia c s. P re se n te d a t 1st In te rn a tio n a l H a e ­ m o p h ilia C o n fe re n c e , B onn. S tra u ss, H . S. (1969). A c q u ire d c irc u la tin g a n tic o a g u ­ la n ts in H e m o p h ilia A . N e w E n g l. J. M e d , 281, 866 - 873. T H E R O L E OF TH E PHYSIOTHERAPIST IN T H E TR EA TM EN T OF T H E T E R M IN A LL Y I L L OR DYING CHILD M . G O O D M A N , B .Sc. (P h y s io th e ra p y ) W itw a te rs ra n d , D .S .E . (U N IS A )* S U M M A R Y T h e role o f the p h y s io th e r a p is t in th e tr e a tm e n t o f the d y in g c h ild is tw o fo ld : F irstly , tr e a tm e n t is d ire cted to w a rd s d e v e lo p in g the c h ild to f u ll p o te n tia l, m a in ­ ta in in g fu n c tio n , p r e v e n tin g d e fo r m itie s a n d relievin g pa in a n d d is c o m fo r t. S e c o n d ly , sh e ha s a role in s u p ­ p o rtin g the p a r e n ts a n d fa m ily . T h e n e e d f o r a d o m i ­ cilia ry p h y s io th e r a p is t is in d ic a ted . 1 a m su re t h a t m o st o f u s p h y s io th e ra p is ts feel in ­ a d e q u a te a n d u n s u r e o f o u rse lv e s w h e n c a lle d u p o n to tr e a t th e te rm in a lly ill o r d y in g c h ild . T h e m o s t c o m ­ m o n a m o n g s t th e s e fa ta l d iseases in p a e d ia tric s a re c y stic fib ro sis, le u k a e m ia , c a n c e r, p ro g re s siv e m u s c u la r d y stro p h ie s a n d p ro g re s siv e g e n e tic d iso rd e rs. “ I t is h a rd to im a g in e a m o re d e v a s ta tin g e x p e rie n c e in th e life o f a fa m ily th a n th e fa ta l illn e ss o r d e a th o f a c h ild . E a c h m e m b e r is a ffe c te d in d iv id u a lly a n d se p a ra te ly w h ile th e fa m ily fu n c tio n in g as a w h o le is th ro w n in to d is ru p tio n .” (S tra k e r a n d S c h m a m a n , 1980). E liz a b e th K iib le r-R o s s in h e r b o o k o n D e a th a n d D y in g (1975) d e sc rib e s fu lly th e stages o f a d ju s tm e n t to a n im p e n d in g d e a th n a m e ly , sh o c k a n d d e n ia l, a n g e r, b a rg a in in g , d e p re ss io n a n d fin a lly a c c e p ta n c e . F a m ilie s to o , go th ro u g h th e s e sta g e s a n d it is im p o r ta n t f o r th e p h y s io th e ra p is t to u n d e r s ta n d th e s e fe e lin g s a n d r e ­ a c tio n s. O n e o f th e m o s t d iffic u lt s itu a tio n s is w h e n th e fa m ily a n d m o re p a r tic u la r ly th e m o th e r, is fe elin g a n g ry . T h e a n g e r m a y b e d ire c te d a t r n y m e m b e r of th e s ta ff a n d if n o t u n d e rs to o d , it m a y re s u lt in fe elin g s o f h o s tility b e tw e e n s ta ff a n d p a r e n ts w h ic h w ill in te r ­ fe re w ith d e c isio n m a k in g a n d tr e a tm e n t o f th e c hild. “ A g o o d w a y o f h e lp in g p a re n ts a n d th e fa m ily o v e r th e ir d e n ia l a n d a n g e r is to e n list th e ir a id in c a rin g f o r th e c h ild . G iv in g th e p a re n ts a se n se o f w o rth a nd fe e lin g t h a t th e y c a n d o s o m e th in g a b o u t th e s itu a tio n c a n go a lo n g w ay to h e lp in g th e m c o m b a t th e ir fe e l­ ings o f g u ilt, fu tility a n d in a d e q u a c y .” (S tra k e r a nd S c h m a m a n , 1981). * P rin c ip a l P h y s io th e ra p is t, J o h a n n e s b u rg H o s p ita l. R e c ie v e d 1 M a y 1981. O P S O M M I N G D ie ro l van die fis io te ra p e u t in die b e h a n d e lin g v an die ste rw e n d e k in d is tw e e v o u d ig : E e r ste n s w o rd b e ­ h a n d e lin g to eg e sp its o p die o n tw ik k e lin g v a n die k in d se p o ten sia a l, o m f u n k s ie te b e h o u , p y n te verlig, en m is v o r m in g te v o o r k o m . T w e e d e n s dra sy to t d ie o n d e r- ste u n in g van d ie o u e rs en fa m ilie by. D ie b e h o e fte aan ’n tu is b e s o e k e n d e fis io te r a p e u t w o r d aangedui. T h e p rim a ry re a s o n fo r a re fe rra l f o r p h y s io th e ra p y tr e a tm e n t w o u ld be to im p ro v e th e p h y sic a l s ta te of th e p a tie n t o r in th e long te rm to im p ro v e th e q u a lity o f life e.g. by k e e p in g th e p a tie n t m o b ile a n d n o t r e v e rtin g to a w h e e lc h a ir to o e arly . T h e se c o n d a ry b e n e ­ fits fro m in v o lv in g th e fa m ily in tr e a tm e n t s h o u ld n o t be u n d e re s tim a te d . A d e fe c t o r d e la y in m o to r d e v e lo p ­ m e n t is o fte n th e firs t sign to b e re c o g n ise d by th e f a m ily th a t so m e th in g m ay b e w ro n g . T h e p a r e n ts m ay re a lis e t h a t th e c h ild is m o v in g a b n o rm a lly o r t h a t , m o v e m e n t is la c k in g long b e fo re th e y a re a w a re of( ̂ visu a l, a u d ito ry o r in te lle c tu a l im p a irm e n t. S im ila rly th e loss o f m o to r a b ility e.g. in m u s c u la r d y s tro p h ie s o r c e re b ra l a n d sp in a l tu m o u rs , m a y be th e firs t sign o f th e d isease. T h e p h y s io th e ra p is t m ay fo r th e s e re a s o n s re c e iv e th e firs t call fo r h e lp , so m e ­ tim e s e v en b e fo re a d iag n o sis is m a d e . T h e a im s o f p h y s io th e ra p y th e n a re: • A c tiv e tr e a tm e n t is th e n in d ic a te d to d e v e lo p th e in ­ f a n t o r ch ild to its m a x im u m p o te n tia l in a ll a re a s, re s p ira to ry , m u s c u lo -s k e le ta l o r n e u ro lo g ic a l. E m ­ p h a s is w o u ld b e in th e a re a w h ic h is m o s t a ffe c te d , e.g, a m o to r o r m u sc u lo -s k e le ta l p ro b le m c a u s in g a d e la y in m o to r d e v e lo p m e n t o r a b n o rm a l d e v e lo p ­ m e n t w o u ld r e q u ir e a n e u ro -d e v e lo p m e n ta l th e ra p y o r o th e r a p p r o p r i a te d e v e lo p m e n ta l p ro g ra m m e . A c h ild w ith c y stic fib ro sis w o u ld r e q u ir e an ex erc ise p ro g ra m m e a im e d a t re a c h in g th e g re a te s t v ita l c a p a ­ c ity p o s s ib le f o r th a t c hild. • I t is i m p o r ta n t to m a in ta in th e s tre n g th a n d m o b ility o f a ll th e m u sc le s a n d th is w o u ld b e e sp e c ia lly in d i­ c a te d in th e tr e a tm e n t o f th e m u s c u la r d y stro p h ie s . I t R ep ro du ce d by S ab in et G at ew ay u nd er li ce nc e gr an te d by th e P ub lis he r (d at ed 2 01 3. ) SEPTEMBER 1981 P H Y S I O T H E R A P Y 71 is a ls o im p o r ta n t to m a in ta in m u sc le s tre n g th d u rin g a le n g th y p e r io d o f b e d re st w h a te v e r th e c o n d itio n . • T h e p r e v e n tio n o f c o n tra c tu re s a n d d e fo rm itie s is o f p rim e c o n s id e ra tio n . T h e p h y s io th e ra p is t m u s t b e f a m ilia r w ith a ll a sp e c ts o f th e d ise a s e a n d its ex ­ p e c te d p ro g re s s io n a n d m u s t n o t w a it f o r th e d e v e lo p ­ m e n t o f a d e f o rm ity b e fo re ta k in g a c tio n . E a rly sp lin tin g , s ta r tin g p e rh a p s w ith n ig h t s p lin ts is es­ se n tia l. I t is th e re s p o n s ib ility o f th e p h y s io th e ra p is t to e n s u re t h a t th e c h ild w ill g row u p to lo o k as n o r m a l as p o ssib le . “ P rid e in p h y s ic a l a p p e a ra n c e a n d p ro w e ss is a t its p e a k in a d o le s c e n c e ” (S tra k e r a n d S c h m a m a n , 1981). G h a stly , u n s ig h tly d e fo rm i­ ties a re d istre s sin g to p a tie n t, fa m ily a n d so c iety . I r e fe r h e re e sp e c ia lly to th e u n tr e a te d m u sc u lo -d y s- tro p h ie s w h o c a n n o t e ven sit in a c h a ir d u e to se v e re sp in a l d e fo rm itie s . T h e s e d e fo rm itie s in v o lv e th e rib c age a n d le a d to r e s p ir a to r y a n d c a rd ia c fa ilu re . A c o m b in e d p ro g ra m m e o f e x erc ise, sp lin ta g e a n d p o s itio n in g n e ed s to b e s ta rte d e a rly in o r d e r to m in im is e su c h d e fo rm itie s. ® T h e c h ild m u s t b e a ssiste d to re m a in as in d e p e n d e n t as p o ss ib le f o r as lo n g as p o ssib le . In d e p e n d e n c e , a t w h a te v e r s ta g e o r w h a te v e r level, is th e b a c k b o n e to th e in d iv id u a l’s in te g rity a n d fe e lin g o f w o rth . C re e p in g o r c ra w lin g , w a lk in g w ith o r w ith o u t aids, s ta n d in g u p fro m a c h a ir a n d sittin g d o w n a g ain , a c tiv itie s o f d a ily living lik e fe e d in g , d re ssin g , to ile ttin g etc. h e lp to do this. T h e s e a c tiv itie s a n d skills a lso le sse n d e m a n d s m a d e o n th e fa m ily o r sta ff. I t is in th is a r e a t h a t th e in g e n u ity o f th e p h y s io th e ra p is t is e sse n tia l in te rm s o f a id s a n d a d a p ta tio n s . S h e n e e d s to w o rk in c lo se c o -o p e ra tio n w ith th e o c c u p a tio n a l th e ra p is t w h e re v e r p o ssib le . • A s th e d ise a s e p ro g re s se s th e a im s o f tre a tm e n t c h a n g e a n d b e c o m e m o re p a llia tiv e . W h e re p o ss ib le th e th e r a p is t h e lp s to a lle v ia te th e p a in a n d m a k e th e c h ild as c o m fo rta b le as p o ssib le . T h e fin a l cause of d e a th , w h a te v e r th e c o n d itio n , is o f te n p n e u m o n ia a n d r e s p ira to ry fa ilu re . W h e n th e r e a lis a tio n t h a t a c u re o r e v e n a f u n c tio n a l im p ro v e m e n t is n o t p o s ­ sib le a n d a f te r c o n s u lta tio n a n d c a re fu l c o n s id e ra tio n w ith all m e m b e rs o f th e te a m , a c tiv e tr e a tm e n t to p ro lo n g life m a y b e p r o lo n g in g s u ffe rin g a n d c o uld th e n be c o n tra in d ic a te d . T h e a im s o f p h y s io th e ra p y w o u ld b e to m a k e b r e a th in g e a s ie r a n d p r e v e n t th e p a tie n t fro m c h o k in g in t h e ir o w n se c re tio n s , by v e ry c a re fu l su c tio n in g . S u d d e n t e r m in a tio n o f th e ra p y s h o u ld b e a v o id e d . “ A n in e -y e a r-o ld d e sc rib e d h o w f rig h te n e d s h e w as w h e n n o b o d y ta lk e d to h e r a b o u t h e r d o w n h ill c o u rse . I t w as like th e y w e re g e ttin g re a d y fo r m e to d ie .” (C y n th ia B irre r, 1979). T h e c h ild c o u ld b e m a d e m o re c o m ­ fo rta b le by c h a n g in g p o s itio n s a n d su g g e stin g fr e ­ q u e n t a lte ra tio n o f p o sitio n . T h is s o rt o f a d v ic e a n d m a n a g e m e n t c o u ld c h a n g e fro m w eek to w eek, or even d a ily , as th e c o n d itio n d e te rio ra te s . • T h e p h y s io th e ra p is t p la y s a la rg e p a r t in s u p p o rtin g th e fa m ily d u rin g th is tim e o f stress. T h e d iag n o sis o f a fa ta l d ise a s e m ay b e m a d e e a rly in th e life of th e c h ild a n d th e p a r e n ts n e e d to r e ta in so m e h o p e a n d a n tic ip a tio n f o r th e fu tu re , h o w e v e r s h o r t i t m a y be. “ I t is th is g lim p se o f h o p e w h ic h m a in ta in s th e m th ro u g h th e d a y s, w eek s o r m o n th s o f su ffe rin g .” (E liz a b e th K iib le r-R o ss , 1978). T h e th e r a p is t sh o u ld sh o u ld n e v e r sa y t h a t th e r e is n o m o re t h a t c a n be d o n e f o r th e ch ild a n d t h a t tr e a tm e n t m u s t b e t e r ­ m in a te d . I t is th e s e w o rd s t h a t se n d th e p a r e n ts in to d e s p a ir a n d in to a d e s p e ra te se a rc h f o r a n y a lte rn a tiv e t r e a t ­ m e n t w h ic h m a y b e co stly a n d in e ffe c tu a l. I n itia lly th e c h ild m a y be tre a te d d a ily a n d la te r w e ek ly or m o n th ly , as in d ic a te d . A c o m p re h e n s iv e h o m e p r o ­ g ra m m e m u s t be in c lu d e d a n d its success w ill d e p e n d o n th e skill o f th e p h y s io th e ra p is t to m o tiv a te th e p a re n ts to c a rry it o u t a f te r e x p la in in g e x a c tly w h a t is r e q u ire d a n d te a c h in g th e m e x a c tly h o w to e x e c u te it. I n v iew o f th e fa c t th a t th e in fa n t o r c h ild is g ro w ­ ing a n d d e v elo p in g , th e tr e a tm e n t w ill be v a rie d as th e n e e d s o f th e c h ild c h a n g e , e.g. a id s w ill b e c o m e to o sm a ll a n d ex erc ise w ill c h a n g e as th e ch ild grow s, a lth o u g h t h e re a so n fo r d o in g th e e x erc ise will re m a in th e sa m e. P a re n ts s h o u ld b e m a d e to fe e l fre e to c o n ­ ta c t th e th e r a p is t a t a n y tim e w ith o u t fe e lin g g u ilty , sh o u ld th e n e e d a rise. A w o rd o f a d v ic e o r a s y m p a ­ th e tic e a r o n th e te le p h o n e is o fte n th e m o s t c o m ­ fo rtin g a n d su p p o rtiv e . T h e p h y s io th e r a p is t m a y o fte n sp e n d m o re tim e th a n a n y o t h e r m e m b e r o f th e te a m w ith th e p a tie n t a n d fa m ily by v irtu e o f th e n a tu r e of th e tr e a tm e n t. H e r a ss o c ia tio n w ih th e fa m ily m a y b e o v e r m a n y y e a rs a n d she m a y fin d h e rs e lf d e e p ly e m o tio n a lly in v o lv e d — sh e m a y n e e d t h e s u p p o rt of th e o th e r m e m b e rs o f th e te a m to h e lp h e r s o r t o u t h e r ow n fe elin g s, e.g. th e so c ial w o rk e r o r p sy c h o lo g ist. S he is p a r t o f th e te a m w h ic h c o n sists o f c o n s u lta n ts a n d o th e r m e d ic a l p e rs o n n e l, so c ial w o rk e r, p s y c h o lo ­ gists, sp e e c h th e ra p is ts , o c c u p a tio n a l th e ra p is ts , n u rse s a n d s p iritu a l lea d ers. T h e m e m b e rs o f th e te a m w ill v a ry d e p e n d in g on th e p a r ti c u l a r n e e d s o f th e c h ild a n d th e y w ill n e e d to w o rk c lo sely to g e th e r. R e g u la r case c o n fe re n c e s w ill n e ed to b e h e ld . T h e s e services a re a v a ila b le a t m o s t la rg e h o s p ita ls a n d in s titu tio n s . B u t, w h a t o f th e fa m ily w ho w ishes to k e e p th e ir ch ild a t h o m e fo r as lo n g as p o ssib le ? I t is h e re t h a t th e se rv ice s o f a d o m ic ilia ry p h y s io th e ra p is t w o u ld be m o s t b e n e fic ia l. S u ch a t h e r a p is t c o u ld still f u n c tio n as p a r t o f th e te a m a t th e h o sp ita l, b u t w o u ld also b e a b le to c a rry o u t h e r tre a tm e n t in th e c o m fo rt a n d s e c u rity o f th e c h ild ’s h o m e . A fte r th e d e a th o f th e c h ild th e fa m ily w ill n e e d th e a s s u ra n c e t h a t e v e ry th in g p o ss ib le w as d o n e fo r th e ir c h ild a n d t h a t h e d ie d p e a c e fu lly . T h e p h y s io th e ra p is t, h a v in g b e e n fu lly in v o lv e d , w ill be a b le to c o n trib u te to th is a ssu ra n c e , so t h a t th e fa m ily w ill a lso k n o w t h a t th e ir ch ild re a lis e d h is fu ll p o t e n ­ tial, h o w e v e r lim ite d . R eferen ces B irre r, C. (1979). T h e d y in g c h ild : D e a th a n d dying. U n iv e rs ity o f th e a ir. P u b lic a tio n o f th e S o u th A f r i­ c a n B ro a d c a s tin g C o r p o r a tio n IS B N 0869720481. F rie d m a n , C h o d o ff, M a s e n a n d H a m b e r g (1963). B e h a ­ v io u ra l o b s e rv a tio n s o f p a r e n ts a n tic ip a tin g d e a th o f a c h ild . P a ed ia trics, 32, 6 1 0 - 625. Irw in, C. (1977). T h e c o n c e p t o f d e a th in c h ild re n . T h e L e e c h , J o u r n a l o f W itw a te r s r a n d M e d ic a l S c h o o l. 47 (a). K iib le r-R o ss, E . (1975). D e a th th e final sta g e o f g ro w th . P r e n tic e H a ll. N e w Je rse y . U S A . K iib le r-R o ss, E. (1978). O n d e a th a n d d y in g . T a v isto c k , L o n d o n . K iib le r-R o ss , E. (1974). Q u e s tio n s a n d a n sw e rs on dying. M c M illa n . N ew Y o rk . S tra k e r, G ., S c h m a m a n , F . (1980), C o u n s e llin g th e p a r e n ts o f th e d y in g c h ild . L e c tu re s U n iv e rs ity of W itw a te rs ra n d (U n p u b lish e d ). R ep ro du ce d by S ab in et G at ew ay u nd er li ce nc e gr an te d by th e P ub lis he r (d at ed 2 01 3. )