SEPTEMBER 1981 P H Y S I O T H E R A P Y 67 F a r b e r, S. (1966). C h e m o th e ra p y in th e tre a tm e n t of le u k a e m ia a n d W ilm s tu m o u r. J .A .M . A . , 198, 826 - 828. G e o rg e, S. L., A u r, R. J. A ., M a u e r. A . M . a n d S im one, J. V. (1979). A r e a p p ra is a l o f th e r e s u lts o f sto p p in g th e ra p y in c h ild h o o d le u k a e m ia . N e w E n g l. J. M e d ., 300, 2 6 9 - 2 7 3 . H a n e s, B., G a r d n e r , M . B., L o o sli, C . G ., H e id b re d e r, G ., K o g a n , B ., M a ry la n d e r, M . a n d H e u b n e r. R . J. (1970). P e t a sso c ia tio n s w ith se lected h u m a n cancers: A h o u se h o ld q u e s tio n n a ire survey. J. N a ll. C a n c er In st., 45, 1 1 5 5 - 1162 K a p lan , H . S. (1974). L e u k a e m ia a n d ly m p h o m a in ex­ p e rim e n ta l a n d d o m e s tic a n im a ls . Ser. H a e m a t o l , 7, 94 - 103. K a ra b u s , C. D . a n d H a rtle y , P . S. (1981). P ro g n o s is in a c u te ly m p h o c y tic le u k a e m ia . S u b m itte d to S A M e d i­ cal C ongress, P re to ria . M iller, R . W . (1967). P e rso n s w ith e x c e p tio n a lly h ig h risk o f le u k a e m ia . C a n c e r R e s ., 27, 242 0 - 2423. N e sb it. M . E ., S a th e r, H . N ., R o b iso n , L. L ., O rte g a, J., L u tm a n , P. S., D ’A n g io , G . J. a n d H a m m o n d , G . D . (1981). P re s y m p to m a tic c e n tra l n e rv o u s system th e ra p y in p re v io u s ly u n tre a te d c h ild h o o d a c u te ly m ­ p h o b la s tic leu k a e m ia : C o m p a ris o n o f 1 800 r a d a n d a n d 2 400 r a d . L a n c e t 1, 461 - 466. S c h a lle r, J. (1972). A rth ritis as a p re s e n tin g m a n if e s ta ­ tio n o f m a lig n a n c y in c h ild re n . J. P ediatr., 81, 793 - 795. T h o m a s, E . E)., S a n d e rs, J. E ., F lo u r n a y , N ., J o h n s o n , F . L ., B u c k n er, C. D ., C lift, R . A ., F e fe r, A ., G o o d e ll, B. W ., S to rb , R . a n d W e id e rn , P. L . (1979). M a r r o w tra n s p la n ta tio n f o r p a tie n ts w ith a c u te ly m ­ p h o b la s tic le u k a e m ia in re m issio n . B lo o d , 54, 468- 476. W ein stein , H . J., M a y e r, R . J., R o s e n th a l, D . S., C a m itta , B. M ., C o ra l, F . S., N a th a n , D . G . a n d F re i, E . (1980). T re a tm e n t o f a c u te m y e lo g e n o u s le u ­ k a e m ia in c h ild re n a n d a d u lts. N e w E ngl. J. M e d ., 303, 473 - 478. W itts, L . J. a n d o th e rs (1971). D u r a tio n o f su rv iv a l of c h ild re n w ith a c u te le u k a e m ia . B rit. M e d . / . , 4, 7 - 9 . T H E CHILD WITH HAEM O PH ILIA C. D . K A R A B U S , M .B . C h.B ., M .M e d . (P aed ), D .C .H .,M .R .C .P ., F .R .C .P .E .* S U M M A R Y H a e m o p h ilia s are b lee d in g d iso rd e rs d u e to an in ­ h e rite d d e fe c t in p r o d u c tio n o f c lo ttin g fa cto rs. I n S o u th A fr ic a 867 h a e m o p h ilia c s h a v e been re g istered a n d 309 o f these are b o y s u n d e r 15 y e a r s o f age. H a e m o p h ilia A a n d B m a k e up a b o u t 85 - 9 0 % o f a ll cases. T h e y are in h e r ite d in a s e x -lin k e d m a n n e r a n d th u s a ffe c t m a le s o n ly . T h e m a n ife s ta tio n s o f h a e m o p h ilia are due to b leeding chara c te ristica lly in v o lv in g the jo in ts. T he d iso rd er v aries in se v e rity a n d in th e m o s t se v ere ly a ffe c te d ch ild re n , re p e a te d h a e m a rtliro se s m a y lea d to crippling. E arly a d m in istra tio n o f c lo ttin g fa c to r, p r e ­ fera b ly g iv e n a t th e c h ild ’s h o m e , p h y s io th e r a p y to p re v e n t m u sc le w a stin g a n d reg u la r a ss e s sm e n t by a c o -o rd in a te d te a m o f paediatrician, p h y sio th e ra p ist, o r­ th opaedic su rg eo n a n d d e n tis t a t the h o s p ita l are n eces­ sary fo r su c c e ssfu l m a n a g e m e n t. T h e se fa c ilitie s can he Tbrganised o n ly at a c o m p re h e n s iv e h a e m o p h ilia tre a tm e n t “centre treating a large n u m b e r o f bleeders. H a e m o p h ilia is a te rm used f o r a g ro u p o f life -lo n g b lee d in g d iso rd e rs re s u ltin g fro m a n in h e rite d d e fe c t in th e p ro d u c tio n o f o n e o r o th e r o f th e p la s m a c lo t­ tin g fa c to rs. T h e s e p ro te in s , re s p o n s ib le f o r n o rm a l b lo o d c o a g u la tio n , a re d e sig n a te d b y th e R o m a n n u m e ra ls I to X I I I . T h e c o m m o n e s t d is o rd e r is h a e m o ­ p h ilia A o r cla ssic h a e m o p h ilia w h ic h w a s p ro b a b ly re co g n ise d as e a rly as th e se c o n d c e n tu ry . T h e B a b y lo n ia n T a lm u d d e sc rib e s a d e c is io n o f R a b b i S im o n b e n G a m a lie l to ex cu se fro m th e r ite o f c ir­ cu m c isio n th e n e w b o rn so n o f a w o m a n w h o se siste rs’ sons b led to d e a th on th e b a s is th a t ‘th e r e a re fa m ilie s w h o se b lo o d is lo o se , w h ile in o th e rs it c o a g u la te s ’. In 1803 th e firs t m o d e rn d e s c rip tio n w as g iven b y J. C. O tto . H a e m o p h ilia w a s c alled th e ‘b le e d e rs d ise a s e ’ a nd la te r ‘th e d ise a s e o f K in g s ’ b e c a u se o f its o c cu r- H a e m a to lo g y /O n c o lo g y S ervice, R e d C ro ss W a r M e m o ria l C h ild r e n ’s H o s p ita l a n d D e p a r tm e n t of P a e d ia tric s, U n iv e rs ity o f C a p e T o w n . R e ce iv e d 1 M a y 1981. O P S O M M IN G H e m o filie is ’n b lo e d in g sie k te te w y te aan 'n oorge- e rjd e d e fe k in die v e rva a rd ig in g v an sto lH n g sfa k to r e . In S u id - A fr ik a is 867 lie m o filie ly e r s g eregistreer en 309 g e va lle is s e u n s o n d e r 15 jaar. H e m o filie A en B be- slaan o m tr e n t 85 - 9 0 % van a lle g evalle. H u lle w o rd o o rg e e rf in ’n s e k s g e s k a k e k le m a n ie r en ta s d u s slegs m a n lik e lye rs aan. D ie k e n m e r k e v an h e m o filie is te w y te aan b lo e d in g s, w at s p e s ifie k g e w rig te a antas. D ie sie k te v arieer in d ie gra a d v a n a a n ta stin g en in d ie k in ­ d e rs w at ergste a a n g e ta s is, k a n h e rh a a ld e h e m a r tr o s e tot erge k r e u p e lh e id lei. V ro e e to e d ie n in g v a n s to llin g sfa k - tor, v e r k ie s fik tuis, fis io te r a p ie oin sp ie r v e rv a l te voor- k o m en g e re e ld e e v a lu e r in g d e u r ’n g e k o d r d in e e r d e span besta a n d e uit ’n kin d e rsp esia lis, fis io te ra p e u t, o rto p e d ie se c h iru rg en ta n d a rts by die h o s p ita a l h n o d ig v ir sitk- s :s v o lle h a n te rin e . H ie rd ie fa siliteite k an sleg s bv ’n om - v a tte n d e h e m o filie b e h a n d e lin g se n tr u m , w a t ’n g ro o t a a n ta l b lo eie rs b e h a n d el, g e o rg a n ise er w ord. re n c e a m o n g d e s c e n d a n ts o f Q u e e n V ic to ria in th e ro y a l fa m ilie s o f B rita in , S p a in a n d R u ssia . H a e m o p h ilia A is d u e to a d e fic ie n c y in f a c to r V I I I c lo ttin g a c tiv ity w h ile h a e m o p h ilia B (C h ristm a s D ise a se) re su lts fro m d e fe c tiv e f a c to r I X p ro d u c tio n . B o th a re in h e rite d in a re ce ssiv e X -lin k e d m a n n e r a n d th e re fo re , w ith ra re e x c e p tio n s, a re m a n ife s t o n ly in m ales. C lin ic a lly h a e m o p h ilia A a n d B a re in d is tin g u is h ­ a b le a n d to g e th e r th e y m a k e u p a b o u t 85 - 9 0 % o f all c ases o f h a e m o p h ilia . H a e m o p h ilia A is 5 to 6 tim e s m o re c o m m o n th a n h a e m o p h ilia B. O f th e o th e r h a e ­ m o p h ilia s , V o n W ille b ra n d d ise a s e is th e th ird m o st c o m m o n , c o m p risin g a b o u t 1 0 % of p a tie n ts . A ll th e o th e r clotting: fa c to r d e fic ie n c ie s to g e th e r m a k e u p less th a n 5 % o f cases. T h e s e d is o rd e rs a re a u to s o m a lly tra n s m itte d a n d th e r e f o r e m a y a ffe c t fe m a le s as well as m ale s. H A E M O P H IL IA A A N D B H a e m o p h ilia is re la tiv e ly u n c o m m o n b u t n o t ra re . T h e in c id e n c e o f h a e m o p h ilia A a n d B h a s b e e n fo u n 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. ) 68 F I S I O T E R A P I E SEPTEMBER 1981 to b e b e tw e e n 10 a n d 15 p e r 100 0 0 0 m a le s in s u r ­ veys d o n e in th e U .S .A . a n d v a rio u s E u r o p e a n c o u n ­ trie s. In S o u th A fric a (K a ra b u s , 1981) 867 h a e m o ­ p h ilia c s h a v e b e e n id e n tifie d a n d o f th e to ta l 706 (8 1 % ) h a v e h a e m o p h ilia A o r B g iv in g a p re v a le n c e o f 5,4 p e r 100 0 0 0 m a le s. T h e fig u re f o r w h ite s is th e sa m e as th a t in o th e r c o u n trie s w hile th e in c id e n c e in b la c k s is m u c h low er. T h e r e a re 309 b ovs u n d e r 15 k n o w n to h a v e h a e m o p h ilia A o r B in th e R e p u b lic a n d in th is a g e g ro u p b la c k b le e d e rs o u tn u m b e r w hites. IN H E R IT A N C E A N D N A T U U R E O F D E F E C T T h e g en es f o r h a e m o p h ilia A a n d B a re lo c a te d a t s e p a ra te sites o n th e X c h ro m o s o m e . S in c e th e r e a re n o o p p o sin g g e n e s o n th e Y c h ro m o s o m e , th e d is o rd e r is e x p ressed o n ly in th e m a le (X Y ). F e m a le (X X ) c a r ­ rie rs o f th e t r a it a re g e n e ra lly u n a ffe c te d , as th e y a re a b le to p ro d u c e s u ffic ie n t c lo ttin g f a c to r u n d e r th e c o n tro l o f th e ir n o rm a l X c h ro m o s o m e . A h a e m o p h ilic m a le c a n n o t tra n s m it th e d is o rd e r to h is so n s b u t a ll h is d a u g h te rs m u s t b e c a rrie rs o f h a e m o p h ilia . A c a r ­ r ie r fe m a le h a s a 50% risk o f b e a rin g a h a e m o p h ilic so n a n d h e r c h a n c e o f h a v in g a c a r r ie r d a u g h te r is th e sa m e. I n a b o u t o n e th ird o f cases, th e re is n o fa m ily h is to ry o f th e d is o rd e r a n d a s p o n ta n e o u s h a e ­ m o p h ilic m u ta tio n on th e X c h ro m o s o m e m u s t h a v e o c c u rre d . T h is d e fe c tiv e g e n e c a n th e n b e tra n s m itte d to su b s e q u e n t g e n e ra tio n s. F o r m a n y y e a rs h a e m o p h ilia w as c o n s id e re d to b e d u e to a q u a n tita tiv e d e fic ie n c y o f c lo ttin g fa c to r. F a n tl a n d c o -w o rk e rs (1956) d isc o v e re d t h a t h a e m o p h ilia c s did p ro d u c e a c lo ttin g f a c to r m o le c u le w h ic h did n o t f u n c tio n n o rm a lly . H a e m o p h ilia is th e r e f o r e d u e to a q u a lita tiv e a n d n o t a q u a n tita tiv e a b n o rm a lity o f th e c lo ttin g fa c to r. T h e p re se n c e o f a f a c t o r V I I I p r o te in w h ic h h a s re d u c e d c lo ttin g a c tiv ity h a s b e e n a u se fu l m e a n s o f d e te c tin g fe m a le c a rrie rs o f th e d iso rd e r. M A N IF E S T A T IO N S A ll th e p ro b le m s in h a e m o p h ilia re s u lt fro m e x cessive b le e d in g a n d its e ffe c t o n th e f u n c tio n o f jo in ts , m u sc le s a n d o th e r o rg a n s. T h e se v e rity o f th e b le e d in g in h a e ­ m o p h ilia v a rie s fro m c ase to case, in g e n e ra l b e in g r e la te d to th e a m o u n t o f fa c to r V I I I o r IX c lo ttin g a c tiv ity p re se n t. T h is m a y ra n g e fro m 5 to ,40%. S e v e re b le e d e rs h a v e less th a n 2 % c lo ttin g a c tiv ity , m o d e ­ ra te ly se v e re 2 - 5 % a n d m o d e ra te to m ild ly a ffe c te d in d iv id u a ls m o re th a n 5 % . T h e a m o u n t o f f a c to r p re s e n t re m a in s c o n s ta n t th r o u g h o u t th e life o f th e h a e m o ­ p h ilia c . In h a e m o p h ilia A a n d B, all a ffe c te d m e m b e rs o f a fa m ilv h a v e th e sa m e level o f c lo ttin g a c tiv ity a n d th u s, u su a lly , o f se v e rity o f sy m p to m s . B lee d in g m a n i­ fe s ta tio n s a re m o s t fr e q u e n t a n d d a m a g in g d u rin g c h ild ­ h o o d . I n se v e re ly a ffe c te d cases, ex ce ssiv e b ru is in g m a y b e n o tic e d d u rin g in fa n c y as th e b a b y s ta rts craw lin g . L a te r as h e le a rn s to w a lk , h a e m a to m a s o n th e f o r e ­ h e a d a n d fa c e a n d b le e d in g fro m a c u t lip o r b itte n to n g u e a re n o t in f r e q u e n t as a re s u lt o f fa lls a n d b u m p s. T h e c h a ra c te ris tic b le e d in g in to jo in ts u su a lly b e co m es o b v io u s f r o m tw o to th r e e y e a rs o f age, c o m ­ m o n ly in v o lv in g th e a n k le s a n d k n e e s. I n th e o ld e r c h ild b le e d s in to th e e lbow jo in ts a r e a ls o fre q u e n t. O th e r jo in ts su c h as th e h ip s a n d s h o u ld e rs a re less o fte n a ffe c te d . T h e fre q u e n c y o f th e b le e d in g e p iso d es d e p e n d s on th e se v e rity o f th e c lo ttin g d e fe c t a n d in c h ild re n w ith less th a n 1 % a c tiv ity b le e d s m a y o c c u r w eekly. T h e a m o u n t o f tr a u m a n e c e s s a ry to in d u c e b le e d in g m a y b e m in im a l in se v e re c ases w h e re a s m ild ly a ffe c te d in d iv id u a ls m a y p la y s p o rt su c h as te n n is o r so c c e r w ith little o r n o b le e d in g . B lee d in g in to jo in ts is th e m a in c a u s e o f disa b ility in h a e m o p h ilia . S e v e re o r re p e a te d h a e m a rth ro s e s re su lt e v e n tu a lly in th e ty p ic a l h a e m o p h y lic a r th r o p a th y . T h e sy n o v ia l tissu e s a re th ic k e n e d , a c h ro n ic e ffu s io n as a re s u lt o f sy n o v itis m a y b e p r e s e n t a n d jo in t m o b ility is re s tric te d . M u s c le w a stin g a r o u n d th e jo in t is the ru le , u n le ss in te n siv e p h y s io th e ra p y is giv en . W eak m u sc le s lead to in sta b ility o f th e jo in t a n d la c k o f p ro ­ te c tio n a g a in st tr a u m a w h ic h m a y c a u s e f u r th e r b le e d ­ ing. U n le ss p re v e n tiv e m e a s u re s a re ta k e n , th is vicious cycle o f b le e d in g , jo in t d a m a g e , m u sc le w a stin g , in ­ sta b ility a n d f u r t h e r b le e d in g m a y r e p e a t itself until th e jo in t is to ta lly d e stro y e d . E v e n tu a lly th e jo in t m ay b e c o m e a lm o s t im m o b ile o r c h ro n ic se v e re p a in m ay n e c e ssita te a rth ro d e s is o r j o in t re p la c e m e n t. R a d io - lo g ic a lly , th e j o in t sp a c e b e c o m e s n a rro w e d , a rtic u la r s u rfa c e s a re irre g u la r a n d d e fe c ts in a lig n m e n t m ay o c c u r. I n th e k n e e th e re is o f te n a v a lg u s d e fo rm ity of th e leg w h ic h re s u lts in a n a b n o rm a l s tra in being p la c e d on th e a lre a d y d a m a g e d jo in t. B le e d in g m a y a lso in v o lv e m u sc le s a n d re s u lt in( m a jo r lo ss o f b lo o d if th e th ig h , b u tto c k o r re tro p e ri­ to n e a l m u sc le s a re in v o lv e d . N e rv e s a n d b lo o d vessels m a y b e c o m p re ss e d if b le e d in g o c c u rs in to th e calf, fo r e a r m o r ilio p so a s m u sc le s a n d s e rio u s d a m a g e m ay fo llo w u n less in te n siv e tr e a tm e n t is g iv en . B lee d in g into th e re n a l tr a c t is n o t u n c o m m o n in o ld e r c h ild re n and m a y c a u s e o b s tru c tio n to u rin e flow . T h e m o s t serious ty p e o f h a e m o r r h a g e a n d th e m a jo r c a u se o f d eath in h a e m o p h ilia is in tra c ra n ia l b le e d in g . T h is m a y be re la te d to tr a u m a to th e h e a d b u t in se v e re h a e m o ­ p h ilia c s n o t in fre q u e n tly th e re is n o h is to r y o f any p re c ip ita tin g c au se. I n v iew o f th is a n y h a e m o p h ilia c w ho c o m p la in s o f a se v e re o r p ro lo n g e d h e a d a c h e s h o u ld b e g iv en tre a tm e n t fo r a p o te n tia l cerebral h a e m o rrh a g e . M A N A G E M E N T T h e o v e ra ll m a n a g e m e n t o f th e c h ild w ith h a e m o ­ p h ilia is c o m p lic a te d a n d e x p en siv e . T h e c ost o f c lot­ tin g f a c to r a lo n e n e e d e d to tr e a t a sin g le jo in t bleed in a b o y o f 10 m a y w ell a m o u n t to R 2 5 0 . I n a d d itio n to th e p a e d ia tric ia n o r h a e m a to lo g is t, th e m a n a g e m e n t te a m sh o u ld a ls o in c lu d e a p h y s io th e ra p is t, o rth o p a e d ic su rg e o n , c lin ica l n u rse , so c ial w o rk e r a n d dentist. L a b o r a to r y fa c ilitie s sh o u ld in c lu d e te c h n o lo g is ts train ed in c o a g u la tio n a n d a n a d e q u a te su p p ly o f tr e a tm e n t m a te ria ls . I n view o f th e c o m p le x ity o f m a n a g e m e n t a n d th e re la tiv e ly sm a ll n u m b e r o f h a e m o p h ilia c p a tie n ts, th e re is little d o u b t th a t c e n tr a lis a tio n is e sse n tia l. In th e U S A , B rita in a n d E u r o p e la rg e h a e m o p h ilia cen­ tre s h a v e b e e n e sta b lish e d w h e re th e c o m p re h en siv e c a re n e e d e d b y th e p a tie n t c a n b e p ro v id e d . I t h a s been su g g e ste d (L ev in e, 1980) t h a t a m in im u m n u m b e r o f 250 h a e m o p h ilia c s a re re q u ire d in o r d e r to k e e p th e services a t a h a e m o p h ilia c e n tre fu n c tio n in g a d e q u a te ly . T reatm ent o f B leed in g E p isod es F re s h fro z e n p la s m a w as firs t u s e d as a m e a n s of re p la c in g th e m issin g c lo ttin g fa c to r b y G r a h a m e t al., in 1949. T h e effe c tiv e n ess o f th ese tra n s fu s io n s was lim ite d by th e la rg e v o lu m e n e c e ssa ry to ra is e a p a tie n t’s c lo ttin g f a c to r su ffic ie n tly to sto p b le e d in g . T h e p lasm a h a d to b e s to re d d e ep fro z e n to r e ta in its p o ten c y a n d th is m e a n t th a t c h ild re n h a d to c o m e to h o sp ital fo r tre a tm e n t. T h e u su a l h o s p ita l d e la y s, in cluding w a itin g fo r th e p la s m a to th a w , in e v ita b ly led to the c h ild m issin g sc h o o l a n d fa llin g b e h in d in his e d u ca ­ tio n . M o re c o n c e n tra te d fo rm s o f f a c to r V I I I a n d IX a re n o w a v a ila b le as c ry o p re c ip ita te o r f a c to r IX con- 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 69 r e n tr a t e a n d th e s e c a n b e d rie d fo r e asy s to ra g e a n d r e c o n s t i t u t i o n . E a rly t r e a tm e n t a s so o n as b lee d in g c o m m e n ce s will re d u c e p a in a n d d isa b ility . I t sh o u ld he e m p h a sise d t h a t th e p a tie n t a n d n o t th e d o c to r i s th e b e st ju d g e o f w h e th e r a b lee d h a s s ta rte d . H o m e tr e a tm e n t w ith a d m in is tra tio n o f c o n c e n tra te bv th e m o th e r o r e ven b y th e c hild h im s e lf s h o u ld now be th e r u le f o r m o s t se v e re b le e d e rs . T h is re d u c e s tre a tm e n t d e la y a n d d e c re a s e s a b se n c e fro m sc h o o l. T h e h a e m o p h ilia c ’s life c a n b e m u c h m o re n o rm a l •is h e is n o lo n g e r tie d to a h o sp ita l; in a d d itio n ex ­ penses in v o lv e d in h o s p ita l v isits a re sp a re d . A c lose re la tio n s h ip b e tw e e n th e ch ild o n h o m e tr e a tm e n t a n d th e h o sp ital, m u st, h o w e v e r, still b e m a in ta in e d . P a tie n ts sh o u ld be a b le to te le p h o n e f o r a d v ic e a b o u t tre a tm e n t an d c h ild re n s h o u ld be se e n a t th e h a e m o p h ilia c e n tre th re e to f o u r tim e s p e r y e a r fo r a c h e c k b y th e tea m o f p a e d ia tric ia n , p h y s io th e ra p is t, o rth o p a e d ic s u r­ geon a n d d e n tis t. P o te n tia lly d a n g e ro u s b le e d in g s u c h as su spected c e re b ra l b le e d in g m u s t a lw ay s b e tre a te d a t ^hospital. T h e a m o u n t o f f a c to r re p la c e m e n t g iv e n d e p e n d s on the se v e rity o f th e b le e d . F o r th e a v e ra g e jo in t b le e d a b o u t 10 u n its o f fa c to r V I I I o r 20 u n its o f f a c t o r IX per k ilo g ra m b o d y w e ig h t is g iv en in tra v e n o u s ly . T h is will raise th e level o f f a c to r V I I I o r I X to 2 0 % w h ic h is s u ffic ie n t to sto p b le e d in g . A s th e e ffe c t o f in fu se d c lo ttin g fa c to r is o n ly te m p o ra ry , r e p e a t d o se s m a y b e n e ce ssa ry a t 6 - 1 2 h o u r in te rv a ls u n til a ll p a in d is­ a p p ea rs. L a rg e r d o se s g iven fo r lo n g e r p e rio d s m a y b e n e ed e d fo r m o re se rio u s b le e d in g . T h e in v o lv e d lim b sh o u ld be sp lin te d to p re v e n t m o v e m e n t a n d n o w e ig h t be arin g sh o u ld be allo w e d u n til f u n c tio n is re sto re d . P h y s io th e ra p y is o n e o f th e m o s t im p o r ta n t a sp e c ts o f tre a tm e n t. T h e k n e e jo in t is m o s t c o m m o n ly in ­ volved in b le e d in g . Q u a d ric e p s w a stin g r a p id ly fo llo w s a h a e m a rth ro sis. T o p re v e n t th is, s ta tic q u a d ric e p s e x e r­ cises a re s ta rte d w ith in h o u rs o f th e b le e d (as soon as m o v e m e n t is p a in free ) a n d th is is fo llo w e d by stra ig h t leg ra isin g a n d e x te n sio n ex erc ises a g a in s t g ra d u a te d re sista n c e . I t is e x tre m e ly im p o rta n t, a n d v e ry d iffic u lt, to m o tiv a te sm a ll c h ild re n to d o p h y s io th e ra p y a t h o m e. P a re n ta l c o -o p e ra tio n m u s t b e o b ta in e d a n d re p ea te d e x p la n a tio n s o f th e im p o rta n c e o f p h y s io th e ra ­ py m ay be ne ce ssa ry . O rth o p a e d ic m e a su re s su c h as tra c tio n o r se rial jw e d g in g o f p la s te r o f p a ris sp lin ts to re s to re fu ll ex ­ t e n s i o n to a jo in t m a y b e n e ed e d . H o f m a n n (1981) has em phasised th e im p o rta n c e o f m a in ta in in g n o r m a l jo in t alig n m e n t a n d a d v ises s u p ra c o n d y la r f e m o ra l o s te o to m y to c o rre c t th e v a lg u s d e fo rm ity w h ic h o f te n fo llo w s r e ­ c u rre n t h a e m a rth ro s e s o f th e k n e e . H e feels th a t p h y ­ sio th e ra p y is u n lik e ly to b e e ffe c tiv e u n til a lig n m e n t is co rrected. Prophylactic Treatm ent A lth o u g h m o s t h a e m o p h ilia c e n tre s a d v is e tre a tm e n t c o n c e n tra te fo r b le e d in g e p is o d e s o n ly , E tz el iT-1 - ^ aS mac*e a g o o d c ase fo r tre a tin g se v e re h a e m o ­ p h iliacs p ro p h y la c tic a lly . B y r e g u la r tw ice w e e k ly a d ­ m in istra tio n o f fa c to r c o n c e n tra te s th e p a ti e n t ’s c lot- r i a, ' t y is m a in ta in e d a t a level a t w h ic h h e is u n likely to b lee d fre q u e n tly . A lth o u g h th e c o st o f su c h p ro p h y la c tic t r e a tm e n t is h ig h — in S o u th A fric a n term s a b o u t R 6 0 00 p e r y e a r fo r a 25 k g b o y — this m ust b e o ffse t a g a in s t th e p o ss ib ly o n ly slig h tly lo w e r •>°h a c u te tr e a tm e n t o f r e c u r r e n t b le e d in g e p iso d es