The D a ily P h y s io th e r a p y R eg im en Physiotherapy, A u g u s t 1986, vol 42 no 3 7 3 in C ystic F ibrosis? L. M. DAVIDS SUMMARY ! The aim of this paper is to question the accepted physiotherapy regimen for children with Fibrocystic Disease. Is it the most effective way of keeping the lungs clear of secretions? J How much are we imposing added stress on 1 an already stressed family? Is it not possible to ! design a programme which reduces the stress and is socially acceptable? The traditional physiotherapy regimen of j breathing exercises, postural drainage and per- j cussion is described. Forced expiratory tech- ' nique is described as well as various forms of nebulisation. The psychological impact on the family of a | chronic, life-threatening disease, is discussed. ! Compliance with set physiotherapy regimens j and the family's (especially the mother's) reac­ tion to them is discussed. The most recent literature on the effective- ; ness of traditional physiotherapy techniques is | reviewed. j From this review as well as from personal experience, it is concluded that a rigid physio­ therapy regimen is stressful and compliance is frequently poor. Suggestions are made how to overcome this. It is stressed that this paper deals with the home programme, not with the ^ hospital programme for acute exacerbations of y the disease. IN T R O D U C T I O N C y s tic F ib r o s is ( C F ) is th e m o s t c o m m o n o f th e g e n e tic a lly d e te r m i n e d d is e a s e s a m o n g C a u c a s i a n s ' a n d as s u c h is th e m o s t c o m m o n c a u s e o f c h r o n ic s u p p u r a ­ tive lu n g d is e a s e in w h ite c h ild r e n .2 T h e c a lc u la te d h e te r o z y g o te r a te in C a u c a s ia n s is a p p r o x i m a t e l y 1:20. L. M. D avids C o n tr o l P h y sio th e ra p ist, R ed C ro ss W a r M e m o ria l C h il­ d r e n ’s H o sp ita l, R o n d e b o sc h , C ape P a p e r read a t C o n g re ss o f the S o u th A frica n S ociety of P h y s io th e ra p y , J o h a n n e s b u rg . A pril 1985 OPSOMMING Die doel van hierdie eksemplaar is om die aanvaarde fisioterapie regimen vir kinders met Fibrosistiese Siekte te ondervra. Is dit die mees doeltreffende manier om die longe vry van sekresies te hou? Hoeveel ekstra spanning le ons op 'n familie wat al reeds gespanne is? Is dit nie moontlik om ’n program te skets wat die spanning verminder en sosiaal aanvaarbaar is nie? Die tradisionele fisioterapie regimen van asemhaling oefeninge, posturale dreinering en beklopping word beskryf. Die gedwonge ekspi- ratoriese tegniek sowel as verskeie vorms van nebulisasie word beskryf. Die s ie lk u n d ig e slag van 'n c h ro n ie s e lewens-dreigende siekte op die familie word bespreek. Die nakoming van vaste fisioterapie leefreels en die reaksie van die familie (en veral die moeder) daartoe word bespreek. Die mees onlangse literatuur oor die doeltref- fendheid van tradisionele fisioterapie tegnieke word nagegaan. Van hierdie oorsig asook van persoonlike ondervinding word dit afgelei dat ’n stywe fisio­ terapie regimen spannend is en dat nakoming dikwels swak is. Voorstelle word gemaak hoe dit oorkom kan word. Dit word beklem dat hierdie eksemplaar oor die tuis-program handel en nie oor die hospitaal-program vir akute opvlammings van die siekte nie. T h e r e p o r t e d in c id e n c e o f C F in w h ite p o p u l a t i o n s v a rie s b e tw e e n 1 :1 0 0 0 to 1:7 0 0 0 . A p a r ti c u l a r ly h ig h in c id e n c e o c c u r s in th e d e s c e n d a n ts o f th e ‘D o r s la n d T r e k k e r s ’ fo u n d in S o u t h W e s t A fric a . T h e c a lc u la te d c a r r ie r ra te is 1:12 in th is g r o u p . 3 C F is e x tr e m e l y r a r e a m o n g S o u t h A f r ic a n b la c k s a n d to d a te n o b la c k c h ild a tt e n d s th e C F c lin ic a t th e R e d C r o s s W a r M e m o r ia l C h i ld r e n ’s H o s p it a l. T h e in c id e n c e a m o n g th e c o lo u r e d c o m m u n i ty is n o t k n o w n b u t it is lo w e r t h a n t h a t o f th e w h ite s in S o u t h A f r ic a .3 It is g e n e ra lly a c c e p te d t h a t c h e s t p h y s i o t h e r a p y p la y s a la r g e ro le in th e t r e a tm e n t re g im e n f o r C F a n d t h a t th e i m p r o v e m e n t in th e s u r v iv a l o f th e s e p a ti e n ts is in 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. ) 74 Fisioterapie, A u g u s tu s 1986, d e e l 42 no 3 p a r t d u e to th e u s e o f p h y s i o t h e r a p y .4 T h e p h y s i o t h e r a ­ p is t p la y s a v e ry im p o r t a n t ro le in th e lives o f C F c h ild r e n a n d t h e ir fa m ilie s. T h e tim e h a s c o m e to a sse ss th e d e m a n d s w e m a k e u p o n th e p a r e n t s a n d th e c h ild a n d to c o n s id e r w h e t h e r we a re n o t m a k i n g life e v e n m o r e d if f ic u lt f o r th e fa m ilie s c o n c e r n e d . T h is w as b r o u g h t h o m e to m e w h e n a r e m a r k m a d e b y th e m o th e r o f a re c e n tly d e c e a s e d ch ild w as re p o rte d to m e. T h e ch ild w as se v e re ly a ffe c te d a n d d ie d a t a p p r o x im a t e l y 7 y e a rs o f a g e. W h e n th is h a p p e n e d th e m o th e r s a id , “T h a n k g o o d n e s s I d o n ’t h a v e to d o th e p h y s io a n y l o n g e r ”. I t w a s th e p h y s io ­ th e r a p y re g im e n d e m a n d e d o f th is m o th e r w h ic h se e m e d to h e r to b e th e w o r s t a s p e c t o f th e d ise a s e . T H E T R A D I T I O N A L P H Y S I O T H E R A P Y R E G IM E N T h is c o n s is ts o f p o s t u r a l d r a in a g e w ith p e rc u s s io n , v i b r a ti o n , b r e a th in g e x e rc is e s a n d c o u g h in g . P o s tu r a l d r a in a g e c o n s is ts o f a v a r ie ty o f p o s i tio n s in w h ic h th e p a ti e n t is p la c e d w h e re g r a v ity a ss is ts th e d r a in a g e o f s e c re tio n s . A c c o r d i n g to H o d s o n a n d G a s - k e ll4 it m a y t a k e u p to 15 m in u te s in a n o ld e r c h ild to d r a i n a n y o n e a r e a a n d s e v e ra l a r e a s m a y n e e d to be d r a in e d . A t r e a t m e n t se s s io n c o u ld la s t u p to a n h o u r 2 - 3 tim e s a d a y . T h e c h i l d ’s b e d n e e d s to be tip p e d a n d / o r a f o a m w e d g e m a y be u se d . P e r c u s s io n a n d v ib r a ti o n m u s t be p e r fo r m e d b y th e m o t h e r o r s o m e o t h e r m e m b e r o f th e fa m ily . W e fin d th e u s e o f m e c h a n ic a l v i b r a to r s m o r e a c c e p ta b le to b o t h m o th e r a n d c h ild . C o u g h in g is d o n e w h e n s e c re tio n s r e a c h th e la r g e r a irw a y s . A s p a r t o f th e d a ily re g im e n s o m e f o r m o f i n h a la tio n t h e r a p y is in c lu d e d . 1. M u c o ly ti c s a) M is ta b ro n 1:1. N o t g en erally liked b u t th e m o s t e ffe c tiv e m u c o ly tic , b) B i s o l v o n 1:4. V e r y u s e f u l in p a ti e n ts w h o h a v e sin u s itis . 2. B r o n c h o d ila to r s a) V e n to lin '/2 1:1 b) I p r a d o l 1:4 U sed f o r th e c h ild r e n w ith b r o n c h o s p a s m . 3. A f te r p o s t u r a l d r a in a g e n e b u liz e d a n ti b io ti c s m a y be used. A t p r e s e n t w e a re u s in g N e tilm y c in 75 m g b d . T h is m a y b e c o m b in e d w ith C a r b e n ic illi n in 1 g b d . H u d s o n d i s p o s a b le n e b u liz e rs a re g e n e ra lly u se d . A v a rie ty o f c o m p r e s s o rs a re a v a ila b le c o m m e rc ia lly . P S Y C H O S O C I A L I M P L I C A T I O N S C F is a c h r o n ic , f a ta l d is e a s e . T h e r e is n o c u re a l t h o u g h life e x p e c ta n c y h a s in c r e a s e d a s a re s u lt o f im p ro v e d tr e a tm e n t. S o th e p r o b le m h a s b e e n e x te n d e d . E a c h in d iv i d u a l p a ti e n t h a s to c o p e w ith his illn e ss fo r a m u c h lo n g e r p e r io d . 5 W e n o w h a v e a d o le s c e n t s a n d y o u n g a d u lts a tt e n d in g C F clin ics. E v e n in m ild e r c a s e s t h e r e a r e h o s p i ta l a d m is s io n s f o r a c u t e e x a c e r b a tio n s . T h e r e is a n in e v ita b le s tre s s o n th e c h ild a n d h is fa m ily r e s u ltin g in a v a r y in g d e g re e o f p s y c h o s o c ia l d y s f u n c tio n . A n u m b e r o f s t u d ie s h a v e d e a l t w ith th e i m p a c t o f h o m e t r e a t m e n t o n th e c h ild a n d his fa m ily . B u r t o n 5 f o u n d t h a t o f t h e w h o le t r e a t m e n t r e g im e n f r o m r e p la c e m e n t e n z y m e s to p h y s io th e r a p y , it w a s th e p h y ­ s i o t h e r a p y t h a t o c c a s io n e d th e g r e a te s t p r o t e s t f r o m th e c h il d r e n a n d th e g r e a te s t d is lik e o f a d m in i s te r i n g it by th e p a r e n ts . M i k k e ls o n e t a l.6 f o u n d t h a t th e fa c t o f h a v in g to tr e a t th e c h ild d a ily w as a c o n s t a n t r e m in d e r o f th e p r e s e n c e o f th e d is e a s e a n d its f a ta l o u tc o m e . M o s t p a r e n t s w e re r e lu c ta n t to a d m i t t h a t th e y f o u n d th e t r e a tm e n t tim e - c o n s u m i n g 7 a n d s o m e m a d e a c o n s c io u s e f f o r t to k e e p so b u s y w ith th e t r e a tm e n t to e n a b le th e m to d e n y n e g a tiv e th o u g h ts . M o s t i m p o r t a n t, D e W e t7 f o u n d t h a t th e p a r e n ts te n d e d to o v e r e s tim a te th e e ffe c ts o f t h e i r h o m e t r e a t ­ m e n t in b o t h a n e g a tiv e a n d a p o s itiv e w a y . “ W h e n th e p a ti e n t w as h e a lth y it w as se e n a s a re s u lt o f p e rs e v e rin g w ith t r e a tm e n t. A l te r n a tiv e ly w h e n th e p a ti e n t w a s ill th is w a s se e n as a f a ilu r e o n th e i r p a r t. D e t e r i o r a t i o n w a s s e l d o m a s c r i b e d t o th e n o r m a l c o u r s e o f th e illn e s s ”. R e c e n t s tu d ie s s h o w t h a t o n t h e w h o le c h ild r e n a r e j c o p in g re la tiv e ly w ell w ith life a n d e m o ti o n a l d i s t u r b ­ a n c e is n o t a n in e v ita b le o u tc o m e o f th e d i s e a s e .8 B y w a te r 9 f o u n d t h a t it w a s th e m o th e r s w h o h a v e a s ig n if ic a n t te n d e n c y to w a r d s d e p r e s s io n . T h e f a th e r s se e m to e s c a p e th is p o s s ib ly b e c a u s e th e y a re e m p lo y e d o u ts id e th e h o m e a n d a re n o t c o n ti n u a ll y re m in d e d o f th e d ise a s e . W e fin d a t th e R e d C r o s s W a r M e m o r ia l C h i ld r e n ’s H o s p ita l t h a t th e c h ild r e n o f th e lo w e r s o c io - e c o n o m ic g r o u p s s h o w m in im a l c o m p lia n c e w ith a p h y s io th e r a p y re g im e n . I f th e c h ild d islik e s it, t r e a tm e n t is s t o p p e d . A ls o , u n d e r s t a n d i n g o f th e im p l ic a t io n s o f th e d ise a s e is s lig h t a n d few m o th e r s se e m s u ffic ie n tly c o n c e r n e d to le a r n m o r e a b o u t th e c o n d itio n . S T U D I E S O N C H E S T P H Y S I O T H E R A P Y C h e s t p h y s i o th e r a p y is a n a c c e p te d p a r t o f th e t r e a t ­ m e n t r e g im e n f o r C F . In th e la s t 10 y e a rs th e re a l b e n e fits o f th is tim e - c o n s u m in g t r e a tm e n t h a v e b e e n th e s u b je c t o f a n u m b e r o f s tu d ie s . 1. P r y o r a n d W e b b e r 10 e v a l u a t e d th e f o r c e d e x p ir a ti o n t e c h n iq u e ( F E T ) a n d f o u n d it p r o d u c e d m o re s e c r e ^ tio n s in a s h o r t e r p e rio d o f tim e t h a n c o n v e n ti o n a l p e rc u s s io n a n d v ib ra tio n s . It w a s d o n e in c o n ju n c tio n w ith p o s t u r a l d r a in a g e , th e r e b e in g n o a t t e m p t to s e p a r a t e th e tw o te c h n iq u e s . T h e p a tie n ts p r e fe r r e d F E T a n d as it w a s p e r f o r m e d by th e m s e lv e s it m a d e th e m in d e p e n d e n t o f o u ts id e h e lp . 2. W o n g e t a l . ' 1 p r o v e d t h a t g r a v ity d o e s a ffe c t th e m u c u s t r a n s p o r t r a te in C F in th e tr a c h e a . A lt h o u g h th e y re c o m m e n d p o s t u r a l d r a in a g e th e r e is s till n o in d ic a tio n o f w h e th e r th e m u c u s t r a n s p o r t r a te is a ffe c te d in th e s m a lle r a irw a y s o r w h e t h e r th e v a rie ty o f p o s t u r a l d r a in a g e p o s i tio n s is n e c e ssa ry . 3. D e s m o n d e t a l . ' 2 p e r fo r m e d a lo n g - te r m t r ia l o n w ell C F p a ti e n ts . T h e y f o u n d t h a t r e g u la r c h e s t p h y s i o t h e r a p y g a v e l i t t l e i m m e d i a t e f u n c t i o n a l im p r o v e m e n t b u t a 3 w e e k p e r io d w i th o u t p h y s i o ­ th e r a p y led t o a w o rs e n in g o f f u n c ti o n a l s ta tu s . 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. ) T h e s e p a ti e n t s a ll h a d m ild t o m o d e r a te a irflo w o b s t r u c ti o n . T h e d e t e r i o r a t i o n w as re v e rs e d a f te r r e g u la r c h e s t p h y s io t h e r a p y w as re s u m e d . 4. A s t u d y d o n e in D e n m a r k b y A n d e r s o n e t a l. 13 c o n s id e r e d t h e e ffe c t o f p o s itiv e e x p ir a t o r y p r e s s u r e ( P E P ) o n th e a m o u n t o f s p u t u m p r o d u c e d a n d o n th e s k in o x y g e n te n s io n ( P s0 2). It w a s f o u n d t h a t th e use o f P E P w ith o r w i t h o u t p o s t u r a l d r a in a g e p r o d u c e d th e la r g e s t a m o u n ts o f s p u t u m a n d t h a t th e P , 0 2 w a s s ig n if ic a n tly in c r e a s e d b y th e u s e o f P E P w i th o u t p o s t u r a l d r a in a g e . C o n v e n ti o n a l c h e s t p h y s io th e r a p y d e c re a s e d th e P s0 2 a n d p ro d u c e d c o n ­ s id e r a b ly less s p u t u m . T r e a tm e n t tim e w as h a lv e d w h e n p o s t u r a l d r a in a g e w as e x c lu d e d . F E T w as u se d t h r o u g h o u t to h e lp r e m o v e s e c re tio n s . 5. A s tu d y b y R o s s m a n e t a l. 14 u s e d X e n o n s c a n s to d e m o n s t r a t e lu n g c le a r a n c e a n d f o u n d t h a t v ig o r o u s re g im e n te d c o u g h s e s s io n s (in s ittin g ) w e re a s e ffe c ­ tiv e f o r c le a r in g s e c re tio n s as t h e r a p is t a d m in is te r e d p h y s i o t h e r a p y . T h is is o n e o f th e fe w s t u d ie s to c o n tr o l f o r c o u g h a lo n e . T h e s e c re tio n s c le a r e d w e re p r in c ip a lly in th e la rg e a irw a y s . A n u m b e r o f s tu d ie s h a v e b e e n d o n e to in v e s tig a te th e u se o f e x e rc is e in s te a d o f o r as a n a d j u n c t to c h e s t p h y s i o th e r a p y . T h e c h il d r e n c h o s e n f o r th e s e s tu d ie s w e re n o t se v e re ly a ffe c te d a n d c o u ld c o p e w ith e x e rc is e p r o g r a m m e s . S w im m in g w a s f o u n d to b e p a r tic u l a r ly h e lp fu l. 6. Z a c h e t a l . l> f o u n d t h a t m u c u s c le a r a n c e in c re a s e d o n s w im m in g d a y s a n d t h a t th e r e w as i m p ro v e d v e n ti l a t o r y f u n c tio n . D a ily p h y s i o t h e r a p y w a s c o n ­ t in u e d . P r e v io u s ly K e e n s e t a l . '6 p r o v e d t h a t v e n tila ­ to r y m u sc le e n d u r a n c e c o u ld be im p r o v e d by sp e c ific v e n ti la t o r y m u sc le e n d u r a n c e tr a i n i n g o r u p p e r b o d y e n d u r a n c e e x e rc is e s . 7. A y e a r la t e r Z a c h e t a l . n c o m p ile d a t o ta l tr a i n i n g p r o g r a m m e o f v ig o r o u s p h y s ic a l e x e rc is e a n d s p o r t. T h is la s te d f o r 17 d a y s a n d n o p h y s i o th e r a p y w as g iv e n . T h e r e w a s a m a r k e d im p r o v e m e n t in th e p e a k e x p i r a t o r y flo w r a te w h ic h w a s m a i n t a i n e d a f te r th e p r o g r a m m e h a d e n d e d . It w a s a ls o n o te d t h a t th e fr e q u e n c y o f c o u g h in g a n d v o lu m e o f e x p e c to r a te d s p u t u m re a c h e d a m a x i m u m b e tw e e n 3 - 5 d a y s a n d th e n d e c r e a s e d to b e c o m e s lig h t by th e e n d o f th e p r o g r a m m e . 8. O r e n s t e in e t a / . 18 r a n a n e x e rc is e t r ia l c o n s is tin g p r in c i p a ll y o f jo g g in g . A t th e e n d o f 3 m o n th s th e y fo u n d a sig n ific a n t im p r o v e m e n t in e x e rc is e to le ra n c e . T h e r e w a s a ls o a g r e a t e r c le a r a n c e o f s e c re tio n s . T h e y c o n s id e r e d t h a t e x e rc is e w a s as e ffe c tiv e as p h y s io th e r a p y in th e r e m o v a l o f s e c re tio n s . O re n s te in s tre sse s t h a t o n ly c h ild r e n w ith a v ita l c a p a c ity o r F E V , o f m o r e t h a n 5 0 % o f th e e x p e c te d n o r m s h o u ld d o th is ty p e o f e x e rc is e . A ls o u n til m o re tr ia ls h a v e b e e n d o n e he is w a r y o f p re s c r ib in g e x e rc is e in s te a d o f p h y s i o t h e r a p y . D I S C U S S I O N T h e s ta tic p h y s io th e r a p y re g im e n o f p o s tu r a l d r a in a g e , p e r c u s s i o n , v i b r a t i o n s a n d i n t e r m i t t e n t c o u g h in g is Physiotherapy, A u g u s t 1986, vol 42 no 3 c o m i n g u n d e r fire f r o m a ll q u a r te r s . If d o n e p r o p e r l y th is t r e a t m e n t is e x tr e m e ly tim e - c o n s u m in g a n d n o t p a r t i c u l a r l y c o m f o r ta b le . S m a ll c h ild r e n h a te to re m a in s till f o r a n y le n g th o f tim e a n d th e o l d e r o n e s b e c o m e b o r e d . T h e o n u s o f c a r r y in g o u t th e t r e a tm e n t g e n e ra lly fa lls o n th e m o th e r a n d d is p u te o v e r th e t r e a tm e n t a d d s to th e s tre s s o f h a v in g a c h ild w ith a l e th a l d o se . P h y s io t h e r a p y is a im e d a t m o b iliz in g a n d re m o v in g s e c re t io n s f r o m th e lu n g s . I t c a n n o t p r e v e n t a c u te lu n g in f e c ti o n . I t c a n n o t p r e v e n t e v e n t u a l d e t e r i o r a t i o n o f th e lu n g tis s u e . It d o e s n o t im p r o v e l u n g f u n c tio n . W h a t w e c a n d o is t o n o r m a li z e th e c h i l d ’s life as m u c h as p o s s ib le w ith in th e lim its o f t h e c o n d iti o n . F r o m v a r io u s s tu d ie s it c a n b e se e n t h a t th e r e a r e a n u m b e r o f w a y s t o m o b iliz e s e c re tio n s w h ic h t a k e less tim e a n d a r e less c o n s t r ic t in g t o th e c h ild a n d th e m o th e r . A ls o as th e c h ild g e ts o ld e r he w o u ld be a b le to p e r f o r m his t r e a tm e n t in d e p e n d e n tly . G e n e r a l e x e rc is e , s w i m m i n g , j u m p i n g , e tc . a ll m o b i l i z e s e c r e t i o n s . B re a th in g se ssio n s w ith a P E P m a s k m o b iliz e se c re tio n s . D ir e c te d c o u g h b o th m o v e s a n d r e m o v e s s e c re tio n s f r o m th e la rg e a irw a y s a n d th e tr a c h e a . T e a c h in g c o u g h in g is p e r h a p s th e m o s t i m p o r t a n t a s p e c t o f th e p h y s i o t h e r a p i s t ’s a d v ic e to p a r e n ts . T h is is f r e q u e n tly n e g le c te d , f a r m o r e tim e b e in g s p e n t o n p o s t u r a l d r a in a g e a n d p e r c u s s io n . It is p o s s ib le to te a c h a c h ild o f tw o y e a r s to c o u g h a n d e x p e c to r a t e . T h e s o o n e r th e c h ild c a n d o th is t h e b e tte r . I t o b v ia t e s th e t r a u m a o f b e in g s u c tio n e d f o r r e g u la r s p u tu m s p e c im e n s a n d e n s u re s t h a t th e c h ild w ill c o u g h e ffe c tiv e ly . I f p a r e n t s a re n o n - c o m p li a n t w ith t r e a tm e n t th e p h y ­ s i o t h e r a p i s t s h o u ld o r g a n iz e r e g u la r a tte n d a n c e a t th e P h y s io th e r a p y D e p a r tm e n t. H e re th e c h ild c a n be ta u g h t to d o th e t r e a tm e n t h im s e lf. O b v io u s ly th e s im p le r a n d m o r e e n jo y a b l e th e p r e s c r i p t io n th e g r e a t e r th e lik e li­ h o o d th e r e w ill be o f k e e p in g th e c h e s t c le a r. It m u s t be e m p h a s iz e d t h a t th e p h y s io th e r a p y re g im e n h a s to be p la n n e d i n d iv i d u a lly f o r e a c h c h ild a n d f a m ily . T h e c h ild r e n w h o a re s e v e re ly a ffe c te d c a n n o t live s u c h a n a c tiv e life a n d w ill r e q u ir e a m o re c o n v e n ­ tio n a l ty p e o f p r o g r a m m e . T h e c h ild r e n m u s t a ls o be p r e p a r e d f o r h o s p it a l a d m is s io n s b e c a u s e a c tiv ity th e r e is u s u a lly lim ite d by d r ip s . A l th o u g h it is th e p a r e n t s ’ r e s p o n s i b ilit y to see t h a t th e c h ild c le a rs h is s e c re tio n s r e g u la r ly th e y s h o u ld a lw a y s fe el fre e to c o m e to th e P h y s io th e r a p y D e p a r t m e n t w h e n e v e r th e y n e e d a d v ic e . C O N C L U S I O N P h y s io t h e r a p y f o r m s a n e s s e n t ia l p a r t o f th e life o f a C F p a ti e n t. In o r d e r to e n s u r e as n o r m a l a c h ild h o o d a n d a d o le s c e n c e as p o s s ib le w e m u s t be m o r e d y n a m ic in o u r a p p r o a c h . W e m u s t tr y t o l ig h te n th e b u r d e n o n th e f a m ily a n d y e t be as e ffe c tiv e as p o s s ib le . R e feren ces 1. H olzel A. T h e q u e st fo r the basic d efect in C ystic Fibrosis. P h y s io th e r a p y 1975; 61(8): 238-239. 2. L ip o w H W a n d M c Q u itty JC . C y stic F ib ro s is. In: R u d o lf A M , ed. P ediatrics, N o rw a lk , C o n n e c tic u t: A p p le to n - C e n tu ry -C ro fts , 1982; 1433-1440. 75 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. ) 76 Fisioterapie, A u g u s tu s 1986, dee142 no 3 3. H ill ID a n d B ow ie M D . C y stic F ib ro s is . M o d e r n M e d i­ cine 1984; 9: 77-86. 4. H o d s o n M E a n d G askell D V . P h y s io th e ra p y . In: H o d so n M E , N o r m a n A P a n d B a tte n J C , eds. C y stic F ibrosis. L o n d o n : B ailliere T in d a ll, 1983; 219-241. 5. B u rto n L. T h e f a m i l y life o f sic k children. L o n d o n : R o u tle d g e & K eg an P a u l, 1975; 3,95. 6. M ik k e ls o n C , W a e c h te r E a n d C r itte n d e n M . C y stic F ib ro sis: a fa m ily ch allen g e. C h ild ren T o d a y 1978; 7(4): 22-26. 7. D e W et B. T h e b irth o f a child w ith a co n g e n ita l a n o m aly : so m e p sy c h o so c ia l im p lic a tio n s f o r th e fam ily . D e c e m b e r 1984. D is s e rta tio n fo r P h D a t U n iv ersity o f S te lle n b o sc h , p303. 8. D r o t a r D et al. P sy c h o s o c ia l f u n c tio n in g o f th e child w ith cystic fib ro sis. P ediatrics 1981; 67(3): 338-343. 9. B y w a ter E M . A d o le s c e n ts w ith cystic fib ro sis: p sy c h o ­ social a d ju s tm e n t. A r c h D is C h ild 1981; 56: 538-543. 10. P ry o r J A a n d W e b b e r BA. A n e v a lu a tio n o f th e fo rce d e x p ir a tio n te c h n iq u e as a n a d ju n c t to p o s tu r a l d ra in a g e . P h y sio th e r a p y 1979; 65(10): 304-307. 11. W o n g L W et al. E ffects o f g ra v ity o n tra c h e a l m ucus t r a n s p o r t ra te s in n o rm a l su b je c ts a n d in p a tie n ts w ith cystic fib ro sis. P ediatrics 1977; 60(2): 146-152. 12. D e s m o n d K J el al. I m m e d ia te a n d lo n g -te rm effects o f ch est p h y sio th e ra p y in p a tie n ts w ith c y stic fib ro sis. J P ed ia tr 1983; 103(4): 538-542. 13. A n d e rso n J B el al. Im p ro v in g th e k e tc h u p b o ttle m e th o d w ith p o sitiv e e x p ir a to ry p re ss u re , P E P . 1982-1983; stu d y d o n e a t H e rle v H o s p ita l, D e n m a rk . 14. R o s s m a n C M e t al. E ffect o f ch est p h y s io th e ra p y on th e re m o v a l o f m u c u s in p a tie n ts w ith cy stic fib ro sis. A m R e v R e sp ir D is 1982; 126(1): 131-135. 15. Z a c h s M S el al. E ffect o f sw im m in g o n fo rc e d e x p ir a tio n a n d sp u tu m c le a ra n c e in c y stic fib ro sis. L a n c e t 1981; ii: 1201-1203. 16. K eens T G et al. V e n tila to ry m u sc le e n d u ra n c e tr a in in g in n o rm a l su b je cts a n d p a tie n ts w ith cystic fib ro sis. A m R e v R e s p ir D is 1977; 116: 853-860. 17. Z a c h M S e t al. C ystic fib ro sis: p h ysical ex ercise versus ch est p h y sio th e ra p y . A r c h D is C h ild 1982; 57: 587-589. 18. O re n ste in D M e t al. E x ercise a n d c y stic fib ro sis. The P h ysicia n a n d S p o r ts M e d ic in e 1983; 11(1): 57-63. F u r th e r re a d in g 1. C e rn y F J e t al. H o s p ita l th e r a p y im p ro v e s e x ercise to le r ­ a n ce a n d lu n g fu n c tio n in c y stic fib ro sis. A m J D is C h ild 1984; 138(3): 261-265. 2. C y stic F ib ro s is F o u n d a tio n . G u id e to D ia g n o sis a n d M a n a g e m e n t o f C ystic F ib ro sis. A tla n ta , G e o rg ia , 1971. | 3. M a x w ell M . Review o f lite ra tu re o f p h y s io th e ra p y in cystic fib ro sis. P h y sio th e r a p y 1980; 66(7): 245-246. 4. M io t A. C ystic fibrosis a n d th e role o f th e p h y sio th e ra p is t. S o u t h A fr ic a n J o u r n a l o f P h y sio th e r a p y 1981; 37(3): 72-74. 5. T ra v is G. C h ro n ic illness in children. S ta n fo rd : S ta n fo rd U n iv e rsity P re ss, 1976. For O rthopaedic Appliances, S u rg ica l corsets, Cervical collars, C hildren's shoes & boots, A rtific ia l limbs, Latest in plastic m odification. For H irin g and sellin g o f H o spita l E quipm ent and Sick room requisites - Wheel chairs, w alking aids, Commodes, H o spita l beds, Traction apparatus V is it th e o rth o p a e d ic m e c h a n ic ia n s A. C. MILLER & Co. T e c h n ic ia n s r e g is te r e d w ith th e S.A. M e d ic a l a n d D e n ta l C o u n c il 275 BREE STREET JOHANNESBURG Telephone (011) 337-4763 P.O. Box 3412 Johannesburg Try New Flotation Pads to keep you free from bedsores Now “ NEW B O N M A T ” and ‘NEW PARAMAT” com e up w ith be tte r q u a lity yet lo w e r p rice s than co n ve n tio n a l flo ta tio n pads Rand Medical Supplies Randburg Centre 449, Jan Smuts Ave., Blairgowrie, Randburg 2194 Box 7166, Johannesburg. Tel. 789-2203/789-2286 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. )