CYSTIC FIBROSIS AND TH E R O L E OF T H E PHYSIOTHERAPIST A N N E M 1 0 T , B.Sc. (P h y sio ), (W itw a te rs ra n d ), J . M . P E T T I F O R , M .B . B .C h ., F .C .P . (S.A .), P h .D . (M ed), I. R E E F , M .B . B .ch., F .R .C .P .* F I S I O T E R A P I E SEPTEMBER 1981 S U M M A R Y C y stic fib r o s is is an in h e r ite d disease, the m a jo r p r o b le m s b e in g p a n c re a tic m a la b so r p tio n a n d p ro g ressive respiratory in v o lv e m e n t, a lth o u g h n u m e r o u s o th er org a n s m a y also be in v o lv e d . T h e p u lm o n a r y p a th o lo g y a n d the role o f the p h y sio th e ra p ist in th e tre a tm e n t o f cystic fib r o s s is discussed. T h e p h y s io th e r a p is t p la y s an i m ­ p o r ta n t role in th e m a n a g e m e n t o f the c h ild w ith cystic fib r o sis, n o t o n ly fr o m the p o in t o f v ie w o f m a in ta in in g m a x im a l p u lm o n a r y fu n c tio n b u t also b ecause she c o m e s in to close c o n ta c t w ith the f a m ily a n d can h e lp in o p ­ tim isin g the ac ce p ta n c e o f the ch ild in to the fa m ily situ a tio n . IN T R O D U C T IO N C y stic fib ro sis w as firs t d e sc rib e d as a c lin ic a l e n ­ tity so m e f o r ty y e a rs a g o ; h o w e v e r, th e c h a ra c te ris tic e le c tro ly te a b n o rm a litie s in th e sw ea t, w h ic h h a v e b e ­ c o m e th e c r ite r ia f o r d iag n o sis, w ere o n ly d isc o v e re d tw e n ty y e a rs la te r. C y stic fib ro sis, a g e n e tic d ise ase i n ­ h e rite d as a n a u to s o m a l re ce ssiv e , is c h a ra c te ris e d by a g e n e ra lis e d d y s fu n c tio n o f e x o c rin e g la n d s . T h is leads to th r e e m a jo r f e a tu re s ; p a n c re a tic in su ffic ie n c y a n d in te s tin a l m a la b s o rp tio n , c h ro n ic p u lm o n a ry d ise ase, a n d e le v a te d c o n c e n tra tio n s o f so d iu m a n d c h lo rid e in th e sw ea t. M a n y o th e r o rg a n s, su c h as th e liv e r a n d m a le re p ro d u c tiv e sy ste m , a re in v o lv e d as w ell, b u t th e ir in v o lv e m e n t is u su a lly o v e rsh a d o w e d b y th e p ro b le m s p ro d u c e d b y th e p a n c r e a tic a n d r e s p ira to ry disease. O v e r th e p a s t tw o d e c a d e s, s ig n ific a n t s trid e s h a v e b e en m a d e in th e tr e a tm e n t o f th e c y stic fib ro sis, so t h a t n o w it is th e e x c e p tio n r a th e r th a n th e ru le th a t s u ffe re rs d o n o t re a c h a d o le sc e n c e . D e s p ite th e s e a d ­ v a n c e s in tr e a tm e n t, th e b a s ic b io c h e m ic a l d e fe c t o f th is d ise ase, w h ic h a ffe c ts so m a n y o rg a n s, is still u n ­ k n o w n . A lth o u g h p a n c r e a tic in su ffic ie n c y w ith its c o n - c o m m ita n t m a la b s o r p tio n a n d f a ilu re to th riv e , is o fte n th e firs t c lin ic a l c lu e t h a t th e p a tie n t is s u ffe rin g fro m c y stic fib ro sis, it is u s u a lly th e p ro g re s siv e p u lm o n a ry in v o lv e m e n t w ith e v e n tu a l r e s p ira to ry fa ilu r e t h a t lea d s to t h e e a rly m o rta lity in th e dise ase. I t is f o r th is r e a ­ so n th a t th e c o n tro l o f th e p u lm o n a ry c o m p lic a tio n s h a s p la y e d su c h a n im p o r ta n t ro le in th e im p ro v e m e n t in p ro g n o sis t h a t h a s o c c u rre d o v e r th e p a s t tw e n ty y e ars. T H E P A T H O L O G Y O F T H E R E S P IR A T O R Y T R A C T I N C Y ST IC F IB R O S IS T h e m a jo r a re a o f in v o lv e m e n t in c y stic fib ro s is is in itia lly th e sm a ll a irw ay s. A t b i r th th e lu n g s a re h isto lo g ic a lly n o rm a l. H o w e v e r th e p a ti e n t is p ro n e to in fe c tio n . I n f e c tio n le a d s to h y p e r tr o p h y o f th e m u c o u s su b -m u c o sa l g la n d s a n d a n in c re a s e in v isc o sity a n d p r o ­ d u c tio n o f th e r e s p ira to ry m u cu s. T h e in fe c tio n o f c ystic * C y stic F ib ro s is C lin ic , D e p a r tm e n t o f P a e d ia tric s , J o h a n n e s b u r g H o s p ita l, P r iv a te B ag X 3 9 , J o h a n n e s ­ b u rg , S o u th A fric a . R e c e iv e d 1 M a y 1981. O P S O M M IN G S istiese fib r o s e is 'n o o r e r flik e sie k te , w aarvan die h o o fp r o b le m e p a n k re a sm a la b so r p sie en progressiew e re spiratoriese v ersa kin g is, h o e w e l verske ie a n d e r organe o o k b e tr o k k e k a n w ees. D ie lo n g p a to lo g ie en die rol v an die fis io te r a p e u t in sistiese fib r o s e w o rd b espreek. D ie fis io te r a p e u t sp e e l ’n b e la n g rik e rol in die h a n tering v an die k in d m e t sistiese fib r o se , nie slegs v an die oog- p u n t o m m a k s im a le lo n g fu n k s ie te b e h o u nie, m a a t^ o o k d a a r sy in n o u e k o n ta k m e t die fa m ilie k o m en kai^ h elp o m die a a n v a a rd in g van die k in d in d ie fa m ilie - situasie te b e vo rd er. fib ro sis is a b ro n c h io litis , a ffe c tin g p re d o m in a n tly th e sm a ll b ro n c h i a n d b ro n c h io li. T h is le a d s to u lc e ra tio n a n d s c a rrin g o f th e sm a ll a irw a y s w ith b ro n c h io la r s te n o sis a n d o b lite ra tio n . E x c e ss m u c u s p r o d u c tio n lea d s to o b s tru c tio n w ith a s s o c ia te d a ir tr a p p in g o r c o lla p se o f alv e o li, T h e in fe c tio n g ra d u a lly sp re a d s to in v o lv e th e s u rro u n d in g p a re n c h y m a a n d th e la rg e r b ro n c h i. T h e ty p ic a l p a tte r n o f c h ro n ic b ro n c h itis w ith o b s tru c tio n o f th e la rg e a n d s m a lle r a irw ay s, h y p e r­ in f la tio n o f th e lu n g s a n d b r o n c h ie c ta s is is th e n seen. T h e b ro n c h ie c ta s is c la ssic a lly in v o lv e s a ll se g m e n ts of th e lu n g . C o n c o m m ita n t w ith th e s e c h a n g e s is th e d e v e lo p m e n t o f p u lm o n a ry h y p e rte n s io n le a d in g to rig h t v e n tr ic u la r h y p e r tr o p h y a n d c o r p u lm o n a le . A f u r th e r a s p e c t o f th e p u lm o n a ry p a th o lo g y w h ic h h a s n o t b e e n a d e q u a te ly e x p la in e d is th e in c re a s e d in c id e n c e o f a s th m a -lik e b ro n c h o s p a s m re sp o n siv e to b ro n c h o d ila to r o r s te ro id th e ra p y . T h u s c lin ic a lly in th e e a rly sta g e s o f th e d ise ase, th e o n ly sy m p to m m a y b e a d ry r e p e titiv e c o u g h w h ic h o f te n pa sse s u n n o tic e d by th e m o th e r o r g e n e ra l prac-< titio n e r. T h is m a y p ro g re s s to a p ic tu r e s im ila r t/ b ro n c h io litis in th e in fa n t, w ith ta c h y p n o e a , h y p e r ­ in f la tio n a n d e x p ira to ry r h o n c h i. T h e c o u g h m a y b e ­ c o m e p a ro x y sm a l a n d in th e y o u n g in f a n t is o fte n a s s o c ia te d w ith v o m itin g . R e c u r r e n t e p is o d e s o f p a tc h y b r o n c h o p n e u m o n ia a lso o c c u r, th e s e o f te n b e in g a sso ­ c ia te d w ith S ta p h y lo c o c c u s a u re u s in fec tio n s. L a te r in th e d ise a s e th e c o u g h is u s u a lly p ro d u c tiv e , b u t th e a m o u n t o f s p u tu m v a rie s c o n s id e ra b ly d e p e n d in g on th e se v e rity o f th e b ro n c h ie c ta s is a n d th e p re se n c e o r a b ­ se n c e o f a c u te in fe c tio n . C lu b b in g o f th e fin g e rs is o f te n se e n in y o u n g c h ild re n s u ffe rin g f r o m c ystic fib ro sis, m o re p a r tic u la r ly in th o s e w ith m o re sev ere lu n g in v o lv e m e n t. A s th e d ise a s e p ro g re s se s, th e ch ild b e c o m e s p ro g re s siv e ly m o re d isa b le d , w ith c e n tra l c y a ­ n o sis a p p e a rin g a n d b e c o m in g p e rs is te n t. R ig h t-s id e d c a rd ia c f a ilu re m a y o c c u r, a n d e p is o d e s o f h a e m o p ty sis b e c o m e m o re fre q u e n t. P n e u m o th o r a x h a s b e e n r e ­ p o r te d to o c c u r p a rtic u la rly in th e o ld e r p a tie n t. A s th e p a tie n t b e c o m e s o ld e r, th e in c id e n c e o f c o lo n iz a tio n o f th e re s p ir a to r y t r a c t w ith P s e u d o m o n a s a e ruginosa b e c o m e s m o re c o m m o n . O n c e e s ta b lis h e d , e ra d ic a tio n o f th e o rg a n is m is a lm o s t im p o s sib le d e s p ite v ig o ro u s tre a tm e n t w ith a p p r o p ria te in tra v e n o u s a n tib io tic s . T h e ro le t h a t th is c o lo n is a tio n p la y s in th e p ro g re s siv e d e ­ te r io r a tio n of r e s p ira to ry f u n c tio n is u n c le a r, as a re th e 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 73 re a so n s w hy c o lo n iz a tio n sh o u ld o c c u r in s e m e p a tie n ts w ith c y stic fib ro sis a n d n o t in o th e rs . If tre a tm e n t is to be o f a n y b e n e fit, it is o b ­ v ious t h a t th e ra p y s h o u ld be a im e d a t p re v e n tin g th e p ro g re s siv e s tr u c tu r a l d a m a g e th a t o c c u rs d u e to re ­ c u rre n t in fe c tio n s a n d th e in c re a s e d s e c re tio n o f viscid m u cu s. W h y th e p a tie n t w ith c y stic fib ro sis s h o u ld b e p ro n e to r e s p ir a to r y in fe c tio n e a rly o n in th e d ise a s e is u n c le a r; h o w e v e r th e r e a re tw o k n o w n d e fe c ts in re s p i­ ra to ry fu n c tio n . F irs tly , c ilial a c tiv ity o f th e r e s p ira to ry m u c o u s m e m b ra n e s is a b n o rm a l. I t is t h o u g h t t h a t n o r ­ m al c ilial a c tio n is a n im p o r ta n t m e a n s o f c le a rin g b a c te ria a n d d e b ris fro m th e s m a lle r a irw ay s. T h u s in t h e p a tie n t w ith c y stic fib ro sis th is m e a n s o f b r o n ­ chial to ile t is im p a ire d . S e c o n d ly , th e in c re a s e d s e c re ­ tio n o f v iscid m u c u s te n d s to o b s tru c t a irw a y s a n d p ro v id e s a g o o d n a id u s f o r b a c te ria l p ro life ra tio n . T h u s th e ra p y s h o u ld b e a im e d a t tw o m a in a r e a s : • th e p r e v e n tio n o f o r v ig o ro u s tr e a tm e n t o f r e s p ira to ry in fe c tio n s a n d th e a d e q u a te c le a ra n c e o f th e v isc id m u cu s. T H E R O L E O F T H E P H Y S IO T H E R A P IS T I N T H E T R E A T M E N T O F C Y ST IC F IB R O SIS T h e p h y s io th e ra p y tre a tm e n t o f a c h ild w ith cystic fib ro sis is a re la tiv e ly r o u tin e m a tt e r a n d w ill o n ly be d iscussed b rie fly . H o w e v e r, o u r p a tie n t is n o t sim p ly a c h ild w h o se lu n g s p ro d u c e e xcessive q u a n titie s o f th ic k a n d te n a c io u s se c re tio n s . H e is a c h ild d isa b le d , to a g re a te r o r a lesse r d e g ree , by a d ise a s e fo r w hich th e re is. a t th e m o m e n t, n o k n o w n c u re . I n a d d itio n , c y stic fib ro sis is a fa m ily d ise a s e a n d it w ill a ffect e very m e m b e r o f th e fa m ily to so m e e x te n t. T o f u r th e r c o m p lic a te th e p ic tu re , it is a g e n e tic a lly in h e rite d d isease. C o n s e q u e n tly , w h ile b e in g p rim a rily c o n c e rn e d w ith c le a rin g th e a irw a y s o f o u r p a tie n t, w e s h o u ld b e a w are th a t o u r ro le as p h y s io th e ra p is ts m u s t go m u c h f u rth e r. W e a re h a n d lin g a d isa b le d c h ild , a n d a p o ­ te n tia lly d is a b le d fa m ily , a n d w e s h o u ld a im , in o u r tre a tm e n t, to a c h ie v e th e h ig h e s t p o ss ib le q u a lity o f life, b o th fo r th e c h ild , a n d f o r his fa m ily . I t is o u r p u rp o s e to s h a re so m e o f th e th o u g h ts w e h ave o n s e c u rin g as b rig h t a f u t u r e as p o ss ib le f o r th is u n it o f p e o p le . O u r ro le s ta r ts w hen th e d ia g n o s is is m a d e . T h e d o c ­ to rs w ill a lr e a d y h a v e g iv en th e d ia g n o s is to th e p a re n ts, a n d d isc u sse d th e p ro g n o sis w ith th e m . H o w ­ e v e r , m o s t p a re n ts , a t so m e sta g e, will w ish to d iscuss it f u r th e r w ith us. T h e r e a re tw o p o in ts to re m e m b e r w hen re p ly in g to th e q u e s tio n s th a t o n e w ill be asked. O ne is t h a t we a re n o w a w a re t h a t c y stic fib ro sis c a n be p re s e n t in a v e ry m ild fo rm , a n d t h e o th e r is th a t th e in itia tio n o f e a rly tre a tm e n t, c o u p le d w ith th e a d ­ v a n ce s in th e ra p y , h a v e im p ro v e d th e p ro g n o sis e n o rm ­ ou sly (see p a th o lo g y ). W e m u s t th e r e f o r e re m e m b e r th a t th e d ise a s e h a s a v e ry v a ria b le , a lth o u g h in e v ita b ly d o w n w a rd c o u rse , so t h a t th e p ro g n o sis m u s t b e given c a u tio u sly , a s th e d ia g n o s is is n o t sy n o n y m o u s w ith d e a th in c h ild h o o d . O u r in itia l re s p o n s ib ility is to in s tru c t th e p a r e n ts in th e n a tu r e o f th e d ise a s e as it a ffe c ts th e lu n g s, a n d to e n su re t h a t th e y u n d e r s ta n d v e ry c le a rly th a t, in th e th e e v e n t o f p u lm o n a r y in v o lv e m e n t, th e ir c h ild ’s p ro g ­ n osis d e p e n d s v e ry la rg e ly on th e e ffe c tiv e e lim in a tio n of se c re tio n s a n d th e c o n tro l o f in fe c tio n . T h is in f o r ­ m a tio n m u s t b e g iv en se n sitiv e ly , so t h a t th e p a re n ts a re n o t left w ith th e fe elin g t h a t th ey th e m se lv e s will be so lely re s p o n s ib le f o r a n y a c u te e x a c e rb a tio n o f th e ir c h ild ’s p u lm o n a ry p ro b le m . T o q u o te L in d y B u rto n (1974) — “ L ittle is w o rse f o r p a re n ts th a n le a rn in g t h a t th e ir c h ild h a s a life- th re a te n in g illness. T h e d ia g n o s is its e lf im p lie s p a in a n d h e lp le ssn e ss, a n d se e m s to p re c lu d e a ll jo y fu l e x p e c ta ­ tio n s f o r a n o rm a l sa tis fy in g life to g e th e r. A s su c h , it is r e s p o n d e d to, n o t ju s t as a th r e a t f o r th e fu tu re , b u t as a re al a n d a c tu a l loss b e g in n in g a t th e m o m e n t th e n e w s is b ro k e n .” P e o p le will re s p o n d to th is d ia g n o s is d iffe re n tly , d e ­ p e n d in g o n a v a rie ty o f b a c k g ro u n d f a c to rs . D e n ia l of e ith e r th e d ia g n o s is o r th e p ro g n o sis is n o t u n c o m m o n , p a rtic u la rly by th e fa th e rs . H o w e v e r, th is d e n ia l c a n in fa c t, c u sh io n th e p a r e n ts a g a in s t re a lity , th u s m a k in g a n in to le ra b le s itu a tio n to le r a b le fo r th e m o m e n t. I t h a s b e e n fo u n d th a t th is in itia l d e n ia l is re d u c e d w h e re th e p a re n ts a re in tim a te ly in v o lv e d w ith th e c a re o f th e ir a fflic te d c h ild . T h e k n o w le d g e t h a t th e m o th e r c an , a n d in d e e d m u st, do a tre m e n d o u s a m o u n t to h e lp h e r ch ild a c h ie v e th e b e s t p o ssib le q u a lity o f life se e m s to go a long w ay in a llo w in g h e r to a c c e p t the d iag n o sis. I t o ffe rs a c h a lle n g e , a n d c a n b e c o m e a n o th e r d e v ic e f o r m a k in g th e in to le ra b le to le ra b le , a n d fo r d im in ish in g th e fe e lin g s o f g u ilt a n d lo w e red se lf­ e ste e m t h a t so m e p e o p le sh o w w h e n to ld th a t th e ir c h ild h a s a g e n e tic a lly tr a n s m itte d disease. T h e s e a b o v e fa c ts c o lo u r o u r d isc u ssio n s w ith th e p a re n ts . T h e m o th e r is, fo r o b v io u s re a so n s , m o re in ­ v o lv ed w ith th e tre a tm e n t, b u t we m u s t m a k e e v ery e ffo rt to e n s u re th e in v o lv e m e n t, n o t o n ly o f th e fa th e r, b u t in d e e d th e w h o le fam ily. H a v in g e x p la in e d w hy th e t r e a tm e n t is e sse n tia l, a n d h a v in g s tr u c tu r e d th e e x p la n a tio n to s u it th e in ­ d iv id u a l, w e stre ss t h a t th e h o m e p ro g ra m m e s h o u ld a c h ie v e th e m a x im u m in th e s h o rte s t p o ssib le tim e , s h o u ld be c o m fo rta b le a n d as m u c h fu n as th e fa m ily can m a k e it. L o n g , d istre s sin g a n d b o rin g tr e a tm e n t se ssio n s o v e r th e y e ars w ill c a u s e a n in itia l r e s e n tm e n t a n d a n e v e n tu a l re b e llio n in th e c h ild . T h is w e m u s t try to a v o id . W e te a c h th e p o sitio n s f o r p o s tu ra l d ra in a g e , th e te c h n iq u e s o f p re c u ss io n , v ib r a tio n a n d a ssiste d c o u g h ­ ing a n d th e use o f th e n e b u lis e r o f c h o ic e . F o r th e re a s o n s d e sc rib e d a b o v e , we d o n o t h a n d o u t lists o f sp e c ific a c tiv itie s. W e p r e fe r to e n c o u ra g e th e m o th e r to d e v e lo p h e r ow n in d iv id u a l h o m e p ro g ra m m e w ith in th e b r o a d fra m e w o rk o f w h a t is n e c e ssa ry . W e h a v e fo u n d t h a t so m e m o th e rs fe el a te rrib le g u ilt a b o u t tr a n s m ittin g a n illn e ss to th e ir c h ild re n , w hile o th e rs feel a d re a d fu l d e s p a ir w h e n th e y look in to t h e fu tu re . T h e s e fe elin g s w ill m a n ife s t th e m se lv e s in v a rio u s w ays. T h e s e w e m u s t re c o g n ise fo r w h a t th e y a re — in d iv id u a l w ays o f c o p in g w ith th e i r p r o b ­ lem s. O n e m ig h t fin d a g g re ssio n , a n d th is o n e sh o u ld ig n o re ; o r o n e m ig h t fin d a n o v e rz e a lo u sn e ss , a n d this o n e sh o u ld try to d isc o u ra g e . W e h a v e se e n so m e m o th e rs b e c o m e so o b se ssed w ith th e c h ild ’s tre a tm e n t, p a r tic u la r ly th e p h y s io th e ra p y sid e o f it, th a t th e y allo w th e c h ild v e ry little o p p o rtu n ity f o r a n y o th e r a c tiv ity d u rin g th e d a y . O th e rs d e v e lo p fix a tio n s a b o u t se c u rin g a p ro d u c tiv e c o u g h , a n d su b je c t t h e ir c h ild re n to th e m o s t e x tr a o r d in a r y in d ig n itie s in o r d e r to a c h ie v e th e ir o b je c tiv e . O n e n e e d s to e x p la in t h a t a ll “ c y stic s” a re n o t h a rb o u rin g b u c k e ts-fu ll o f se c re tio n s in th e ir lu n g s a ll th e tim e , a n d t h a t w h ile k e e p in g th e lungs c le a r is im p o rta n t, h e lp in g th e c h ild to le a d as n o r m a l a n d as h a p p y a life as p o ssib le , is o f even g re a te r im p o rta n c e . H a l le r (1970) h a s c o m m e n te d — “ I f the ch ild is p e rm itte d to fo c u s o n his d ise ase, o r his d e ­ fo rm ity , so t h a t th e im a g e d o m in a te s h im , h e will r e ­ m a in c h ro n ic a lly ill d e s p ite e x c e lle n t c lin ic a l c are . I f a c h ild c an be c o n v in c e d t h a t h is d is a b ility is o n ly re la tiv e , h a lf o f th e th e r a p e u tic b a ttle is w o n .” W e s h o u ld give g u id e lin e s o n th e fre q u e n c y o f h o m e tre a tm e n t. W e a d v ise th a t, if th e ch ild is p r o ­ d u c tiv e , th e p ro g ra m m e s h o u ld b e c a rrie d o u t tw ice a d a y , fir s t th in g in th e m o rn in g a n d la s t th in g a t n ig h 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. ) 74 F I S I O T E R A P I E SEPTEMBER 1981 if p o ssib le , n o t im m e d ia te ly a f te r a m e a l; t h a t th e f r e ­ q u e n c y sh o u ld b e ste p p e d u p if th e se c re tio n s in c re a s e , a n d th a t o n e tr e a tm e n t p e r d a y is e n o u g h if th e c h ild ’s c o u g h is u n p ro d u c tiv e . In th e case o f a y o u n g ch ild w ho o n ly show s signs o f m ild p u lm o n a ry in v o lv e m e n t, w e a d v ise th a t a s h o r t p ro g ra m m e o f p o s tu ra l d r a in ­ a ge a n d “ b r e a th in g e x e rc is e s” s h o u ld b e c a rrie d o u t o n c e a d a y . T h is is to a c c u s to m th e c h ild to th e tr e a t ­ m e n t so th a t it b e c o m e s sim p ly a p a r t 'o t h is d a ily life. H o p e fu lly , th is a p p r o a c h will m a k e it e a s ie r f o r th e c h ild to a c c e p t th e tr e a tm e n t w h e n it re a lly b e c o m e s n e ce ssa ry . W e stre ss th e i m p o r ta n c e o f p h y sic a l a c tiv ity — a n y a c tiv ity a p p r o p ria te to th e a g e o f th e c h ild . T h is is b o th f o r th e p u rp o s e o f m o b ilisin g se c re tio n s a n d p ro d u c in g a c o u g h , a n d f o r t h e se n se o f w e ll-b e in g t h a t a n y su c h a c tiv ity a lm o st in e v ita b ly b rin g s, p ro v id e d o f c o u rse th a t it is d o n e w ith in th e lim its o f th e c h ild ’s c a p a c ity . W ith o u t h a v in g d o n e a n y c o n tro lle d s tu d ie s o n th is s u b je c t, it is d iffic u lt to say w h e th e r a “ well c y stic ” jo g s b e c a u se h e is w ell, o r w h e th e r jo g g in g m a k e s a “ c y stic ” well. A n o th e r o f o u r re s p o n s ib ilitie s is to in s tru c t th e m o th e rs in in fe c tio n c o n tro l, te a c h in g th e m h o w to c le a n th e e q u ip m e n t u se d so as to m in im is e th e d a n g e r o f re in fe c tio n . W ith m o re a n d m o re “ cy stic s” su rv iv in g in to th e ir te e n a g e y e ars it is b e c o m in g o f p a r a m o u n t im p o rta n c e to in v e s tig a te , a n d te a c h w ays o f h e lp in g th e te e n a g e r to c le a r h is o w n lu n g s, th u s s e c u rin g as f a r as p o s ­ sib le th e in d e p e n d e n c e so n e c e ssa ry f o r th e tr a n s i­ tio n fro m a d o le s c e n c e to a d u lth o o d . T h e s e m e th o d s in ­ c lu d e s e lf-n e b u lis a tio n , s e lf-p o s tu ra l d ra in a g e u sin g th e fo rc e d e x p ira tio n te c h n iq u e , p o ss ib ly m e c h a n ic a l p e r ­ c u ssio n s, a n d se lf-a ss iste d c o u g h in g , s u c h as c o u g h in g a g a in s t a c lo sed g lo ttis. I t h a s b e e n su g g e ste d t h a t v ig o ro u s a n d s e lf-d ire c te d c o u g h in g m a y b e a ll t h a t is r e q u ir e d fo r e ffe c tiv e b ro n c h ia l to ile t. C o n ta c t w ith t h e c h ild o n a d m iss io n to h o s p ita l fo r tr e a tm e n t o f a n a c u te in fe c tio n is o f te n a tim e w hen th e ro le o f p h y s io th e ra p y c a n be r e in fo rc e d . H e r e a g a in , th e a c tu a l p h y s io th e ra p y tr e a tm e n t is sim p le , b u t it is c o m p lic a te d b y th e fa c t th a t th e c h ild h a s, in a sense, b e c o m e a p ro d u c t o f h is d ise a s e in te rm s o f h is b e ­ h a v io u ra l re s p o n s e s to it. N o c h ild c a n a c tu a lly e n jo y b e in g h o o k e d u p to a n in tra v e n o u s d rip , b e in g tip p e d , p e rc u ss e d a n d m a d e to c o u g h v ig o ro u sly , a n d th e “ c y stic ” c h ild re n a re n o e x c e p tio n . A s w ith a n y c h ild w h o h a s a c h ro n ic d e b ilita tin g a n d so c ially u n a c c e p t­ a b le illness, re s e n tm e n t a n d la c k o f c o -o p e ra tio n m a y o fte n b e p re se n t. I h e se b e h a v io u r p a tte rn s s h o u ld be re c o g n iz e d fo r w h a t th ey a re, a n d we s h o u ld try, if n e c e s­ sa ry w ith th e h e lp of th e p s y c h ia tris t a tta c h e d to the te a m , to se c u re th e c o -o p e ra tio n o f th e c h ild . A ll o f this m e a n s th a t we a re n o t d e a lin g w ith a c h ild w h o sim p iy re q u ire s “ c h e s t p h y s io th e ra p y ” . W e h a v e fo u n d t h a t o n e m u s t se t a c h ie v a b le goals, d e p e n d in g o n th e c h ild ’s c o n d itio n a t t h a t m o m e n t, a n d th e n v ery firm ly a n d g e n tly , in sist o n h is c o -o p e ra tio n . I t is a lso v ery n e c e s­ sa ry to a llo w th e m o th e r to p a r tic ip a te in th e c a re of h e r c h ild , w h ile still e n su rin g t h a t she h a s th e re st th a t sh e n e e d s as w e ta k e o v e r th e re s p o n s ib ility f o r c le a rin g his c h est. F in a lly , in th e te rm in a l sta g e s of th e dise ase, th e p h y s io th e r a p is t’s r o le sh o u ld b e c o m e m o re su p p o rtiv e . P h y s io th e ra p y s h o u ld n o t b e w ith h e ld b u t s h o u ld b e te m p e re d w ith th e u n d e rs ta n d in g th a t o n e ’s ro le no w is to t r y a n d a c h ie v e m a x im u m c o m fo rt fo r th e d y ing c h ild . l I n th is p a p e r it is o b v io u sly im p o s sib le to go into" th e m e th o d s o f p h y s io th e ra p y e m p lo y e d in e a c h o f th e a g e g ro u p s o f c h ild re n s u ffe rin g c y stic fib ro sis. H o w ­ e v er, it is a p p a r e n t t h a t p h y s io th e ra p y a n d th e p h y s io ­ th e r a p is t play c e n tra l ro les in th e h a n d lin g o f c h ild re n w ith c ystic fib ro sis. I t is o u r h o p e t h a t th is a rtic le h a s h e lp e d in d e fin in g th e e x te n d e d ro le s o f t h e p h y ­ s io th e ra p is t a n d in o u tlin in g th e p a th o p h y s io lo g y of th e lu n g d ise ase a sso c ia te d w ith c y stic fib ro sis. R e fe re n c e s B u rto n L. (1 9 ? 4 ). T o le ra tin g th e in to le ra b le — th e p ro b le m s fa c in g p a r e n ts a n d c h ild re n fo llo w in g d ia g ­ n o sis in: C a re o f th e ch ild fa c in g d e a th . R o u tle d g e a n d K e g a n P a u l. L o n d o n . P p 16 - 34. H a lle r, J. A. (1970). A h e a lth y a ttitu d e to w a rd s c h ro n ic illn e ss — q u o te d by B u rto n L. (1974). T o le ra tin g th e in to le ra b le — th e p ro b le m s fa c in g p a r e n ts a n d c h ild re n fo llo w in g d ia g n o s is in: C a re o f th e child fa c in g d e a th . R o u tle d g e a n d K e g a n P a u l. L o n d o n . P p 1 6 - 3 4 . R e id L . (1980). C a r d io p u lm o n a ry P a th o lo g y , in: P e r ­ sp e c tiv e s in C y stic F ib ro s is — P ro c e e d in g s o f th e 8 th I n t e r n a ti o n a l C y stic F ib ro s is C o n g re ss. E d . J. M . S turgess. C a n a d ia n C y stic F ib ro s is F o u n d a tio n . T o ro n to . P p . 1 9 8 - 2 1 4 . T R E A T M E N T N O T E : A CASE O F INTRACTABLE PRURITIS VULVAE T R EA TM EN T WITH PULSED ULTRASOUND C. A . L IG G 1 N S , M .C .S .P ., H .T ., D ip .T .P .* A 6 7 -y e a r-o ld m a rrie d A fric a n w o m a n w as a d m itte d to h o s p ita l o n 6 A p ril 1979 a f te r a g y n a e c o lo g ic a l in v e s­ tig a tio n . H e r m a in c o m p la in ts w ere v a g in a l p a in a n d itc h in e s s o f th e v u lv a o f 12 m o n th s d u r a tio n . P e lv ic e x a m in a tio n sh o w e d a tro p h y o f th e la b ia a n d v a g in a l m u c o s a a n d a c e rv ic a l p o ly p . A d ia g n o s is o f c h ro n ic v u lv al d y stro p h y a n d p ru ritis v u lv a e w as m a d e . W h ile a n in p a tie n t sh e w as fu lly in v e s tig a te d fo r * S e n io r L e c tu re r, H e a d o f D e p a r tm e n t o f P h y s io ­ th e ra p y , U n iv e rs ity o f D u rb a n -W e s tv ille . W h ile a n in -p a tie n t sh e w as fu lly in v e s tig a te d fo r R e c e iv e d 1 A u g u s t 1981. d ia b e te s ; g lu co se to le ra n c e test su g g e ste d d ia b e te s m elli- tu s in a m ild fo rm . T h e p o ly p w as re m o v e d u n d e r g e n e ra l a n a e s th e tic a n d a v u lv al b io p sy ta k e n ; th is p ro v e d to b e n e g a tiv e . T h e p a tie n t w as d isc h a rg e d on 10 A p ril. S he w as re -a d m itte d in M a y 1979 w ith a m a rk e d p r u r itis v u lv ae . A t th e tim e it w as c o n sid e re d th a t this c o u ld h a v e b e e n a g g ra v a te d by h e r d ia b e te s so sh e w as p u t on a d ia b e tic re d u c in g d ie t. In a d d itio n , lo ca l a p p li­ c a tio n o f o e s tro g e n to th e v u lv a w as trie d , b u t to no effect. S he w as th e n re fe rre d to th e S kin C lin ic a n d ste ro id c re a m w as p re sc rib e d . T h e re w as d im in u tio n of 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. )