SOCI ALI ZATI ON OF CHI LDREN AND ADOLESCENTS
W I TH CYSTI C FI BROSI S: SUPPORT FOR NURSI NG CARE
1Tainá Maués Pelúcio Pizzignacco2
Regina Apar ecida Gar cia de Lim a3
Pizzignacco TMP, Lim a RAG. Socializat ion of childr en and adolescent s w it h cy st ic fibr osis: suppor t for nur sing car e. Rev Lat ino- am Enfer m agem 2006 j ulho- agost o; 14( 4) : 569- 77.
The aim of t his paper is t o ident ify t he rout ine ( school, work, free t im e act ivit ies and relat ionships wit h friends and fam ily) of children and adolescent s w it h Cyst ic Fibrosis ( CF) t hrough t heir experiences and ident ify sit uat ions t hat can affect t hese r out ines. The obj ect s of analysis of t his r esear ch ar e childr en and adolescent s wit h CF who at t end a t eaching hospit al in a cit y of t he St at e of São Paulo - Brazil. I t is a qualit at ive research, w it h dat a collect ion based on open in t er v iew s an d pat ien t ch ar t s. Th e dat a br ou gh t t h e f ollow in g t h em es: m isleading knowledge about t he disease, concern wit h self- im age, search for self- care and hope of im provem ent in t he fut ur e. The r esult s ev idence t he r eper cussion of CF in t hose pat ient s’ socializat ion pr ocess, ev idencing t h e im por t an ce of h ealt h pr ofession als k n ow in g abou t t h ese dem an ds an d in cor por at in g t h em in t o t h e car e plan, w it h a v iew t o effect iv e int er v ent ions t o pr om ot e infant - j uv enile gr ow t h and dev elopm ent .
DESCRI PTORS: cy st ic fibr osis; child; adolescent ; nur sing car e; pediat r ic nur sing
EL PROCESO DE SOCI ALI ZACI ÓN DE NI ÑOS Y ADOLESCENTES
CON FI BROSI S QUÍ STI CA: APOYO PARA LA ATENCI ÓN DE ENFERMERÍ A
La finalidad de est e est udio es conocer el día a día ( escuela, t rabaj o, act ividades de ocio e int eracciones con la fam ilia y am igos) del niño y del adolescent e con Fibrosis Quíst ica ( FQ) a part ir de sus propias vivencias e ident ificar sit uaciones que puedan int er fer ir en esa cot idianidad. Los suj et os de la inv est igación son niños y adolescent es port adores de Fibrosis Quíst ica baj o seguim ient o en un hospit al- escuela del int erior del Est ado de São Paulo, Brasil, con edad ent re 7 y 18 años. Efect uam os una invest igación con aproxim ación cualit at iva. Los dat os em pír icos fuer on r ecopilados m ediant e el análisis de r egist r os m édicos y ent r ev ist as abier t as, r ev elando los siguient es t em as: conocim ient o equivocado sobre la enferm edad; preocupación con el aut oim agen; búsqueda p or au t ocu id ad o y esp er an za d e m ej or as en el f u t u r o. Los r esu lt ad os ev id en cian las r ep er cu sion es d e la Fibrosis Quíst ica en el proceso de socialización de esos pacient es, es im port ant e que los profesionales de salud con ozcan esas d em an d as y las in cor p or en al p lan d e cu id ad os, con v ist as a in t er v en cion es ef ect iv as q u e pr om uev an el cr ecim ient o y desar r ollo infant o- j uv enil.
DESCRI PTORES: fibr osis quíst ica; niño; adolescent e; at ención de enfer m er ía; enfer m er ía pediát r ica
O PROCESSO DE SOCI ALI ZAÇÃO DE CRI ANÇAS E ADOLESCENTES
COM FI BROSE CÍ STI CA: SUBSÍ DI OS PARA O CUI DADO DE ENFERMAGEM
O p r esen t e est u d o t em p or ob j et iv o con h ecer o d ia- a- d ia ( escola, t r ab alh o, at iv id ad es d e lazer e in t er ações com a fam ília e am igos) da cr ian ça e do adolescen t e com Fibr ose Císt ica ( FC) , a par t ir de su as próprias vivências, bem com o ident ificar sit uações que possam int erferir nesse cot idiano. Os suj eit os da pesquisa são crianças e adolescent es port adores de Fibrose Císt ica em acom panham ent o num hospit al- escola do int erior do est ado de São Pau lo. Tr at a- se de pesqu isa com abor dagem qu alit at iv a, com colet a de dados em pír icos r ealizada m edian t e an álise de pr on t u ár ios e en t r ev ist a aber t a. Dos dados, em er gir am os segu in t es t em as: con h ecim en t o eq u iv ocad o sob r e a d oen ça; p r eocu p ação com a au t o- im ag em ; b u sca p elo au t ocu id ad o e esper ança de m elhor ias no fut ur o. Os r esult ados ev idenciam as r eper cussões da Fibr ose Císt ica no pr ocesso d e socialização d esses p acien t es, salien t an d o a im p or t ân cia d os p r of ission ais d e saú d e con h ecer em essas d em an d as e i n co r p o r ar em - n as ao p l an o d e cu i d ad o s, v i san d o a i n t er v en çõ es ef et i v as q u e p r o m o v am o cr escim ent o e o desenv olv im en t o infan t o- j uv enil.
DESCRI TORES: fibr ose císt ica; cr iança; adolescent e; cuidados de enfer m agem ; enfer m agem pediát r ica
1
Research insert ed in t he int egrat ed proj ect CNPq ( Process No 550713/ 2002- 6) and FAPESP ( Process No 01/ 10764- 0) ; 2 Undergraduat e St udent , Scient ific I nit iat ion Grant Holder, e- m ail: t at apizzi@hot m ail.com ; 3 Advisor, Associat e Professor, e- m ail: lim are@eerp.usp.br. Universit y of São Paulo at Ribeirão Pret o College of Nursing, WHO Collaborat ing Cent re for Nursing Research Developm ent
I NTRODUCTI ON
C
y s t i c Fi b r o s i s ( CF) , a l s o k n o w n a sMu cov iscid osis, is a ch r on ic, au t osom al, r ecessiv e,
h er ed it ar y an d st ill in cu r ab le d isease. Th e g en e of
Cy s t i c Fi b r o s i s i s l o c a t e d o n t h e l o n g a r m o f
chr om osom e 7 and is r esponsible for pr oducing t he
CFTR m em brane prot ein, which conduct s ions bet ween
in t r a an d ex t r acellu lar dom ain s. I n ciden ce lev els of
t he disease am ount t o 1 in every 1,500/ 2,000 in t he
sout h of Brazil. This is sim ilar t o levels in t he European
Ca u c a s i a n p o p u l a t i o n . Ra t e s d e c r e a s e i n o t h e r
Br azilian r eg ion s, t h at is, 1 case f or ev er y 1 0 , 0 0 0
bir t hs( 1).
As a result of deficient ion t ransport in cellular
m em br anes, t hick secr et ions ar e pr oduced fr om t he
e x o c r i n e g l a n d s , w h i c h e n d u p o b s t r u c t i n g t h e
canaliculi and duct s t hrough which t hey are secret ed,
pr ev ent ing t heir ar r iv al at t heir place of act ion( 1). As
t h er e ar e v ar iou s m u t at ion s in t h e CF g en e, sig n s
and sym pt om s can be m ore or less int ense, alt hough
t he upper airways and pancreas are always affect ed( 2).
N o w a d a y s , l u n g s y m p t o m a t o l o g y
m an if est at ion s r epr esen t t h e h igh est m or bidit y an d
m ort alit y rat es. The t hick m ucus clogs t he lungs and
b e c o m e s a c u l t u r e m e d i u m f o r b a c t e r i a a n d
m i c r o o r g a n i s m s , c a u s i n g r e p e a t e d p n e u m o n i a s ,
b r o n c h i e c t a s i s , p n e u m o t h o r a x , a m o n g o t h e r
com plicat ion s. On e ot h er big com plicat ion of Cy st ic
Fibrosis is t hat it affect s t he digest ive t ract , especially
cau sin g pan cr eat ic in su f f icien cy, w h ich im pedes t h e
pr odu ct ion an d/ or sen din g of digest iv e en zy m es t o
t h e d i g e st i v e t r a ct , ca u si n g p o o r d i g e st i o n . Th i s
pr oblem is m ainly due t o t he non- absor pt ion of fat s,
w h i c h c a u s e s s t e a t o r r h e a a n d p r o t e i n - c a l o r i c
m alnut rit ion in children and, t hus, affect s t heir weight
an d st at u r e dev elopm en t . Repr odu ct iv e or gan s an d
liv er can also be affect ed( 1).
Cy st i c Fi b r o si s ca n b e d i a g n o sed t h r o u g h
different m et hods. The m ost com m on one is t he Sweat
Te st . I t i s co n f i r m e d b y t w o m e a su r e m e n t s w i t h
ch l o r i d e co n cen t r at i o n s su p er i o r t o 6 0 m Eq / l( 1 ). I n
gener al, t he m ot her suggest s t he pr obable diagnosis
t o doct or s by m ent ioning t hat , w hen she k isses t he
baby ’s for ehead, t he k iss “ t ast es salt y ”( 2).
I n Brazil, Decree GM/ MS No 822 by t he Healt h
Min ist r y, issu ed on Ju n e 6t h 2 0 0 1 , est ab lish ed t h at
neonat al screening, bet t er known as t he “ Heel Prick”,
w h ich w as on ly u sed t o d et ect con g en it al d iseases
lik e Hy pot hy r oidism and Pheny lk et onur ia, w ould also
allow for t he early det ect ion of ot her diseases, including
Cy st ic Fibr osis( 3 ). Alt h ou gh t h is Decr ee h as been in
f or ce sin ce it s pu blicat ion , m an y h ealt h in st it u t ion s
st i l l d o n o t p er f o r m co m p l et e n eo n a t a l scr een i n g
because t hey hav e not been qualified by t he Single
He a l t h Sy st e m ( SUS) . Th i s q u a l i f i ca t i o n r e q u i r e s
com pliance wit h all st eps of t he process, ranging from
n eon at al scr een in g u n t il t h e f ollow - u p of d et ect ed
cases, as well as t he availabilit y of a m ult idisciplinary
t eam . Nowadays, only few privat e and public hospit als,
considered t o be cent ers of excellence for diagnosing
a n d t r e a t i n g t h e s e d i s e a s e s , p e r f o r m c o m p l e t e
screening. A com plem ent ary Decree( 4) also det erm ines
on free out pat ient , hospit al and m edicat ion t reat m ent
for CF pat ient s.
Tr eat m ent of CF is aim ed at m inim izing t he
signs and sym pt om s of t he disease and im peding it s
pr ogr ession . I t m ain ly in v olv es m ain t ain in g air w ay s
clean an d h u m id if y in g t h e m u cu s t o m ak e it m or e
f l u i d a n d f a c i l i t a t e e x p e c t o r a t i o n ; r e s p i r a t o r y
physiot herapy and using cort icost eroids and ant ibiot ics
t o av oid infect ions. Moreov er, nut rit ional supplem ent s
w i t h v i t a m i n s, sa l t s, l i q u i d s, e x t r a ca l o r i e s a n d
digest iv e en zy m es, bef or e m eals an d sn ack s, h av e
cont r ibut ed t o k eep pat ient s healt hy( 1).
I n 1938, 70% of childr en diagnosed w it h CF
died bef or e t h eir f ir st an n iv er sar y. As a r esu lt , t h e
disease was classified as a pot ent ially let hal pediat ric
p at h olog y. I n r ecen t y ear s, cou n t less st u d ies h av e
pr oduced br oader k now ledge about t he disease and
h av e led t o t h e u se of a t r eat m en t m or e coh er en t
wit h physiopat hology, which has provided an increase
in t hese pat ient s’ life expect ancy. Mean life expect ancy,
as docum ent ed by t he Epidem iological Cyst ic Fibrosis
Records in 1998, corresponded t o 32 years, alt hough
t his v ar ies accor ding t o t he count r y ’s socioeconom ic
and scient ific developm ent levels, t reat m ent condit ions
and pat ient s’ age at t he m om ent of diagnosis( 5).
Nowadays, a global epidem iological t ransit ion
is in cou r se, d u e t o t ech n olog ical ad v an ces in t h e
h ealt h ar ea as a w h ole, allow in g ch r on ic d iseases,
including CF, t o be cont r olled m or e easily. This fact ,
par t icular ly CF, is ev idenced by t he gr ow ing num ber
of adolescent s, young adult s and even m arried adult s
living w it h t he disease.
Socializat ion pr ocess
According t o developm ent t heories by Erikson
int er act ion w it h t heir env ir onm ent t hr ough r epeat ed
and varied act ions, which allows t hem t o get fam iliar
w i t h i t a n d e x p l o r e i t b e t t e r ; i n t h i s p r o ce ss, a
p r eced in g p h ase is n eed ed f or t h e d ev elop m en t of
t h e n ex t on e. I n t h e case of ch ild r en w it h ch r on ic
disease, depending on w hich phase of life sy m pt om s
st ar t t o ap p ear i n , t h e i m p act o n t h ei r co g n i t i v e,
em ot ion al an d social dev elopm en t w ill h appen in a
part icular way, t hus im peding fut ure developm ent s( 6).
So ci a l i za t i o n i s d e f i n e d a s “ a b r o a d a n d
co n si st e n t i n t r o d u ct i o n o f a n i n d i v i d u a l i n t o t h e
obj ect iv e w or ld of a societ y or a sect or of it ”. I t can
b e r an k ed as p r im ar y an d secon d ar y socializat ion .
The pr im ar y t y pe is w hat t he indiv idual ex per iences
during childhood and t hrough which ( s) he becom es a
m em b er o f a so ci et y ; t h e seco n d a r y t y p e i s a n y
su b se q u e n t p r o ce ss t h a t i n t r o d u ce s t h e a l r e a d y
socialized indiv idual int o new sect or s of societ y. The
fam ily is responsible for t he child’s first insert ion int o
t he obj ect ive social world, t o t he ext ent t hat it offers
t he lear ning of cult ur al elem ent s, such as language,
habit s, uses, cust om s, v alues, st andar ds, behav ior al
st an d ar d s an d at t it u d es an d , m ain ly, p r om ot es t h e
form at ion of basic personalit y and ident it y st ruct ures( 7).
Wit h r espect t o t he socializat ion of childr en
and adolescent s of school age who suffer from chronic
diseases, t hese ar e at t he int er sect ion of t he healt h
an d ed u cat ion sy st em s, in v iew of t h e sep ar at ion
bet ween bot h in m ost count ries’ t eaching inst it ut ions.
Hen ce, t h er e n eed s ar e n ot in t eg r at ed , cr eat in g a
deficiency in one of t he sect ors, usually t he educat ional
side, as healt h is ur gent . The lack of com m unicat ion
b et w een t h ese t w o sect or s an d ed u cat or s’ lack of
k n ow ledge ar e t h e m ain edu cat ion al con st r ain t s f or
t hese st udent s( 8).
Th e p r ob lem s t h ese ch ild r en ex p er ien ce in
school ar e dir ect ly r elat ed t o t he collat er al effect s of
t reat m ent , t o signs and sym pt om s of t he disease and
t o feelings lik e r ej ect ion, st r ess and isolat ion out of
sham e fr om t heir colleagues( 8). Dur ing cr isis per iods
of t he disease and due t o const ant hospit alizat ions,
children and adolescent s wit h CF m iss school and oft en
cannot pass t o t he next year because of t heir absences,
w hich discour ages t hem so st udy( 2).
To t h e ex t en t t h a t t h e l i f e ex p ect a n cy o f
ch ild r en an d ad olescen t s w it h CF in cr eases, issu es
r elat ed t o ch oosin g a p r of ession , r elat ion sh ip s an d
r epr oduct ion becom e m or e int ense and ex pect at ions
for t he fut ure st art t o appear, which can creat e anguish,
depr ession an d of t en t r eat m en t aban don m en t . I t is
dur ing t his t r ansit ion per iod fr om childhood t o adult
ag e t h a t f eel i n g s o f sel f - co n t r o l , i m m o r t a l i t y a n d
fr eedom of choice com e up. Tr eat m ent abandonm ent
o c c u r s b e c a u s e h e a l t h c a r e , w h i c h u s e d t o b e
at t r i b u t ed t o an o t h er p er so n , l i k e t h e m o t h er f o r
exam ple, is not at t ribut ed t o t he pat ient him - / herself( 2).
Furt herm ore, during t his phase, new social at t ribut ions
em er g e, su ch as w or k or t h e con t in u it y of sch ool
c o m m i t m e n t s w h i c h , t o g e t h e r w i t h t h e r a p e u t i c
com m it m ent s, lead t o a full agenda and ex haust ion
t o perform self- care, which m ay becom e less frequent
or r elegat ed t o t he back gr ound( 9).
I n spit e of t h eir t u r bu len t life an d con st an t
changes in t heir healt h st at e w hich, in t ur n, r equir e
ch an g es in t h e st r u ct u r e of car e an d in t h e f am ily
n u cleu s, m ost r elat iv es of ch ildr en an d adolescen t s
w it h CF and t hese pat ient s t hem selves consider t heir
l i v e s a s n o r m a l . Th i s p h e n o m e n o n i s k n o w n a s
n or m alizat ion an d occu r s in st igm at ized gr ou ps lik e
t he chr onically ill( 9).
I n t his sense, concer n about t hese childr en
and adolescent s’ qualit y of life has also been a focus
of int erest for researchers in t he area. Thus, t he Cyst ic
Fi b r osi s Qu est i on n ai r e w as d ev el op ed i n 1 9 9 7 . I n
Br a zi l , t h e v a l i d a t i o n o f t h i s i n st r u m e n t i s b e i n g
c o n d u c t e d b y Pr o f . Ta t i a n a Ro s o v , f r o m t h e
Dep ar t m en t of Ped iat r ics an d Reh ab ilit at ion of t h e
Fed er al Un iv er sit y of São Pau lo - Pau list a Med ical
Sch ool. Th is q u est ion n air e w ill m ak e it p ossib le t o
analyze t he im pact of t reat m ent and t he disease it self
on t he qualit y of life of Brazilian CF pat ient s( 10).
The a im of t his st udy is t o inv est igat e how
t he socializat ion pr ocess of childr en and adolescent s
wit h CF is occurring, based on t heir own experiences.
This research is j ust ified by t he possibilit y t o ident ify,
in t he daily lives of t hese pat ient s, aspect s t hat need
nur sing int er v ent ions, w it h a v iew t o im pr ov ing t heir
qualit y of life.
METHOD
This is a descript ive and explorat ory st udy( 11).
The answ er s t o t he r esear ch quest ions w er e sought
in t he qualit at ive approach, due t o t he charact erist ics
of t he st udy obj ect and t he pr oposed obj ect iv e. We
b eliev e t h is ap p r oach is p ar t icu lar ly v alu ab le, as it
p er m it s d iscov er in g t h e m ean in g of ev en t s, social
Th e r esear ch p r oj ect w as ap p r ov ed b y t h e
Resear ch Et h ics Com m it t ee of t h e st u dy in st it u t ion .
Th e s u b m i t t e d p r o t o c o l c o n t a i n e d t h e f r e e a n d
inform ed consent t erm , w hich described t he research
in sim ple language( 13). Before t he int erview, t his t erm
w as r ead an d d iscu ssed w it h t h e p ar en t s, ch ild r en
and adolescent s. On t his occasion, any doubt s w er e
clar ified and, aft er t he childr en and adolescent s had
agr eed t o par t icipat e, bot h par ent s and par t icipant s
signed t he t er m .
The st udy inst it ut ion is a t eaching hospit al and
reference for care delivery t o children and adolescent s
w it h CF, locat ed in t h e in t er ior of t h e st at e of São
Paulo. St udy par t icipant s w er e eight ( 8 ) childr en or
adolescent s w it h a diagnosis of CF, in t he age range
from 7 t o 18 years, m ale and fem ale, who were under
clinical and hospit al follow - up at t he st udy hospit al.
The choice of t his age range was due t o t he fact t hat
t h ese pat ien t s ar e in t h e ph ase of logical t h in k in g,
an d can t h er efor e com m u n icat e t h eir ideas v er bally
and giv e m eaning t o t he ex per iences( 6 ). We defined
t h e n u m b er of p ar t icip an t s w h en d at a p r ov id ed u s
w i t h su f f i ci e n t su p p o r t t o u n d e r st a n d t h e st u d y
p h en om en on .
We used open int erviews as a dat a collect ion
st r at eg y, d u e t o t h e f act t h at t h is t ech n iq u e g iv es
access t o descr ipt iv e dat a in t he subj ect s’ language
and because it allow s t he r esear cher t o int er act iv ely
develop an idea about how subj ect s int erpret aspect s
of t he world( 12).
Th e in t er v iew w as h eld at t h e par t icipan t s’
hom es ( 6 ) or at t he clinic ( 2 ) and consist ed of t w o
p a r t s: t h e f i r st p a r t , f o r i d e n t i f i ca t i o n , g a v e u s
i n f o r m a t i o n a b o u t s e x , a g e , e d u c a t i o n l e v e l ,
p r of ession / occu p at ion , w or k p lace an d or ig in . Th e
secon d con t ain ed t w o gu idin g qu est ion s: 1 . Tell m e
w hat y ou know about y our disease; 2. Do y ou t hink
y ou ar e differ en t fr om y ou r fr ien ds / acqu ain t an ces
w h o d o n o t h a v e Cy st i c Fi b r o si s? We l o o k e d f o r
com plem ent ar y dat a on t he pat ient char t s, w hen w e
exam ined t he diagnosis dat a and t herapeut ic schem e.
Moreover, w e used a field diary, w here w e regist ered
t he im pr essions obt ained fr om each int er view , giving
sp ecial at t en t ion t o n on - v er b al com m u n icat ion an d
fam ily r elat ion s.
Dat a in t er p r et at ion st ar t ed on t h e b asis of
t he fully t ranscr ibed t apes. We analy zed each set of
dat a ( in t er v iew , f ield diar y n ot es an d pat ien t ch ar t
dat a) per part , seeking t he codes, t hat is, phrases or
words t hat give m eaning t o t he inform at ion cont ained
i n em p i r i cal d at a( 1 2 ). Nex t , w e g r o u p ed t h e co d es
accor ding t o t heir sim ilar it ies, w hich r esult ed in t he
t hem es. The children’s and adolescent s’ report s w ere
ident ified according t o t he order of t he int erviews ( E.1
t o E. 8 ) . Fo r each si t u at i o n , w e i n d i cat ed t h e ag e
because we believe t his is an im port ant indicat or ( E.1,
1 8 y ear s) .
RESULTS
Dat a analysis allowed us t o ident ify 4 t hem es,
w h i ch a r e d i r e ct l y o r i n d i r e ct l y r e f l e ct e d i n t h e
socializat ion pr ocess. Th ese ar e:
Mist ak en k now ledge about t he disease
Most in t er v iew ees cou ld not ex plain ex act ly
w h a t Cy st i c Fi b r o si s i s. Ma n y o f t h e m m i x e d u p
inform at ion, like in t he following exam ples: I t ’s genet ic.
I know m ore or less, I don’t know very m uch. I know it ’s m ore or
less sim ilar t o Down’s Syndrom e. I t ’s from t he sam e chrom osom e,
I t hink it is, but I really don’t know for sure. ( E.1, 18 years) . I
k now it ’s w it hout or gan t her e and t hat y ou need dr ugs for
digest ion... ( E.3, 17 years) .
Ot her part icipant s associat ed t he disease wit h
prevailing signs and sym pt om s, as well as wit h specific
drugs: The disease I have...I cough a lit t le when I have quit e a
lot of cat arrh.... ( E.4, 10 years) . Cyst ic Fibrosis is a t hing in t he
lungs. Then, each t im e you’re going t o eat you have t o t ake t he
enzym e, but t hen you have t o do t he PULMOZYME® [ inhalat ion
m edicat ion] , if not it t urns int o pneum onia. ( E.6, 10 years) .
D a t a e v i d e n ce d t h a t sci e n t i f i c w o r d s l i k e
g en et ics, ch r om osom e an d h er ed it ar y ap p ear ed in
t h e r ep or t s q u it e f r eq u en t ly, alt h ou g h w it h ou t an y
act ual m eaning, em phasizing t he t echnicist language
o f t h e i n f o r m a t i o n t h ey r ecei v ed . Fo r ex a m p l e: I
explained t hat it ’s a genet ic disease...t hey asked what it was and
I said it was a genet ic disease! ( E.4, 7 years) .
A com m unicat ion bar r ier is cr eat ed bet w een
professionals and pat ient s, due t o t he use of t echnical
an d ab st r act lan g u ag e. I n m an y sit u at ion s, h ealt h
p r o f e s s i o n a l s o v e r e s t i m a t e p a t i e n t s a n d f a m i l y
m em bers’ knowledge, based on t he prem ise t hat t hey
m ast er t heir scient ific univ er se.
Ma n y CF p a t i e n t s p r e f e r t o m a i n t a i n t h e
d i se a se a se cr e t b e ca u se t h e y h a v e i n su f f i ci e n t
knowledge. By ignoring it , t hey cannot develop posit ive
coping st rat egies and t hen st art t o hide it as a form of
on t elling t o friends, not like t hat ; it ’s j ust t hat , like t hat , I only
t ell it t o t he closest ones, m ore close like t hat , you know? ( E.3, 17
years) . Nobody suspect s. Yes, and t hey will not know, I ’m never
gonna t ell ( E.1. 18 years) .
Then oft en hide t he disease because t hey do
n ot k n ow t h e t r an sm ission m ech an ism s an d , t h u s,
seek t o av oid people st igm at izin g t h em becau se of
t h e p o ssi b i l i t y o f co n t a g i o n , l i k e i n t h e f o l l o w i n g
exam ples: So, I t hink t hat people around should becom e aware
of what it is, you know? I t hink it ’s a bit sim ilar t o AI DS, like, you
say you have AI DS everybody im m ediat ely...does not want t o sit
dow n ( E.1. 18 years) .
Con cer n abou t self - im age
I t is when t hey st art school t hat children and
adolescent s wit h CF st art t o perceive t hey are different
from t heir colleagues( 2). Som et im es, t hese differences
a r e e v i d e n ce d b y t h e i r o w n co l l e a g u e s, ca u si n g
con st r ain t an d sh am e in pat ien t s, accor din g t o t h e
following report s: Two girls keep m aking fun of m e because
I ’m short ! I say, so what ? So what if I ’m short ? ( E.4, 7 years) I ’m
a bit short er t han m y classm at es... ( E.5, 10 years) .
I n p at i en t s w h o ar e st i l l g o i n g t o sch o o l ,
d if f er en ces g o b ey on d p h y sical ch an g es; t h er e ar e
a l s o r e s t r i c t i o n s t h e d i s e a s e i m p o s e s a n d t h e
cont inuously pr esent t r eat m ent , as m ent ioned: They
don’t have it , but I do, I can’t run because I ’m gonna cough. And
if I run a lot , j um p a lot , t hen I ’m gonna get t ired, feel bad ( E.6,
10 years) . I cough a lot and take a lot of drugs, the others [ children]
only t ake t hem when t hey are ill, I t ake t hem all t he t im e ( E. 4, 7
years) .
I n t he sit uat ions t he children experienced, we
obser v ed t h ey f elt u n com f or t able abou t t h e im age,
a l t h o u g h a s so m e t h i n g t h a t ca n n o t b e ch a n g e d .
Adolescent par t icipant s, on t he ot her hand, r ev ealed
t o be m or e con cer n ed abou t t h eir body im age w it h
respect t o changes and t heir possible int ervent ions: I
pract iced swim m ing for 7 years. I chose t his sport t o develop
t he lung, so t hat I wouldn’t get barrel chest and hunchback and
short w it h m ore or less 1.50 m ...[ ironic] . ( E.2, 18 years) .
Concer n about self- im age is also a st r at egy
t o fight t he st igm a, since it is an at t em pt t o m inim ize
t he phy sical sy m pt om s of t he disease and be equal
t o ot her persons: I was t hin, it bot hered m e, I couldn’t t ake
m y shirt of close t o friends... Then, I st art ed t o buy supplem ent s
from a supplem ent shop... So, I don’t know if it ’s t rue, I saw it on
t he I nt er net t hat , ov er t im e, t he m uscles of Cy st ic Fibr osis
gradually at rophy, I don’t know, I don’t know if t his is t rue. But
I didn’t st ick t o t his eit her t o do it [ weight lift ing] no, it ’s m ore t o
have, how do you say, t o have a bet t er physique act ually. ( E.1, 18
years) .
Body alt er at ion du e t o t h e pr ogr ess of t h e
disease is one of t he few com plicat ion childr en and
ad olescen t s m ay h av e som e con t r ol ov er, w h et h er
b y ex er cisin g or b y p r ev en t in g com p licat ion s af t er
t r eat m en t . Th is m ech an ism can b e classif ied as a
dir ect ed coping st rat egy, t hat is, or ient ed t owar ds a
specific st r essor.
Sear ch f or self - car e
We f o u n d t h a t m o st i n t e r v i e w e d p a t i e n t s
perform ed self- care in t heir search for independence:
Yes, so, since about t wo years, I t ook hold, you know? I st art ed
t o cope wit h t hings alone. I go t o t he hospit al alone, I t ake t he
prescript ions, if a prescript ion is m issing I say t his, I solve it
m yself. ( E.1, 18 years) . No...m y m ot her, lat ely, and m y fat her
j ust say: ‘you have t o do t his, you have t o do t hat , do t hat ot her
t hing’... I cannot keep depending on t hem m y whole life, I have t o
learn t o find m y way, go out and arrange m y life. ( E.2, 18 years) .
Th e i n t er v i ew ed ch i l d r en a n d a d o l escen t s
m ent ioned t hat t he disease cr ossed t heir daily liv es,
especially because care t ook a lot of t im e. Treat m ent
f or Cy st ic Fib r osis is con st an t , h ap p en s ev er y d ay
and several t im es per day, t hus t aking a lot of t im e.
Th is af f ect s t h e r ealizat ion of ot h er act iv it ies, lik e
working or playing for exam ple: I don’t have a lot of t im e
f or ot h er t h in gs. Becau se, u n t il I st ar t ed t o do t h e TOBI®
[ inhalat ion ant ibiot ic] , I had t o do 4 inhalat ions per day. Then I
had t o, what is it like, I had t o do t he PULMOZYME ® first , wait a
while...t hen, I only had t o do t he PULMOZYME®, spit , all of t his
t ook one hour. Then I had t o wait for at least 3 hours t o do t he
TOBI®, because I had t o do it wit h clean lungs. So t oget her t hat ’s
3 hours already, so, t hat m eans, if you have t o do som et hing like
working, there is no way. Soon afterwards, you had to do everything
again. Just wit h t he m achine on t hat ’s 40 m inut es st raight . So I
on ly f in ish at n igh t r eally . ( E. 1 , 1 8 y ear s) . Af t er I d o t h e
PULMOZYME®, I feel light t o be able t o play! ( E.4, 7 years) .
Alt hough t hey considered t he t herapy int ense
and exhaust ing, children and adolescent s in t his st udy
t u r n ed t o sel f - car e as a st r at eg y t o co n t r o l t h ei r
act iv it ies, sin ce t h is allow s t h em t o plan t h eir daily
lif e bet t er an d m in im ize t h e im pact of t h e disease,
especially in t h e sch ool en v ir on m en t , as m en t ion ed
below: So, we t ry, seeing t he possibilit ies and t he t im e I have
available during t he day t o do it . I don’t live in favor of Cyst ic
Fibrosis, it lives wit h m e and I t ake it along. I f I st op m y life t o
drugs m yself, because I know m y t im es, so I don’t get confused.
But I don’t t ake t he drug in school, I don’t eat when I ’m t here...I ’m
asham ed! ( E.3, 17 years) . I don’t take m edication in school, there’s
no reason why, because som et im es I have a snack, so I ’d have t o
t ake it wit h t he pancrease, you know? But I don’t t ake it , no, j ust
a snack, a j uice, I don’t t hink it ’s really necessary t o t ake it . ( E.1,
18 years) .
Hope for im pr ov em ent s in t he fut ur e
As CF is a gen et ic disease, t h e on ly v iable
c u r e i s g e n e t h e r a p y( 1 ). N e v e r t h e l e s s , t h e
int erviewees’ hopes were m ore direct ed at new drugs
and t her apies. The dissem inat ion of new t r eat m ent s
in t he m edia has cont ribut ed t o keep up t hese hopes,
for exam ple: Because m y m om said t hat , in Europe, t hey are
m aking a drug t o cure...t hey’re going t o cure it . ( E.5, 10 years) .
Th er e’s t h at v accin e [ ag ain st Pseu d om on as] , w h ich is n ot
available in Brazil yet . ( E.4, 7 years) . Yes, t he t rend is for t hings
t o get bet t er and bet t er, it ’s bet t er already! Over here, in Brazil,
m ucoviscidosis pat ient s used t o be 30 years old at m ost . ( E.2,
18 years) . Yes, I hope it im proves because of t he drug, which is
evolving. ( E.3, 17 years) .
Ch i l d r e n a n d a d o l e s c e n t s i n t h i s s t u d y
m ent ioned anot her for m of hope t hat t heir condit ion
w ill im p r ov e in t h e f u t u r e, an d d esir e t o h av e t h e
disease recognized by t he populat ion in general. They
suggest ed st r engt hening t he Br azilian Associat ion in
Su p p or t of Mu cov iscid osis ( ABRAM) , cam p aig n s t o
d i s s e m i n a t e t h e d i s e a s e a n d g r e a t e r m e d i a
i n v o l v e m e n t i n k n o w l e d g e d i sse m i n a t i o n . I n t h i s
respect , t hey report ed: I t hink t hat , if t he disease were well
explained, t here would be less prej udice... ( E.1, 18 years) . Look,
in Brazil, it only works when you go t o t he m edia...AI DS, Diabet es,
Cholest erol, Cancer, all of t hese t hings worked out , people accept
it , because it ’s in t he m edia. Cyst ic Fibrosis is not in t he m edia,
I also t hink it won’t be unt il som e daught er of a fam ous person is
born wit h t his disease... ( E.2, 18 years) . Ah, nobody really knows
what Cyst ic Fibrosis act ually is! ( E.4, 7 years) . I t hink t hat it ’s
m issing [ inform at ion] , in m y cit y I ’m t he only one who has it . No
one underst ands it w ell! ( E.7, 12 years) .
DI SCUSSI ON
I n r e l a t i o n t o t h e t h e m e o f m i s t a k e n
knowledge about t he disease, com m unicat ion bet ween
t h e h eal t h t eam , p at i en t s an d f am i l y m em b er s i s
f u n d a m e n t a l t o u n d e r s t a n d t h e o b s e r v e d
p h en om en on . Th e p r of ession als’ w or ld is b ased on
science and t echnology and t heir language is basically
const r uct ed in t his rat ionalit y. Fam ilies, childr en and
ad o l escen t s, o n t h e o t h er h an d , ar e i m m er sed i n
changes in t heir daily lives as a result of t he disease
and t heir int eract ions are support ed by t heir affect ive
life. As language is one sym bolic com m unicat ion form ,
sy m bols do n ot alw ay s h av e t h e sam e m ean in g for
f am ilies an d pr of ession als( 1 4 ). Hen ce, as m en t ion ed
a b o v e , w o r d s w h o s e m e a n i n g s s e e m t o b e
under st andable in t he ey es of pr ofessionals m ay be
e m p t y, o r h a v e a n o b s c u r e s e n s e f o r c h i l d r e n ,
adolescent s and t heir r elat iv es.
Th e i n d i v i d u al s’ d i sease an d d ev el o p m en t
cross several t im es during t he lives of chronic pat ient s
and t he cognit ive dom ain, t hat is, how t hey underst and
a n d a s s e s s t h e d i s e a s e a n d i t s s t r e s s o r s , w i l l
d e t e r m i n e h o w t h e y w i l l h a n d l e t h i n g s w h e n t h e
d i se a se i s i n t e r f e r i n g i n t h e i r l i v e s. W h e n t h e se
indiv iduals ar e childr en, t he m eaning of t he disease
w ill depen d on w h at is said t o t h em , on w h at t h ey
experienced and t heir developm ent al abilit ies( 15). Thus,
t he childr en’s and adolescent s’ k now ledge about t he
d i se a se w i l l b e d e t e r m i n a n t f o r t h e m t o d e v e l o p
p osit iv e cop in g st r at eg ies, w h ich w ill m in im ize t h e
st ressors t hat w ill nat urally com e up in t heir lives as
chr onic pat ient s.
Co n c e r n w i t h s e l f - i m a g e r e f l e c t s s o c i a l
pr ocesses; one older one, called n or m alizat ion, and
t he ot her, cont em porar y, called t he cult of t he body.
A l t h o u g h d i s t i n c t , i n t h e c a s e o f c h i l d r e n a n d
adolescent s w it h Cy st ic Fibr osis, bot h ar e used w it h
t he sam e pur pose, t he sear ch for nor m alit y.
I n spit e of indiv idual m anifest at ions, st igm a
and discr im inat ion ar e social const r uct ions. St igm a is
def in ed as “ an at t r ibu t e t h at is deeply depr eciat iv e
and t hat , in t he ey es of societ y, ser v es t o discr edit
t he person who has it ”. St igm at ized persons are seen
as possessing an unw ant ed differ ence, t hus, st igm a
ap p ear s in societ y t h r ou g h d if f er en ces, d ev iat ion s.
D i scr i m i n a t i o n , o n t h e o t h e r h a n d , i s a n a ct o f
et hnocent rism in societ y and m eans not liking what is
d i f f e r e n t( 1 6 ). H e n ce , n o r m a l i z a t i o n a p p e a r s a s a
st rat egy t o cope w it h t he st igm a, t o t he ext ent t hat ,
t h r o u g h a ct i o n s, p e o p l e a t t e m p t t o m i t i g a t e t h e
d if f er en ce an d t u r n it s ex ist en ce closer t o w h at is
n or m al( 9 ).
Before t hey went t o school, m any CF children
of school age did not hav e an idea about t he ex act
d im en sion of t h eir d if f er en ces in r elat ion t o ot h er
t hat t hey are short er, t hat t hey cough and expect orat e
m o r e an d t h at t h ey t ak e d r u g s f r eq u en t l y an d i n
g r eat er q u an t it ies t h an m ost of t h eir colleag u es( 2 ).
Ch ild r en of sch ool ag e n eed t o f eel eq u al t o t h eir
co l l e a g u e s. I n t h i s a g e r a n g e , t h e y p e r ce i v e t h e
ph y sical differ en ces an d dist in gu ish dev iat ion s fr om
nor m alit y. Childr en consider ed “ nor m al” indicat e t he
d ef ect s o f o t h er s w h o a r e “ d i f f er en t ” f r o m t h em ,
cau sin g sh am e an d con st r ain t s( 1 7 ). Wh en p r ob lem s
r elat ed t o t he disease and t r eat m ent appear dur ing
childhood or adolescence, social adj ust m ent problem s
ar e ex p ect ed . Wh en ch ild r en an d ad olescen t s w it h
ch r on ic d isease d o n ot k n ow t h e m ean in g of t h eir
d i se a se a n d a r e d i f f e r e n t f r o m t h e o t h e r s, t h e i r
accept ance by t heir colleagues is put at r isk( 8). Ev en
i f sch o o l ap p ear s as an o b st acl e f o r ch i l d r en an d
adolescent s wit h CF, it can help, providing experiences
t h a t ca n i n cr e a se t h e i r se l f - e st e e m , su ch a s t h e
d ev elop m en t of sk ills an d in ser t ion in t o a h ealt h y
en v ir on m en t( 2 ).
Th e n o r m a l i za t i o n p r o ce ss p e r m e a t e s a l l
phases in t he liv es of fam ilies and pat ient s w it h CF,
a l t h o u g h t h e st r a t e g y u se d t o m a i n t a i n i t v a r i e s
accor din g t o t h e ph ase of t h e disease. Besides t h is
co p i n g m e ch a n i sm , d u e t o t h e t r a j e ct o r y o f t h e
disease, rout ine care and t he fact t hat it is a congenit al
d i se a se , f a m i l i e s a n d p a t i e n t s d o n o t h a v e a n y
experience wit h anot her t ype of life and consider t heir
life as nor m al( 9).
Th e n or m alizat ion pr ocess also includes
so-called pr oblem - solv in g copin g st r at egies, w h ich u se
sp ecif ic act ion s t o solv e st r essor s, w h ich ar e also
specific for t he disease. Norm alizat ion is observed by
d ef i n i n g t h e ch i l d a n d a d o l escen t a s n o r m a l , n o t
differ ent fr om it s healt hy peer s, w hen t hey est ablish
r o u t i n e s w i t h t h e t r e a t m e n t a n d s e l f - c a r e . Th e
n o r m a l i t y a t t r i b u t e i n d i ca t e s t h e f o r m a t i o n o f a
cognit iv e schem e, t hr ough w hich t he child st ar t s t o
i n t e r p r e t h i s/ h e r d i se a se - r e l a t e d e x p e r i e n ce s a s
som et hing nat ur al and fam iliar( 15).
The biggest st igm as in CF are relat ed t o t he
fact t hat t hese children and adolescent s are physically
different from ot hers in t he sam e age range. The fact
t hat t hey are t hinner and, som et im es, present corporal
changes like clubbing of t he fingers and barrel chest ,
accen t u at es t h i s d i f f er en ce, an d t h i s m ak es t h em
i n cr easi n g l y t u r n t o n o r m al i zat i o n p r o cesses. Th e
body- relat ed st igm a is also present in ot her diseases,
a n d i s d e t e r m i n e d b y t h e w a y so ci e t y cl a ssi f i e s
p h y si cal n or m al i t y an d v al u es cor p or al at t r i b u t es.
Since t he 1980’s, t he w or ld has been obser v ing t he
growing valuat ion of m uscles and healt hy appearance,
encouraged by t he indust rial increase in product s t hat
in dicat e beau t y an d h ealt h at t h e sam e t im e. As a
result , t his st im ulat es t he cult of t he body and healt hy
appear an ce an d ex clu des people w h o do n ot f ollow
t hese st andar ds. This m ay occur w it h Cy st ic Fibr osis
pat ient s, w hose disease is phy sically v isible.
When t hey sear ch for self- car e, childr en and
adolescent s w it h CF once again m ay be r ecur r ing t o
nor m alizat ion, t hr ough t he pr oblem - solv ing st rat egy,
w h ich also pr om ot es t h e sear ch f or k n ow ledge an d
t r eat m ent adher ence. This st r at egy is based on t he
organizat ion of care t o decrease t he disease’s im pact
on t heir daily lives( 15). I n a st udy of adult s w it h CF,
m an y p at ien t s m en t ion ed t h at t h ey d id n ot lik e t o
p er f or m self - car e ev er y d ay or m an y t im es d u r in g
t he day as, t his way, t he disease becom es increasingly
pr esent in t heir liv es, filling t heir agenda m or e t han
t hey would like t o. However, t hey perform ed self- care
because t hey were afraid of t he disease’s evolut ion( 9).
Par t i cu l ar l y f o r ad o l escen t s, sel f - car e i s a cr u ci al
com pon en t in t h e t r an sit ion f r om pediat r ic t o adu lt
care, const it ut ing a fundam ent al inst rum ent t o develop
self - est eem , m ain ly f or p at ien t s w h o d ep en d ed on
t heir parent s’ care for a long t im e( 15).
I n r epor t ing t heir hopes for im pr ov em ent in
t h e f u t u r e, r e l a t e d t o t h e r a p e u t i c m e a su r e s, t h e
children and adolescent s who part icipat ed in t his st udy
r est r ict ed t heir anx iet ies t o less com plex issues and
did not quot e t he cure of t he disease, perhaps due t o
a lack of knowledge about t he im plicat ions of a genet ic
d isease. How ev er, p ar t icip an t s’ g r eat est h op e w as
r e l a t e d t o t h e d i s s e m i n a t i o n o f t h e d i s e a s e b y
com m unicat ion channels, school and associat ions. This
r esu l t i s esp eci al l y i m p o r t an t f o r t h i s st u d y, as i t
em p h a si zes t h e n eed f o r b et t er st r u ct u r ed so ci a l
suppor t t o t his client ele.
One of t he coping st rat egies for children and
ad olescen t s w it h ch r on ic d isease is t h e sear ch f or
social suppor t fr om fr iends, fam ily and pr ofessionals,
as t h ey con sider t h is is on e of t h e m ost sign ifican t
ways t o cont rol st ressors caused by t he disease. Social
suppor t can be of help in differ ent phases in chr onic
p a t i e n t s ’ l i v e s , w h e t h e r b y p r o v i d i n g e m o t i o n a l
suppor t , solv ing disease- r elat ed quest ions and giv ing
care and dist ract ion in difficult periods. I n m ost cases,
t his support is provided by t he parent s. However, when
in cr eases, w h ich is especially im por t an t f or sch
ool-age ch ildr en an d adolescen t s. Th e sear ch for social
support from friends varies according t o t he children’s
an d adolescen t s’ ages an d con t ex t s. Th e st igm a of
t he disease influences t he choice of suppor t , m ak ing
t hem t ur n t o people w ho hav e dem onst r at ed loy alt y
and sensit iv it y, seek per sons w ho ex per ience sim ilar
si t u a t i o n s o r si m p l y h i d e t h e d i se a se f r o m t h e i r
friends( 15). The sear ch for people w ho exper ience t he
sam e sit uat ion, t hat is, ot her CF pat ient s, is a t ype of
so ci a l su p p o r t b a se d o n n o r m a l i z a t i o n si n ce , i n
cont act s wit h people who share t he sam e experiences,
ch i l d r en an d ad o l escen t s i d en t i f y t h em sel v es an d
b ecom e eq u al t o t h eir p eer s. Th is is an im p or t an t
support , as it allows for t he exchange of experiences
r e l a t e d t o t h e d i s e a s e , c o p i n g s t r a t e g i e s a n d
socializat ion .
CONCLUSI ONS AND I MPLI CATI ONS
We believe t hat t his st udy offers considerable
con t r ibu t ion s t o u n der st an d h ow Cy st ic Fibr osis can
int er fer e in t he socializat ion pr ocess of childr en and
a d o l e sce n t s w i t h t h i s d i se a se . Re co m m e n d a t i o n s
e m e r g e d f r o m t h e p a r t i ci p a n t s’ e x p e r i e n ce s a n d
suggest : elabor at ion of educat iv e folder s; lect ur es in
schools; pr opaganda and subj ect s on t elev ision and
st r engt hening leagues and associat ions t hat fight for
t he cause, so t hat pat ient s, r elat ives and societ y get
t o know t he disease and st art t o cope wit h CF and it s
pat ient s in a com pr ehensiv e and r espect ful w ay.
We also consider t hat t hese pat ient s’ relat ion
wit h school, work, friends and fam ily is im paired, not
because of t he disease in it self but due t o t he lack of
posit iv e coping st r at egies. Mist ak en k now ledge about
Cy s t i c Fi b r o s i s u n c h a i n s n o n - p o s i t i v e c o p i n g
st r at egies, w hich lead t o dist ancing fr om colleagues,
in cr eased f am ily pr oblem s an d dif f icu lt ies t o at t en d
environm ent wit h great er socializat ion, such as school
and w or k .
W i t h a v i e w t o p u t t i n g p a r t i c i p a n t s ’
suggest ions in pract ice, t here is a need for art iculat ion
am o n g h eal t h p r o f essi o n al s, ci v i l so ci et y an d t h e
St at e. The lat t er should develop healt h and educat ion
p o l i ci e s a i m e d a t t r a i n i n g p r o f e ssi o n a l s w h o a r e
cap ab l e of d eal i n g w i t h t h ese d em an d s; est ab l i sh
g r eat er ar t icu lat ion b et w een h ealt h an d ed u cat ion
sect o r s t o d el i v er i n t eg r al car e t o t h ese ci t i zen s.
Pr ofessionals should pr ov ide clear infor m at ion about
t he disease, not only t o pat ient s, but also t o relat ives.
An d societ y n eed s t o w elcom e t h ese ch ild r en an d
adolescen t s w it h ou t an y st igm a, as t h is is t h e on ly
w ay t o int ensify t heir r elat ion w it h school, w or k and
f r ien d s an d , t h u s, t o g u ar an t ee a b et t er q u alit y of
lif e.
ACKNOW LEDGEMENTS
Thanks t o t he m ult idisciplinary Cyst ic Fibrosis
t eam at t h e Ribeir ão Pr et o Medical Sch ool Hospit al
d as Cl ín i cas an d t o t h e Su p p o r t Gr o u p f o r Cy st i c
Fibr osis Pat ient s and Relat iv es.
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