Professional Documents
Culture Documents
TEZ DE DOCTORAT
- rezumat -
Doctorand,
Hogea (Lzrescu) Lavinia Maria
Conducator tiinific,
Prof. univ. dr. Rcanu Ruxandra
BUCURETI
2012
Doctorand,
Hogea (Lzrescu) Lavinia Maria
Conducator tiinific,
Prof. univ. dr. Rcanu Ruxandra
BUCURETI
1
2012
CUPRINS
n loc de introducere...........................................................................3
Argument.............................................................................................5
Capitolul I............................................................................................6
Repere teoretice privind sntatea i boala
I.1. Hemofilia o boal rar........................................................................................10
I.2. Hemofilie i ereditate............................................................................................11
I.3. Tabloul clinic al hemofiliei...................................................................................13
I.4. Formele clinice ale hemofiliei...............................................................................21
I.5. Hemofilia n lume.................................................................................................23
I.6. Manifestri hemoragice i complicaii..................................................................25
Capitolul II........................................................................................35
Aspecte psihosociale ale coagulopatiei cronice
II.1. Handicap, deficiene, dizabiliti clasificare internaional..............................37
II.2. Profilul psihologic al bolnavului hemofilic.........................................................40
II.3. Impactul bolii asupra pacientului i familiei.......................................................42
II.4. Alexitimia frecven, caracteristici...................................................................52
II.5. Comportamentul obsesiv-compulsiv caracteristici, manifestri.......................61
II.6. Tratamentul hemofiliei din perspectiv psihologic............................................63
Capitolul III.......................................................................................68
Calitatea vieii i deschiderea psihosocial privind bolile cronice
III.1. Concept omniprezent: calitatea vieii (definiii, accepii)..................................70
III.2. Dimensiunile calitii vieii i interrelaii...........................................................71
III.3. Factorii determinani ai calitii vieii................................................................76
III.4. Instrumente de msurare a calitii vieii...........................................................81
III.5. Copilria i calitatea vieii..................................................................................85
III.6. Implicaii ale evalurii calitii vieii (pentru pacient, familie, organizaii,
comitete).......................................................................................................................86
Capitolul IV.......................................................................................89
Metodologia cercetrii
IV.1. Scopul cercetrii.................................................................................................89
IV.2. Obiectivele cercetrii.........................................................................................90
IV.3. Ipotezele cercetrii.............................................................................................91
IV.4. Loturile investigate (caracteristici, criterii de includere / excludere)................92
IV.5. Instrumente utilizate n cercetarea noastr.........................................................96
IV.6. Procedura de lucru (specificiti).....................................................................106
Capitolul V......................................................................................107
Interpretarea rezultatelor cercetrii
Concluzii..........................................................................................168
Bibliografie......................................................................................183
Anexe
3
Argument
Trim ntr-o lume n care cunotiinele privind tratamentul hemofiliei s-au dezvoltat,
totui, din pcate acesta nu se poate aplica la nivel global. Cu toate c, n ultimele decenii
s-au nregistrat numeroase progrese n identificarea bolii, punerea corect a diagnosticului,
diagnosticul prenatal, existena produselor terapeutice, de aceste beneficii se bucur doar o
parte dintre bolnavii hemofilici, acetia provenind din mediile dezvoltate. ns, pentru cei
muli, hemofilia sever continu s se manifeste ca i pn acum, sub forma unui dezastru
personal i social [Poenaru D.V., et al., 2005]. Din pcate, dei se nregistreaz un numr de
aproximativ 1.585 pacieni, Romnia se confrunt cu aceast situaie, innd cont de faptul c
bugetul alocat acestei afeciuni este foarte sczut.
S trieti cu hemofilie nseamn s duci o povar n spate destul de grea. nc din
copilrie n viaa zilnic a hemofilicilor trebuie luate o serie de precauii, cum ar fi: evitarea
loviturilor i cderilor; alegerea de preferin a cmilor cu mneci lungi, a hainelor i
pantalonilor matlasai, a nclmintei care acoper clciele; purtarea cu sine a unei truse
farmaceutice care conine cele necesare unei perfuzii. De asemenea, extraciile dentare
trebuie s fie efectuate n mediu spitalicesc, sporturile violente i periculoase sunt interzise.
Totodat, meseriile manuale sunt excluse din orientarea profesional. Toate acestea condiii
conduc la limitarea comportamentului adaptativ, individul devine incapabil de a realiza
sarcinile propuse. Repetatele insuccese l fac s devin anxios i descurajat, pentru alte
motive dect boala n sine.
Boala afecteaz psihicul bolnavului prin durata ei (pe tot parcursul vieii), dar i prin
complexele de inferioritate pe care i le creeaz, n raport cu persoanele sntoase de aceeai
vrsta.
De aceea este important s tim cum gndete un hemofilic, ce simte i cum se
comport pentru ai mbuntii calitatea vieii i starea general a sntii.
Totodat, este important s cunoatem caracteristicile psihologice al acestor pacieni
pentru a ghida i conduce terapeutica necesar afeciunii lor, pentru a interveni n
armonizarea terapeuticii medicale cu psihoterapia.
Capitolul I
Repere teoretice privind sntatea i boala
suficient de factor de coagulare pentru ca formarea cheagului de snge s aib loc i astfel
boala s nu se manifeste. Dar ea va fi purttoare (car-rier), putnd transmite mai departe
X-ul cu defect copiilor si [erban, M., et al., 2006].
Formarea deficitar a
Capitolul II
Aspecte psihosociale ale coagulopatiei cronice
Conceptul de fenomen psihosocial definete rezultatul conlucrrii, apropierii i
contopirii psihologicului cu socialul, reprezentnd un nou nivel de realitate pentru care sunt
definitorii alte determinri calitative.
Examinarea acestei categorii de fenomene se face prin raportarea la conceptul de
contiin social, ele fiind considerate drept primul nivel structural al contiinei, peste care
se suprastructureaz planul ideologic. Fenomenele psihosociale nu sunt numai fapte
spirituale, de contiin, i nu se regsesc n totalitate n interiorul ei, ci se muleaz nemijlocit
pe condiiile sociale de existen, sunt fenomene de via reale, dimensiuni ale vieii cotidiene
ale oamenilor care dispun de o corporalitate traductibil n indicatori obiectivi [Sillamy, N.
1995].
Comportamentul reprezint forma cea mai expresiv a mbinrii socialului cu
psihologicul, realizat cu participarea contiinei dar fr s se cuprind integral n categoriile
contiinei; se refer la totalitatea reaciilor pe care o fiin vie le exprim n mod organizat
fa de incitaiile incluse n factorii de mediu; se constituie, de fiecare dat, bazat pe o alegere
7
(selecie) dintr-o mulime de reacii posibile, alegere finalizat pentru meninerea n condiii
optime a formei i funciilor fiinei respective ca tot [chiopu, U., 1997].
Formarea personalitii umane este rezultatul unui proces complex de socializare, n
care interacioneaz factorii personali, de mediu i culturali.
II.1.
10
Capitolul III
Calitatea vieii i deschiderea psihosocial privind bolile cronice
n tratamentul unei afeciuni cronice unul din scopurile principale este reprezentat de
reducerea impactului bolii asupra pacientului, de mbuntire a calitii vieii acestuia.
Aadar, nu putem vorbi de evaluarea calitii tratamentului unei afeciuni cronice fr s
facem referire la calitatea vieii bolnavilor care beneficiaz de acel tratament.
11
12
Capitolul IV
Metodologia cercetrii
IV.1. Scopul cercetrii
Studiul de fa i propune s evalueze calitatea vieii pacienilor hemofilici, precum i
evidenierea unor particulariti psiho-clinice specifice acestora. Datele obinute pot fi utile
personalului psiho-medical ce-i desfoar activitatea n acest domeniu i pot contribui la
orientarea specific a interveniei psihoterapeutice sau de consiliere.
gradul
de
handicap
al
pacienilor;
situaia
Capitolul V
Interpretarea rezultatelor cercetrii
Ipoteza 1 Exist diferene semnificative statistic ntre pacienii hemofilici i
persoanele sntoase n privina domeniilor calitii vieii.
Pentru verificarea acestei ipoteze s-au folosit rezultatele obinute n Anamnez, iar
pentru msurarea domeniilor calitii vieii (general, fizic, psihologic, independen, relaii
14
M
domeniul
AS
Nonhemofilici
AS
Vrsta
Vrsta
Vrsta
16-25 ani
26-35 ani
36-45 ani
AS
AS
AS
12,33
3,26 15,40
2,19 14,28
3,12 13.97
2,70
11,82
3,45
11,84
1,18 11,87
1,85 11,78
1,76 11,93
1,11
12,86
1,11
11,87
1,28 12,65
1,25 12,21
1,23 12,55
1,44
11,97
1,33
12,03
1,21
11,91
0,80 12,07
0,97 11,73
0,84
11,95
1,46
11,49
1,13 12,16
1,16 11,95
1,21 11,83
1,12
11,28
1,08
11,26
1,36 12,59
0,96 12,25
1,23 11,46
1,47
11,28
1,35
11,65
2,99 13,33
1,74 12,72
2,64 12,81
1,78
10,89
2,98
general
domeniul
fizic
domeniul
psihologic
domeniul
independen
domeniul
relaii
sociale
domeniul
mediu
nconjurtor
domeniul
spiritualitate
Se remarc diferene semnificative statistic ntre cele dou loturi n aproape toate
domeniile calitii vieii studiate [Tabel. 5.]. Diferene extrem de semnificative statistic
remarcndu-se n cadrul domeniului general al sntii, mediul nconjurtor, domeniul
psihologic, relaii sociale i n domeniul spiritualitate/religie/convingeri personale (p< .001;
p< .05).
15
Ipoteza lucrrii este unilateral, n vederea testrii ipotezei s-a realizat o ANOVA
univariat 2 (persoane care sufer de hemofilie i persoane care nu sufer de hemofilie) X 3
(vrsta 16-25 ani, 26-35 ani, 36-45 ani) pentru a investiga diferenele dintre grupuri i
efectul de interaciune pentru fiecare domeniu n parte: calitatea vieii generale, domeniul
fizic, psihologic, independen, relaii sociale, mediu nconjurtor i spiritualitate.
Prelucrarea statistic a confirmat parial ipoteza, exist diferene semnificative statistic
ntre hemofilici i nonhemofilici, hemofilicii au un nivel mai sczut al calitii vieii pe
domeniile general, psihologic, relaii sociale, mediu nconjurtor i spiritualitate comparativ
cu nonhemofilicii.
n ce privete efectul variabilei vrst asupra domeniilor calitii vieii, a fost
confirmat ca semnificativ statistic doar n cazul domeniului mediu nconjurtor, pentru
celelalte domenii efectul fiind nesemnificativ statistic. De asemenea, efectul de interaciune
lot, vrst i domeniile calitii vieii este semnificativ doar n cazul unui singur domeniu al
calitii vieii, respectiv spiritualitatea.
Ipoteza 2 Exist diferene semnificative statistic n ceea ce privete nivelul
comportamentelor obsesiv-compulsive la pacienii hemofilici, comparativ cu cei din
lotul de control.
Ipoteza lucrrii este unilateral, n vederea testrii ipotezei s-a realizat o ANOVA
univariat 2 (persoane care sufer de hemofilie i persoane care nu sufer de hemofilie) X 3
(vrsta 16-25 ani, 26-35 ani, 36-45 ani) pentru a investiga diferenele dintre grupuri i
efectul de interaciune. Pentru a valida aceast ipotez invocm urmtoarele argumente
cantitative:
Tabel. 13. Tabel sumar ANOVA 3X2 factorial pe dimensiunea comportamente
obsesiv-compulsive
Sursa dispersiei
SS
Df
MS
Lot
336,244
336,244
7,286*
.008*
Vrsta
138,281
69,140
1,498
.226
Lot X vrsta
26,348
13,174
.285
.752
Eroare
8952,936
194
46,149
Total
22359,00
200
Din cele trei raporturi F, prezentate n tabelul 13, doar unul este semnificativ statistic:
efectul principal F lot [F (1, 194)= 7,286, p < .01 (M= 9,67 versus M= 6,22)]. Aceasta
nseamn c exist diferene semnificative statistic ntre hemofilici i nonhemofilici,
hemofilicii au un nivel mai mare al comportamentelor obsesiv-compulsive, comparativ
cu nonhemofilicii, datorit faptului c efectul principal pentru loturi (hemofilici versus
nonhemofilici) se prezint ca semnificativ statistic.
Ipoteza 3 Presupunem c exist diferene semnificative statistic n ceea ce
privete nivelul alexitimiei, n sensul c subiecii din lotul clinic vor avea un nivel mai
mare al alexitimiei, comparativ cu cei din lotul de control.
Ipoteza lucrrii este unilateral. n vederea testrii ipotezei s-a realizat o ANOVA
univariat 2 (persoane care sufer de hemofilie i persoane care nu sufer de hemofilie) X 3
(vrsta 16-25 ani, 26-35 ani, 36-45 ani) pentru a investiga diferenele dintre grupuri i
efectul de interaciune. Pentru a valida aceast ipotez invocm urmtoarele argumente
cantitative:
SS
Df
MS
Lot
3839,481
3839,481
31,261**
.000**
Vrsta
542,461
271,230
2,208
.113
Lot X vrsta
601,229
300,615
2,448
.089
Eroare
23827,0
194
122,820
Total
34594,5
200
Din cele trei raporturi F, prezentate n tabelul 14, doar unul este semnificativ statistic:
efectul principal F lot [F (1, 194)= 31,261, p < .001, (M= 69,83 versus M= 55,76)]. Aceasta
nseamn c ipoteza lucrrii este confirmat, deci exist diferene semnificative statistic
ntre hemofilici i nonhemofilici, hemofilicii au un nivel mai mare al alexitimiei,
comparativ cu nonhemofilicii, datorit faptului c efectul principal pentru loturi (hemofilici
versus nonhemofilici) se prezint ca semnificativ statistic.
17
fizic,
domeniul
relaii
sociale
sunt
predictori
pentru
nivelul
forma
Alexitimia
hemofiliei
forma
sntate
funcionare
domeniul
general
fizic
relaii sociale
hemofiliei
alexitimia
.00
sntate
-.16
-.32
funcionare
-.27*
-.20*
.52*
fizic
-.03
-.23
.13
.25*
general
domeniul
relaii sociale
Conform datelor din tabelul 17 n care este prezentat matricea de corelaii ntre
predictorii inclui n model, nu s-a identificat o situaie de multicoliniaritate. Analiznd
tabelul 18 observm c ntre forma hemofiliei, alexitimia, sntate general, durere, domeniul
mediu nu exist corelaii foarte puternice, ceea ce indic faptul c posibilitatea apariiei unei
situaii de multicoliniaritate este redus [idem].
18
Regresie
R
Ajustat
.076
.006
-.004
.494
.244
.203
Coeficieni
Coeficieni
Model 2
Nestandardizai
standardizai
Beta
Eroarea
Sig.
-2.991
.004
standard
Interceptul
-28.411
9.498
forma
.669
1.302
.048
.514
.609
alexitimia
.248
.054
.444
4.579
.000
sntate
.088
.044
.217
1.985
.050
funcionare
-.082
.035
-.261
-2.356
.021
fizic
1.711
.580
.279
2.947
.004
hemofiliei
general
relaii sociale
Alexitimia are o valoare pozitiv (b=.248), prin urmare se stabilete o relaie direct
ntre alexitimie i nivelul comportamentelor obsesiv-compulsive. Astfel, atunci cnd
alexitimia crete cu o unitate, nivelul comportamentelor obsesiv-compulsive tinde s creasc
cu o unitate (mai precis cu 0.24) n condiiile n care se pstreaz constant influena celorlali
predictori.
Sntatea general are o valoare pozitiv (b=.088), prin urmare se stabilete o relaie
direct ntre sntatea general i nivelul comportamentelor obsesiv-compulsive. Astfel,
atunci cnd aceasta crete cu o unitate, nivelul comportamentelor obsesiv-compulsive tinde s
creasc cu o unitate (mai precis cu 0.08) n condiiile n care se pstreaz constant influena
celorlali predictori.
19
Comporta
forma
mente
sntate
funcionare
domeniul
hemofiliei
obsesiv-
general
fizic
relaii sociale
compulsive
forma
hemofiliei
comportamen
te
.076
-.16
-.03
-.27*
-.18
.52*
-.03
.13
.13
.25*
obsesiv-
compulsive
sntate
general
funcionare
fizic
domeniul
relaii sociale
20
Regresie
R
Ajustat
.002
.000
-.010
.549
.302
.264
Suma
Df
Media
Regresie 1
ptratelor
Regresia
.083
0.83
Reziduri
15316.027
98
156.289
Total
15316.110
99
Model
Suma
Df
Regresie 2
Ptratelor
Regresia
4618.473
923.695
Reziduri
10697.637
94
113.805
Total
15316.110
99
Sig
.001
.982
Sig
8.116
.000
ptratelor
Media
ptratelor
n urma analizei datelor se observ c primul model nu explic mai bine dect cel
bazat pe medie dispersia datelor, F(1,98)= .982, p> .05. Modelul numrul 2, care cuprinde pe
lng forma hemofiliei i nivelul comportamentelor obsesiv-compulsive, sntate general,
funcionare fizic, domeniu relaii sociale, este semnificativ statistic mai bun dect cel obinut
pe baza mediei cu valorile F(5,94)= 8.116, p< .01. Din cei cinci predictori, trei (nivelul
comportamentelor obsesiv-compulsive, sntate general, domeniu relaii sociale) se prezint
semnificativ statistic dup cum se poate vedea n tabelul 24. Acest model este capabil s
explice n form ajustat o proporie de 26,4% (R ajustat = .264) din evoluia dispersiei
nivelului comportamentelor obsesiv-compulsive, n vreme ce restul de 73,6% rmne
neexplicat, ntruct se datoreaz altor factori, neintrodui n modelul de regresie.
21
Coeficieni
Coeficieni
Model 2
Nestandardizai
standardizai
Beta
Eroarea
Sig.
8.459
.000
standard
Interceptul
109.161
12.904
forma hemofiliei
-1.675
2.240
-.067
-.748
.457
comportamente
.736
.161
.410
4.579
.000
sntate general
-.241
.074
-.331
-3.262
.002
funcionare fizic
.050
.061
.090
.822
.413
relaii sociale
-2.987
.999
-.271
-2.990
.004
obsesiv-compulsive
CONCLUZII
Concluziile ce s-au desprins n urma analizei datelor sunt urmtoarele:
Compararea scorurilor celor dou loturi a relevat diferene n aproape toate domeniile
calitii vieii n defavoarea hemofilicilor.
Cele mai afectate domenii ale calitii vieii hemofilicilor sunt: domeniului general al
sntii, mediul nconjurtor, domeniul psihologic, relaii sociale i n domeniul
spiritualitate/religie/convingeri personale (p< .001; p< .05). Aceast evaluare semnific
existena problemelor cu munca sau cu alte activiti zilnice, datorate sntii fizice i a
problemelor emoionale, interferena marcat i frecvent cu activitile sociale normale
datorit problemelor fizice sau emoionale. Hemofilicii lotului consider c sntatea lor
general este precar i c exist posibilitatea ca aceasta s se nruteasc n viitor.
Scorurile afectate ale acestor domenii influeneaz negativ calitatea vieii.
Resursele financiare ale hemofilicilor sunt profund afectate, deoarece o mic parte din
aduli sunt angajai. Accesibilitatea i calitatea serviciilor medicale sunt evaluate negativ, iar
posibilitile lor de recreere i de petrecere a timpului liber sunt semnificativ reduse.
De asemenea se remarc diferene semnificative ntre cele dou loturi ale studiului cu
privire la nivelul comportamentelor obsesiv-compulsive: F (1, 194)= 7,286, p < .01 (M= 9,67
versus M= 6,22). Lipsa factorilor de coagulare pune viaa pacientului hemofilicc n pericol,
23
una invers, ceea ce nseamn c activitatea zilnic scade semnificativ n condiiile n care
nivelul comportamentele de protecie, de asigurare este unul crescut.
Atunci cnd un membru al unei familii este diagnosticat cu hemofilie, att pacientul,
ct i cei apropiai i pun o serie de ntrebri:
Ce este hemofilia?
Ce a cauzat aceast boal?
Cte tipuri de hemofilie exist?
Care sunt complicaiile?
Care sunt metodele terapeutice?
Toate aceste informaii pot fi obinute prin ntrebri i discuii cu cadrele specializate
i/sau o documentare din surse adecvate: cri i reviste de specialitate, internet, etc.
n continuare, vom ncerca, rspunznd la aceste ntrebri, s realizm un microghid
de ngrijire psihosocial util att pacientului, ct i familiilor acestuia.
Hemoflia A este o boal genetic transmis de mam i manifestat doar la sexul
masculin. Este cea mai frecventa coagulopatie, cauza fiind cantitatea insuficient sau chiar
absena total a factorului de coagulare VIII din sange.
Atunci cnd:
-
II.
Informaii de specialitate
Un barbat hemofilic i o femeie sntoas vor avea biei clinic sntoi pentru c
mostenesc cromozomul X de la mam. n schimb, toate fetele vor fi purtatoare de boal,
pentru c motenesc cromozomul X de la tat. Acestea vor transmite la randul lor, ntr-un
procent de 50%, boala bieilor i n acelai procent, statutul de purttoare, fetelor. Este
25
posibil ca i familiile care nu au avut un istoric al bolii s fie afectate la un moment dat, prin
mutaii spontane ale genelor care codific informaia pentru factorul VIII. Defectele genice inserii, deleii, mutaii cu substituie de aminoacizi, determin prin amploarea lor severitatea
bolii.
III.
Clasificarea hemofiliei
IV.
Semne clinice
V.
Tratamentul medical
VI.
Deschideri psihosociale
parte medici specialiti din diverse domenii, psihologi, asisteni sociali. Dei acest lucru nu
este ntotdeauna posibil datorit resursele financiare limitate, totui profesionitii din
domeniul sntii ar trebui s fie contieni de toate problemele care pot s apar la pacienii
hemofilici [Barlow, et al., 2007; Beeton, et al., 2007; Bossard, et al., 2008; Bottos, et al.,
2007; Cassis, R.M.Y., 2004].
Alturi de problemele de ordin medical, pacienii cu hemofilie se confrunt cu
numeroase provocri de ordin social i economic. Iar aa cum se poate observa din acest
studiu, tratamentul medical nu este suficient pentru mbuntirea calitii vieii, sunt
necesare i interveniile pentru diferitele probleme psiho-sociale.
Tulburarea de sngerare este o afeciune cronic care impune limitri, dar poate duce
i la schimbri pozitive cum ar fi capacitatea de a nva i auto-contientia. Psihoterapiile,
terapiile alternative i serviciile sociale pot ajuta persoanele hemofilice s fac fa limitri.
Strategiile pentru a face fa fizic, mental, emoional i social trebuie s includ educaie
individual, consiliere familial, resurse, sprijin din partea comunitii, sprijin din partea
celorlali hemofilici, accesibilitate la tratament profilactic, produse de nlocuire a factorului
de coagulare, accesul la fizioterapie i chirurgie corectiv [idem].
S-a constat c accesibilitatea la tratamentul medical are un impact mare asupra
calitii vieii att pentru pacieni, ct i pentru familia acestuia. ntr-un studiu s-a remarcat c
cei care triesc n zonele unde consumul de factor de coagulare este ridicat calitatea vieii este
mai bun n comparaie cu cei care locuiesc ntr-o zon cu consum sczut de factor de
caogulare [Bullinger, Von Mackensen, 2004; Tedgard, 2008].
VII.
IX.
X.
30
BIBLIOGRAFIE
1. Aarason, N.K., Meyeravitz BE, Bard M., et al. (1991). Quality of life research in
oncology: past achievements and future priorities. Cancer, 67:839-843.
2. Abshire, T.C., Brackmann, H.H., Scharrer, I., et al. (2000). Sucrose formulated
recombinant human antihemophilic factor VIU is safe and efficacious for treatment of
hemophilia A in home therapy. Thromb Haemost, 38:811-816.
3. Abshire, T.C., Kenet, G. (2004). Recombinant factor Vila: review of efficacy, dosing
regimens and safety in patients with congenital and acquired factor VIII or IX
inhibitors. J Thromb Haemost, 2(6):899-909.
4. Achembac, T.M., McConaughy, S.H, Howell, C.T. (1987). Child/adolescent
behavioural and emotional problems: implication of cross-informnat correlation for
31
health questionnaire): an instrument for measuring clinical results. Med Clin Barc,
104: 772-776.
21. Anderson, G., Hussey, P.S. (2001). Comparing health system performance in OECD
countries. Health Aff, 20: 219-232.
22. Anderson, G., Kaplan, R.M., Bery, C.C. (1989). Interday reliability of function
assessment for a health status measure. The quality of well-being scale. Med Care
27: 1076-1083.
23. Anderson, J.P., Bushm J.W., Bery, C.C. (1988). Internal consistency analysis: a
method for studying the accuracy of function assement for health outcome and quality
of life evaluation. J Clin Epidemiol, 41:127-137.
24. Anderson, K.L. (1995). The effect of chronic obstructive pulmonary disease on
quality of life. Res Nurs Health, 18:547-556.
25. Anderson, K.L., Benditt, J.O., et al. (1996). Lung volume reduction surgery improves
global quality of life. Am J Respir Crit Care Med, 153:A426.
26. Andronescu, .G., Bulla O. (1987). Hemofilia. Bucureti, tiinific.
27. Antunes, S.V., Vicari, P., Cavalheirc, S., et al. (2003). Intracranial haemorrhage
among a population of haemophilic patients in Brazil. Haemophilia 9(5):573.
28. Apajasalo, M., Rautonen, J., Sintonen, H., et al. (1997). Health-related quality of life
after organ transplantation in childhood. Pediatr Transplant 1(2): 130-113.
29. Arranz, P., Remor, E., Quintana, M., et al. (2004). Development of a new diseasespecific quality of life questionnaire to adult s living with haemophilia. Haemophilia,
10(4):376-382.
30. Astermark, J., Petrini, P., Tengborn, L., et al. (1999). Primary prophylaxis in severe
hemophilia should be started early but can be individualized. Br J Haematol
105:1109-1113.
31. Athanasiu, A. (1983). Elemente de psihologie medical. Medical. Bucureti.
32. Bach, M., Bach, D. (1996). Alexithymia in somatoform disorder and somatic disease:
a comparative study. Psychother Psychosom.
33. Bagby, R.M., Parker, J.D.A., Taylor, G.J. (1994). The twenty-item Toronto
Alexithymia Scale-1. Item selection and cross-validation of the factor structure. J
Psychosom Res 38:2332.
34. Bagby, R.M., Taylor, G.J., Parker, J.D.A. (1994). The twenty-item Toronto
Alexithymia scaleII. Convergent, discriminant, and concurrent validity. Journal of
33
47. Berenbaum, H., Irvin, S. (1996). Alexithymia, anger, and interpersonal behavior.
Psychother Psychosom.
48. Bergner, M., Bobbitt, R.A., Carter, W.B., et al. (1981). The Sickness Impact Profile:
development and final revision of a health status measure. Med Care, 19:787-805.
49. Bernheim, J.L., (1999). How to get serious answers to the serious question: How have
you been? Subjective quality of
publics judgements to inform scales of health related quality of life. Soc Sci Med,
41:1447-1462.
63. Bowling, A. (1997). Measuring health: A review of quality of life measurement scale.
Milton Keynes, Open University Press.
64. Brazier, J.E., Harper, R., Munro, J., et al. (1999). Generic and condition-specific
outcome for people with osteoarthritis of the knee. Rheumatology, 38:870-877.
65. Bressi, C., Taylor, G., Parker, J., et al. (1996). Cross validation of the factor structure
of the 20-item Toronto Alexithymia Scale: an Italian multicenter study. J Psychosom
Res 41:551559.
66. Bullinger, M., Anderson, R., Cella, D., et al. (1993). Developing and evaluating
cross-cultural instruments from minimum requirements to optimal models. Qual Life
Res, 2:451-459.
67. Bullinger, M., Von Mackensen, S. (2004). Quality of life assessment in haemophilia.
Haemophilia, 10 Suppl 1, 9-16.
68. Bullinger, M., Von Mackensen, S., The Haemo- QoL Group. (2003). Quality of life in
children and families with bleeding disorders. J. Pediatr Hematol Oncol, Vol. 25,
Suppl. 1.
69. Bulloch, W., Fildes, P. (1911). Haemophilia. In: Treasury of Human Inheritage.
Cambridge University Press. London, 169-347.
70. Burcu, A. (2005). Factorii determinani ai calitii vieii. Argonaut. Cluj-Napoca.
71. Calman, K.C. (1985). Quality of life in cancer patients-a hypothesis. J med Ethics,
10:124-125.
72. Canclini, M., Saviolo-Negrin, N., Zanon, E., et al. (2003). Psychological aspects and
coping in haemophilic patients:a case-control study. Haemophilia, Sep; 9(5):619-24
73. Carol, S., Burckhardt, L., Anderson, B.A., Hgg, O. (2003). Health and Quality of
Life. Outcomes.
74. Carr, A.J., Gibson, B.A., et al. (2001). Is quality of life determined by expectations or
experience? BMJ, 322:1240-1243.
75. Carr, A.J., Higginson, I., J. (2001). Measuring quality of life: Are quality of life
measures patient centered?. British Medical Journal, 322, 13571360.
76. Casey, R.L., Brown, R.T. (2003). Psychological aspects of hematologic disease.
Child Adolesc Psychiatr Clin N Am, 12(3):567-84.
77. Cassis, R.M.Y. (2007). Psychosocial care for people with haemophilia. Treatment of
36
96. Elander, J., Barry, T. (2003). Analgesic use and pain coping among patients with
haemophilia. Haemophilia, 9(2):202-13.
97. Eldred, L,, Wu, A., Chaisson, R., Moore, R. (1998). Adherence to antiretroviral
pneumocystis prophylaxis in HIV disease. J Acquir Immune Defic Syndr, 18:117-125.
98. Enchescu, C. (2000). Tratat de psihopatologie. tiinific. Bucureti.
99. Engel, G.L. (1980). The clinical application of the biopsychosocial model. Am J
Psychiatry, 137:53554.
100. Epstein, M.H., Nelson, C.M., Polsgrove, L., Coutinho, M., et al. (1993).
Comprehensive Community-Based Aproach to Serving Students with Emotional and
Behavioral Disorders. Journal of Emotional and Behavioral Disorders, 1, 127133.
101. Feinstein, A.R. (1983). An additional basic science for clinical medicine: IV. The
development of clinimetrics. Ann Sci, 27(1):31-35
102. Feld, M., Ruegg, C.J. (2005). Head attak. Scientific American Mind, 16(2): 66-71.
103. Fitzpatrick, R., et al. (1992). Quality of life measure in health care. I: Applications
and issues in assessment. BMJ, 305:1074-1077.
104. Franz, A. (2008). Medicin psihosomatic. Trei. Bucureti.
105. Garret, J.W., et al., (1990). Health status in inflamatory bowel disease: Biological
and behavioral consideration. Gastroenterology, 99:91-93.
106. Geerts E., Van de Wiel, H., Tamminga, R. (2008). A pilot study on the effects of the
transition of paediatric to adult health care in patients with haemophilia and in their
parents: patient and parent worries, parental illness-related distress and healthrelated Quality of Life. Haemophilia, 14, 1007-1013.
107. Ghanizadeh, A., Baligh-Jahromi, P. (2009). Depression, anxiety and suicidal
behaviour in children and adolescents with haemophilia. Haemophilia, 15, 528-532.
108. Ghergu, A. (2001). Psihopedagogia persoanelor cu cerine speciale - Strategii de
educaie integrat. Polirom, Iai.
109. Ghergu, A. (2005). Sinteze de psihopedagogie special - Ghid pentru concursuri
i examene de obinerea gradelor didactice. Polirom, Iai.
110. Ghosh, K. (2004). Management of haemophilia and its complications in developing
countries. Clin Lab Haematol, 26(4):243-51.
111. Giangrande, P.L.F. (2003). A history of haemophilia. Haemophilia World, 10.
112. Gill, T.M., et al. (1994). A critical appraisal of the quality of life measurements. JAMA,
272:620-626.
38
113. Goodman, W.K., Price, L.H., Rasmussen, S.A., et al. (1989). The Yale-Brown
Obsessive Compulsive Scale, I: development, use, and reliability. Arch Gen
Psychiatry, 46:10061011.
114. Gotay, C.C., Korn, E.L., et al. (1992). Quality of life assessment in cancer
treatment protocols: research issues in protocol development. J Natl Cancer Inst,
84:545-579.
115. Greening, L., Stopelbein, L., Chandler, C.C., Elkin, T.D., (2005). Predictors of
Childrens and Adolescents Risk Perception. Journal of Pediatric Psychology, 30, 5,
425435.
116. Gregg-Smith, S.J., et al. (1993). Septic arthritis in haemophilia. J Bone Joint Surg,
75B:368-370.
117. Gregory, M., Boddington, P., Dimond, R., Atkinson, P., Clarke, A., Collins, P.
(2007). Communicating about haemophilia within the family: the importance of
context and of experience. Haemophilia, 13, 189-198.
118. Gringeri, A., Von Mackensen, S. (2008). Quality of life in haemophilia.
Haemophilia, 14 Suppl. 3, 19-25.
119.
Groot, J.M., Rodin, G., Olbsted, M.P. (1995). Alexithymia, depression, and
143. Janicke, D.M., Finney, J.W., (2000). Determinants of Childrens Primary Health
Care Use. Journal of Clinical Psychology in Medical Settings, 7, 2939.,
144. Jenkins C.D., et al. (1990). The mesuarements of health-related status quality of
life: major dimension identified by factor analysis. Soc Sci Med, 25-32.
145. Jenkinson, C., et al. (1993). The SF-36 Health Suvey Questionnaire. Auditorium,
2:7-12.
146. Jenney, M.E.M., Campbell, S. (1997). Measuring quality of life. Arch Dis Child,
77:347-350.
147. Jones, P. (2002). Living with haemophilia. Oxford University Press.
148. Joranson, D.E., Ryan, K.M., et al. (2000). Trends in medical use and abuse of
opioid analgesics, 283:1710-1714.
149. Jung H, et al. (2000). Comaparison of patientspreferences and evaluation
regarding aspects of general practice care. Fam Pract, 17:236-241.
150. Jung, H., et al. (1997). What makes a good general practitioner: do patients and
doctors heave different views? Br J Gen Pract, 47:805-809.
151. Kalinin, V.V., Zemlyanaya, A.A., Krylov, O.E., Zheleznova, E.V. (2010)
Handedness, alexithymia, and focus laterality as risk factors for psychiatric
comorbidity in patients with epilepsy. Epilepsy Behav.
152. Keller, A., Terrier, F., et al. (2002). Pelvic haemophilic pseudomotor: management
of a patients with high level of inhibitors. Skeletal Radiol, 31(9):550-552.
153. Kemnitz, S., Moens, P., et al. (2002). Avascular necrosis of the talus in children
with haemophilia. J Pediatr Ortop, 11 (1):73-77.
154. Keyes, L.M. (2007). Promoting and Protecting Mental Health as Flourishing: A
Complementary Strategy for Improving National Mental Health. American
Psychologist, 62, 2, 95108.
155. Kingston, H.M., (1995). Queen Victorias Gene: haemophilia and the royal
Family. BMJ, 311:1106-1107.
156. Kitchens, C.S. (1980). Occult hemophilia. Johns Hopkins Med J, 146:225-259.
157. Knitzer, J. (1993). Children's Mental Health Policy: Challenging the Future.
Journal of Emotional and Behavioral Disorders, 1, 816.
158. Kooiman, C.G. (1998). The status of alexithymia as a risk factor in medically
unexplained physical symptoms. Compr Psychiatry, 39(3):152-159.
159. Krystal, H (1988). Integration and Self Healing: Affect, Trauma, Alexithymia.
41
L.
(2008).
Asistena
social
persoanelor
cu
dizabiliti.
www.weebly.com/ 2010.
185. Manea, M., Manea, T. (2004). Psiologie Medical. Tehnic. Bucureti.
186. Mannucci, P.M., Ham-Wasserman Lecture (2002). Hemophilias and Releted
Bleedading Disorders: A story of Dismay and success. Haematology,1-9.
187. Mannucci, P.M., Tuddenham, E.G.D. (2001). The Hemophilias-From Royal Genes
to Gene Therapy. N Engl J Med, 344(23): 1773-1779.
188. Maryam, A., Tooyserkani, M.A.B., Sedigheh, K. (2011). The Moderating Role of
Positive and Negative Affects on the Relationship Between Alexithymia and
Experience of Pain in Chronic Pain Patients
217-226.
207. Nemiah, J.C. (1977). Alexithymia. Theoretical considerations. Psychother
Psychosom 28(1-4):199-206.
208. Nemiah, J.C., Freyberger, H., Sifneos, P.E. (1976). Alexithymia: a view of the
psychosomatic process. In: Hill OW, Modern trends in psychosomatic research, vol.
3. London, Buttersworth, 430-9.
209. Nemiah, J.C., Sifnoes, P.E. (1970). Psychosomatic illness and problem of
communication. Psychother Psychosom.
210. OBoyle, C.A., et al. (1992). Individual quality of life in patients undergoing hip
replacement. Lancet, 339:1088-1091.
211. Oancea, C. (2002). Tehnici de sftuire/consiliere. Vavila, Bucureti.
212. Oliver, A. (2003). Putting the quality-adjusted life years. Public Health Med, 25:812.
213. Olsson, C.A., Bond, L., Johnson, M.W., Forer, D.L., Boyce, M.F., Sawyer, S.M.
(2003). Adolescent chronic illness: a qualitative study of psychosocial adjustment.
Ann Acad Med Singapore, 32(1):43-50
214. Orley, J., Kuyken, W., ed al. (1994). Quality of life assessment: international
perspectives. Springer-Verlag, Berlin, 41-57.
215. Orley, J., Kuyken, W., et al. (1994). Quality of life assessment in health care
setttings. Springer Verlag. Heidelberg.
216. Otto, J.C. (1803). An account of an haemorrhagic disposition existing in certain
families. Med Repos, 6:1-4.
217. Paglierani, S. (2002). The Psychology of Mintal health and Illness-Dedining Health
and Illness more Accurately. online la http:// theemergencesite.com/ Tech/
Psychology-of-Mental-Health-Illness.htm.
218. Palomo, N.A.L., Alcoverro, F.O., Hurtado, R.G., Guerrero, P.A. (2003). Thought
disorders in obsessive-compulsive disorder. Actas Esp Psiquiatr 31:100102.
219. Parker, J., Taylor, G., Bagby, M. (2001). The Relationship Between Emotional
Intelligence and Alexithymia. Personality and Individual Differences, 30:107-115.
220. Parker, J.D., Bagby, R.M., Taylor, G.J. (1991) Alexithymia and depression: distinct
or overlapping constructs?. Compr Psychiatry 32: 387394.
221. Parker, J.D., Taylor, G.J., Bagby, R.M., Acklin, M.W. (1993). Alexithymia in panic
disorder and simple phobia: a comparative study. Am J Psychiatry 150:11051107.
45
222. Pearlman, R.A., et al., (1988). Quality of life in chronic diseas: perception of
elderly patients. J Gerontol, 43:25-30.
223. Penica, S., Williams, K.E. (2008). The use of a psychological intervention to
increase adherence during factor administration in a child with haemophilia.
Haemophilia, 14, 939-944.
224. Penka, M., Smejkal, P. (2009). Genetic and therapeutic risk factor for inhibitor.
Oral presentation. Octapharma inhibitor course, Bratislava.
225. Perquin, C.W., Hazebroek-Kampscheur, A.A.J.M., et al., (2001). Chronic pain
among children and adolescents: psysician consultation and medication use, 16:229335.
226. Pierre, U., et al., (1998). Proxy use of the Canadian SF-36 in rating health status of
the disabled elderly. J Clinic Epidemiol, 984-988.
227. Poenaru, D.V., erban, M., Branea, I.L. (2005) Artropatiile hemofilice. Bucureti,
Academia Romn.
228. Pollmann, H., Richter, H., et al. (1999). When are children diagnosed as having
severe haemophilia and when do they start to bleed? A 10-year single centre PUP
study. Eur J Pediatr, 158: S166-170.
229. Postel, J. (1998). Dicionar de psihiatrie i de psihopatologie clinic. Univers
Enciclopedic, Bucureti.
230. Predescu, V. (1998). Psihiatrie. Medical. Bucureti, vol. I.
231. Provorotov, V.M., et al. (2009). Alexithymia. http://nature.web.ru.
232. Rachman, S., Hodgson, R. (1980). Obsession and compulsions. Prentice-Hall:
Englewood Cliffs.
233. Rapport pour le SPF, 2004.
234. Rcanu, R. (2001). Psihologia sntii: de la credine i explicaii la sistemul de
promovare al ei. Iai. Polirom.
235. Rcanu, R. (2007). Introducere n psihodiagnoz clinic. Bucureti. Universitatea.
236. Rodak, M.S., Bernadette, F. (2002). Hematology Clinical Principle and
Applications. W.B. Saunders company, chapter 43,632-642.
237. Rodriguez-Merchan, E.C., (1996). Effects of haemophilia on articulation of
children and adults. Clin Orthopedics Related Res, 328:7-13.
238. Roghaye, A., Mirjalili, M.A.B., Sedigheh, K. (2011). The Moderating Role of SelfEfficacy on the Relationship Between Alexithymia and Severity of Pain in Chronic
46
Pain Patients, Original Research Article Procedia - Social and Behavioral Sciences,
Volume 30:149-153.
239. Roosendaal, F.R., et al., (1990). Modern haemophilia treatement: medical
improvments and quality of life. J Intern Med, 228:635-640.
240.
Roosendaal, G., et al. (2000). Articular cartilage is more susceptible to blod induced
269. Sullivan, M., et al., (1995). The Swedish SF-36 health survey. Soc Sci Med,
41:1349-1358.
270. Szucs, T.D., et al., (1996). Socioeconomic impact of haemophilia care: results of a
pilot study. Haemophilia, 2:211-217.
271. chiopu, U. (coord.). (1997). Dicionar de psihologie. Babel. Bucureti.
272. erban, M., Ioni, H., Poenaru, D.V. (2011). Ghid de tratament musculo-scheletal
n hemofilie. Timioara. Brumar.
273. erban, M., Barna, L., Lctuu, A., Malia, I., erban, V. (2006). Hemofilia pe
nelesul tuturor. Timioara. Brumar.
274. erban, M., Mihailov, D., Lctuu, A., et al. (2009). Multicentric Evaluation of
health quality of life in romanian haemophiliacs. Boston, Poster.
275. erban, M., Schramm, W. (2001). Hemostazeologie clinic. Timioara. Brumar.
276. Tariq, S.M., et al. (2001). Comprehensive haemophilia care in developing countries.
Ferozsons.
277. Taylor, G.J. (1977). Alexithymia and counter-transference. Psychother Psychosom,
28: 141-7.
278. Taylor, G.J. (1984). Alexithymia: concept, measurement, and implications for
treatment. Am J Psychiat.
279. Taylor, G.J., Bagby, R., et al. (1997). Disorders of Affect Regulation: Alexithymia
in Medical and Psychiatric Illness. Cambridge: Cambridge University Press.
280. Taylor, G.J., Bagby, R.M. (2004). New trends in alexithymia research. Psychother
Psychosom, 73:6877.
281. Taylor, G.J., Bagby, R.M., Luminet, O. (2000). Assessment of alexithymia: selfreport and observerrated measures. In: Bar-On R, Parker JDA (eds). The handbook
of emotional intelligence: theory, development, assessment, and application at home,
school, and in the workplace. San Francisco: Jossey-Bass/Pfeiffer; 301-19.
282. Taylor, G.J., Bagby, R.M., Parker, J.D. (1991). The alexithymia construct. A
potential paradigm for psychosomatic medicine. Psychosomatics, 32(2):153-164.
283. Taylor, G.J., Parker, J.D., Bagby, R.M., Acklin, M.W. (1992). Alexithymia and
somatic complaints in psychiatric outpatients. J Psychosom Res 36:18.
284. Taylor, G.J., Taylor, H.S. (1997). Alexithymia. In M. McCallum, W.E. Piper (Eds.)
Psychological mindedness: A contemporary understanding. Munich: Lawrence
Erlbaum Associates.
49
285. Tedgard, U., Ljung, R., Bullinger, M., Von Mackensen, S. (2008). Quality of life in
haemophilia carriers preliminary results of the ESCHQoL study. Session B-2.5:
Multidisciplinary free papers 01. 29 FP 05. Haemophilia.
286. Teitel, J.M., et al., (2004). Home management of haemophilia. Haemophilia,
10:118-133.
287. TenHouten, W.D., Hoppe, K.D., Bogen, J.E., Walter, D.O. (1985). Alexithymia
and the split brain. IV. Gottschalk-Gleser content analysis, an overview. Psychother
Psychosom.
288. Terry, P., Humphreys, L.M., Wood, J.D.A., (2009). Alexithymia and satisfaction in
intimate relationships. Personality and Individual Differences, Volume 46, Issue 1,
Pages 43-47.
289. Testa, M.A., Simonson, D.C. (1996). Assessment of quality of life outcomes. N
Engl J Med, 334:835-840.
290. Thailer, S.A., Friedman, R., Harshfield, G.A., Pickering, T.G. (1985). Psychologic
differences between high, normal-, and low-renin hypertensives. Psychosom Med.
291. Thapar, A., Holmes, J., et al. (1999). Genetic basis of attention deficit and
hyperactivity. Br J Psychiatry, 174:105-111.
292. The Flanagan Quality of Life Scale: Evidence of Construct Validity
293. The WHOQoL Group, (1994). The development of the World Health Organization
Quality of Life Assement Instrument. Heildleberg: Springer-Verlag. Qual Life, 2:153158.
294. Tizzano, E.F., Cornet, M., Domenech, M., Baiget, M. (2002). Modifier genes in
haemophilia: Their expansion in the human genome. Haemophilia, 8:250-254.
295. Todarello, O., Casamassima, A., Marinaccio, M. et al. (1984). Alexithymia,
immunity and cervical intraepithelial neoplasia: a pilot study. Psychother Psychosom.
296. Todarello, O., Taylor, G.J., Parker, J.D., Fanelli, M. (1995). Alexithymia in
essential hypertensive and psychiatric outpatients: a comparative study. J Psychosom
Res.
297. Trippoli, S., et al., (2001). Multivariate analysis of factors infuencing quality of life
and utility in patients with haemophilia. Haematologica, 86:722-727.
298. Troop, N.A., Schmidt, U.H., Treasure, J.L. (1995). Feelings and fantasy in eating
disorders: a factor analysis of the Toronto Alexithymia Scale. Int J Eat Disord.
299. Trzepacz, A.M., Vannata, K., Davies, W.H., Stehbens, J.A., Noll, R.B. (2003).
50
Psychosom.
311. Watson, D., Suls, J., Haig, J.. (2002). Global self-esteem in relation to structural
models of personality and affectivity. J Pers Soc Psychol, 83(1):185-197.
312. White, G., Shapiro A., et al. (1998). Clinical evaluation of recombinant factor IX.
Semin Hematol, 35:33-38.
313. White, G.C., Rosendaal, F., Aledort, L,M., et al. (2001). Definitions in hemophilia:
recommendation of the scietific subcommittee on factor VIII and factor IX of the
51