HK J Paediatr (new series) 2008;13:239-252

Psychological Vulnerability and Resilience in Children andAdolescents with Thalassaemia Major

ASM FUNG, LCK LOW, SY HA, PWH LEE

Abstract Objectives: Chronic childhood illness may be a risk factor for psychosocial or psychiatric disturbances.Yet, children with chronic illnesses may also show resilience and active coping with varying degrees ofsuccess or failure. The present study aims to outline the patterns of coping and adjustment of patientswith thalassaemia major, and identify specific developmental issues associated with living with the chronicillness. Method: Seventy-three patients receiving treatments at Queen Mary Hospital were assessed witha set of semi-structured interview and questionnaires instruments covering the children's perceptions oftheir quality of life, illness and treatment. Their psychological adjustment was assessed through measureson life satisfaction, the extent and nature to which they felt they had been adversely affected by the illnessand treatment, and their views of the future and of themselves. Results: The patients had multiple concernsand dissatisfactions with their illness, treatments, and health status, but many of their concerns were alsocommonly noted in normal healthy children. Resilience is demonstrated in a proportion of patients. Therewere clear developmental trends showing that patients had changing concerns over different stages oftheir development. Older patients had more adverse impact as chronicity and mental exhaustion in copingwith the illness seemed to be an important factor affecting adjustment. Conclusions: The results indicatethe need for psychosocial interventions, especially for the older patients with thalassaemia major.

Key words Adjustment; Coping; Developmental patterns; Resilience; Thalassaemia major

Department of Clinical Psychology, Queen Mary Hospital,102 Pokfulam Road, Hong Kong, China

ASM FUNG BSocSc, MSocSc(Clin Psy), PhD

Department of Paediatrics and Adolescent Medicine,The University of Hong Kong, Queen Mary Hospital,102 Pokfulam Road, Hong Kong, China

LCK LOW MBChB, FHKAM(Paed)SY HA MBBS, FHKAM(Paed), FHKAM(Path)

Department of Psychiatry, The University of Hong Kong,Queen Mary Hospital, 102 Pokfulam Road, Hong Kong,China

PWH LEE BSocSc, MSocSc(Clin Psy), PhD

Correspondence to: Dr ASM FUNG

Received May 23, 2008

Introduction

Children living an extended life with a chronic physicalillness have to face up a lifetime of medical treatments anddaily reminders of vulnerability and dependency.1 A child'sreactions to the treatment demands often vary dependingon his/her stage of cognitive, emotional and social maturity.Diminished quality of life, uncertainties over the entirelifespan, and disruptions to the family system associatedwith chronic illnesses are legitimate areas of professionalconcern.2

Thalassaemia major or Cooley's anaemia is an inheriteddisorder characterised by absent or decreased productionof one of the normal globin chains of haemoglobin in thered blood cells. Three point four and five percent of theHong Kong population are carriers of beta-thalassaemias

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and alpha-thalassaemias respectively.3 It is estimate that 400patients suffer from thalassaemia major in Hong Kong.

Developmental Implications of Having ThalassaemiaAn infant with thalassaemia major appears normal at

birth. Signs of the disease develop a few months afterwardsand become progressively severe. Pallor, irritability, poorappetite, and failure to thrive are generally noted at the endof the first year. Subnormal growth rates for height, weight,and skeletal maturation are common especially inadolescence. Disorders of puberty are common in bothmales and females but normal pubertal development ispossible in optimally treated children.4

With improved treatment and extended life expectancy,the stresses and hardships involved in survivingthalassaemia are increasingly recognised. Adolescent andadult patients face difficulties which early sufferers of theillness did not have as the latter may have perished withprevious less advanced medical interventions. Difficultiesinvolving education and career development, heterosexualrelationships, and forming one's own family have to be dealtwith. Hilgartner et al5 studied 72 thalassaemia subjects andtheir families and reported that these adolescent and youngadults expressed great concerns over their uncertainprognosis, the genetic transmission, fertility, and bodyimage. Low self-esteem and fear of dependency were alsocommon.5

The tasks of health care providers are different from thatof two or three decades ago. A multifactorial developmentalframework is advocated6-8 as the impact of chronic illnesson children and their families are not uniform and notnecessarily adverse, and a complex array of factors are ofteninvolved in determining the final outcomes. Children aredeveloping and have different needs and capacitiesdepending on the stage of their development. The level ofcognitive and emotional development, comprehension ofillness and medical procedures, stress and coping capacities,parent-child relationships, family homeostasis, and themeanings attached to the illness may all have an impact onthe ultimate adjustment of the child patient concerned.9,10

Developmental IssuesThe nature and extent of psychological and emotional

difficulties precipitated by the illness often varies dependingon the age and developmental level of the afflicted child.11

During infancy and pre-school years, the child patient maynot be able to understand the seriousness of the illness, orto anticipate the long-term implications of the illness andtreatment. Treatment issues are often handled by the parents.

A common difficulty at this stage involves ensuring thechild's compliance and increasing his/her tolerance to thedistressing procedures. Confrontations between parent andchild over day to day care-taking tasks are common. Inthalassaemia patients, the cumbersome daily subcutaneouschelation may pose a particular problem. During early andmiddle school years, academic performance and peerinfluence become prominent. Hospitalisations disruptschool attendance and lead to poor academic achievement,social estrangement, and strained peer relationships. Duringschool age, given comparisons with peers, sick childrenmay be forced to acknowledge differences betweenthemselves and others, sometimes with deleteriousconsequences on their self-esteem and personalitydevelopment. As poorly controlled thalassaemia oftendistorts facial appearance and brings about stunted growth,it is not uncommon for these children to be singled out.The adolescent patient gradually takes on moreresponsibility for his/her self-care so that parents need onlyprovide occasional surveillance and reminders. Adolescentsand young adults have to slowly yet painfully come to termswith real and imagined restrictions in work or socialopportunities, reduced fertility and a limited life-expectancy. Further frustrations await the maturing adultas he/she has to face squarely with thwarted aspirationsand demands, against a background of compromisedphysical ability and limited life opportunities.

Family InfluencesThe influence of the family may be critical across all

stages of the illness. A major illness in a child precipitatesa crisis in the family.6 The chronic illness may disrupt theentire fabric of a family,12 as pre-existing hopes andaspirations have to be relinquished, in place of which areuncertainties, painful anticipations of restrictions andreduced potential for the child, as well as adaptations tomultiple changes in routine and habits.13 Given the oftenunpredictable and fluctuating course of thalassaemia,family members may be driven into a state of constantapprehension. Some families harbor a pronounced thoughcovert expectation that things may get worse as time goesby. It has also been reported that some families may becomesocially isolated as a result of the child's illness.14-16

Yet, a chronic illness may strengthen family unity andadaptation. Pless and Satterwhite17 noted that parents ofchronically ill children felt that the illness served as aunifying force in the family so much so that sensitivity offamily members to each other was enhanced. Markovaet al18 noted in their study of parents of haemophiliac

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children that half of the parents felt that the illness hadpromoted the family's solidarity. It appears that the family'scapacity to perceive meaning in the chronic illnessexperience may be an important mediator of the child'smental and physical health.

Aims of the Study

While a developmental understanding of the differingneeds of the chronically ill child has been documented to acertain extent, there is a dearth of systematic informationavailable on the developmental needs of patients withthalassaemia from their childhood to young adulthoodyears. The present study aimed to map out patterns of copingand adjustment to thalassaemia, and identify specificdevelopmental issues faced by these patients. The resultsobtained may be clinically useful in informing andimproving service provisions in the long-term care of thepatients. The aims of the study were:1. To identify the developmental changes in needs,

perceptions, and challenges faced by patients withthalassaemia;

2. To identify the nature of the coping processes engagedby patients with thalassaemia at different stages of theirdevelopment.

Patients and Method

Our study was designed to look into the developmentalissues confronted by the entire population of thalassaemiapatients who were being followed-up at Queen MaryHospital, a university teaching hospital in the southern partof Hong Kong. The hospital is by far the most establishedgeneral hospital on Hong Kong Island, and treats themajority of patients with thalassaemia who resides in HongKong. A comprehensive assessment of the patients'psychosocial and family functioning was included.

SubjectsPatients with thalassaemia major receiving treatment at

Queen Mary Hospital were included. The subjects weremanaged by the Department of Paediatrics and AdolescentMedicine and the Department of Medicine. A total of 73subjects were included. Consent was obtained from boththe parents and the subjects themselves, who were briefedon the aims of the study, and were provided with time andopportunity to ask questions about the study. No subject or

parent refused to participate. The study was approvedby the Ethics Committee of the Faculty of Medicine, theUniversity of Hong Kong. All subjects were assessed ona standard set of semi-structured interview andquestionnaire instruments. The subjects were assessedwhen they were admitted to the hospital for monthlyblood transfusions.

MeasuresInstead of adopting a simple questionnaire measure

approach, the authors adopted an in-depth interviewapproach so that relevant issues could be adequately coveredand collection of quality data better assured throughestablishing a trusting rapport with all the subjects. A semi-structured interview schedule as well as a set of standardquestionnaires were used in the study and administeredthrough a clinical assessment interview. Both patients andtheir parents were interviewed but assessed separately. Thepresent paper involves only the use of the semi-structuredinterview schedule on the patients. The assessment schedulemay be obtained from the first author.

The semi-structured interview schedule was designedto obtain both qualitative and quantitative data, and assessa number of areas pertinent to the subjects' perceived qualityof life and psychological adjustment. Assessments coveredthe subjects' current concerns, views of their illness andtreatment, self-perceptions and their wishes for the future.

The interview format included the use of a consistentnumber of open ended prompts. An initial free responsewas allowed on most questions, and subjects wereencouraged to express their ideas and thoughts freely inrelation to the specific areas assessed. Standard probes intospecific areas were administered subsequently, and subjectswere asked to rate their responses on a 3-point scaleindicating "mild" through "moderate" to "very much so".When appropriate, a 4-point scale was used with "not atall/not applicable" added as a possible response.

Each subject was also administered two selected verbalsubtests of an intelligence test commonly used locally (theWechsler scales) to obtain a prorated verbal IQ as a quickindicator of their intelligence. The assessment with eachsubject took about an hour.

Data AnalysesTo arrive at an understanding of the developmental

patterns in the subjects' adjustment, the subjects weredivided into four groups. These are: the preschool group(aged under 6 years and not yet admitted in primary school);the primary school group (aged between 6 and 12 years

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and attending primary school); the secondary school group(aged between 12 to 18 years and receiving secondaryschool education); and the young adult group (over 18 yearsold and working). As the preschool group was unable torespond meaningfully to the interview schedule, theirparents were interviewed separately. For the purpose of thispaper, only the results obtained by the last three groups ofpatients and only descriptive, qualitative data were analysedand reported here.

On questions where subjects were asked to make ratings(e.g. their levels of concern about and the degree to whichthey feel stressed by the illness stresses; their level oftoleration of treatment etc.), the averages of those rated as2 or above were reported and discussed. The socioeconomicstatus of the family was classified according to the methoddevised by Lee et al19 in their study on the development ofa language screening scale for Hong Kong Chinese children.This was done by summing the separate scores assigned tomother's education and father's occupation.

Results

I. Subject CharacteristicsThe subject sample consists of 42 females and 31 males.

The mean age of the subjects was 15.42 years (SD: 7.4years, range: 30 months to 35 years old). Nine subjectswere aged below 6 and attending kindergarten, 14 were inprimary school and 23 were in secondary schools. Twosubjects had completed secondary school and were

receiving vocational training. Twenty-five young adultsubjects were included who were either working or seekingemployment. The average number of years of schoolattendance was 8.42. One subject was married. Most ofthe working subjects were engaged in clerical jobs. Theaverage monthly income is $5,300 with a standard deviationof $3,671. The mean age of diagnosis of the illness was6.18 months (SD: 4.23). The average prorated verbal IQwas 92.32 (SD: 17.44), which is not very different fromthe IQ scores obtained among children and young adultwith thalassaemias in studies done elsewhere.20,21

II. Developmental PatternsA. Concerns

The patterns of self-volunteered and prompted concernswere analysed. Subjects in the primary school groupvolunteered more worries relating to issues of immediateconcerns, such as school work, health and treatment.Subjects in the secondary school group had more concernsabout health and school, while those in the young adultgroup expressed more concerns related to the future, healthand family-related affairs. Consistently, both self-volunteered and prompted concerns were most frequentlyrelated to the illness condition (Figure 1).

The patients also indicated being most concerned withphysical deterioration and such treatment procedures asblood transfusion and chelation. In particular, the youngadult subjects expressed more concerns over treatment, theirphysical vulnerability, and possibility of their impendingmortality (Figure 2).

Figure 1 Self-volunteered current concerns.

Adjusting to Thalassaemia Major243

B. Illness Related StressesSubjects' self-volunteered illness stresses include

treatment covering chelation, hospitalisation andcomplications as well as concerns over own health and thehealth of significant others (Figure 3). When prompted, thesubjects reported being most stressed by pain, boredom andgeneral fatigue (Figure 4). When asked to rate theirtoleration of such treatment procedures as chelation,venipuncture, hospitalisation, and blood transfusion, theresults indicated that regardless of age groups, chelationwas regarded as most intolerable. Similar findings have beenreported in a study involving patients in the United Kingdomand Cyprus and nearly 50% of the patients reported thatthey disliked chelation.22 Toleration of various aspects ofthe treatment also deteriorated with age (Figure 5). Theyounger subjects regarded chelation as bothersomeprimarily because of its accompanying discomfort. Theolder subjects, however, rejected chelation mostly becauseof its effects on limiting their life activities.

Most subjects indicated that their academic and workperformance had been adversely affected by thalassaemia.In addition, their leisure activities, mobility, self-care, andrelationships with others were also reported to be hindered.Disrupted school performance, difficulty in seeking a jobin the future, being a burden to the family, and inability toenjoy good physical health and good personal developmentin the future were also common issues mentioned. A clear

developmental trend was noted in that the older the subjects,the more handicaps and hindrances were reported (Figures6 & 7).

C. Positive Aspects of the IllnessNot all subjects were totally negative about their illness.

The majority of the subjects somehow managed to reportsome positive aspects about their illness. The youngersubjects valued having more secondary gains (e.g. of beingloved and attended to, being more easily allowed to havetheir way at home, being treated more nicely etc.) andhaving increased chances for knowing friends who wereco-patients. Interestingly, the older subjects valued havingbetter medical knowledge as a result of the illness and alsolearning to see things in a wider perspective and being moreappreciative of others' points of view (Figure 8).

D. Self-perceptions and Views Towards the FutureAn average of 10% and 12% of the subjects could not

think of any personal strengths and weaknesses respectively.Most of those who could do so consider their strengths asbeing their social skills, their personality characteristics(e.g. being more caring) and their achievement (e.g. havinggood school results, being good at a particular skill likedrawing, sports, etc.), while their weaknesses relate to theiremotional characteristics (e.g. not cheerful, moody),personality (e.g. being passive and introverted, diffident,

Figure 2 Prompted current concerns.

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Figure 3 Self-volunteered illness stress.

Figure 4 Prompted illness stress.

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Figure 6 Handicaps.

Figure 5 Degree of toleration of different aspects of treatment.

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Figure 8 Positive things about the illness.

Figure 7 Handrances.

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impatient etc.), their having an illness and their socialinteractions. In a way, the patterns and strengths noted alsoindicate the important areas of life concerns of the subjects,being social acceptance, personal character, as well aspersonal resources for ensuring a better life. It is noted thatyounger subjects emphasized their strengths andweaknesses in terms of achievements, while older subjectsplaced more emphasis on personality characteristics andsocial relationships (Figures 9 & 10).

Regarding subjects' wishes and aspirations, the resultsindicated that most subjects expressed wishes with themesrelated to their own illness and their family members' well-being. While younger subjects expressed wishes for betterschool performance and achievement, the older subjectgroups expressed more wishes related to future developmentand career opportunities. Younger subjects aspired moretowards idealistic goals. Older subjects, on the other hand,were clearly more down to earth, with their aspirations beingmore practical and real life oriented (Figures 11 & 12).

E. CopingThe coping strategies used by the subjects were

categorised according to the conceptual scales of coping

described by Carver, Scheier and Weintraub.23 The mostcommonly used strategies were active coping, mentaldisengagement, acceptance and positive reinterpretation.Developmentally, the younger subjects preferred moreactive forms of coping and a stronger reliance on socialsupport. Older subjects tended to use more cognitivestrategies, e.g. positive thinking and mental disengagement( Figure 13).

Discussion

Differences in needs, perceptions and coping patternsof subjects with thalassaemia across their developmentalstages were evidenced. Younger subjects gave fewerspontaneous responses. Understandably, they were less ableto express their concerns, as their self-reflective abilitiesare less developed than their older counterparts. Withgrowing maturity, there was a distinct shift of concerns froma focus on the immediate environment and oneself to thefuture and the larger social environment. The ability to de-center, become less egocentric, and think in abstract termsdevelops with maturity.24 As subjects grow older, it is

Figure 9 Self-perception of strengths.

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Figure 11 Three wishes.

Figure 10 Self-perception of weaknesses.

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Figure 12 Aspirations.

Figure 13 Coping strategies.

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inevitable that they should become more in touch withreality issues such as their own personal development, socialacceptance, financial independence and well-being, andwhat might hold for them in the future.

Illness related concerns relating to reduced lifeexpectancy, anticipated illness complications, and lack ofcure, were obvious in all subject groups but were moreprominent with increasing age. Illness and physicalvulnerability concerns also extended to worries for familymembers and friends. Older subjects, probably as a resultof prolonged adverse personal and vicarious experienceswith chronicity, consistently reported being more stressedand strained by their illness, and showed the least tolerancefor various medical treatments. Older subjects were alsomore conscious of others' views and discriminations. Theywere also more self-conscious of their adversely affectedphysical appearance, and expressed frustrations towardstheir limited job opportunities which they attributed to theiroutward appearance. They also considered that the illnesshad negatively affected their academic performance andtheir heterosexual relationships. They were highly diffidentrelating to the opposite sex because of their delayed sexualdevelopment, awkward physical appearance and thehereditary nature of the illness. Older subjects were alsoclearly noted to be more concerned about being a burdento the family. They were more vigilant of their illnessprogression as well as potential health hazards that theirillness and treatment may bring. The growing awarenessof death also emerged as a key issue with increasing age.

Differences in cognitive abilities, knowledge aboutmedical procedures and social maturity may account forsome of the developmental differences. The older subjects'increased understanding and awareness of health andillness-related issues may be attributable to increasedsensitization and a heightened sense of vulnerabilitydeveloping through the long years of having thalassaemia.Reality constraints, as well as the subjects' notabledifferences from their "normal" peers also prompted a senseof inferiority and self-consciousness. In addition, chronicityper se would have exerted more stress and strain on anyoneforced to bear with thalassaemia simply as a function ofnumber of years of having the illness burden. In this respect,older subjects were clearly worst off than their youngercounterparts. With advancing time, there seemed to be lessdifferentiation in terms of what is more or less acceptableto the patients. Anything illness-related seemed to haveacquired a conditioned aversive impact. It may also be truethat fewer of the older subjects may have benefited fromthe more advanced and less aversive treatment regimens

available nowadays.Therefore, with better treatment, less stress and strain

from the illness, and a less matured cognitive development,younger subjects apparently harboured a more optimisticattitude towards their illness and its treatment. The adventof hypertransfusion regime, continuous improvements inthe efficacy of filters during blood transfusion, and promiseof bone marrow transplantation may together have broughtabout more positive and enhanced tolerance of the varioustreatment procedures in the younger populations as well astheir parents. The younger subjects showed higheraspirations, more robust self-confidence, and a lesstarnished self-esteem compared to their older counterparts.With the advent of oral chelators, the burden of illness onthe overall functioning of the subjects is expected to befurther modulated or reduced in the years to come.

The findings highlighted a more intense need forsupportive psychosocial interventions with the older groupof subjects with thalassaemia, as over time, they would haveshouldered the bulk of adverse influences from their illnesscompared to younger subjects. To ensure that they continueto comply with treatment as they grow older, betterpsychosocial support not only from their natural socialcircles but also from the treatment team is important.22

It is interesting to note that hospitalisation was notparticularly rejected by the majority of subjects in the twoschool age groups. A possible interpretation may be thatschool age subjects took hospitalisation as a readilyavailable, temporary refuge from the pressures of schoolwork and peer pressure. In contrast, being more in touchwith reality issues, older subjects resented routinehospitalisation because of its disruptive effects on theirwork. Routine hospitalisation also exposed subjects tohigher risk of having to reveal their health status tocolleagues and employers. The potential bias anddiscrimination towards chronically ill individualsunfortunately may still be rampant in the real world in whichour subjects find themselves.

Our findings also indicate that the developmental themesnoted in our subjects seem to echo those of normalteenagers. The same struggles with developing a sense ofidentity, self-esteem, as well as the ambivalence towardsdependency issues were obvious. Consistent trendsassociated with the subjects' developmental level wereevident in the wishes volunteered. Younger subjects wishedfor better school performance and to have more possessions.Adolescent subjects, on the other hand, expressed wishesrelated to the social realm, their family, friends, and theoutside world. They were also more concerned with their

Adjusting to Thalassaemia Major251

future development, health, job prospects, heterosexualrelationships and potential for having their own families.While their concerns were in line with the usual taskscommonly seen in adolescents, our subjects seemed to feelsuch issues as being more pressurising because of thehindrances brought about by the illness. Thalassaemia, apartfrom its adverse influences on physical status andfunctioning, clearly exerted additional burdens on youngpatients in their process of negotiating their owndevelopmental hurdles.

Confronted with a life long illness, the subjects'adjustment and coping strategies varied with differentdevelopmental stages. Younger subjects were more actionoriented with a stronger reliance on seeking help fromothers. However, older subjects, burdened with years oftreatment related hassles as well as being more aware ofthe longer-term implications of thalassaemia, seemed tofind positive actions no longer appropriate in mostcircumstances. Instead, they tried to cope with their negativemood by adopting more positive thinking. They alsoactively tried to avoid thinking about unresolved issues, orof the negative but unavoidable hardships associated withthe illness. These differing emphases in coping strategiesmay reflect differences in the nature of problemsencountered across different age groups. As problemsassociated with increasing age become less likely to beamenable to total resolution − more cognitive and emotionalcoping tactics are necessary and evidenced. This finding isconsistent with findings in the literature concerning changeof coping strategies associated with age. As active copingmay predominate when constructive actions are possible,emotion-focused coping takes precedent when stressors hadto be endured instead of gotten rid of.25 Thus, when therealistic implications of a chronic illness sink in over time,the subjects may gradually adopt emotion-focused copingstrategies in place of more direct, active coping methods.

It was also noteworthy to mention that despite the long-term care required, there were few complaints ordissatisfaction expressed towards members of the healthcare team. This may be due to the highly personalisedservices provided. Subjects were routinely given'preferential' treatments in that they were admitted into thesame ward with familiar and friendly ward staff. Priorarrangements were also routinely made for patients ofsimilar age to be admitted on the same day so that theycould establish a better social network and comradeshipwith fellow patients. Flexible arrangements for admissionsalso helped subjects cater better to their school and workconstraints on the patients. Given the finding that patients'

level of satisfaction with medical care is important inaffecting ultimate adjustment,22,26-30 such flexibility andconsiderations in service provisions are clearly worthemulating.

Likewise, despite the long-term dependency on familymembers, it was reassuring to note the relatively fewcomplaints against family members. In fact, much of thewishes of the subjects were for the well-being of their ownfamily members. Despite that a major illness in a child mayprecipitate a crisis in the family6 and disrupt the very fabricof a family,12 it was apparent from our data that a chronicillness like thalassaemia may, on the contrary, strengthenfamily unity and adaptation. Pless and Satterwhite17 notedthat parents of chronically ill children reported that theirillness served as a unifying force in the family such thatfamily members' sensitivity to each other was enhanced. Itappeared that the family's capacity to perceive meaning inthe chronic illness experience may also be an importantmediator of the child's mental and physical health. Oursubjects' responses were clearly evident of their sense ofgratitude and well wishes towards family members.

Summary and Conclusions

The findings indicated that all subjects assessedexpressed varying concerns and dissatisfactions towardstheir illness, health and treatment. Nevertheless, the adverseimpact of the illness was not all encompassing, and mostsubjects had a life and focus outside of their illnessregardless of age. It was also noted that most subjects sharedsimilar concerns as their healthy counterparts of the sameage.

Clear developmental trends were noted in thatolder subjects had a greater range of concerns whichencompassed physical appearance, emotional well-being,and issues related to work and heterosexual relationships.They were also more socially-oriented and less egocentricwith more concern for others, and also more concerns abouthow others view or evaluate them. They had worries abouttheir illness, and were less tolerant of their illness andtreatments. They harboured the most intense resentmenttowards their illness as they regarded themselves as havingsuffered adverse consequences not only in the physicalbut also psychosocial realms. They felt that theircompetitiveness in choice of jobs, career development andheterosexual relationships were hampered by their physicaldevelopment and scholastic achievement. The prospect ofhaving a family of their own was regarded as poor because

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of insecurity feelings towards their own physical andfinancial well-being. They therefore regarded their futureas being bleak.

Yet, against the harsh realities of current and futurethreats, most subjects' view of themselves, the illness, andtheir future were not uniformly negative. The adaptivenessin learning to see things with a more balanced perspective,with negative as well as positive foci was evident in somesubjects, who demonstrated that resilience amidst a lifelongillness was not unreachable.

Admittedly, the study has its limitations, one of whichstems from the fact that only patients with thalassaemiamajor seen in one hospital were included. While there isthe inevitable concern about the generalisability of the dataobtained from a single center study to the entire group ofpatients in the territory, the findings of the study are at leastuseful as a guide for future study and development ofpsychosocial interventions for patients with thalassaemiaacross the different age groups. The focus of psychosocialinterventions clearly needs to be flexible, child-centred, andin line with the specific developmental issues and concernsof the children. Adolescent patients seem to have a greaterneed for psychosocial interventions given their more intensereactions towards the illness, the chronicity of theirafflictions, and the need to struggle for a better sense ofindependence and security.

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