Parents are important primary caregivers as they are the keyindividuals who take on the crucial role of caring for children withthalassemia.1 Thalassemia is a severe chronic inherited disorder of globinsynthesis in which the production of globin chains is partially orcompletely suppressed. This abnormality requires long-term treatment andsignificant treatments for thalassemia are blood transfusions and ironchelating therapies. Children with thalassemia encounter the impacts fromthis inherited disease and its treatment and they need to continue to receivecare throughout their childhood from their parents.2,3 In addition, parentsencounter multiple challenges that affect their quality of life when caringfor their children with thalassemia.4,5 In fact, the child’s quality of lifedepends largely on the corresponding parents’ quality of life. Therefore, ifparents are overwhelmed by caring for their children, without beingprovided the support system necessary to solve their problems, this in turncan also adversely affect the quality of life for children with thalassemia.
The empirical studies searched from January 1st, 2009 to March 1st,2018, showed that many studies illustrated parents’ experiences, andcomprised: physical distress such as sleep problems, weakness, fatigue,failing to take care of oneself, loss of good health; psychological distresssuch as anxiety, worry about their child’s condition and their future, stress,guilt, uncertainty; social distress such as loss of social activity, socialalienation, social death; and spiritual distress such as hopelessness,inexpressible wishes of having an ideal society, stigmatization etc.6-10Similarly, in a quantitative study of the burden of caregivers for childrenwith thalassemia9 it was found that 66 % of parents reported they had nohappiness in their lives and 82% of them revealed concerns for their child’sfuture.
In addition, culture and context in each area of the study is aninfluential factor on parents’ experiences.11,12 For example, there is the studyon Southeast Asian and Asian Indian parents of children with thalassemiawho lived in the United states.13 The research results showed that the parents not only suffered from caring for their child, it also found thatthese parents encountered many obstacles to access health careservice systems. This was due to limitations around language,lack of health insurance etc. Furthermore, to some context inIran,8 parents reported their stigma and social death from theinaccurate beliefs about the cause of chronic inheritedillnesses like thalassemia. False beliefs held by people insociety led to parents being seen as disgusting and beingexcluded from society. For the most part, financial burden wasacknowledged to be one of the challenges parents faced dueto exorbitant treatment expenses and other expenses incurredfrom caring for their child (such as traveling to hospital, andparents’ daily expenses for taking care of their child duringhospitalization etc).7-10 This demonstrates that there is a needfor health care providers to understand parents’ experiencesas there might be both similarities and differences in points ofconcern and vulnerable issues in each area.
This understanding would be useful to improve ourcomprehensive understanding of parents’ experiences and tobetter promote quality of life for parents and for their childrenwith thalassemia who rely on parental care. The literaturereview showed similar and different results on the topic of thisintegrative review. Therefore, this integrative review will helpthe researcher to synthesize knowledge about parents’experiences who care for their children with thalassemia andwill provide a perception of these experiences with the aim ofimproving healthcare practice.
Aim of this review
The purpose of this integrative review was to synthesizeparents’ experiences caring for children with thalassemia.
Design and search strategy
PRISMA’s systematic reviews for examining andunifying literature was utilized.14 MEDLINE, PsycINFO,SCOPUS, and CINAHL were searched from January 1st, 2009to March 1st, 2018. The keywords used were ‘parent’,‘parents’, ‘mother’, ‘father’, ‘perception’, ‘perspective’, ‘view’‘experience’, ‘caring’, thalassemia’, ‘children’, ‘child’, ‘baby’.Relevant research articles were retrieved from the databasesuggestions. Journal articles were included in this review ifthey:
Data extraction and synthesis
A thematic synthesis was used for this integrative reviewto collate the findings.15 Data extraction and synthesisinformation were based on objective, sample and setting,methodology, gathering of data, data analysis, conclusions,strengths and limitations and future implications.
Strategies to enhance rigor in integrative reviews
The PRISMA statement consisted of a 27-item checklistand 4 phase flow diagram to meet the standard reporting of asystemic review with rigor. The PRISMA’s framework wasdesigned for a systematic review and meta-analysis. However,in terms of an integrative review, this method was a challengein combining multiple data sources. Therefore, an integrativereview was chosen with an integration concept with samplesprovided of all the processes applied and illustrated byPRISMA.
Data Abstraction/ data evaluation
The empirical report included types of methodology inqualitative research and consisted of five qualitativeapproaches. According to the data evaluation of PRISMA, theydescribed the evaluation in overall terms of quality. The authorchose from 628 articles and determined the exclusion criteriaas follows: no full text, no qualitative approach, not in English,published more than 10 years ago, considered a duplicate,representative of primary data sources and/ or irrelevant.Finally, the author identified 5 articles that met the criteria setout to answer the research questions of this study. A total of623 articles were removed and 5 articles were selected fromat least 98 articles on parents’ experiences caring for childrenwith thalassemia. Data were collected from focus groups,semi-structured and face-to-face interviews conducted inJordan, Iran, Malaysia, and The United States. (Table 1)
Six themes were identified: concern about children inthe future; social death and stigmatization; absence of supportnetwork; psychological distress; frustration with treatmentand; the need for good coping strategies. Details as follows:
Concern about children in the future: parents showedgreat concern about their children’s future due to the parents’awareness of the impacts of thalassemia and its treatment. Thisincludes a gradual worsening of the condition in children withthalassemia. This then leads to the parents’ heightened concernabout their children’s future including factors such as progressionof the disease, concern about treatment and complications,children’s education, job, employment and life prospects,developmental growth, delayed menses, self and body image,marriage and starting a family. As an example, in the wordsof parents:
Figure 1: PRISMA flow diagram on the outcomes of search strategies.
Table 1: The relevant selected articles followed PEO format for qualitative research.16
“Life was full of problems, when you had [sic]the childrenwith thalassemia, the problems were complex. As childrengot older, when they grew up, they need the job, life and marriage.When I thought about these topics, my body startstrembling. My son was a slow student, he suffered from manyeducational problems. The teacher complained him when hedidn’ t do his homework, he was [sic] encountered with manyproblems.” 18
“Her friends had all obtained menstruation, why hadthey had their menses but my child had not? When she askedme. Why she had not gotten her period. Why not? I did notknow how should [sic] will I talk to her (Group 3 – parent)” 17
“Last week, I heard this girl got engaged and the mandidn’t know she had thalassemia. When the man knew, he didnot want to marry her. (R.U., father, Pakistani)” 13
Social death and Stigmatization: the parents spent moretime providing continual care for their children and thisaffected the parents as they no longer visited their relatives asoften, nor did they have the chance to participate in socialactivities. Furthermore, in some areas it was shown that parentssuffered from a lack of real understanding or from misconceptionsabout the cause of thalassemia in others. The inaccurate beliefof their community about thalassemia led some people in thecommunity to cast blame and some treated the children andtheir parents like criminals or implied they were punished byGod. This impacted parents, with some concealing their child’sillness, and others refusing to participate or discuss theirchildren with thalassemia with others. Unfortunately, someparents experienced exclusion or stopped having a relationshipwith their relative altogether. As an example, in the words ofparents:
“I’m away from my friend because I did not have time,I’m really busy…I need to stay beside my son …he was veryimportant for me more than my friends.” 10
“People treated [us] unwisely and stigmatize us. Theytalked about my child’s illness unfairly... Unfortunately, thatwas how they viewed thalassemia and blamed this disease.” 10
“I usually did not tell anyone about it besides our family,because of somebody who didn’t know us but they heardfrom rumors that my kids had thalassemia and they thoughtthat I had disease. ‘Oh, she had AIDS, HIV’.” 13
Absence of a support network: the parents reflected ontheir experiences caring for children with thalassemia andspoke of an absence of support networks. In addition, theylacked information about thalassemia, felt social isolation, andan absence of professional and health care system support, andfinancial support.
Absence of information about thalassemia: parentsreflected on the absence of information on thalassemia suchas: the causes of the disease; treatments, and its side effects and; lack of information to enhance the parents’ role in caringfor their child/children. As a result, parents could not interpretmany of the clinical signs and symptoms of thalassemia andparents found it difficult when confronted with unpleasantsymptoms experienced by their child/children. As an example,in the words of parents:
“I didn’t understand exactly what thalassemia was. Atthe first time, the doctor told me that my child had anemia andasked me if I had thalassemia and I answered no . . . the doctortold me that thalassemia was an inherited disorder . . . but Idid not know really what the doctor meant. (Participant 14)” 10
“Sometimes I asked nurses and doctors about my son’sproblems, but . . . they were busy. (Participant 8)” 10
“I could not just travel due to many restrictions, includingwhere to go to attach the blood bags and if he felt bad, whatI should do, these all were limitations.” 18
Absence of social, professional and health care systemsupport: parents of children with thalassemia were confrontedwith social alienation due to inaccurate belief and superstitionabout the causes of the disease. This adversely affected parentswho felt loneliness and helplessness. In addition, some parentsexperienced an absence of professional support and deficienciesin health care systems. For instance, inadequate expertise ofthe healthcare team, insufficiently equipped healthcare teamand also not enough experienced nurses in intravenous linesadministration. This led to parents feeling dissatisfied . As anexample, in the words of parents:
“We need advice or counseling sessions by an expert.We would be angry because he would not listen to us.” 17
“Lack of supportive behaviors in health care personneland lack of holistic care for supporting the families mightreflect many obstacles that required effective ways to cope withmany challenges [sic] issue.” 8
Absence of financial support: parents worried about lackof financial support because of the characteristics ofthalassemia that require continuous long-term treatmentthroughout their children’s lives. This means, parents need tospend a lot of money to cover the cost of transportation whenvisiting hospital for follow-ups or for blood transfusions, thecost of special food requirements and relaxation activities todecrease tension and anxiety of children. Meanwhile, parentsoften had to leave their jobs to take children to hospital andthis was a financial burden. As an example, in the words ofparents:
“I need to pay for the cost of transportation or need topay for the taxi every time when I took [sic] my child tohospital and I also need to buy special diet for my child.” 10
“Sometimes, I did not have a single cent of money in mypocket to pay for the taxi. But since my child had an appointment I had to take her to hospital. When she was onblood transfusion she told me, she was hungry. I cried and wasfeeling very sad as I had no money in my pocket.” 17
Psychological distress: parents’ psychological distressoccurred for the first time when parents became aware of thediagnosis of their child’s thalassemia. They felt shock, disbelief,and sadness. After that, signs and symptoms caused bypathophysiology of thalassemia showing in their childincluded the effects of its treatments that their child encountered.These included fatigue, exhaustion, failure to thrive, easy tofall prey to infection due to low immunity, growth and delayedsecondary characteristics, body changes, body image, sufferingfrom such treatments as drilling blood, blood transfusion, ironchelation, loss of activities with peer group, school absenteeismetc. This created tension on the part of the parents. Furthermore,parents reflected on problems that occurred within the familyrelated to having children with thalassemia as parents tendedto spend more time taking care of children with thalassemiawhen compared with other children. The sick child’s conditionincreased the feeling of love towards children with thalassemiaand this in turn created a feeling of discrimination andjealousy among the other children. Moreover, in some partsof this study where there was a high prevalence rate ofthalassemia caused by consanguinity through marriage, parentsfelt guilty because they perceived themselves as the cause ofillness in their child. Finally, parents were also concerned thathealthy female children within the family may lose theiropportunity of having a happy marriage as prospectivehusbands may choose not to marry them because they are partof a family with thalassemia. These factors adversely affectedparents and caused tremendous psychological distress. As anexample, in the words of parents:
“I am very sad; it is a true disaster when I saw my sonin pain and unlike other boys. (Participant 3)” 10
“It scared me so much. I also had a healthy child thatI [was] concerned she might lose her opportunity in hermarriage life because [she] did not have the man [who] wantsto marry her after [he] saw the sick child in the family.” 18
“There were many problems involved with kept [sic]healthy child besides the sick child. Some of the problems wereinvolved to parents and some to the child; Diet problems andpreparing separate meals for the sick child, paying attentionto the special needs of these children, different [sic] of skincolor appearance and change in their facial shape, whencompared with that of the healthy one, these were allproblems.” 18
Frustration with treatment: parents reflected on theirfrustration with treatment which had occurred in the past andwas still ongoing in the present, in particular concerningineffective health care systems that could not prevent the birthof children with thalassemia. There was also the inadequacyof the thalassemia diagnostic test in the past that led to havingthe children with thalassemia. Therefore, parents now had tosuffer, having to purchase blood for required blood transfusionsfor their children with thalassemia. Moreover, some parentsshared their experience of not having access to a desferal pumpand medications that affected the children who then receivedineffective treatment since they were forced to share onedesferal pump with other patients. In addition, some parentsreported issues with access to health care systems such asliving in remote areas. This meant parents faced difficulties intravelling to the hospital, and some parents had to move toanother city in order to be close to the thalassemia center.Barriers encountered also included language limitations ofcertain groups in society. As an example, in the words ofparents:
“I was usually seeking for someone who could donateblood for my child. I donated my own blood several times.Some soldiers were ready to sell their blood. I had to find themto pay for and purchase their blood.” 8
“Our English skills were adequate for communicationin every day. However, we felt difficulty to understand medicalterminology when the conversation [was] with the healthcareteam” 13
The need for good coping strategies: parents tried tocope with their suffering arising from caring for their children.The coping strategies focused on improving their children’slives and well-being. Parents tried to search for informationabout thalassemia through communication with physicians andnurses, they tried to cope with self-education, tried to instill asense of normality in their children, and tried to trust in theirfaith and religion to maintain their hope for treatment. As anexample, in the words of parents:
“It never affected her. I treated her as a normal child.I always told my family that, ‘you did not need to be pity her’.Since my daughter was a child, as she was growing up, I kepttelling her about her disease, what was wrong with her, andshe had taken it very good.’’ 13
“But most of all, I put the most of my trust in Godbecause he could do miracles that no doctors out here coulddo. And so, I believed we went through life…most of oursuffering, most of the things we went through made us strongerspiritually and as a person.’’ (B.R., mother, Hmong)” 13
“I was able to cope with my problems through strongfaith in my religion. I was Muslim. I believed in the Qada’ andQadar to deal with my suffering. I was hopeful that one daymy child would be cured of their disease.” 17
The synthesis results identified six themes: concernabout children in the future; social death and stigmatization;absence of support network; psychological distress; frustrationwith treatment, and; the need for good coping strategies.However, we re-conceptualized all of the themes to considerthe latent significant challenges and issues within the themesthat were shown. We identified two important points:psychosocial and financial problems. Both need to beconsidered to enhance the potential ability of parents who carefor their children with thalassemia.
The author found that psychosocial problems weresignificant in the synthesis of the articles. In brief, the resultsfrom all of the articles in this integrative review showedpsychosocial problems parents faced from caring for theirchildren with thalassemia. These problems were caused bymany factors such as disease, treatment and its side effect,life-changing impact in the day-to-day social activities of theparents, the sick child and their sibling/s. All of thesereflected the source of the problem and the parents’ need forsupport to alleviate their suffering.19-22 Suggestions arisingfrom this integrative review have been outlined to helpdevelop a holistic nursing intervention8 to support parents andto promote the potential ability of parents to cope with theirsuffering successfully. Programs could include the promotionof parents’ role in taking care of their children, and thedevelopment of a thalassemia database to share necessaryinformation through digital technology. One example, is settingup a Line group connecting parents of children with thalassemiaas a useful channel for them to immediately access informationthey want.23 In addition, genetic counseling programs wereadded to this integrative review. The goal of genetic counselingwas to promote a better understanding of the consequences ofthe disease, increasing knowledge of the risk of disease ortransmission of disease, reducing anxiety, making the rightchoices, finding ways to prevent the transmission ofabnormalities to children and to make informed decisions aboutfamily planning. In brief, genetic counseling was an importantnursing intervention to help parents to alleviate suffering andto find different ways to solve their problems. Finally, geneticcounseling was seen an important strategy of the health caresystem to decrease the incidence rate of new patients. 17,24
However, the author found that one of the most crucialissues was social death and stigmatization, and this wasprevalent in the study in Iran.8 People in society believedthalassemia was caused by a supernatural power or was apunishment from God, or because someone had donesomething wrong against the norms of society. These viewswere reflected in most of the people in Iran, who still lackedinformed knowledge about thalassemia. This led to the childand families being criticized by society resulting in socialdeath. Families had to conceal their child’s illness. The studyalso reflected the disparity in society, and this needs to beresolved urgently by all parties in order to allow children andfamilies to live in society with dignity and well-being. Due tothese challenging issues, parents still faced limitations in termsof their vulnerability related to belief and social values. Therefore,further study is needed to better understand this vulnerabilitywith an ethnographic research as an approach to reach therequired research questions. After that, the information gainedfrom a qualitative approach could be used to construct anappropriate nursing intervention to promote a properunderstanding of the disease to people in society.
Financial problems seemed to be a universal cause ofsuffering of parents with children with thalassemia since thedisease requires prolonged care and treatment and frequenthospitalization. It was the greatest problem seen, especiallyamong parents in the lower socio-economic group.9,13,18 Thisreflects the need for financial support as a necessary factor tohelp parents to reduce suffering from caring for their child andto promote the quality of care and the quality of life in boththe parents and their child. Therefore, financial support shouldbe considered by policymakers, at least in the form of healthinsurance, to be determined as a health right for citizens toalleviate the parents’ suffering arising from caring for theirchildren with thalassemia.25 Moreover, most of the studiesraised unemployment as one of the burdens facing parents asthey frequently needed to take leave from their jobs to taketheir child to hospital.17 Therefore, policymakers shouldcooperate with employers to determine guidelines to helpparents.
This study looked at how health care providers need tohelp parents to cope with their lives by applying specificstrategies. These include: promoting the caring role of parents;developing a thalassemia database; implementing a geneticcounseling program and; policymakers creating appropriatehealth insurance policies. Further study is needed to understandsome topics where the understanding is still limited to helphealthcare providers to provide the appropriate nursingintervention to alleviate parents’ suffering. Moreover, thesenursing interventions should integrate a holistic approachparticularly when it comes to the relevant issues of religion,belief, and society’s values.26
According to the results of this integrative review,parents of children with thalassemia faced many challengesarising from caring for their child. These comprised concernabout children in the future, social death and stigmatization,absence of support networks, psychological distress, frustrationwith treatment, and the need for good coping strategies. Theseresults show that health care providers should be concernedwith the suffering of parents who care for children withthalassemia. This is because parents are crucial and play animportant role in providing quality care for their child.Therefore, to attain a better quality of life for both parents andtheir children, health care providers should provide theappropriate holistic nursing care or interventions to alleviatesuffering. They should provide the appropriate supportprogram for challenges and issues in caring for children with thalassemia. In particular, psychological support should begiven to enhance the capacity of parents’ skill to cope withtheir situation. Finally, these improvements will enhanceparents’ capacity to sustain their role in taking care of theirchildren, which will lead to a better quality of life for parentsand children alike.