Thalassemic Children Research Articles

Red blood cell alloimmunizations in beta-thalassemia patients in Casablanca/Morocco: Prevalence and risk factors.

We aimed to study the prevalence and risk factors of red cell alloimmunization in β-thalassemic patients. Retrospective study during 9 years (2009-2018) was conducted on 160 β-thalassemic patients transfused regularly in pediatric department of children's hospital in Casablanca, Morocco. The main clinical, demographic and transfusional characteristics of alloimmunized and non-alloimmunized patients were compared. Red blood cells units transfused were leukodeplated and phenotypically matched for RH-KELL systems and for other systems after immunization. Screening and antibody identification were performed by gel-filtration method on BIO-RAD caseds using 3 and 11 red blood cells panels. To detect autoantibodies, autocontrol and direct antiglobulin tests were carried out using polyspecific coombs (IgG/C3d) gel cards. The prevalence of alloimmunizations was 8.75% during the study period. Seventeen alloantibodies identified were directed mainly against antigens of KELL and RH systems: KEL 1 (35.29%), RH 3 (23.52%), RH1 (11.76%), Kpa (11.76%), RH2 (5.88%). Red blood cells autoantibodies had been detected in 6 of 14 (42.85%) of alloimmunized patients versus 12 of 146 (11.76%) of non-alloimmunized patients (P<0.01). Presence of autoantibodies, transfusional interval<3weeks and gender were associated with high rate of red cells alloimmunization. This study proves the data of literature. The presence of red cell autoantibodies appears to be a major risk factors for alloimmunization in thalassemic children, and could advocate specific transfusion guidelines.

PS1575 USE OF THE NEW DEFERASIROX FORMULATION IN PEDIATRIC THALASSEMIA PATIENTS: ONE YEAR RESULTS

Background:Thalassemia is the most common and severe chronic hemolytic anemia in Greece, the National Registry recently reporting more than 2000 transfusion‐dependent patients. As a combined result of disease pathophysiology and transfusion treatment, thalassemic patients present with iron overload from an early age. Deferasirox (DFX), the newest of chelators used to excrete iron, was until recently administered in the form of dispersible tablets. However, the same active substance was licensed as film coated tablets (FCT) in an effort to overcome issues related to drug intolerance. Because of the same active substance being used, no clinical trials in iron overloaded patients were required from regulatory authorities.Aims:Aim of the present study was to evaluate safety and efficacy of the new DFX formulation in pediatric patients with transfusion‐dependent thalassemic syndromes.Methods:Pediatric patients with transfusion‐dependent thalassemia, followed at a single pediatric center, were enrolled. Patients were prospectively evaluated as to hepatic function, glomerular and tubular renal function, and liver and cardiac iron overload over a twelve‐month period following initiation of DFX FCT. Drug related adverse events were recorded. Statistical analysis was performed by SPSS 23, Pearson Correlation and Wilcoxon test.Results:Out of 19 patients enrolled 12 (63%) were males. Mean patient age was 13.8 years (7–18 years). Prior to DFX FCT administration 14/19 patients (73%) were on the previous DFX formulation (dispersible tablet, DT) and 5/19 patients on a different iron chelator. At the end of study, mean ferritin values showed no statistically significant change. With regards to renal function, 18/19 patients (94.7%) presented with a reduction in glomerular filtration rate (GFR) at some time point during follow up, however, in no case was reduction found to be more than &gt; 33% compared to baseline. Transient increase of spot urinary protein: creatinine ratio was reported in 4/19 patients (21%), and of spot urinary calcium: creatinine ratio in 18/19 patients (94.7%) – in no case increase reaching statistical significance. Mild, transient elevation of alanine transaminase (ALT) and of alkaline phosphatase was reported in 3/19 patients (15.7%) and of aspartate transaminase (AST) in 2 patients (10.5%). No statistically significant correlation was found between DFX FCT dose and laboratory parameters studied, i.e ferritin, GFR, protein: creatinine and calcium: creatinine ratio, AST, ALT or ALP. With regards to cardiac and liver magnetic resonance imaging (MRI), assessment at the beginning and end of study was performed in 8 out of 19 patients (42%). All patients presented with a reduction in liver iron, which reached the limit of statistical significance, and a statistically significant reduction in cardiac iron. Reported drug‐related adverse events were mild gastrointestinal symptoms in 3/19 patients (15.8%) and skin rash in 1 patient (5.2%).Summary/Conclusion:Study results demonstrated that the new DFX formulation is effective in reducing iron overload in thalassemic children, while maintaining a satisfactory safety profile. Improvements in formulation's mode of administration can increase treatment adherence and minimize iron toxicity. That, in combination with documented safety and efficacy, may improve quality of life for patients with transfusion‐dependent thalassemia.

Barriers Hindering Maternal Care of Children Suffering from Thalassemia: An Assessment Study

Background: Thalassemia is a group of genetic disorders of blood, characterized by decreased synthesis of one or two types of polypeptide chain (α or β) that form abnormal human hemoglobin molecules. Aim of this study: was to assess barriers hindering maternal care of their children suffering from thalassemia. Research design: A descriptive design was utilized in carry out this study. Setting: The study was conducted in Pediatric Hematology Outpatient Clinic at both Ain Shams University and Zagazig University Hospitals. Subject: A Purposive sample composed of 100 mothers (50 from each study setting) provided care to their children suffering from thalassemia. Tools of data collection: An interviewing questionnaire sheet that was designed by the researcher to assess barriers hindering maternal care of their children suffering from thalassemia. Results: Most of the studied mothers were having physical, psychological, nutritional, educational barriers and sleep disorders that hinder care of their children suffering from thalassemia. Conclusion: There are barriers that prevent the mothers from provide care of children with thalassemia and considers physical barriers as the main barriers are meeting the mothers when care her child with thalassemia. Recommendations: Periodic assessment of maternal practices in care of their thalassemic children and Identify barriers hindering maternal care.

Assess Quality of Life of Children with Beta Thalassemia Major

Background: It is imperative to know further about QOL in children to assess and improve the care that they receive. Quality of life varies from other forms of medical assessments in that it emphases on the people's own opinions of their welfare and other features of life. Aim of study: Assess the quality of life among children with beta-thalassemia. Design: A descriptive research design was utilized in this study. Setting: the study was carried out at the outpatient hematology clinic at Mansoura University children's hospital in Dakahlia governorate. Sample: the study sample was collected from 124 children with beta thalassemia major. Results: Almost all the studied children who complied with blood transfusion had low QOL. Also, the majority compliant with chelation therapy had lowQOL. The studied thalassemic children experienced moderate QOL related to physical, emotional, and school functioning and experienced high QOL regarding social functioning. Conclusion: the highest percentage of the studied children had moderate levels of QOL. There was a statistically significant association between children's QOL and their knowledge. Moreover, there was a positive relation between children's ages and their total scores of QOL. Recommendation: regularand continuous health education programs are essential for children suffering from beta thalassemia and their mothers.

Relationship between serum ferritin and endocrinopathies in thalassemic children: A hospital-based study

Background: Thalassemia syndromes are a heterogeneous group of Mendelian disorders characterized by lack of/decreased synthesis of either ?/? globin chains of hemoglobin. It results in ineffective erythropoiesis and lysis of mature red blood cells in the spleen. Thalassemic patients require multiple blood transfusions which led to iron overload. Objective: This study evaluated endocrinopathies in thalassemic patients of age 5–18 years and relationship with serum ferritin level. Materials and Methods: This is a hospital-based cross-sectional study on 70 transfusion dependent thalassemic children of 5–18 years attending the department of pediatrics, of a tertiary care hospital in Assam. Their height, sexual maturity rating, serum ferritin, calcium, alkaline phosphatase, phosphate, thyroid stimulating hormone, random blood sugar, and fasting blood sugar were recorded. The data were analyzed statistically and p &lt;0.05 was considered to be statistically significant. Results: The study population (70) consisted of 40 males and 30 females. In this study, 91.67% (22 of 24 cases) had delayed puberty, 50% (35 of 70) were stunted, 37.14% (26 of 70 cases) were hypocalcemic, 31.43% (22 of 70) had hypothyroidism, and 2.85% (2 of 70) were diabetic. The mean serum ferritin level was 2845±859.40 ng/ml in delayed puberty while it was 2027.50±21.92 ng/ml (p&gt;0.05) in thalassemic children with normal puberty. The mean serum ferritin in stunted thalassemic children was 2909.86±820.93 ng/ml and it was 1421.43±283.41 ng/ml (p&lt;0.001) in normal height thalassemics. The mean serum ferritin in hypothyroid thalassemics is 2806.59±929.57ng/ml and is 1885.75±846.89 ng/ml (p&lt;0.001) in euthyroid cases. The mean serum ferritin in diabetic thalassemics was 2772.50±1092.48 ng/ml and it was 2147.16±965.63 ng/ml (p&gt;0.05) in nondiabetic thalassemics. Conclusion: Growth retardation and endocrinal complications significantly occur in transfusion-dependent thalassemic children. Adequate chelation therapy helps in controlling serum ferritin levels thereby enabling chronically transfused thalassemic children to grow normally without early endocrinal complications.

Awareness among Parents of Beta Thalassemia Major and Intermedia Patients in Three Centers in Baghdad and Al-Nasiriyah, Iraq in 2017

Background: Thalassemia is an autosomal recessive disease common in Iraq with a prevalence of 35.7 per 100000. Beta thalassemia major is a life-threatening condition with many complications which if not managed could cause death at an early age. This cross-sectional study aimed to assess the awareness of parents/caregivers of children with beta-thalassemia major and intermedia, as enhancing awareness is the first and the most important step in all prevention programs&#x0D; Methods: We conducted this study in three thalassemia centers (two in Baghdad and one in Al-Nasiriyah) from July 20th, 2017 to September 20th, 2017. This study involved 193 parents of thalassemic children under the age of 15 who come to the centers frequently for blood transfusion. The study assessed the awareness questionnaire through interviews. Data analysis was performed using the Statistical Package for the Social Sciences program (SPSS) version 24.&#x0D; Results: We found that awareness was low in many aspects. The highest knowledge was for foods that thalassemic patients shouldn't eat and for the early manifestation signs in thalassemic patients which is 94.8% and 86%, respectively. The lowest knowledge was for the consideration of human immune deficiency virus as a transfusion-transmitted disease and for the chance of having an affected child when both parents are carriers which were 37.3% and 11.9%, respectively.&#x0D; Conclusion: Parents have low awareness of thalassemia in many aspects. Thalassemia care centers may need to provide more education for the parents of patients with thalassemia.

Quality of Life of Thalassemic Children and the Assessment of Effectiveness of Health Care Package in Terms of Knowledge for the Parents on Care of Thalassemic Children

Quality of Life of Thalassemic Children and the Assessment of Effectiveness of Health Care Package in Terms of Knowledge for the Parents on Care of Thalassemic Children

Coping Strategy Enhancement for Families Who Have Children with Thalassemia Through Family Counseling at Sukabumi Thalassemia Foundation

&#x0D; &#x0D; &#x0D; Family copyng strategy is one of the factors so that families with thalassemia children can survive and function properly. The purpose of this study is to determine whether family counseling had an effect on improving family copyng strategy, by identifying 1). What are the characteristics of children and families in the Sukabumi branch thalassemia foundation? 2). What is the process of family counseling? 3). What are the results of implementing family counseling? This study used a qualitative approach with the action research method. Three families with thalassemia children with main data collection techniques by providing indeepth interview, supplemented by observations and study documentation. The results showed that children with thalassemia had transfused between 3 to 10 years, and routinely performed transfusions every week to every month. Family counseling provides solutions to problems faced by families as an effort to improve family strategy copyng. Active families seek support from extended families, doctors and friends. Families always think positively and organize themselves not to display negative emotions and improve spiritual life. The conclusion of this study indicates that family counseling can have a positive influence on improving family copyng strategy. Family counseling as an intervention that involves all family members also strengthen relationships between family members. The practical implication of the family counseling process requires commitment from all family members, the willingness to openly convey what is felt and thought.&#x0D; &#x0D; &#x0D;

Seroprevalence of Toxoplasma gondii Infection Among Β-Thalassemia Major Pediatric Population: Implications for Transfusion Transmissible Toxoplasmosis.

Children with β-thalassemia major who regularly receive blood transfusion are at risk of developing transfusion-transmitted infection. Toxoplasmosis is a common and a serious parasitic disease with high prevalence and could be transmitted through blood transfusion from healthy asymptomatic donors. However, screening Toxoplasma gondii before blood donation has not been considered. The objective of this study was to determine the prevalence of T. gondii antibodies among thalassemia children undergoing blood transfusion. In a case-control study, serum samples from 211 thalassemia children and 100 control children were investigated for Toxoplasma IgM and IgG using the enzyme-linked immunosorbent assay. Positive serum samples for IgG antibodies to T. gondii were further subjected to IgG avidity enzyme-linked immunosorbent assay. The seroprevalence of Toxoplasma infection among thalassemia children was 23.2% and 53.6% for IgM and IgG anti-Toxoplasma antibodies, respectively. Whereas in the control group, the prevalence was 5% and 18% for IgM and IgG anti-Toxoplasma antibodies, respectively. There is a significant statistical difference between thalassemia and control groups regarding the prevalence of toxoplasmosis. From these positive IgG samples, 65.5% have low avidity indicating recent infection while 38.73% have high avidity indicating past infection. Due to the high serologic infection rate of toxoplasmosis among thalassemia pediatric population in this study with no existing effective therapies and no available T. gondii vaccine, appropriate strategies are critical for reducing the risk of that infection. Screening of blood for T. gondii antibodies should be considered before transmission to those children especially in countries with a high prevalence of toxoplasmosis.

Knowledge, Attitude and Practices of Parents of Thalassemic Children in District Patiala, Punjab, India

Introduction: β-thalassemia is an autosomal recessive single gene disorder characterized by defective production of hemoglobin and excessive destruction of Red Blood Cells. The defect causes an abnormal development of Red Blood Cells and ultimately anemia. The cultural and religious scenario in Muslims, Khatris and Punjabis migrated from Pakistan is such that consanguineous marriages are quite common. There is no concept of premarital screening of counseling of individuals with a family history of disease. The only way to prevent the disease and to reduce the morbidity and mortality is by educating the general population. For this reason, present study was taken to evaluate the knowledge, attitude and practices of parents of Thalassemic children. Objectives: To assess the Knowledge, Attitude and Practices of Parents of Thalassemic Children. Methods: The study was conducted on parents of 100 patients of thalassemia who are coming regularly for blood transfusion in Thalassemia Day Care Centre (TDCC) run by Patiala Thalassemic Children Welfare Association (PTCWA) at Rajindra Hospital, Patiala to collect the relevant data for the study to evaluate the Knowledge, Attitude and Practices (KAP) of parents of thalassemic children. Results: Mean age of the patients was 8.36 years. 66% patients were males and 34% were females. 41% of the patients were from Khatri community and 59% were from other communities. Family history of thalassemia was present in 23% of the cases and history of consanguineous marriage was present in 4% of the cases. Seventy six percent of the parents knew about the prenatal diagnosis that could be performed to prevent the birth of thalassemic children. Knowledge regarding the treatment options available was found more in parents from Bania, Khatri and Kamboj communities. More positive attitude of parents towards prevention of thalassemia was found in parents who have answered 11-15 questions from Knowledge questionnaire. Conclusion: There should be a community based educational efforts to increase awareness of this problem especially to the people from low socioeconomic class and from rural area. Control of thalassemia requires treatment of the individual patients as well as a community based educational effort to increase the awareness of this problem.

Neurocognitive Function and Its Related Potentials in Children with Beta Thalassemia Major: An Egyptian Study.

BACKGROUND:Repeated blood transfusions and hemolysis in β-Thalassemia major children lead to iron overload in various organs, including the brain which may cause neurodegeneration.AIM:To evaluate intelligence quotient in children with β-thalassemia major and healthy counterparts and to assess risk factors that cause cognitive problems.SUBJECTS AND METHODS:This case-control study was performed on 50 children aged 6-16 years old with β-thalassemia major as patients group and compared with 50 healthy children as a control group of matched age, sex, and social class. Cognitive functions were evaluated by using the Wechsler Intelligence Scale for Children. Serum ferritin and iron were measured by ELISA.RESULTS:There were significantly lower mean performance and full-scale IQ scores of patients group in comparison with controls, whereas no significant differences between both groups as regards to a verbal IQ score. In thalassemic children, block design, comprehension and arithmetic were negatively correlated with age of disease onset, duration of illness and onset of chelation therapy. Serum iron and ferritin were negatively correlated with similarities and digit span. Serum iron levels were negatively correlated with performance IQ score.CONCLUSION:Children with β-thalassemia major need to receive more academic attention and cognitive assessment to improve their IQ.

Comparative Evaluation of BMI, Dental Age, Salivary Alkaline Phosphatase Levels, and Oral Health Status in Children with β Thalassemia Major.

ABSTRACTPurposeThalassemia poses clear systemic and oral health problems. Clinicians must be aware of various dental treatment needs in these patients. This study was undertaken to assess body mass index (BMI), dental age, salivary alkaline phosphatase levels, malocclusion, and treatment needs in children with β thalassemia major.Materials and methodsA total of 100 children within the age group of 2–15 years (50 thalassemic and 50 healthy controls) were included. Dental age, oral health status, malocclusion, and intervention urgency index was recorded. Unstimulated whole saliva samples were collected to assess the salivary alkaline phosphatase levels. The recorded data were statistically analyzed.ResultsA significantly greater number of thalassemia children were found to be in the underweight category. A significantly high OHI-S scores and low gingival index scores were observed in thalassemia children. Salivary alkaline phosphatase levels were significantly low in thalassemia children compared to the control group. Intervention urgency index scores were significantly high in thalassemia group of children.ConclusionThalassemia children had low BMI, gingival index scores, salivary alkaline phosphatase levels and higher prevalence of malocclusion, and high OHI-S index scores compared to the healthy control group. Intervention urgency index scores revealed greater dental treatment needs in children with thalassemia.How to cite this articleRani ST, Reddy ER, et al. Comparative Evaluation of BMI, Dental Age, Salivary Alkaline Phosphatase Levels, and Oral Health Status in Children with β Thalassemia Major. Int J Clin Pediatr Dent 2019;12(4):303–306.

Sociodemographic determinants associated with parental knowledge of screening services for thalassemia major in Lahore.

Objective:To assess the sociodemographic factors affecting parental knowledge regarding availability of screening services for thalassemia major.Methods:A cross sectional study was conducted among biological parents of thalassemic children at three registered centers of blood transfusion in Lahore. A sample of 186 parents was selected through systematic random sampling. A pretested questionnaire was used to collect data. IRB clearance was obtained and informed consent was taken before data collection. Data was analyzed at SPSS version 23 and chi square test was applied and p value was fixed at ≤ 0.05 as significant.Results:Parents of registered thalassemic children have adequate knowledge about Disease transmission and screening services. 91% of the participants knew that thalassemia major gets transmitted through parents. Cousin marriage was mentioned as a source of transmission by 77% of the participants. 91% of the parents knew about prenatal screening and 89% about premarital screening. Knowledge of parents was significantly associated with male gender, higher education and income.Conclusion:Male gender, higher education and income are associated with better knowledge of parents.

Types of Thalassemia predict the optimal time of surgery for biliary stone in Children.

Introduction: Biliary stone is the most common surgical problem in thalassemia (thal) children. The optimal time for surgery is currently unknown, it should be delayed as long as the patients remain asymptomatic. This study aimed to determine the optimal time of biliary stone surgery according to type of thalassemia. Method: This is a retrospective study of the 56 patients younger than 18 years who underwent surgery for biliary stone disease in Srinagarind Hospital between 2006-2016. There were 39 thalassemia patients. Of these, 8 patients of unknown type of thalassemia were excluded. We classified the remains (31 patients) into 5 groups as follow: 16 β thal E (51.6%), 4 Hb H-CS (12.9%),4 Hb AE Bart’s-CS (12.9%),3 other major thal (9.6%) and 4 other minor thal (12.9%). All clinical and surgical data were analyzed. Result: The respective minimal age of stone detection for β thal E, Hb H-CS, Hb AE Bart’s-CS, other major and other minor thal was 3, 12, 10, 12 and 8 years. The minimal age (years) of symptom occurrence was 9 for β thal E, 12 for Hb H-CS and 12 for other major thal. There was no symptom observed in Hb AE Bart’s-CS and other minor thal until 15 years of life. Half of the patients with Hb H-CS experienced symptom, and required surgery before 15. Conclusion: Type of thalassemia could predict optimal time of surgery. We suggest the surgery can be deferred until 15 for Hb AE Bart’s-CS and Other minor thal, or until 9 regardless of types of thalassemia.

Need for a universal thalassemia screening programme in India? A public health perspective.

Thalassemia is one of the significant public health concerns as the carrier rate and disease numbers are increasing worldwide. The increase in number is because of consanguineous marriage which has a deep-rooted norm among many people globally. Besides various clinical and psychological problems associated with thalassemia, a lifelong treatment aspect makes it much more difficult for a person or family to sustain with thalassemia or thalassemia-affected children. Though the government has come up with a screening programme for thalassemia, given the fact that it is optional, people tend to ignore it. Examples from Pakistan and Iran remind us to have a mandatory prenatal screening programme which is very much cost-effective. With a highly recommended notion, we suggest that it should be universal to have an antenatal screening programme to avert thalassemia-related deaths.

Quality of life in transfusion dependent thalassemic children

Quality of life in transfusion dependent thalassemic children

Family planning practices in couples with children affected by β-thalassemia major and its relationship with their education: An epidemiological study.

BACKGROUND:Although prevention the birth of children with β-thalassemia major (β-TM) is an important health issue, it is rarely explored. With this background, the study was designed to find family planning practices and related factors of couples with children affected by β-TM.MATERIALS AND METHODS:This was a cross-sectional, observational study, conducted among 324 parents with children affected by β-TM attending a tertiary care health facility in Eastern India. Data were analyzed using univariate and multivariable logistic regression using SPSS.RESULTS:Of 324 parents, 89.5% were sexually active, 44.8% of which were currently using family planning method to prevent the birth of another child with the oral contraceptive pill most preferred (46.9%) followed by tubectomy (33.9%). Those who were sexually active, but not desirous of a child were 223, of which 41.7% had the unmet need for family planning with religious belief (41.9%) being the most predominant reason followed by unaware of methods (26.9%). In multivariable analysis, religion, caste, per capita monthly income, mothers’ education level, and working status were significant predictors of unmet need for family planning adjusted with others.CONCLUSIONS:Burden of unmet need for family planning among the study population was alarmingly high. Parents of the thalassemic children should be counseled in easily comprehensible local language to increase the rate of contraception among them.

The Relationship between Serum Level of Ferritin and Cardiac Troponin T (cTnT) in Children with Major Beta-Thalassemia

Beta-thalassemia is one of the most common inherited blood disorder result from a defect in production of beta globin chain due to mutations in the HBB gene on chromosome 11, range from minor to sever transfusion dependent major beta-thalassemia. Major beta-thalassemia regarded as one of the major health problems in endemic regions as the Mediterranean basin, part of North and West Africa and South East Asia. Frequent blood transfusions cause progressive iron overload and hemochromatosis in the body which is the major complication of treatment lead to accumulation of iron in many organs and meanly in heart. The aim of the study is to determine the relation between serum ferritin and serum cTnT in children with major beta-thalassemia. A cross sectional study was carried out in Kirkuk City from 16th of January 2018 to 15th of March 2018. A total of 60 children with major Beta thalassemia admitted to thalassemia center in Azadi Teaching Hospital, and 30 non major Beta thalassemia children (control group) their age ranged between 2-10 years old, were investigated for s. cTnT and s. ferritin. There was a highly significant increase in s. ferritin level in children with major beta-thalassemia as compare with control of same age and gender. Also, There was a difference between the level of serum cTnT level in children with major beta thalassemia more than control, but not significant of same age and gender.

Well-being, familial risk, and transfusion interval in thalassemia-affected families: A two-step cluster analysis.

BACKGROUND:Thalassemia is an inherited blood disorder which is one of the common genetic disorders among children that is increasing worldwide.AIM AND OBJECTIVE:This study aimed to identify clustering patterns among thalassemia-affected families based on their well-being, transfusion interval, and risk factors using two-step cluster analysis (TCA).METHODS:The study design is a descriptive, analytical cross-sectional study. The study sample consisted of 125 thalassemia children along with either one of the parents who referred to a thalassemia treatment center of a pediatric hospital in Chennai, Tamil Nadu. The parents and children were evaluated using the Ryff Psychological Well-being Scale and Kidscreen Questionnaire respectively. Data were analyzed using SPSS version 16.0 software.RESULTS:Out of 125 parents, 86 were father (68.8%) and 39 were mother (31.2%). The mean age of parents was 38 years. Similarly, out of 125 thalassemia-affected children, the mean age of children was 13 years. Six clusters were deducted from the TCA. Parents’ well-being variable does not have discriminating power to form cluster division. Three cluster formations were meaningful.CONCLUSION:TCA, in this study, helps in finding the clusters of families with thalassemia-affected children associated with poor well-being and familial risks, which require attention for medical counseling.

Literature Review: Stress and Mother Life Quality with Thalassemia Children Major Ages 0–18 Years

Thalassemia is a blod disorder which various countries pay specific attention to, both develoing and developed countries. Children with thalassemia need long-term treatment and excessive cost. It has become a burden for the family. Mother as a caregiver for thalassemia child is often confrontedby a stressful life related to the child’s illness and the following treatment as well as other couses of stress; such as fear of loss, anxiety, frustration and sadness, and overwhelmed thinking of the child’s recovery can affect the mother’s daily activities, also the quality of life. This systematic review aimed to identify the stress and quality of life of mother with thalassemia children aged 0–18 years. Articles were collected through Google Scholar, PubMed, Proquest, Ebscho databases using the keyword Stress, Qulity of Life, Mother with Thalassemia Child including both quantitative and qualitative studies. The used articles only which were full-text, written in English, and published in 2007 until 2017. There were five articles met the criteria. The results of literature review revealed that the stress faced by the mothers especially when the children must undergo the blood transfusion for once a month. Stress was shown with psychological symptoms that can affect health. The mother’s stress was assessed using the Parental Stress Scale (PSS), and for the quality of life measured by HRQOL SF-12v2, with Likert scale, the lowest score 0 and the highest 100. Found that if the mother’ quality of life also decreased. Conclusion: mothers with thalassemia children then to experience stress, resulting in both physical and psychological impact on the mothers’ health, thus affecting the quality of life. By this literature review, expected that will be more research related to stress and quality of life of mothers with thalassemia children, thus reducing stress on mothers that affect the quality of life.