European Journal of Cardiovascular Medicine

Thalassemia is an autosomal recessive genetic disorder that results in reduced production of hemoglobin which is an iron-rich protein found in red blood cells which functions to transport oxygen from the lungs to all parts of the body (Black & Hawk, 2014). The prevalence of thalassemia in the world is around 20% of the population carrying α thalassemia and 5.2% carrying a significant variety of hemoglobin disorders including β thalassemia and thalassemia (WHO, 2019). Every year around 56,000 babies are born with thalassemia major (Weidlich et al., 2016). According to the Thalassemia International Federation (TIF), around 68,000 children are born with thalassemia every year in the world. The most common thalassemia is beta thalassemia with 80 to 90 million people (1.5% of the global population) reported to be carriers of this thalassemia trait. It is also reported that around 23,000 children are born in the world with beta thalassemia every year (Taher et al, 2013). The prevalence of beta thalassemia carriers in the Maldives is 18%, Free 14%, Sardinia 10.3% and Southeast Asia 3-5%. In Southeast Asia, the high number of thalassemia carriers causes quite serious public health problems because it is related to human survival (Cappellini et al, 2014).
The Indonesian Thalassemia Foundation (YTI) and the Indonesian Association of Parents of Thalassemia Sufferers (POPTI) conducted thalassemia screening in the community from 2009 to 2017 with the results of screening 1,184 people as thalassemia carriers. Meanwhile, based on data from Cipto Mangunkusumo Hospital, as of October 2016, there were 9,131 thalassemia patients recorded throughout Indonesia (Ministry of Health, 2018). According to Dr. Mohammad, Hoesin General Hospital (RSMH) registration data between June 2010 and April 2018 showed 287 patients suffering from thalassemia, and 145 (50.5%) of them were aged 7 to 18 years (Venti et al., 2018). West Java Province has the highest number of thalassemia sufferers in Indonesia. There are as many as thalassemia sufferers in Indonesia 5,501 patients. 1,751 or around 35% came from West Java. There are around 300 people who care for thalassemia in the city of Bandung. Meanwhile for Greater Bandung around the 800s. Most patients seek treatment at the hospital. Hasan Sadikin and RSUD in their respective areas. This was reported by the Indonesian Thalassemia Foundation-Association of Parents of Thalassemia Sufferers (YTI-POPTI, 2021).
Thalassemia causes children to experience mental retardation, physical deformities, growth retardation, and delayed puberty. The impact on physical appearance, namely bone deformities and short stature, and poor self-image. Apart from that, it can cause serious complications such as heart failure, cardiac arrhythmias, liver disease, endocrine complications and patients are easily infected (Ismail et al., 2018). One of the therapies given to thalassemia patients is blood transfusion. Blood transfusion is the mainstay treatment for sufferers of thalassemia major and intermedia. Chronic transfusion prevents most of the serious growth, skeletal, and neurologic complications in thalassemia major (Abolwafa et al., 2018). The results of this study also emphasize the importance of maintaining pre-transfusion Hb levels of at least 9-10.5 g/dL (Abolwafa et al., 2018).
Supriyanti (2019) wrote that 83.8% of the 63 respondents studied did not comply with blood transfusions. This non-compliance has an impact on the child's growth and development, oxygenation to the brain, resulting in a decrease in the child's cognitive abilities. One of the reasons why children do not comply with transfusions is because of financial conditions (Aziz, 2019). The results of research by Rima and Siska (2018) found that non-compliance with blood transfusions in thalassemia children was caused by low family support.Family support is the most influential thing because family support is a situation that can be beneficial for an individual that is obtained from other people who can be trusted so that a person will know that there are other people who pay attention, appreciate and love him. Family support Refers to the actions taken by others when providing assistance, and is understood to be of four types namely: emotional support; information support; and assessment support, and instrumental support (Friedman, Bowden, & Jones, 2010 in Artamia, Rakhmawati, and Saladin, 2019).
Children with thalassemia experience physical, emotional, social and school dysfunction. Therefore, family support is very necessary for children suffering from thalassemia. The role of the family, especially parents, is very influential in undergoing continuous treatment and there is no certainty of recovery, especially for young children who need protection and love from their parents, so that children have confidence that their parents will not neglect them. about their illness. Family support, especially from parents of thalassemia sufferers, is the presence of the family as the support needed by parents of thalassemia sufferers, that there are resources that provide a sense of psychological comfort, make individuals loved, cared for, appreciated and have their true nature. its existence is recognized by group members.
The family has a strong influence in determining treatment, the support provided is instrumental support, assessment support, informational support and emotional support. Therefore, the support provided by families in the form of recommendations for regular blood transfusions has an impact on improving the welfare of parents who have children with thalassemia to take their children to health services (Ghorbanpor, 2020). Based on the results of research by Ghafoor (2016) in Pakistan, 60% of parents are not aware of the basic things about thalassemia in children because these parents are illiterate, live in rural areas, and have a low level of knowledge.
This influences family support for their children Based on the research results of Rima and Siska (2019), it shows that the family support of parents in the Thalassemia Room at Bhayangkara Setukpa Lemdikpol Hospital, Sukabumi City is supportive, including always providing encouragement or motivation so that the majority of parents are compliant in taking them to undergo blood transfusion treatment. This shows that the support provided by the family will reduce parental non-compliance in patients. Based on the results of preliminary research, thalassemia sufferers on average receive blood transfusions for 50 children every month at Pasteur Bandung Regional Hospital. Researchers' observations of thalassemia families in hospitals showed that the families accompanying the child for blood transfusions were mostly parents Judging from compliance, families take their children for transfusions according to the predetermined schedule. Apart from that, there are also families who take their children for transfusions later than the specified time for various reasons.
The lack of family support provided is assessment support, where families should give praise and appreciation to thalassemia children who undergo blood transfusions. Apart from that, based on data in October 2022, there were 10 children out of 50 who did not have blood transfusions according to schedule. Blood transfusion is in the form of red blood cells which have the function of increasing Hb (Hemoglobin) so that oxygenation in tissues can be optimally improved. Continuous and regular transfusions can help replace dead cells. This transfusion must be carried out throughout life for sufferers of thalassemia major and is carried out periodically and continuously over a period of every 4-5 weeks (Rohimah and Puspasari, 2020).

According to Angastiniotis (2019), in patients who do not undergo regular transfusions, there is an increase in erythropoiesis more than normal and a decrease in hemoglobin levels which can cause symptoms such as dizziness, lethargy, fatigue caused by chronic anemia due to not having regular transfusions. Patient compliance with blood transfusions is important in the management of thalassemia sufferers. Factors that influence compliance include; socio-economic factors, side effects of treatment, health service facilities, and family support. In these four factors, family support cannot be ignored, because family support is one of the factors that has a significant contribution and acts as a reinforcement that influences patient compliance (Artamia, Rakhmawati, and Salahuddin, 2019).
Based on the background above, the author is interested in conducting research on the relationship between family support and blood transfusion compliance in children with thalassemia.
RESEARCH PURPOSES
To determine the relationship between family support and blood transfusion compliance in children suffering from thalassemia major

This research is non-experimental research, namely quantitative research that is correlational in nature, including a systematic investigation of the relationship between variables where the research is carried out by developing the relationship between existing variables, namely the independent variable and the dependent variable (Grove & Gray, 2019).
This research was conducted to determine the relationship between family support and blood transfusion compliance in children with thalassemia major in the Children's Care Room at Pasteur Regional Hospital, Bandung. This research design uses a cross sectional method, where this research only uses one time to measure or observe data on the independent and dependent variables only once at a predetermined time. Not all subjects in this study had to be observed on the same day or time, but both the independent variable and the dependent variable were only assessed once (Nursalam, 2015). In this study, the samples were 55 families suffering from thalassemia in the Children's Care Room at Pasteur Regional Hospital, Bandung in 2023.
Sampling in this study was non-probability sampling with a total sampling technique, namely the technique of determining samples from the total population. The instruments to determine family support used in this research consist of informational support (numbers 12, 13, 14, 15, 16), assessment or appreciation support (numbers 17, 18, 19, 20, 21, 22), instrumental support (number 7, 8, 9, 10, 11), and emotional support (numbers 1, 2, 3, 4, 5, 6). This questionnaire consists of 22 statements which are measured on a Likert scale (1-4) with good statement values: 4 = always, 3 = often, 2 = sometimes, and 1 = never, based on this scale the score given by the respondent can be achieved is a minimum of 22 to a maximum of 88. Meanwhile, the instrument used to measure blood transfusion compliance is an observation sheet in the form of a patient's schedule for carrying out blood transfusion therapy. The patient is said to be compliant if the patient has undergone a blood transfusion according to schedule in the last 3 months. Non-compliant if the patient has not had a blood transfusion according to schedule in the last 3 months one or more times. Bivariate analysis in this study used the chi square test