Rajiv Gandhi University of Health Sciences, Bangalore, Karnataka s2

RAJIV GANDHI UNIVERSITY OF HEALTH SCIENCES, BANGALORE, KARNATAKA

PROFORMA FOR REGISTRATION OF SUBJECTS FOR DISSERTATION

1. / NAME OF THE CANDIDATE AND ADRESS / Mrs. G.R. SREEMATHY
1st YEAR, M.Sc(N),
GOUTHAM COLLEGE OF NURSING, WEST OF CHORD ROAD, MANJUNATH NAGAR, RAJAJINAGAR,
BANGALORE – 560 010
2. / NAME OF THE INSTITUTION / GOUTHAM COLLEGE OF NURSING, WEST OF CHORD ROAD, MANJUNATH NAGAR, RAJAJINAGAR,
BANGALORE – 560 010
3. / COURSE OF STUDY AND SUBJECT / 1st YEAR MSc NURSING
PEDIATRIC NURSING
4. / DATE OF ADMISSION / 16-OCT-2007
5. / TITLE OF THE TOPIC / A STUDY TO ASSESS THE EFFECTIVENESS OF STP “ON CARE OF CHILDREN WITH THALASSEMIA” IN KNOWLEDGE AND ATTITUDE AMONG MOTHERS IN SELECTED GOVERNMENT HOSPITALS AT BANGALORE

6 Brief Resume of the intended work

6.1 Need for the Study

‘The child of today for the world of Tomorrow’
Children in the age group of 0-14 years constitute 40% of the population. The well being of these children are the responsibility of parents as well as health personnel. As they are the most vulnerable section which undergo various types of health problems. The risk is connected to growth, development and survival. The primary health care in children aims at prevention and promotion of health.
Among all the childhood diseases, hematological and hereditary diseases are most life threatening disease conditions which affects in their early life. It affects upon birth, severely affecting their ability to survive on their own due to chronic anemia resulting from an inherited hemoglobin disorder. With currently available medical treatment, afflicted children have a substandard quality of life and a shortened life expectancy. The selective pressure that have made the thalassemias are so common are not known but are assumed to relate to the geographic distribution of Malaria.1,2
Among the world total population, 4.5% are the carriers of hemoglobinopathies, about 15 million people are thalassemia patients and 240 million people are carriers of B-Thalassemia. The largest concentration of thalassemia patients seen in South Asia, Sri Lanka, Bangladesh, Pakistan, Middle East countries and Italy.3
Every year, around 8000-10,000 children with thalassemia are born, accounting for 10% of the annual world incidence. The carrier rate of B-Thalassemia varies from 1to3% in South India; 3to15% in North India. Its prevalence is high in ethnic groups among Gujarathies, Punjabies, Sindhis, and Lohanas etc. Over 30 million people are carriers of thalassemia gene in our country.2,4
Thalassemia, depending on its severity, the disease may cause an enlarged spleen, bone weakness and growth problems and may require transfusions starting in early childhood. The blood transfusions lead to the most serious complications. Children with this Illness usually end up also suffering from other complications such as heart disease, cirrhosis of the liver, diabetes, facial deformities, and spinal cord abnormalities.
All forms of Thalassemia like Thalassemia Minor, Thalassemia Major, Alpha-thalassemia, beta-thalassemia, delta thalassemia are transmitted only through heredity. It cannot be caught from another child or person who has it. The disease is passed on through parents who carry the Thalassemia gene in their cells. A ‘carrier’ has one normal gene and one Thalassemia gene in all body cells, a state sometimes called having the ‘Thalassemia trait’. Most carriers lead completely normal healthy lives.5
Many promising researches are going on to find a cure for Thalassemia, like, fetal hemoglobin induction, gene therapy, cord blood and bone marrow transplant, alternative iron removal therapy, blood substitutes etc. While some efforts are being undertaken by non-profit organizations in India to create awareness of this illness among high-risk communities in India, currently there are no efforts directed towards non-resident Indians in countries like United States of America.5
The Bangiya Purohit Saba, an association of Hindu priests in West Bengal, have decided that none of its members will perform wedding rituals unless both the bride and the groom undergo pre marriage blood tests for AIDS and Thalassemia, according to the association’s joint secretary, Samantha Chatterjee.6
A study on “Fractures in Transfusion Dependent Beta- Thalassemia”, aims to analyse the incidence of fractures in beta-thalassemia patients to identify causative factors. A total of 105 TDBT patients were selected for study. There were 85 males and 20 females with a 4:1 ratio. All patients and their families were interviewed using a questionair regarding the onset, course andtreatment of the disease, with a study periodof 10 years. Ten of 85 males and 4 of 20 females sustained fractures. 12 out of 95 patients with thalassemia major and 2 of 10 patients with thalassemia intermedia sustained fractures. 8 of the 63 under transfused (Hb<8mg/dl) and 6 of the 42 well transfused cases (Hb>8gm/dl) suffered fractures.7
A10 years Cohort study conducted to determine the epidemiological aspects and spectrum of hemoglobinopathy, cases in Orissa.1015 cases of anemia were analyzed, and family studies were carried out to confirm the diagnosis. Out of 1015 cases, beta- thalassemia syndrome was prevalent in Brahmin, Karan etc.8
Under the Thalforum articles & news-Thalassemia Community Forum, Thalassemic child’s parent’s campaign for awareness had expressed that, a need to spread awareness about Thalassemia. The Society of India (TSI) also feels the need for such campaigns since lack of awareness is the only impediment in thalassemic prevention.9
So, there is a great need for the people to be aware of all the consequences of thalassemia and it is the most important area where the health personnel should take serious measures to create an understanding and awareness among the public regarding Thalassemia and its risk factors. And even the Gene Therapy and Genetic Counseling
also helps a lot in this aspect.
Many researchers during their clinical experience find that there is a lack of knowledge and awareness among parents and care givers about the onset of disorders of thalassemia, and its life threatening complications which makes the children to lead a miserable life. And it is the responsibility of the health personnel to make them accept and understand the consequences and how to manage. Because the complications of thalassemia like growth retardation, fractures, heart diseases etc occurs in their early lives which cannot be prevented, and it affects the child’s daily activities. Mothers consistently reported a lack of knowledge about thalassemia and sickle cell diseases in the health system and they expressed a stressful lifestyle, emotional hardships such as helplessness, fear of their childs death and anxiety over being separated from their child. So, the parents and caregivers should be provided sufficient knowledge on thalassemia and awareness regarding the importance of physical care, diet schooling, psychological and emotional support for children as well as parents which in turn helps to improve the health and prolong the lives of children by controlling the complications as much as possible. Hence the researcher is interested to take up the study in this aspect to provide knowledge and try to develop positive attitude among mothers in caring thalassemic children.

6.2

REVIEW OF LITERATURE

/ The present study divided into five groups:
a. Studies related to incidence of thalassemia
A study on Hereditary hemolytic disorders among the Ashram School Children in Mayurbhanj district of Orissa. A total number of 465 Ashram school children aged 6-15 years from Mayurbhanj tribal screened. The beta-thalassemia was detected in Santal 8.0%, Kolha 2.0%, Bhumiz 1.7%. Other tribal 3.8%. Sickle cell hemoglobinopathies and beta-thalassemia are prevalent in this district among the tribes, but the frequency is low. This shows that the antimalarial drugs should be administered with caution as these cause hemolytic anemia, sometimes fatal also. This pattern is consistent with the characteristic features of tribal populations in India.10
A study on sickle cell anemia associated with alpha-thalassemia in Malaysian Indians were found to have high frequency of Hb S. They studied 12 patients with sickle cell anemia ranging age from 4-61 years and 30 sickle cell traits age from7-63 years. All 12 sickle cell anemia patients were found to have alpha-thalassemia out of 30 sickle traits.24 had Hb S traits with alpha-thal-2, with additional gene abnormalities.Bgl restriction analysis showed that the alpha-thal2 was mostly of the rightward deletion alpha-thal genotype. The cickle cell anemia patients and Hb S trait carriers had very high levels of Hb S and low levels of Hb 12. The Hb S trait carriers with alpha-thal 2 had relatively low levels of Hb S.11
b. A study related to growth and development of children with thalassemia
A cross sectional study on Growth of children with b-thalassemia major, 68 children with B-Thalassemia Major in Hong Kong , 75% of the girls and 62% of boys over the age of 12years were the third percentile in height. A high prevalence of this incidence reported from Turkey and India. A main cause is the growth hormone deficiency due to thalassemia present in 24% and 50%of due to Hypogonadism in which 47%of girls, amenorrhea in 23%, menstrual irregularity in 14% and arrest of sexual maturation in 13% of 1861 pts. So, they concluded that despite of advanced Medical Therapies, growth retardation and hypogonadism are problems in transfusion dependent pts with T.M.12
A study on Effect of High caloric diet on Nutritional parameters of children with Thalassemia Major, to test the hypothesis that impaired growth might be corrected partially or totally. 30 randomly selected children with thalassemia studied. The dietary intake evaluated by recall methods appears to be adequate compared with 30 normal children. Nutritional status assessed by measuring the body Mask Index (BMI), mid arm conference (MAC), triceps skin fold thickness (SMT) and serum albumen and insulin (IGF-1), before and after 8 weeks of high caloric diets, (130-150%).The nutritional status with thalassemia were significantly decreased compared with control group, but after supplementation of high calorie diet for 8 weeks, BMI, MAC SFT of thalassemic children increased significantly.13
c. A study related to treatment of Thalassemia
A Study on comparative Efficacy of Desferrioxamine, Deferiproneandine Combination Iron Chelation in thalassemic in India. A prospective study from 2000 to April 2001 in an urban setting, 30 thalassemic children having received more than 20 blood transfusions and a serum ferritin greater than 1500mg/ml were enrolled and randomized into 3 groups. The assessment done by 24hrs urinary excretion and measurement of serum Ferritin levels after 6 months of follow-up. Statistical deference assessed by applying analysis of variance. The results were, Ferritin levels after 6 months maximally decreased in group 1, there was a significant difference between group 1 and group 2, no difference noticed between group1& groupp3. So, they concluded that DFX is most effective chelating drug in iron overloaded multi transfussional thalassemic patient.14
d. The studies related to consequences of Thalassemia
A comparative Study on A Comparative evaluation of Thalassemia Major and Thalassemia Minor in Thalassemia Heart Disease They have compared clinical and
Echo-cardiographic global parameters in 131 patients (meanage-28 6 years) and 74-age matched TI patients, are classified into two subgroups with respect of the presence of heart disease and pulmonary hypertension. .The results were Congestive heart failure 4 in five patients with TM (3.8% age range 25-29 yrs), and in two TI 2.7% age 37-40yrs). Systolic Left Ventricular Dysfunction in patients with TM (8.4%). Lastly they concluded that Regular lifelong transfusion and chelation therapy in TM patients prevented premature heart disease and pulmonary hypertension.15
A Study on spectrum of viral hepatitis in Thalassemic children receiving multiple- blood transfusions. in India, with an aim to investigate the prevalence of infection with hepatitis viruses in children with thalassemia receiving multiple blood transfusions. Sera from 50 children aged 5-15 years were included for the presence of hepatitis A virus (HBV, HBsAG,HAV), hepatitis B virus(HBV, HBsAG, HCVRNA) and hepatitis Evirus(HEV, IgM anti- HEV), IgM anti hepatitis D virus (HDV) was looked for only in HBsAg or Ig M anti- HBc positive sera .No child had evidence of recent HAV OR HDV. Infection. Ig anti, HAV was positive in 12 children. One patient had acute HBV infection. Nine pts were HBsAg positive. HCV infections in 15 cases, 6-HCV RNA positive. The conclusion was thalassemic pts receiving multiple blood transfusions often acquire hepatitis B (20%), C (30%) infections. Recent hepatitis B infections was documented in 10% in this one-point study.16
e. A Study related to Knowledge and Attitude of parents on Thalassemia
A cross sectional descriptive survey was conducted to asses the attitude towards genetic diagnosis in Pakistan; A survey of medical and legal communities and parents of thalassemic children. Five hundred and seventy doctors, One seventy eight medical students, eighty nine parents of thalassemic children were included in the survey. The groups showed considerable differences in their attitudes towards thalassemia. A large proportion of 88.5% agreed to the idea of genetic diagnostic screening, especially the parents of thalassemic children. Premarital carrier screening was favored by 77%. Prenatal screening was most favored by the parents of thalassemic children 94.4%. Likewise a majority of parents of thalassemic children were in favor of abortion in case of an affected
fetus. The parents of the affected children strongly favor genetic screening.17
A structural research tool was developed to asses the current level of knowledge, attitudes regarding thalassemia and thalassemia screening in the Chinese community of Toronto. It was administered by trained interviewers to 160 Chinese health fair attendees and health service users. Statistical software was used for data entry and analysis. Approximately half of the sample had heard of thalassemia and more than 3/4th of the sample wanted to know more about this condition. So, the study concluded that knowledge and awareness of thalassemia in the Chinese community are low. Health Professionals, community educators and the media need to work together to raise community levels of awareness of thalassemia.18

6.3 STATEMENT OF PROBLEM

/ A study to assess the effectiveness of STP on care of children with thalassemia in knowledge and attitude among parents in selected hospitals at Bangalore.

6.4 OBJECTIVES OF THE STUDY

/ 1. To assess the knowledge and attitude of parents on care of children with thalassemia.
2. To assess the effectiveness of structured teaching program on care of children with thalassemia among parents.
3. To find out association between knowledge and attitude scores and demographic variables.

6.5 OPERATIONAL DEFINITIONS

1. Effectiveness: Refers to the evaluation of knowledge regarding Thalassemia among the parents determined by pretest and posttest knowledge scores. Knowledge: Refers to the correct responses of subjects on self administered questionnaire on Thalassemia.
2. Attitude: The responses of subjects in the level of understanding of structured interview schedule on Thalassemia.
3. Children: patients who are suffering from THALASSEMIA.
4. STP: Refers to statistically organized planned teaching program providing information regarding Thalassemia, etiology, clinical manifestations, laboratory test, management, knowledge on taking care of children in their daily life which helps to prolong their lives and efforts to control the complications of the disease.
5. Thalassemia: Thalassemia syndromes are heterogeneous group of hereditary disorders of reduced hemoglobin synthesis.
6. Demographic Variables: Refers to age, sex, education , type of family, number of siblings, family income.

6.6 HYPOTHYSIS

H1 There will be significance difference between pretest and posttest on knowledge and attitude among parents of Thalassemic children.

6.7