INTRODUCTION:

Thalassemia is an inheritedchronic disease of alpha or beta globin biosynthesis. They are associated with significant mortality and morbidity. Chronic blood transfusion with RBC’s improved oxygen levels prolong life for patients with beta thalassemia major, but inevitable side effects, notably iron overload, usually prove fatal by age 30. Each year 8000 children are born with thalassemia in India, which contributes around 10% of the annual world incidence¹. The early onset of symptoms, multiple somatic complaints, frequent blood transfusions and subsequent absence from school, not to mention the overall uncertainty of prognosis make large demands on the emotional and interpersonal resources of the child and the family². It is therefore, not surprising that various authors have reported high rates of psychopathology in children with thalassemia.

The chronic diseases are a theme of extra relevance for reflection on the human living process, considering that, even with the advances in science and technology in the sphere of early diagnosis of diseases and their appropriate therapeutics, which often allow the control of their evolution and cure, this group of diseases promotes organic, emotional and social changes that require constant care and adaptation³.The aim of the management or control of thalassemia is to reduce suffering and improve the quality of life of the individuals; however, the patients are not completely cured. This process requires extensive hospitalizations, even during the diagnosis period with frequent visits.

Children with chronic thalassemia, generally need to undergo aversive medical procedures, hospitalizations, and worsening of their physical condition, and may have their physical and emotional development affected. This may lead to the presentation of psychological maladjustments arising from the disease and from the treatment. Thus, hospitalization permeates their processes of growth and development, modifying, to a greater or lesser degree, alienating them from their families and environment⁴.

Thalassemia presents high indices of morbidity and mortality and has gained a prominent position in the changes happening in the global epidemiological profile⁴. In this sense, thalassemia in children currently constitute a subject of great concern for healthcare professionals, due to their limiting aspects and the consequences of their treatment, even being outpatient treatment, causing distress and suffering for the affected person, as well as their family.

Adolescents with chronic conditions often have alterations triggered in their growth and development process, mainly due to the aggressiveness of the established therapeutic model. In addition to this aspect, they also have the associated risk of disease progression and failure of their spleen and cardiac functions, leading to even more serious complications⁵.

Therefore, it was considered relevant to enquire whether the knowledge that the adolescents with thalassemia have about their illness, and self-care requirements related to it, gives them a foundation for the continuation of the therapy in a way that is appropriate and prevents relapses. Therefore, it is necessary that healthcare professionals must be aware of the need for the investigation of the necessity for self-care of adolescents in order to implement interventions that address the singularity of each specific moment. For the individuals with thalassemia, when they assume an active role in the health-disease process, they contribute to the reduction of recurrent hospitalizations and of suffering resulting due to the disease. Thus, it may be comprehended that in order to promote a better quality of life in their process of adaptation and the course of disease, the guidance of the adolescents and their family is of great value for the improvement of their clinical condition and control of the disease⁵. Given this situation, it is essential to investigate the self-care necessity in adolescents with thalassemiaas a basis for the implementation of measures that promote self-care within the human necessities of these individuals.

Thus, it was asked: what are the deficits in the self-care requirements in adolescents with thalassemia? Is it possible to develop interventions that minimize these deficits? Given these questions, this study aimed to investigate the self-care requirements associated with thalassemia, in adolescents, in light of the theoretical framework of Orem, to identify nursing diagnoses in the self-care deficits and to develop interventions, with the adolescent, at the moment of the identification of the deficits in the self-care requirements.

METHODOLOGY:

In order to investigate the self-care deficits in adolescents with thalassemiaan exploratory-descriptive qualitative approach was used. This method was chosen because it allows the revelation of little known social processes relating to particular groups, as well as providing greater familiarity with the object of study in order to make it explicit.

The study was conducted in thethalassemia ward of a teaching hospital, located in Bangalore, during the period of September to November 2013. The universe considered for this study was adolescents diagnosed with thalassemia and treated. For the selection of the participants, the following criteria were considered: to be present for consultation during the period of data collection, to have a history of thalassemia for more than one year, female adolescents aged between eight to 18 years and accompanied by the caregiver. Purposive method was adapted to select 10 female adolescents for the study.

The study was conducted taking into consideration the ethical aspects of research involving human beings, as recommended by Ethics Committee of the Hospital. Informed consent to participate in the study was obtained from the legal representative of the adolescents.

Data collection was conducted through means of semi-structured interviews with the adolescents and their caregiver and it might be relevant to mention that during the interview the caregiver was asked to confirm the data related to the time of diagnosis and the family income. The data collection instrument was based on the General Theory of Nursing, since it aimed to investigate the self-care requirements in the developmental self-care and health deviations associated with thalassemia in adolescents, in light of the theoretical framework of Orem.

The data were recorded using electronic media (MP3), with the aid of a field diary to avoid forgetfulness, considering recording criteria such as clarity, reliability and legibility. Before the data collection began, the researcher was present at the thalassemia ward, at the moment that preceded the consultation with the haematologist in-charge, which was used to initiate contact with the adolescent and caregivers. This contact was intended for the introduction of the researcher and presentation of the prior informed consent of the study.

Even before the consultation with the haematologist, the interview was initiated with those who had agreed to participate in the investigation. At the same time the interviews were conducted and the self-care deficits of adolescents with thalassemia identified, nursing interventions were implemented, which were directed towards the adolescents and, consequently, their caregivers, since some interventions needed for comprehension of by caregivers for their involvement.

The interviews were transcribed and, using the notes in the field diary, the history of the adolescent was constructed, and used subsequently to augment the discussions regarding the self-care requirements identified during the interview. Next, the nursing diagnoses related to each deficit was identified, using NANDA nursing diagnostic criteria. After the identification of the diagnoses, further interventions were formulated; with a view to contribute to the care of other adolescents. The NANDA nursing diagnosisis a system of unified nursing language, instrumental terminology for the nursing practice that facilitates cross-combination of local terms with the existing terminology⁶.

RESULTS AND DISCUSSION:

The study was conducted with 10 female adolescents, and their age ranged from 8 and 13 years. The study was conducted during the subjects’ visit to the hospital to confirm the diagnosis and to start the treatment and, from then on continued follow up during monthly or bi-monthly outpatient consultations; the schedule established due to the progression or control of the disease. The adolescents had a confirmed diagnosis of thalassemia. The duration of treatment and monitoring of the adolescents was from a minimum of four years to a maximum of 10 years. All of them were from low income families.

Considering the described self-care requirements, to assess the history of the adolescents, it can be inferred that the self-care requirements of these subjects are not found in isolation, on the contrary, they are interlinked, since the children experience a pathological process in a period of life where they go through physical and psychological changes, in addition to expanding their relational bonds.

Universal self-care requirement

Through the analysis of the histories of adolescents the nursing diagnoses in the following universal self-care requirements were identified: demand/maintenance of a sufficient intake of food (risk for nutritional deficit), alteration in the promotion of the functioning and development of the human being/potential and in the limitation and desire to be normal (negative self-image).

The universal self-care requirements are essential for all human beings and are present at all the phases of the life cycle, considering that they are associated with maintaining the integrity and functioning of the human body and general well-being. These requirements correspond to: sufficient inhalation of air and ingestion of food and water, provision of care associated with the processes of elimination and excretion; maintaining a balance between activity and rest and between solitude and social integration; prevention of damage to life, to the functioning and to the well-being; promotion of human functioning and development in social groups according to the potential, known limitations and the desire to be normal⁷.

Food intake is one of the factors responsible for adequate growth and development during childhood. For this reason, the risk for nutritional deficit is a nursing diagnosis that should be monitored, in order to minimize the risks of complication. The need for feeding involves restrictions and re-adaptation to the lifestyle of an adolescent affected by thalassemia, which can compromise the nutritional status of the child. Furthermore, rapid intervention in nutritional support can minimize the impact, leading to clinical benefits, even in the absence of an effective treatment for the underlying condition.

By analysing the histories it can be perceived that the adolescents and their caregivers were aware of the need to follow a special diet. However, this adaptation in the lifestyle was not always practised. Therefore, it is important that these adolescents and their family members have accurate information regarding the importance of following the diet indicated, with the need for dietary restrictions and the problems arising from failure to follow this guidance being made clear. Given the above, nursing interventions are needed, such as dietary guidance for the adolescents with thalassemia and their caregivers, using clear and easy to comprehend language, encouraging the development and practice of self-care, which would enable order to make them active members involved the health-disease process.

The self-image is the view that each individual has of himself, including the shape, size, body proportions, the feelings in relation to it and its parts according to their individual judgment. The self-image emerges in the interaction of people with their social context, stemming from established relationships with others and oneself, being regarded as the knowledge and recognition that we have of ourselves, how we sense our potentials, feelings, attitudes and ideals, the most realistic possible image, finally, what we make of ourselves⁸.

Adolescents affected by thalassemia experience many limitations and constant adaptations, beyond the necessity of submission to pharmacological therapy. Therapeutics in a majority of cases causes reactions reflected in the bodily image. In this way, the self-image may be compromised and reflected in the feelings of interaction with others, especially in adolescents⁹.

The compromised self-image in adolescents, noted during this study, was identified in the statements of discomfort with their own bodies, when they had abnormal facial features, short stature or perceive themselves as having a smaller stature than others of the same age group. This perception of self can be reflected in the quality of life and in the self-esteem of these children, since they are at the stage of psychological development and in the process of formulation of this perception¹⁰.

Fewer studies, however, have examined the challenges faced by caregivers/ parents of children affected by chronic medical illness. In their review, Choy et al (2012) describe outreach strategies and offer guidance for providing sensitive education about thalassemia and the related self-care satisfaction strategies. The authors based their suggestions with the experiences and addressed issues extrapolated from the existing literature about self-care requirements in adolescents with thalassemia¹¹.

The study conducted by Mayo et al (2011) showed similar findings as the present study viz., certain adaptations for adolescents with thalassemia are required such as following a diet regimen, adherence to the treatment. However, this adaptation in the lifestyle was not always adopted. Therefore, it is important that nurses should provide adequate information to the adolescents and their family regarding the importance of following the diet indicated, with the need for dietary restrictions and explain the problems arising from failure to follow this guidance ¹².

Aiming to reduce the losses originating from the perception of the self-image, the nurse can inform the adolescent and the parent about the effects that the disease and the pharmacological treatment can cause in the life of the child, especially those that are visible to their eyes and the eyes of others, such as bodily changes. Furthermore, it is important to encourage the adolescent to share their fears, anxieties and dissatisfaction about their image with their caregiver and with the professionals responsible for their monitoring.

Requirement of developmental self-care

Deficits in the requirements of developmental self-care were confirmed, with the identification of the nursing diagnosis of inadequate growth, in the adolescents involved in this study. The requirements of developmental self-care correspond to the demands that occur during the adaptations related to normal situations or to crises during the life cycle, such as infancy, adulthood and aging, pregnancy and childbirth, situations of marriage, divorce or separation, and situations of changes in the life course.Infant growth constitutes a continuous process with specific characteristics in each phase of life which are influenced by various factors, which relate to biological, genetic and ethnic determinants; and the exogenous, related to the nutritional, cultural, environmental and social factors¹³.

Among the problems triggered by these factors, thalassemia as a chronic disease is highlighted, because it provokes significant physiological changes in the body and in the production of substances that help the growth spurt, such as growth factor, or the hematologic function, such as erythropoietin, which stimulates the formation of erythrocytes and with this there is improved cellular oxygenation and, consequently, provides adequate nutrition for the cell. Because of this, the earlier the children present haematological alterations, the greater the alterations in their rhythm of growth, weight/height ratio and other development¹⁴.

Thalassemia affects the growth of children and adolescents compromising the organs directly and indirectly involved with the disease, due to chronic anaemia, low oxygen carrying capacity of the haemoglobin, associated complications, infections and therapeutic side effects¹⁵.

Ratanachadawan et al (2010) in their study, on self-care need for children with chronic diseases, recommended that the need for developmental self-care is primary than other self-care needs because other self-care needs could be achieved through being a normal or near normal individuals. Other studies have also emphasized on the need to maintain the optimal level of developmental care in children with chronic disease¹⁶. In this aspect, it is important to direct the nursing actions towards adolescents with thalassemia with a view to prevent inadequate growth. Accordingly, the guidance to adolescents and their families should emphasize on treatment and nutrition. It also requires the professionals to involve themselves in the process, so that they feel co-responsible for the development and growth of children, with minimal compromise.

Self-care in the health deviations

During this study, it was realized that the effective control of the prescribed therapy is not always achieved, as exemplified in the statements of some children, where they related that they do not properly follow the suggested diet and do not observe the effects of treatment because of their low socio economic status.

The requirements of health deviations related to functional and genetic problems as well as to the clinical diagnosis and mode of treatment. These occur when individuals find themselves in situations where they do not react to an event of loss of sensation or where they do not control their functions and potentials of self-care, for example: when they are sick, have accidents, are incapacitated or are under medical diagnosis or medical treatment. Considering these view points, subsequent nursing diagnoses were identified: Ineffective management of therapeutic regimen related to not following the recommended diet and deficient health knowledge related to not understanding the precise diagnosis¹⁷.

The ineffective management of the therapeutic regimen occurs when the individual, affected by a particular acute or chronic disease, is subjected to treatments that require alterations in functioning, or in the previous lifestyle, and cannot integrate these adjustments into their routine, in addition to not staying attentive to the results and/or discomfort presented. The Health Belief Model and other tools have identified many personal variables associated with adherence, such as, the patient’s perception of susceptibility to disease, severity of the illness and of the efficacy of the proposed therapy¹⁸.

The other critical factor to consider while discussing adherence by adolescents is during their developmental stage. Adolescence is a crucial time for physical, pubertal and cognitive maturation, in addition to psychosocial changes, including identity formation and the development of independent social relationships. During these years, the child will move from complete dependence to a more autonomous lifestyle. All these changes arise from the use of medication, alterations in eating habits, and inclusion of exercise in daily life, among others¹⁹.

Adolescents with a chronic disease should be aware of the need to follow the treatment plans that are being submitted avoiding, in this way, some harm that may arise, such as an exacerbation of the disease and difficulties to achieve a cure or to stabilize it. For this to be achieved, the adolescent must have the support and guidance of the parent/care giver, who should also receive support from the healthcare professionals. Health education is fundamental to nursing care, as it may determine how individuals and families are able to perform the behaviour that leads to self-care²⁰.

Healthcare professionals, by identifying the factors that cause or contribute to the impairment of the efficacy of this control, can collaborate by providing simple and direct instructions to the adolescent and caregiver on how to control the therapeutic regimen, making them active participants for patient care. Deficient health knowledge was another nursing diagnosis identified in the self-care requirement in the health deviations. It can be said that this relationship arises from the lack of control of the disease and the absence of the family members in the decision-making process about the nature of the disease and treatment.

Knowledge about the health-disease process provides a feeling of greater control and responsibility regarding health, encouraging one to assume an active role in the management of their health. It is worth noting that it is necessary that healthcare professionals understand how the client interprets their illness, as it is the client who deals with it daily and learns the pattern of their symptoms²¹.

The adolescent with thalassemia and their caregivers accumulate knowledge about the chronic disease and how to perform self-care in diverse ways. This knowledge is constructed from popular beliefs, personal experiences or experiences of others who are around them, as well as from information obtained from healthcare professionals and the media²¹. It is necessary to remember that their socioeconomic and education levels directly influence this process. Therefore, it is necessary for care actions to be performed with flexibility, through interactive dialogue that comprehends the process experienced by the family.

Considering that Orem's theory proposes the participation of the individual in the healthcare process in a conscious and enlightened way, because the adolescent may not have this capacity. It is the duty of those responsible for the care of the adolescent to assume the condition of therapeutic self-care agents. In this process, the agents have the function of providing the necessary self-care for themselves or for another person, receiving cultural and experimental support influences. With the nursing actions being directed more towards guidance and clarification for the caregivers and adolescents, the interventions will be performed in the educational supportive system, which, according to Orem's theory, is applied when the client needs nursing assistance to acquire knowledge and skill.

Final considerations

Orem's theory helps to identify that thalassemia provokes significant modifications in the life of the adolescent and their caregivers/family members, often requiring from these people radical changes in their lifestyles. However, it is necessary that they possess adequate tools to deal with the situations provoked by the disease, which corresponds to availability information, guidance and materials to assist in the observation and control of the disease. Information regarding the conditions of health-disease is essential for the individual with thalassemia and/or for their caregiver. Thus, they may become responsible for the care in the individual and social context, being able to undertake important decisions about health behaviour that are more favourable, leading to a better quality of life.

The study showed that, among the modifications necessary for adolescent are, the differentiated diet is the most difficult to follow. It was perceived that alterations in the self-image and the growth of the adolescent are reasons for discomfort during the experience of the disease. Furthermore, it was identified that, although the information related to the diseases is easily accessible and provided by the healthcare professionals, there were still people who did not know the chronic condition of the adolescents in its entirety, a situation that is perceived to be related to the level of literacy. It can be inferred that the way that this information is being transmitted by the professionals is inadequate for comprehension by the families.

The guidance regarding self-care dispensed by the nurse to adolescents and their care giver/ family members must be in a language which could be easily understood, considering the totality and singularity of these individuals. In this way, it could be possible to evolve ways and means for the adolescents with thalassemia to be co-responsible in their care process, able to cope with the help of their caregivers, with changes in their life for the pursuit of their wellbeing.

To implement health education, it is important that favourable environment is provided for exchange of information and that the nurse uses and develops sensitivity to meet the care needs. Thus, there are tools to identify the needs of each individual, allowing the choice of effective methods to reduce the cultural distance between adolescents, their caregiver and the nurse.

SUMMARY AND CONCLUSION:

This disease is inherited with significant mortality and morbidity. It is difficult for children / adolescents to live with alterations required by the disease. As self-care deficits do occur, care givers need to take up responsibility of attending their needs, encourage for activities of daily living.

One of the limitations of the study was the limited number of patients and their parents/ caregivers. However, the systematic study has opened up a range of possibility for future study aimed at educational strategy for developing horizontal educational programme. There is a need to exchange values for evolving suitable guidelines for nursing care process.

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Received on 31.05.2014 Modified on 20.06.2014

Int. J. Adv. Nur. Management 2(2): April- June, 2014; Page 55-60