SOCIALIZATION OF CHILDREN AND ADOLESCENTS WITH CYSTIC FIBROSIS: SUPPORT FOR NURSING CARE

O presente estudo tem por objetivo conhecer o dia-a-dia (escola, trabalho, atividades de lazer e interações com a família e amigos) da criança e do adolescente com Fibrose Cística (FC), a partir de suas próprias vivências, bem como identificar situações que possam interferir nesse cotidiano. Os sujeitos da pesquisa são crianças e adolescentes portadores de Fibrose Cística em acompanhamento num hospital-escola do interior do estado de São Paulo. Trata-se de pesquisa com abordagem qualitativa, com coleta de dados empíricos realizada mediante análise de prontuários e entrevista aberta. Dos dados, emergiram os seguintes temas: conhecimento equivocado sobre a doença; preocupação com a auto-imagem; busca pelo autocuidado e esperança de melhorias no futuro. Os resultados evidenciam as repercussões da Fibrose Cística no processo de socialização desses pacientes, salientando a importância dos profissionais de saúde conhecerem essas demandas e incorporarem-nas ao plano de cuidados, visando a intervenções efetivas que promovam o crescimento e o desenvolvimento infanto-juvenil.


INTRODUCTION Cystic Fibrosis (CF), also known as
Mucoviscidosis, is a chronic, autosomal, recessive, hereditary and still incurable disease.The gene of Cystic Fibrosis is located on the long arm of chromosome 7 and is responsible for producing the CFTR membrane protein, which conducts ions between intra and extracellular domains.Incidence levels of the disease amount to 1 in every 1,500/2,000 in the south of Brazil.This is similar to levels in the European

Caucasian population. Rates decrease in other
Brazilian regions, that is, 1 case for every 10,000 births (1) .
As a result of deficient ion transport in cellular membranes, thick secretions are produced from the exocrine glands, which end up obstructing the canaliculi and ducts through which they are secreted, preventing their arrival at their place of action (1) .As there are various mutations in the CF gene, signs and symptoms can be more or less intense, although the upper airways and pancreas are always affected (2) .
Cystic Fibrosis can be diagnosed through different methods.The most common one is the Sweat Test.It is confirmed by two measurements with chloride concentrations superior to 60mEq/l (1) .In general, the mother suggests the probable diagnosis to doctors by mentioning that, when she kisses the baby's forehead, the kiss "tastes salty" (2) .
In Brazil, Decree GM/MS No 822 by the Health Ministry, issued on June 6 th 2001, established that neonatal screening, better known as the "Heel Prick", which was only used to detect congenital diseases like Hypothyroidism and Phenylketonuria, would also allow for the early detection of other diseases, including Cystic Fibrosis (3) .Although this Decree has been in force since its publication, many health institutions still do not perform complete neonatal screening because they have not been qualified by the Single Health System (SUS).This qualification requires compliance with all steps of the process, ranging from neonatal screening until the follow-up of detected cases, as well as the availability of a multidisciplinary team.Nowadays, only few private and public hospitals, considered to be centers of excellence for diagnosing and treating these diseases, perform complete screening.A complementary Decree (4)  interaction with their environment through repeated and varied actions, which allows them to get familiar with it and explore it better; in this process, a preceding phase is needed for the development of the next one.In the case of children with chronic disease, depending on which phase of life symptoms start to appear in, the impact on their cognitive, emotional and social development will happen in a particular way, thus impeding future developments (6) .
Socialization is defined as "a broad and consistent introduction of an individual into the objective world of a society or a sector of it".It can be ranked as primary and secondary socialization.
The primary type is what the individual experiences during childhood and through which (s)he becomes a member of a society; the secondary type is any subsequent process that introduces the already socialized individual into new sectors of society.The family is responsible for the child's first insertion into the objective social world, to the extent that it offers the learning of cultural elements, such as language, habits, uses, customs, values, standards, behavioral standards and attitudes and, mainly, promotes the formation of basic personality and identity structures (7)   .
With respect to the socialization of children and adolescents of school age who suffer from chronic diseases, these are at the intersection of the health and education systems, in view of the separation between both in most countries' teaching institutions.
Hence, there needs are not integrated, creating a deficiency in one of the sectors, usually the educational side, as health is urgent.The lack of communication between these two sectors and educators' lack of knowledge are the main educational constraints for these students (8)   .
The problems these children experience in school are directly related to the collateral effects of treatment, to signs and symptoms of the disease and to feelings like rejection, stress and isolation out of shame from their colleagues (8) .During crisis periods of the disease and due to constant hospitalizations, children and adolescents with CF miss school and often cannot pass to the next year because of their absences, which discourages them so study (2)   .
To the extent that the life expectancy of children and adolescents with CF increases, issues related to choosing a profession, relationships and reproduction become more intense and expectations for the future start to appear, which can create anguish, depression and often treatment abandonment.It is during this transition period from childhood to adult age that feelings of self-control, immortality and freedom of choice come up.Treatment abandonment occurs because health care, which used to be attributed to another person, like the mother for example, is not attributed to the patient him-/herself (2) .
Furthermore, during this phase, new social attributions emerge, such as work or the continuity of school commitments which, together with therapeutic commitments, lead to a full agenda and exhaustion to perform self-care, which may become less frequent or relegated to the background (9)   .
In spite of their turbulent life and constant changes in their health state which, in turn, require changes in the structure of care and in the family nucleus, most relatives of children and adolescents with CF and these patients themselves consider their lives as normal.This phenomenon is known as normalization and occurs in stigmatized groups like the chronically ill (9) .
In this sense, concern about these children and adolescents' quality of life has also been a focus of interest for researchers in the area.Thus, the Cystic School.This questionnaire will make it possible to analyze the impact of treatment and the disease itself on the quality of life of Brazilian CF patients (10)   .
The aim of this study is to investigate how the socialization process of children and adolescents with CF is occurring, based on their own experiences.
This research is justified by the possibility to identify, in the daily lives of these patients, aspects that need nursing interventions, with a view to improving their quality of life.

METHOD
This is a descriptive and exploratory study (11) .
The answers to the research questions were sought in the qualitative approach, due to the characteristics of the study object and the proposed objective.We believe this approach is particularly valuable, as it permits discovering the meaning of events, social practices, individual perceptions and actions (12) .The submitted protocol contained the free and informed consent term, which described the research in simple language (13) .Before the interview, this term was read and discussed with the parents, children and adolescents.On this occasion, any doubts were clarified and, after the children and adolescents had agreed to participate, both parents and participants signed the term.
The study institution is a teaching hospital and reference for care delivery to children and adolescents with CF, located in the interior of the state of São Paulo.Study participants were eight (8) children or adolescents with a diagnosis of CF, in the age range from 7 to 18 years, male and female, who were under clinical and hospital follow-up at the study hospital.
The choice of this age range was due to the fact that these patients are in the phase of logical thinking, and can therefore communicate their ideas verbally and give meaning to the experiences (6)   .We defined the number of participants when data provided us with sufficient support to understand the study phenomenon.
We used open interviews as a data collection strategy, due to the fact that this technique gives access to descriptive data in the subjects' language and because it allows the researcher to interactively develop an idea about how subjects interpret aspects of the world (12) .
The interview was held at the participants' homes (6)  Data interpretation started on the basis of the fully transcribed tapes.We analyzed each set of data (interview, field diary notes and patient chart data) per part, seeking the codes, that is, phrases or words that give meaning to the information contained in empirical data (12) .Next, we grouped the codes according to their similarities, which resulted in the themes.The children's and adolescents' reports were identified according to the order of the interviews (E.1 to E.8).For each situation, we indicated the age because we believe this is an important indicator (E.1, 18 years).Data evidenced that scientific words like genetics, chromosome and hereditary appeared in the reports quite frequently, although without any actual meaning, emphasizing the technicist language of the information they received.For example: I explained that it's a genetic disease..

.they asked what it was and I said it was a genetic disease! (E.4, 7 years).
A communication barrier is created between professionals and patients, due to the use of technical and abstract language.In many situations, health professionals overestimate patients and family members' knowledge, based on the premise that they master their scientific universe.

Concern about self-image
It is when they start school that children and adolescents with CF start to perceive they are different from their colleagues (2) .Sometimes, these differences are evidenced by their own colleagues, causing constraint and shame in patients, according to the following reports:

years). I cough a lot and take a lot of drugs, the others [children]
only take them when they are ill, I take them all the time (E.4, 7 years).
In the situations the children experienced, we observed they felt uncomfortable about the image, although as something that cannot be changed.
Adolescent participants, on the other hand, revealed to be more concerned about their body image with respect to changes and their possible interventions:  But I don't take the drug in school, I don't eat when I'm there...I'm ashamed!(E.3, 17 years).I don't take medication in school, there's no reason why, because sometimes I have a snack, so I'd have to take it with the pancrease, you know?But I don't take it, no, just a snack, a juice, I don't think it's really necessary to take it.(E.1, 18 years).

Hope for improvements in the future
As CF is a genetic disease, the only viable cure is gene therapy (1) .Nevertheless, the interviewees' hopes were more directed at new drugs and therapies.

DISCUSSION
In relation to the theme of mistaken knowledge about the disease, communication between the health team, patients and family members is fundamental to understand the observed phenomenon.The professionals' world is based on science and technology and their language is basically constructed in this rationality.Families, children and adolescents, on the other hand, are immersed in changes in their daily lives as a result of the disease and their interactions are supported by their affective life.As language is one symbolic communication form, symbols do not always have the same meaning for families and professionals (14) .Hence, as mentioned above, words whose meanings seem to be understandable in the eyes of professionals may be empty, or have an obscure sense for children, adolescents and their relatives.
The individuals' disease and development cross several times during the lives of chronic patients and the cognitive domain, that is, how they understand and assess the disease and its stressors, will determine how they will handle things when the disease is interfering in their lives.When these individuals are children, the meaning of the disease will depend on what is said to them, on what they experienced and their developmental abilities (15)   .Thus, the children's and adolescents' knowledge about the disease will be determinant for them to develop positive coping strategies, which will minimize the stressors that will naturally come up in their lives as chronic patients.
Concern with self-image reflects social processes; one older one, called normalization, and the other, contemporary, called the cult of the body.
Although distinct, in the case of children and adolescents with Cystic Fibrosis, both are used with the same purpose, the search for normality.
In spite of individual manifestations, stigma and discrimination are social constructions.Stigma is defined as "an attribute that is deeply depreciative and that, in the eyes of society, serves to discredit the person who has it".Stigmatized persons are seen as possessing an unwanted difference, thus, stigma appears in society through differences, deviations.
Discrimination, on the other hand, is an act of ethnocentrism in society and means not liking what is different (16) .Hence, normalization appears as a strategy to cope with the stigma, to the extent that, through actions, people attempt to mitigate the difference and turn its existence closer to what is normal (9) .
Before they went to school, many CF children of school age did not have an idea about the exact dimension of their differences in relation to other children with the same age.In school, they perceive more and that they take drugs frequently and in greater quantities than most of their colleagues (2) .
Children of school age need to feel equal to their colleagues.In this age range, they perceive the physical differences and distinguish deviations from normality.Children considered "normal" indicate the defects of others who are "different" from them, causing shame and constraints (17) .When problems related to the disease and treatment appear during childhood or adolescence, social adjustment problems are expected.When children and adolescents with chronic disease do not know the meaning of their disease and are different from the others, their acceptance by their colleagues is put at risk (8) .Even if school appears as an obstacle for children and adolescents with CF, it can help, providing experiences that can increase their self-esteem, such as the development of skills and insertion into a healthy environment (2) .
The normalization process permeates all phases in the lives of families and patients with CF, although the strategy used to maintain it varies according to the phase of the disease.Besides this coping mechanism, due to the trajectory of the disease, routine care and the fact that it is a congenital disease, families and patients do not have any experience with another type of life and consider their life as normal (9) .
The normalization process also includes socalled problem-solving coping strategies, which use specific actions to solve stressors, which are also specific for the disease.Normalization is observed by defining the child and adolescent as normal, not different from its healthy peers, when they establish routines with the treatment and self-care.The normality attribute indicates the formation of a cognitive scheme, through which the child starts to interpret his/her disease-related experiences as something natural and familiar (15) .When they search for self-care, children and adolescents with CF once again may be recurring to normalization, through the problem-solving strategy, which also promotes the search for knowledge and treatment adherence.This strategy is based on the organization of care to decrease the disease's impact on their daily lives (15) .In a study of adults with CF, many patients mentioned that they did not like to perform self-care every day or many times during the day as, this way, the disease becomes increasingly present in their lives, filling their agenda more than they would like to.However, they performed self-care because they were afraid of the disease's evolution (9) .
Particularly for adolescents, self-care is a crucial component in the transition from pediatric to adult care, constituting a fundamental instrument to develop self-esteem, mainly for patients who depended on their parents' care for a long time (15) .
In reporting their hopes for improvement in the future, related to therapeutic measures, the children and adolescents who participated in this study restricted their anxieties to less complex issues and did not quote the cure of the disease, perhaps due to a lack of knowledge about the implications of a genetic disease.However, participants' greatest hope was related to the dissemination of the disease by communication channels, school and associations.This result is especially important for this study, as it emphasizes the need for better structured social support to this clientele.
One of the coping strategies for children and adolescents with chronic disease is the search for social support from friends, family and professionals, as they consider this is one of the most significant ways to control stressors caused by the disease.Social support can be of help in different phases in chronic patients' lives, whether by providing emotional support, solving disease-related questions and giving care and distraction in difficult periods.In most cases, this support is provided by the parents.However, when friends give support, the range of socialization , lung symptomatology manifestations represent the highest morbidity and mortality rates.The thick mucus clogs the lungs and becomes a culture medium for bacteria and microorganisms, causing repeated pneumonias, bronchiectasis, pneumothorax, among other complications.One other big complication of Cystic Fibrosis is that it affects the digestive tract, especially causing pancreatic insufficiency, which impedes the production and/or sending of digestive enzymes to the digestive tract, causing poor digestion.This problem is mainly due to the non-absorption of fats, which causes steatorrhea and protein-caloric malnutrition in children and, thus, affects their weight and stature development.Reproductive organs and liver can also be affected

Fibrosis
Questionnaire was developed in 1997.In Brazil, the validation of this instrument is being conducted by Prof. Tatiana Rosov, from the Department of Pediatrics and Rehabilitation of the Federal University of São Paulo -Paulista Medical or at the clinic (2) and consisted of two parts: the first part, for identification, gave us information about sex, age, education level, profession/occupation, work place and origin.The second contained two guiding questions: 1.Tell me what you know about your disease; 2. Do you think you are different from your friends / acquaintances who do not have Cystic Fibrosis?We looked for complementary data on the patient charts, when we examined the diagnosis data and therapeutic scheme.Moreover, we used a field diary, where we registered the impressions obtained from each interview, giving special attention to non-verbal communication and family relations.
analysis allowed us to identify 4 themes, which are directly or indirectly reflected in the socialization process.These are: Mistaken knowledge about the disease Most interviewees could not explain exactly what Cystic Fibrosis is.Many of them mixed up information, like in the following examples: It's genetic.I know more or less, I don't know very much.I know it's more or less similar to Down's Syndrome.It's from the same chromosome, I think it is, but I really don't know for sure.(E.1, 18 years).I know it's without organ there and that you need drugs for digestion... (E.3, 17 years).Other participants associated the disease with prevailing signs and symptoms, as well as with specific drugs: The disease I have...I cough a little when I have quite a lot of catarrh.... (E.4, 10 years).Cystic Fibrosis is a thing in the lungs.Then, each time you're going to eat you have to take the enzyme, but then you have to do the PULMOZYME ® [inhalation medication], if not it turns into pneumonia.(E.6, 10 years).
Although they considered the therapy intense and exhausting, children and adolescents in this study turned to self-care as a strategy to control their activities, since this allows them to plan their daily life better and minimize the impact of the disease, especially in the school environment, as mentioned below: So, we try, seeing the possibilities and the time I have available during the day to do it.I don't live in favor of Cystic Fibrosis, it lives with me and I take it along.If I stop my life to live it, I'll become completely ill!(E.2, 18 years).I control theSocialization of children and adolescents... Pizzignacco TMP, Lima RAG.Rev Latino-am Enfermagem 2006 julho-agosto; 14(4):569-77 www.eerp.usp.br/rlaedrugs myself, because I know my times, so I don't get confused.
The dissemination of new treatments in the media has contributed to keep up these hopes, for example: Because my mom said that, in Europe, they are making a drug to cure...they're going to cure it.(E.5, 10 years).
There's that vaccine[against Pseudomonas], which is not available in Brazil yet.(E.4,7 years).Yes, the trend is for things to get better and better, it's better already!Over here, in Brazil, mucoviscidosis patients used to be 30 years old at most.(E.2, 18 years).Yes, I hope it improves because of the drug, which is evolving.(E.3, 17 years).
Since the 1980's, the world has been observing the growing valuation of muscles and healthy appearance, encouraged by the industrial increase in products that indicate beauty and health at the same time.As a result, this stimulates the cult of the body and healthy appearance and excludes people who do not follow these standards.This may occur with Cystic Fibrosis patients, whose disease is physically visible.
changes like clubbing of the fingers and barrel chest, accentuates this difference, and this makes them increasingly turn to normalization processes.The body-related stigma is also present in other diseases, and is determined by the way society classifies physical normality and values corporal attributes.