Nursing interventions in monitoring the adolescent with Cystic Fibrosis: a literature review

ABSTRACT Objectives: to search for nursing interventions focused on the improvement of quality of life and promotion of self-care of adolescents suffering from the Cystic Fibrosis. Method: literature review. The inclusion criteria were: primary studies and studies with interventions developed by nurses in the adolescent population with Cystic Fibrosis, using Portuguese, Spanish, French and English with no time limit, and supported by the databases Scopus, Web of Science and CINAHL. The search expressions were: nursing AND care AND adolescent AND "Cystic Fibrosis" AND ("quality of life" OR "self-care"). Results: a total of 59 articles was retrieved; 8 matched the criteria chosen. Nursing interventions targeted at adolescents with Cystic Fibrosis and their family members were identified. These interventions were organized according to the nurses' role, namely caregiver, coordinator, counsellor, researcher, trainer and care partner. Conclusions: nursing interventions targeted at following up the adolescent during the entire therapeutic process, involving the presence of parents/significant others, since both the adolescent and family have to be responsible for self-care. Healthcare professionals should be capable of identifying the specific needs of patients with chronic disease and their family, permitting a better understanding and adaptation to the health-disease transition process.


Introduction
Adolescence is characterised by major fluctuations, particularly in physical terms -of rapid and accelerated growth until maturity; at a cognitive level -from the abstract to the fully established capacity for abstract thinking; in terms of identity -where the body image causes a variety of concerns until it becomes clearly defined; regarding relations with parents -from the definition of boundaries, through major conflicts until the achievement of emotional and physical separation; in terms of relationships with peer groups -search for the acceptance of friends, fear of rejection, to an interest in individual friendship to the detriment of the group; sexuality -fluctuating between self-exploration and the formation of stable relationships with others; and major mood swings until the establishment of greater emotional stability in terms of mental health (1) .
If adolescence may be described as a troubled life cycle, Fibrosis is transmitted in a recessive autosomal form, meaning that this disease is passed on by both parents of the child. Various authors refer to an incidence that fluctuates between 1:2000 and 1:1500 of newborn infants in the European population. It is less frequent in Africans and rare in Asians (2)(3) . Cystic Fibrosis affects various organs and is characterised by the dysfunction of the exocrine glands. The secretions are very thick, due to alteration in the functioning of exchanges of water and salt in the exocrine gland cells. These secretions will cause obstruction in various organs, and manifest themselves in the lungs, pancreas, intestines, reproductive system and sweat glands. Therefore, patients can present various clinical manifestations, isolated or together in relation to the affected organ: chronic cough, recurrent pneumonia, low weight, deficient food absorption, pancreatitis, meconium ileus and elevated sweat chloride (2)(3) . Increased   Association, recommends that a nurse should "work in partnership with the adolescent and family/significant person, in any context in which the adolescent is found (hospitals, continuous care, health centres, school, community, home, …), so as to promote the highest status of health possible, provide care to a healthy or sick adolescent and provide education towards health as well as identify and mobilise resources to support the family/significant person" (8) .
It has become necessary to carry out research on published scientific articles, which identify nursing interventions that improve the quality of life and selfcare of the adolescent with Cystic Fibrosis.  However, eight repeated articles were found in the three databases, thus resulting in 14 final articles for analysis. From these 14 articles, it was not possible to have full access to 6 articles, which was necessary for a complete analysis. Later attempts were made to contact these authors but one had a wrong email address, three articles required payment for full access, one author was already deceased and one did not reply.

Method
In Figure 2, the article selection process is summarized.
In order to identify the nursing interventions provided to adolescents with Cystic Fibrosis, eight articles were analysed with respect to: study objectives, research design, results (nursing interventions) and conclusions.
The articles were assessed by another researcher, independently, and subsequently classified according to: "type of study"; "objectives", "nursing interventions" and "main conclusions".

Results
The 8 articles included in this review show different research methods: two studies are descriptive/ reflexive; one is exploratory/descriptive; one is a case study; one is a survey; one is correlational; one is a content analysis and one is a qualitative update. The analysis of the articles relative to the study design highlights various particularly salient aspects, such as their qualitative method and objectives, which refer to the area of knowledge and understanding (description, understanding, reflection and exploration).

Nursing interventions targeting adolescents
with Cystic Fibrosis and their family members were identified. These interventions will be displayed and organized according to the nurses' role, namely caregiver, coordinator, counsellor, researcher, trainer, and care partner. Nursing interventions Support at a psychological, social and emotional level to the patient and family; interventions at the moment of diagnosis, at the first administration of intravenous medication; at the beginning of school, college, employment; in care to be provided at home (intravenous medication, enteral feeding), in transition phases, in genetic counselling, in infertility treatments, in heart/lung transplants, in dependence on oxygen and ventilation support, in terminal care and death; education of the patient, family and society; in linkage, coordination and communication between the work team members and the patient's advocate for the defence of his interests.

Conclusions
The Cystic Fibrosis specialist nurse is part of the multidisciplinary team.
In the future, various areas should be explored, namely in the direct provision of care (there should be one fulltime nurse for every 50 patients with Cystic Fibrosis at specialised attendance centres); in the construction of a consulting model; in research; in counselling, psychology, education and palliative care.

Title and author of the article Playing for time: adolescent perspectives of lung transplantation for Cystic Fibrosis. Christian, D'Auria and Moore 1999
Type of study Case study

Objectives
Understand the events implied in the adolescent's decision-making in relation to carrying out lung transplantation.

Nursing interventions
Identification and planning of interventions which help people with Cystic Fibrosis, in a final state of pulmonary disease, to build a promising future; communication among all team members.

Conclusions
The study identified nurses (both in clinical practice and research) as the members of a multidisciplinary team in a key position to ensure the follow-up and psychosocial support to the individual, during the waiting period before carrying out a transplant and during the period of physical decline.

Type of study Descriptive/ reflexive
Objectives Reflect on the creation of health services that meet the needs of patients with Cystic Fibrosis.

Nursing interventions
Creation of measures to proceed with the transition of these youths to adult services; family support and offer of information, coordination of the transition process and communication; guidance plan in relation to the transition date; preparation of an educational programme; coordination of the transition process; administrative support and participation of the primary healthcare team.

Conclusions
The nurses provide special attention to change of environment (school, employment) of the patients; hence, they focus on ensuring that the existence of effective service planning and coordination is reflected in a successful transition of health services.

Type of study Survey
Objectives Learn more about the expectations of health professionals who care for youths with Cystic Fibrosis on self-care behaviour.

Nursing interventions
Preparation of education in the areas identified as most lacking and most adapted to the different ages.

Conclusions
Knowing the ages when adolescents become autonomous in certain self-care activities means that the created guidelines of attendance are more personalised and directed at adolescents with Cystic Fibrosis.

Type of study Qualitative, exploratory and descriptive
Objectives Learn more about the daily life of children and adolescents with Cystic Fibrosis from their actual experience and identify situations that might affect their daily life.

Nursing interventions
Extra care in the use of scientific and very specific language; knowledge of nurses about coping strategy mechanisms; encouragement of responsible self-care; guidance in the process of normalisation of daily life; interventions directed at increasing the existing potential in adolescents.

Conclusions
Health professionals should coordinate their efforts with the state and civil society, in order to boost the development of health and education, providing clear information on the disease to both the patients and their family.
Title and author of the article Predictors of self-care in adolescents with Cystic Fibrosis: a test of Orem's theory of self-care and self-care

deficit. Baker and Denyes 2008
Type of study Correlation study

Objectives
Offer scientific evidence on the work of nurses involving youths with Cystic Fibrosis, studying the predictors of selfcare through Orem's theory.

Nursing interventions
Identification of the profile of self-care of the adolescent; promotion of health; improvement of quality of life

Conclusions
The nurse should be capable of developing a specific action protocol for health promotion, establishing evidencebased nursing and contributing to the significant improvement of the health potential of young people with lifethreatening chronic disease.

Type of study Update
Objectives Understand the repercussions of Cystic Fibrosis as a stigmatising disease in the life of adolescents with Cystic Fibrosis and their family.

Nursing interventions
Learn more about the stigma associated to the disease and articulate the healthcare plan with better compliance with the treatment, process of socialisation, family relationship.

Conclusions
Recognition of the stigma of the disease by healthcare professionals leads to a better planning of care, assessment of treatment compliance and promotion of quality of life.

Type of study Qualitative with content analysis according to Milles and Huberman 2003
Objectives Explore the experience of parents and adolescents with Cystic Fibrosis relative to the transition to the adult attendance centre.

Nursing interventions
Strategies of support to families and adolescents with Cystic Fibrosis; involvement of families in the therapeutic process.

Conclusions
Knowledge of the experience of young people and their family in the process of transition to an adult healthcare unit is important since the identification of the family system as a single whole contributes to the improvement and finetuning of the nursing care provided. Caring for an adolescent with a chronic disease, such as cystic fibrosis, involves a multidisciplinary work and this is also one of the nurses´ responsibilities.
This professional is responsible for interacting with community services and governmental institutions, aiming to provide the best quality care to these patients, namely in what concerns pre and post pulmonary transplantation (9)(10)(11) .
Results also show clear evidence on the nurse's role as a caregiver, particularly in what concerns inhaled and intravenous medication administration, enteral and parenteral nutrition, ventilator weaning and oxygen administration (9) . However, in addition to these nursing interventions, related to the know-how, the educational domain is also highlighted by several authors as an important competence of nurses who provide support to patients and also to their family caregivers, thus contributing to extended home care (9)(10)(11)(12) .
In this literature review, all studies refer to nursing interventions, mainly focused on communication processes, considered an important and efficient method to provide emotional and psychologic support, aiming at tailor-made care plans.
Encouraging the involvement of family members in the therapeutic process and the support to the family as the most important promoter of the adolescent wellbeing is a key factor that will enable the adolescent to better adapt to the chronic disease (12) .
Emphasis should also be given to the nurses' role in training these adolescents and their family members to pay special attention to feeding, nebulization, oxygen therapy, which will most likely result in benefits to the adolescent and avoid hospital readmissions (9) .
The nurse, as a health team member who interacts closely with the adolescent and family in the transition health-illness process, is able to establish a close relationship and offer emotional support, contributing to the autonomy of the adolescents and their family members (9,(11)(12)(14)(15) .
Developing the potential of each individual with cystic fibrosis and advocate for the patient's legal interests and protection is also one of the nurses' competencies (9)(10)(11) .
The partnership of care and the identification of critical areas, such as the stigma associated with this disease, contribute to improve treatment compliance and enhance quality of life (16) .
Many authors study the transition to adult health services, since the developments in care for adolescents and training provided to families has helped to increase these patients' life expectancy, thus becoming a new area of knowledge to be explored (13,15) . Authors have reflected on the need to implement measures to help these adolescents' transition to adult health services, as well as the focus areas that nurses should consider, namely the identification of the family support and information provided, coordination of the transition and information process (13,15) .
The evidenced-based research and practice developed by nurses empowers them with the necessary skills to enable a better health promotion and support the development of guidelines underlying nursing practice (10,14) .
The provision and management of care, the early identification of the adolescents´ needs, the interpersonal and therapeutic relationship established between nurses and adolescents/ families are important skills of nurses who provide care and support to this population. These are specific interventions, comprising specific areas such as primary, secondary and tertiary care, and are targeted at patients and family members from the moment of the disease diagnosis until the patients´ death (9)(10)(11)(12)(13)(14)(15) .