Cystic Fibrosis as Family Health Issue
Cystic Fibrosis –as a family health issue- is a common issue faced by many different families across the globe. The autosomal genetic disease is a life threatening condition that commonly appears as early as childhood. Preventive measures available are genetic diagnostics and tracing the history of the disease through family genogram. As a family issue, cystic fibrosis can lead to imbalance in the family’s psychological and emotional estate due to the feelings of guilt, denial and burdens of care needed for the survival of the ill member of the family.
The overall household population of Australian community comprising of 17% has been found to be affected by disabling genetic illnesses, such as cystic fibrosis (Wertz and Fletcher, 2004 p.106). According to Ballard (1999), cystic fibrosis affects approximately 1 in every 2500 children in Australia (p.85). In Australia, 800 children with case of Cystic fibrosis are presently living in New South Wales, while approximately 2500 infected children are living nationwide (Ballard, 1999 p.85). Cystic fibrosis, as a family health issue, has caused significant disruption in the area of family’s psychological and emotional well-being. The main issue explored in this report is the impacts of cystic fibrosis patients on family health. Cystic fibrosis affects primarily the exocrine glands of the body, which immediately results in excessive production of thick mucus that can block different pathways of body systems (Ballard, 1999 p.85). In the body of the discussion, the hallmark involves the confrontations commonly done by the family members that lead to family stress among its members. The study cites the etiologies, impacts, nursing interventions and evidence-based interventions in resolving family health issue associated to cystic fibrosis. Health care needs and transitions of care required by cystic fibrosis patient further complicate family’s task in providing care to their patient. Based on studies identified within the discussion, the primary contributing factors of family stress in rendering care for their cystic fibrosis patient are (a) lack of proper understanding of the disease and required management, and (b) increasing needs of the patient requiring progressive care regimen. In order to resolve this family issue, we have recommended CP-FEP as a learning program aimed at establishing the capacity of the family to render self-administration care regimen for their patient. Nursing recommendation has chosen CP-FEP due to its practical and self-care natured strategy of resolving the family’s care needs for their patient. The study is useful for families with possibilities or actual patients suffering from cystic fibrosis; since cystic fibrosis is one of the major causes of preterm deaths, decrease of lifespan and chronic disabilities among children. By understanding the care regimens and concepts of the disease, the family can better manage their patients leading to the improvement of both patient and family’s quality of life. The study limits itself in discussing the effects of a family member with cystic fibrosis towards the family health tasks, which include the psychological and emotional balance of the family confronting the care demands of their patient.
a. Background of the Problem
Cystic fibrosis – a life threatening childhood hereditary disease – has been found to affect the status of parenting and stability of family health. It is important to understand that cystic fibrosis, as a family issue, can lead to (a) feelings of guilt, especially among the parents, (b) disruption in healthy parenting, and (c) disrupts the usual family cycle due to the shortened life span of the patient. According to Fiese (2006), the status of a child with cystic fibrosis greatly affects the family functioning making the family more vulnerable to psychological and emotional distress (p.84). Added by Kuhse and Singer (2006), family health is at risk of experiencing stress and disruptions compared to families with normal setup (p.130). Considering the terminal nature of the childhood illness, the conditions of the child become increasingly difficult to manage; hence, it is likely for families with cystic fibrosis case to experience difficulties in adjustments and parenting (Hoghughi and Long, 2004 p. 191).
b. Purpose of the Study
The primary purpose of the study is to explore the health-associated problems experienced by families in managing their patient with cystic fibrosis. The study aims to determine the (a) etiologies of the family health care issue and (b) provide appropriate recommendations to resolve the family health issue. The impacts of cystic fibrosis to family health can be devastating, especially in the launching systems of the family, stability of the parents and the members, and the financial requisites of children with this condition. According to Pryor, Prasad and Prasad (2002), genetic diseases, such as cystic fibrosis, place strain on the marital relationship disrupting the strength and stability of parental domain, which eventually affects other members of the household (p.283). With family as the major element of care, the complexities of the family health impacts of this disease are important components to study and analyze aiming to formulate evidence-based recommendations and appropriate nursing interventions for both parents and patient.
c. Definition of Terms
Cystic Fibrosis – it is a hereditary condition affecting the exocrine glands of different vital organs, such as lungs, pancreas, liver and intestines, which increases the level of mucus production and eventually leads to multi-organ failure (Nelson and Martin, 2000 p.308).
Family health – is a state of holistic well-being of the family system manifested by the development and continuous interaction among the physical, mental, emotional social, economic, cultural and spiritual dimensions (Pardeck, 2006 p.195),
Family Stress – a condition wherein the family’s level of health, state of stability and capacity to intervene accordingly are compromised by a stressful event occurring within or outside the family circle, such as financial constraints, illnesses, failure to launch, etc (Johnson, Hayes and Field et al., 2000 p.309).
III. Results and Discussions
a. Description of the Health Issue
The ever-growing and life-threatening childhood disease – Cystic Fibrosis – affects mostly the childhood and teenage population of Caucasians and White population (Chisholm-Burns, Wells and Schwinghammer et al., 2008 p.138). According to Harris (2006), cases with cystic fibrosis in Australian families have shown increase in life span for both sexes, especially among males with some reaching their launching phase (males 44 years and females 28 years) (p.427). Nonetheless, the impacts of the disease to the health of the family remain. The disease generally disrupts the normal process of mucous secretions in the vital organs causing blockages, damages and eventually, multi-system failure, which can lead to death (Pilitteri, 2006 p.262). Blumenfield and Strain (2006) emphasize that the autosomal cystic fibrosis is prevalent among Australian population due to the capacity of the children with the disease to reach their launching systems within their limited life span (p.900).
Caring for a child with cystic fibrosis requires daily adherence to nursing activities, such as chest physiotherapy, recreational activities, child surveillance, etc. According to Fiese (2006), family members experience varying degrees of disruption in the general health of family relations and adapted routines due to the different adjustments every member must make in order to suit well in the caring process of the child (p.84). Moreover, cystic fibrosis is not only the issue of immediate family members but most of all the genetic origin – patriarchal or maternal – of the disease. Kuhse and Singer (2006) point out that the family side carrying the genetic trait is at risk of experiencing guilt, hostility from the opposite party and eventually family stress (p.130).
b. Contributing Factors of the Health Issue
Strong feelings of guilt in the source party from either parties, resentment in the child according to the degree of dependency and capacity for self-care, and restrictions mandated by the condition and treatment processes are the most common causes of family stress and destabilization (Pryor, Prasad and Prasad, 2002 p.284-285). According to the study of Powers, Gerstle and Lapey (2001), the foremost etiologies affecting the patient’s quality of life and family health condition are (a) the child’s social problems, (b) social and role limitations, (c) bodily pain, (d) physical functioning, (e) family activities and (f) general health of the child. The family’s balance and capacity to confront the needs of their patient trigger family stress, especially among the parents. According to Fierse (2006), “families who has a child with cystic fibrosis experienced difficulty communicating, monitoring affect, and controlling the behavior of other members of the family compared to families without an ill child” (p.85). The disruption of family hierarchy, appropriate authority and functions dramatically cause family stress risking destabilization and inappropriate family management (e.g. parenting, early launching, etc.). As supported by the study of Reiss, Gibson and Walker (2005), transitioning age of the patient with cystic fibrosis can lead to frequent adjustment periods for all members of the family. If one fails to adapt appropriately, the consequence of action immediately bounces to other members of the family causing family strain and imbalance of emotional/psychological health. Illness management burdens the family members due to financial and routinary constraints; meanwhile, family attitude towards the patient becomes overprotective, limiting and restricting, which eventually disrupts the normal process of growth and family development.
c. Impact of the Health Issue
Families confronted by childhood progressive illness suffers different psychological and emotional confrontations, especially with the increasing needs and adjustments the patient have to undergo every transition. In the comparison study of Janicke, Mitchell and Stark (2005), 28 families of children with cystic fibrosis were compared to 27 families of non-ill and age-matching peers. The assessment aims to discover the family differences in (a) task accomplishment, (b) communication, (c) affect management, (d) interpersonal involvement, (e) behavior control and (f) role allocation. At the end of the study, families of cystic fibrosis children obtained lower scores compared to families of non-ill children. According to Crane and Marshall (2005), transitioning care needs of cystic fibrosis patient largely depend on the families participation, while complex treatment programs, such as aggressive antibiotics, synthetic enzymes and others, can be very demanding on the families placing parents under the great burden of carrying-out the child’s needs (p.384).
In effect, family members become strained and eventually manifest feelings of depression, denial, guilt and other unhealthy psychological defense mechanisms (Blumenfield and Strain, 2006 p.900). According to Spirito and Kazak (2005), the health of the family in terms of stability, balance of powers and communication systems become affected by the erratic care needs of the patient with cystic fibrosis (p.50). Australian families most of the time are unaware of handling the different symptoms and manifestations of the illness causing further psychological distress and emotional decline (Blumenfield and Strain, 2006 p.900). Nonetheless, the interventions of the family, despite of the burden, remains essential to the survival the patient.
d. Nursing Actions
The hallmark of nursing intervention for families with cystic fibrosis patient is the adequate and appropriate teachings on home care giving emphasis on (a) chest physiotherapy, (b) medication administration and (c) regular patient monitoring (Hatfield, 2004 p.267). Therefore, the first nursing intervention that needs to be rendered by the health care provider is the establishment of reciprocal learning process built in appropriate rapport and trusting relationship. Evidences from the study recommend that family members’ (a) lack of knowledge in preparation for transition and (b) changing challenges of the child are the immediate nursing needs of the families (Reiss, Gibson and Walker, 2005). The study of Reiss, Gibson and Walker (2005) emphasize the value of reciprocal nurse-family relationships based on mutual trust and support in caring of patient with cystic fibrosis. The rationale of the intervention states that the nurse fills in the gap of understanding in cystic fibrosis management, which, if done appropriately, can reduce the stress and strains experienced by the family. According to Blumenfield and Strain (2006), Australian families have shown wide support and utilization of educational materials on cystic fibrosis, especially the diagnostic early prevention schemes (e.g. newborn screening, sweat testing, etc.) (p.900).
In the second nursing intervention, the nurse must establish and evaluate adequate knowledge of every family member involved in the care process of the patient. The main principle of the nursing intervention is to teach the family the expected manifestations and challenges of the disease encouraging right attitude for preparation and knowledge in managing the disease. According to Janicke, Mitchell and Stark (2005), planning the care of the patient with the health care provider is also a recommended intervention since the family can voice out their queries, while the health care provider renders appropriate resolutions to resolve these conflicts. Furthermore, the nurse must emphasize the value of adhering to the daily tasks of care required by the patient, such as chest physiotherapy and medications. If the parents plan to have another pregnancy, it is always best to encourage them for genetic testing.
e. Evidence-based Recommendations
Considering the culture and family tradition of Australian families, it is always important establish patient-provider rapport prior to engaging health care management. According to Bartholomew, Parcel and Kok et al. (2006), the nurse providing learning intervention for family members must emphasize the value of enhancing the family’s self-management skills through the standard CF Family Education Program (CF-FEP) (p.271). According to Drotar (1998), CF-FEP is a self-management education program designed to be implemented as an integral part of CF care in home care or health care settings (p.p205). Since the program is self-administered by the family and requiring only occasional supervision from the health care provider, CF-FEP can be a better alternative of management due its cost-effective and family oriented approach.
Problems affecting the health of a family with a cystic fibrosis patient are most of the time caused by insufficient knowledge in managing the care transitions and needs of the patients. In response to this problem, CF-FEP is formed to help the family implement self-administered patient care. Guided by the health care provider, family members are taught to correct positioning for chest physiotherapy, medication administration, and essential topics and signs family members must learn to better monitor their patient (Bartholomew, Parcel and Kok et al., 2006 p.271). Furthermore, the care management establishes profound interactions between patient and family members developing feelings of responsibility and attachment among all members. Since the procedure is economical and effective with full family participation, CF-FEP is the most considerable recommendation for management procedures.
In conclusion, cystic fibrosis is a genetic, progressive and chronic disease manifesting mostly during childhood. The condition is life threatening and usually causes dramatic decline on a person’s life span. Families with members suffering from cystic fibrosis are greatly confronted by the risk of developing compromised family health in relation in to psychological, emotional and interactional domains. Some of the acknowledged initial impacts of the condition to family members are denial, guilt, depression, feelings of blame and hostility against the source gene, which can be either patriarchal or maternal. Due to the chronic nature of the disease, family members are continuously burdened by the care needs of their patient. Without having the right knowledge in managing the disease, the family experiences great deal of stress in caring for their patient.
As the person grows, the patient confronts another transition of care needs, such as increasing the dosage of medicine, increasing the frequency of physiotherapy, and further limiting self-care management and capacities of the patient. For families who do not entirely understand the nature and management of the illness, these changes can dramatically result to family conflicts, disrupted family stability and comprised patient care. Hence, as a resolution, the standard nursing program CF – FEP has been recommended to foster the knowledge of the families in rendering self-administered care for their patient. The advantage of utilizing CF-FEP is development of patient-family attachments and fostering interaction, affect and bonds within the involved members.
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