What is one disease that frustrates the medical community and patients alike, whose etiology is unknown, can occur at all ages, may affect virtually any organ or system of the body, mimics the clinical features of multiple diseases, presents with flares and remissions, and has no cure? Systemic lupus erythematosus (SLE) is the chronic autoimmune disorder requiring skill in diagnosis and the ability to predict and prevent or effectively manage flares of disease activity. SLE is a disease challenging the expertise of healthcare professionals working in tandem with patients who must be responsible and take charge of managing their changing health status. 1
INCIDENCE OF PHENOMENON AND IMPACT ON SOCIETY
Lupus, which originates from the Latin term for wolf, was combined with erythema to name this disease, lupus erythematosus. In 1828, doctors described the disease that presented frequently with a facial rash that looked like the face of a wolf. 2 Lupus is now recognized as a common disease occurring worldwide, and it affects an estimated 500,000 Americans, although the incidence is likely much higher. SLE shortens life expectancy, creates significant morbidity, and accounts for substantial total health expenditures. 3
The usual onset of SLE occurs between 15 and 45 years of age, with 80% of cases occurring in women during their childbearing years. The incidence of the disease has more than tripled over the past 4 decades, with the incidence in African-Americans (1:250) in comparison to Whites (1:1000). 1 The disease is rare in childhood but a sibling of a patient with SLE is 20 times more at risk than those without an immediate family member diagnosed with SLE. 4 Interestingly, it also is noted that smokers are almost 7 times more likely to develop SLE than nonsmokers and there is a higher risk for school teachers, most likely from the high exposure to infectious agents (such as Epstein-Barr virus), which has been associated with a higher risk of SLE. 5
The socioeconomic impact of patients with SLE is clearly demonstrated in research studies. Approximately 40% of patients with SLE quit work within 4 years of the diagnosis, and many are required to make significant work modifications if they are able to continue in the same work environment. Determining factors include physical demands of work, severity of condition at time of diagnosis, and educational level. 4
SLE is a multisystem autoimmune disease involving both humoral and cellular aspects of the innate and acquired immune systems. These autoimmune reactions are directed against constituents of the cell nucleus, especially DNA. Autoantibodies are produced against the nuclear antigens, cytoplasmic antigens, and blood cell surface antigens. When the autoantibodies bind to their specific antigens, complement activation occurs and accumulation of immune complexes occurs within the blood vessel walls. It is with this development of ischemia within the blood vessel walls that leads to the thickening of the internal lining, fibrinoid degeneration, and thrombus formation. It is at this point where specific manifestations of SLE become present, dependent on which cell types or organs are involved. 6 Disease states can be as simple as troublesome alopecia to life-threatening kidney and brain disorders.
SLE shortens life expectancy, creates significant morbidity, and accounts for substantial total health expenditures.
Patients with SLE produce a wide spectrum of autoantibodies, with approximately 95% producing antinuclear antibodies (ANA). 7 Autoantibodies are produced in other systemic diseases, but antibodies to double-stranded DNA and anti-Sm antibodies to polypeptides that complex with certain species of nuclear NRA are very specific for SLE. The activity of the disease can be monitored by levels of ANA, complement, cryoglobulins, and circulating immune complexes measured by the Raji cells assay. 8 The major histocompatibility complex (MHC), MHC-linked, and non-MHC genes have been found to be the most susceptible in SLE, thus would provide appropriate for diagnostic testing. The human leukocyte antigen (HLA) class II genes appear to have the strongest SLE MHC association. 9
Current research demonstrates an estimated 20 different genetic factors that may be involved in the activities which make persons susceptible to SLE. The “concordance rate of SLE in monozygotic twins is approximately 25 to 70%. If a mother has SLE, her daughter's risk of developing the disease is 1:40 and her son's risk is 1:250” (section 3). 1 One study has found defects in four genes that regulate apoptosis, whereas another has identified an inefficient gene that promotes the build up of immune complexes that can cause kidney damage. Human leukocyte antigen (HLA) is a genetically regulated molecule that traps part of the antigens and presents them for destruction by antibodies and T-cells. 4 Other external factors, such as viruses, fatigue, hormones, prescription drug use, chemicals, and sunlight can promote or increase the likelihood of development of SLE. 10 The presence of another autoimmune disorder also dramatically increases the chance of developing SLE. 4 Other triggers may also be possible in an already susceptible person.
The diagnosis of SLE is based on the established criteria for classification originally developed in 1982 and updated in 1997 (Table 1). If four of the criteria are present at any time during the course of disease, a diagnosis of SLE can be made. 11
Because the mechanisms that normally prevent the immune system from attacking an individual's own body are defective, effective lupus specific treatments and therapies have been very difficult to develop. 12 Management of SLE has resulted in general treatment options dependent on its manifestations and severity. Mild disease typically requires little or no therapy. Arthralgias are usually controlled with nonsteroidal antiinflammatory drugs (NSAIDs). Aspirin is helpful but higher doses may cause liver toxicity. Antimalarial uses for joint and skin manifestations are common with hydroxychloroquine 200 mg qd to bid dosing. Opthalmologic exams are advised every 6 months due to retinal toxicity of this particular drug. 13
Severe disease requires immediate therapy with high-dose prednisone and immunosuppressive drugs. Specific dose regimens are used depending on the diagnosis. The use of cytotoxic agents has been found to be beneficial in controlling disease exacerbations, and reducing the need for steroid requirements. 14 Simple avoidance of sun and ultraviolet light is essential in the prevention of flares. 15
Interestingly, the simple close working relationship between the healthcare provider and patient often can prevent or lessen the severity of exacerbations. Increased patient awareness/health promotion and self-monitoring of general health, periodic lab work, acute intervention during exacerbations and regular physical assessments is essential. Close medical supervision is vital during surgical procedures and or pregnancy. Flares and vascular thrombosis may occur with oral contraceptive use, and miscarriage risk is elevated for those with antiphospholipid antibodies. 13 Essential psychosocial support should always be offered with counseling regarding genetic, family, and multitude of issues present with any chronic illness.
Recent developments in immunology, oncology, and endocrinology have resulted in many potential therapies for SLE. Treatments include the use of genetically engineered molecules, immunosuppression, bone marrow transplantation, and immunoablative therapy. 14 The FDA has approved a new screening test that is the first significant diagnostic breakthrough for SLE in more than 4 decades. The test, called the “Anti-SR protein antibody assay,” can identify 50% to 75% of lupus patients who react positively to SR proteins (Immunotherapy, 2002) which are present only in patients with lupus. The Centers for Disease Control (CDC) is currently working with state health departments to develop a state-based registry of persons with SLE to monitor trends and better characterize those with this challenging disease. 16
The theoretical models the advanced practice nurse (APN) can apply to care of the SLE patient is Selye's General Adaptation Syndrome (GAS), the health behavior models Stages of Change, and Health Promotion (HPM). Selye's work evolves around the theory of stress response which suggests there is an initial reaction to stress that is identified as shock, followed by a countershock phase, and then eventually gradual resistance develops to the stressor. Selye defines stress as “the non-specific response of the body to any demand made upon it.” 17(p86) If the level of stress exceeds the limits of tolerance and the adaptive capacity is overworked, then the result may be permanent damage or possibly death. 17 It is essential that the body is allowed a time of adaptation and repair; damage, chronic disease, senescence, or death may occur if the regeneration phase is not allowed to occur. Robinson and Kish 18 identify the importance of an individual's need to develop stress responses to maintain a state of homeostasis. The APN can assess the patient with SLE during an exacerbation and initiate appropriate procedures to return them to a level of homeostasis. This can be followed up with a plan to assess the patient status and set achievable goals.
The Stages of Change model was developed to better understand the individual's place within the change process and focuses on his or her current stage rather than level of change. 19 The patient with SLE certainly is in an evolving process of change and has the impetus to consider change for the benefit of his or her health. Individuals are at varying levels of motivation and at different points in the process of changing a health behavior. In this transtheoretical model, five stages provide for the person to enter and exit at any point, and also relapse at any stage. 18 The APN can develop an educational plan that assists with motivating and educating patients and helping patients to set realistic, obtainable goals as they persevere with their chronic SLE. Utilization of the HPM by the APN provides for an approach that includes the individual characteristics, experiences, and psychological and sociocultural factors. 18 The key to health-promoting behaviors is the development of personal commitment to a plan of action with specific strategies that the APN can implement in concert with patient input.
ADVANCED PRACTICE NURSING CARE PLAN
The development of a nursing care plan by the APN for the patient with SLE requires the collaboration and management of symptoms that interfere with the patient's daily functioning (Table 2). The incidence of exacerbation is always present, but the focus is to adapt to an effective and manageable plan with the typical symptoms noted for the patient with SLE.
SLE is a complex and challenging disease that involves managing many facets. The effects on the patient's life can be devastating or simply a series of frustrations and intrusions on an otherwise “normal” life. An effective understanding of the pathophysiology, recognition, treatment, and care of patients with this disease can enable most to lead relatively normal lives. The APN is a part of the team that positively affects the patient by possessing the skills, knowledge, and expertise to manage this chronic disease. The future looks promising in technological advances that may hold the answer to a cure for this disease. Patients with SLE are often admitted to critical care units for an exacerbation of their SLE or for another problem. It is essential that critical care nurses know about the management and treatment of patients with SLE.
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