2.0 ANCC Contact Hours Abstract Juvenile idiopathic arthritis (JIA) is the most common chronic pediatric illness in the United States. The disease encompasses a group of heterogeneous chronic arthritis conditions that begin before age 16 years and persist for more than 6 weeks. Formerly termed juvenile rheumatoid arthritis (JRA), JIA now includes polyarticular, oligoarticular, psoriatic, enthesitisrelated arthritis, systematic arthritis, and undifferentiated arthritis. Diagnosis is based on clinical and laboratory features. Treatment includes immunosuppressant therapy, non-steroidal anti-inflammatory drug (NSAIDS), and biologic therapies. This can affect all aspects of an adolescent’s life including physiologic, psychosocial, and spiritual components; therefore, this article discusses a comprehensive approach to care management with transition of care as a critical feature in adolescent healthcare. Keywords: Biologic therapy; Chronic illness; Juvenile idiopathic arthritis; Transition to adult care.
Growing up with Juvenile Idiopathic
ARTHRITIS J Amy McKeever, PhD, CRNP, WHNP-BC and Michelle M. Kelly, PhD, CRNP
uvenile idiopathic arthritis (JIA) is the most common chronic pediatric illness in the United States and affects approximately 240,000 children and adolescents under the age of 18 years (Arthritis Foundation, 2013). The disease is responsible for inflicting significant musculoskeletal pain, growth limitations, nutritional status impairment, and decreased health-related quality of life (HRQOL) as well as potential conflict and stress among families and peers (Khan et al., 2012; Seid, Opipari, Huang, Brunner, & Lovell, 2009). Juvenile idiopathic arthritis, formerly termed juvenile rheumatoid arthritis encompasses a group of eight heterogeneous chronic arthritis conditions with onset prior to the age of 16 years with symptoms persisting beyond 6 weeks (Prakken, Albani, & Martini, 2011). The JIA conditions are grouped based upon clinical and laboratory findings to enable the use of evidence-based treatment protocols to expedite diagnosis and improve patient outcomes (Prakken et al., 2011). A multidisciplinary management approach is required for JIA, which includes: pharmacological therapy, physical and occupational rehabilitation, and psycho-educational interventions with the patient and
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family, school accommodations, as well as comprehensive nurse navigation and interdisciplinary case management (Russo et al., 2012). An exemplar case is provided in Box 1.
Pathophysiology
The etiology of JIA is unknown, but is thought to be both genetic and environmental, with environmental factors triggering autoimmune deregulation (Lin, Wang, Gershwin, & Chiang, 2011; McDonagh, Shaw, & Southwood, 2006; McMahon & Tattersall, 2011). T cells, particularly CD 8 T cells, play a role in the immune deregulation. Laboratory evaluations of JIA patients point to an increased numbers of T cells from the blood circulation in the joint synovial fluid. In addition, increased levels of cytokines, specifically interleukin 1 through interleukin 12 (IL 1–IL 12) have been reported in the serum samples and synovial fluid of JIA patients (Martini, 2012). Siblings and first degree relatives of patients with JIA have higher rates of autoimmune arthritis; rates are reported from 25% to 40% and rates of JIA among siblings are 15- to 30-fold greater than that of the general pediatric population (Prahalad et al., 2010). The HLA-A2, January/February 2015
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HLA-DRB1*11, and the HLA-DRB1*08 genes have been implicated in JIA (Martini, 2012). Researchers speculate that a child or adolescent who is genetically predisposed to JIA may experience an environmental trigger causing deregulation in the immune response (Lin et al. 2011). Such triggers may include viral infections such as influenza A, rubella, and parvovirus B 19. Mycobacterium pneumonia has also been associated with the onset and exacerbation of JIA. Other factors suspected to trigger the onset of JIA include: maternal smoking during pregnancy (influences the immune system deregulation of the fetus), weather changes (extreme changes in temperature have been implicated in exacerbations and flares), recent vaccination (vaccinations can trigger the immune response with lymphocyte activation), and stress (increases IL-6 production) (Martini, 2012).
Diagnosis Diagnosing JIA can be a difficult task that relies on identification of clinical features. Classification of JIA is based upon the International League of Associations for Rheumatology Taskforce on Classification of ChildJanuary/February 2015
Juvenile idiopathic arthritis is the most common pediatric chronic illness in the United States. hood Arthritis (Table 1) (McMahon & Tatersall, 2011). Hallmark features of arthritis include persistent joint pain, morning stiffness lasting one or more hours, and daily activity limitations (Modica, Sukumaran, & Milojevic, 2012). Children and adolescents with systemic JIA may have a prolonged disease course with a progression to oligoarthritis (affecting one to four joints) or polyarthritis (affecting five or more joints), and may present with the following symptoms: prolonged fever, myalgia, rash, elevated inflammatory markers (erythrocyte sedimentation rate [ESR]), elevated c-reactive protein (CRP), anemia, thrombocytosis, abdominal pain, lymphadenopathy, and splenomegaly. Patients with oligoarthritis frequently MCN
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Exemplar Case of Juvenile Idiopathic Arthritis A 10-year-old girl presented to her pediatrician after a viral infection with right knee swelling. A work-up for Lyme disease was negative. ANA and R.A. labs were negative. On exam her knee was painful, warm to touch with a slight effusion on the right. She was referred to rheumatology. After an uneventful course of 2 years, she began with episodes of joint swelling of her knees, wrists, and 4th and 5th metacarpal joints. She responded well with corticosteriod therapy (Prednisone), and NSAIDs (Naproxen and Meloxicam) but had to discontinue them due to gastrointestinal side effects. On year 3 she was started on Etanercept 25 mg/weekly sq after she began to experience decreased range of motion and functional mobility with amplified pain syndrome in her knees, her feet, and hands. After 4 months into treatment, she responded with improved range of motion, decreased amplified pain, and improved functional mobility. She was also sent for outpatient occupational and physical therapy.
have documented elevated levels of antinuclear antibody antibodies (ANA) serum levels along with joint inflammation, and some patients may present with uveitis (Modica et al., 2012). A comprehensive clinical history and physical assessment with specific attention to the musculoskeletal system is critical. The musculoskeletal examination of a patient suspected of having JIA includes: careful assessment of joints with particular attention to joint swelling due to effusion or hypertrophy, warmth of the joint, pain with palpation or range of motion, and limited range of motion (Modica et al., 2012). Tracking the disease course for the first 6 months is a priority for an accurate diagnosis and prompt treatment. Parents and adolescents may be encouraged to use digital photographs and smartphone calendars to monitor joint swelling and symptoms. Laboratory evaluation includes complete blood cell count, comprehensive metabolic panel, ANA, ESR, CRP, rheumatoid factor, lupus anticoagulant, and HLA B27 gene
Table 1. Classification of Juvenile Idiopathic Arthritis Arthritis Classification
Clinical Manifestation
Prevalence in Males Versus Female
Systemic
Arthritis in one or more joints with daily fever or preceded by daily fever for 2 weeks and accompanied by one of the following: an evanescent nonfixed erythematous rash, generalized lymphadenopathy, hepatomegaly, or splenomegaly or serositis
Affects males and females equally Can begin in childhood
Oligoarticular
Arthritis affecting one to four joints during the first 6 months of disease. Two subcategories: Persistent oligoarthritis: affecting not more than four joints during the disease course Extended oligoarthritis: affecting more than four joints during the disease course
Affects girls more frequently than boys Peaks age of onset: 2–4 years Most common joint affected: Knee
Polyarthritis (rheumatoid factor negative)
Arthritis affecting five or more joints during the first 6 months. Rheumatoid factor test negative
Affects girls more than boys Biphasic onset distribution: Early onset 2–4 years Later peak onset 6–12 years
Polyarthritis (rheumatoid factor positive)
Arthritis affecting five or more joints during the first 6 months. Rheumatoid factor test positive on two separate occasions
Affects girls more than boys Late childhood or adolescence
Psoriatic arthritis
Arthritis and psoriasis, or arthritis alone and at least two of the following: Dactylitis, nail pitting, onycholysis, psoriasis of a first-degree relative
Affects girls more than boys Biphasic onset distribution: Early onset 2–4 years Later peak onset 6–12 years
Enthesitis-related arthritis
Arthritis and Enthesitis or arthritis or Enthesitis with at least two of the following: Presence or history of sacroiliac joint pain, presence of HLA B27 antigen, onset of arthritis in male over 6 years of age, acute anterior uveitis, sacroiliitis with inflammatory bowel disease, Reiter syndrome or acute uveitis in a firstdegree relative
Affects boys more than girls Late childhood or adolescence
Undifferentiated arthritis
Arthritis that persists for at least 6 weeks but does not fulfill criteria for any other category Arthritis that fulfills two or more categories in the JIA spectrum above
*Table recreated using information from McMahon and Tattersall (2011).
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Table 2. Pharmacological Management of Juvenile Idiopathic Arthritis Pharmacological Agent
Pharmacological Drug Class
Dose
Clinical Implications
Adalimumab (Humara)
Human monoclonal antibody anti-TNF antibody
24 mg/m2
Treatment for polyarticular JIA: monotherapy or with methotrexate
Anakinra (Kineret)
Interleukin-1 receptor antagonist
1 mg/kg/day
Used in systemic JIA
Etanercept (Enbrel)
TNF inhibitor Alpha & Beta TNF receptorimmunoglobulin-fusion protein
0.4 mg/kg subcutaneously twice per week
Treatment for JIA - First approved biologic treatment for JIA Used in R.A. factor + and - Psoriatic arthritis, systemic arthritis
Infliximab (Remicade)
TNF inhibitor Chimeric murine-human monoclonal anti-TNF antibody
3 mg/kg intravenous infusion at 0, 2, and 6 weeks. Maintenance dosing every 6 weeks
Not currently FDA approved for JIA, but is used in clinical practice and has demonstrated improved results in RCTs Used often with Crohn disease
Tocilizumab
NR-Interleukin (IL6) receptor antagonist
2–8 mg/kg intravenous infusion at 0 week Maintenance every 2 weeks
Used with active/refractory or systemic JIA
Abatacept
Inhibits T-cell stimulation
10 mg/kg/4 weeks intravenous infusion
Used with active/refractory or systemic JIA that is TNF-unresponsive
typing. Radiological imaging is often the first step in evaluating suspected joint involvement. Magnetic resonance imaging and ultrasonography (US) are also used to detect early JIA disease (McMahon & Tattersall, 2011).
Management Pharmacological Management
Inhibiting cytokines is the priority for pharmacological treatment for the management of JIA (Horneff, 2013). The initial pharmacological therapy for the patients with JIA includes NSAIDs. If synovitis continues after treatment with NSAIDs, immune suppression to decrease joint inflammation is indicated (Lee, 2013). First-line oral immunosuppressant agents often used include: methotrexate—a folic acid antagonist, sulfasalazine—a dihyrofolate reductase inhibitor, and indomethacin—a NSAID/COX 2 inhibitor. The first-line biologic agents for the treatment of JIA are the tumor necrosis factor (TNF) inhibitors including etanercept, adalimumab, and infliximab (Goldzweig & Hashkes, 2011; Horneff). Second-line biologic agents include toclizumab, abatacept, anakinra, canakinumab, and tocilizumab. Other biologics indicated under review include rituximab, golimumab, and certolizumab (Table 2) (Gartlehner, Hansen, Jonas, Thieda, & Lohr, 2008; Horneff; Sandborg & Mellins, 2012). Complementary Alternative Medicine Management
Use of complementary alternative medicine (CAM) modalities has increased in the JIA patient (Wang, 2012; Ward, Stebbings, Cherkin, & Baxter, 2013; Zebracki, Holzman, Bitter, Feehan, & Miller, 2007). It is estimated that 34% to 90% of patients with JIA use CAM January/February 2015
modalities (April et al., 2009). The most common reported CAM modalities reported include: dietary alternations (herbal remedies, and vitamin and mineral supplements) (60–70%), prayer (56%), massage therapy (50%), meditation (30%), topical skin creams for pain relief and joint inflammation (30%), aromatherapy (25%), touch therapy (20%), and herbal medicine (20%) (Merkes, 2010). Adolescents with JIA reported that CAM therapies are primarily used for minimizing symptoms and decreasing pain. Forty-five percent of JIA adolescents who use CAM modalities fail to report their use of CAM to their rheumatologist for concern of disapproval (Feldman et al., 2004; Wang, 2012; Ward et al., 2013). Adolescents and their families may feel more comfortable sharing their CAM use with nursing professionals, particularly if questions regarding its use are posed in a nonjudgmental manner. Researchers noted that parents may choose CAM as their first-line therapy to manage their child’s chronic illness due to misconceptions about pharmacological treatment and fear of adverse effects (Wang, 2012; Ward et al., 2013). Because there has been limited research in the use of CAM with JIA adolescents, there is little evidence to support its effect on symptoms or adherence to standard medical treatments (Ward et al., 2013). Risk of Associated Disease
Opportunistic infections and childhood malignancy are two conditions thought to be associated with treatment modalities used for children with JIA. These complications pose significant concerns for parents and adolescents as they make treatment decisions. Secondary data analysis of records from two United States medical databases, the US Medicaid Analytic extract database and the US Surveillance, Epidemiology and End Results MCN
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(SEER) database from 2000 to 2005, was used to determine standardized incidence rates and risks related to treatment options (Beukelman et al., 2012, 2013). Beukelman et al. (2013) concluded that individuals with JIA have a two-fold increase in opportunistic infections; however, the risk was not related to immunosuppressive medications but was thought to be associated with autoimmune disease. Most prevalent opportunistic infections were herpes zoster, Salmonella, and Coccidioides with herpes zoster being the most frequently treated infection. Based upon the SEER data, it was determined that those individuals with JIA have a 1.4 to 4.5 times higher standardized rate of malignancy than children with attention deficit hyperactivity disorder or asthma. Regardless of treatment modality, children with JIA have an increased risk of malignancy (Beukelman et al., 2012, 2013). Impact of Chronic Illness
Chronic pediatric illness affects the adolescent and family on physical, psychosocial, social, behavioral, educational, and financial levels (Barlow & Ellard, 2006). Many adolescent patients relate their overall well-being to their perceived psychosocial well-being, their status with peers, and their perceived body image. When the disease interferes with JIA adolescents’ ability to socialize, attend school regularly, and participate in age-related activities with their peers, their psychosocial well-being, sense of normalcy, and peer relationships are negatively affected (Sanzo, 2008; Schanberg, Gil, Anthony, Yow, Rochon, 2005). For example, Russo et al. (2012) reported that families with a child or adolescent diagnosed with JIA selfreported a change in family and social relationships. Of 33 JIA patients surveyed, 50% reported prolonged school absences due to disease flares, 70.8% reported halting organized sports, 66.6% reported attending regular physical
Figure 1. Model of Transitional Care for Adolescents with Juvenile Idiopathic Arthritis
Pharmacological Management
Psychological Health
Independent Living
ADOLESCENT Education College/ Vocational Training
Reproductive Issues
Parental Involvement
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Social Issues
Juvenile idiopathic arthritis encompasses eight chronic arthritis conditions in children under the age of 18 years.
and occupation rehabilitation sessions, and 50% reported needing an assistive aid for their affected joints (Russo et al., 2012). When asked about their social interaction having an impact on their disease status, 75% reported that they failed to tell anyone about their disease for fear of shame or discrimination, 91.6% relate that their disease interferes with their daily living and HRQOL, and 41.6% reported that their JIA had a negative impact on their body image. Additionally, 91% of adolescents with JIA were worried about the current and future state of disease control, their body image, and current and potential disabilities related to JIA (Russo et al., 2012). Tong, Jones, Craig, and Singh-Grewal (2012) interviewed JIA patients using qualitative methods and identified six themes about how their disease had an impact on their life. Themes identified included: aversion to being different, striving for normality, stigma and misunderstanding, suspension in uncertainty, managing treatment, and desire for knowledge about their disease. In addition, Tong and colleagues noted that school and social functioning were recognized by JIA adolescents as an important part of their HRQOL. An adolescent with JIA also reported that pain and fatigue were two disease factors that negatively affected academic performanc and school attendance. For example, the early morning routines and later activities for athletics and clubs involved in high school are not optimal for the JIA client as proper sleep and periods of rest are paramount to control disease flares (Khan et al., 2012). Promotion of normal routines is paramount, and is facilitated by adequate disease management, and regular activity (Khan et al., 2012). January/February 2015
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Table 3. Recommendations for Successful Transitional Care Programs Providers/Nurses • Design a structured transition program with step-by-step processes that begin in early adolescence • Appoint a key healthcare provider (nurse/APN) to begin the transitional program • Work systematically to foster independence with adolescent beginning early in adolescence • Have parents included in visit, but allow adolescent to lead the discussion during visit • Work at each visit to encourage ownership and independence of disease management • Discuss self-management of pharmacological agents • Discuss life issues: work, career, sexuality, intimate relationships, higher education • Provide multidisciplinary referrals or consultation
Young Adults
Parents
• Voice your opinion • Meet with adult care providers before selecting one • Obtain access to chart and medical records • Participate in healthcare decisions • Communicate clearly with healthcare provider and staff healthcare needs and concerns about chronic illness • Attend visits without a family member • Verbalize understanding and willingness to begin transfer of care • Schedule own appointments • Verbalize understanding of disease management regimens • Set goals for education and employment • Verbalize understanding of impact of substance use, smoking, and pregnancy on disease • Use nurse navigator to assist in transition
• Arrange meeting with pediatric rheumatologist to discuss transfer plans • Assist adolescent if needed in meeting adult rheumatologist • Communicate with adolescent the need for ownership of care • Allow adolescent to schedule own appointments • Allow adolescent to attend visits alone prior to transfer • Allow adolescent to take ownership of disease management • Meet with pediatric nurse/ANP who will assist in coordinating transition
Transitional Care The American Academy of Pediatrics (2011) considers comprehensive transitional care programs for adolescents with chronic illness a healthcare priority. Advancement in the treatment of JIA with biologic therapies and immunosuppressant therapy has increased HRQOL. As children born with complex chronic illnesses are now surviving into adulthood and leading independent lives, programs transitioning from pediatric care to adult care are of critical importance (Osterkamp, Costanzo, Ehrhardt, & Gormley, 2013; Paone, Wigle, & Saewyc, 2006; Sable et al., 2011). Transition of care refers to the continuum of care that begins during the pediatric period and ends when the adolescent is transitioned to their adult healthcare provider and into the adult healthcare system with all interdisciplinary referrals in place. The term transition of care is often misused assuming that transition of care only refers to the movement of the adolescent’s medical records. However, that is only one small component of the transitional program (Paone et al., 2006). McDonagh et al. (2006) have identified key components of effective transitional care including: skills training for the adolescent (e.g., communication, decision making, assertiveness, and self-management), inclusion of the caregiver/parent, young-person centered, interdisciplinary/multidisciplinary focused, involves pediatric and adult healthcare services, and holistic focused (Lipstein, Meuthing, Dodds, & Britto, 2013). Preparation must begin early to ensure adolescents develop the knowledge and skills to take ownership of their chronic illness and disease management. Healthcare providers must be well educated and aware of the developmental issues that present in the adolescent years. The nurse is a critical provider to navigate, educate, and coordinate this transition. As adolescents move from parental control to independent living, their healthcare is of critical January/February 2015
Adolescents should take an active role in their disease management as this is a crucial step in the transition of care. importance (Van Staa, Jedeloo, Van Meeteren, & Latour, 2011). According to Barlow and Ellard (2006), the major concerns of adolescents with chronic illness who move into adult care are going into the unknown, going into a different world, disrupting relationships and ways of working, a shift in roles and responsibilities between the adolescent and adult, and recommendations for improving processes and personal growth. Recommendations for successful transition include: improving patient and parental preparation and improving communication between the pediatric and adult rheumatologist (Table 3) (Van Staa et al., 2011).
Clinical Nursing Implication In healthcare facilities, the nurse is a key member of the healthcare team focused on development and implementation of transitional care for the chronically ill adolescent (McDonagh et al., 2006). As transition of care is an ongoing process that begins in early adolescence and continues through early adulthood, the important features of disease management for the JIA patient in transition of care include: self-awareness of disease; identification of potential triggers to flares; symptom management with appropriate rest, pain medication, and complementary therapy; the comprehensive understanding of drug treatment protocol and potential toxicities; and preparing the adolescent for lifestyle changes with higher education and employment. Critical issues for the nurse to address for adolescent patient MCN
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Clinical Implications
• Transitional care assists adolescents to successfully care for their chronic illness, achieve life goals, and to function independently leading a productive life.
Amy McKeever is an Assistant Professor, Villanova University, College of Nursing, Villanova, PA. She can be reached via e-mail at [email protected] Michelle M. Kelly is an Assistant Professor, Villanova University, College of Nursing, Villanova, PA. She can be reached via e-mail at [email protected] The authors declare no conflict of interest.
• Transitional care begins in early adolescence and should continue until adult care is well-established.
DOI:10.1097/NMC.0000000000000096
• Successful transitional care is based upon an individualized approach based upon the JIA’s particular disease.
References
• The nurse is a key member of the transitional healthcare team for pediatric and adolescent patients with chronic illness.
• Transitional care requires care coordination with the interdiscinplinary healthcare team.
include patient education such as risks of substance use with the adolescent’s pharmacological therapy, ramifications of sexual behavior with immunosuppressant therapy, unintended pregnancy and teratogens, potential complications associated with pregnancy, as well as the importance of family planning measures to prevent or delay pregnancy. Discussions on family planning with acceptable contraceptive options must be included in the transitional care program, including risk reduction education on delaying or abstaining from sexual intercourse, and risk reduction measures if the adolescent is already sexually active. Adolescent and young adult females must be well informed about risks and complications of pregnancy with their chronic illness. Those females with JIA are at increased risk throughout the pregnancy, and management with a multidisciplinary team is necessary to reduce maternal and fetal morbidity and mortality (McDonagh et al., 2006). Successful transition must be individualized to consider the developmental level of the JIA patient, the severity of the adolescent’s particular JIA illness, their disease stability, their personal goals, and the ability of that patient to take ownership of their disease. Transitional care requires planning, care coordination, and collaboration among the interdisciplinary team members as well as the JIA patient and their family, with facilitation by the nurse. The objectives of an effective transition of care is for the adolescent to reach a level of independence in caring for their chronic illness in young adulthood, to achieve their goals in life, and to be able to function as a productive member of society while at the same time living and managing a chronic illness. Nurses must encourage adolescents to take ownership of their disease management and health promotion activities that are critical pieces in transitional care (Condon, Gormley, & Hussey, 2009; Sanzo, 2008). There are large number of chronically ill children and adolescents throughout the United States. Nurses are key members of the interdisciplinary health team leading the successful transition of care. Transitional care is about addressing how adolescents can move into adulthood and learn how to live successfully and manage their chronic illness from a holistic perspective using the concept of resilience. Resilience means how a patient can manage extrinsic factors of life and risk factors (e.g., workload, school, stress, and role) in order to balance any adverse effects from their chronic illness and still maintain a normal lifestyle. ✜ 14
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American Academy of Pediatrics. (2011). Supporting the health care transition from adolescence to adulthood in the medical home. Pediatrics, 128(1), 182-200. doi:10.1542/peds.2011-0969 April, K. T., Feldman, D. E., Zunzunegui, M. V., Descarreaux, M., Malleson, P., & Duffy, C. M. (2009). Longitudinal analysis of complementary and alternative health care use in children with Juvenile idiopathic arthritis. Complimentary Therapies in Women, 17(4), 208-215. doi:10.1016/j. ctim.2009.03.003 Arthritis Foundation. (2013). Medical Care. Helping your kids take an active role in their healthcare. Retrieved from www.kids getarthritistoo.org/living-with-ja/daily-life/transition/growing-upwith-arthritis.php Barlow, J. H., & Ellard, D. R. (2006). The psychosocial well-being of children with chronic disease, their parents and siblings: An overview of the research evidence base. Child Care, Health & Development, 32(1), 19-31. doi:10.1111/j.1365-2214.2006.00591.x Beukelman, T., Haynes, K., Curtis, J. R., Xie, F., Chen, L., Bemrich-Stolz, C. J., …, Safety Assessment of Biological Therapeutics Collaboration. (2012). Rates of malignancy associated with juvenile idiopathic arthritis and its treatment. Arthritis & Rheumatism, 64(4), 1263-1271. doi:10.1002/art.34348 Beukelman, T., Xie, F., Baddley, J. W., Chen, L., Delzell, E., Grijalva, C. G., …, SABER Collaboration. (2013). Brief report: Incidence of selected opportunistic infections among children with juvenile idiopathic arthritis. Arthritis & Rheumatism, 65(5), 1384-1389. doi:10.1002/art.37866 Condon, C., Gormley, J., & Hussey, J. (2009). A review of the physical activity levels of children with juvenile arthritis. Physical Therapy Reviews, 14(6), 411-417. doi:10.1179/108331909X12540993898215 Feldman, D. E., Duffy, C., De Civita, M., Malleson, P., Philibert, L., Gibbon, M, …, Dobkin, P. L. (2004). Factors associated with the use of complementary and alternative medicine in juvenile Idiopathic arthritis. Arthritis & Rheumatism, 51(4), 527-532. doi:10.1002/art.20536 Gartlehner, G., Hansen, R. A., Jonas, B. L., Thieda, P., & Lohr, K. N. (2008). Biologics for the treatment of juvenile idiopathic arthritis: A systematic review and critical analysis of the evidence. Clinics of Rheumatology, 27(1), 67-76. doi:10.1007/s10067-007-0654-6 Goldzweig, O., & Hashkes, P. J. (2011). Abatacept in the treatment of polyarticular JIA: Development, clinical utility, and place in therapy. Drug Design, Development and Therapy, 5, 61-70. doi:10.2147/DDDT.S16489 Horneff, G. (2013). Update on biologicals for treatment of juvenile idiopathic arthritis. Expert Opinion: Biologic Therapies, 13(3), 361-376. doi: 10.1517/14712598.2013.735657 Khan, N. A., Spencer, H. J., Abda, E. A., Alten, R., Pohl, C., Ancuta, C., …, QUEST-RA group. (2012). Patient’s global assessment of disease activity and patient’s assessment of general health for rheumatoid arthritis activity assessment: Are they equivalent? Annals of the Rheumatic Diseases, 71(12), 1942-1949. doi:10.1136/annrheumdis-2011-201142 Lee, Y. C. (2013). Effect and treatment of chronic pain in inflammatory arthritis. Current Rheumatology Reports, 15(1), 300. doi:10.1007/ s11926-012-0300-4 Lin, Y. T., Wang, C. T., Gershwin, M. E., & Chiang, B. L. (2011). The pathogenesis of oligoarticular/polyarticular vs systemic juvenile idiopathic arthritis. Autoimmunity Reviews, 10(8), 482-489. doi:10.1016/j.autrev.2011.02.001 Lipstein, E. A., Muething, K. A., Dodds, C. M., & Britto, M. T. (2013). “I’m the one taking it”: Adolescent participation in chronic disease treatment decisions. Journal of Adolescent Health, 53(2), 253-259. doi:10.1016/j. jadohealth.2013.02.004 Martini, A. (2012). Systemic juvenile idiopathic arthritis. Autoimmunity Reviews, 12(1), 56-59. doi:10.1016/j.autrev.2012.07.022 McDonagh, J. E., Shaw, K. L., & Southwood, T. R. (2006). Growing up and moving on in rheumatology: Development and preliminary evaluation of a transitional care programme for a multicentre cohort of adolescents with juvenile idiopathic arthritis. Journal of Child Health Care, 10(1), 22-42. doi:10.1177/1367493506060203 McMahon, A.-M., & Tattersall, R. (2011). Diagnosing juvenile idiopathic arthritis. Paediatrics and Child Health, 21(12), 552-557. doi:10.1016/j. paed.2011.07.005 January/February 2015
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Merkes, M. (2010). Mindfulness-based stress reduction for people with chronic diseases. Australian Journal of Primary Health, 16(3), 200210. doi:10.1071/PY09063 Modica, R. F., Sukumaran, S., & Milojevic, D. (2012). Pediatric musculoskeletal examination for juvenile arthritis. Pediatric Annals, 41(11), 1-14. doi:10.3928/00904481-20121022-08 Osterkamp, E. M., Costanzo, A. J., Ehrhardt, B. S., & Gormley, D. K. (2013). Transition of care for adolescent patients with chronic illness: Education for nurses. Journal of Continuing Education in Nursing, 44(1), 38-42. doi:10.3928/00220124-20121101-52 Paone, M. C., Wigle, M., & Saewyc, E. (2006). The ON TRAC model for transitional care of adolescents. Progress inTransplantation, 16(4), 291-302. Prahalad, S., Zeft, A. S., Pimentel, R., Clifford, B., McNally, B., Mineau, G. P., …, Bohnsack, J. F. (2010). Quantification of the familial contribution to juvenile idiopathic arthritis. Arthritis and Rheumatism, 62(8), 2525-2529. doi:10.1002/art.27516 Prakken, B., Albani, S., & Martini, A. (2011). Juvenile idiopathic arthritis. The Lancet, 377(9783), 2138-2149. doi:10.1016/s0140-6736(11)60244-4 Russo, E., Trevisi, E., Zulian, F., Battaglia, M. A., Viel, D., Facchin, D., …, Martinuzzi, A. (2012). Psychological profile in children and adolescents with severe course Juvenile Idiopathic Arthritis. The Scientific World Journal, 2012, 841375. doi:10.1100/2012/841375 Sable, C., Foster, E., Uzark, K., Bjornsen, K., Canobbio, M. M., Connolly, H. M., …, Williams, R. G. (2011). Best practices in managing transition to adulthood for adolescents with congenital heart disease:The transition process and medical and psychosocial issues: A scientific statement from the american heart association. Circulation, 123(13), 1454-1485. doi:10.1161/CIR.0b013e3182107c56 Sandborg, C., & Mellins, E. D. (2012). A new era in the treatment of systemic juvenile idiopathic arthritis. The New England Journal of Medicine, 367(25), 2439-2440. doi:10.1056/NEJMe1212640
Sanzo, M. (2008). The child with arthritis in the school setting. Journal of School Nursing, 24(4), 190-196. doi:10.1177/1059840508319630 Schanberg, L. E., Gil, K. M., Anthony, K. K., Yow, E., & Rochon, J. (2005). Pain, stiffness, and fatigue in juvenile polyarticular arthritis: Contemporaneous stressful events and mood as predictors. Arthritis and Rheumatism, 52(4), 1196-1204. doi:10.1002/art.20952 Seid, M., Opipari, L., Huang, B., Brunner, H. I., & Lovell, D. J. (2009). Disease control and health-related quality of life in juvenile idiopathic arthritis. Arthritis and Rheumatism, 61(3), 393-399. doi:10.1002/art.24477 Tong, A., Jones, J., Craig, J. C., & Singh-Grewal, D. (2012). Children’s experiences of living with juvenile idiopathic arthritis: A thematic synthesis of qualitative studies. Arthritis Care & Research, 64(9), 13921404. doi:10.1002/acr.21695 Van Staa, A. L., Jedeloo, S., Van Meeteren, J., & Latour, J. M. (2011). Crossing the transition chasm: Experiences and recommendations for improving transitional care of young adults, parents and providers. Child: Care, Health and Development, 37(6), 821-832. doi:10.1111/ j.1365-2214.2011.01261.x Wang, C. (2012). Role of Tai Chi in the treatment of rheumatologic diseases. Current Rheumatology Reports, 14(6), 598-603. doi:10.1007/ s11926-012-0294-y Ward, L., Stebbings, S., Cherkin, D., & Baxter, G. D. (2013). Yoga for functional ability, pain and psychosocial outcomes in musculoskeletal conditions: A systematic review and meta-analysis. Musculoskeletal Care, 11(4), 203-217. doi:10.1002/msc.1042 Zebracki, K., Holzman, K., Bitter, K. J., Feehan, K., & Miller, M. L., (2007). Brief report: Use of complementary and alternative medicine and psychological functioning in Latino children with juvenile idiopathic arthritis or arthralgia. Journal of Pediatric Psychology, 32(8), 1006-1010. doi:10.1093/jpesy/jsmo33
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15
Growing up with Juvenile Idiopathic
ARTHRITIS J Amy McKeever, PhD, CRNP, WHNP-BC and Michelle M. Kelly, PhD, CRNP
uvenile idiopathic arthritis (JIA) is the most common chronic pediatric illness in the United States and affects approximately 240,000 children and adolescents under the age of 18 years (Arthritis Foundation, 2013). The disease is responsible for inflicting significant musculoskeletal pain, growth limitations, nutritional status impairment, and decreased health-related quality of life (HRQOL) as well as potential conflict and stress among families and peers (Khan et al., 2012; Seid, Opipari, Huang, Brunner, & Lovell, 2009). Juvenile idiopathic arthritis, formerly termed juvenile rheumatoid arthritis encompasses a group of eight heterogeneous chronic arthritis conditions with onset prior to the age of 16 years with symptoms persisting beyond 6 weeks (Prakken, Albani, & Martini, 2011). The JIA conditions are grouped based upon clinical and laboratory findings to enable the use of evidence-based treatment protocols to expedite diagnosis and improve patient outcomes (Prakken et al., 2011). A multidisciplinary management approach is required for JIA, which includes: pharmacological therapy, physical and occupational rehabilitation, and psycho-educational interventions with the patient and
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family, school accommodations, as well as comprehensive nurse navigation and interdisciplinary case management (Russo et al., 2012). An exemplar case is provided in Box 1.
Pathophysiology
The etiology of JIA is unknown, but is thought to be both genetic and environmental, with environmental factors triggering autoimmune deregulation (Lin, Wang, Gershwin, & Chiang, 2011; McDonagh, Shaw, & Southwood, 2006; McMahon & Tattersall, 2011). T cells, particularly CD 8 T cells, play a role in the immune deregulation. Laboratory evaluations of JIA patients point to an increased numbers of T cells from the blood circulation in the joint synovial fluid. In addition, increased levels of cytokines, specifically interleukin 1 through interleukin 12 (IL 1–IL 12) have been reported in the serum samples and synovial fluid of JIA patients (Martini, 2012). Siblings and first degree relatives of patients with JIA have higher rates of autoimmune arthritis; rates are reported from 25% to 40% and rates of JIA among siblings are 15- to 30-fold greater than that of the general pediatric population (Prahalad et al., 2010). The HLA-A2, January/February 2015
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HLA-DRB1*11, and the HLA-DRB1*08 genes have been implicated in JIA (Martini, 2012). Researchers speculate that a child or adolescent who is genetically predisposed to JIA may experience an environmental trigger causing deregulation in the immune response (Lin et al. 2011). Such triggers may include viral infections such as influenza A, rubella, and parvovirus B 19. Mycobacterium pneumonia has also been associated with the onset and exacerbation of JIA. Other factors suspected to trigger the onset of JIA include: maternal smoking during pregnancy (influences the immune system deregulation of the fetus), weather changes (extreme changes in temperature have been implicated in exacerbations and flares), recent vaccination (vaccinations can trigger the immune response with lymphocyte activation), and stress (increases IL-6 production) (Martini, 2012).
Diagnosis Diagnosing JIA can be a difficult task that relies on identification of clinical features. Classification of JIA is based upon the International League of Associations for Rheumatology Taskforce on Classification of ChildJanuary/February 2015
Juvenile idiopathic arthritis is the most common pediatric chronic illness in the United States. hood Arthritis (Table 1) (McMahon & Tatersall, 2011). Hallmark features of arthritis include persistent joint pain, morning stiffness lasting one or more hours, and daily activity limitations (Modica, Sukumaran, & Milojevic, 2012). Children and adolescents with systemic JIA may have a prolonged disease course with a progression to oligoarthritis (affecting one to four joints) or polyarthritis (affecting five or more joints), and may present with the following symptoms: prolonged fever, myalgia, rash, elevated inflammatory markers (erythrocyte sedimentation rate [ESR]), elevated c-reactive protein (CRP), anemia, thrombocytosis, abdominal pain, lymphadenopathy, and splenomegaly. Patients with oligoarthritis frequently MCN
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Exemplar Case of Juvenile Idiopathic Arthritis A 10-year-old girl presented to her pediatrician after a viral infection with right knee swelling. A work-up for Lyme disease was negative. ANA and R.A. labs were negative. On exam her knee was painful, warm to touch with a slight effusion on the right. She was referred to rheumatology. After an uneventful course of 2 years, she began with episodes of joint swelling of her knees, wrists, and 4th and 5th metacarpal joints. She responded well with corticosteriod therapy (Prednisone), and NSAIDs (Naproxen and Meloxicam) but had to discontinue them due to gastrointestinal side effects. On year 3 she was started on Etanercept 25 mg/weekly sq after she began to experience decreased range of motion and functional mobility with amplified pain syndrome in her knees, her feet, and hands. After 4 months into treatment, she responded with improved range of motion, decreased amplified pain, and improved functional mobility. She was also sent for outpatient occupational and physical therapy.
have documented elevated levels of antinuclear antibody antibodies (ANA) serum levels along with joint inflammation, and some patients may present with uveitis (Modica et al., 2012). A comprehensive clinical history and physical assessment with specific attention to the musculoskeletal system is critical. The musculoskeletal examination of a patient suspected of having JIA includes: careful assessment of joints with particular attention to joint swelling due to effusion or hypertrophy, warmth of the joint, pain with palpation or range of motion, and limited range of motion (Modica et al., 2012). Tracking the disease course for the first 6 months is a priority for an accurate diagnosis and prompt treatment. Parents and adolescents may be encouraged to use digital photographs and smartphone calendars to monitor joint swelling and symptoms. Laboratory evaluation includes complete blood cell count, comprehensive metabolic panel, ANA, ESR, CRP, rheumatoid factor, lupus anticoagulant, and HLA B27 gene
Table 1. Classification of Juvenile Idiopathic Arthritis Arthritis Classification
Clinical Manifestation
Prevalence in Males Versus Female
Systemic
Arthritis in one or more joints with daily fever or preceded by daily fever for 2 weeks and accompanied by one of the following: an evanescent nonfixed erythematous rash, generalized lymphadenopathy, hepatomegaly, or splenomegaly or serositis
Affects males and females equally Can begin in childhood
Oligoarticular
Arthritis affecting one to four joints during the first 6 months of disease. Two subcategories: Persistent oligoarthritis: affecting not more than four joints during the disease course Extended oligoarthritis: affecting more than four joints during the disease course
Affects girls more frequently than boys Peaks age of onset: 2–4 years Most common joint affected: Knee
Polyarthritis (rheumatoid factor negative)
Arthritis affecting five or more joints during the first 6 months. Rheumatoid factor test negative
Affects girls more than boys Biphasic onset distribution: Early onset 2–4 years Later peak onset 6–12 years
Polyarthritis (rheumatoid factor positive)
Arthritis affecting five or more joints during the first 6 months. Rheumatoid factor test positive on two separate occasions
Affects girls more than boys Late childhood or adolescence
Psoriatic arthritis
Arthritis and psoriasis, or arthritis alone and at least two of the following: Dactylitis, nail pitting, onycholysis, psoriasis of a first-degree relative
Affects girls more than boys Biphasic onset distribution: Early onset 2–4 years Later peak onset 6–12 years
Enthesitis-related arthritis
Arthritis and Enthesitis or arthritis or Enthesitis with at least two of the following: Presence or history of sacroiliac joint pain, presence of HLA B27 antigen, onset of arthritis in male over 6 years of age, acute anterior uveitis, sacroiliitis with inflammatory bowel disease, Reiter syndrome or acute uveitis in a firstdegree relative
Affects boys more than girls Late childhood or adolescence
Undifferentiated arthritis
Arthritis that persists for at least 6 weeks but does not fulfill criteria for any other category Arthritis that fulfills two or more categories in the JIA spectrum above
*Table recreated using information from McMahon and Tattersall (2011).
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Table 2. Pharmacological Management of Juvenile Idiopathic Arthritis Pharmacological Agent
Pharmacological Drug Class
Dose
Clinical Implications
Adalimumab (Humara)
Human monoclonal antibody anti-TNF antibody
24 mg/m2
Treatment for polyarticular JIA: monotherapy or with methotrexate
Anakinra (Kineret)
Interleukin-1 receptor antagonist
1 mg/kg/day
Used in systemic JIA
Etanercept (Enbrel)
TNF inhibitor Alpha & Beta TNF receptorimmunoglobulin-fusion protein
0.4 mg/kg subcutaneously twice per week
Treatment for JIA - First approved biologic treatment for JIA Used in R.A. factor + and - Psoriatic arthritis, systemic arthritis
Infliximab (Remicade)
TNF inhibitor Chimeric murine-human monoclonal anti-TNF antibody
3 mg/kg intravenous infusion at 0, 2, and 6 weeks. Maintenance dosing every 6 weeks
Not currently FDA approved for JIA, but is used in clinical practice and has demonstrated improved results in RCTs Used often with Crohn disease
Tocilizumab
NR-Interleukin (IL6) receptor antagonist
2–8 mg/kg intravenous infusion at 0 week Maintenance every 2 weeks
Used with active/refractory or systemic JIA
Abatacept
Inhibits T-cell stimulation
10 mg/kg/4 weeks intravenous infusion
Used with active/refractory or systemic JIA that is TNF-unresponsive
typing. Radiological imaging is often the first step in evaluating suspected joint involvement. Magnetic resonance imaging and ultrasonography (US) are also used to detect early JIA disease (McMahon & Tattersall, 2011).
Management Pharmacological Management
Inhibiting cytokines is the priority for pharmacological treatment for the management of JIA (Horneff, 2013). The initial pharmacological therapy for the patients with JIA includes NSAIDs. If synovitis continues after treatment with NSAIDs, immune suppression to decrease joint inflammation is indicated (Lee, 2013). First-line oral immunosuppressant agents often used include: methotrexate—a folic acid antagonist, sulfasalazine—a dihyrofolate reductase inhibitor, and indomethacin—a NSAID/COX 2 inhibitor. The first-line biologic agents for the treatment of JIA are the tumor necrosis factor (TNF) inhibitors including etanercept, adalimumab, and infliximab (Goldzweig & Hashkes, 2011; Horneff). Second-line biologic agents include toclizumab, abatacept, anakinra, canakinumab, and tocilizumab. Other biologics indicated under review include rituximab, golimumab, and certolizumab (Table 2) (Gartlehner, Hansen, Jonas, Thieda, & Lohr, 2008; Horneff; Sandborg & Mellins, 2012). Complementary Alternative Medicine Management
Use of complementary alternative medicine (CAM) modalities has increased in the JIA patient (Wang, 2012; Ward, Stebbings, Cherkin, & Baxter, 2013; Zebracki, Holzman, Bitter, Feehan, & Miller, 2007). It is estimated that 34% to 90% of patients with JIA use CAM January/February 2015
modalities (April et al., 2009). The most common reported CAM modalities reported include: dietary alternations (herbal remedies, and vitamin and mineral supplements) (60–70%), prayer (56%), massage therapy (50%), meditation (30%), topical skin creams for pain relief and joint inflammation (30%), aromatherapy (25%), touch therapy (20%), and herbal medicine (20%) (Merkes, 2010). Adolescents with JIA reported that CAM therapies are primarily used for minimizing symptoms and decreasing pain. Forty-five percent of JIA adolescents who use CAM modalities fail to report their use of CAM to their rheumatologist for concern of disapproval (Feldman et al., 2004; Wang, 2012; Ward et al., 2013). Adolescents and their families may feel more comfortable sharing their CAM use with nursing professionals, particularly if questions regarding its use are posed in a nonjudgmental manner. Researchers noted that parents may choose CAM as their first-line therapy to manage their child’s chronic illness due to misconceptions about pharmacological treatment and fear of adverse effects (Wang, 2012; Ward et al., 2013). Because there has been limited research in the use of CAM with JIA adolescents, there is little evidence to support its effect on symptoms or adherence to standard medical treatments (Ward et al., 2013). Risk of Associated Disease
Opportunistic infections and childhood malignancy are two conditions thought to be associated with treatment modalities used for children with JIA. These complications pose significant concerns for parents and adolescents as they make treatment decisions. Secondary data analysis of records from two United States medical databases, the US Medicaid Analytic extract database and the US Surveillance, Epidemiology and End Results MCN
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(SEER) database from 2000 to 2005, was used to determine standardized incidence rates and risks related to treatment options (Beukelman et al., 2012, 2013). Beukelman et al. (2013) concluded that individuals with JIA have a two-fold increase in opportunistic infections; however, the risk was not related to immunosuppressive medications but was thought to be associated with autoimmune disease. Most prevalent opportunistic infections were herpes zoster, Salmonella, and Coccidioides with herpes zoster being the most frequently treated infection. Based upon the SEER data, it was determined that those individuals with JIA have a 1.4 to 4.5 times higher standardized rate of malignancy than children with attention deficit hyperactivity disorder or asthma. Regardless of treatment modality, children with JIA have an increased risk of malignancy (Beukelman et al., 2012, 2013). Impact of Chronic Illness
Chronic pediatric illness affects the adolescent and family on physical, psychosocial, social, behavioral, educational, and financial levels (Barlow & Ellard, 2006). Many adolescent patients relate their overall well-being to their perceived psychosocial well-being, their status with peers, and their perceived body image. When the disease interferes with JIA adolescents’ ability to socialize, attend school regularly, and participate in age-related activities with their peers, their psychosocial well-being, sense of normalcy, and peer relationships are negatively affected (Sanzo, 2008; Schanberg, Gil, Anthony, Yow, Rochon, 2005). For example, Russo et al. (2012) reported that families with a child or adolescent diagnosed with JIA selfreported a change in family and social relationships. Of 33 JIA patients surveyed, 50% reported prolonged school absences due to disease flares, 70.8% reported halting organized sports, 66.6% reported attending regular physical
Figure 1. Model of Transitional Care for Adolescents with Juvenile Idiopathic Arthritis
Pharmacological Management
Psychological Health
Independent Living
ADOLESCENT Education College/ Vocational Training
Reproductive Issues
Parental Involvement
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Social Issues
Juvenile idiopathic arthritis encompasses eight chronic arthritis conditions in children under the age of 18 years.
and occupation rehabilitation sessions, and 50% reported needing an assistive aid for their affected joints (Russo et al., 2012). When asked about their social interaction having an impact on their disease status, 75% reported that they failed to tell anyone about their disease for fear of shame or discrimination, 91.6% relate that their disease interferes with their daily living and HRQOL, and 41.6% reported that their JIA had a negative impact on their body image. Additionally, 91% of adolescents with JIA were worried about the current and future state of disease control, their body image, and current and potential disabilities related to JIA (Russo et al., 2012). Tong, Jones, Craig, and Singh-Grewal (2012) interviewed JIA patients using qualitative methods and identified six themes about how their disease had an impact on their life. Themes identified included: aversion to being different, striving for normality, stigma and misunderstanding, suspension in uncertainty, managing treatment, and desire for knowledge about their disease. In addition, Tong and colleagues noted that school and social functioning were recognized by JIA adolescents as an important part of their HRQOL. An adolescent with JIA also reported that pain and fatigue were two disease factors that negatively affected academic performanc and school attendance. For example, the early morning routines and later activities for athletics and clubs involved in high school are not optimal for the JIA client as proper sleep and periods of rest are paramount to control disease flares (Khan et al., 2012). Promotion of normal routines is paramount, and is facilitated by adequate disease management, and regular activity (Khan et al., 2012). January/February 2015
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Table 3. Recommendations for Successful Transitional Care Programs Providers/Nurses • Design a structured transition program with step-by-step processes that begin in early adolescence • Appoint a key healthcare provider (nurse/APN) to begin the transitional program • Work systematically to foster independence with adolescent beginning early in adolescence • Have parents included in visit, but allow adolescent to lead the discussion during visit • Work at each visit to encourage ownership and independence of disease management • Discuss self-management of pharmacological agents • Discuss life issues: work, career, sexuality, intimate relationships, higher education • Provide multidisciplinary referrals or consultation
Young Adults
Parents
• Voice your opinion • Meet with adult care providers before selecting one • Obtain access to chart and medical records • Participate in healthcare decisions • Communicate clearly with healthcare provider and staff healthcare needs and concerns about chronic illness • Attend visits without a family member • Verbalize understanding and willingness to begin transfer of care • Schedule own appointments • Verbalize understanding of disease management regimens • Set goals for education and employment • Verbalize understanding of impact of substance use, smoking, and pregnancy on disease • Use nurse navigator to assist in transition
• Arrange meeting with pediatric rheumatologist to discuss transfer plans • Assist adolescent if needed in meeting adult rheumatologist • Communicate with adolescent the need for ownership of care • Allow adolescent to schedule own appointments • Allow adolescent to attend visits alone prior to transfer • Allow adolescent to take ownership of disease management • Meet with pediatric nurse/ANP who will assist in coordinating transition
Transitional Care The American Academy of Pediatrics (2011) considers comprehensive transitional care programs for adolescents with chronic illness a healthcare priority. Advancement in the treatment of JIA with biologic therapies and immunosuppressant therapy has increased HRQOL. As children born with complex chronic illnesses are now surviving into adulthood and leading independent lives, programs transitioning from pediatric care to adult care are of critical importance (Osterkamp, Costanzo, Ehrhardt, & Gormley, 2013; Paone, Wigle, & Saewyc, 2006; Sable et al., 2011). Transition of care refers to the continuum of care that begins during the pediatric period and ends when the adolescent is transitioned to their adult healthcare provider and into the adult healthcare system with all interdisciplinary referrals in place. The term transition of care is often misused assuming that transition of care only refers to the movement of the adolescent’s medical records. However, that is only one small component of the transitional program (Paone et al., 2006). McDonagh et al. (2006) have identified key components of effective transitional care including: skills training for the adolescent (e.g., communication, decision making, assertiveness, and self-management), inclusion of the caregiver/parent, young-person centered, interdisciplinary/multidisciplinary focused, involves pediatric and adult healthcare services, and holistic focused (Lipstein, Meuthing, Dodds, & Britto, 2013). Preparation must begin early to ensure adolescents develop the knowledge and skills to take ownership of their chronic illness and disease management. Healthcare providers must be well educated and aware of the developmental issues that present in the adolescent years. The nurse is a critical provider to navigate, educate, and coordinate this transition. As adolescents move from parental control to independent living, their healthcare is of critical January/February 2015
Adolescents should take an active role in their disease management as this is a crucial step in the transition of care. importance (Van Staa, Jedeloo, Van Meeteren, & Latour, 2011). According to Barlow and Ellard (2006), the major concerns of adolescents with chronic illness who move into adult care are going into the unknown, going into a different world, disrupting relationships and ways of working, a shift in roles and responsibilities between the adolescent and adult, and recommendations for improving processes and personal growth. Recommendations for successful transition include: improving patient and parental preparation and improving communication between the pediatric and adult rheumatologist (Table 3) (Van Staa et al., 2011).
Clinical Nursing Implication In healthcare facilities, the nurse is a key member of the healthcare team focused on development and implementation of transitional care for the chronically ill adolescent (McDonagh et al., 2006). As transition of care is an ongoing process that begins in early adolescence and continues through early adulthood, the important features of disease management for the JIA patient in transition of care include: self-awareness of disease; identification of potential triggers to flares; symptom management with appropriate rest, pain medication, and complementary therapy; the comprehensive understanding of drug treatment protocol and potential toxicities; and preparing the adolescent for lifestyle changes with higher education and employment. Critical issues for the nurse to address for adolescent patient MCN
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Clinical Implications
• Transitional care assists adolescents to successfully care for their chronic illness, achieve life goals, and to function independently leading a productive life.
Amy McKeever is an Assistant Professor, Villanova University, College of Nursing, Villanova, PA. She can be reached via e-mail at [email protected] Michelle M. Kelly is an Assistant Professor, Villanova University, College of Nursing, Villanova, PA. She can be reached via e-mail at [email protected] The authors declare no conflict of interest.
• Transitional care begins in early adolescence and should continue until adult care is well-established.
DOI:10.1097/NMC.0000000000000096
• Successful transitional care is based upon an individualized approach based upon the JIA’s particular disease.
References
• The nurse is a key member of the transitional healthcare team for pediatric and adolescent patients with chronic illness.
• Transitional care requires care coordination with the interdiscinplinary healthcare team.
include patient education such as risks of substance use with the adolescent’s pharmacological therapy, ramifications of sexual behavior with immunosuppressant therapy, unintended pregnancy and teratogens, potential complications associated with pregnancy, as well as the importance of family planning measures to prevent or delay pregnancy. Discussions on family planning with acceptable contraceptive options must be included in the transitional care program, including risk reduction education on delaying or abstaining from sexual intercourse, and risk reduction measures if the adolescent is already sexually active. Adolescent and young adult females must be well informed about risks and complications of pregnancy with their chronic illness. Those females with JIA are at increased risk throughout the pregnancy, and management with a multidisciplinary team is necessary to reduce maternal and fetal morbidity and mortality (McDonagh et al., 2006). Successful transition must be individualized to consider the developmental level of the JIA patient, the severity of the adolescent’s particular JIA illness, their disease stability, their personal goals, and the ability of that patient to take ownership of their disease. Transitional care requires planning, care coordination, and collaboration among the interdisciplinary team members as well as the JIA patient and their family, with facilitation by the nurse. The objectives of an effective transition of care is for the adolescent to reach a level of independence in caring for their chronic illness in young adulthood, to achieve their goals in life, and to be able to function as a productive member of society while at the same time living and managing a chronic illness. Nurses must encourage adolescents to take ownership of their disease management and health promotion activities that are critical pieces in transitional care (Condon, Gormley, & Hussey, 2009; Sanzo, 2008). There are large number of chronically ill children and adolescents throughout the United States. Nurses are key members of the interdisciplinary health team leading the successful transition of care. Transitional care is about addressing how adolescents can move into adulthood and learn how to live successfully and manage their chronic illness from a holistic perspective using the concept of resilience. Resilience means how a patient can manage extrinsic factors of life and risk factors (e.g., workload, school, stress, and role) in order to balance any adverse effects from their chronic illness and still maintain a normal lifestyle. ✜ 14
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American Academy of Pediatrics. (2011). Supporting the health care transition from adolescence to adulthood in the medical home. Pediatrics, 128(1), 182-200. doi:10.1542/peds.2011-0969 April, K. T., Feldman, D. E., Zunzunegui, M. V., Descarreaux, M., Malleson, P., & Duffy, C. M. (2009). Longitudinal analysis of complementary and alternative health care use in children with Juvenile idiopathic arthritis. Complimentary Therapies in Women, 17(4), 208-215. doi:10.1016/j. ctim.2009.03.003 Arthritis Foundation. (2013). Medical Care. Helping your kids take an active role in their healthcare. Retrieved from www.kids getarthritistoo.org/living-with-ja/daily-life/transition/growing-upwith-arthritis.php Barlow, J. H., & Ellard, D. R. (2006). The psychosocial well-being of children with chronic disease, their parents and siblings: An overview of the research evidence base. Child Care, Health & Development, 32(1), 19-31. doi:10.1111/j.1365-2214.2006.00591.x Beukelman, T., Haynes, K., Curtis, J. R., Xie, F., Chen, L., Bemrich-Stolz, C. J., …, Safety Assessment of Biological Therapeutics Collaboration. (2012). Rates of malignancy associated with juvenile idiopathic arthritis and its treatment. Arthritis & Rheumatism, 64(4), 1263-1271. doi:10.1002/art.34348 Beukelman, T., Xie, F., Baddley, J. W., Chen, L., Delzell, E., Grijalva, C. G., …, SABER Collaboration. (2013). Brief report: Incidence of selected opportunistic infections among children with juvenile idiopathic arthritis. Arthritis & Rheumatism, 65(5), 1384-1389. doi:10.1002/art.37866 Condon, C., Gormley, J., & Hussey, J. (2009). A review of the physical activity levels of children with juvenile arthritis. Physical Therapy Reviews, 14(6), 411-417. doi:10.1179/108331909X12540993898215 Feldman, D. E., Duffy, C., De Civita, M., Malleson, P., Philibert, L., Gibbon, M, …, Dobkin, P. L. (2004). Factors associated with the use of complementary and alternative medicine in juvenile Idiopathic arthritis. Arthritis & Rheumatism, 51(4), 527-532. doi:10.1002/art.20536 Gartlehner, G., Hansen, R. A., Jonas, B. L., Thieda, P., & Lohr, K. N. (2008). Biologics for the treatment of juvenile idiopathic arthritis: A systematic review and critical analysis of the evidence. Clinics of Rheumatology, 27(1), 67-76. doi:10.1007/s10067-007-0654-6 Goldzweig, O., & Hashkes, P. J. (2011). Abatacept in the treatment of polyarticular JIA: Development, clinical utility, and place in therapy. Drug Design, Development and Therapy, 5, 61-70. doi:10.2147/DDDT.S16489 Horneff, G. (2013). Update on biologicals for treatment of juvenile idiopathic arthritis. Expert Opinion: Biologic Therapies, 13(3), 361-376. doi: 10.1517/14712598.2013.735657 Khan, N. A., Spencer, H. J., Abda, E. A., Alten, R., Pohl, C., Ancuta, C., …, QUEST-RA group. (2012). Patient’s global assessment of disease activity and patient’s assessment of general health for rheumatoid arthritis activity assessment: Are they equivalent? Annals of the Rheumatic Diseases, 71(12), 1942-1949. doi:10.1136/annrheumdis-2011-201142 Lee, Y. C. (2013). Effect and treatment of chronic pain in inflammatory arthritis. Current Rheumatology Reports, 15(1), 300. doi:10.1007/ s11926-012-0300-4 Lin, Y. T., Wang, C. T., Gershwin, M. E., & Chiang, B. L. (2011). The pathogenesis of oligoarticular/polyarticular vs systemic juvenile idiopathic arthritis. Autoimmunity Reviews, 10(8), 482-489. doi:10.1016/j.autrev.2011.02.001 Lipstein, E. A., Muething, K. A., Dodds, C. M., & Britto, M. T. (2013). “I’m the one taking it”: Adolescent participation in chronic disease treatment decisions. Journal of Adolescent Health, 53(2), 253-259. doi:10.1016/j. jadohealth.2013.02.004 Martini, A. (2012). Systemic juvenile idiopathic arthritis. Autoimmunity Reviews, 12(1), 56-59. doi:10.1016/j.autrev.2012.07.022 McDonagh, J. E., Shaw, K. L., & Southwood, T. R. (2006). Growing up and moving on in rheumatology: Development and preliminary evaluation of a transitional care programme for a multicentre cohort of adolescents with juvenile idiopathic arthritis. Journal of Child Health Care, 10(1), 22-42. doi:10.1177/1367493506060203 McMahon, A.-M., & Tattersall, R. (2011). Diagnosing juvenile idiopathic arthritis. Paediatrics and Child Health, 21(12), 552-557. doi:10.1016/j. paed.2011.07.005 January/February 2015
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Merkes, M. (2010). Mindfulness-based stress reduction for people with chronic diseases. Australian Journal of Primary Health, 16(3), 200210. doi:10.1071/PY09063 Modica, R. F., Sukumaran, S., & Milojevic, D. (2012). Pediatric musculoskeletal examination for juvenile arthritis. Pediatric Annals, 41(11), 1-14. doi:10.3928/00904481-20121022-08 Osterkamp, E. M., Costanzo, A. J., Ehrhardt, B. S., & Gormley, D. K. (2013). Transition of care for adolescent patients with chronic illness: Education for nurses. Journal of Continuing Education in Nursing, 44(1), 38-42. doi:10.3928/00220124-20121101-52 Paone, M. C., Wigle, M., & Saewyc, E. (2006). The ON TRAC model for transitional care of adolescents. Progress inTransplantation, 16(4), 291-302. Prahalad, S., Zeft, A. S., Pimentel, R., Clifford, B., McNally, B., Mineau, G. P., …, Bohnsack, J. F. (2010). Quantification of the familial contribution to juvenile idiopathic arthritis. Arthritis and Rheumatism, 62(8), 2525-2529. doi:10.1002/art.27516 Prakken, B., Albani, S., & Martini, A. (2011). Juvenile idiopathic arthritis. The Lancet, 377(9783), 2138-2149. doi:10.1016/s0140-6736(11)60244-4 Russo, E., Trevisi, E., Zulian, F., Battaglia, M. A., Viel, D., Facchin, D., …, Martinuzzi, A. (2012). Psychological profile in children and adolescents with severe course Juvenile Idiopathic Arthritis. The Scientific World Journal, 2012, 841375. doi:10.1100/2012/841375 Sable, C., Foster, E., Uzark, K., Bjornsen, K., Canobbio, M. M., Connolly, H. M., …, Williams, R. G. (2011). Best practices in managing transition to adulthood for adolescents with congenital heart disease:The transition process and medical and psychosocial issues: A scientific statement from the american heart association. Circulation, 123(13), 1454-1485. doi:10.1161/CIR.0b013e3182107c56 Sandborg, C., & Mellins, E. D. (2012). A new era in the treatment of systemic juvenile idiopathic arthritis. The New England Journal of Medicine, 367(25), 2439-2440. doi:10.1056/NEJMe1212640
Sanzo, M. (2008). The child with arthritis in the school setting. Journal of School Nursing, 24(4), 190-196. doi:10.1177/1059840508319630 Schanberg, L. E., Gil, K. M., Anthony, K. K., Yow, E., & Rochon, J. (2005). Pain, stiffness, and fatigue in juvenile polyarticular arthritis: Contemporaneous stressful events and mood as predictors. Arthritis and Rheumatism, 52(4), 1196-1204. doi:10.1002/art.20952 Seid, M., Opipari, L., Huang, B., Brunner, H. I., & Lovell, D. J. (2009). Disease control and health-related quality of life in juvenile idiopathic arthritis. Arthritis and Rheumatism, 61(3), 393-399. doi:10.1002/art.24477 Tong, A., Jones, J., Craig, J. C., & Singh-Grewal, D. (2012). Children’s experiences of living with juvenile idiopathic arthritis: A thematic synthesis of qualitative studies. Arthritis Care & Research, 64(9), 13921404. doi:10.1002/acr.21695 Van Staa, A. L., Jedeloo, S., Van Meeteren, J., & Latour, J. M. (2011). Crossing the transition chasm: Experiences and recommendations for improving transitional care of young adults, parents and providers. Child: Care, Health and Development, 37(6), 821-832. doi:10.1111/ j.1365-2214.2011.01261.x Wang, C. (2012). Role of Tai Chi in the treatment of rheumatologic diseases. Current Rheumatology Reports, 14(6), 598-603. doi:10.1007/ s11926-012-0294-y Ward, L., Stebbings, S., Cherkin, D., & Baxter, G. D. (2013). Yoga for functional ability, pain and psychosocial outcomes in musculoskeletal conditions: A systematic review and meta-analysis. Musculoskeletal Care, 11(4), 203-217. doi:10.1002/msc.1042 Zebracki, K., Holzman, K., Bitter, K. J., Feehan, K., & Miller, M. L., (2007). Brief report: Use of complementary and alternative medicine and psychological functioning in Latino children with juvenile idiopathic arthritis or arthralgia. Journal of Pediatric Psychology, 32(8), 1006-1010. doi:10.1093/jpesy/jsmo33
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January/February 2015
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