Course:KIN366/ConceptLibrary/JuvenileRheumatoidArthritis
| Movement Experiences for Children | |
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| KIN 366 | |
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| Instructor: | Dr. Shannon S.D. Bredin |
| Email: | shannon.bredin@ubc.ca |
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Juvenile rheumatoid arthritis commonly also referred to as juvenile idiopathic arthritis in North America or juvenile chronic arthritis in Europe (Doudkani-Fard et al., 2014). This disease is the most common chronic autoimmune disorder that occurs in childhood and encompasses a subset of disorders (Doudkani-Fard et al, 2014). Typically juvenile rheumatoid arthritis is associated with stiff and inflamed joints, pain and lethargy (Cavallo, April, Grandpierre, Majnemer, & Feldman, 2014). This disorder only appears in childhood, as individuals must present the onset of symptoms for a minimum of 6 weeks, before the age of 16 to be diagnosed with juvenile rheumatoid arthritis (Doudkani-Fard et al., 2014). Though rheumatoid arthritis, developed in adulthood, share similar symptoms, it differs from juvenile rheumatoid arthritis because this disorder in adulthood is thought to be a permanent, lifelong disorder while in the majority of cases children out grow this condition (Hochberg, 1981). Currently there is no definite cause of juvenile rheumatoid arthritis but it is speculated that both genetic and environmental components play a role (Ravelli & Martini, 2007). Treatment is classically targeted towards pain management with the use of nonsteroidal anti-inflammatory drug (NSAIDS) and corticosteroid injections (Schneider & Passo, 2002). However, physical activity has become increasingly utilized as a treatment option to reduce inflammation, increase muscular strength and range of motion and maintain bone mineral density (Philpott, Houghton, & Luke, 2010).
Subtypes
The global disorder of juvenile rheumatoid arthritis is divided into 5 different classifications.
Systemic Arthritis
Systemic arthritis develops throughout childhood and distinguishes itself from other forms of juvenile rheumatoid arthritis because of its distinct rash and fever that occurs along side arthritic symptoms (Schneider & Passo, 2002). Anemia, low red blood cell levels is often present, along with potential inflammation of internal organs and lymph nodes (Ravelli & Martini, 2007).
Oligoarticular Arthritis
Oligoarticular arthritis affects up to four different joints throughout the body for the first six months of the disease onset (Ravelli & Martini, 2007). Oligoarticular arthritis is then divided into persistent and extended components. Persistent oligoarticular arthritis affects a maximum of four joints throughout the progression of the disease, while extended oligoarticular arthritis can progress to affect greater than four joints after the first six months (Schneider & Passo, 2002).
Polyarticular Arthritis
If five joints or more are affected by arthritic symptoms during the first six months of obtaining the disease, the disorder is then classified as polyarticular arthritis (Schneider & Passo, 2002).
Enthesitis Arthritis
Enthesitis refers to the inflammation at the insertion of the tendon on the bone (Ravelli & Martini, 2007). This condition exhibits enthesitis symptoms along with arthritic symptoms. Lower extremities are typically affected first, specially, the insertion of the Achilles tendon on the calcaneus (Ravelli & Martini, 2007).
Psoriatic Arthritis
Psoriatic arthritis is characterized by arthritic symptoms accompanied by a psoriatic rash (Ravelli & Martini, 2007). As well, dactyliti, inflammation of the fingers and onycholysis, separation of the nail from the skin on the finger are also associated with this disorder (Schneider & Passo, 2002).
Signs & Symptoms
Each case of juvenile rheumatoid arthritis possesses a variety of symptoms and rarely incidents are uniform but the primary characteristic is the presence of arthritic symptoms including, pain and stiffness in the joints for a minimum of 6 weeks before the age of 16 (Doudkani et al., 2014). Arthritis is defined as inflammation of the joints leading to severe pain during movements around affected joints and fatigue (Cavallo et al., 2002). Inflammation is due to fluctuations in ion levels and growth factors impeding nociceptors, pain receptors within joints (Van Oort, Tupper, Rosenberg, Farthing, & Baxter-Jones, 2013).Typically joint pain is worse in the morning and subsides throughout the day (Cavallo et al., 2002). As the disorder progresses, joints may become bent, deformed and unable to be straightened (Cavallo et al., 2002). In addition, rashes, fevers and inflammation of other aspects of the body are also associated with juvenile rheumatoid arthritis (Ravelli & Martini, 2007).
Growth Disturbances
Growth disturbances occur in children with juvenile rheumatoid arthritis when inflammation expands towards the location of a growth plates on the bones of the extremities (Ravelli & Martini, 2007). This inflammation may result in over growth of the bones of the extremities as the inflammation causes accelerated growth of ossification centers (Schneider & Passo 2002). Under development of bones may appear due to adverse effects of corticosteroid therapy, which reduces linear bone growth or because of the catabolic nature of the active arthritis disease (Schneider & Passo, 2002). Therefore, globalized short stature is a symptom associated with juvenile rheumatoid arthritis, however to combat this abnormality growth hormone is often supplemented (Schneider & Passo, 2002). Functional abnormalities such as leg length discrepancies are also common, as well as various cosmetic imperfections (Schneider & Passo, 2002).
Osteoporosis
Osteoporosis is defined as the loss of both the mineral and matrix constituents found in bone, resulting in a decreased bone density (Ravelli & Martini, 2007). This poses a problem for child development because during puberty, growth is accelerated to reach skeletal maturity and insufficient bone density throughout this period will increase the child’s vulnerability to brittle bones and subsequent fractures (Schneider & Passo, 2002). Fractures are a common consequence of osteoporosis and are often seen in children with juvenile rheumatoid arthritis. Decrease in bone density is a result of inadequate calcium intake, corticosteroid therapy, insufficient weight barring activities, delayed puberty and due to the nature of the arthritis disease activity (Schneider & Passo, 2002). Calcium and vitamin D supplements are often recommended to prevent osteoporosis and maintain an appropriate level of bone density (Schneider & Passo).
Cause
The cause of juvenile rheumatoid arthritis remains unknown; however there are many hypothesized theories as to the origin (Ravelli & Martini, 2007). It is likely that both environmental and genetic factors are responsible for this disorder (Ravelli & Martini, 2007). The genetic component is thought to involve several genes within the HLA region of chromosome 6; genes associated with inflammation and immunity responses (Ravelli & Martini, 2007; Weiss & Ilowite, 2005). It is also speculated that psychological stress can trigger arthritis if the necessary genes are present; as well joint trauma, irregular hormone levels, and bacterial or viral infections are potential causes though none of these theories have been proven to be the correct origin in all juvenile rheumatoid arthritis cases (Weiss & Ilowite, 2005).
Diagnosis
The first step of diagnosis of juvenile rheumatoid arthritis is to eliminate different types of arthritis and other possible diagnoses (Doudkani-Fard et al., 2014). Unfortunately, there are no concrete laboratory tests to confirm the diagnosis because a cause has not yet been identified (Doudkani-Fard et al., 2014). However, tests such as blood cell count, analysis of blood proteins, immunoglobulin examination and antibody investigation are typically completed to measure inflammation (Doudkani-Fard et al., 2014). If juvenile rheumatoid arthritis is speculated, radiology and magnetic resonance imaging (MRI) are then completed to assess the joints, because early diagnosis is important to prevent irreversible damage to the joints (Doudkani-Fard et al., 2014). Adenosine deagminase has also been proven to be a suitable biomarker for diagnostic testing (Doudkani-Fard et al., 2014). However, due to the lack of knowledge regarding the cause of the disease and the wide range of symptoms associated with juvenile rheumatoid arthritis, confirming a diagnosis in patients is often a difficult and inconclusive process (Schneider & Passo, 2002).
Treatment & Management
There is no known cure for juvenile rheumatoid arthritis, therefore treatment emphasizes pain management, maintenance of physical function and prevention of permanent joint damage (Schneider & Passo, 2002; Ravelli & Martini, 2007). However, questionnaires and counselling sessions have been incorporated into the treatment to assess the patients’ quality of life and ensure that both the patient and their family are well supported (Schneider & Passo, 2002).
Medication
NSAIDS such as ibuprofen is first given to relieve joint pain and is typically used because of its limited side effects, intra-articular corticosteroid therapy is then utilized to reduce inflammation and stiffness if the NSAIDS do not provide sufficient relief (Schneider & Passo, 2002). Methotrexate therapy is another common treatment option, as it has been shown to decrease cartilage break down and typically is effective within 6-12 weeks of using this therapy (Ravelli & Martini, 2007).
Side Effects
Though methotrexate therapy is the most beneficial treatment option in diminishing arthritic symptoms to combat juvenile rheumatoid arthritis, it is also associated with negative effects on the liver and stomach ulcers. Therefore, methotrexate therapy is often paired with folic acid to offset the adverse effects (Ravelli & Martini, 2007). Corticosteroid therapy, though effective as a treatment option for individuals with juvenile rheumatoid arthritis have the potential to become toxic and increase an individual heart rate to dangerous levels (Ravalli & Martini, 2007). In addition, corticosteroid therapy has been known to produce increases in depression and anxiety related symptoms (Ravelli & Martini, 2007). This highlights the importance of emotional and psychosocial resources in terms of treatment options as opposed to purely treatment of physiological abnormalities.
Stem Cell Transplants
Bone marrow transplants are a relatively new form of treatment used to regenerate degrading cartilage in severe cases (Kim, 2010). However the current evidence is inconclusive regarding the success of this technique, but there has been beneficial outcomes for approximately 60-65% of patients with the use of this treatment option (Kim, 2010).
Questionnaires
The Childhood Health Assessment Questionnaire (CHAQ) measures the degree of disability of those with juvenile rheumatoid arthritis, this questionnaire scores on a scale of 0 to 3, 0 representing no disability and 3 representing the most severe case of disability (Schneider & Passo, 2002). The Juvenile Arthritis Quality of Life Questionnaire (JAQQ) and Childhood Arthritis Health Profile (CAHP) measure quality of life and physical function respectively in this population (Schneider & Passo, 2002).
Physiotherapy
Children with juvenile rheumatoid arthritis classically have physiotherapists to prevent or resolve biomechanical abnormalities (Ravelli & Martini, 2007). Massage and soft tissue release is also recommended to release stress on the joints (Ravelli & Martini, 2007). Specially, sports such as swimming and cycling are recommended because these activities provide less stress and weight barring strain on the joints (Ravelli & Martini, 2007). Physiotherapists work with patients to insure proper biomechanics and fix any abnormalities that may cause additional stress or developmental delays (Ravelli & Martini, 2007).
Statistics
As of 2011 it has been recorded that approximately 1 in 6 Canadians currently report having arthritis and 3 out of every 1,000 of Canadian children (The Arthritis Society, 2015). Therefore, approximately 24,000 children in Canada, under the age of 18 suffer from a form of arthritis (The Arthritis Society, 2015).
Physical Activity Trends
Due to the pain and stiffness associate with juvenile rheumatoid arthritis, children with this disorder are more likely to live a more sedentary lifestyle compared to healthy children of equal age (Cavallo et al., 2014). Sedentary lifestyles are highly correlated to increased risk factors for all-cause mortality in adulthood (Klepper, 2003). Specifically, less than 40% of children with juvenile rheumatoid arthritis reach the recommended 60 minutes of physical activity each day (Cavallo et al., 2014), compared to 60% of healthy children that reach the same recommendations. In terms of participation in physical activity, whether within the school environment or in afterschool activities, children with juvenile rheumatoid arthritis have been documented to be less active and refrain from physical activity in general, especially in terms of moderate to vigorous intensity (Cavallo et al., 2014). Decrease in daily activity levels is likely correlated with the decline in cardiopulmonary and aerobic capacity and range of motion seen in this population (Apti, Kasapcopur, Mengi, Ozturk, & Metin, 2014). In addition, muscular strength, size and power are aspects recorded to be reduced in children with juvenile rheumatoid arthritis, compare to age matched controls (Klepper, 2003). It is important to note that research does not support the idea that physical activity exasperates disease related symptoms, such as increases in joint swelling (Cavallo et al., 2014). On the contrary, to patients beliefs, exercise has been shown to decrease pain and chronic inflammation, improve bone health and benefit immune function (Cavallo et al., 2014). Inflammation is minimized as circulation increases with physical activity and increases in heart rate and blood flow (Van Oort et al., 2013). Anemia, decreased oxygen carrying capacity, is a symptom of juvenile rheumatoid arthritis, which may contribute to decreased aerobic capacity, resulting in muscle atrophy and weakness (Klepper, 2003). Whether this decrease in participation is due to the pain and often abnormal biomechanical abnormalities associated with the disorder or the influence of parental figures restricting their child’s activities to maintain safety and prevent injury, it is important from all children to engage in physical activity to enhance cognitive, social and physical development (Cavallo et al., 2014). Physical activity is critical in childhood to create friendships, gain self-confidence and self-esteem and maintain a healthy lifestyle and appropriate body weight (Cavallo et al., 2014). In children with juvenile rheumatoid arthritis, levels of quality of life are often lower than other children without this disorder, it is hypothesized that the lack of involvement in activities with their peers result in feelings of depression and social isolation (Cavallo et al., 2014). However, research been shown that children with juvenile rheumatoid arthritis report spending more time with family members and engage more frequently in activities with family as opposed to friends (Cavallo et al., 2014).
Physical Activity Recommendations & Benefits
Difficulty may arise when participating in physical activities whether structured or unstructured play, as many children with this disorder are restricted in their movement and types of activities they are comfortable participating in (Schneider & Passo 2002. Participation in weight barring activities have been noted in this population, therefore sports such as swimming and cycling are recommended because these activities provide less stress and weight barring strain on the joints (Cavallo et al., 2014; Ravelli & Martini, 2007). Water exercises specifically have shown to increase hip range of motion and decrease the number of affected joints (Apti et al., 2014).
Physiotherapy is the main source of guidance patients with juvenile rheumatoid arthritis utilize when seeking guidance and attempting to maintain physical fitness and range of motion, however, these sessions can become time consuming and expensive (Sandstedt, Fasth, Eek, & Beckung, 2013). Therefore, home-based exercise programs are recommended and show approximately 70% adherence rates (Sandstedt et al., 2013). Home based exercise programs are targeted to increase both physical function, such as cardiopulmonary capacity and range of motion, of individuals with juvenile rheumatoid arthritis, as well as quality of life and psychological well being assessed by the CHAQ (Apti et al., 2014). Exercise programs typically include weight bearing activities, such as walking or jogging, simple free weights and muscle strength training exercises (Sandstedt et al., 2013). Jump rope has become an increasingly popular recommendation for children with juvenile rheumatoid arthritis as is it well tolerated by the individual, resulting in marked increases in muscular strength and bone density (Sandstedt et al., 2013). A minimum of six week home based exercise program recommended for individuals with juvenile rheumatoid arthritis, as it is typically pain free and show numerous beneficial health outcomes as well as a 6% increase in muscle hypertrophy, and also increases in energy levels and decrease in medication use (Sandstedt et al., 2013 & Philpott et al., 2010). Exercise bouts of 30 to 60 minutes and frequencies between 1 to 3 times a week are recommended (Klepper, 2003). Flexibility and stretching exercises, before and after exercise sessions are also recommended (Philpott et al., 2010). In addition, supervised resistance exercise can also be added into exercise routines (Klepper, 2003).
Though physical activity results in numerous health benefits, it is important to slowly increase intensity and duration, and discontinue participation in any activities that exasperates pain. (Difficulty also arises due to the lack of specific safe guidelines for physical activity participation, particularly in special populations, therefore it is important to watch progression slowly and participation in activities tailored to each individuals abilities and preferences (Philpot et al., 2010).
References
The Arthritis Society (2015). Arthritis in Canada: Facts & figures. Retreieved from: http://www.arthritis.ca/document.doc?id=925
Cavallo, S., April, K. T., Grandpierre, V., Majnemer, A., & Feldman D. E. (2014). Leisure in children and adolescents with juvenile idiopathic arthritis: A systematic review. PLoS ONE, 9(10). doi:10.1371/journal.pone.0104642
Dogru Apti, M. D., Kasapcopur, O., Mengi, M., Ozturk, G., & Metin, G. (2014). Regular aerobtic training combined with range of motion exercises in juvenile idiopathic arthritis. Biomed Research International, 1–6. doi:10.1155/2014/748972
Doudkani-Fard ,M., Ziaee, V., Moradinejad, M. H., Sedaghat, M., Haghi-Ashtiani, M. T., & Ahmadinejad, Z. (2014). Sensitivity and specificity of adenosine deaminase in diagnosis of juvenile idiopathic arthritis. Medical Journal of the Islamic Republic of Iran, 28(113). Retrieved from: http://www-ncbi-nlm-nih-gov.ezproxy.library.ubc.ca/pmc/articles/PMC4313457/pdf/MJIRI-28-113.pdf
Hochberg, M.C. (1981). Adult and juvenile rheumatoid arthritis: Current epidemiologic concepts. Epidemiology Review, 3: 27-44. Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/7030763
Klepper, S.E. (2003). Exercise and fitness in children with arthritis: Evidence of benefits for exercise and physical activity. Arthritis Care & Research 49(3), 435-443. doi:10.1002/art.11055
Kim, K.N. (2010). Treatment of juvenile rheumatoid arthritis. Korean Journal of Pediatrics, 53(11): 936-941. doi:10.3345/kjp.2010.53.11.936
Philpott, J., Houghton, K., & Luke, A. (2010). Physical activity recommendations for children with specific chronic health conditions: Juvenile idiopathic arthritis, hemophilia, asthma and cystic fibrosis. Paediatric Child Health, 15(4), 213-218. doi:10.1097/jsm.0b013e3181d2eddd
Ravelli, A., & Martini, A. (2007). Juvenile idiopathic arthritis. The Lancet, 369(9563): 767-778. doi:10.1016/s0140-6736(07)60363-8
Sandstedt, E., Fasth, A., Eek, M.N., & Beckung, E. (2013). Muscle strength, physical fitness and well-being in children with adolescent and juvenile idiopathic arthritis and the effect of an exercise programme: a randomized controlled trial. Pediatric Rheumatology, 11.7. doi:10.1186/1546-0096-11-7
Schneider, R., & Passo, M.H. (2002). Juvenile rheumatoid arthritis. Rheumatic Disease Clinics of North America, 28(3), 503-530. doi:10.1016/s0889-857x(02)00016-9
Van Oort, C., Tupper, S.M., Rosenberg, A.M., Farthing, J.P., & Baxter-Jones, A.D. (2013). Safety and feasibility of a home based six week resistance training program in juvenile idiopathic arthritis. Pediatric Rheumatology, 11:46. doi:10.1186/1546-0096-11-46
Weiss, J.E., & Ilowite, N.T. (2005). Juvenile idiopathic arthritis. Pediatrics Clinic of North America, 52(2):413-442. doi:10.1016/j.pcl.2005.01.007