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Juvenile Rheumatoid Arthritisby Ryan Cochran
Description and History Juvenile Rheumatoid Arthritis is one of the most common chronic diseases prevalent in children, which includes ages 0-16 (although cases in babies under 6 months are rare). The normal onset age is between 1 and 3 years, while the average rates of incidence and prevalence are reported to be between 10 to 20 in 100,000 and 56 to 113, respectively (Cakmak, 2005). First identified by George F. Still in 1897, today it affects more than 250,000 children in the United States alone, occurring in more girls than boys (seffera.net). The criteria for the disease are as follows:
Like adult rheumatoid arthritis, juvenile rheumatoid arthritis is an autoimmune disease, which means the body's immune system attacks its own healthy cells and tissues (chw.org). JRA is considered to be a multifactorial condition meaning that "many factors" are involved in causing the health problem. The factors are usually both "nature" and "nurture," where a combination of genes from both parents, in addition to unknown environmental factors, produces the trait or condition. This explains the fact that JRA is more prevalent in girls than boys as one gender is affected more frequently than the other in multifactorial traits. It may be that a virus or bacterium triggers the development of JRA in children with certain genetic profiles. These genetic profiles are detected in some children with JRA and are considered genetic markers for JRA. However, not all children with the markers develop JRA, and children without the markers can develop the condition (mayoclinic.com). There are many effects and complications associated with JRA, including skin rash, inflammation of the eyes, and/or swelling of the lymph nodes and spleen (Stopka, 2005). The above symptoms are more often associated with one of the three types of JRA, systemic JRA. Systemic refers to the disease affecting many parts of the body, as opposed to a few specific places. The attacks that come with the systemic type of the disease include spiking fevers once or twice a day, malaise, and skin rash; they also may be unpredictable, lasting for months, subsiding, and reappearing (Stopka, 2005). It is the least common type of JRA (20%) and an equal number of boys and girls have this type (seffera.net). It is also the most serious, as many organs can be affected including the heart, lungs, and liver. As mentioned above there are two other forms of the disease, polyarticular and pauciarticular. Fifty to sixty percent of the total number of children with JRA have the former, which is characterized by five or more joints being affected. Polyarticular arthritis is often symmetrical, affecting both sides of the body. This type is particularly prevalent in teenage girls (affecting them 3/1 over boys), although it often does appear as early as age eight. The pauciarticular type can be further broken down into early onset pauciarticular (mainly girls) and late onset pauciarticular (mainly boys), and affects less than five joints (Tanvir, 2004). Bigger joints, such as the knee, are usually associated with this subgroup of the disease. Children affected with this type of JRA are also at a higher risk of developing uveitis, inflammation of the eye that can lead to blindness; however, all children with JRA should be checked for uveitis (Tanvir, 2004). While a review of the literature reveals that there are differing estimates on the percentage of cases that cause severe, life-altering symptoms (17% to 50%), it is believed that with proper care 70% of the cases can be controlled to prevent permanent crippling (Palmer, 2005). Fifty percent of these children "grow out" and have no future complications later in life. Diagnosis and Treatment To successfully treat the symptoms of all kinds of diseases encompassed under the "umbrella" term Juvenile Rheumatoid Arthritis a "holistic," multidisciplinary approach is necessary (Cakmak, 2005). The involvement of many professionals will also improve the prognosis of the child. These professionals may include pediatric doctors, physiotherapists, occupational therapists, psychologists, and dieticians. Another essential element in the management of JRA is physical therapy - without it joint pain and inflammation would trigger a vicious cycle that often leads to permanent crippling. In this section I will outline the medical treatments and advances trying to combat JRA and include ways in which a rehabilitative program and adapted physical education can help children with JRA. In any case, the phrase that basically sums up the attitude everyone involved in helping children with JRA should have is "An ounce of prevention is worth a pound of cure," as treating the symptoms after the fact is time-consuming, expensive, and not nearly as effective (Cakmak, 2005). In diagnosing JRA, every effort should be made in making an accurate diagnosis, taking into account the quote above, and also because of the frequency of confusing the symptoms with Acute Lymphocytic Leukemia (ALL) (Jones, 2006). Currently, there is no diagnostic test that definitively tells that a child has JRA; rather reported symptoms, laboratory tests, and imaging tests are used to make a diagnosis. Joint inflammation usually must be present for at least six weeks, continuously and then the child is given such laboratory tests as: 1) antinuclear antibody (ANA), 2) arthrocentesis (joint aspiration) 3) complement, 4) complete blood count (CBC), 5) creatinine, 6) erythrocyte sedimentation rate (ESR or sed rate) 7) hematocrit, 8) rheumatoid factor (RF), 9) white blood cell count (WBC). To show the extent of the damage to the bone and further diagnose JRA, imaging tests are done. They include: 1) x-ray, 2) computed tomography scan (Also called a CT or CAT scan.), 3) magnetic resonance imaging (MRI), 4) bone scan (Stanfordhospital.com). After the diagnosis, nonsteroidal anti-inflammatory drugs, disease modifying antirheumatic drugs, biological response modifiers, glucocorticoids, and/or analgesics can be used to help with the symptoms. The nonsteroidal anti-inflammatory drugs are ibuprofen and naproxen - commonly known as Advil and Aleve. I will not go into detail about the other groups, however, in studies done the disease modifying antirheumatic drugs methotrexate and leflunomide were shown to be effective in controlling the symptoms of JRA (mayoclinic.com). As was stated before, along with diagnosis and medication, rehabilitative efforts are of the utmost importance in maintaining proper joint functioning in patients with JRA. Patients with JRA are inclined to keep their joints in a position where joint volume is greatest and the swollen synomvium is at its most comfortable position. This “most comfortable” position is usually the flexion posture and therefore extensor muscles are usually weak and underdeveloped. This phenomenon can also result in growth retardation. Having said that the objectives of physical therapy and rehabilitative programs are as follows:
Standard physical therapy modalities, such as heat, cold, massage, electrical stimulation, and ultrasound are effective in treating JRA; however exercise and adapted activity also play a role in accomplishing the goals bulleted above. Aquatic exercises are particularly useful because they are low impact and allow children to move and exercise without undue stress to their joints. Sessions of these aquatic exercises should last around a half hour and be of moderate intensity. They are much preferred over sports that include extensive use of the ankles and knees, such as basketball and football. All exercises are best performed later in the day, as joints are often stiff and painful after waking. Along with exercise, stretching is vital for improving ROM. Stretching twice a day using the "contract-relax" technique is recommended. In this technique the patient's joint is placed in passive extension, and while the patient is actively flexing, the therapist forces the joint to extension. After holding this position for a couple of seconds the therapist contracts the joint up to extension while the patient relaxes. Strength training is also an important aspect of combating the effects of JRA. The training should be done during the chronic period, three days per week, and can be done with Theraband, free weights, and weight machines under supervision (Cakmak, 2005). Two Recent Articles When doing research about JRA I found two articles that I found very interesting. The first concerns the drug thalidomide. This drug became infamous for its devastating teratogenic effects in the late 1950's and early 1960's, when given to pregnant women as a sedative and antinauseant. The drug became approved by the FDA in 1998 to treat cancer and complications due to HIV/AIDS, and a study has now seem to show that it is helpful in treating severe systemic onset JRA. The study which included thirteen children from the United States and Brazil concluded that thalidomide dramatically affected for the positive these children, after all other treatment had failed. While the study was small and uncontrolled, the implications are worth noting and perhaps it is best summed up by the quote at the end of the article: "But what perhaps most interesting about this report is its suggestion that a drug that devastated the lives of thousand of children may now, with careful use, offer other children real therapeutic benefit" (Pediatic Alert, 2004, pg.144). The second article has to do with total knee replacements in kids
with severe JRA. Patients who had "severe osseous destruction"
and using wheelchairs and walkers were given total knee arthroplasty
is both knees. The results were positive, as six of the eight patients
were able to walk about freely without the use of aides and were essentially
pain-free. The other two also showed marked improvement. The results
were obtained by clinical observations prior to surgery and then a mean
of 15.5 years after the surgery. The study concluded by saying that
for a population with severe pain and disability at a young age this
procedure is a "reasonable option" (Palmer, 2005). References Jones, F. (2006). …And of childhood leukemia vs. juvenile rheumatoid arthritis. Pediatric Alert, 31(10), 57. Palmer, D.H., Mulhall, K.J. (2005). Total knee arthroplasty in juvenile rheumatoid arthritis. Journal of Bone and Joint Surgery, 87(7), 510-514. Pediatric Alert. (2004). Thalidomide for severe
juvenile rheumatoid arthritis? 29(24), 143-144. Tanvir, J. (2004). Handling a possible case of juvenile rheumatoid arthritis. Pulse, 64(47) 46. www.chw.org PELINKS4U is a non-profit program
of Central Washington University dedicated to promoting active and healthy
lifestyles
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