Juvenile rheumatoid arthritis: physical therapy and rehabilitation.

Abstract: Juvenile arthritis is one of the most prevalent chronic diseases in the childhood period (ages 0 to 16 years). This disease was first defined in the first half of the 16th century. In the course of time, its differential diagnosis and characteristics have been determined, and it has been classified. Incidence and prevalence values are 10 to 20 in 100,000 and 56 to 113 in 100,000, respectively. Various factors are suggested for its underlying cause. Its denomination is also in dispute. Treatment of juvenile arthritis includes education, medical treatment, physical therapy, and occupational therapy. This article summarizes the objectives and methods of physical therapy and rehabilitation that are important parts of treatment.

Key Words: juvenile rheumatoid arthritis, pediatrics, physical therapy and rehabilitation, splint, orthosis.

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Juvenile arthritis is one of the most common chronic diseases in the childhood period (ages 0-16). It may lead to functional deficiency in the musculoskeletal system and blindness. Juvenile Rheumatoid Arthritis (JRA) is arbitrarily defined as arthritis beginning before the age of 16 years. In most instances, the onset age is between 1 and 3 years, and it is rarely diagnosed before 6 months of age. Despite the fact that incidence and prevalence vary among countries, the average rates of incidence and prevalence are reported to be 10 to 20 in 100,000 and 56 to 113 in 100,000, respectively. (1,2) The etiopathogenesis of JRA is summarized in the Table.

Diagnostic Criteria of Juvenile Rheumatoid Arthritis

Criteria include (1) onset before age 16 years; (2) arthritis involving one or more joints or presence of at least two of the following findings: (a) limitation in range of motion (ROM), (b) tenderness or pain with joint movement, (c) increased fever; (3) disease persisting 6 weeks or longer; (4) clinical features of (a) polyarthritis: inflammation in five or more joints, (b) oligoarthritis: inflammation in fewer than five joints, (c) systemic: characteristic arthritis that develops with fever; and (5) exclusion of other juvenile arthritis.

A multidisciplinary approach is necessary for the successful treatment of JRA, with the active involvement of pediatric rheumatologists, physiatrists, physiotherapists, occupational therapists, psychologists, and dieticians. (3) This article summarizes the methods and objectives of physical therapy, an essential element in the management of JRA.

Joint pain and inflammation trigger a vicious cycle that often ends in joint damage and chronic deformities. A comprehensive rehabilitation program must start early to restore loss of function and prevent permanent handicap. (4) An appropriate rehabilitation program is indeed as important as initiating appropriate and timely medical treatment. The essential objective is to preserve the functional capacity and autonomy of the child. (5) The phrase, "An ounce of prevention is worth a pound of cure" is especially true for children who have JRA. In fact, preventing contractures, muscle weakness, osteoporosis, and the disability resulting from these conditions is less time-consuming, painful, and expensive than treating the patient after the development of these problems. (6,7)

The approach to rehabilitation management of childhood rheumatic disease differs in many ways from that of adult disease. Among the special considerations are the effects of musculoskeletal inflammation in a growing and developing individual and the tendency of children to tighten their joints into positions of comfort, with fewer problems resulting from ligamentous laxity and instability. (8)

Rationale for Rehabilitation

Patients with JRA tend to keep their joints in "the most comfortable positions." This is the position in which the joint volume is maximum, and swollen synovium is at its most comfortable position. Because this position is frequently the flexion posture for the joints, weakness of extensor muscles and contracture of flexor muscles rapidly develops. Complete extension of the joint cannot be performed. Flexor contracture leads to loss of function (eg, difficulty in grasping and walking). Over the course of time, compensatory contractures develop, resulting in more marked loss of function. (6,9) Limping and walking with knee flexion are walking disorders that must be rapidly recognized and treated. (10,11)

Because of pain, tiredness, and stiffness, children with JRA are less active than their peers. Reduced mobility may lead to systemic muscle weakness, decreased flexibility, cardiovascular reserves, and exercise capacity. (6,12)

Growth retardation is frequently seen in children with JRA, and its prevention is one of the purposes of rehabilitation. Children may repeatedly fall due to a decrease in fine motor skills and joint development as well as impairment of balance control. Adaptation to changes in position is also often inefficient in children with JRA. Therefore, neurodevelopmental and neurosensory stimulation techniques must be part of the treatment. (8)

Osteoporosis is also prevalent in children with JRA as the result of steroid use, nutritional disorders, and decrease in the quantity of load carried by the joints. (6) Cetin et al (13) and Celiker et al (14) documented that lumbar vertebral bone density was significantly lower in children with JRA as compared with a control group and that this was especially evident in those using steroids.

Objectives of Rehabilitation

Objectives of physical therapy and rehabilitation in JRA include the following (15): (a) controlling pain, (b) preventing limitation and restoring ROM in affected joints, (c) maintaining and improving muscle strength, (d) increasing and maintaining endurance for activities of daily living, (e) minimizing the effects of inflammation, and (f) ensuring normal growth and development.

To achieve these objectives, raising awareness about the disease in patients and families is one of the most essential components of treatment. A study by Andre et al (16) documented that through education, physical exercises, coping strategies, and problem-solving skills, families became more involved in the treatment, better understood their children's condition, and were able to find more efficient solutions, with a subsequent improvement in the quality of life of their children.

In JRA, home-based programs are important to support the treatment. There are three basic rules for home programs: first, the most appropriate treatment is the simplest, least painful, and least expensive; second, stretching and strengthening exercises should be customized according to the daily activities that the patient is unable to perform; and third, all treatments must be followed closely by a health care professional.

Physical Therapy Modalities and Exercise

Standard physical therapy modalities are heat-cold treatment, massage, electrical stimulation, and ultrasound. These modalities are aimed to relieve pain and stiffness, prepare for exercise programs, reduce contractures, and provide training for specific muscle groups. (6)

Heat treatment

Heat treatment is used especially for decreasing the rigidity of joints, increasing the flexibility of the fibrous tissue in joint capsules and tendons, and decreasing pain and muscle spasms. The effect of heat depends on several factors, including optimum temperature (ideally 40 to 45.5[degrees]C); duration of fever (3 to 30 minutes), rate of temperature change, and area treated. Taking a hot shower may relieve morning stiffness; taking a bath before going to bed may control night pains. Applying heat treatment before exercises, especially stretching exercises, will increase the efficiency of the treatment. Ultrasound as a source of deep heat is especially useful in the hip joint. Since, however, it may have an effect on growing plates and cemented prostheses, it should be applied with care in these regions. (6,8,15,17)

Cold treatment

Cold treatment is used for analgesic and vasoconstriction purposes in inflamed joints during the acute period. Potential adverse effects are cold urticaria, cryoglobulinemia, Raynaud phenomenon, and frostbite from overuse. It is usually applied for 20 minutes. A hyperemic reaction develops thereafter. Nevertheless, as many children dislike cold, it cannot be used frequently. (6, 15)

Massage

Massage can relieve the pain and prevent adhesions in the subcutaneous tissues. It is applied with heat treatment and generally before stretching exercises. (15) Field et al (18) recommend massage treatment as a relaxation therapy. Researchers report that urinary norepinephrine levels decreased after massage and that more orderly sleeping was observed thereafter. A 15-minute daily massage treatment by a family member for 30 days reduced anxiety levels in both the person who applied the massage and in the child. Furthermore, this was associated with a reduction of cortisol levels in the child's saliva and a decrease in the pain. (19)

Electrical stimulation

Short-term electrical stimulation is useful in children with excessive muscle atrophy and in those who cannot exercise. (15)

Therapeutic exercises

Therapeutic exercises form the basis of the treatment for children with JRA. This program should include all kinds of exercises: aquatic exercises, positioning, passive ROM exercises, and isometric exercises. Aquatic exercises frequently decrease the pain and prevent muscle spasms. Therefore, swimming and Tai Chi are recommended rather than those sporting activities that include extensive use of the ankles such as basketball, football, and gymnastics. Exercises performed to improve aerobic capacity should be moderate in intensity and should not last more than 30 minutes per day. (6,8) Bacon et al (20) demonstrated that in children who had exercised in water for 6 weeks, hip rotation angles were significantly improved and other ranges of motion were also enhanced. Klepper (12) studied the outcomes of intensive exercises and showed that an 8-week intensive aerobic exercise program practiced 2 days in the hospital and 1 day at home for 60 minutes per day improved the physical well-being without increasing the activity of disease. After the active phase subsides, stretching exercises are the main exercises performed to improve ROM. The stretching exercises must be continued for a 10-second period, 5 to 10 times in each session twice a day (10-second hold, 20-second relax). For this purpose, the best suitable exercise is one with a "contract-relax" technique. The patient's joint is placed in passive extension, and while the patient is asked to actively flex, the therapist forces the joint to extension, and after holding this position for a couple of seconds, the therapist contracts the joint up to extension while the patient relaxes. (6) Exercises for increasing the strength of muscles are important in the chronic period. Accordingly, progressive exercises could be taught to the patient (with dumbbells, weights, and Theraband). A recommended program is 3 days per week of customized resistance exercise. (6,15,21)

Splint/orthosis

Resting splints are used to rest the joint and are placed in an appropriate position in the acute period. Because immobilization complications may rapidly develop and lead to dependence, full bed rest is not recommended. (17) Since ankylosis develops faster compared with adults, positioning is very important in preserving the maximum function. The joint should be held in the extension position to the extent that the inflammation allows. Wrist splints, ring splints for fingers, and splints that prevent flexion contracture in knees are frequently used to that purpose. (6) Customized splints prepared by an experienced therapist are more effective than ready-made splints. (22)

In dynamic splint applications, the objective is to exert adequate force that the tissue can tolerate, provide sufficient joint volume, and ensure that the splint is used for a period that is sufficient to attain the requested target. Therefore, it is recommended to use splints that exert lesser forces for 10 to 12 hours. (6)

Functional splints are used to protect the joint in the course of activity. Stabilizer splints used in wrist deformities are the most classic examples of such splints. The most frequently used splint for the lower extremities is the ankle-foot orthosis. It has been proven that ankle-foot orthosis improves the load distribution in the hind foot, mid foot, and forefoot. (6,23)

In cases in which permanent contractures develop in joints, a "stabilizer splint" must be used as an orthosis. Orthoses are used in patients for stabilizing the flexion contractures by stretching. Gradual casting is also recommended for this purpose. The recommended technique is removing the cast at 24- to 48-hour intervals and applying aggressive stretching for 20 minutes, before returning the cast to a position that forces maximal extension. The cast should stay on for at least 23 hours. (6)

Protection Methods

It is difficult to correct permanent deformities once they have occurred in a growing musculoskeletal system. Therefore, the primary target should be the prevention of these deformities. Recommendations are summarized below. (6)

Spine

Children should avoid keeping the neck in flexion positions for long periods, such as sitting and leaning over schoolbooks. Chair and table heights should be adjusted. The pillows should be thin. Active ROM exercises should emphasize extension and rotation. Children should use a cervical collar when studying, and an inclined table is recommended to avoid holding their necks in flexion continuously. The cervical collar prevents spasm by keeping cervical muscles warm and decreases the risk of flexion contracture that may arise in the future. Children should also sleep in the prone position for half an hour a day to prevent forward flexion position. Supported chairs and book holders should be used for the appropriate sitting position. (10,11,24) It should be taken into consideration that the bursa in the atlantoaxial joint, after being inflamed, may lead to atlantoaxial subluxation. Patients should be encouraged to use soft collars for protective purposes and to use hard collars in the event of subluxation. The Canadian soft collar is easily used and tolerated. (25)

The thoracic region of the spine tends to develop kyphosis and is associated with forward shoulder position and frequently exaggerated lumbar lordosis secondary to hip flexion contractures. Maintaining spinal extension, such as sleeping in extension positions, doing back extension exercises, and practicing good posture, allows for better function even if the spine fuses. Deep breathing and regular respiration exercises are also recommended. (6,8)

Jaw

The temporomandibular joint is involved in polyarticular JRA and leads to pain when opening the mouth and difficulty in chewing. Moreover, in the case of growth anomaly (micrognathia), joints undergo comparatively more stress due to closing problems. In regard to protective measures, it is recommended that the patient hold the mouth in a slightly open position and bite big morsels such as a hamburger to increase muscle power. (6)

Shoulder

Preserving the ROM in the shoulder joint, which has wide range of motion in all directions, can be ensured by such activities as reaching for high objects, combing hair, playing volleyball with a soft ball, and painting. It is important that shoulders be kept in a neutral position. (6) Thus children should not be allowed to sit in soft armchairs, which places the child in a forward flexion position. (8)

Elbow

Limitation of extension up to 10[degrees] is normal in women. Extension and supination are usually limited first. Full flexion of this joint is important for maintenance of activities of daily living. Therefore, active ROM exercises are recommended, holding arms at full extension when sitting and standing, using night splints (especially when flexion contracture begins to develop), and extensor muscle strengthening exercises. Complications of ulnar entrapment neuropathy caused by synovitis and rheumatoid nodules should be carefully monitored. Patients should be instructed to avoid directly touching their elbows to hard surfaces. (6,8)

Wrist

Loss of extension and ulnar deviation at the radiocarpal joints are often the first limitations noted. A night resting splint is recommended in addition to the active extension exercises. Keyboarding should be encouraged because long-term handwriting (holding pens) leads to fatigue. Recommendations include playing volleyball with a soft ball and painting. Median nerve entrapment neuropathy may coexist with wrist stiffness. (6)

Finger

Metacarpophalangeal joint involvement is frequent in the polyarticular type. Terminal flexion and extension are limited. Active and passive ROM exercises, preserving muscle power with squeezing a sponge and not allowing excess load onto the loose joints, are recommended. (6)

Hip

The hips are major weight-bearing joints. They are involved especially in rheumatoid factor (positive) and the systemic form of JRA. In hip involvement, primarily extension and internal rotation are limited. Weakening of the extensor muscles occurs by allowing excessive load onto the femur head from compensatory knee flexion contracture, tension of the hamstring muscles, and excessive energy consumption while walking. To prevent these pathologies, it is recommended that patients sleep in the prone position 2 times per day for 30-minute durations, sleep in the prone position at night, and stretch and strengthen the extensor muscles. (6,8) In the case that hip flexor contractures develop, traction at nighttime may be applied. Traction cannot be performed until the hip flexion contracture is reduced to 10 to 15[degrees]. (8) If traction is applied in the presence of significant flexion contracture, lumbar lordosis is exaggerated, and the child will have back pain.

Knee

Extension and flexion limitations are often observed. To sit down and stand up from a chair, full extension and 110[degrees] flexion are required. If these angles are preserved, the strength of the quadriceps is also maintained and the child will be functional. Encouraging maintenance of medial quadriceps strength by straight leg raises with the foot rotated out 45[degrees], maintaining patellar mobility, and correcting the differences in length of legs is required. Exercises should be repeated 25 to 30 times. Night splints should be applied in case flexion contractures begin to develop. Recommended activities include swimming, ascending and descending stairs, and kicking a ball. (6,8)

Ankle

Ankles should be in a neutral position for heel strike and an orderly walking pattern. Stretching the ankles in different directions after warming and gradual casting when necessary are recommended to regain ROM. Wearing appropriate shoes and slightly raising the heels relieve pain and provide a comfortable walking environment. (6)

Foot

Patients with JRA often have small, wide feet with high arches, due to premature closure of tarsal and metatarsal joints. This may limit pronation and supination of the mid-foot. Sometimes, the longitudinal arch may collapse. Plantar fascia can tighten and metatarsal adduction can be observed. To retain flexibility, active and passive ROM exercises, picking up marbles from the carpet to strengthen intrinsic foot muscles, and using an arch support in the shoes are recommended. Shoes with thick soles and ankle supports are recommended for these patients. (6)

Assistive devices

Adaptive devices are appropriate for those children whose daily activities are still limited despite all these measures. Comb handles are extended, thicker spoons are used, and a shoehorn is used to put on shoes. Clothes with easy openings and/or Velcro can facilitate putting on and removing clothing. To encourage fluid intake, angled glasses can be used in children with cervical stiffness and in children who cannot extend their heads. Only a few children need wheelchairs. Lightweight wheel chairs are preferred. (10,11,26)

Conclusion

JRA is a disease that can be managed by close follow-up and timely application of appropriate treatment modalities, enabling children to reach adult ages without development of disabilities. Early diagnosis, appropriate treatment, education, and teamwork of the family, patient, and physicians are factors that increase the chances of success.

Table. Etiopathogenesis in juvenile rheumatoid arthritis (a)

Group

Genetic factors           HLA
                          TCR
                          Immunoglobulins
                          Peptide carriers
Triggering factors        Different infectious agents directed at the
                            same target
                          An infectious agent not yet defined
                          Other environmental factors
Abnormal immune response  Autoimmunity?
                          Hyperactivity?
                          Dysfunctional inflammatory cells in
                            inflammation region
                          Accumulation of inflammatory mediators
Chronic synovitis         Continuation of antigenic stimulus?
                          Self-oriented?

(a) HLA, human leukocyte antigen; TCR, T-cell receptor.

Acknowledgment

The authors express great appreciation to Family Bilecik for their continous support and their kind gesture of publishing and delivering free of charge patient education materials in Turkey.

August 10, 2004

Please see Christopher R. Morris' editorial on page 136 of this issue.

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RELATED ARTICLE: Key Points

* Juvenile arthritis is one of the most common chronic diseases in the childhood period.

* Juvenile arthritis may lead to functional deficiency in the musculoskeletal system and blindness.

* Juvenile rheumatoid arthritis is arbitrarily defined as arthritis beginning before the age of 16 years; nevertheless, onset before 6 months of age has been considered rare.

* Treatment of juvenile arthritis includes education, medical treatment, and physical and occupational therapy; a multidisciplinary approach is necessary for successful treatment.

* This report summarizes the methods and objectives of physical therapy, which is one of the essential parts of treatment.

Aysegul Cakmak, MD, and Nalan Bolukbas, MD

From the Department of Physical Medicine and Rehabilitation, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.

Reprint requests to Dr. Aysegul Cakmak, Istanbul Faculty of Medicine, Istanbul University, 34390 Sehremini, Istanbul, Turkey.