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| REVIEW ARTICLE |
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| Ahead of print publication |
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Role of rehabilitation in comprehensive management of juvenile idiopathic arthritis: When and how?
Pranav Raman Chickermane1, Nittu Devassy Panjikaran2, Suma Balan1
1 Department of Rheumatology and Clinical Immunology, Amrita Institute of Medical Sciences, Kochi, Kerala, India
2 Department of Physical Medicine and Rehabilitation, Amrita Institute of Medical Sciences, Kochi, Kerala, India
| Date of Submission | 18-Mar-2022 |
| Date of Acceptance | 04-Sep-2022 |
| Date of Web Publication | 25-Oct-2022 |
Correspondence Address:
Suma Balan,
Department of Rheumatology and Clinical Immunology, Amrita Institute of Medical Sciences, Ponekkara, Kochi - 682 041, Kerala
India
 Source of Support: None, Conflict of Interest: None DOI: 10.4103/injr.injr_55_22
Juvenile idiopathic arthritis (JIA), the most common chronic rheumatological disorder in children, can result in significant disability and poorer health-related quality of life. Rehabilitation aimed at pain management, optimizing musculoskeletal function, improving endurance and achieving independence in activities of daily living, and participation in age-appropriate activities, is a vital component in the comprehensive management of children with this condition. Rehabilitation strategies depend on the phase of the disease and focus on pain alleviation in the active phase and improving mobility and function in the inactive phase. Rehabilitation in JIA is multidisciplinary and includes exercise therapy, physical modalities, orthotic and assistive devices, and gait training. Exercise therapy has demonstrated improvement in muscle strength, bone mineral density, exercise capacity, and quality of life, without negative consequences of pain or exacerbation of arthritis. Common exercise interventions for children with JIA include mobilization, strengthening, aerobic exercises, Pilates-based exercises, aquatic therapy, and recreation. Physical modalities such as thermotherapy, cryotherapy, electrotherapy, ultrasound, and low-power laser therapy are commonly employed. Orthotic devices play an important role in joint protection, prevention and reduction of joint deformities, and assistance with function and gait. Assistive devices are prescribed to improve functional ability and independence in activities of daily living in children with disabilities. There have been recent advances in the field of rehabilitation with the advent of robotics, virtual reality, and telerehabilitation.
Keywords: Exercise therapy, juvenile idiopathic arthritis, orthoses, physical modalities, rehabilitation
| Introduction | |  |
Juvenile idiopathic arthritis
Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatological disorder in the pediatric age group. JIA encompasses a clinically heterogeneous group of arthritides of unknown cause, with onset before the age of 16 years and persisting for >6 weeks.[1] The International League of Associations for Rheumatology has classified JIA into seven subtypes based on the clinical features present during the first 6 months of illness, namely, systemic arthritis, oligoarthritis (persistent or extended), polyarthritis rheumatoid factor (RF)-positive, polyarthritis RF-negative, enthesitis-related arthritis (ERA), psoriatic arthritis, and undifferentiated arthritis.[2] Recently, the Pediatric Rheumatology International Trials Organization has proposed revised classification criteria for JIA, comprising four categories: systemic JIA, RF-positive JIA, enthesitis/spondylitis-related JIA, and early-onset antinuclear antibody-positive JIA.[3]
The goals of treatment of JIA include control of signs and symptoms of the disease, prevention of structural damage, optimization of function and fitness, growth and development, improvement in health-related quality of life (HRQOL), and social participation. There has been increasing evidence supporting the concept of “the window of therapeutic opportunity” in JIA, i.e., early aggressive treatment with traditional and/or biological disease-modifying antirheumatic drugs (DMARDs) may modify the disease course with favorable long-term outcomes.[4],[5],[6],[7] There have also been therapeutic advances in the management of this condition with the availability of an expanding armamentarium of drugs, especially biological DMARDs over the past few years. This has led to advocacy for the treat-to-target strategy for the management of JIA with clinical remission as the primary target.[8] The treatment of JIA involves a multidisciplinary approach which includes a pediatric rheumatologist, ophthalmologist, orthopedic surgeon, dermatologist, physiatrist (physical medicine and rehabilitation doctor), physiotherapist, occupational therapist, psychologist, and social worker.[8]
JIA can result in significant disabilities such as decreased range of joint mobility, joint deformities, growth disturbances, and impaired physical function.[9] The functional abilities of JIA patients have been reported to be lower than those of normal children.[10] Children with JIA have a poorer HRQOL compared to their healthy peers.[11] This highlights the importance of rehabilitation in the comprehensive management of JIA.
Rehabilitation in juvenile idiopathic arthritis
Physical medicine or rehabilitation is not only physiotherapy, it also involves the utilization of physical modalities and stretching and strengthening techniques, toward energy conservation, pain relief, and joint protection. The early commencement of appropriate medical treatment can help to reduce deformities, but the presence of deformity will require the use of assistive devices and vocational planning. Thus, a multidisciplinary approach is recommended toward targeting musculoskeletal problems ranging from inflamed joints, weakness, and joint deformities. The rehabilitation process should be adapted to the child's current locomotor system and function.
In the context of JIA, rehabilitation aims to reduce pain, improve range of motion (ROM) in affected joints, prevent the development of deformities and correct existing deformities, improve muscle strength, promote function, normal growth and development, improve endurance, achieve independence in activities of daily living, and participation in age-appropriate activities.[12]
Each phase of the disease requires different strategies [Table 1]. While in the active phase of the disease pain alleviation is the main focus, the inactive phase requires strategies for improving motility and function.[13] Patient education and counseling play a vital role and help in ensuring patient adherence and active participation in the rehabilitation plan. | Table 1: Rehabilitation strategies according to the phase of juvenile idiopathic arthritis
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Initial assessment
The initial evaluation on referral to rehabilitation services includes a clinical examination to look for any joint effusion, synovial thickening, tenderness, crepitus, deformity, instability, limitation in active and passive range of movements, muscle atrophy, limb-length discrepancy, posture and gait, assessment of pain, muscle strength, physical function, and HRQOL.
The pain intensity Visual Analog Scale (VAS), Wong–Baker Faces Pain Rating Scale, the Oucher scale, and Varni/Thompson Pediatric Pain Questionnaire (PPQ) are useful in the assessment of pain in children with JIA. The VAS consists of a 100-mm horizontal line with the terms “no pain” at the left and “worst pain possible” at the right. The child (or parent, in the case of children younger than 8 years of age) makes a vertical mark on the line to indicate his or her level of pain. The Wong–Baker Faces Pain Rating Scale comprises six line-drawn faces with descriptive terms from “no hurt” to “hurts worse.”[14] The Oucher scale is a self-reported pain intensity scale, consisting of two vertical pain scales: a six-color photographic faces scale for younger children and a numerical rating scale of 0 (“no hurt”) to 10 (“the biggest hurt you could ever have”) available for children older than 8 years.[15] The Varni/Thompson PPQ is a child or parent-reported questionnaire assessing the intensity of pain, sensory, affective, and evaluative qualities of pain and the location of pain in children.[16],[17] The PPQ includes a VAS, color-coded body diagram, and open-ended questions and is broken down into three forms for young children (5–7 years), children (8–12 years), and adolescents (13–18 years).
Goniometer, a tool used to measure the range of movement, is a protractor with one axis that joins two arms, one of which is stationary and the other movable. The angle of movement for a joint is measured by aligning goniometer arms with the limb to be tested.[12] Jamar hydraulic hand dynamometer and Martin-type squeeze dynamometer-vigorimeter are devices used to measure isometric grip strength and dynamic movement grip strength, respectively.[18] In manual muscle testing, isometric force is measured by asking the patient to push maximally against the examiner's hands with the muscle group being tested.[19]
Tools for the assessment of physical function in children include Juvenile Arthritis Functional Assessment Report (JAFAR), Juvenile Arthritis Functional Assessment Scale (JAFAS), Childhood Health Assessment Questionnaire (C-HAQ), and Juvenile Arthritis Status Index (JASI). The JAFAR measures physical function by assessing the child's ability to independently perform 23 activities of daily function in the preceding week.[20] The JAFAS, which is administered by a physiotherapist or occupational therapist using simple equipment in a clinical setting, measures performance in completing 10 basic daily tasks.[21] The C-HAQ measures functional status for 30 activities across eight domains, namely, dressing and grooming, arising, eating, walking, hygiene, reach, grip, and activities.[22] The JASI consists of two parts. The first part measures activities of daily living and functional mobility and includes 100 functional items divided into five groups (self-care, domestic, mobility, school, and extracurricular).[23] The second part involves the identification of up to five tasks that are most problematic for a given child and these are reassessed at follow-up.
The Juvenile Arthritis Quality of Life Questionnaire and the Pediatric Quality of Life Inventory (PedsQL) are the commonly employed tools to assess HRQOL in children with JIA.[24],[25] The commonly used tools for the assessment of pain, range of movements, muscle strength, physical function and health related quality of life are enlisted in [Table 2]. | Table 2: Tools for the assessment of pain, range of movements, muscle strength, physical function, and health-related quality of life
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Exercise therapy
Exercise therapy is a vital component of the standard of care for children with JIA.[1] Common exercise interventions for children with JIA include mobilization, strengthening, aerobic exercises, Pilates-based exercises, aquatic therapy, and recreation.[26]
In children with JIA, exercise therapy has demonstrated improvement in muscle strength, bone mineral density, exercise capacity, and quality of life, without negative consequences of pain or exacerbation of arthritis.[27],[28],[29],[30] In a systematic review and meta-analysis evaluating exercise therapy in JIA, Kuntze et al. included nine randomized control trials encompassing various exercise interventions such as aquatic therapy, strengthening, proprioception, aerobic exercises, and Pilates. Despite substantial heterogeneity in exercise interventions, they observed that exercise therapy was associated with improved outcomes.[31] Exercise programs focusing on strength, flexibility, and balance using supervised or partially supervised training sessions were commonly associated with improvements in pediatric activity, body function, and structure and quality of life. However, they found that high-impact exercise led to pain, hence not recommended for these children.[31]
In actively inflamed joints, gentle active or active-assisted ROM exercises should be performed. Passive stretching should be applied with extreme caution considering the risk of rupture of the joint capsule in case of large effusions.[26] As the inflammation subsides, active-assisted exercises should be initiated.
The strengthening program always starts with isometric (static) contraction (no change in the muscle length or joint position) to reduce joint stress on exercising inflamed or damaged joints. All exercises must be practiced to submaximal effort to prevent muscle atrophy. In the next stage, it is advanced to isotonic (dynamic) contraction in which the load is fixed but the joint position and muscle length change. Isotonic exercises are to be avoided in patients with acutely inflamed joints. When a muscle shortens on contraction it is known as concentric contraction and when it lengthens in contraction it is known as eccentric contraction. Isokinetic contraction usually requires maximal force of contraction which is not recommended in patients with arthritis unless the joint is relatively normal.[26]
After initial control of pain and inflammation, aerobic activities such as walking, cycling, swimming, water aerobics, and low-impact aerobic dance which increase aerobic capacity and functional ability are recommended.[26] The exercise intensity, duration, and frequency should be adjusted as tolerated by the patient. Tai Chi exercise is one exercise that can be done to improve flexibility, strength, balance, and proprioceptive training. Improvement in muscle strength and functional ability due to aerobic activity usually becomes apparent only after 1.5–3 months. All strenuous activities need to be avoided, in case, the patient has an acute flare-up or inflammation of any joint. Any joint pain persisting for >2 h after the exercise is an indication for reducing the intensity and duration of the program. Supervised aerobic exercises are better than home programs.
Pilates-based exercise (named after Joseph Pilates) is a popular method for improving torso or core strength. It also confers benefits including improved spinal and joint mobility, proprioception, and balance and coordination training. Mendonça et al. reported that Pilates-based exercises, comprising floor exercises and exercises with the reformer, stability chair, and Cadillac and Ladder Barrel, had a greater positive physical and psychosocial impact on HRQOL in children and adolescents with JIA in comparison to the conventional exercise program consisting of warm-up, workout, and cooling down.[32]
Aqua therapy is widely recommended as the buoyancy of the water offloads the joints and reduces the effect of gravity, which can be utilized to mobilize joints. Joint mobilization should be done in the early stages before a deformity sets in, as it becomes very difficult to achieve full ROM once a contracture is formed. Pool temperatures between 83 F° (28.3°C) and 88 F° (31.1°C) are usually comfortable for most aquatic exercises. Takken et al. conducted two studies evaluating the efficacy of aquatic exercise programs in children with JIA.[33],[34] In a prospective pilot study enrolling 25 children with JIA, a 15-week aquatic exercise program was associated with improvement in HRQOL. However, there were no significant positive effects on functional ability, joint status, or physical fitness.[33] The second study, a randomized controlled trial enrolled 54 children. A 20-week aquatic exercise program resulted in 55% decrease in the number of swollen and tender joints; however, the results only tended toward statistical significance. The study did not show significant positive effects on functional ability, quality of life, and physical fitness.[34] Fragala-Pinkham et al. observed significant improvement in functional mobility, walking endurance, muscle strength, and range of the joints and pain relief in children following aquatic physical therapy.[35]
Recreational exercises done in groups have a better compliance as it is enjoyed by children and involves strengthening, mobilization, and aerobic activities in combination. It gives a sense of psychosocial well-being and social contact and elevates the child's mood.
The Ottawa panel for evidence-based clinical practice guidelines for structured physical activity (2017) recommended Pilates, cardio karate, and home and aquatic exercises to improve quality of life, pain, functional ability, ROM, and decrease the number of active joints affected.[36]
The proprioceptive neuromuscular facilitation technique is a hold-relax technique or a slow reversal-hold-relax technique, usually employed to improve exercise efficiency.
Fatigue, which is commonly observed in children with JIA, can be improved with aerobic activities as was shown in a study by Houghton et al., in which a reduction in fatigue was observed in patients who adhered to the program for 6 months.[37]
In patients with sacroiliitis, the posture is usually loss of lumbar lordosis, increased thoracic kyphosis, compensatory extension of the cervical spine, and hip and knee flexion. The center of gravity shifts downward and forward. A 5–10-min warm-up before the commencement of exercise is recommended. The exercise program for these patients includes stretching and strengthening exercises with posture correction. Deep breathing techniques and spirometry exercises help to improve respiratory function in patients with progressive axial disease. Strategies to improve compliance to exercise therapy are outlined in [Table 3].
Physical modalities
Physical modalities such as thermotherapy (heat), cryotherapy (cold), electrotherapy, and ultrasound and low-power laser therapy are commonly employed in rehabilitation of children with JIA.
Thermotherapy
Thermotherapy can be divided into superficial and deep heat and is used in subacute to chronic inflammation. Superficial heating agents increase the temperature within the skin and subcutaneous fat. Due to poor penetration (<1 cm), superficial heat generally only affects cutaneous blood flow and cutaneous nerve receptors. In addition to increasing local blood flow, the higher cutaneous temperature also has an analgesic effect. Heat causes skeletal muscle relaxation by decreasing the stimulus threshold of muscle spindles and decreasing the gamma efferent firing rate.[38] The commonly used superficial heat modalities include hot packs, electrical heating pads, paraffin baths, infrared, and hydrotherapy. A paraffin bath (paraffin and mineral oil mixture) is a simple and efficient method for applying superficial heat, especially to small joints of the body such as the interphalangeal joints. Infrared is a superficial, dry heat modality, which tends to elevate superficial temperatures more than moist heat. Compared to the other superficial heat modalities, an infrared lamp can increase the temperature without touching the patient, making it the only superficial heating method appropriate for patients with skin defects. Hydrotherapy treats the patient through the medium of water and reduces pain, edema, and muscle spasm. In contrast to superficial heat, penetration of deep heat is up to 3–5 cm.
Deep heat modalities include ultrasound, short-wave diathermy (SWD), and microwave diathermy. The mechanism of heat transfer of ultrasound is conversion, which refers to the transformation of energy (e.g., sound or electromagnetic) into heat. Heat is produced when acoustic energy is absorbed, especially at or near the surfaces of structures with high attenuation coefficients such as bone. Based on the limited data from animal studies showing microscopic changes to the histology of the physis (growth plate) following the use of therapeutic ultrasound, although there are no reports of growth disturbance in humans, this modality should be used with due caution in the pediatric population.[39] The application of therapeutic ultrasound around the physis should be avoided.[39]
SWD converts electromagnetic energy into thermal energy. Continuous SWD is the technique of choice when uniform elevation of temperature is required in deep tissues and inside joints. Subacute or chronic conditions respond well to continuous SWD. SWD has good bone penetrance and can result in heating of the epiphyseal plates in the long bones of children, consequently affecting growth. Hence, caution should be exercised while using this modality in children.[38]
Cryotherapy
Cryotherapy is a treatment modality which involves lowering local tissue temperature. Its major physiological effects include decreasing the nerve conduction velocity of pain fibers, causing vasoconstriction, and reducing joint swelling and muscle spasms.[38] This modality is especially useful in acute pain relief and to reduce inflammation. Cryotherapy involves the use of ice packs, ice massages, cold whirlpools, vapocoolant sprays, and contrast baths. Contrast baths produce alternating vasoconstriction and vasodilation, which reduce local edema and are used to treat subacute swelling. Cryotherapy should not be used in patients with cold hypersensitivity, Raynaud's phenomenon, cryoglobulinemia, or paroxysmal cold hemoglobinuria.
Electrotherapy
The mechanisms of action of electrotherapy in pain management include segmental inhibition of pain signals to the brain and the dorsal horn of the spinal cord (Melzack and Wall gate control theory) and activation of descending inhibitory pathways and stimulation of the release of endogenous opioids and other neurotransmitters (serotonin, gamma-aminobutyric acid, etc.).[38] The used modalities are transcutaneous electrical nerve stimulation and interferential current therapy.
Low-level laser therapy
Low-level laser therapy (LLLT) involves the use of relatively low light energy (<100–200 mW).[38] The potential physiologic benefits of LLLT appear to be nonthermal; it can have a stimulating effect on target tissues. LLLT is useful in decreasing pain and morning stiffness.
| Orthoses | | |
An orthosis is defined as a device attached or applied to the external surface of the body to improve function, restrict or enforce motion, or support a body segment.[40] Orthoses provide protection, help to correct deformities, offloads the joint, and assist in gait.[40],[41] Orthotic devices such as resting hand splints are used for achieving proper positioning of joints, reduction of inflammation and pain, and functional improvement at rest. They are useful when the joint is still mobile and not in fixed joints.
Keeping the orthosis lightweight, comfortable and cosmetic is very important to improve the patient compliance to wear the splint. The patient and his or her family need to be educated and counseled regarding how to don and doff the orthosis, the goal to be achieved, and the reason for positioning the limb in a particular way. Activity modification and functional splints are used for supporting the affected joint while performing a task. The commonly used orthoses along with their indications are summarized in [Table 4].
Assistive technology
Assistive technology is a technology used by individuals with disabilities to perform functions that might otherwise be difficult or impossible. Assistive devices serve to enhance functional ability, promote independence in activities of daily living, and maintain engagement in meaningful activities in individuals with disabilities.[42] Assistive devices frequently used in children with JIA include aids for personal care and protection (aids for dressing and personal hygiene), aids for eating (adapted cutlery and cups, universal cuff) [Figure 1], aids for personal mobility (wheelchairs, walking aids, and crutches), aids for housekeeping (knives and peelers with ergonomic handles), adaptations to homes and other premises (raised toilet seats, shower chairs, and lifts and ramps), and aids for handling products and goods (aids for opening containers and grip function, reach extenders, and carrying aids).
Kinesio Taping
Kinesio Tape is an elastic therapeutic tape that is applied to reduce pain and increase ROM, reduce swelling, and provide mechanical support.[43] It improves local circulation in the taped area by increasing the cutaneous and subcutaneous interstitial area and facilitating lymphatic drainage. It provides sensory feedback and helps in small muscle contraction by pulling the fascia which helps in increasing strength.
Hand function training
Hand function training aims to make the patients independent. Occupational therapy to improve hand function and transfers employs the use of devices such as zipper hooks, chopsticks, and pegboards to improve dexterity and strength [Figure 2]. This training also includes feeding techniques with putty and finger dexterity training for buttoning and unbuttoning.
Serial casting
Some children with mild-to-moderate contractures of the knees, ankles, elbows, wrists, and small joints of the hands benefit from serial casting. Serial casting involves temporary straightening and casting of the affected joint for 2–3 days. This is followed by the removal of the cast, physical therapy, and application of a new cast with the joint stretched to a greater degree than before. The procedure is repeated with the joint a little straighter each time and is continued until maximal straightening has occurred [Figure 3]. Thereafter a resting splint is prescribed for use at night for 3–6 months.
Recent advances
There have been advances over the past two decades with the application of the technologies of robotics and virtual reality (VR) in the field of rehabilitation medicine strategies. A robot refers to “a machine that performs various complex acts of a human being” and “automatically performs complicated, often repetitive, tasks.” The robotic systems include therapeutic robots such as large stationary exercise robots or wearable exoskeletal robots (powered braces) and functional robots such as assistant robots for activities of daily living and companion robots.[44]
VR is defined as a human–computer interface that allows a user to interact with a computer-generated environment, using multiple sensory channels.[45],[46] The virtual environment can provide users with visual, auditory, or tactile feedback. VR can be used to enhance conventional therapy (VR-augmented rehabilitation) or can replace conventional interventions altogether (VR-based rehabilitation).[46]
Orthotics have advanced to a level that with a given deformity, a three-dimensional-printed orthosis can be customized in a few hours [Figure 4]. | Figure 4: 3D-printed orthosis for finger deformities. 3D: Three dimensional
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Telerehabilitation
The COVID-19 pandemic has forced health-care decision-makers to consider alternative modes for delivering health care, such as telehealth. Telehealth refers to the use of telecommunications and virtual technology to deliver health care, including patient rehabilitation (telerehabilitation).[47],[48] Telerehabilitation is also useful for patients who have difficulty to access therapy and might not be able to visit the treatment center frequently. However, they may need regular rehabilitation programs as joint range might be compromised by the disease activity, leading to complications such as disuse atrophy and weakness.
| Conclusion | | |
Rehabilitation is a vital component in the comprehensive management of children with JIA. Rehabilitation strategies depend on the phase of the disease and focus on pain alleviation in the active phase and improving mobility and function in the inactive phase. Rehabilitation in JIA is multidisciplinary and includes exercise therapy, physical modalities, orthotic and assistive devices, and gait training.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]
[Table 1], [Table 2], [Table 3], [Table 4]
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