Nonmedical Therapies in Pediatric Rheumatic Diseases

Acknowledgements

Gay Kuchta, OT, is acknowledged for her contributions in the previous editions that provided a sound basis for this revised chapter. Machteld Huber, MD, PhD, is acknowledged for sharing her expertise on the My Positive Health model.

Over the last decades, great advances have been made in the medical management of the pediatric rheumatic diseases. Despite this, children with rheumatic disease report a poorer health-related quality of life than their healthy peers. Optimal treatment of rheumatic diseases of childhood requires early and ongoing intervention by an interdisciplinary team of health professionals. As the medical management of rheumatic diseases has evolved, the spectrum of problems dealt with by therapists has changed considerably. Today’s therapist must pay attention to both frank and subtle functional challenges in physical, emotional, social, and educational spheres, as well as long-term outcomes of health. With the increased physical possibilities for the pediatric rheumatic patient, patients are expecting to pursue more physically demanding sports and leisure time activities.

These spheres can be recognized in a novel perspective on health as introduced by Huber et al., defining “health as the ability to adapt and self-manage, in face of social, physical and emotional challenges.” Especially in very young children with a pediatric rheumatic condition, adaptation is a commonly observed behavior; for example, the toddler with arthritis in one knee refrains from walking and starts “bottom shuffling” to adapt to a decreased ability to bear weight through the lower extremity. Convincing an older child who suffers from chronic fatigue to practice self-management techniques, such as pacing and energy conservation during school or leisure activities, may be a challenge for both parents and health professionals. However, self-management has proven to be a potent modifier for health in children.

Based on the My Positive Health model, the novel concept of health by Huber, a tool for children (8 to 16 years) and youth (16 to 25 years) has been developed and made available to inventory children’s health perception (publication in progress). This self-reflection tool can be used to start communication with children and their parents to assess the challenges and barriers they face in the course of a disease. It may prove helpful to collaboratively define the physical, emotional, and social needs of children and their families to improve their health. The six constructs of the model ( Fig. 15.1 ) provide a basis for the five sections that structure this chapter and that will enable readers to find their way to successful treatment strategies.

The chapter is organized in five sections that outline a series of topics of relevance to many pediatric rheumatic conditions and that will contribute to the health of the child ( Fig. 15.2 ).

Although the majority of patients seen in a pediatric rheumatology clinic have juvenile idiopathic arthritis (JIA), therapists are now involved in the treatment of children with the full spectrum of rheumatic diseases. Each section therefore will include generic and disease type–specific information on how symptoms are revealed through clinical observation, anamneses (history), and examination ( Fig 15.3 shows a brief overview on clinical findings and functional consequences); how they can be measured through clinimetrics and what the treatment strategies could be. Whenever possible, therapy interventions ( Table 15.1 and Boxes 15.1 to 15.7 ) are based on evidence. Often, the limited information from studies in childhood rheumatic diseases must be supplemented by information from adult populations with similar diseases and by personal experience. The therapeutic team varies and may contain physical and occupational therapists, nurses, exercise physiologists, social workers, psychologists, and pharmacists as well as physicians. All members must work closely with each other, and the child and family, to provide consistent and comprehensive care.

TABLE 15.1

Red Flags for Therapy Interventions

Multiple system involvement	Activity restrictions imposed by other subspecialties Degree of impaired vision and functional implications Central nervous system and cognitive restrictions
Comorbidities	Psychiatric conditions Developmental delay
At-risk social situation	Guardianship issues with restricted access Ability of family to cope with treatment plan Ability of family to financially cope with treatment recommendations Social service involvement
Abnormal imaging or lab results	Bony changes that limit or determine specific therapeutic interventions (ankyloses, subluxation, abnormal bone growth, osteoporosis, excessive joint swelling) Severe anemia that affects function and therapeutic interventions (pool, aerobic activities) Uncontrolled disease Joint instability, especially of the cervical spine

BOX 15.1

Areas of Occupational and Physical Therapy Intervention

Assess and teach pain management
Assess body functions and structures, document impairments, develop treatment plan to address findings
Assess and document activities and athletic skills, and minimize functional restrictions
Develop consistent, reliable measures of changes over time in (1) musculoskeletal health (e.g., joint range, muscle strength, musculoskeletal flexibility, and health-related fitness and endurance), (2) motor-proficiency, and (3) physical literacy
Develop, upgrade, and negotiate with the child and family an exercise program to address the identified deficits and define the expected outcome
Educate on the continued need and safety of physical activity
Evaluate and teach coping skills for symptom management
Evaluate and teach self-management skills
Teach ergonomics
Identify the need for and refer to vocational counseling
Provide ongoing patient and family support
Facilitate integration into school and community
Advocate for the child with the family, school, and community
Refer to and liaise with community therapists
Liaise with sport coaches and trainers
Facilitate transition to adult care

BOX 15.2

Indications to Refer to Occupational and Physical Therapy

Active disease
Avoidance of activity or inability to keep up because of pain or weakness
Significant morning stiffness
Overwhelming fatigue
Change in quality of sleep
Marked mood or behavioral change, especially isolation from peers
Inability to do normal activities in a timely manner
Regression of age-appropriate development
Restricted/asymmetrical movement
Growth abnormalities
Reduced school attendance or output (>10 days absence in the past 4 months)
Reduced or abstinence of participation in physical education lessons, sports and active recreation

BOX 15.3

Occupational and Physical Therapy Interventions: Uncontrolled Disease

Disease parameters are high, pain can be present even at rest; fatigue and weakness are marked

Goal: Minimize Symptoms

Direct assessment to address patient’s stated problems
With juvenile dermatomyositis (JDM), assess that children are safe ambulating and swallowing
Provide frequent reassessment to monitor changes, keep interventions to a minimum
Teach pain management and coping skills
Teach fatigue management, especially with connective tissue disease
Maintain range, strength, and muscle flexibility using exercises, splinting, and positioning
Reinforce education on disease management
Intervene at school with all diagnoses
Provide adaptive devices as indicated by the child
Teach sleep hygiene and screen time management
Teach coping strategies for systemic symptoms (Raynaud phenomenon, uveitis)

BOX 15.4

Occupational and Physical Therapy Interventions: Controlled Disease

Disease activity is reduced, pain is occasional, and energy, strength and flexibility are improving

Goal: Maximize Function

Full assessment to identify and monitor persisting deficits and their impact on the body mechanics and function
Modify pain and fatigue management techniques
Improve function in self-care
Increase joint range, muscle strength, flexibility, and endurance
Identify and retrain faulty movement patterns
Improve exercise tolerance and balance
Increase participation in family, school, sports, and leisure activities, especially physical activities
Review and monitor necessary school interventions to reflect changing physical status
Introduce the concepts of self-image and self-efficacy to child and family
Teach coping skills such as time management and pacing activities
Review understanding of disease management as the child matures
Interventions reviewed every 4 to 12 weeks
Interventions delivered via a short home program (10 minutes), regular clinic visits or occasionally inpatient rehabilitation

BOX 15.5

Occupational and Physical Therapy Interventions: Clinical Remission

Disease is in remission either with or without medications

Goal: Normal Function

Semiannual to annual full assessment to determine whether physical and psychosocial sequelae of previous issues persist
Assess for ongoing pain and fatigue issues
Focus on:
- Abnormal movement patterns
- Mechanical malalignments
- Sleep hygiene and screen time management
- Risk-taking behaviors
- Physical and psychological developmental milestones
Work on fine motor control, balance, endurance, power
Work on physical literacy (motivation, confidence, competence, knowledge, and understanding)
Promote physical and emotional independence
Promote integration/participation in activities, sports, and the community
Reinforce self-image as a healthy, active individual
Encourage healthy life choices

BOX 15.6

Occupational and Physical Therapy Interventions: Ongoing Chronic Disease

Low-grade active disease or disease quiescent with residual restrictions and damage

Goal: Optimize Function within Limitations

Full assessments with any disease flares
Annual assessment to monitor deficits
Provide targeted exercises for issues amenable to change
Address changing pain patterns and causes, teach advanced pain coping skills if needed
Determine need for adaptations to the environment of the home, school, or car
Teach effective adaptive movement patterns
Assess the need for and teach the use of mobility aids
Assess and plan for preoperative and postoperative interventions
Promote independence in self-care and participation in leisure, work, and physical activities: problem solve around personal and environmental restrictions
Refer to vocational assessment
Provide documentation for financial support or accommodation at university/college
Foster progression toward independent living

BOX 15.7

Less Frequently Used Interventions

Less utilized therapeutic interventions:

Serial casting with or without concurrent intraarticular joint injections
Splints: working or resting, orthotics
Adaptations to accommodate joint restrictions (rocker bars on shoes, raised toilet seats)
Orthopedic manual physical therapy techniques
Electrical muscle stimulation
Electrical modalities for controlling pain (transcutaneous electrical nerve stimulation [TENS], interferential current [IF], laser)
Ambulation training with aids
Wheelchair assessment, fitting and training in use

Joint and Muscle Health

Physical activity and exercise directly affect joint and muscle health and are resources to enable individuals to live full, productive lives. Exercises enable individuals to engage in greater physical activity and social interaction. Integrity of the articular and muscular systems is essential for an individual to adapt and self-manage physical challenges. All the rheumatic diseases of childhood affect joints and/or muscles to various degrees. Healthy joint motion, muscle strength, flexibility, and endurance will affect an individual’s physical fitness. Flexibility and muscle strength are components of physical fitness and contribute to the definition of physical literacy. The International Classification of Functioning, Disability and Health places issues involving joints and muscles into the domain of structure and function. Health conditions affecting structure and function will have a domino effect on both activities of daily living and social participation. This influences mental and social health.

Epidemiology of Joint and Muscle Health in Pediatric Rheumatic Conditions

Juvenile Idiopathic Arthritis

Arthritis can occur in any of the rheumatic diseases of childhood. It is almost universally present in patients with JIA at some point in their disease course. A 2015 study of children with JIA receiving contemporary optimum medical management observed that 48% to 78% (rheumatoid factor [RF]+ polyarticular JIA vs. oligoarticular JIA) reached an active joint count of zero within 2 years of diagnosis. Despite a high frequency of disease flares (42.5%) after attaining inactive disease status, these statistics demonstrate that joint inflammation is now transient for many children with JIA. Muscle weakness of the surrounding musculature often accompanies joint inflammation resulting from muscle reflex inhibition. This is particularly evident in the muscles that pull the joint into a “closed packed position” (i.e., joint position with optimal mechanical stability). This muscle weakness persists long after disease remission.

Juvenile Dermatomyositis

Ninety-five percent of children diagnosed with juvenile dermatomyositis (JDM) present with symmetrical muscle weakness. Studies have shown that axial and proximal muscle groups are statistically weaker than distal muscle groups and that lower extremity muscles are weaker than upper extremity muscles. There is a reported prevalence of arthritis in 23% to 64% of JDM patients.

Juvenile Systemic Lupus Erythematosus

Musculoskeletal manifestations occur in 60% to 82% of children diagnosed with juvenile systemic lupus erythematosus (JSLE). Arthritis is the most frequent issue. It occurs early in the disease and often involves multiple joints. Myositis occurs in less than 5% of patients. Fatigue is the predominant presenting feature of JSLE. This can lead to inactivity and deconditioning. Evidence of long-term muscle weakness in this group of patients varies. In a longitudinal study of JSLE patients, almost none had evidence of muscle atrophy or weakness. Another study demonstrated that JSLE patients who were physically active still had significantly lower muscle strength than normal.

Juvenile Scleroderma

In a description of clinical manifestations of juvenile scleroderma (JS), 19% of patients had arthritis, 34% had contractures, and 12% had myositis. Localized scleroderma, or morphea, is the most common type of scleroderma diagnosed in children. It primarily involves the skin, but it can progress to involvement of the underlying fat, muscle, and bone. Fibrotic skin lesions and atrophy of underlying musculature lead to joint contractures and limb shortening. Active disease is often sporadic but continues to be active in 12% to 38% of children with morphea into their adult years. Most tissue damage occurs early in the disease course. ^,

Examining Joint and Muscle Health

When possible, an assessment of affected joints, muscles, and function should be done during the initial clinic appointment. This defines the extent of restrictions and provides a baseline to monitor change with treatment. Pain, anxiety, and fatigue are often barriers to reliable measurement. A full musculoskeletal assessment is recommended within the first few months of disease. The patient’s disease state and proximity to the clinic may dictate the timing of this assessment.

Assessment of Joint Range of Motion and Muscle Flexibility

Restrictions in joint range of motion may reflect intrinsic joint inflammation (increased intraarticular fluid, synovial hypertrophy, pain) or shortening of capsule, ligament, tendon, muscle, or overlying skin. In children with severe, longstanding active joint inflammation, joints may become deformed, subluxed, or ankylosed, resulting in marked limitation, absence of joint motion, or abnormal movement. Although infrequent, these consequences can occur if there are significant delays in initiation of medical treatment or the disease proves resistant to treatment. In disorders such as JS, fibrotic skin lesions and atrophy of underlying musculature lead to joint contractures and limb shortening, and 30% to 50% of patients have significant joint contractures. In JDM, muscle weakness, atrophy, and disuse may limit functional range of motion resulting in secondary periarticular tightening. Accompanying calcinosis in JDM can cause blocks and pain, which severely limits motion.

Passive range of motion of all affected joints should be assessed using a goniometer to obtain consistent, precise, and reproducible measurements. Active range should also be recorded when it differs significantly from passive range, for example, when a quadriceps lag exists or there is decreased active finger flexion secondary to flexor tendon tenosynovitis. The universal goniometer is still the tool of choice for regular clinic work in measuring joint range and has been shown to have good reliability and validity. ^, Increasing availability of smartphone applications and photo and video programs to measure joint range may change the tool of choice as more studies of their reliability and validity become available. This may also herald reliable self-assessment of patients at home, facilitating follow up of patients who are geographically remote. Feasibility of assessor independent measures, such as electro-goniometry, to assess active joint range and flexion and extension torques in JIA are also being explored. The opening and lateral movements of the temporomandibular joint can be measured using a ruler or a TheraBite Range of Motion Scale. The modified Schober test is useful for monitoring lumbar spine mobility in children with enthesitis-related and psoriatic subtypes of JIA. Studies frequently reduce the effects of individual measurement error by using various methods of combining measurements. The Global Range of Motion Score and the Pediatric Escola Paulista de Medicina Range of Motion Scale are standardized and validated methods.

Attention should be paid to the effect of muscles that cross two joints to minimize false measures of joint restriction resulting from insufficient muscle length. Other structures such as skin and subcutaneous tissues are also known to limit joint range of motion and should be taken into account during the assessment. Normal range of motion varies considerably with age and with each individual. Knowledge of a specific child’s normal ranges allows the therapist to recognize minor restrictions that could easily be overlooked. Between 8% and 60% of the pediatric population is hypermobile. ^, A decrease in hypermobility is an important indicator of joint restriction. Functional range requirements are related to age and culture. Attention to the quality of movement and the use of compensatory movement patterns is integral to a range assessment and often indicate functional issues related to loss of range. Abnormal alignment, such as a wrist or knee lateral deviation, should also be documented ( Box 15.8 ) .

BOX 15.8

Functional Assessment of Decreased Flexibility

Observations: signs of pain, quality of movement, efficiency, accuracy, compensatory/adaptive patterns, balance, and endurance
Activities for functional assessment include:
- Upper extremity activities such as weight bearing on palms, reaching overhead, catching balls, dressing, and fine motor activities such as writing
- Lower extremity activities such as walking, running, stairs, squatting up and down from the floor, putting on shoes and socks

You're Reading a Preview

Become a Clinical Tree membership for Full access and enjoy Unlimited articles

Become membership

If you are a member. Log in here