Special Considerations for Physical and Intellectual Disabilities
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This chapter presents background information and special considerations related to exercise testing, prescription, and progression for individuals with physical and intellectual disabilities (IDs), including spinal cord injuries (SCIs) and autism.
The case study that follows describes an adult female who, following a traumatic SCI, received 21 weeks of combined acute and postacute rehabilitation before being discharged and returning to her home. This case study presents guidance for the design of a progressive aerobic conditioning, resistance training, flexibility, and neuromotor training program with the primary goals of returning to work and improving functional capacity and independence. See Box 23.1 for common terminology related to SCI.
Box 23.1 | Terminology for Spinal Cord Injury |
Tetraplegia: Impairment or loss of motor and/or sensory function in the cervical segments of the spinal cord resulting in impaired function in the arms, trunk, legs, and pelvic organs (7). | |
Paraplegia: Impairment or loss of motor and/or sensory function in the thoracic, lumbar, or sacral segments of the spinal cord. Arm function is preserved, but trunk, legs, and pelvic organs may be impaired depending on the neurological level of injury (7). | |
Neurological level of injury: The most caudal segment of the spinal cord with normal sensory and motor function on both sides of the body as determined by clinical neurological examination (7). | |
Complete injury: No motor or sensory function is preserved in the sacral segments S4–S5 (124). | |
Incomplete injury: Some preservation of sensory and/or motor function below the neurological level of injury (7). | |
Spasticity: Disordered sensorimotor control, resulting from an upper motor neuron lesion, presenting as intermittent or sustained involuntary activation of muscles including clinical presentations of hypertonia, hyperreflexia, flexor and extensor spasms, and clonus (98). | |
Hypertonia: Abnormality in reflex mechanisms resulting from upper motor neuron injuries leading to altered balance of excitation and inhibition of spinal motor neurons and presenting clinically as persistent, involuntary muscle activity and “stiffness” in the trunk and/or extremities (89,91). | |
Clonus: Involuntary rhythmic muscle contraction resulting in distal joint oscillations, which may be elicited by a sudden rapid muscle stretch (17,65). | |
Autonomic dysreflexia: Acute severe paroxysmal hypertension caused by uncontrolled sympathetic activity leading to symptoms such as throbbing headaches, profuse sweating, flushing of the skin, and bradycardia (78). Autonomic dysreflexia is most often associated with spinal cord injuries at or above the level of T5 and if left unattended can become a life-threatening medical emergency. | |
Tenodesis grip: A normal anatomical action whereby, as the wrist extends, the fingers flex and naturally fall against the thumb in a position of lateral pinch. | |
Heterotopic ossification: The abnormal formation of calcified tissue within the hip, knee, elbow, or shoulder resulting in limited range of motion of the involved extremity (18). |
Mrs. Case Study-SCI
Mrs. Case Study-SCI is a 32-year-old female weighing 153 lb (69.5 kg) with a height of 67 in (170 cm). Her body mass index (BMI) = 24 kg ∙ m−2. She was injured 4 years ago in a diving accident, which resulted in a C5 burst fracture with retropulsion of bone fragments into the spinal canal. Surgical management of the injury included anterior decompression and stabilization at C4–C6. Following surgery, she was transferred to an out-of-state specialty SCI rehabilitation hospital where she completed 5 weeks of inpatient, 8 weeks of outpatient, and 8 weeks of transitional rehabilitation. No cognitive deficits were indicated following her accident, and she reported being able to follow all directions and commands throughout postacute rehabilitation. Upon discharge, a final neurological examination using the American Spinal Injury Association (ASIA) International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI) was performed. Results from the examination indicated a C5 neurological level of injury with ASIA Impairment Scale (AIS) classification C indicating motor-incomplete tetraplegia. Mrs. Case Study-SCI demonstrates some partial but very weak motor function in the lower extremities as well as present but impaired sensation below her neurological level of injury. Figure 23.1 provides the detailed motor and sensory scores from her neurological examination.
FIGURE 23.1. ASIA ISNCSCI examination results for Mrs. Case Study-SCI at discharge from rehabilitation. (From American Spinal Injury Association. International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI). Atlanta [GA]: American Spinal Injury Association; 2015.)
Mrs. Case Study-SCI reports that she uses a manual wheelchair with power-assisted wheels as her primary means of mobility and lives with her husband in a small, rural community in the Midwest United States. Her home has been modified and made accessible to accommodate her needs. When pushing her wheelchair over long distances or uphill, she complains of arm muscle fatigue, shoulder pain, dizziness, and shortness of breath. She reports feeling “out of shape,” and her posture appears kyphotic with rounded shoulders, forward head, and protruding abdomen. In addition, she reports experiencing “stiffness” in the hip and knee extensors as well as the ankle plantar flexors when transferring to and from her wheelchair. Both parents are living and in good health and occasionally assist her with activities of daily living (ADL) when her husband is unavailable. Mrs. Case Study-SCI indicated that she smokes infrequently during social occasions, and although she was given a home exercise program and desires to be more physically active, she has not participated in a structured exercise program since discharge from transitional rehabilitation. Upon inquiry, she states lack of energy, lack of accessible facilities in her area, and limited knowledge of appropriate exercises as barriers to exercise participation. She owns an assistive standing device (i.e., an EasyStride standing frame), which she uses to stand approximately 45–60 minutes per day, as well as resistance bands and wrist weights in 2-, 4-, and 6-lb (.90-, 1.81-, and 2.72-kg) increments. On occasion, she notes feeling light-headed and dizzy when moving from a prone to seated or standing position but only when she does not take her orally prescribed 10-mg dose of midodrine.
Three years ago, she completed a course of bisphosphonate (Didronel [etidronate disodium]) and nonsteroidal anti-inflammatory drug (i.e., NSAID [indomethacin]) to treat bilateral hip heterotopic ossification (HO). Her most recent x-ray identified mild, nonprogressive HO inferior to the right femoral neck. She reports being on a consistent bowel and bladder management program and is currently taking the following medications to manage neuropathic pain, lower extremity spasticity, neurogenic bladder, and orthostatic hypotension:
Imipramine (antidepressant/bladder incontinence): 50 mg orally once per day
Baclofen (antispasmodic): 20 mg orally three times per day
Midodrine (antihypotensive): 10 mg orally as needed when active
Gabapentin (neuropathic pain): 300 mg orally two times per day
See Table 23.1 for common medications that are used to manage secondary conditions for patients with SCI.
Baseline blood work and cardiovascular measures from her most recent annual physical examination revealed the following:
Total cholesterol: 187 mg ∙ dL−1 (4.84 mmol ∙ L−1)
Low-density lipoprotein cholesterol (LDL-C): 103 mg ∙ dL−1 (2.67 mmol ∙ L−1)
High-density lipoprotein cholesterol (HDL-C): 35 mg ∙ dL−1 (1.35 mmol ∙ L−1)
Fasting blood glucose: 107 mg ∙ dL−1 (5.94 mmol ∙ L−1)
Seated resting blood pressure (BP): 94/60 mm Hg
Seated resting heart rate (HR): 68 bpm
Mrs. Case Study-SCI described the following as her health and fitness goals:
Improve independence with ADL so that she can return to work.
Learn an exercise routine that she can perform independently.
Improve balance and limits of stability while seated.
Decrease body weight in order to assist with mobility and transfers.
Common Medications Used to Manage Secondary Conditions in Spinal Cord Injury |
Generic Drug Name/Brand Name | Use or Condition | Common Side Effects |
Amitriptyline/Elavil | Antidepressant | Drowsiness, dizziness, blurred vision, weight gain, dry mouth |
Imipramine/Tofranil | Antidepressant and neurogenic bladder | Blurred vision, headache, drowsiness, nausea, weight gain/loss |
Nortriptyline/Pamelor | Antidepressant and neuropathic pain | Drowsiness, dizziness, blurred vision, weight gain, dry mouth |
Carbamazepine/Tegretol | Anticonvulsant and neuropathic pain | Nausea, dizziness, drowsiness, dry mouth |
Pregabalin/Lyrica | Anticonvulsant and neuropathic pain | Drowsiness, dizziness, dry mouth, weight gain, swelling in arms/legs |
Gabapentin/Neurontin | Anticonvulsant and neuropathic pain | Drowsiness, dizziness, fatigue, ataxia, sedation, loss of coordination |
Baclofen/Lioresal | Antispasmodic | Drowsiness, dizziness, muscle weakness, headache, fatigue |
Diazepam/Valium | Antispasmodic | Drowsiness, dizziness, fatigue, blurred vision, headache |
Dantrolene sodium/Dantrium | Antispasmodic | Drowsiness, dizziness, weakness, fatigue |
Tizanidine/Zanaflex | Antispasmodic | Drowsiness, dizziness, light-headedness, weakness, dry mouth |
Botulinum toxin/Botox | Antispasmodic/hypertonia | Localized muscle weakness, neck or back pain, shortness of breath |
Midodrine hydrochloride/ProAmatine | Hypotension | Supine hypertension, paresthesia, blurred vision, cardiac awareness, headache |
Warfarin sodium/Coumadin | Anticoagulant | Nausea, persistent bleeding following injury, bruising |
Enoxaparin sodium/Lovenox | Anticoagulant | Fatigue, persistent bleeding following injury, bruising |
The spinal cord is part of the central nervous system and is composed of longitudinally oriented spinal tracts that lie within the vertebral column. These spinal tracts consist of sensory and motor neurons that make it possible for the brain, body systems, and organs to communicate. Figure 23.2 provides an illustration of the spinal column and spinal nerves and a general description of the major muscle groups associated with each spinal cord segment.
FIGURE 23.2. Spinal column and spinal nerves with major muscle groups associated with each spinal cord segment. Reprinted with permission from World Health Organization (WHO). From: Bickenbach J, et al. (2013) World Health Organization and The International Spinal Cord Society , International Perspectives on Spinal Cord Injury (ISCoS).
Damage to the neural elements within the spinal canal can have a profound effect on many body systems and can lead to complete or partial loss of motor, sensory, and autonomic function below the spinal cord lesion level. SCIs can occur as a result of traumatic events or nontraumatic pathologies. Common causes of traumatic SCI include motor vehicle accidents, falls, violence, and occupational or sports injuries (93), whereas causes of nontraumatic SCI include congenital-genetic disorders (e.g., spinal dysraphism, skeletal malformations, hereditary spastic paraplegia) or acquired conditions (e.g., vertebral column degeneration, infectious disease, metabolic and vascular disorders, spinal tumors, inflammatory and autoimmune diseases) (95). The nature and severity of impairments resulting from SCI are dependent on the mechanism, level, and “completeness” of the injury. Injuries to the cervical segments of the spinal cord (C1–C8) result in tetraplegia with impairment or loss of motor and/or sensory function in the arms, trunk, legs, and pelvic organs (bowels, bladder, sexual organs). Injuries to the thoracic (T1–T12), lumbar (L1–L5), or sacral (S1–S5) segments of the spinal cord result in paraplegia and result in impairment or loss of motor and/or sensory function in the trunk, legs, and pelvic organs. Neurological examinations such as the ASIA ISNCSCI are often administered to describe the neurological level and completeness of an injury and to provide a means by which standardized information regarding motor, sensory, and autonomic impairments can be communicated among health care providers. Based on examination findings, individuals with SCI are given an AIS classification based on an “A” to “E” grading system. Table 23.2 provides a description of each AIS grade based on the presence or absence of motor and sensory function below the neurological level of injury.
Table 23.2 | American Spinal Injury Association Impairment Scale Classification |
AIS Grade | Degree of Impairment |
A | Complete: No motor or sensory function is preserved in the sacral segments S4–S5. |
B | Sensory incomplete: Sensory function is preserved below the neurological level of injury including the sacral segments S4–S5. No motor function is preserved more than three levels below the motor level. |
C | Motor incomplete: Sensory plus some motor function is preserved more than three levels below the motor level. Less than half of key muscles below the neurological level of injury have a muscle grade ≥3. |
D | Motor incomplete: Sensory plus motor function is preserved more than three levels below the motor level. At least half of key muscles below the neurological level injury have a muscle grade ≥3. |
E | Normal: Motor and sensory function is preserved and graded as normal. |
Adapted from American Spinal Injury Association. International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI). Atlanta (GA): American Spinal Injury Association; 2015.
Globally, the annual incidence of SCI is estimated to be between 250,000 and 500,000 new cases (129). In the United States, it has been reported that SCI is the second leading cause of paralysis and that approximately 12,500 new cases occur each year (28,94). Approximately 59% of these cases involve the cervical spinal segments, and 41% involve the thoracic, lumbar, and sacral segments (94). Although differences in the average age at injury exist between traumatic SCI (15–29 yr) and nontraumatic SCI (60–70 yr), males continue to make up the majority of those who experience an SCI across injury types (28,93,130). Despite advances in emergency medical management and postacute rehabilitation, the life expectancy of persons with SCI remains lower than the general adult population, with two of the major leading causes of premature death associated with diseases of the respiratory and cardiovascular systems (26,93,130).
Owing to altered muscle morphology/physiology, impaired cardiorespiratory function, and increased sedentary time, persons with SCI are likely to experience diminished muscular strength, endurance, and aerobic capacity (70,111). It is well documented that cardiovascular control is altered after SCI due to disruption of descending input to the autonomic nervous system (ANS), especially for those with complete injuries at or above the level of T5 (125,126). ANS dysfunction can lead to impaired sympathetic regulation of BP, HR, and body temperature. Impaired respiratory function caused by weakness and/or stiffness of the respiratory muscles may also contribute to compromised and limited exercise capacity among persons with SCI (13,126). Further compounding the problem of physical deconditioning are low exercise participation and increased sedentary time among those with mobility impairments (88,105). The combination of these factors, along with pathological neuromuscular adaptations associated with SCI (i.e., muscle atrophy, decrease fat-free mass, and increase fat mass) (19,53), can significantly contribute to impaired functional capacity, decreased independence with ADL, increased risk of cardiovascular and metabolic comorbidities, and decreased life expectancy (26,32,112). Consequently, physical deconditioning and its impact on health and function are major concerns for health care providers and for persons living with SCI and should be addressed using evidence-based countermeasures with measurable health and fitness outcomes.
Preparticipation Health Screening, Medical History, and Physical Examination |
A detailed investigation into the medical and health history of persons with SCI will likely reveal a number of important factors that may or may not necessitate medical clearance but will likely be required in order to determine the most appropriate exercise testing and prescription. Compared with healthy adults and those with other chronic health conditions (i.e., known cardiovascular disease), far less evidence exists regarding the prevalence of adverse events (especially those related to cardiac episodes) during exercise participation among persons with SCI. Although current evidence within this population points to a relatively low risk of serious adverse events during exercise (123,133), it is still necessary to obtain a thorough medical and physical activity history in accordance with the American College of Sports Medicine’s (ACSM) recommendations for preparticipation screening with additional consideration given to the injury-specific conditions associated with SCI (24). Self-guided questionnaires, such as the American Heart Association (AHA)/ACSM Health/Fitness Facility Pre-Participation Screening Questionnaire (11) or the revised Physical Activity Readiness Questionnaire for Everyone (PAR-Q+) (122), may be useful prescreening tools with the latter containing specific questions concerning SCI. Figure 23.3 provides a general decision tree for preparticipation screening for SCI. Additional considerations unique to exercise participation among persons with SCI have been provided in Table 23.3.
FIGURE 23.3. General preparticipation screening considerations for persons with SCI. CV, cardiovascular; Rx, prescription. (Adapted from Zehr E. Evidence-based risk assessment and recommendations for physical activity clearance: stroke and spinal cord injury. Appl Physiol Nutr Metab. 2011;36:S214–31.)
Spinal Cord Injury Considerations for Exercise Participation |
Preparticipation Health Screening, Medical History, and Physical Examination 1. What would be the suggested guidelines regarding preparticipation screening for this patient? 2. Describe the criteria used to justify the final recommendations for exercise participation. |
Information obtained during preparticipation screening will be needed to facilitate selection of exercise tests, equipment, protocols, and individual accommodations. Not dissimilar to exercise testing considerations for other populations, test selection for persons with SCI should be based on the individual health and fitness goals and functional limitations of the participant. A general overview of exercise testing considerations in SCI follows. Table 23.4 provides supplementary content relevant to each of the exercise testing domains described in this section.
Additional Considerations and Significance of Exercise Testing Among Persons with Spinal Cord Injury |
Exercise Testing Domain | Considerations/Significance |
Body composition | Spinal cord injury often results in physical, metabolic, and lifestyle changes that can lead to decreased total daily energy expenditure, reduced resting metabolic rate, and reduced thermic effect of activity (30,92,112). Consequently, there is a high prevalence of obesity among persons with SCI, and the risk of developing comorbidities is greater compared to the general population (54,86). Monitoring changes in body composition, although difficult in SCI, could provide exercise professionals with valuable information related to the effectiveness of exercise interventions aimed at combatting overweight and obesity. |
Cardiorespiratory fitness | Cardiorespiratory fitness has significant health implications for those with SCI and can be affected by a number of factors including level of injury, severity of injury, degree of physical deconditioning, and extent of ANS impairment (112). Consistent with its use among other populations, results obtained from cardiorespiratory exercise testing in SCI can be used to determine relative risk for the development of secondary health conditions, to assist in the development and implementation of the exercise prescription, and to establish baseline criteria for the effectiveness of exercise interventions. Specific cardiorespiratory assessment methods, fitness profiles, and norm-referenced data have been reported for persons with SCI (50,56,111); however, clinicians and exercise professionals should use caution when comparing cardiorespiratory fitness outcomes as existing data is limited to specific modes of testing and SCI characteristics (i.e., level and completeness of injury). |
Muscular strength and endurance | Muscular strength and endurance testing can provide valuable information regarding changes in physical conditioning and maintenance of strength over time. Because of the heterogeneity within the SCI population, norm-referenced outcomes for muscular strength and endurance across all levels of injury and completeness have not been established; however, preliminary reference data has been reported for a small subset of the population (70,111). In the absence of comprehensive norm-referenced data, pre-/posttraining comparisons can be made between the maximum number of completed repetitions or the maximum weight lifted before and after training. |
Flexibility | Whether propelling a manual wheelchair or using an assistive device while walking, persons with SCI are likely to rely heavily on the chest, shoulders, and arms for mobility. Assessing and maintaining range of motion across these joints should be a priority for the exercise professional. |
Neuromotor | Neuromuscular function and movement quality are significantly and negatively affected as a result of SCI. Similar to aging and frail populations, persons with SCI who ambulate at home or in the community may be at an increased risk of falls due to impaired motor control, balance, coordination, and proprioception (107). Neuromotor training may be an effective strategy for improving skill-related components of fitness and may positively affect the structure and function of key brain and spinal centers involved in cognition and motor task performance, such as walking (2,22,127). |
Cardiorespiratory Fitness Testing
Cardiorespiratory exercise test selection should be determined based on consideration of neurological level of injury, motor completeness, and the extent to which the test allows the participant to utilize the largest muscle mass possible. Upon consideration of these factors, exercise professionals may choose from a variety of cardiorespiratory testing protocols and modalities including continuous, discontinuous, submaximal, and maximal assessments; however, exercise professionals should keep in mind that formulas used to estimate maximal aerobic capacity from submaximal testing in noninjured populations may not be valid for persons with SCI. Incremental multistage test protocols can be implemented for arm, leg, or combined cycle ergometry as well as recumbent stepping where workloads are increased every 1–3 minutes by increments of 5–10 W per stage for persons with tetraplegia or 10–25 W per stage for persons with paraplegia (5,56). When available, task-specific manual wheelchair tests can be performed using a stationary wheelchair roller system or a motor-driven treadmill. For wheelchair athletes, sport-specific field tests such as the 12-minute wheelchair propulsion test can be used to estimate peak cardiorespiratory capacity (41,51). Individuals who are ambulatory and present with significant preservation of trunk and lower limb function may be able to perform standardized overground or treadmill-based walking tests (119). It is important to note that individuals with motor-complete SCI above T5–T6 may exhibit lower cardiovascular performance compared to those with lower level paraplegia and motor-incomplete injuries (67,87), and it may be necessary to treat postexercise hypotension and exhaustion with rest, recumbency, and leg elevation following maximal-effort exercise testing (5). In addition to the ACSM’s guidelines for healthy adults, exercise test termination criteria for persons with SCI should include any signs or symptoms of autonomic dysreflexia (AD) or orthostatic hypotension (OH).
Field measures of body composition (e.g., BMI, skinfold measures, bioelectrical impedance, circumference measures) can be performed relatively quickly and require minimal equipment but are often based on specific assumptions regarding fat-free composition that may not be valid in SCI (15,23,112). Laboratory measures may provide greater reliability and validity but often require specialized equipment, are likely to incur greater cost, and may not be easily accessible to exercise professionals and persons with SCI. Recent evidence suggests that predictive equations developed for noninjured populations are a poor fit for those with SCI, and body fat percentage is often significantly underestimated in this population when using field techniques (15,115,132). Dual-energy x-ray absorptiometry may not be the most accessible for all persons with SCI, but the method appears to meet reliability and validity standards, can be less invasive than other measures, and may be obtained in conjunction with routine clinical assessments of bone mineral density.
Muscular Strength and Endurance Testing
Assessment options for muscular strength and endurance among persons with SCI are similar to those found in the general population and include one repetition maximum (1-RM), 10-RM, isokinetic, and isometric testing. Special consideration should be given to the level and severity of SCI, the desired muscle(s) to be tested relative to the personal fitness goals, the extent of preserved motor function, the need for adaptive or accessible equipment, and the presence of range-of-motion (ROM) limitations due to contracture or HO. Traditional assessments, such as the 1-RM and 10-RM, can typically be performed using equipment found in most fitness centers and can be used to assess muscular strength across single or multiple joints. Functional exercise tests, including the modified push-up, modified pull-up, supine-to-sit, seated depression, and prone-on-elbows press-up, can also be used in conjunction with or as a supplement to traditional muscular strength tests such as the horizontal row, lateral pull-down, and bench press (111,112). For individuals with sufficient sparing of trunk and/or lower extremity muscles, it may be possible to incorporate some specific lower extremity and core strength assessments into the test battery (i.e., seated leg press, timed modified prone plank).
For persons with SCI, flexibility and adequate joint ROM are essential to maintaining mobility and decreasing the risk of injury. Upper extremity, trunk, and lower extremity ROM can be assessed using a goniometer and can be used to identify joint ROM deficiencies that might exist or that may begin to develop over time.
A number of functional assessments have been used to monitor changes in neuromotor function over time. For individuals with SCI who are ambulatory, standing balance, dynamic reach, and modified agility tests may be valuable tools for tracking functional overground performance. Specific assessments with potential utility for persons with SCI include the four-stage balance test (25), standing reach test (39,125), Timed “Up and Go” (102), the 30-second chair stand test (72), Edgren side-step test (40), and the agility T-test (100). Alternatively, the Thoracic-Lumbar Control Scale (9) may provide a means of assessing functional trunk control and seated balance for persons with limited or no lower extremity muscle activation and who require a wheelchair as their primary means of mobility. Exercise professionals who wish to incorporate tests of balance, agility, coordination, and proprioception will need to thoroughly assess each participant to determine the most appropriate and safest neuromotor assessments.
Exercise Testing Considerations 3. What are the intended actions and their potential impact on exercise testing of the medications prescribed for Mrs. Case Study-SCI? 4. What exercise test protocols would you select for this participant? Justify your answer. Were the exercise tests appropriate for this participant? Why or why not? 5. What safety precaution(s) need to be taken into account for a participant with SCI performing a 1-RM test? |