Neurons (nerve cells)
Appendix 1
The nerve cell (also known as “neuron” or “neurone”) is the basic unit of the nervous system. Nerve
cells carry information between the central nervous system (CNS) and the rest of the body as electrical
impulses. There are three types of nerve cells:
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Motor nerve cells, which take information from the CNS to a muscle or gland
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Sensory nerve cells, which do the opposite, taking messages from the rest of the body to the CNS
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Interneurons (also known as “relay neurons”), which carry information between nerve cells
Figure A1.1 shows a typical nerve cell. Note the cell body and axon. Information enters the nerve
cell through the dendrites and cell body and exits via the axonal endings. The cell bodies form
the gray matter of the brain, and the axons form the white matter, or the communication tracts.
The whitish color of the white matter is due to the fatty substance, called “myelin,” that covers
the axons; this insulates and speeds up the transmission of electrical impulses. Nerve cells receive,
interpret, and transfer messages as electrical impulses. These electrical impulses form the brain’s
electrical activity, which can be measured and recorded on an electroencephalogram (EEG).
Figure A.1 Nerve cell. Adapted with permission from The Identification and Treatment of Gait Problems in Cerebral Palsy, 2nd edition, edited by James Gage et al. (2009). Mac Keith Press.
Multidisciplinary team members
Appendix 2
The following lists the medical specialties of medical professionals who may be part of the multidisciplinary
team caring for individuals with spina bifida. Some individuals with spina bifida will need many or all these medical specialties; others will not. Additional medical professionals may be added as needed. This list is not exhaustive, and not all teams will include the same specialties.
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Anesthesiology (administration of medication resulting in a sleep-like state needed for surgery)
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Assistive technology (product design and services to enhance the functional capabilities and independence of individuals with disabilities)
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Care coordination (coordination of care for individuals)
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Child life (child development and effective coping through play)
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Complex care (care for individuals with complex health care conditions)
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Critical care (care for individuals who are critically ill)
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Developmental pediatrics (evaluation and treatment of children with developmental, learning, and behavioral conditions) • Gastroenterology (disorders of the digestive system)
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Genetics (evaluation and management of genetic conditions)
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Neurology (disorders of the nervous system)
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Neuropsychology (the relationship of the brain and behavior and how they impact each other)
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Neurosurgery (surgical management of disorders of the nervous system)
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Nursing (holistic care for individuals across the lifespan, including education and health promotion)
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Nutrition care guidance on eating habits, weight management, and overall wellness through dietary planning)
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Occupational therapy (rehabilitative care focused on improving function in everyday activities to promote health, well-being, and independence)
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Orthopedic surgery (surgical management of disorders affecting the musculoskeletal system—the muscles, bones, joints, and their related structures)
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Orthotics (design, manufacture, and management of orthoses)
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Pediatric general surgery (surgical management of children)
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Pediatrics (children and their medical conditions)
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Physical medicine and rehabilitation (PM&R) (enhancing and restoring functional ability and quality of life among those with physical disabilities)
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Physical therapy (rehabilitative care to improve strength and functional mobility)
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Primary care (overall health including routine and preventive care)
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Psychology and psychotherapy (diagnosis and management of mental health conditions)
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Social work (offers social needs and services and provide connections to resources and support systems)
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Speech and language pathology, or language therapy (rehabilitative care focused on communication and swallowing conditions)
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Therapeutic recreation (use of recreational activities for promoting physical, emotional, and social well-being)
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Urology (diagnosis and management of conditions affecting the urinary system)
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Wound care (management of wounds)
Appendix 3
Epilepsy management
Epilepsy management is complex. Epilepsy may evolve over time as the individual gets older, so the evaluation of the condition and its management is ongoing. Since clinical expertise can vary, it is important to know that information about management in this book may be different to practice at different hospitals and treatment centers. Management is not “one size fits all”; it must be customized.
The main goal of epilepsy management is to prevent, reduce, or stop seizures. Some related Important terms to understand include:
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Seizure control: Effective epilepsy management that results in a decrease in frequency, severity, and/or duration of seizures.
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Seizure freedom: A set period without any seizures; the ultimate goal of epilepsy management.
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Remission: A state where an individual with epilepsy is seizure-free for at least six months.
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Resolved: A state where an individual with epilepsy has remained seizure-free for the last 10 years, with no antiseizure medications for the last 5 years, or the individual had an age-dependent epilepsy syndrome and is past the applicable age for this diagnosis (i.e., self-limited neonatal or infantile epilepsy syndromes).
Why manage epilepsy?
Management of epilepsy is important for the following reasons:
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To protect the brain from damage: Epileptic seizures may lead to damage of areas in the brain, especially when they are prolonged or uncontrolled.
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To protect organs and body systems from damage: Epileptic seizures (especially those with motor signs) may lead to injuries and lesions in various body organs (e.g., kidneys or liver), or body systems (e.g., cardiovascular or musculoskeletal systems).
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To prevent status epilepticus: This condition, in which seizures last more than five minutes or occur in close succession (one after the other, without a return to baseline), is life-threatening.
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To prevent SUDEP (sudden unexpected death in epilepsy): This rare complication of epilepsy is named to describe the death of an individual with epilepsy when no other cause of death can be found.
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To ensure safety and prevent injury: Individuals with epilepsy are at an increased risk of accidental injuries from falls, motor vehicle accidents, and accidents around water, fire, and in other activities.
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To improve quality of life: Seizure control correlates with the ability to participate fully in life, including social activities, physical activities, education, employment, driving, and independent living.
How is epilepsy managed?
The management of epilepsy generally includes:
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Pharmaceutical treatments, involving the use of antiseizure medications, either as monotherapy (one medication) or polytherapy (more than one medication).
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Non-pharmaceutical treatments, involving the ketogenic diet, neuromodulation (repetitive electrical discharges administered through a device), and epilepsy surgery
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Other medications or supplements, including vitamins or medical cannabis
Pharmaceutical treatments are generally tried first. However, some epilepsy syndromes and drug-resistant epilepsy are best managed with non-pharmaceutical treatments or other medications or supplements. Pharmaceutical treatments, non-pharmaceutical treatments, and other medications or supplements can be used with the same individual and at the same time.
Management options for epilepsy are shown in Table A3.1.
Table A3.1 Management options for epilepsy
Management
Description
Indications for use
Pharmaceutical treatments
Monotherapy
One antiseizure medication (may try a different medication if the first doesn’t work)
All types of epilepsy, generally tried first
Polytherapy
More than one antiseizure medication given at the same time (may try different combinations)
Non-pharmaceutical treatments
Ketogenic diet
Neuromodulation
Specialized diet with a very low amount of carbohydrates
Repetitive electrical discharges administered through a device (for the management of epilepsy, these devices are surgically implanted)
Used when polytherapy does not work, or when the epilepsy type, epilepsy cause, or epilepsy syndrome is more responsive to non-pharmaceutical management
All types of epilepsy, when monotherapy does not work
Neuromodulation
Surgery to areas of the brain where seizures are thought to start or spread to
Other medications and supplements
Medications
Medications other than antiseizure medication include:
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Immunotherapies (treatments that alter the immune system),
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Steroids (medications with anti-inflammatory properties)
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ACTH (a type of hormone therapy).
Used when the epilepsy type, epilepsy cause, or epilepsy syndrome is known to be responsive to a particular medication
Vitamins
More than one antiseizure medication given at the same time (may try different combinations)
All types of epilepsy, when monotherapy does not work
Medical cannabis
A pharmaceutical form of the cannabis plant.
Used in epilepsy types and epilepsy syndromes known to be responsive to cannabis
Appendix 4
Gait analysis
The precise elements of gait analysis vary slightly between institutions. Gait analysis at Gillette Children’s includes the following elements, described in more detail below:
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Medical history
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X-rays
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Parent-reported (or individual-reported) functional questionnaires
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Two-dimensional video
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A standardized physical examination
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3D computerized motion analysis
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Kinematics: 3D measurement of motion (movement)
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Kinetics: 3D measurement of forces and mechanisms that cause motion
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Electromyography (EMG): Measurement of the activity of muscles
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Pedobarography: Measurement of the pressure distribution under the feet
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Energy expenditure: Measurement of the energy used during walking
Preparing for a gait analysis: What to expect
A typical gait analysis takes about two and a half to three hours. However, it is broken down into the elements listed above, none of which takes very long. The longest portion is usually the physical examination.
For children, two and a half to three hours is a long time to remain cooperative, It’s important to make sure that they are is well rested and well fed before the appointment It’s helpful to have a snack or drink on hand in case they need it to keep up their energy.
The gait lab usually specifies the type of clothing the child should wear to the appointment; for example, loose-fitting shorts and a tank top for girls and just the shorts for boys. Any orthoses and walking aids (e.g., crutches, walkers, or canes) used should also be brought.
The gait lab has a special walkway about 20 meters long, with special plates in the floor over which the child walks. The plates measure the forces produced as they walk. There are also cameras around the room for the two-dimensional video recording.
The elements of gait analysis explained
The following details the different elements of gait analysis at Gillette Children’s and will be a useful guide to gait analysis at most centers. It may be helpful to refer to section 2.5 on typical walking as you read this.
Medical history
Medical history includes information on:
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Birth history
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Developmental milestones
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Any medical problems
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Surgical history
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Current physical therapy program
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Current medications
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Parent (or individual) report on functional walking at home, school, and in the community and other functional skills (e.g., climbing stairs, running, jumping)
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Any complaints of pain
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Any behavioral or learning issues
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Reason for referral to gait lab, including current surgical or treatment considerations
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Goals for treatment
Parent-reported (or individual-reported functional questionnaires)
The following questionnaires are completed:
To measure function in the community
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Gillette Functional Assessment Questionnaire (FAQ), Walking Ability and Higher-Level Functional Skills
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Pediatric Outcomes Data Collection Instrument (PODCI)
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Gait Outcomes Assessment List Questionnaire (GOAL)
To measure goals:
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Gait Outcomes Assessment List Questionnaire (GOAL).
(Details of these measurement tools are included in Appendix 1.)
Two-dimensional video
This visual record is useful for understanding an individual’s gait problems and for comparing gait before and after treatment. The information obtained by observing them walk complements the information obtained from the 3D computerized motion analysis equipment. It is useful to see how the individual walks without any of the marker equipment used in 3D computerized motion analysis. Any problems with posture and balance in standing and walking are also noted. See Figure A4.1.
Figure A4.1 Two dimensional kneeling
Standardized physical examination
The following are typically measured in the physical examination:
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Muscle strength
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Selective motor control
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Muscle tone
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Range of motion and contractures
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Bone deformity
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Fixed and mobile foot deformities
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Leg length
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Extensor lag (the difference between the active and passive knee extension)
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Ligament laxity (loose ligaments)
Gait laboratories usually have very standard protocols for completing each.
3D dimensional computerized motion analysis
3D computerized motion analysis provides a very detailed analysis of walking. The equipment for this is the same technology as that used for animation in the film and video game industries. The clinician, usually a physical therapist, applies small markers to the individual’ body at specific points. See Figure A4.2.
Figure A4.2 Applying markers for 3D computerized motion analysis.
As a person walks, many changes occur at different joints in the body and in different planes. In addition to the many changes that occur in normal walking, a person with CP has a combination of primary, secondary, and tertiary abnormalities that interfere with their walking. The speed of these changes as they walk is far greater than the speed at which the observer can process the changes. The cameras capture the markers at high speed (120 frames per second) and provide far more information than can be gleaned even with a very experienced eye or from watching slow-motion videotape (30 frames per second). The information from the cameras is synchronized with information from the force plates and muscle activity so that all the data is collected simultaneously.
The 3D computerized motion analysis output allows the staff to compare the person’s walking pattern with data collected from normal walking. Once the differences are identified a list of deviations drawn up.
The following describes how Gillette displays gait data; other gait laboratories may produce the data slightly differently.
a) Kinematics
Kinematics is the quantitative 3D measurement of motion (movement). Kinematics show what is happening at the trunk, pelvis, hip, knee, and ankle on both sides of the body and in all three planes of motion. The kinematic graphs show the position and pattern of each body segment at each point in the gait cycle. See Figure A4.3.
Figure A4.3 Kinematic graphs.
The horizontal axis of all graphs represents one full gait cycle. The shaded areas show the gait patterns of typically developing children. The area left of the vertical line represents when the foot is on the ground (stance phase, approximately 60 percent of the gait cycle); the area to the right represents when the foot is in the air (swing phase). The right lower limb is labeled green, and the left is labeled red. The graphs for both legs are synchronized.
The five rows of individual graphs represent the trunk, pelvis, hip, knee, and ankle, respectively. The three columns represent the three planes of motion. From left to right, they are:
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From the back or front: the coronal plane
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From the side: the sagittal plane
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From the top or bottom: the transverse plane
When looking at kinematics, the points to note are how well the person’s graphs (green and red) match normal gait (the shaded area). Does the shape (pattern and position) of the graph for each limb match normal gait? Is the timing of events within the gait cycle (e.g., stance and swing time) normal?
b) Kinetics
Kinetics help to explain the movement seen in the kinematic graphs. Kinetic data is obtained from the special plate data combined with kinematics. See Figure A4.4.
Figure A4.4 Kinetic graphs
The kinetic graphs show:
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First row: The person’s kinematic graphs at hip, knee, and ankle level (i.e., what is happening during the gait cycle). These are just included for reference.
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Second row: The moments generated at these joints during the gait cycle. Remember, a “moment” is a force (e.g., a muscle contraction) acting on a lever (the bone] about a joint, which produces movement.
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Third row: Power at these joints (a combination of how fast the joint is moving and the moment being produced) during the gait cycle.
The moment (second row) and power (third row) graphs provide information about the cause of gait abnormalities—the “why” that explains the “what” of the kinematics (first row).
Again, when looking at kinetics, the points to note are how well the person’s graphs (green and red) match normal gait (the shaded area). Does the shape (pattern and position) of the graph for each limb match normal gait? Is the timing of events within the gait cycle (e.g., stance and swing time) normal?
Kinetic graphs are generated in two planes—the coronal and sagittal planes. The graphs shown are from the sagittal plane.
Electromyography
Electromyography (EMG) is the measurement of the electrical activity of the muscles. It provides information about the amount of muscle activity. There are times in the gait cycle when a muscle is supposed to be working and times when it is supposed to be silent. A typical EMG graph is displayed in Figure A4.5.
Figure A4.5 EMG graph.
The graph of the person having gait analysis is compared with normal gait, which is shown in the colored bars underneath the graph: yellow when the muscle is silent and reddest when it is most active. The blue line shows when the muscle is contracting and when it is not. In the figure, the blue line displays that this muscle is not working correctly because it comes on earlier than it should at the beginning of the gait cycle and lasts a little longer. Also, the muscle is active at the end of the gait cycle when it should be silent.
In addition to looking at activity patterns in individual muscles, activity patterns between muscles are also assessed Some groups of muscles are expected to work together, while others are expected to work opposite each other. While one of the agonist-antagonist pair (addressed in section 2.4) is working, the other is expected to be silent. (The gastrocnemius and the tibialis anterior are an example of an agonist-antagonist pair.)
Information from EMG is used to look for signs of spasticity—whether muscles are turning on and off at appropriate times. It also checks if the muscles are working all together or all on or off at the same time—signs of patterned movement that indicate poor selective motor control. The EMG graphs are studied in conjunction with the kinematic and kinetic graphs.
Pedobarography
Pedobarography is the study of pressures underneath the foot. The person walks across a special mat that senses the pattern and distribution (high or low) of pressure under the feet. It is dynamic because it captures this information while the person is walking. (This technology is used in some sports shops when selling running shoes.) See Figure A4.6.
Figure A4.6 Testing foot pressure.
Figure A4.7 shows plantar pressure graphs of a person’s left and right sides. The colors indicate the magnitude of the pressure: red indicates high pressure and blue indicates low pressure. In gait analysis, any deviations from the normal pattern distribution of pressure under the feet are noted. In this example, the person bears weight only on the ball of their right foot.
Figure A4.7 Plantar pressure graphs of left and right sides.
Energy expenditure
As a result of the deviations in gait, people with CP frequently walk in a manner that is less energy efficient than normal walking. An energy expenditure test provides an objective measure of a person’s energy efficiency during walking.
The person wears a mask that covers their nose and mouth, and the equipment measures the amount of oxygen they inhale and carbon dioxide they exhale. First measured is resting energy expenditure, with the person sitting for 10 minutes. (See figure A4.8.) They then walk for six minutes to measure their movement energy expenditure.
Figure A4.8 Measuring energy expenditure
In labs that do not have this equipment, a timed walk test may be conducted as an indirect indicator of the energy efficiency of walking.
Appendix 5
Transfer Aids and Vehicle Transportation
Transfer Aids
A transfer aid is a form of specialized equipment used to safely transfer an individual from one place to another; for example, moving from their bed to their wheelchair. As children grow, these transfers can become increasingly physically challenging, putting the person assisting and the individual transferring at higher risk of injury. Parents of children who need assistance for transfers have a higher incidence of low back pain than parents of children who can transfer independently.* Specialized equipment may help ease the physical challenge of transfers for parents while enabling the child to safely transfer between settings.
Common transfer aids include but are not limited to:
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Manual hydraulic lift: A freestanding device with a manually operated pump that assists with transfers of the individual in an attached sling. The caregiver operates the pump to raise and lower the individual. The lift has wheels that allow the individual to be moved to the desired location.
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Electric lift: A freestanding electrically operated lift. By pressing buttons or switches, the individual in the attached sling is raised and lowered. As with the manual lift, it has wheels that allow the individual to be moved to the desired location.
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Ceiling lift: A ceiling-mounted motorized system with ceiling tracks for movement to desired locations in the home. It operates similar to a freestanding lift, with a sling for transport and a remote control for moving the individual to the desired location.
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While useful in some situations, lifts do have limitations and they will not work everywhere in the home. For instance, they do not allow for transferring into the bathtub or onto a couch unless there is enough space beneath it for the base of the lift to fit. Ceiling lifts are also limited to the spaces in the home where ceiling tracks are installed.
Vehicle transportation
As children get older, they eventually outgrow commercial child car and booster seats, which then may need to be replaced by specialized equipment to provide adequate safety and postural support. Medical car seats are specialized for this purpose and can accommodate more weight than commercial car seats can.
Transportation should be considered from an early stage as it is difficult to lift an older child into a car seat. In addition, a wheelchair may be too heavy or not possible to fold to put in the trunk. Wheelchair accessible vehicles are specifically designed to accommodate wheelchairs. These vehicles feature a lowered floor or ramp for easy wheelchair entry and exit, wheelchair tie-downs to secure the wheelchair in place, and a vehicle-mounted seat belt to ensure safety for the wheelchair user during transport.
* Ward M, Johnson C, Klein J, McGeary Farber J, Nolin W, Peterson MJ (2021). Orthotics and assistive devices. In: Murphy KP, McMahon MA, Houtrow AJ, editors, Pediatric Rehabilitation Principles and Practice. New York: Springer, pp 196–229.