Communication
The selection of an appropriate communication system depends on the disease and its stage of evolution. Nonelectronic communication systems, including the use of paper and pencil, letter and phrase boards, and direct selection techniques using eye movements, are easily accessible to many patients and often adequate enough for most situations. Some patients with SMA type I and ALS who become aphonic can learn to use a personal computer with keyboard emulator software to drive a voice synthesizer and possibly a printer (74). Simple blinking operates an eye switch activated by infrared limbus-pupil reflection; electro-ocular switches may also be used (75). The Eye Gaze System (LC Tech- nologies, Inc., Fairfax, VA) (Fig. 4) is an integrated system that provides access to the electronic devices in the patient's environment but requires careful positioning for accurate use.

Bach et al. (73) reported the experience of 22 patients with aphonia and ALS who were dependent on 24-hour ventilatory support for an average of 2.8 years while communicating with personal computer/voice synthesizer systems. Five patients used the system for over 5 years. One patient who used the technologic aids for 10 years earned a living by writing prose and poetry for a greeting card manufacturer. The ability to communicate had a positive effect on psychological outlook and longevity. In another study of patients with ALS managed by tracheostomyIPPV without mention of augmentative communication, mean longevity was only 11 months (76).

Fig. 4. Duchenne muscular dystrophy ventilator consumer using the Eye Gaze Electronic Communication and Environmental Control System. The gaze of the user's eye on the computer screen (right monitor) is monitored (left monitor) and triggers the computer.

Patients with functional forearm musculature who have severe shoulder weakness, such as those with facioscapulohumeral muscular dystrophy, benefit from scapular stabilization to the rib cage and scapula-fixing orthoses to prevent winging. These can permit arm abduction, and flexion to greater than 90°.

Mobility
Patients with good trunk stability and function, particularly those with poor endurance, benefit from a motorized scooter, with elevated seats to facilitate transfers, attached baskets for carrying objects, and ventilator trays. They can be readily disassembled into three or four pieces of maximal weight 40 pounds and placed into the trunk or back seat of an automobile. Adapted strollers are available for children who are unable or too young to propel a wheelchair and require positioning support; however, their complexity varies. Like wheelchairs, the bases can have an option that allows the parent to recline a child as necessary, and with some, the base can be quickly reversed to permit parents to conveniently reposition a child with seizures or who requires ventilatory support.

For maximal efficiency, wheelchairs should be of proper size and have accessories to increase the function and quality of life of the user. The value of the wheelchair to the physically challenged individual cannot be overstated. Wheelchair prescription is appropriate for long-distance travel, through shopping malls, and along areas of uneven terrain and at outdoor events for patients with poor endurance or standing balance but who can otherwise walk with a cane, a walker, or orthosis. The ideal wheelchair is a light folding type for ease of transfer in an automobile. A more substantial manual or a power wheelchair is indicated for patients with greater walking difficulties. The patient must be measured for proper fit, and coat and orthotic use must be considered when applicable. Most wheelchairs have an overall width of 24 to 28 inches. Wider, more heavily constructed chairs can be made for obese individuals. A seat belt should be used for those with poor sitting balance or weak trunk musculature.

Two types of lightweight wheelchairs are available, those with rigid and those with nonrigid frames. A rigid-framed chair hasa solid immovable base and an axis that connects one wheel to the other. They are suited for the active individuals who will use the chair on a variety of uneven terrains. Because of the solid axis, these frames provide more stability, a smoother ride, and are more durable. Such wheelchairs support more weight and accessories like ventilators and robot arms. A disadvantage of the rigid-frame wheelchair is that it cannot be folded and put into a car. However, the pin and lock configuration of "quick release" wheels permits rapid wheel removal to decrease the diameter of the chair inhalf for storage in the trunk of an automobile. Wheelchairs with a non-rigid frame have a crossbar connecting one side of the wheelchair to the other. They fold to decrease their width in half. The cross-frame, however, wears out with use, compromising the integrity and stability of the wheelchair. Nevertheless, wheelchair users may prefer a cross-frame wheelchair because of its convenience. Both options should be tried to determine the one most appropriate for the user's lifestyle and environmental restrictions.

Most individuals with neuromuscular conditions require adjustable removable leg rests with heel straps. Offset foot plates and heel loops are used for individuals with severe ankle and foot deformities. The feet are maintained flat on the plates to discourage further deformity. Elevating foot rests increase the turning radius of the wheelchair and are generally used in conjunction with a reclining back in the presence of lower extremity edema, postural hypotension, and pressure sores that cannot be managed effectively in other ways. Individuals with poor endurance may also require a reclining seat. The ability to recline with extended knees reduces the tendency to develop flexion contractures of the knees and hips and allows the user to rest more comfortably. A neck or head support may be necessary for individuals with advanced ALS, infantile SMA, and/or DMD. Neck rests with a forehead strap provide both lateral and anterior-posterior support.

The user has the option of having full-length wheelchair arms or desk arms. Desk arms are preferable because they permit the user to approach and use tables. Full-length arms may be useful to support a lapboard to facilitate the performance of school work, gainful employment, and recreational activities. Elevating arms are an option but are rarely required by users with neuromuscular weakness. However, the wheelchair arms must be removable to facilitate transfers.

Special seating modifications are required for individuals with weakness, instability, and deformity of the trunk, pelvis, or spine. The individual must be positioned and aligned properly to discourage pelvic obliquity. When bony prominences or pelvic obliquity are severe, a proper seating system can provide a stable surface while accommodating the deformities. The pelvis can be supported by a firm seat cushion supporting both ischia to keep the pelvis level and balanced. Several seating systems should be tried for comfort and function before one is prescribed. Commercially available seating systems include the Roho (Roho Inc., Belleville, IL), Jay (Jay Medical Ltd., Boulder, CO), and Avanti (Invacare, Inc., Elyria, OH) systems. Wedges, padded inserts, and lateral trunk supports are added as needed to maximize comfort and support. Seats can also be fully contoured for patients with severe deformity (77). Some seating systems permit users to vary their sitting positions by adjusting the upright sitting posture to increase head and upper extremity control and to reduce skin pressure and pressure ulcers. The "Tilt-in-Space" system (La Bac Systems, Inc.,Denver, CO) shifts the user and the wheelchair seat and back simultaneously to change the seating orientation and shift skin pressures. Users can operate the tilt themselves via a switch.

Motorized wheelchairs are essential for the independent mobility of many severely disabled individuals. The same considerations for standard wheelchairs apply to motorized wheelchairs. In addition, motorized wheelchairs have drive trains and operation systems that must be chosen to satisfy the needs of the particular patient. A front-wheel-drive wheelchair is more maneuverable inthe confined spaces of the home. A rear-wheel drive grips the ground better and is superior for outdoor use. When finger function is present, power wheelchairs can be operated by joy-stick controls. Otherwise, tongue, chin, and sip-and-puff controls are usually used. When neck, finger, and lip musculature are inadequate, any volitional muscle activity can be adapted to operate the chair (Fig. 5).

FIG. 5. A patient with Duchenne muscular dystrophy using a ventilator whose best functional movement is with his biq toe. He uses his toe to trigger his computer.

Although patients who are able to dnve standard wheelchairs are infrequently dependent on daytime ventilator use, motorized wheelchair operators often require daytime support. When it is estimated that daytime ventilatory assistance will be required within 3 years, any prescribed power or standard wheelchair should have the capability of carrying a ventilator tray and holding additional batteries and possibly a charger. Daytime ventilator support is conveniently provided by the delivery ofIPPV via a mouthpiece fixed onto wheelchair controls or, for joy-stick control users, fixed by a metal gooseneck clamp adjacent to the mouth (Fig. 6).

Other considerations when prescribing a motorized wheelchair include its style for personal preference adjustability of the control box for sensitivity of operation; programming chair speed, acceleration, and turning radius; noise; brake efficiency; tire treads and suspension system for smoothness of ride on flat unlevel surfaces or on grass; curb-jumping ability; battery recharger; durability; warranty; and price. Some motorized wheelchairs can stand the patient and can be operated while standing Patients and care providers must be trained to properly and safely operate the wheelchair to prevent accidents and injuries for both the user and pedestrians. Training includes lifting and lowering the wheelchair onto and off of curbs, steps, and ramps.

FIG. 6. Patient with muscular dystrophy whose tracheostomy tube was removed after 5 months of failed ventilator weaning attempts. She uses 24-hour mouthpiece intermittent positive pressure ventilation delivered during daytime hours via a mouthpiece held adjacent to the mouth by a metal gooseneck clamp.

CONCLUSION

The impact of progressive musculoskeletal impairment can become increasingly intrusive on the physical, emotional, and social functioning of affected patients. However, interventions in each stage of disability can optimize function and maintain continued community living despite severe physical disability. Physical medicine interventions therefore increase patient management options and optimize quality of life for patients with neuromuscular diseases. The stage of prolonged survival will be considered in Chapter 40.

REFERENCES

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2. Bach JR. Amyotrophic lateral sclerosis: communication status and e; survival with ventilatory support. Am J Phys Med Rehabil 1993-72-343-349.
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8. Sutherland DH, Olshen R, CooperL, et al. The pathomechanics of gait mUuchenne muscular dystrophy. Dev Med Child Neurol 1981;23:
9. Demos J. Early diagnosis and treatment of rapidly developing Duchenne de Boulogne type myopathy Am J Phys Med 1971-50-271-284
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12. Gardner-Medwin D. Clinical features and classification of the muscular dystrophies. Br Med Bull 1980;36:109-115.
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14. Rideau Y, Duport G, Delaubier A, Guillou C, Renardel-Irani A, Bach JK. barly treatment to preserve quality of locomotion for children with
Duchenne muscular dystrophy Semin Neurol 1995;15:9-17
15. Paul WD. Medical management of contractures in muscular dystrophy.

For remaining references please refer to Motor Disorders, edited by David S. Younger, MD, Lippincott Williams & Wilkins, 1999.

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