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April 2002


Craig Gets Mobile!

By Ginny Paleg, MPT, and Janice Fisher, MS, PT, ATP


Craig, a 6-year-old with osteogenesis imperfecta, is assessed for power mobility options. This power chair comes apart easily to fit in the trunk of a car.
The Hospital for Sick Children in Washington, DC, helps a child with osteogenesis imperfecta move independently.

Craig is 6 years old and has type II-III osteogenesis imperfecta (OI). Craig's mother came to the Hospital for Sick Children, Washington, DC, with three main goals: She wanted Craig to be assessed for power mobility options; she wanted to explore ways for Craig to move and exercise; and she wanted a safe way for Craig to sit on the floor and play with his four brothers. After 6 months of treatment, his successes included swimming, learning to drive a power wheelchair, and being able to sit on the floor independently for the first time. Following is how the team at the Hospital for Sick Children accomplished these goals.

OVERVIEW OF OI
Children born with OI can present with deformity, fractures, and short stature. These children produce faulty collagen, the white fibrous protein that forms the framework for bones, tendons, and ligaments. The skeletons of severely affected children are so fragile that parents and caregivers have been known to accidentally break a child's leg while changing a diaper. There is no known cure for OI. Treatment may consist of inserting metal rods into the largest bones to reinforce them, or wearing rigid plastic braces to protect fragile bones and decrease deformity.

It is an inherited disorder of connective tissue. Overall, OI effects roughly one per 10,000 individuals. Signs and symptoms are fragile bones, short stature, scoliosis, defective dentinogenesis of baby or permanent teeth or both, middle ear deafness, laxity of ligaments, and blue sclerae (whites of the eyes) and tympanic membrane. Patients may have misshapen skulls with a wide intertemporal measurement and small triangular faces. Dentinogenesis imperfecta results in soft, translucent, and brownish teeth. Laxity of ligaments results in hypermobile joints and increased incidence of joint dislocation. The most widely accepted method of categorizing the disorder is the Sillence classification based on clinical, genetic, and radiographic parameters. This has a numeric listing of types I-IV.

Type I is the most common and mildest form of OI. The bones are predisposed to fracture although most fractures occur before puberty. These children present with normal or near-normal stature, loose joints, and low muscle tone. Their sclerae usually have a blue, purple, or gray tint and their faces are triangular. Children with Type I have a tendency toward spinal curvature and hearing loss beginning in their early 20s or 30s.

Type II is the most severe form, frequently causing death at (or shortly after) birth due to respiratory problems. In recent years, some children with Type II have lived into young adulthood. These infants present with numerous fractures and severe bone deformity, small stature, and undeveloped lungs. In addition, they present with deafness, dark blue sclerae, concertina femur, and beaded ribs.

In Type III, the bones fracture easily. A fracture is often present at birth and x-rays may reveal healed fractures that occurred before birth. The children present with short stature, sclerae that have a blue, purple, or gray tint, and loose joints. They also display poor muscle development in arms and legs, barrel-shaped rib cage, triangular face, scoliosis, and respiratory problems. Severe bone deformities are often exhibited, as well as brittle teeth and hearing loss. In Type III, the collagen is improperly formed. The molecular basis for this type of OI has not been fully characterized as it has been for the other types.

Type IV is between types I and III in severity. Bones fracture easily, most before puberty, and these children are shorter than average. Clinically, they present with normal-color sclerae and have mild to moderate bone deformity. In this population, there is a tendency toward scoliosis, barrel-shaped rib cage, and triangular face. These children may also have brittle teeth or hearing loss.

Postural Assessment and Seating System Solutions
Craig was unable to ambulate or propel a manual wheelchair and had always been dependent for all his mobility. We wanted to help him make choices about where he wanted to go, when he wanted to go there. We wanted to try power mobility in hope that it would achieve this goal. Prior to actual power mobility trials, we needed to optimize Craig's seating alignment so that he could use his point of access (his hand) as functionally as possible to operate the wheelchair.

Craig had a simple mat evaluation before beginning his power wheelchair trials. His mat evaluation demonstrated a number of structural deformities in the trunk and pelvis, as well as multiple contractures in his upper and lower extremities. All of these deformities were "fixed" in nature and could not be corrected with manual or structural input. We had a trial wheelchair brought in by an equipment vendor; however, the seating system was very basic and did not accommodate Craig's small size or structural needs. We solved this problem by removing Craig's own seating system from his manual mobility system and mounting it on the trial wheelchair. By using Craig's own seating system, we were able to align his seating posture and meet his structural needs in order to maximize his function during the power wheelchair trials.

Power Wheelchair Access and Mounting Solutions

We wanted to try operating the wheelchair using Craig's upper extremity but Craig demonstrated limited movement in his upper extremities, particularly in his shoulders. He demonstrated more consistent motor responses distally, even though movement was limited in range and strength. First, we tried a common input device, the proportional joystick. Craig was able to access this device, but it was difficult for him, and he fatigued quickly. Craig could not use the joystick and perform consistently for any length of time.

We decided to try specialty electronics and something less conventional. We spoke with our product vendor and borrowed a dynamic finger steering device. This type of control has a box with a hole in it that uses capacitance to trigger the voltage to power the chair. Craig stuck his finger in the hole and moved it in the box. An inanimate object with no electrical charge such as a pencil would have no impact on the power forces of the chair. The chair responds spatially and proportionally to the user's finger movements in the circle. When Craig moves his finger to the top of the circle, the chair moves forward and when he moves it further to the top, the chair moves faster. Right corresponds with right, left with left. This type of control can also be programmed to be more sensitive and responsive to the smallest amount of movement and for other parameters such as acceleration and deceleration. We mounted this custom-fabricated device on a piece of Orthoplast close to Craig's side and at an angle. This allowed easy access to the control for Craig to use with his index finger. He grasped the concept of this control both cognitively and motorically, and soon, Craig was taking himself where he wanted to go.

Power Wheelchair Decision-Making
We had determined Craig's special needs in a seating system. We had also clearly determined our access site and the best control interface for operation: the dynamic finger steering device. After several trials, it was also clear that Craig had the potential to achieve our goal of being an independent power wheelchair user. Now, we needed to determine some specifics of the power base that we were going to choose. Craig lives in a small apartment and needs a small base with a tight turning radius. We chose the mid-wheel drive, mainly because it can turn in very tight spaces. Some other features that are beneficial with mid-wheel drive are its ability to handle obstacles and its very easy steering. We also looked for a system that could come apart easily and fit into his mother's car. Craig is one of five brothers, so there was not a lot of room left for a power chair. We chose a power base that came apart easily (no tools) into five pieces that all weighed less than 35 pounds.

Next, we tackled Craig's desire to be on the floor with his four rambunctious brothers.

Long Leg Sitter
Therapists at the Hospital for Sick Children prefer to get children out of their wheelchairs to experience other positions. We also like them to be able to experience the world from supportive and therapeutic places other than their wheelchairs. The long leg sitter is a therapeutic seating alternative that we frequently use. We make them out of inexpensive foam, and we individually customize the devices to each client's needs. Our long leg sitter proves to be therapeutic in numerous ways. The long leg sitting posture gives a prolonged stretch to the hamstring muscles over both the hip and the knee joints. The long leg sitter allows Craig to sit independently, achieve midline orientation, and frees up his hands to do other activities. The sitter encases Craig with springy foam, giving him tactile and proprioceptive input to clue him into his "body in space." Finally, the sitter allows Craig to sit supported on the floor, a place where his brothers spend a lot of time playing with toys and pets, or watching television.

STRENGTH AND ENDURANCE
Our final task was to come up with a fun activity to improve Craig's strength and endurance.

People with OI are encouraged to exercise as much as possible to promote muscle and bone strength that can help prevent fractures. Swimming or aquatic therapy is a common exercise choice for patients with OI, as water allows independent movement with little risk of fracture. For those who are able, walking, with or without mobility aids, is an excellent exercise. Individuals with OI should consult their physician and/or physical therapist to discuss appropriate and safe exercise.

Craig began a once weekly water-based exercise program. The therapy tank at the Hospital for Sick Children is 98°, and Craig found it more pleasant than his neighborhood pool. We positioned him in an inflatable ring purchased at a local toy store. In this ring, Craig was free to move his legs reciprocally, and to move his arms and hands to splash the water. He was safe under supervision and felt secure.

We played games like catch and ring-around-a-rosy to encourage Craig to kick his feet and splash with his hands. His respiratory rate and heart rate were monitored periodically. The session would end if Craig displayed nasal flaring or other signs of respiratory distress.

Craig's success indicates that aggressive physical therapy and rehabilitation have a major place in the overall care of infants and children with OI.



Ginny Paleg, MPT, is an NDT-certified pediatric physical therapist and Janice Fisher, MS, PT, ATP, is an NDT- and ATP-certified pediatric physical therapist, both at the Hospital for Sick Children in Washington, DC. They are part of the FOCUS team that offers a 5-day intensive mobility rehabilitation program for children who cannot stand and walk independently. Fisher is a clinical specialist and also runs the adaptive equipment clinic at the Hospital for Sick Children. They can be reached via email at: gpaleg@hospsc.org and jfisher@hospsc.org.

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