subject: Technology Transitions Of A Moderate/severely Disabled Student: Longitudinal Case Study [print this page] For most people who don't deal with a disability, the life cycle and daily routines of those who are afflicted with a severe disability are out of sight and mind. Caregivers and parents in the United States who have the responsibility of supporting those with a severe disability can be overwhelmed, lack training, resources, and vision on the opportunities that technology can offer for people with a disability.
What are the challenges of using and training a person with technology that has a severe disability? What can we expect over the life cycle of a person who has a debilitating condition and their access to technology?
The following longitudinal study takes a hard look at one student's experiences with technology in the past, present and what his prospects may be for the future. The report is based on firsthand experience and a review of records from this teacher's experiences and a review of the students files.
Past
James was diagnosed with Angleman Syndrome at 2-1/2. What is Angleman Syndrome?
. . . it is a rare neuro-genetic disorder named after a British pediatrician, Dr. Harry Angelman, who first described the syndrome in 1965. As is characterized by intellectual and developmental delay, speech impediment, sleep disturbance, unstable jerky gait, seizures, hand flapping movements, frequent laughter/smiling and usually a happy demeanor. As is a classic example of genetic imprinting caused by deletion or inactivation of critical genes on the maternally inherited chromosome 15. The sister syndrome is called Prader-Willi syndrome, and is caused by loss of paternal genes. An older, alternative term for AS, happy puppet syndrome, is generally considered pejorative and stigmatizing so it is no longer used, though it remains useful as a diagnostic heuristic.
Dr. Harry Angelman, a pediatrician working in Warrington (then in Lancashire) first reported three children with this condition in 1965. It was initially incorrectly presumed to be rare. In 1987, it was first noted that around half of the children with Angelman syndrome have a small piece of chromosome 15 missing (chromosome 15q partial deletion).
Features include:
* Feeding problems during in 75%
* Delay in sitting and walking
* Absent or little speech (not in all case)
* Receptive and non-verbal communication skills higher than verbal ones
* Poor attention span and hyperactivity
* Severe learning disabilities
* Epilepsy (80%) and an abnormal EEG
* Unusual movements (fine tremors, hand flapping, jerking movements)
* Affectionate nature and frequent laughter
* Wide-based stiff-legged gait, with tendency to hold arms up and flexed while walking.
* Below average head size, often with flattening at the back
* Subtle, but sometimes characteristic facial features (wide mouth, widely spaced teeth, prominent chin, tendency to tongue thrust)
* Poor sleeping pattern
* Strabismus (Squint - crossed eye/s) in 40%
* Scoliosis (abnormal curvature of the spine) in 10%
* Increased sensitivity to heat
* Attraction to/fascination with water
James developed symptomatic generalized epilepsy and severe developmental delay. His seizures were generalized/ tonic/clonic. His seizures typically were observed as isolated of 2-3 minute durations and he could go 2-3 months without having one. He was routinely put on medications to try and control the seizures that included Depakote 275 mg twice a day, Keppra twice a day, Topomax 100 mg twice a day, and Risperdal 0.5 mg twice a day. In the case of severe attacks, he was to be given Lorazepam.
James received services from his public school experiences that included physical therapy. His goals were to demonstrate improved postural stability and motor control for classroom mobility and safety. Upon review of his academic file, it appears he was given several opportunities to access technology which consisted of adaptive technology buttons that allowed him to.
According to his parents, he was very active until recently and was able to crawl and walk with minimal assistance. His parents are divorced and James was transitioned to a group home two years ago.
Present
James is presently 15 years old and has been attending a Special Day Class at a local High school for the past year where the ratio is 2:1. Over the Christmas break, he had surgery to place a metal rod next to his spine to prevent deterioration of his spinal cord and posture. He is spending time in his group home recovering and is expected to return in 3-4 weeks. Complete recovery is expected to take three to four months.
James routine before his operation was to come to class in his wheel chair. He would be checked and changed if needed. Vocalizations of discomfort or pleasure were distinguishable. Typical discomfort seemed to come from being in his chair for extended periods of time or from being constipated. After being placed on the floor on a mat or in a reclining chair, vocalizations would diminish or be replaced with laughter.
James has demonstrated the ability to interact with others. He will reach out and play with long hair if within reach, or reach out to grab someone. He also seems to enjoy playing with adaptive buttons that allow him to listen to music or tapes, but he seems unable to maintain consistent pressure; he enjoys hitting the button off and on.
Future
Angelman Syndrome is not an illness, but a genetic condition, there is no currently available cure for AS. The epilepsy can be controlled by the use of one or more types of anticonvulsant medications. However, there are difficulties in ascertaining the levels and types of anticonvulsant medications needed to establish control, because AS is usually associated with having multiple varieties of seizures, rather than just the one as is normal cases of epilepsy.
Many families use melatonin to promote sleep in a condition which often affects sleep patterns. Many individuals with Angelman Syndrome sleep for a maximum of 5 hours at any one time. Mild laxatives are also used frequently to encourage regular bowel movements and early intervention with physiotherapy is important to encourage joint mobility and prevent stiffening of the joints. Occupational therapy, speech therapy, hydrotherapy and music therapy are also used in the management of this condition.
James will remain in the Special Day Class until he turns 22, in 2014, when he is transitioned out of the public school system; the local Regional Center will appoint a case manager who will step in to help him to be placed in a day activity program that will continue to interact, feed, and care for him.
Implications for Technology
Ideally, it would be nice if a cure was found to reverse or prevent the genetic condition of AS, but saving that all that can be done is to make James as comfortable as possible as he matures in life. Individuals with AS are expected to have a normal life span (Sprowles, 1985). Technology can play a part in the quality of James life by providing him with a means to interact with others and give him opportunities to exercise control over medium he would not otherwise have.
However, technology is only as effective as the people around James who help to support him and provide him with the opportunities to use the technology. In class, we provide James with opportunities to push buttons to communicate, control and to react. If not offered opportunities each day, James soon forgets about his abilities to control and his behavior turns inward and seems to become more vegetative.
Efforts by the parents, family and circle of friends to advocate for James should be created to look out for James best interests and care. The difficulty in caring for James will be isolation, time and complacency. As his family grows older or pass on, his care may fall into the hands of those who do not know him or his abilities.
Low paid staff at resident group homes may not be trained in dealing with or emphasizing with individuals that have AS and fall prey to a "care for" scenario in which the individual is cared for, but not treated with respect or interacted with socially. However, with the development of friends and a program and routine designed to create as much social interaction as possible, James can have a life that is respectful and sensitive to his needs and disabilities.
