Occupational therapy intervention to inspire self-efficacy in a patient with spinal ataxia and visual disturbance

Abstract

We report a case of a patient with severe ataxia and visual disturbance due to vitamin E deficiency, whose self-efficacy was inspired by intervention with an appropriate occupational therapy activity. Before the handloom intervention, her severe neurological deficits decreased her activities of daily living (ADL) ability, which made her feel pessimistic and depressed. The use of a handloom, however, inspired her sense of accomplishment because she could perform the weft movement by using her residual physical function, thereby relieving her pessimistic attitude. This perception of capability motivated her to participate in further rehabilitation. Finally, her eager practice enhanced her ADL ability and quality of life (QOL). The result suggests that it is important to provide an appropriate occupational therapy activity that can inspire self-efficacy in patients with chronic refractory neurological disorders because the perception of capability can enhance the motivation to improve performance in general activities, ADL ability and QOL.

Background

Rehabilitation intervention for patients with motor dysfunction due to degenerative ataxia still remains difficult. Although the effectiveness of rehabilitation programmes for patients with ataxia who have serious disabilities in daily life has been reported,^1–5 rehabilitation studies on patients with ataxia as well as visual disturbance are few because of the difficulty in compensating ataxic symptoms without visual control. Therefore, data accumulation from effective rehabilitation programmes for patients with both conditions is needed.

Case presentation

The diagnostic process of this case of a 62-year-old woman was previously reported.⁶ Briefly, at the age of 48 years, she was diagnosed with ataxia with vitamin E deficiency (AVED), which is known to be caused by mutations in the α-tocopherol transfer protein gene.⁷

She was the sixth-born sibling in a family of six children. Three of her siblings experienced similar symptoms. She developed an unsteady gait at the age of 9 years and began having difficulty with delicate finger movements at the age of 13 years. At the age of 23 years, she abruptly developed visual disturbance caused by retinitis pigmentosa; since then she has been confined to a wheelchair.

After graduation from junior high school, she stayed at home without a job. After her two older sisters married, she lived with her mother, who was her main caregiver. Since the age of 37 years, she attended a rehabilitation programme once a week at our institute. She received training for standing using a standing device, forward crawl movement and walking using parallel bars. Until she lived at the assisted-living facility, she was able to crawl with minor assistance in her house. Her mother died when the patient was 49 years of age, and she began living at an assisted-living facility. Thereafter, her physical ability decreased, as no rehabilitation programme was provided. Her physical functions and abilities probably deteriorated because of disuse when she was in her late 50s. Two years later, she participated in our rehabilitation programme again; however, by that time, she had lost the ability to stand.

On physical examination, she exhibited ataxia, loss of sense of position and passive movement, glove-and-stocking type sensory disturbances, muscular hypotonia, bilateral Babinski's sign, and bilateral absent knee and ankle tendon reflexes. Her score on the revised Hasegawa Dementia Scale was 28/30 points. Her vision was at the counting fingers level (10 cm). She had dysuria (pollakiuria and impending incontinence) and, therefore, needed considerable help with toilet activities. She had difficulty remaining in the sitting position on the bed or in standing position with bar support because of severe truncal ataxia. Since she had difficulty in moving to a bed or toilet stool, she needed two people to assist her. Her score of activities of daily living (ADL) on the Functional Independence Measure (FIM)⁸ was 62/126 points. In addition, her score of self-efficacy on the Generalised Self-Efficacy Scale (GSES)⁹ was low, 4/16 points.

Investigations

Ophthalmoscopy showed bilateral changes typical of retinitis pigmentosa. Brain MRI revealed no cortical or cerebellar atrophy and no ventricular dilation.

Treatment

The disabled-accessible handloom ‘SAORI’¹⁰ was used as part of the patient's rehabilitation in order to motivate her through craft activities that she could perform by herself (figure 1). SAORI activity requires the movement of the lower limbs to lift the warp thread and maintain a stable sitting posture. The workflow also demands movement coordination between the upper and lower limbs to put a weft through the warp threads and to tighten the weft. We selected SAORI weaving as the patient's intervention because we believed that craft activities that she could perform by herself would motivate her, as she was often pessimistic when others enjoyed craft production. Although handloom activity needs movement coordination between the upper and lower limbs, activity to pass a weft through the warp threads and tighten the weft seemed to be appropriate for her because she could use her residual functional movements for it. As expected, she could perform weft movements by herself using the residual physical function with oral instructions and partial assistance from a single staff member.

Figure 1.

SAORI handloom activity. SAORI handloom activity requires the movement of the lower limbs to lift the warp thread and maintain a stable sitting posture. The workflow also demands movement coordination between the upper and lower limbs to put a weft through the warp threads and tighten the weft.

Outcome and follow-up

The patient's rehabilitation time was occupied by the handloom activity. Although she could weave only 3–5 cm of cloth for 1 h per day, the weaving activity stimulated her to imagine the products made of the cloth she had woven. She checked her daily outcome with her residual touch sense of the hand and always asked the staff member ‘How much cloth could I weave today?’ The handloom activity brought her a sense of accomplishment and made her feel happy. However, she was still unwilling to perform other physical and ADL trainings.

Six months later, she said, ‘I want to train my physical ability to be able to weave faster.’ Her motivation prompted her to exercise her lower limbs, which were required to lift the warp thread of the handloom. Furthermore, she started training by sitting on the edge of the bed because the handloom had to be operated in a stable sitting posture. Three months after the training, she was able to sit on the edge of the bed for 15 min, decreasing the need for staff assistance during her weaving. Finally, her self-enhancement motivation further prompted her to perform ADL training to move between the bed and the wheelchair, and to sit up from a supine position. Her motion on the bed extended, and she spent more time in physical and ADL trainings. At around 18 months after the handloom intervention, she was able to move more skilfully between the bed and the wheelchair, and to sit up from the supine position smoothly by herself. This self-accomplishment brought her great fulfilment.

Recently, she acquired the skill of standing up. She could maintain a standing position using a bar with verbal support to modify her tilt. Under observation, she could move from the wheelchair to the bed by herself by using a bar for assistance and a transfer board. While moving from the wheelchair to the toilet bowl, she now needed assistance from only a single person. Her FIM score increased slightly to 65 from 62 points due to her improved transfer ability 24 months after the handloom intervention (table 1). With the increase in ADL ability, her pessimistic expression disappeared. She showed an active attitude about the improvement of her physical functions and ADL ability. Finally her GSES score increased to 11 from 4 points due to her improved scores on activeness in behaviour and anxiety about failure 24 months after the handloom intervention (table 2).

Table 1.

Improvement of Functional Independence Measure (FIM) scores after the intervention of the SAORI handloom

FIM score before and after the intervention
Activity	Before	After
Self-care
Eating	4	4
Grooming	5	5
Bathing	1	1
Dressing—upper body	1	1
Dressing—lower body	1	1
Toileting	1	1
Sphincter control
Bladder management	5	5
Bowel management	5	5
Transfers
Bed, chair, wheelchair	1	3
Toilet	1	2
Tub, shower	1	1
Locomotion
Walk/wheelchair	3	3
Stairs	1	1
Communication
Comprehension	7	7
Expression	6	6
Social cognition
Social interaction	6	6
Problem solving	6	6
Memory	7	7
Total (126)	62	65

Table 2.

Improvement of General Self-Efficacy Scale (GSES) scores after the intervention of the SAORI handloom

GSES score before and after the intervention
	Before	After
Factor I (activeness in behaviour)	2	5
Factor II (anxiety about failure)	1	5
Factor III (social locus of ability)	1	1
Total (16)	4	11

Discussion

AVED is characterised by ataxic symptoms caused by spinal cord posterior column defects similar to Friedreich's ataxia. Usually, these types of ataxic symptoms can be compensated for by visual control. However, the present case was complicated with severe visual impairment, as determined based on visual acuity and visual field, because of progressive retinitis pigmentosa, which is often associated with vitamin E deficiency^{11 12} as vitamin E plays an important role in the prevention of lipid oxidation in the retina. Therefore, the patient could not compensate for the lack of deep sensation through visual control. In addition, her severe disturbances of superficial sensation limited her ADL ability.

Before the handloom intervention, because her cognition was not impaired, the patient in this case felt pessimistic and depressed because of her disease condition. She was excessively pessimistic when she saw that other patients were happy with their completed crafts. In fact, she often said that there was nothing she could do when other patients were happily involved in crafts. Her ADL were limited due to her severe disability, including impaired static reflexes, ataxia, and sensory and visual disturbances. She needed considerable assistance to move. However, due to a lack of motivation, she refused to perform exercises to train her physical functions and ADL.

The handloom intervention changed her attitude from not being able to do anything to being able to perform the task at hand. We presume that the reason why she changed her attitude from being pessimistic to being vigorously involved in loom use was the sense of accomplishment with her handloom work that she performed by using her residual physical functions. The use of the handloom succeeded in making her aware of her residual touch sensation. The joy of accomplishment seemed to encourage and motivate her to engage in more rehabilitation programmes. This motivation prompted her to receive further sitting exercises and training to increase the strength of the lower limb muscles, which are needed for better handloom work. Her acquired ability of sitting on the edge of the bed made her conscious of the ADL training outcomes. She repeatedly practiced ADL motions that she was previously unable to complete by herself and, finally, she achieved success in one task after another.

In the present case, handloom intervention triggered the patient to perceive her capability of successfully performing a behaviour. This perception of capability, which is thought to be related to self-efficacy,¹³ led to the further accumulation of successful experiences such as physical training and ADL motions. As a result, less physical assistance was required. The self-efficacy theory was proposed by Bandura.¹³ His research has confirmed the relationship between self-efficacy and behavioural change; that is, performance mastery experiences produce higher, stronger and more generalised expectations of personal efficacy.¹⁴ We assessed the patient's self-efficacy before and after the intervention by the GSES, which has been widely used as a reliable and valid questionnaire of general self-efficacy in Japan.^{9 15 16} The GSES scores demonstrated that the activeness in behaviour was brought to her and her anxiety about failure was reduced after the handloom intervention.

Self-efficacy seems to act as an important mediator in the association between disability, and physical and quality of life (QOL) outcomes. Determining and testing an appropriate intervention activity that can inspire the perception of capability and self-efficacy in patients with refractive neurodegenerative diseases is important in order to enhance motivation to improve general activities, ADL ability and QOL.

Learning points.

• Handloom intervention caused this patient with spinal ataxia and visual disturbances to gain perception of her capability of successfully performing a behaviour and self-efficacy.

• Self-efficacy can bring patients self-enhanced motivation to improve their general activities and activities of daily living (ADL) ability.

• It is important to provide an appropriate activity to inspire self-efficacy in patients with chronic refractory neurological disorders in order to enhance their motivation to improve ADL and quality of life.