Assessing Functional Vision in young children with multiple disability and visual impairment

Presented in March, 2000

Assessing vision within a programme of early intervention for infants and children (0-7 years) who have additional or multiple disabilities and visual impairment (MDVI)

Compiled for ATCVI after discussion, feedback and contributions from members of this association by Marianna Buultjens, Coordinator of the Scottish Sensory Centre, Edinburgh.

Introduction

1 Primary Strategy of Process

2 Secondary Strategies

3 Case Studies

3.1 Case Study One: Six month old girl

3.2 Case Study Two: Girl aged 17 months

3.3 Case Study Three: "Peter", One year 10 months

3.4 Case Study Four: "Sally", Four years and 6 months

3.5 Case Study Five: ‘Gordon’, Five years

4 Appendices

Introduction

Parents and all who serve this group of young children wish to see a Vision Assessment Service Model which is efficient and effective. The criteria for such a service would include the following:

• provide the most comprehensive and appropriate service for the child’s needs;
• be simple and allow participation and local access for parents to the service;
• enable the passing on of useful information to those supporting the child’s development and learning;
• facilitate exchange of information between the health, social work and educational services;
• avoid duplication of effort.

Efficient transfer of information is vital, but difficult to achieve. Routes of communication for each VI Service could be identified, or established to ensure this efficiency.

Agreement on and specification of which assessments/tests should be carried out by which personnel would help in the following ways:

• cut down on clinical time spent on assessments which could be done by others;
• provide baseline information to help clinicians decide which investigation or further testing should be done;
• avoid the frustration of parents having to answer the same questions repeatedly and repeated administration of the same assessments.

The following model or process, has been drawn up through consultation with ophthalmologists and teachers of children with visual impairment. Each VI service or education authority, will need to ‘customise’ it to local needs through a process of discussion and negotiation with local parents and professionals.

Marianna Buultjens, August 1998

1 Primary Strategy of Process

1.1 Referral

Although methods of referral will vary from area to area and service to service, note should be taken of section 2 in Vision for the Future (1995) especially 2.2 Interagency identification and referral.

1.1.1 Local documents should incorporate information on useful contacts in other services.

1.2 Initial assessment

1.2.1 History

General background (from parents, Record of Needs, Assessment Centres, School Profiles, Ophthalmologists, Community Medicine, orthoptists, therapists, paediatricians, home visiting teachers, nursery staff).

Vision history (from parents and medical information which is often difficult to get, depending on where child lives and medical services responsible).

Communication history (method of communication and potential).

Current developmental level (information as for General Background, supplemented by own assessments).

Family background (information on extended family, any relevant medical history, ethnic and language background, issues such as non-accidental injury).

1.2.2 Gathering the above information, especially from parents, might be done by a simple questionnaire, eg, of questionnaire attached (4.2).

1.2.3 Examination of Threshold Vision - methods and materials (Importance of another pair of hands and eyes when assessing, also video camera).

Light perception (Vision for Doing Sections 9: Response to Light & 10; Responses to Reflected Light).

General Observations (Precision Vision Tests 19 & 20, Heidi and grating paddles).

Acuity (Lea - Gratings; Cardiff Cards; BUST/LH; Kay Cards).

Contrast Sensitivity (Precision Vision (PV) Contrast Sensitivity Tests; Heiding Heidi (PV); Vision for Doing Section 14).

Colour Vision (PV Quantitative Colour Vision test with large caps; home-made materials). Important to get information from paediatric neurologist if likelihood of colour vision being absent.

Dark adaptation (PV Cone adaptation test: Observation).

Visual Fields (Vision for Doing Sections 13a-c; structured and unstructured observation throughout the day at home or school; use of video to supplement this and to help note child’s strategies such as eccentric viewing and head postures).

Visual Sphere (distance at which child pays attention to visual stimulation).

NB For all the above, an approximate estimate of the speed of accomplishing each task is important. Number of seconds taken could be recorded. Statistics could be acquired over time. An initial grading of ‘normal’ or ‘slow’ could be given.

2. Secondary Strategies

For infants and young children with MDVI 0 - 7 years. This section needs to be developed individually and collaboratively to include further information needed and examples of intervention and methodology. The paper by Professor Gordon Dutton (attached) on the assessment of the performance of children with brain damage in terms of time, place and person, is a useful guide (4.4).

2.2 Type of Visual Deficit

2.2.1 Eye/Optic nerve Damage.
2.2.2 Brain damage.
2.2.3 Light sensitivity, eg, cone dystrophy.
2.2.4 Oculomotor dysfunction/accommodation difficulties.

2.3 Additional Disabilities

2.3.1 Impaired Hearing.
2.3.2 Motor disability, eg, Cerebral Palsy.
2.3.3 Communication and language difficulties.
2.3.4 Epilepsy.
2.3.5 Specific types of cerebral visual dysfunction, eg, blindsight, inability to recognise faces, inability to see moving objects or still objects.
2.3.6 Social and communication disorders, eg, autism.

The attached case studies exemplify various approaches to assessment and intervention.

2.4 Application of outcome of Vision Assessment. (See 3).

3. Case Studies

3.1 Case Study One: Six month old girl

Background
Six month old girl who is MDVI. Cortical visual impairment and cerebral palsy and developmental delay. She is the youngest of three children, living in a rural area of Scotland. Her parents contacted RNIB to find out about services available for their daughter. They had been told that their daughter would not be eligible for services as in nursery provision until she reached aged 2 years.

The parents were extremely concerned that their daughter needed to be in a stimulating environment from an early age - they know that Early Intervention is vital - on all fronts. RNIB put them in touch with Westerlea Early Education Unit in Edinburgh, the Bobath Centre in Glasgow and the Royal Blind School Nursery who have an early education group on Fridays. The parents have decided on Westerlea - the mother bringing the child herself.

They have local services of a physio once a fortnight, a pre-school home visiting teacher weekly and some input from the local VI teaching service. The pre-school visiting teacher is looking for support, and the VI teacher’s post has recently become vacant.

The parents have asked for assistance in assessing the vision of their daughter and gathering information about her functional and potential vision. The availability of the Prevision Vision materials could be most useful in this case, where there may be an opportunity to network with the local pre-school VI service and Westerlea. The parents being the key players. RNIB could facilitate this as the link.

prepared by Anne Taylor, Family Services Offices, RNIB

3.2 Case Study Two: Girl aged 17 months
(for the purpose of this study to be known as Tracy)

3.2.1 Referral
Tracy was referred at the age of 11 months by the local Community Medical Officer to the local Pre-school assessment team, (Prescat). The referral was within a few weeks passed to the Early Years Learning Support Team. Medical report was included in the referral. The case was allocated to one of the teachers who initially had difficulty making contact, as the house appeared to be empty when she made pre-arranged visits. There was then a 7 week gap before the next successful visit, after which the referral was made to the visual impairment specialist. The first joint visit (LS and VI teachers) took place 4 weeks later.

3.2.2 Initial Assessment

(i) History/General Background
Tracy was born after an emergency section, due to foetal distress. She was hospitalised for the 1st 2 months of life due to respiratory problems. Before discharge an ultrasound showed mild dilation of ventricles in brain. Mum was told to "expect problems".

When Tracy was aged 10 months, her mother was anxious regarding her development. Referral from Community Medical Officer gave a diagnosis of microcephaly and spastic quadriplegia, and gave a description of Tracy’s physical development and degree of spasticity. "In prone position she keeps her head up. When picked up by the armpits, her legs stiffen and scissor. Her arms go into spasm on and off, and hands fisted and held in prone position."

Tracy on medication 3 times daily for seizures. Seizures have now stopped, and she is much calmer generally.

Tracy was being seen by hospital based paediatrician on a 6 weekly basis at time of referral, (aged 11 months), but was not being visited by any community based therapists. Referral was also made at this time to Speech and Language Therapy Services for advice on feeding, as she could not eat anything other than milk and soup, choking on anything more solid. This service has now been commenced, and therapist is working on de-sensitising Tracy’s mouth and facial area around it. There has already been a slight degree of success.

Tracy now receives home based physiotherapy on a twice weekly basis, the programme concentrating at present on head control.

(ii) Vision History
Tracy was referred to Ophthalmology at age of 4 months for intermittent squint. At time of referral to Prescat, she still had not been seen and this was to be "chased up" by CMO. Appointment was given for 2 months later. (Tracy now aged 13 months).

She has now been seen by ophthalmology dept. on 2 occasions. On referral to this vision impairment specialist, a request was made to the department for information on Tracy’s vision, - after her second appointment, which was imminent at that time. A report was sent immediately thereafter - by the orthoptist who saw her on that occasion.

On the first visit Tracy was seen by a consultant ophthalmologist. He found no evidence of any visual response. Examination showed "fairly normal" fundi control and no need for spectacles. On the second visit, Mother had felt Tracy was seeing more, but orthoptist was unconvinced. It was felt that any inconsistent minimal response to ceiling lights may be due to involuntary convergence. She did, however feel that at times Tracy seemed to fixate on her Mum’s face. If results are still inconclusive in 3 months time, referral will be made to another hospital for electrodiagnostic tests.

(iii) Family background
Tracy is the first child of the parents. Father works away from home and Tracy is looked after by her Mother alone for most of the time. There is a very supportive extended family who live near. Maternal Grandfather is wheelchair bound.

3.2.3 Examination of Threshold Vision

(i) Initial Observation
Tracy moves her eyes around a lot. She seemed to occasionally focus on one’s face when addressing her, but on moving aside her gaze stayed static. She will turn her head towards sound, but there was no appearance of focusing on any rattles or soft toys, no matter how brightly coloured or boldly contrasting in colour.

(ii) Light Perception
Material used: Vision for Doing, Section 9 Learner’s Response to Light. Tracy responds consistently to sunlight, daylight, camera flash , room light being switched on/switched off. In daytime it was difficult to get the room dark enough to use a torch to full advantage, but she did occasionally fixate briefly on filtered torchlight. Mum reports that she will occasionally gaze at a table lamp at a distance of just over a metre. A fibre optic curtain was left to be used in darkness. In daylight Tracy consistently gazed at it for a few seconds at a time. When the effect was enhanced by placing hologram foil behind the fibres, she gazed for more than 30 seconds.

Material used: Vision for Doing, Section 10 Response to Reflected Light. For this we (LS teacher and VI teacher) started with a Christmas decoration - a faceted?! ball. There was a definite stilling from Tracy and a gaze at the ball which lasted a few seconds before she moved. This was at positions 1-6. Secondly we used a board 8ins by 8ins covered with silver diffraction foil. Tracy immediately gazed at this, and attention times were much longer. This was in positions 1-9. This has been a consistent response. The same response has been obtained from a tin with a 4 inch diameter covered in pink foil, but the attention time is shorter. When tester wore a tinsel wig there was a very definite focusing, and a definite smile on each occasion the head was shaken to move the tinsel.

(iii) Acuity
It does not appear at this point that Tracy is likely to have measurable visual acuity, but a trial of "Gratings" would be helpful, if available.

(iv) Visual Fields
Unstructured observation. Tracy appeared to be showing a more consistent response when board was to the left of mid-line. She also appeared to be focusing more when objects were at eye level. These observations were made by staff present, Mum and educational psychologist.

Vision for Doing Section 13a Responses in Visual Field (Upper half).
Using the Christmas ball, there were occasional brief stillings of gaze, but this will be better assessed using video.

Section 13b Responses in Visual Field (Lower half).
The response here appeared to be more consistent. Again, this will be studied using video. We will also continue to use other materials and settings.

At present the scoring for both halves would be "Aware".

(v) Visual Sphere
The distance at which Tracy is aware of light reflective material is around 30cm.

3.2.4 Secondary Strategies

(i) Type of Visual Deficit
There appears to be no damage to the eye or the optic nerve. The extent to which the brain is damaged, and in which area, is not yet known.

(ii) Additional Difficulties
Hearing has not yet been assessed, but there does not appear to be a hearing impairment.

(iii) Motor Disability Tracy has quadriplegia
She has frequent spasms in arms and legs. She does make some little movements when bells are attached to wrists, and smiles during this. She does not hold anything, but has just begun to move her fingers in a grasping movement when her hand is placed on crinkled soft foil.

(iv) Communication and Language Difficulties
Tracy vocalises a lot. Babbles are of repetitive sounds. She has just begun to make a "tutting" sound, which she will do in a conversation with her Mum.

(v) Epilepsy
Seizures continue to be controlled by medication.

(vi) It is not known if there is any type of cerebral visual dysfunction.

3.2.5 Applications of outcome of assessment to IEP

(i) Further Assessment
Responses to sound
Sense of touch
Sense of smell
Sense of taste
Most of this will be carried out by LS teacher, whose visits will alternate with VI support teacher, with constant liaison and one set of notes.

(ii) Continuing assessment of vision
Using Vision for Doing Sections 11 and 12 assess Tracy’s response to moving objects and eye or head movements
Using video recording, observe response in upper and lower visual fields.
Continue, with parents, to observe and note responses to visual stimuli in various settings and with a wide variety of materials.
Observe any eye contact when face is held very close to Tracy’s.
Seek more information after any further ophthalmological examinations.

(iii) Working With Parents
Mum particularly is keen to read any available information. Texts given to her so far - "Show Me What My Friends can See", "One Step at a Time". Keen to follow out suggestions.
Tape given to Mum of action songs to encourage body awareness.
Mum watched RNIB video, "The World in Our Hands 1" - "Our Baby is Blind".
Told about "The Little Room" Dad is making a version, as there is not one available in our area.
Mum daily coming up with ideas to stimulate Tracy.

(iv) Meanwhile
Introducing activities to raise level of awareness using suggestions from Vision for Doing and also "A Sensory Curriculum" by Flo Longhorn.

(v) Liaison with Speech and Language therapist; Physiotherapist; Paediatric OT; Health visitor; Case Psychologist.
prepared by Lorna Hall, VI Support Teacher.

3.3 Case Study Three: "Peter", One year 10 months

3.3.1 Referral

GP and health visitor. Peter has been in another area until age 1 year 8 months.

3.3.2 History

Congenital micropthalmy of right eye and anophthalmy of left. General developmental delay. No further information on vision or development available from previous health authority. Makes known what he does not want by crying. If frustrated, Peter bites his own arm and screws up his face. Smiles and laughs when happy. No speech or sign yet. May have a hearing loss but does respond to voice and recognises people and sounds. Current developmental level: delayed by several months. Child is not showing interest in environment sufficient for exploration. No attempt to crawl or walk. Uses a baby-walker to get about at home. Bottom shuffles elsewhere.

Self-help: beginning to use a spoon if it is loaded for him. Finger feeding. Spout cup. Wonderful sense of rhythm.

Family History: Mother’s second marriage. First marriage produced 3 children, of whom 2 died of cystic fibrosis in infancy. Second marriage produced 3 children of whom Peter is the second. Current family on "At risk" register. Previous incidence of abuse. Mother very supportive and loving toward children. No known history of visual impairment in family before now. Home visits should not be undertaken alone. Arrange to meet parents in Centre because father can be very resistant to what he sees as interference.

3.3.3 Examination of Threshold Vision

Light perception. Enjoys playing with toys that light up and flash. Holds them, very close to his eyes, using his own hands. Enjoys watching reflected light and is attracted to shiny surfaces. Always moves towards the French window. Flicks his fingers in front of his eyes.

Acuity: Prevision Vision Gratings: preliminary trial, shows Peter interested in 0.5 cpm at 40 metres. Intention is to repeat and confirm this.

Contrast sensitivity: Precision Vision Hiding Heidi will be used. No information on colour vision. The paediatric neurologist will be consulted.

Dark adaptation: observation only. Peter is able to distinguish objects in dim light, but is instantly happier when the light is switched on.

Visual field: very limited. Peter’s eye is turned towards the inner corner. In order to obtain a better view, he often pokes his finger into his eye to manually change the position of the eyeball

3.3.4 Secondary Strategies

(i) Type of visual deficit
Absence of left eye. Very small right eye.
There has not been a neurological examination.
Light sensitivity is not apparent.

(ii) Additional Disabilities
Impaired hearing mainly due to repeated glue ear infections. Grommets are to be fitted.
No apparent motor disability.
Delayed language. Understands simple one-word utterances. Is beginning to babble himself and has a vocabulary of about 6 words, eg, hello, ta-ta.
No epilepsy.
No apparent specific visual dysfunction.
Good relationships with carers and family.

3.3.5 Other services involved

Physiotherapist, occupational therapist, Assessment Centre staff, education psychologist (soon), GP, ophthalmologist.

3.3.6 Application of above information

Further assessment of vision: Gratings, Hiding Heidi, faces.
Stimulation of current vision by use of lights, shiny play objects, eg, tin covered with reflective paper (Good for banging, rolling, opening to discover shiny and noisy contents, putting in and out, closing, banging, etc).

Use of this vision in making cup, plate, etc, visible for Peter. Use of sound and vision to encourage standing and walking.

Programme of environmental awareness raising; variety of textures, sounds, involvement of Peter in everyday activities like opening and shutting doors, picking things up, answering the telephone, etc.

Co-operation with the OT and physiotherapist to stimulate overall development.

Co-operation with speech and language therapists to use sensory stimulation to encourage language. Use his sense of rhythm to help with speech and general learning. Peter loves music, which can then be used as a vehicle for further learning.

Alison Duthie, Visiting Teacher for VI

3.4 Case Study Four: "Sally", Four years and 6 months

3.4.1 Referral

Sally is four years and six months old. She has attended the nursery unit of a school for children with complex learning difficulties for two years on a part time basis. She was referred by the head teacher of her school.

3.4.2 History

Sally was seen by Dr D two years ago. Sally’s Mum reports that he said that her eyes were normal and the problem was with the brain processing the information. He believed that Sally could see light but "time would tell" as to whether Sally would develop her vision further. I have not had access to this report but hopefully I will in the near future. Sally has profound learning difficulties, cerebral palsy (spastic quadriplegia) and epilepsy.

3.4.3 Examination of Threshold of Vision

I used "Vision for Doing" to assess Sally’s vision. I began by looking at the other senses and found that Sally’s level of response was mainly at an awareness level and sometimes at an attention level. It was difficult to know exactly what constituted a response, for school staff as well as for me, as Sally has very limited gross movements and makes very frequent and apparently random fine movements of her head, arms, mouth and especially eyes. Consequently I decided to use the following method suggested by "Vision for Doing".

First I took a sample of Sally’s exact movements every 10 seconds for between two and five minutes with no sensory stimuli present. Secondly I repeated the exercise with a visual stimuli present. I could then compare Sally’s behaviour and conclude that any significant change in her behaviour when a visual stimulus present must mean she could see it. No difference would mean that she could not. Using this method I could that Sally’s slight head, mouth, arm movements and most noticeably eye movements, were significantly less when a torch was shining, particularly with a red filter. This was also the case with torchlight shining on reflective material, and torchlight shining on fluorescent yellow and pink material (2 graphs showing samples of Sally’s movements attached). I found no significant difference to her behaviour with or without visual stimuli presented in normal lighting conditions. However I did not have the opportunity to observe Sally’s response to bright sunlight.

These are the recommendations that I made:

(i) Sally needs a sensory curriculum in which her responses to sound, smell, touch, taste and possibly a very small amount of residual vision can be developed. I would suggest a ‘sensory bank’ of sensory stimuli could be set up along the school lines discussed by Flo Longhorn in her book ‘A Sensory Curriculum for Very Special People’. The ‘Vision for Doing’ manual also has some very useful ideas about how to move Sally from one level of response to the next.

(ii) Sally’s responses in a dark room or multi-sensory room need to be closely observed. Sally makes almost constant very small movements of her head, arms, mouth or eyes. Staff need to look out for a ‘stilling’ response to visual stimuli, and for eye movements to decrease. Sally is not able to fixate on visual stimuli but may be keeping her eyes still in order to take in the information.

(iii) Similarly Sally’s responses to sunlight need to be closely observed. Does she shut her eyes or put her head down or turn towards it? Sally responded to coloured filters with torchlight and staff could try a range of coloured filters outside on a sunny day.

(iv) The RNIB booklet and video ‘Movement, Gesture and Sign’ would be relevant for staff to look at. Sally would benefit from the ‘Movement’ section ideas.

(v) I would recommend a ‘Little Room’ along the lines suggested by L Nielsen to encourage Sally to begin to reach out and explore her world, starting with a small confined environment.

(vii) Aromatherapy massage would increase Sally’s awareness of touch. Massage or gentle stroking with a variety of textures would also increase Sally’s awareness of her body.

(vii) Sally showed some response to very bright fluorescent objects highlighted by torchlight and if possible I would like to observe Sally’s response to fluorescent materials under ultra-violet light.

Louise France, Visiting Teacher for VI

3.5 Case Study Five: ‘Gordon’, Five years

Gordon aged 5 years at a special school for multi-disabled children aged 5-18.

3.5.1 Referral
The Headteacher of the school referred to the Visual Impairment Service for assessment because Gordon has a visual impairment and the TVI made regular visits to the school to work with the children. Gordon had recently joined the school from his local Nursery.

History: (Source School)
Microcephalic. Quadriplegic. Total care required in all areas. Appropriate postural support required at all times with regular change of position and use of aids to prevent/reduce swelling of his feet. Gordon’s health has improved considerably since he had an operation to correct gastric reflux. He eats well and is generally alert and happy. His attendance is good. Gordon is regularly seen by a Physiotherapist who advises the school on aids, programmes and problems. He does daily exercises and he especially relaxes in the hydrotherapy pool where he is able to move his arms and legs. His positions include the side lying board with vibrator, standing frame, long legged sitting chair, multi wedge and James Leckey chair.

Record
A Record of Need was opened for Gordon before he started school.

Eye condition: (Source School and Ophthalmologist)
Cortical Visual Impairment and Myopia.

Communication: (Source School)
Gordon expresses his happy or sad feeling and needs by eye, facial, body movements and some vocalisation. He enjoys a music and movement programme each morning and he can show pleasure and some anticipation.

Family Background: (Source School and Parents)
Gordon lives at home with Mum and Dad and two older siblings. His grandparents play an important role in this close knit, loving and caring family.

3.5.2 Initial Assessment

Gordon was assessed within the school in general and particularly in the black and white stimulation area and the multi sensory room. Assessment was carried out on an individual basis and also in small groups. Ideas for assessment were taken from Vision for Doing and books by L Nielsen and F Longhorn. Assessment tools were basically the resources of the school and the only vision test that I had available, Keeler Preferential Looking. Unfortunately I had no access to Lea Hyvärinen’s assessment materials.

3.5.3 Examination of Threshold Vision

Acuity. No specific details are known at this stage but acuity appeared to be 6/24 binocular without correction, using the Keeler test.

Contrast sensitivity. Does alert to light and much pleasure shown when in the black and white stimulation area.

Colour vision. Unknown but much pleasure was consistently shown when red or green were the main colours in the multi sensory room.

Dark adaptation. No suitable materials for testing.

Visual fields. Responses made, to the right and to the left.

3.5.4 Functional Vision

I am undergoing this stage of the assessment at present but initial observations showed that Gordon responds with eye, facial and head movements to varying visual stimuli. Responses are very slow and very often it is difficult to tell if he is focusing. Vocalisations are very limited and are generally made when he does not like something eg certain tastes from the sensory curriculum lessons. I hope to be able to assess Gordon more fully if I am able to have access to Lea Hyvärinen’s materials.

Christine Stones, Visiting Teacher for VI

4. Appendices

4.1(a) Reading

4.1 (b) Materials Available on Loan to Support Vision Assessment
Scottish Sensory Centre:
Two Light boxes
Seven sets of LH Cards
(The above donated by Dr Lea Hyvärinen to this initiative).
Heiding Heidi Contrast Sensitivity Cards: 1 set
Heidi Paddles: 1 set "Little Room" Lilli Nielsen (Structure only available - person borrowing would need to provide the items to be hung from frame.)
For reference in Centre "Ideebanken’. Folder with Lilli Nielsen’s ideas for sensory materials.

The above are available for loan for members of the Scottish Sensory Centre Library.

4.2 Eyesight Questionnaire. Professor G N Dutton

4.3 Assessment of Visually Impaired Infants. Dr Lea Hyvärinen

4.4 Visual Problems in Children with Brain Damage