Special Topics Paper 1 - For Merge

8/8/2019 Special Topics Paper 1 - For Merge

1/38

Special Topics - OTS 294

The Role of Assistive Technology in meeting Activities of Daily Livingof Adolescents with Upper limb Dysfunction arising from Failures of

formation

by Pamela Williams

1


2/38

Introduction

Upper limb congenital malformations are pretty rare but can result in severe limitation or

loss of function in all areas of occupation because the use of arms and hands are essential for

the performance of many activities. Children with impairment and disability, arising from upper

limb dysfunction may require the provision of aids, equipment and other accommodations which

can help them to successfully adapt and respond to meeting the demands of differing

environmental contexts which they are going to encounter as they develop and grow to maturity.

Assistive technology can be a valid means of helping to develop skills or maintain skills and can

facilitate greater levels of independence.

However, there appears to be little written about the lived experience of children with

congenital upper limb dysfunction and their utilization of assistive technology for meeting self-

care/personal activities of daily living. A key area of focus of this paper is to explore how

assistive technology is used by adolescents with congenital upper limb dysfunction, when they

are at an age where the desire for increasing autonomy, awareness of body image/self-identity

and need to assert their independence away from with primary caregivers are likely to emerge as

important factors during this transitional period. Questions being addressed include, Do

adolescents with upper limb dysfunction have ease of access to the right types of aids which

enable them to competently manage essential self-care routines (eg personal grooming tasks

such as washing, drying and brushing hair and dressing activities), if so what routes are followed

for obtaining these aids/assistive technology and how readily available are they? Do the assistive

aids currently available make a real qualitative difference to both the main service user and their

care givers (if applicable) in respect of factors such as time, effort and organization required for

carrying out self-care tasks, or do alternative strategies/techniques and problem-solving bridge

the gap in the absence of assistive technology which could make a difference in these areas?

2


3/38

What are the concerns of the primary caregivers (within families) in these situations and how are

issues of independence, during this period of transition for their child, managed?

Before these issues can be addressed, it is important to understand the pathogenesis of

congenital upper limb conditions and their course as these factors determine the extent to which

the functional anatomy and biomechanics of the upper limb are likely to be affected and greatly

influence future management and therapeutic interventions. Subsequently the next section of this

paper provides an overview of the literature on etiology and classification of congenital upper limb

deficiencies.

Etiology and Classification of Congenital Upper limb Deficiencies

Congenital anomalies are uncommon. They affect 1- 2 % of newborns. Of these

approximately 10% of children have upper extremity irregularities (Kozin, 2003). Normal fetal

development occurs in three stages (1) the pre-differentiation period (weeks 1 and 2), (2) the

embryonic period (weeks 3-8) and (3) the fetal period (weeks 9 term) (Van Heest, 1996). Eight

weeks after fertilization embryogenesis is complete and all limb structures - bones, ligaments,

joints and muscles are in place and undergoing rapid growth. The ongoing process of normal

skeletal ossification for the upper limb results in the formation of the long bones - clavicle,

humerus , radius and ulna the short bones - metacarpals, proximal, middle and distal

phalanges of the hand and flat bones the scapula and carpals. Primary centers of

ossification are present in all fetal long bones by week 12. Most congenital anomalies of the

upper extremity occur during the fetal phase of development (Kozin, 2003). Upper limb

deficiencies are defined as reduction defects of the humerus, radius, ulna, hand and phalanges.

(Ephraim et al, 2003).

Most frequently malformations of the hand and arm result from gene variation which give

rise to specific syndromes or with associated abnormalities of other organ systems, (Van Heest,

3


4/38

1996). Additionally, exposure to environmental teratogens or indeed gene- environmental

interaction may result in limb deficiencies (Ephraim et al, 2003). However, the causes of some

upper limb anomalies are not always known.

Morrissey and Weinstein (2001) note that the classification of congenital limb anomalies

has not been clearcut and most orthopedic clinicians tend to use a combination of systems to

categorize and to precisely describe these deficiencies . However, a widely adopted system is

that which was proposed by C.H Frantz and R. ORahilly in 1961 and augmented by A.B

Swanson in 1976. (Froster-Iskenius & Baird, 1990). This is also now augmented by a system of

classification developed by the International Standards Organization (ISO) and the International

Society for Prosthetics and Orthotics (ISPO). (Morrissey & Weinstein, 2001). However for ease

of reference this paper will use the descriptive terminology applied under the Frantz and O

Rahilly system . This system places upper limb malformations within seven categories. These

are as follows:

1) Failure of formation of parts -

2) Failure of differentiation

3) Duplication

4 ) Overgrowth

5) Undergrowth

6) Congenital Constriction band syndrome

7) Generalized skeletal abnormalities

1. Failure of formation of Parts:

Failure of formation describes upper arms, forearms, wrists, hands or fingers that do not

form completely or at all.

Transverse deficiencies, i.e. those which extend across the entire width of the limb,

linclude all naturally occurring congenital amputations which can apply to the shoulder, arm,

forearm, carpals metacarpals and phalanges . The most common transverse deficiency is the

4


5/38

unilateral below- elbow amputation. Van Heest (1996) notes that this generally lends itself well

to early fitting of a prosthesis . A child of between 6 12 months of age can be fitted with a

simple paddle prosthesis initially with more sophisticated prostheses being applied as the child

develops.

Longitudinal deficiencies cover the gamut of deformities ranging from phocomelia, where

the hand is attached to the trunk, to anomalies of the radius, ulnar and digits. Phocomelia is

uncommon and is usually associated with the drug thalidomide having been taken in the first

trimester of pregnancy. Surgery is not usually indicated in these cases and fitting of a prosthesis

can be difficult due to the extreme shortening of the limb. (Kozin, 2003). Deficiencies of the

radius most commonly result in partial to complete absence of this bone. The ulna in this

instance is often subject to growth retardation and bowing. The thumb can be absent in upto

85% of cases and remaining digits may be stiff. Hand function is reduced in these instances due

to associated loss of thumb and digit function as well as acute deviation at the wrist joint.

Bilateral incidence ranges from 40-60% in these cases. Unilateral deformities occur twice as

often on the right side as on the left and boys are affected more frequently than girls in a 3:2 ratio.

Radius deficiencies are commonly associated with named syndromes such as Holt- Oram

syndrome or Thrombocytopenia Absent Radius (TAR) syndrome. The complete absence of

skeletal parts of a limb is known as Amelia. (Froster-Iskenius & Baird, 1990)

2. Failure of Differentiation

Failure of differentiation, or separation, occurs when the basic unit is not fully formed.

Synostoses, (or the joining of two bones by the ossification of connecting tissues) of the radius

and ulna, carpus and metacarpals are examples of a lack of complete differentiation of parts in

the upper limb. Van Heest (1996) notes that Syndactyly, or fusion of two fingers, is the most

common congenital upper limb condition in the United States. Syndactyly can be successfully

treated via surgical intervention providing this conducted when the child is between 6-18 months

5


6/38

of age in order to avid tethering of skeletal growth and to facilitate subsequent normal digital

development (Van Heest, 1996)

3. Duplication

Duplication, or polydactyly most commonly involves an extra thumb or little finger and

central polydactyly is rare ( Morrissey & Weinstein, 2001). Polydactlyly of the little finger, without

complications, can usually be successfully treated with excision during the newborn phase with

normal hand function being preserved but more complex polydactylies may necessitate excision

and reconstruction. Because of the functional importance of the thumb preaxial polydactyly

(thumb duplication) surgery is not usually effected within the first year of birth as excision can

easily disrupt thenar musculature and damage collateral ligaments. Central polydactyly will either

entail leaving the digits conjoined, or attempting reconstruction to a five digit hand. However,

even with reconstruction the involved digit may be subject to poor alignment and is likely to be

smaller, weaker and stiffer. (Morrissey and Weinstein, 2001)

4. Overgrowth

A section of the hand or arm may be too large or too small affecting joint structure and

function. However overgrowth deformities may affect the entire limb, forearm, hand, or digits.

Most commonly, macrodactyly - non-hereditary enlargement of a digit - will result in seventy

percent of children having an adjacent digit affected. (Van Heest, 1996)

5. Undergrowth

Van Heest (1996) states that undergrowth, or hypoplasia denotes complete formation of

a part during the embryonic period of gestation but incomplete growth during the fetal period.

The whole limb or any of its parts may be affected and can also occur with longitudinal anomalies

of adjacent parts. The thumb is most commonly affected and hypoplasia in this instance can

range from minor diminution with full function to total absence. Hand surgeon consultation is

necessary to determine gradation of involvement and most appropriate procedures.

6


7/38

6. Congenital Constriction band syndrome

This results from tight bands of tissue around the arm, forearm, wrist or fingers. It can

range from being mild to severe. If severe, part of the hand may be lost with amputation arising

distal to the constricting band. Surgical interventions are recommended for all types of banding

except those which present as amputations. (Morrissey & Weinstein, 2001). If a secondary and

severe deformity is present such as gross distal lymphedema then interventions may occur when

a child is still neonate otherwise surgical interventions within first 18 months is appropriate. (Van

Heest, 1996)

7. Generalized skeletal abnormalities

This category covers a spectrum of malformations but includes congenital dislocations

such as those occurring at the elbow. These may arise, for example from ulnar abnormalities and

limitations in motion and reduced function of the forearm will result ( Morrisey & Weinstein,

2001). In this instance the shoulder, wrist,or trunk need to be used to compensate and facilitate

recreational activities and activities of daily living. This presentation is very uncommon and may

not always be remedied by surgical interventions although elbow reconstruction might be feasible

in some case.

Abnormalities which involve limited proximal and distal radial physeal growth result in

wrist deviation and bowing of the ulna . (Morrisey & Weinstein, 2001). Additionally with radial

skeletal malformations there may be paralleled soft tissue deficiencies on the preaxial side of the

hand, wrist and forearm and wrist extensors, pronator-flexor muscles, radial nerve and radial

artery may all be severely affected. Children with longitudinal deficiencies of the radius may

need to compensate for the absence of opposition by using spherical grip and lateral pinch and

application of adaptive techniques for managing hygiene, feeding, and dressing are often

necessary.

7


8/38

The congenital abnormalities previously described highlight how performance in key

activities of daily living are likely to be impaired from such malformations and the long-term

effects of these pervasive conditions. However, surgical, medical and therapeutic interventions

can potentially and substantially lessen deformity and improve function. Although failure of

formation anomalies appear least common they are probably the most likely to have most impact

on upper limb and hand function and are subsequently the main focus of this paper.

Global Incidence of Upper Limb Deficiencies

Morrissy & Weinstein (2001) note that in general there is little information on the

incidence of congenital limb deficiencies. However, Giele et al (2001) in an eleven year

population study of Western Australia found the prevalence of babies born with upper anomalies

was 1 in 506. 46% of these had another nonhand congenital anomaly, 51% had bilateral hand

anomalies, and 17% had multiple different hand anomalies. The most frequently occurring

irregularities resulted from failures of differentiation (35%) duplications (33%), and failures of

formation (15%). Upper limb anomalies were more frequent for boys, preterm and post-term

births, multiple births and older mothers. No significant differences were found for ethnic groups,

left and right sides or in babies who survived birth or those who died shortly after birth.

Current and accurate data regarding the global incidence for all congenital disorders

which affect the upper limb is difficult to gain. In the developed world national registries are

maintained which provide statistics pertaining to incidence and epidemiology and focus in the

literature in this field highlights concerns about management of surgical amputations arising

from diseases such as diabetes and cancer or traumatic injury and implications for subsequent

rehabilitation. (Ephraim et al, 2003). However, trying to find information pertaining to incidence

of congenital malformations in the developing world is more difficult. Little could be found by way

of statistical data for these populations and they do not appear to be well represented in research

literature. There may be a number of reasons for this. Historically all types of birth defects have

8


9/38

been considered by multiple cultures to be stigmatizing or to be punishments for misdeeds and

sins and a visitation of evil upon the child and family (Brent, 2004). In cultures where such belief

systems still persist children with these birth defects are less likely to come to the attention of

health and medical authorities/organizations who are a main source for keeping records.

Additionally, where resources are scarce there may not be the commitment or infrastructure to

support collection of data for these relatively rare conditions. True incidence of upper limb

congenital malformations may also not be known due to non- recording of stillbirths or pre-natal

detections of such deformities which may result in terminations.

Brief Overview of the Functional anatomy of the Upper Extremity

The upper limb is a remarkable and complex example of natural biomechanical

engineering. A fully developed and unimpaired arm is comprised of multiple structures which

allow smooth, efficient movements to occur. Hand motions are made more effective through

proper positioning via the elbow, shoulder joint and shoulder girdle. Greatly simplified, the upper

limb comprises of the shoulder girdle complex, the shoulder joint, the three long bones of the

humerus, radius and ulnar , elbow and radioulnar joint, wrist and hand. The hand itself consists

of 27 bones, 30 joints and 33 muscles and is innervated from three peripheral nerves. (Stanley &

Tribuzi, 1992).

The upper extremity is connected to the trunk via the sternum and skeletal attachment

occurs at the sternoclavicular joint. (McMinn et al, 1993). However, most arm movements are

facilitated by the combined actions of the three joints of the shoulder which consist of the

sternoclavicular joint, the acromioclavicular and glenohumeral joint which is a lax, multi-axial ball

and socket joint. It is supported by a network of muscles, ligaments and soft tissue and is one of

the most mobile joints of the body. (Hamill & Knutzen, 1995). The sternoclavicular joint in

isolation has little stability but acts as an axis for rotation of the shoulder girdle and absorbs

lateral shock. (Jacobs, 1999). The acromioclavicular joint facilitates elevation and depression of

9


10/38

the scapula. (Hamill & Knutzen, 1995). The range of motion at the shoulder joint is extensive. It

allows the arm to be moved through approx 180 degrees of flexion or abduction, 60 degrees of

hyperextension, 75 degrees of hyper-adduction, 90 degrees of internal and external rotation, 135

degrees of horizontal flexion and 45 degrees of horizontal extension. (Hamill & Knutzen,1995).

The elbow joint is comprised of the distal end of the humerus, the proximal end of the

ulna and the proximal end of the radius. whilst the radioulnar joint consists of proximal, distal and

middle joints stabilized by ligaments.

The wrist joint is made up of the distal ends of the radius and ulna and two rows of carpal

bones and its movements are flexion, extension, radial deviation and ulnar deviation. (Jacobs,

1995).

The combination of shoulder and elbow/radioulnar joint movements, in concert with the

hand, enables us to effect a myriad of movements essential for participation in occupations that

require prehension, that is to say the use of the hand and fingers to grasp or pick up objects.

(Anderson et al, 2002). Without this capability actions required for dexterously and precisely

manipulating tools and utensils, catching a ball or fine motor tasks such as rolling a bead between

the fingers are not possible. The wrist and hand are capable of precision and power because of

multiple joints being controlled by a large number of muscles which originate in the forearm and

enter the hand as tendons. Additionally the hand communicates a wealth of sensory information

and feedback and deficits to the hand can result in loss of tactile discrimination and absence of

capability for discerning the nature of objects by the sense of touch.

The Challenges of Upper limb Deficiency and the Facilitation of Occupational Adaptation

Due to the rarity of congenital upper limb deficiencies many clinicians and healthcare

professionals may have limited or no experience of these conditions. However it is important that

patients are able to access organized programs where knowledge and experience is available.

10


11/38

The management of pediatric limb deficiency is a specialized and complex area of clinical

practice. A multi-disciplinary team approach to these patients and families requires effective

communication and ideally teams working with this type of presentation should be comprised of a

physician, surgeon, prosthetist, physical therapist, occupational therapist, social worker or child

psychologist who have knowledge of normal child development and are aware of the deviations

from the norm which will occur as the child develops. With upper extremity deficient children

there are many factors which come into play for influencing interventions. Therefore, assessment

and treatment planning need to focus on function, growth, cosmetic deformity and the concerns of

the family and child if successful outcomes are to be achieved. (Morrissey & Weinstein, 2001;

Meier & Atkins, 2004).

Function

Interventions aimed at improving function for congenital upper limb or hand problems

should address the ability to place the limb and hand in space, deficiencies in grasp, release, or

pinch capabilities and improvement or maintenance of skin sensibility and mobility, and giving

consideration to the degree to which physeal abnormalities are likely to affect musculoskeletal

deformity both short-term and long-term. In some cases non-operative care may be indicated

whilst in others reconstructive surgery or amputation may be advised as a means of optimizing

function but much will depend on the condition being presented and the associated

complications. (Waters, 2001).

If a decision is made to fit a child born with congenital upper limb absence with an initial

passive prosthesis, subsequent to amputation, then this can be made as early as when the infant

is three months old . This is the age at which they begin to sit up and start to use their hands

together for gripping activities and is also when the device can start to begin to become

incorporated as part of their body image by parents thereby increasing the likelihood of their

acceptance of the prosthetic.(Stark, 2001) However, before proceeding with the construction of

any prosthesis there will always need to be a full evaluation and examination of the limb to

determine current shape, extent of any scarring and bony areas which are likely to affect design,

11


12/38

comfort and flush fit. Children are potentially likely to have a series of prosthetic fittings as they

grow and as their needs change. If a child retains a prosthesis it is likely to be activated when

they are about 18 months of age for below elbow amputee and about 3 years of age for an above

elbow amputee. As a child grows greater considerations need to be given for balancing function

with cosmetic concerns. It is at subsequent evaluations that advantages and disadvantages

about component parts are likely to be discussed with both the child and parents. Outcomes of

these discussions will determine the composition of terminal devices, wrist, elbow, hinges, control

mechanisms, harnessing and socket fitting. Assessments of range of motion and muscle

strength will be recorded to determine how best to make the socket so that it causes least

restriction of movement and facilitation of prehension with a terminal device should be a primary

objective. (Stark, 2001)

Growth

A childs physical and emotional status constantly changes as he or she develops.

Consequently the rehabilitation approach must be responsive to these maturational changes with

regards to congenital upper limb deficiencies because developmental milestones are used as a

guide when prescribing prosthetic devices. (Jain, 1996; DiCowden 1990). In the first year of life

a child is physically developing strength proximo-distally and is learning to co-ordinate

movements of trunk, and head with gross motor functioning of the limbs. For transradial

unilateral and transverse forearm anomalies, Initial fitting of a light-weight supracondylar

prosthesis, with a self-suspending socket, may be effected from 3 9 months of age to assist in

gross motor development tasks, such as creeping and pulling to standing for example. At two

years of age a child is attuned to processing information through the five senses, is able to

ambulate and is beginning to develop manual dexterity. If initial fitting is made at this stage

however, there may be a rejection of the prosthesis because the child is already starting to

develop compensatory techniques by using their feet or trunk but these may be counter-

productive to the development of normal movement patterns if left unchecked (Jain, 1996).

Children with bilateral deficiencies also require early fitting but may not always benefit more from

12


13/38

bilateral prostheses unless they can be an adjunct to improving sitting and standing balance and

the weight and complexity of hardware can be a major source of frustration for a child.

Unilateral fitting in these instances may still be advisable with this being applied to the side where

the child has developed a leading foot pattern (if this is known). However, children between 2- 6

years of age can usually be fitted with a myoelectric or body-powered prosthetic with a terminal

device providing that they do not have other cognitive and developmental limitations which might

interfere with understanding 2-step commands, attention span or the ability to relate to

bimanual prehension activities. The ability to use a prosthesis competently by age 2 is

significant because at this stage of development the child is asserting some degree of self-

sufficiency. Effort is concentrated in self-feeding, independent toileting, obtaining objects in the

environment unassisted and gaining self-control over the body. This process can be delayed if

the child is necessarily having to remain dependent on a caregiver for these seminal tasks and

they may be significantly below their age level in relation to motor skill development an

subsequent intellectual growth (DiCowden, 1990).

Children with an amputation at the elbow level can generally be fitted with a lock and unlock

elbow cable operated or switch control prosthetic, which acts as an assisting limb, at between 35

-48 months if they can demonstrate understanding of the relationship between objects and the

immediate environment but the Utah Myolectric elbow is not suitable for children under 14 (Jain,

1996). At around 3 4 years of age children are going to become more aware of their limb

deficiency and of the visible difference between themselves and other children but will not yet

have capacity for building their own coping strategies. (Tonnacour and Shorgan, 2004) . This

can be a difficult phase as the child may display social discomfort just as they are about to

commence school.

From six through to puberty children continue to develop co-ordination and social skills

which may be demonstrated in involvement with scholastic endeavors, sports and other extra-

curricular activities. This is the stage of development where Piagets concept of concrete

operations is formalized by the individual being able to make connections between concrete

13


14/38

relationships. For instance they more readily understand mathematical quantities and proportions

and can relate to basic scientific principles. Additionally, according to Eriksons theory of lifespan

development feedback from peers , as well as adults, begins to have significance at this age

and the child can achieve sense of competency with attaining age appropriate tasks or can have

feelings of inferiority if they are not able to develop skills and to be recognized.

DiCowden (1990) highlights that rehabilitation needs to actively take account of the needs of

the child with congenital deficits or chronic at each particular age stage because disability can

have dramatic developmental impact upon the child. Dicowden (1990) also advocates for

multidisciplinary rehabilitation services being set up which assess and treat children all the way

from infancy to late adolescence and which provide comprehensive evaluations taking into full

consideration intellectual and psychosocial functioning in addition to physical and medical factors.

The use of prescribed prosthetics, or other assistive technology is therefore subject to the

childs different stages of development which will inherently bring them changing needs and

challenges. Certainly, as children with congenital deficits reach adolescence they may need to

deal with a number of psychosocial issues around independence, self-identity and body-image

and these are discussed in a later section of this paper.

Cosmetic Deformity

Upper limb anomalies are a very apparent visible difference. As such individuals can be

subject to stigma and negative perceptions associated with disfigurement with subsequent

consequences of poor body image and loss of confidence. Depending on the nature and extent

of the anomaly, some deformity may possibly be diminished through surgical interventions either

through amputation or reconstruction. However, all children will gradually become aware of how

socially orientated society is and by the time of adolescence most children do not want to be seen

as different from their peers. However, children with this pronounced type of upper limb

impairment need to come to terms with their difference whilst also being expected to be become

more independent. Therefore challenges exist for rehabilitation teams to be able to provide

14


15/38

prosthetic devices which when in use are not only functional but also look aesthetic and lifelike.

Additionally, any other assistive devices or technology used also has a requirement to be age

appropriate and appealing for this group.

FamilyConcerns

The parents of an infant born with congenital upper limb dysfunction are likely to initially

experience a sense of grief and guilt, in addition to lacking information about the nature of the

cause and likely progression of their childs condition and how this will influence future

development. Therefore, it is imperative that they have access to guidance from professionals

who can provide education and advice which will enable them to make informed decisions about

their child at each stage of growth. These decisions will have lifelong consequences for the child

as they have a condition which they will need to adapt to and accommodate rather than having an

illness that has an end point or a cure. In some circumstances functional use of a childs upper

limb may be enhanced by amputation but this may be a difficult recommendation for parents to

accept. DiCowden(1990) states that the family is the most important mediator of a childs

development, and subsequently if parents remain grieved by their childs difficulties they cannot

give the necessary feedback and guidance required for rehabilitation.

Parents may benefit from counseling, psychotherapy and referral to support groups where

they can be introduced to families who have gone through similar experiences. This can help

allay fears and anxieties about the future, begin to give resolution to feelings of loss and

disappointment whilst leading them to a point of acceptance and realistic hope that their child can

reach their full potential. (Morrissy, Giavedoni, & Coulter O Berry ,2001) .

Although initially born dependent, normally developing children, gradually move to greater

independence and are capable of demonstrating considerable physical and psychological

adaptability. Loss of upper limb function can greatly impact on all areas of occupational

performance but children born with congenital disabilities affecting this extremity can also thrive if

15


16/38

they are provided with the right resources, assistive devices, technology and accommodations.

The utilization of adapted performance techniques can also enable them to acquire skills and

strategies for developing competence and mastering the environmental contexts in which they

are placed. However, if a child does not have access to the appropriate remediations reduced

opportunities for developing skills and autonomy is likely to result in diminished sense of personal

causation, self-efficacy and self-esteem (Stoller, 1998).

Children with a unilateral upper limb congenital deficiency are likely to cope well in most

situations without a prosthesis or other assistive devices. They can learn to use their intact hand

for most activities whilst often using their deficient, or shorter arm for steadying. Anomalous

limbs are often still functional but orthoses and limb reconstruction are options for maximizing

function. However, sometimes amputation maybe indicated but the goal should be to create the

most functional limb possible which allows the individual to gain most life-satisfaction. (Carroll &

Edelstein, 2006)

Celikyol (1984) notes that problem-solving and the pursuit of sometimes unorthodox

solutions are crucial strategies for effectively interacting with the environment and for facilitating

the management and performance of activities of daily living when prosthetic aids may not

always be wanted or feasible. Individuals with bilateral upper-limb absence, as in the case of

phocomelia, may prefer to use their feet as arm substitutes for functional activities rather than

cope with prostheses.

The Thalidomide Society has highlighted some of the difficulties which can be experienced

as a result of congenital bilateral upper limb loss. Many of these individuals have become adept

at using their feet and toes as substitutes for arms and legs but are now finding that over-use of

their lower limbs are resulting in knee and hip problems arising from joint degeneration which is

affecting their ability to manage some tasks. Many thalidomide impaired people report that the

length of time take for personal tasks such as washing and drying hair and dressing may take

16


17/38

four times longer than for anyone else. Even slight Injury (such as a stiff shoulder) can often

result in not being able to carry out personal care activities until the injury has healed. Meal

preparation can also be difficult and dangerous. For instance the cutting of vegetables, and the

carrying of hot dishes, or lifting and moving of pans is often done in close proximity to the body

because of reduced arm length and the lack of thumb and fingers means reduced grip and

strength. Where hands are articulated at the shoulder it may not be possible to carry items at all.

Additionally medication bottles or blister packs may be difficult to open because of a lack of a

thumb or fingers. Also the lack of the fine pincer grip movements makes the picking up of small

items such as coins and pills difficult to achieve.

However, perhaps one the greatest challenges for children with this type of presentation as

they grow older is learning to accept themselves as they are irrespective of the physical

difference created by the upper limb deficiency.

Psychosocial Issues and Adolescents with Congenital Upper Limb Dysfunction.

The formation of a coherent personal identity is, in accordance with theories of

psychosocial development, a primary task of adolescence. The model of life span development

as posited by Erikson (1963) considers that during this period of transition failure to reach this

goal may result in confusion relating to sexual identity, choice of employment and other roles

performed as an adult. This stage of life may also be characterized by turmoil and stress as

adolescents experience the physical changes of puberty, and increasing expectations from

society at large as well as from family and peers as to how they should behave. The process of

puberty often carries with it concerns and anxieties over physical appearance and body image

and a desire to fit in with peers whilst also striving for a sense of ones own uniqueness and

17


18/38

forging adult relationships away from parents and the family. Over the past thirty years there

has been a large amount of research interest in adolescent development. However this is in

relation to the general population. Chamberlain and Kent (2005) state that this process maybe

all the more challenging when childhood is marred by congenital or acquired disability. Rumsey

and Harcourt (2004) also note that transitional periods including changing schools, jobs or

neighbourhood can be particularly challenging for those with a physical difference because they

need to develop new strategies for dealing with the reactions of unfamiliar people and for forming

new relationships which go beyond initial encounters. Children with disability may experience

less exposure to everyday events and therefore have fewer opportunities to learn and explore in

the same ways as their age-related peers. Hostler et al (1989) affirm that the literature

concerning psychosocial outcomes for children with disabilities points to themes of poor social

adjustment, a sense of isolation and loneliness, fewer same-sex and opposite sex friends of

similar age, increased dependence on others, less assumption of responsibility and less than

average knowledge of sex.

Adamson (2003) however, notes that overall, research literature regarding the

psychosocial development, and personal experiences of adolescents with physical disabilities, is

sparse and this appears to be particularly apt for individuals with congenital upper limb

dysfunction. Adamson also highlights that there needs to be further exploration of the ways in

which young persons with disabilities perceive and evaluate themselves.

Van Dortsten (2004) also reports that current reviews of the literature fail to identify

particular recommendations or innovations for guiding and integrating psychological, medical

evaluation and treatment approaches for limb loss. However, young children are not initially

intellectually aware of physical differences. In fact it is most likely the parents who will have

difficulty adjusting to their childs disability. Initial reactions are likely to be those of shock,

anxiety, guilt and fear for the future, and there maybe some difficulty with the initial bonding

process (Rumsey and Harcout, 2004) . Parents may be concerned about the reactions of other

18


19/38

people and how this will affect childs feelings and level of self-acceptance as they develop.

(Carroll & Edelstein, 2006). Families can vary considerably in their strategies for coping with a

child with a physical difference. These may range from open discussion , denial of its existence

or over-protection. (Rumsey and Harcourt, 2004). Parental concerns, however, may in turn be

transmitted to the child and assimilated with subsequent consequences for the childs own

perceptions of emerging body image and feeling of self-worth (Rumsey and Harcourt, 2004).

The child themselves may at some stage indeed begin to experience some degree of self-

consciousness as they progress towards puberty and may also be more vulnerable to teasing

and bullying from peers. It is at this time that some children may try to hide their deficiency,

particularly if they feel the effects of social stigma, even though may they have accepted the

appearance of the arm/hand previously. There may, for the first time, be signs of resentment and

a feeling of injustice about their disability. Again, however, there is little in the academic

literature which highlights how adolescents with this presentation deal with issues of body image

at this stage of the life span. Van Dorsten (2004) notes that no data exists to clarify whether body

image issues are greater for individuals with congenital limb loss, or for persons with acquired

limb loss arising from traumatic accident or vascular disease.

Parental support at times of transition in a childs life is always important but it is likely to

be crucial for the child who may have experienced various limb fittings (if prostheses are

involved) surgical procedures and other therapeutic interventions, which call for them to develop

adaptive coping styles. Biddiss et al (2007) highlight that strong social support networks

consisting of parents, teachers, friends and classmates have a positive impact on the

psychosocial health of children with disability. In fact peer acceptance of individuals with upper

limb loss generally appears to be good for children and adults alike. In terms of personal

adjustment and adaptation children with congenital limb loss have a higher level of social

adjustment in contrast to adults with acquired upper extremity deficiencies. This may be

attributed to the fact that persons with a congenital condition have a lifetime of incorporating their

disability into their identity. Persons born with disabilities begin to shape meanings ascribed to

19


20/38

assistive technology early on and are more likely to consider it to be a means for facilitating

performance of tasks which they might not otherwise be able to undertake. (Louise - Bender

Pape et al, 2002). Those individuals with an acquired impairment, or amputation which has

arisen from sudden trauma or a state of acute, or even progressive disease are likely to have to

make more significant and critical changes in lifestyle, employment and social roles. In such

instances the use of assistive aids and prostheses maybe viewed as an inadequate substitute

and may not compensate for the loss of function of the limb and serve as a negative reminder of

this for the individual. However there is evidence which suggests that upto 19% of adolescents

with upper limb deficiencies are likely to reject prostheses and aids.

There are a possible range of reasons for this. Postema et al (1999) and Biddess et al

(2007) note that there is a shift in functional needs, from motor skills necessary for play and

exploration, to cognitive skills at this stage of development which exact less extensive use of the

hand, but more importantly if parents have negative expectations of their childs prosthesis then

the likelihood of it being rejected is higher in this instance. Biddiss et al (2007) also note that for

older pediatric users cosmesis has higher priority as a design feature than functional utility.

Secondly, adolescents may negatively reassess the role of their prosthesis as they form a more

autonomous identity. This is also a time when image counts for dating and appearance is tied in

with self-worth. De Tonnacour and Shorgan (20004) note that grief for the absence of a perfect,

symmetrical body can be intensely felt at this stage and can become an important element adding

to the normal crisis of adolescence. Subsequently adolescents with a visible difference or

disfigurement may be more susceptible to lowered sense of self worth which may turn to

depression. (Rumsy and Harcourt, 2004). Louise- Bender Pape et al (2002) emphasize that the

extent to which assistive technology and other devices are perceived as acceptable and helpful

tools depends on the individuals point in the life cycle, their place and role within the family,

cultural heritage and the social consequences of assistive technology use. Bad fitting

experiences which have resulted in pain may also contribute to non-use of a prosthesis. (Carroll

& Edelstein, 2006).

20


21/38

Factors Affecting use of Assistive Technology Devices for the Upper Limb, with Specifc

Reference to Prostheses

Stoller (1998), states that in relation to human occupation assistive devices can be influential

in a number of ways. Firstly, assistive devices and technology can facilitate the undertaking of

tasks which may previously not have been possible. Furthermore, assistive devices can

remediate or compensate for deficits in developmental skills with results of the individual being

better able to organize existing skills into occupational behaviors which meet with societys

expectations and the external demands of the environment. Additionally, assistive devices can

significantly reduce the amount of time and effort expended in undertaking tasks which adds to

efficiency and performance competence. However, assistive technology is not without its

limitations and it is important for both the users and providers of assistive technology to have

realistic expectations of what can be achieved. Although assistive technology can be a powerful

remediation tool it may not always be feasible to apply it for effecting full restoration of lost

functional capabilities. This is pertinent in relation to loss of function with the upper limb.

Given the complexities and intricacies of the anatomical structures and physiology of the

upper limb it is not possible to replicate all the movements of the natural hand and arm and any

mechanical replacement is a poor imitation. Society in general, appears to be better at

accommodating lowerlimb disability whereas there appear to be less environmental adaptations

eg lever-type faucet handles, for those with upper-limb deficiency. There is also the issue of

providing devices tailored to meeting the self-care needs of individuals with upper limb difficulties.

Manufacturers may be deterred from committing funds and resources for improving components,

materials, and investing in research for this relatively small but specialist market. (Carroll &

Edelstein, 2006) However, the most widely available assistive technology devices available for

21


22/38


23/38

prescribed and accepted by individuals with a below elbow amputation. They have the

advantages of requiring less natural energy being expended by the recipient, and the need

for harnessing is reduced or eliminated but they are very costly. (Carroll & Edelstein,

2006). However, they have the benefit of appearing more life-like and yield more grip

strength when required for functional activities. (Herr et al. n.d)

A Terminal device (TD) is the part of a prosthesis which substitutes for the anatomic hand.

Many TDs are commercially manufactured and can easily be removed from or added to a

prosthesis with a rapid disconnect wrist unit. Additionally by placing a rubber washer or O-ring

on the threaded screw at the base of the TD, a user can gain better control over incremental

rotation of the hand and this of benefit in sports such as baseball, basketball, or other recreational

activities such as photography or the playing of musical instruments. Some units also allow two

angles of wrist flexion to occur and this is benefit to anyone who cannot reach the midline of the

body. Furthermore, wrist flexion is particularly essential for helping with feeding, dressing, oral

and facial hygiene and toileting (Carroll & Edelstein, 2006).

Future TDs are likely to incorporate sensory feedback. Long-term goals of present research

in this area are to integrate prostheses directly into the bodys sensory nervous system and to

design TDs which can replicate multiple grasp patterns and wrist movements. Additionally,

electrodes may be implanted into muscle or attached directly and in conjunction with

microprocessor technology the user may be able to select pre-programmed series of movements.

For instance a person with a shoulder disarticulation could wear a prosthesis which is

programmed for facilitating eating. The hand would hold a fork, whilst the wrist rotates and the

elbow would flex to bring the food to the mouth with only one signal being required to trigger this

sequence of movements. (Carroll & Edelstein, 2006)

Brenner (2002) notes that electronic technology has made significant strides in the field of

prosthetics but has subsequently led to increasing complexity and a greater range of options

23


24/38


25/38

Clinical funding is still a requirement to ensure that new or modified assistive technology

aids and advices -prosthetics or otherwise - can actually be properly tested, applied and

accessed by appropriate patient groups and evaluated for their efficacy as treatment techniques

and for meeting the individual needs of users.

Irrespective of these technological innovations, children as they develop, may opt to only

use a prosthesis only for certain specific sporting activities or social events. Indeed some of the

most popular adaptive and prosthetic designs have been developed by individuals who wanted to

find better ways of participating in their favorite sports and recreational activities. The choice of a

prosthetic aid depends on many factors which need to be assessed for by a clinical team.

Principally these are in the domains of concordance, comfort of the device, particularly in relation

to socket configuration, cosmesis and physiological and psychosocial factors of upper-limb

disability as these are very pertinent to the issue of rejection of a device which tends to occur

after one or two years of successful usage. Certainly an interesting and consistent finding in

relation to adolescent prosthesis wearers is that they do opt to abandon use of prostheses at this

stage of their life but there are few studies which have actively addressed the design priorities,

and personal factors influencing device usage, of the pediatric and population via qualitative

comments and reflective feedback (Biddiss, Beaton and Chau, 2007). More longitudinal

research studies and methodological approaches which more readily incorporate service user

opinion, for example focus groups could help bridge this limitation.

Whatever the perspective of the team or level of advancement in technology, it is the

childs wishes, where they are able to start expressing these, which should always be seen as

paramount. Patience, compliance and participation are often increased with greater

understanding but the best device in the world is of no use at all if a person does not engage with

it (Carroll and Edelstein, 2006). Higher levels of prosthesis acceptance may be anticipated

where the device is inexpensive to produce, lightweight and fit for purpose, biomechanically

sound, durable and easy to repair or replace and cosmetically acceptable. As expectations of

25


26/38

technology grow, and patients become more informed of what might be available to them,

providers of assistive devices, whether this is a prosthetist or indeed an occupational therapist,

will need to stay abreast of new products and techniques.

The Role of the Occupational Therapist with Adolescents with Upper Limb Difference

In many respects the Occupational Therapist who works with adolescents with upper limb

will, at varying times assume the roles of educator, advocate and researcher on behalf of the

user. A key aim of intervention with this client group is to maximize function and autonomy,

whilst also enhancing quality of life via the appropriate access to, and utilization of, assistive

technology devices and aids. The therapist is likely to educate and train both parents and the

adolescent about the strategies, techniques and equipment which might enable greater

independence and efficiency of energy to be achieved regarding self-care activities such as

feeding, toileting, grooming and dressing whilst also bearing in mind the developmental and

physiological changes which may be occurring at particular times. (Celikyol,1984). Many

children with congenital upper limb differences have, by the time they reach adolescence, learnt

to use problem-solving strategies for carrying out essential activities of daily living and can be

remarkably adaptable in most situations but consideration needs to be given to personal care,

health, social, economic and leisure choices. This is particularly relevant for individuals who

experience bilateral upper limb loss (Stoeker, 2004).

Adolescent clients may prefer to adapt or modify how something can be accomplished

rather than use equipment because this may reduce feelings of dependency. Mutual problem-

solving which involves parents is keyas their attitudes, participation and interest shapes and

influences training programs and treatment interventions. In fact there is likely to be lesser need

26


27/38

for specific interventions in many areas but the therapist may help adolescents and their families

deal with specific personal, social and vocational effects of upper limb disability and issues which

arise at times of transition. For instance these may include progression to high school, leaving

home for the first time which brings expectations of being more independent of family, and

obtaining employment.

It is essential for the therapist to work collaboratively and holistically with the adolescent. In

order to establish an occupational profile and design interventions which reflect their priorties and

life situation the therapist should aim to use age-appropriate assessment instruments for

gathering information, identifying the current strengths as well as potential problem areas.

Appropriate assessments include the Adolescent Role Assessment (ARA), The Occupational

Circumstances Assessment Interview Rating Scale (OCAIRS) and The School Setting

Interview (Henry, 2003). The ARA is a semi-structured interview procedure which covers areas

of childhood play, family and peer socialization, school functioning, occupational choice and

anticipated worker role as an adult. The OCAIRS focuses more specifically on occupational

participation and adaptation and addresses personal causation, values and goals, interests, roles,

habits, and skills. The SSI can be used to identifying any unmet needs for school environment

accommodations.

In the context of working with adolescents with upper limb difference the therapist also

needs to remain updated on the providers of assistive technology products, and to be aware of

resources, agencies/organizations and latest developments which the client might need to be

directed to in order to make informed choices about what is available and is going to best meet

needs. At times, there may be the necessity for a client to make use of both simple and

complex devices in order to meet functional needs and to satisfactorily undertake key everyday

tasks. Artificial hands, Prostheses and individually designed orthoses are definitely examples of

more complex devices but other widely available items can be utilized or adapted for domestic,

self-care/personal and other instrumental life skills. However, where amputation is effected in

27


28/38

adolescence the occupational therapist is likely to have considerable involvement in providing

instruction in the control and use of prosthetic devices. In relation to prosthetic controls training

emphasis may be placed on body control motions, grasp/release and co-ordination of movements

using a TD and in relation to wider prosthetic usage focus will be on activity based training

pertaining to self-care, recreation and home, work or school evaluations as needed. (Carroll &

Edelstein, 2006).

To illustrate the range of assistive technology currently available, for assisting adolescents

to maximize their ability to undertake basic personal/ self-care tasks and reduce the need for

physical assistance from other, some appropriate devices, aids and techniques are now

mentioned. Additionally assistive technology may be required to lend educational and vocational

support for this group of adolescents as they transition to adulthood and some current advances

in computer technology are also outlined in addition to reference to environmental controls .

Devices for self-care and dressing - In the case of unilateral impairment, there may be

reliance on use of the residual limb and the other fully functional limb for carrying out tasks

mostly one-handed . For bilateral amputees feet may adeptly be used in place of the upper limb

for many activities. Dressing can be made easier through use of items such as shoes with

Velcro laces that can be closed with one hand, shirts/blouses with Velcro fastening instead of

buttons or us of a PantClip (available from Sammons Preston) that can help unilateral upper-

extremity amputees pull-up and button their own pants. Button loops/hooks can help upper

extremity amputees more readily guide buttons through buttonholes and dressing sticks can also

be used to position garments.

For meeting grooming and hygiene needs commercially available dental floss holders can

aid with dental hygiene and battery operated toothbrushes can be used by. Body/hair washing

drying and brushing can be problematic but ingenuity, trial and error learning and perseverance

are often factors which contribute to independent success in these areas. For instance showers

can be adapted with Hygenic body washers (large rectangular pads that attach to the wall with

28


29/38

suction cups) and hair brushes can be strategically wall-mounted and brushed up against and

hair-driers might similarly be wall-mounted. (Bowers, 2001).

Toiletting for bilateral upper-extremity amputees can be challenging. Installed bidets for use

with a commode can be useful in the home and there are more sophisticated, but also more

expensive toilet systems, such as the Closomat which combines a water stream for cleansing and

air for drying. The control handles can be manipulated using a mouth stick with a long metal

hook (Bowers, 2001). However, physical assistance may sometimes be needed in some

circumstances when the individual is away from the home environment.

Feeding and Meal Preparation - Light-weight, combination knife and fork Cutlery sets (such

as those available from Maddak,Inc) allow for one-handed users to utilize a single device for

cutting and eating food. A device called The Eatery (also available from Maddak Inc) is also a

relatively simple device that enables bilateral upper-extremity amputees to eat independently

without using prostheses. At the other end of the spectrum switch activated systems such as the

Winsford Feeder (available from Sammons Preston) which is operated by head or chin

movements and brings a spoon from food to the mouth of the user, may be a means of helping

individuals with no arms to eat independently. Being able to cook and make meals for oneself is

an important life skill which adolescents may begin to experiment with prior to leaving home for

the first time. For meal-preparation spiked cutting/chopping boards can be invaluable for one

handed users and automatic can-openers such as the one touch can-opener can ease

frustration of trying to open cans. Jars can be opened a variety of ways with modified techniques

and devices. For instance a unilateral upper limb amputee who is fitted with a prosthesis can

grasp the middle of the container with the terminal device and then use the sound hand to

unscrew the lid. Alternatively if only one hand is being used, the jar can be secured with a piece

of dycem under it, and then the hand pushes down on the lid and then turns it. There are other

commercial aids to assist with this task, but are best being tried first wherever possible before

being purchased to see if they work for the particular individual user.

29


30/38

Technology for facilitating educational participation for Adolescents with Upper limb

Differences

In many instances there may not be the need to use specific complex technological or

assistive devices with children with upper limb difference but this will depend upon the extent and

type of presentation which will affect how the adolescent is able to participate in school activities.

Some adolescents with unilateral limb loss may require only minor accommodations in the

classroom which then enable them to fully engage with the curriculum. These students, may for

example, be very capable of performing hand-writing tasks without specific remediation and can

be proficient at one-handed typing, or using standard keyboards rather than requiring adapted

versions. Likewise some students with bilateral limb absence, may be able to use writing

instruments dexterously with their toes, but may not want to do so in front of their school peers,

and so assistive devices and computer technology may be preferable and appropriate for

increasing access to the learning environment for these students. Where prostheses and

terminal devices are in use, they should enable the student to have functional grasp and strength

for pencil and paper work, keyboarding and being able to hold and place items (such as

backpacks, cans of soda) effectively. Any re - fittings occurring at this time are likely to include

requests from both parents and the child for prostheses which combine cosmesis with function.

(Ramdial, Wierzba, Kurtz and Hubbard, 1997) However, in addition to the personal preferences

and skill levels of individuals, the technology required always needs to be based on assessment

of needs, cost and training requirement considerations and what can realistically be provided in

relation to facilitating inclusion and engagement for the student in question.

Computers

Computer technology can open up the world for individuals with upper limb absence and

gives them access to a social life, information, support and even employment which becomes a

major area of focus when adolescents are on the brink of leaving school and deciding what they

become in their adult life. Some competence with computing skills is expected with most jobs

30


31/38

these days and personal computers are also used routinely for managing aspects of our everyday

lives, such as online banking and shopping in addition to being a source of leisure activities, but

for many people with no hands, or only one-hand, typing, using a keyboard and a mouse may be

difficult. (Bowers, 2001) Major advances have been made in computer technology and many

products have been developed which can allow computers to be operated totally hands-free

(Bowers, 2001) . Such products include foot-controlled mouse consoles, or headmouse devices

which are activated by movements of the users head. A sip/puff switch or software is used to

perform the click function. Additionally, other integrated infrared/sound/touch switches can make

it possible for users to operate numerous devices and software with just an eye blink or other type

of body movement. Several speech recognition/ voice activated software packages (such as

Dragon Speaking 9) are now also available for providing access to the internet and facilitating

written applications on the computer.

Electronic Aids for daily Living and Environmental Controls

Electronic technology in rehabilitation has created further potential for increasing

independence for individuals with upper limb loss in the form of environmental control. Through

environmental control systems it is possible to operate for example lights, telephones, and

televisions, in a living or work area by use of residual control motions to activate either

microswitches, pneumatic switches or voice- controls. Mouth sticks, universal cuffs or

splints/orthotics may assist with accessing the systems but may not always be appropriate or

necessary. Before recommending any Electronic Aids for Daily Living (EADL) thorough

evaluation should take into account all environments where the EADL will be used, access

methods, feedback mechanisms and requirements, flexibility and regularity of use, installation

and cost. (Bain, 2003).

Driving Adaptations

The ability to drive may be of importance to the adolescent at this time and can be a major

asset for independence. The occupational therapist can give vital information and advice about

31


32/38

possible vehicular modifications that can be made and can refer parents and the child to the

appropriate agency responsible for carrying out driving evaluations. (Morrissey et al, 2001).

Many individuals with upper limb deficiencies drive successfully and sometimes without

adaptations being made to vehicles. This is easier when cars are automatic transmission.

However, modifications can be made which may include spinning knob conversions being made

to steering columns, and windshield wiper controls and signal indicators being foot-operated

switches/pedals.

Implications for Future Practice and the importance of Increasing Occupational Therapy

training and Awareness of Assistive Technology applicable to Upper limb Loss

This paper has explored an area of congenital physical disability which is generally

unfamiliar to most occupational therapists unless they develop expertise in upper limb

dysfunction. Whilst occupational therapists may at some points in their practice come into

contact with the adult amputee population it has been highlighted that children born with upper

limb loss are, by the very nature of the rarity but also complexity of their conditions, seldom seen

routinely in occupational therapy clinical settings. Children with upper-limb deficiencies are

usually managed long-term by specialist multi-disciplinary facilities which are equipped to

address the numerous assessment and treatment considerations needing to be given to factors

of age, effects of maturation on function and level of deficiency of the limb, possible surgical

interventions, and psychosocial influences which can have a significant impact on body image,

successful management of transitions, rehabilitation programs, and outcomes for positive

occupational adaptation. A key aim of this paper was to investigate how adolescents with

congenital upper limb limb loss utilize assistive technology in their everyday contexts with

particular emphasis on managing self-care activities of daily living.

At the beginning of the paper a number of questions were posed which appear not to

have been addressed in mainstream occupational therapy journals or healthcare research

32


33/38

literature pertaining to the adolescent population with this type of upper limb deficit and their

particular needs in this area of occupational performance. These questions were directed at

establishing if current assistive technology options can fully meet functional requirements for

activities of daily living and if they dont then what alternative strategies/techniques bridge the gap

which could make a difference in these areas.

There are numerous factors which shape personal preference choices for assistive

technology and some of these have been described in this paper . Although overgeneralizations

cannot be made about all adolescents who comprise this treatment population it has become

apparent from undertaking this inquiry that there are usually two clear paths which teenagers

with congenital upper limb deficiencies ultimately chose between. These are either the use of

customized and functional myoelectric prostheses which can be adapted for purpose with

differing terminal devices, or actual rejection of mechanical and complex devices in favor of

problem-solving approaches and some utilization of simpler aids, modifications and

environmental adaptations. With regards to the former, children whose deficiencies are terminal

transverse, for example below- elbow or above elbow, and who have had early initial and

successful fittings of prostheses as infants, at around 6 months of age, are good candidates

(Wenner, 1995). In relation to the latter, rejection most often occurs where children are either

bilateral amelic or phocomelic. If children with this level of deficiency are taught early on to use

their feet as hand substitutes they develop a high level of prehension, strength, flexibility and

sensibility and can accomplish most activities successfully and independently. Irrespective of

whatever route is taken, it is most important that children with upper limb difference cultivate a

strong self-identity and body image as they develop. Lack of this is acknowledged within the

prosthetics literature to be a probable contributory factor for rejection of prostheses in

adolescence but as yet has remained largely unexplored in research studies.

Further research for gaining the service user-perspective for this group is pertinent.

Unless it is known what these adolescents think themselves about the assistive technology

33


34/38

available and their relationships to it as active users and consumers, can occupational therapists,

truly say that they are providing the best evidence-based practice, treatment interventions

rehabilitative approaches, for this pediatric population?

Biddiss & Chau (2007), comment that conducting good quantitative studies in this area is

challenging because of the heterogeneous population characteristics and the difficulty of

recruiting sufficiently sized populations on which to draw results. However, in relation to

looking at the personal factors which shape use of assistive technology qualitative and descriptive

research methods, such as case studies and in-depth interviews can be more appropriate for

gaining insights and information about the lived experience.

Healthcare professionals involved with considering the role of assistive technology for this

client group need to stay informed and aware of the alternatives which most appropriately and

beneficially meet needs. However, due to the infrequent nature of congenital upper limb loss

there is a paucity of experienced clinicians whether prosthetists, physicians, therapists or

psychologists with a depth of understanding of this area of pediatrics. Presently, occupational

therapists in training receive little instruction in relation to assistive technology, biomechanics and

specific upper-limb devices as they relate to the pediatric population. Although Occupational

Therapy is crucial in the rehabilitation of amputees and other upper limb conditions most

occupational therapy students seldom encounter children with the presentations described in

this paper or prosthetic users during their fieldwork or training experiences. Post-qualification

there appear to be limited opportunities for advancing theoretical knowledge and gaining practical

competencies in this field. One or two-day intensive workshops being run at OT schools by

OT practitioners specializing in this field and continuing education programs which address skills

in this area would appear to be necessary in order to increase the knowledge base of therapists

who may at some point encounter children with upper limb difference, outside of their usual

arena of practice.

34


35/38

In conclusion, this paper has highlighted the different means by which assistive technology

can be an important adjunct to the facilitation of independence for those who experience differing

degrees of upper extremity limb loss and the attendant limitations and physical dysfunction which

can arise from this presentation. However, assistive technology should fit with the person

rather than vice versa and ultimately decisions made as to how this is purposefully incorporated

, into real life everyday contexts ultimately rest with the individual.

35


36/38

References

Adamson, L. (2003) Self-Image, Adolescence and Disability, American Journal of Occupational

Therapy. 57, 578-581.

Anderson, D.M., Keith, J., Novak, P.D. & Elliot. M. (2002) Mosbys Medical, Nursing, & Allied

Health Dictionary (6th ed). Mosby. Missouri.

Bain, B (2003) Chapter 31: Assistive Technology in Occupational Therapy. In E.B. Crepeau,

E.S. Cohn & B.A.B. Schell (Eds). Willard and Spackmans occupational therapy(10th ed.

pp659 675). Philadelphia: Lippincott, Williams &Wilkins.

Biddiss, E., Beaton, D., & Chau, T. (2007) Consumer design priorities for upper prosthetics.

DisabilityandRehabilitation: AssistiveTechnology. 2, 346-357.

Biddiss, E., Chau, T. (2007). The Roles of predisposing characteristics, established need, and

enabling resources on upper extremity prosthesis use and abandonment. Disability and

Rehabilitation: Assistive Technology. 2, 71- 84.

Bowers, R (2001) Assistive Devices Give Upper- Extremity Amputees a Helping Hand. First Step,

2, 1-4 retrieved from http:/www.amputee-coalition.org./first_step/firststepv2_slal4.html

on March 22, 2008.

Brenner C.D.(2002) Prosthetic Principles: The Need for Early Intervention.Chapter 8: Atlas of

Limb Prosthetics: Surgical, Prosthetic and Rehabilitation Principles. Retrieved from

http://www.oandplibrary.org/alp/chap 08_02.asp on April 8th 2008.

Carroll, K. & Edelstein, J.E. (2006) Prosthetics and Patient Management: A comprehensive

clinical approach. Slack Incorporated. New Jersey.

Celikyol, F. (1984) Prostheses, Equipment, Adapted Performance: Reflections on thes Choices

for the training of the Amputee. Occupational Therapy in Healthcare. 1, 89-

Chamberlain. M.A. & Kent. R.M. (2005) The Needs of Young People with Disabilities in

Transition from Paediatric to Adult Services. Europa Medicophysica. 41, 111 -123.

De Tonnacour, C & Shorgan, N. (2004) Pyscho-social considerations in the Rehabilitation of

Children with Congenital Limb Loss. The Association of Childrens Prosthetic Orthotics

Clinics, 10, 24-26

36
http://www.oandplibrary.org/alp/chap%2008_02.asp


37/38

DiCowden, M (1990) Pediatric Rehabilitation: Secial patients,special needs. The Journal of

Rehabilitation. 56, 13 17.

Ephraim, P.L., Dillingham, T.R., Sector, M., Pezzin, L.E. & Mackenzie E.J. (2003) Epidemiology

of Limb Loss and Congenital Limb Deficiency: a Review of the Literature. Archivesof

Physical Medicine & Rehabilitation. 84, 747- 61.

Erikson, E.H (1963) Childhood and Society (2ndEd). Norton. New York.

Froster - Iskenius, U.G & Baird, P.A (1990) Amelia: Incidence and Associated Deficits in a large

Population. Teratology. 41, 23 - 31.

Giele, H., Giele, C., Bower, C and Allison, M. (2001) The incidence and epidemiology of

congenital upper limb anomalies: A total population study. The Journal of Hand Surgery.

26, 628-634.

Hamill, J. & Knutzen. K.M (1995)Biomechanical Basis of Human Movement. Williams & Wilkins.

Philadelphia.

Hostler, S., Gressard, R.P., Hassler, C.R. & Linden, P.G (1989) Adolescent Autonomy Project:

Transition Skills for Adolescents with Physical Disability. Childrens Health Care. 18, 12

18.

Jain, S (1996) Rehabilitation in Limb Deficiency. 2. The Pediatric Amputee.Archives of Physical

Medicine and Rehabilitation. 77, S9- S13.

Kozin, S.H. (2003) Upper Extremity Congenital Anomalies. The Journal of Bone and Joint

Surgery. 85, 1564 1576.

Louis-Bender Paper, T., Kim, J. & Weiner, B. (2002). The shaping of individual meanings

assigned to assistive technology: a review of personal factors. Disability and

Rehabilitation. 24, 5-20.

McMinn, R.M.H., Hutchings, R.T., Pegington, J & Abrahams, P. (1993)Color Atlas of Human

Anatomy (3rdedition ). Mosby. St Louis.

Morrissey, R.T. & Weinstein, S.L. (eds) (2001) Lovell and Winters Pediatric Orthopaedics Vol 2

(5th edition). Lippincott Williams & Wilkins. Philadelphia.

37


38/38

Postema,K., Van Der Donk, V., Van Limbeek, J., Rijken, R.A.J., & Poelma, M.J. (1999)

Prosthesis rejection in children with a unilateral congenital arm defect. Clinical

Rehabilitation. 13, 243 249.

Ramdial, S., Wierzba, S., Kurtz, I. & Hubbard, S. (1997) A Challenging Bilateral Upper

Extremity Powered Prosthetic Fitting. Journal of the Association of Childrens Prosthetic-

Orthotic Clinics. 3, 12-16.

Rumsey, N & Harcourt, D. (2004). Body Image and Disfigurement: Issues and Interventions.

Body Image. 1, 83 -97

Stanley B.G., & Tribuzi S.M. (1992)Concepts in Hand Rehabilitation. F.A. Davis. Philadelphia.

Stark, G (2001) Upper Extremity Limb Fitting. retrieved from http:// www.amputte-

coalition.org/inmotion /jul_aug-01/primer.html on March 23 2008. .

Stoeker, W (2004) Foot Skills and Other Alternatives to Hand Use in R.H Meier & D.J. Atkins

(Eds)

Functional Restoration of Adults and Children with Upper Extremity Amputation. (pp133-134).

Demos Medical Publishing. New York.

Stoller L.C. (1998) Low-Tech Assistive Devices: A Handbook for the school Setting. Therapro.

Framingham

Surman, G., Bonellie, S., Chalmers, J., Colver.,A., Dolk, H., Hemming, K., King, A., Kurinczuk,

J.J., Parkes, J., & Platt, M.J., (2006) Journal Public Health (Oxford). 28, 48 56.

Van Heest, A. (1996) Congenital Disorders of the Hand and Upper Extremity, Pediatrics Clinics of

North America. 43, 1113 1133.

Wenner, S.M (1995) Chapter 69: Prosthetic Management of the Upper-Deficient Child. In J.M.

Hunter, E.J. Mackin, A.D. Callahan (eds.) Rehabilitation of the Hand: Surgery and

Therapy, Volume II(4

th

ed. pp1203 -1210). Missouri: Mosby.

38
http://www.amputte-coalition.org/inmotion%20/jul_aug-01/primer.htmlhttp://www.amputte-coalition.org/inmotion%20/jul_aug-01/primer.html

Documents

Special Topics Paper 1 - For Merge