Occupational Therapy for Children With Upper Limb Deficiencies

Wendy Hill BScOT

Vivian J. Yip OTD, MA, OTR/L

Neither of the following authors nor any immediate family member has received anything of value from or has stock or stock options held in a commercial company or institution related directly or indirectly to the subject of this chapter: Wendy Hill and Dr. Yip.

ABSTRACT

Comprehensive care of a child with an upper limb difference includes occupational therapy. To provide the best outcomes for this patient cohort, it is helpful to review prosthetic and nonprosthetic care and prosthetic and therapy options, as well as understand the role of the occupational therapist. The importance of family-centered care and the involvement of a comprehensive team should be recognized. Considerations are needed to accommodate the uniqueness of each child, family, and situation.

Introduction

Children are adaptable. A child born without an arm or a hand, with a limb difference, or who has an acquired upper limb amputation is capable of learning how to complete necessary tasks and participate in desired activities. If children are fitted with a prosthesis at an early age, encouraged to wear it, and taught how to use it, they will learn how to accomplish important activities using the prosthesis. If a child (or their family) chooses not to wear a prosthesis, he or she will learn to do these activities in other ways, often using other parts of the body to compensate for the missing limb or by incorporating adaptive equipment or tools into their daily life.

Unilateral congenital transradial limb deficiency is a common level of limb loss seen in pediatric prosthetic clinics throughout the world.¹^,²^,³^,⁴ The treatment of upper limb deficiency differs slightly from clinic to clinic, but most clinics offer prosthetic fitting as a common treatment option for children. Regardless of the decision to elect or decline a prosthetic fitting, the child should be regularly evaluated by the management team. The team of specialists in an upper limb clinic can assess the child as he or she develops and address concerns or issues that may arise or evolve over time. Experienced clinicians can be identified through the Association of Children’s Prosthetic and Orthotic Clinics (ACPOC). This organization promotes multidisciplinary team development and collaboration and supports research and education for professionals involved in caring for children who need orthopaedic interventions.⁵ Information on clinical teams who provide care for children with limb differences is available from ACPOC and can serve as an additional resource.

The prosthetic options for children with upper limb loss include passive or cosmetic prostheses, body-powered or cable-activated prostheses, and externally powered prostheses. When children become involved in extracurricular activities and sports, more than one type of prosthesis may be required. Children who are fitted with a prosthesis often have an active-grasping prosthesis for daily use and may also have a passive prosthesis with activity-specific recreational attachments or a passive cosmetic option.

It is important to be aware of the psychological effect experienced by a family or a child with a limb deficiency. The desire to have a prosthesis may be rooted in the child’s or the family’s desire to appear “normal” to the outside world, possibly to avoid stares and questions from strangers, or to assuage the guilt parents may feel regarding the cause of the limb deficiency. A prosthesis may help the child and/or the family come to terms with the child’s condition, and a cosmetic prosthesis may promote comfort in social situations. However, addressing the root of these concerns through a qualified counsellor is important. A prosthesis will not resolve feelings of guilt or social discomfort.

As children develop, they will decide if they need a prosthesis and what type of prosthesis they prefer. Being fit with prostheses during childhood allows the exploration of various options and provides experience on which to base prosthetic decisions later as life activities and/or priorities change.

The Team and First Assessment

The members of the clinical team will vary depending on the clinic but may consist of a physician (pediatrician or physical medicine specialist), an orthopaedic surgeon, a prosthetist, an occupational therapist (OT), a physical therapist, a social worker, a case manager, a child-life specialist, a nutritionist, a psychologist, and a psychiatrist. The first encounter with the clinical team can be intimidating for families, so it is important not to overwhelm them by introducing too many care clinicians at the initial meeting. One of the main goals of the first meeting is to build rapport with the family and child because the clinic may be providing care for many years. Some clinics treat children until they are 18 years of age, whereas others follow the patient into adulthood. If the family is having difficulty accepting or coping with the limb loss, further psychosocial counselling should be recommended.

Pediatric care involves the entire family unit. The activities or interests that are important to the family will form the basis of the child’s treatment by the team. If the family wants the child to be fit with a prosthesis, information and appropriate prosthetic options should be presented by the prosthetist and/or the OT. When funding is secured, the prosthetist will begin building an appropriate prosthesis. The OT typically has a role in evaluating the child as he or she works with a temporary prosthesis and may provide feedback to assist the prosthetist so that the device can be fabricated in the most functional position for this stage of development. The child and the family will then attend prosthetic training sessions with the OT to learn how to use the prosthesis and incorporate it into daily activities.

The role of an OT is to maximize independence in occupations (meaningful and purposeful everyday life activities).⁶ These include the activities of daily living (ADLs) and the instrumental activities of daily living (IADLs). ADLs are activities oriented toward taking care of one’s own body. For a child, this may include bathing, grooming, hygiene, toileting, dressing, and feeding. IADLs may include play participation, exploration, school activities, and social participation with peers and the community.⁶ As children develop, these activities will change and may include participation in hobbies, sports, and work activities. The OT provides skilled services in collaboration with the family and the team to facilitate full engagement in the child’s everyday life activities. For a child with upper limb loss, the OT assists the family in solving problems in accomplishing ADLs and/or IADLs with or without a prosthesis. The OT evaluates the development, behavior, and performance skills of the child to establish family-centered goals and interventions and provides information and resources to educate the family so that the child can work toward optimal participation in ADLs and IADLs.

The involvement of other team members can help the family to accept and cope with the limb difference. The OT will follow the child’s development, help the parents focus on the child’s strengths and abilities, and address concerns about the child’s ability to participate in future activities.⁷ Many aspects of the child’s life, health,⁸ and relationships may be included in the team assessment (Table 1).

TABLE 1 Items That May Be Included in the Team Assessment

Birth history

Medical history

Previous therapy interventions

Health history of parents, siblings, and other extended family members who may have a limb difference

Developmental status

Cognitive function

School-grade

level and performance

Socioeconomic status of parents and/or guardians

Family and child’s daily routine

History of the patient’s use of a prosthesis if one was previously used

Activities of daily living assessment and instrumental activities of daily living assessment—how they are completed (with assistance, using equipment, age appropriateness)

Identification of individuals who provide the most care for the patient

Patient and parent social skills, thoughts about limb difference, coping skills and techniques

Inspection of the residual and sound limbs related to skin integrity, residual limb length, pain, edema, bony prominences, range of motion, and strength

Discussion of interests, goals, and the things the family wants the child to accomplish (with or without a prosthesis) (aesthetics versus function)

Information about prostheses and other adaptive equipment and strategies, as appropriate

Unilateral Transradial Deficiency

Prosthetic Considerations

When a family decides to pursue prosthetic fitting for their child, the options for appropriate types of prostheses should be presented at the first consultation appointment and at subsequent appointments over the years as new technologies or components become available and the needs of the child or family have changed. The options for a prosthesis are dependent on the age of the child, the level of amputation, and the availability of funding. Practice guidelines vary regarding the age when the first prosthesis is prescribed. A 2006 review of the literature determined that the age of the first fitting varied worldwide, with the age ranging from 2 to 25 months.⁹ The aim of the study was to determine if fitting a prosthesis before 2 years of age was related to lower rates of rejection and better functional outcomes at an older age. The authors concluded that the guidelines for prosthetic prescription procedures are based on experience, not on evidence. Currently, the literature shows that most clinics that fit prostheses to children with upper limb differences
agree that there are benefits to fitting a passive prosthesis between the ages of 3 and 9 months.²^,¹⁰

Shaperman¹¹ conducted a survey of fitting practices for children with transradial limb loss in pediatric prosthetic clinics throughout North America. She concluded that most children with unilateral transradial limb absence have their first fitting when they can sit (approximately at age 6 months) and are later fit with an active-grasping prosthesis when the child demonstrates awareness of cause and effect and attempts to hold objects (between ages 10 and 18 months). A recent survey of clinicians working with children in different parts of the world reported that most clinics fit an active prosthesis, either body-powered or externally powered, between 1 and 3 years of age.¹⁰ Because there is limited evidence-based research on the best age for fitting the first prosthesis, it is important to collaborate with the child’s parents to determine what is in the best interest of their child and family.

There are various reasons why children with congenital upper limb differences may be fitted with a prosthesis at different ages or stages of development. The home situation may be overwhelming for some families, and they may be unable to comply with the requirements of prosthetic care when the child is a toddler. A family may want to wait until the child is able to verbalize his or her wants and needs or may not have access to care when the child is young. Regardless of the reason, prosthetic fitting in an older child has its own considerations. The child will likely be able to express his or her opinions, and his or her level of motivation will need to be considered. It is likely that an older child will have adapted to efficiently completing ADLs with compensatory techniques and will not find a prosthesis useful for these tasks. If fitted, the child may only consider a cosmetic option for special occasions or an activity-specific prosthesis to participate in sports or activities such as art or playing musical instruments. If the child decides to incorporate an active prosthetic option into ADLs and IADLs, he or she must be prepared to allot time for training with the prosthesis to achieve proficient use.

The First Passive Prosthesis

The goals of fitting the first passive upper limb prosthesis at an early age are twofold. First and foremost, it allows the family to establish a consistent wearing pattern and become accustomed to incorporating the prosthesis into the family’s lifestyle. When a child age 2 or 3 years is fit with a prosthesis for the first time, parents often struggle to keep the prosthesis on the child. This may be because the child is accustomed to using his or her residual limb in daily activities and the added weight, heat, and diminished sensation while wearing the prosthesis forces the child to do tasks in an unfamiliar manner. Second, a prosthesis that is fitted around the time the child is mastering sitting can be incorporated into the acquisition of gross motor skills. It provides length extension and support on the missing side to aid with sitting balance, reaching forward while sitting, prone play, crawling, and pulling to a standing position (Figure 1).

When a child wears a prosthesis as a regular part of the daily routine, he or she learns to manipulate toys in the true midline of the body, instead of a skewed midline closer to the missing limb when a prosthesis in not worn. The prosthesis helps establish a more upright posture from a young age and encourages body symmetry. The neuronal group selection theory of brain development described by Meurs et al⁹ implies that if a child wears a prosthesis during the early phases of development, a representation of the limb with a prosthesis will be established in the brain. If the prosthesis is used as the child develops motor skills, the child will have motor functioning abilities with and without the prosthesis.⁹ The clinical experiences of the authors of this chapter reinforce this theory because children who establish an early wearing pattern tend to incorporate the use of the device into their daily routines and use it to accomplish daily tasks. A study by Hahamy et al looked at cortical reorganization in children born with one hand who do not use a prosthesis. They determined that the residual limb or other body parts that function in place of the hand (lips, feet) are represented in the area of the brain associated with hand function.¹²

FIGURE 1 Photograph of a child with a passive upper limb prosthesis that provides support in prone play.

The prosthetist and OT provide the parents with a home program when the passive prosthesis is fitted. This program should include instructions for initial wearing time and how to progress to full-time wear. Full-time wear is considered to be all waking hours, with removal of the prosthesis for napping and bathing. The parents should monitor the child for signs (such as localized areas of redness around the brim of the socket or on the distal end of the residual limb) that the prosthesis is becoming too small and in need of adjustment.

The home program should also provide instruction for hygiene and cleaning of the residual limb and the prosthesis. The inner socket should be cleaned daily with a soft, moist cloth (with a mix of soap and water or alcohol and water) or a baby wipe. The outside of the prosthesis should be cleaned as often as the child’s hand is cleaned, such as when there is obvious dirt or food on it. Certain types of prosthetic gloves are more prone to staining than others. If there is a polyvinyl chloride glove covering the hand, it will be easily stained by food dyes, ink, clothing dyes, and other agents. Stains should be cleaned promptly with alcohol or they will be very difficult to remove. Many
of the newer prosthetic hands and gloves for children are made of silicone and are more easily cleaned.

As part of a home program, it is helpful to include a list of toys or activities that will encourage use of the prosthetic arm in bimanual play. If the child does not pay attention to the side with the prosthesis, interesting toys can be attached over the hand with straps or handles. Toys that can be placed in the mouth or that make noise may be highly interesting to very young children. A fun game can be made by placing toys with large handles on the prosthetic hand and encouraging the child to remove them. The child can be encouraged to hold down a toy with the prosthesis while playing with or manipulating the toy with the other hand. Deep pots or buckets filled with blocks or smaller toys to dump or pick up are fun for some children. Playing with large balls or large stuffed animals encourages bimanual arm use, as does playing pat-a-cake or other rhyming songs with arm gestures.

Regular follow-up with the family is essential to monitor progress with the wearing schedule, comfort, and fit of the prosthesis as well as the child’s achievement of developmental milestones. These will be factors in deciding when to fit the next prosthesis. If the prosthesis is too snug before the child is considered developmentally ready, the prosthesis can be lengthened or the socket can be adjusted to allow more time for the development of gross motor skills and improved cognitive readiness.

It is important that the fitting process does not have unnecessary delays that would leave the child without a prosthesis after a wearing schedule has been established. If possible, the fitting for a new prosthesis should be started before the previous device has been completely outgrown. This will ensure any progress made in wearing and using the prosthesis is not lost.

The First Active Prosthesis

The appropriate time to fit a child with the first active prostheses varies among practitioners and in different parts of the world, although most agree that it should occur before age 3 years.¹⁰ Some centers follow a developmental approach to fitting a child with a myoelectric hand, meaning that the child is considered ready if he or she is cognitively ready and is walking and no longer requires arm extension for balance. Other centers fit a body-powered prosthesis when the child meets their readiness criteria; an externally powered prosthesis would be considered if the child proves to be a good user of the body-powered device. In Germany, the family is given the choice of either a myoelectric or body-powered prosthesis when the child is between the ages of 2 and 4 years.¹³ In the United Kingdom, prosthetic centers follow guidelines established by the British Society for Rehabilitation Medicine. These guidelines recommend fitting children for a first passive prosthesis between the ages of 4 to 15 months, and then fitting the first active prosthesis, either a body-powered device or a myoelectric device, between the ages of 15 months and 3 years.¹⁴

When a child has outgrown the passive prosthesis and is considered ready for a prosthesis with active prehension, the team, including the family, must determine what type of prosthesis will best meet the child’s needs. To ensure the child’s success with an active prehension prosthesis, the family must be committed to attending the training appointments and complying with the requirements of the home program.

Body-Powered Prostheses for Young Children

When the child has outgrown the first passive prosthesis and/or has demonstrated a readiness to use a terminal device for grasping, an active-grasping prosthesis should be considered. The criteria to determine readiness are as follows: (1) the child can follow simple one-or two-step directions; (2) he or she has an attention span of at least 5 to 10 minutes; (3) the child attempts to hold objects with his or her residual limb or the terminal device; and (4) the child tolerates being handled by the OT.¹⁵^,¹⁶

During general development, the child may be ready for formal training between 20 and 26 months of age.¹⁵^,¹⁶ The child’s behavior, ability to attend to simple instructions, and motor development will influence the progress during prosthetic training. A home program for the family will guide parents how to assist the child in learning the control motions of opening and closing the terminal device and promote follow-through with using the prosthesis.

Toddlers with a transradial deficiency are typically fit with the elbow preflexed in the socket. This allows the terminal device to be in a midline position without the child exerting much effort, and the prosthesis will be prepositioned adequately for two-handed activities. When the prosthesis is positioned with the elbow in extension, which is more appropriate for adults or older children, toddlers tend to ignore the prosthesis, and more effort is needed for appropriate midline use of the prosthesis.¹⁵^,¹⁶ A well-balanced harness that is slightly snug and does not have a center ring will help the very young child easily learn the control motion.

Initial prosthetic training should begin at a height-appropriate table for the child to learn the basic control motion. The OT should sit behind the child and use both hands to guide the child. For voluntary opening terminal devices, one hand should be on the shoulder joint for stabilization and one hand on the forearm of the prosthesis to assist with humeral flexion for opening the terminal device¹⁵^,¹⁶ (Figure 2). The therapist should call attention to the open terminal device with verbal cues. The child is encouraged to use the sound hand to place an object in the open terminal device while the OT guides the prosthesis from humeral flexion to humeral extension to close the terminal device securely on the object. It is important to use repetitive bimanual tasks to teach the control motion, including activities such as stringing beads, opening nesting barrels, and opening markers. The child should be encouraged to use the prosthesis for gross motor activities and all daily tasks. For voluntary closing terminal devices, training will be similar, however humeral flexion will close the terminal device and humeral extension will open the device. Clinics’ fitting philosophies
of when to fit voluntary opening versus voluntary closing terminal devices may vary and should be individualized to the child’s development and the intended tasks for the prosthesis.

FIGURE 2 Clinical photograph of a therapist positioning a transradial prosthesis to assist with terminal device operation.

As the child progresses and understands the basic control motion, he or she needs to learn various prosthetic skills and refinements. These include prepositioning the terminal device, accurate placement of objects inside the terminal device, opening the terminal device close to the body, keeping the terminal device closed when reaching forward, and refining the basic control motion to include shoulder abduction. All of these skills are learned by the child through practice and performing age-appropriate bimanual activities on a daily basis and during prosthetic training with the OT. Training activities and functional tasks described in the following section on externally powered prostheses also can be used in body-powered prosthesis training.

Externally Powered Prostheses for Young Children

Externally powered prostheses generally refer to those devices operated using myoelectric control. However, activation also can be accomplished with switches, touchpads, or linear transducers. Children with longitudinal deficiencies who have digits at the distal end of the residual limb may prefer to use the movement they have in the distal end to push against or activate a switch or touchpad to control a prosthetic hand.

Egermann et al¹³ described the following keys to success in a pediatric upper limb prosthetic rehabilitation program. A myoelectric prosthesis should be fitted at a specialized center, and the child should be managed by a specialized multidisciplinary team. The child should train with an OT and be regularly monitored to assess use of the prosthesis and for changing prosthetic needs. The center should provide timely support for maintenance and repair of the prosthesis.

Myoelectrode Site Selection

When fitting a young child with the first myoelectric prosthesis, a simple one-muscle system is often used to operate the hand. When the muscle is contracted, the hand opens. When the muscle is not contracting, the hand closes and remains closed. This is often referred to as the “cookie crusher” control strategy. Initially, the electrode sensitivity will be set quite high so that any activity of the muscle will cause the hand to open. This is intentional because it draws attention to the hand and reinforces the cause-effect concept. In some fitting centers, children are fitted right away with a two-muscle system where the extensors are used to open the hand and flexors are used to close the hand. Dr. Liselotte Hermansson,¹⁷ an occupational therapist in Sweden, describes a structured training program for young children learning to use a myoelectric prosthesis using two muscles.

When fitting a toddler or very young child with a myoelectric prosthesis, finding appropriate muscle sites to place an electrode is generally not challenging. The muscles can be palpated during the assessment; however, the electrode is generally placed on the forearm extensors close to the lateral epicondyle. The size of the standard electrode will usually encompass much of the extensor muscle belly, producing a strong signal when the muscle is contracted.

During the appointment to select the myoelectrode site, it is a good idea to place an electrode on the forearm in the approximate location on the extensor muscle and secure it with a cuff or strap if the child allows. A single-function toy modified to operate with a standard electrode can be used to establish the cause-effect concept at this early stage. A toy that lights up, makes sounds, or moves is highly reinforcing for young children (Figure 3). When the child is asked to wiggle his or her “little arm,” the child sees that something happens as a result. This will encourage the child to continue to activate the muscle to see the resulting action of the toy. This technique works well when the child is sitting quietly, either with the parents or with the OT, and is able to attend to the toy. A small room with a limited number of people is recommended for this training. The OT may want to gently hold the child’s forearm when he or she is being asked to wiggle it so the child does not wiggle the whole arm instead of the muscles in the forearm. A child’s prosthetic hand also can be used in place of the toy for the purpose of site selection and cause-effect training. Some children respond well to a prosthetic hand as opposed to a toy; however, because the hand is not attached to the child’s forearm, operating a hand in this way is still an abstract concept and may not generalize into the ability to control the prosthetic hand after the prosthesis is fabricated.

When the child is old enough to have a sustained attention span of at least 10 minutes and the ability to follow simple instructions, the myoelectrode site selection process and socket fitting can take place with the aid of computer software. Several manufacturers have software for evaluating and visualizing muscle signals, but the MyoBoy tester (Ottobock) is most often used for pediatric patients because it is the only software with a virtual child’s prosthetic hand.¹⁸ The MyoBoy tester allows muscle signals to be viewed in real time, and the child can see a virtual hand moving in real time as he or she activates the electrode (Figure 4). If the child can isolate the muscle on command, the electrode sensitivity can be adjusted to an appropriate level, eliminating guesswork. For young children,
the computer software may not be motivating, so attention will be quickly lost. If this happens, it is more important to know that the child understands the concept of contracting the muscle to cause something to happen than to sit for extended periods of time practicing this control. It may be necessary to distract the child with other toys or games to ensure that he or she keeps the socket on long enough to ensure a good fit and intimate contact with the electrode.

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