Rehabilitation of the Child with Cancer
David W. Pruitt
Mary A. McMahon
Susan D. Apkon
The comprehensive rehabilitation of children with cancer requires an interdisciplinary team approach across the continuum of care. Extraordinary improvement in survival of children with a wide variety of cancer types is the result of advances in treatment previously discussed in this text. Accompanying this success are new challenges resulting from morbidity in the survivors, due to the cancer itself or interventions utilized to treat the cancer. Physical, psychological, and social function may be affected. Minimizing the consequences of these sequelae on future quality of life (QOL) is the overall goal of rehabilitation.
The interventions of cancer rehabilitation can be grouped into one of four broad categories, often referred to as the Dietz classification. Preventative rehabilitation emphasizes early intervention and education in order to prevent or delay the symptoms of tumor progression or treatment.1 Interventions might include range of motion in order to maintain joint flexibility in a spastic extremity or prophylaxis for deep venous thrombosis in inpatient rehabilitation. Restorative rehabilitation attempts to assist patients’ return to their premorbid functional status without substantial residual disability. Supportive rehabilitation occurs in those patients who sustain permanent, residual impairments as a result of their cancer or its treatment and focuses on maximizing functional independence in an optimal environment. Palliative rehabilitation is utilized in those patients with a recurrent or progressive cancer and focuses primarily on comfort, caregiver education, minimizing burden of care, and appropriate equipment recommendations.
The International Classification of Functioning, Disability, and Health, known more commonly as ICF, provides a standard language and framework for the description of functioning and disability (see Fig. 44.1). In ICF, the term functioning refers to all body functions, activities and participation, while disability is similarly an umbrella term for impairments, activity limitations, and participation restrictions.2 Impairment refers to the loss or abnormality of psychological, physical, or anatomic structure or function, and is at the organ system level of function. Specific impairments, in this case different types of cancer, may have an impact on the child’s age-appropriate activity, affecting mobility, self-care, communication, cognition, and/or psychological and social function (Table 44.1). Disability is the limitation in activity, in the manner or within the range considered normal, due to impairment. The same impairment may or may not result in activity limitations in different children. Participation restrictions are external to the individual, such as those imposed by architectural or attitudinal barriers, and occur when an impairment or disability prevents a person from engaging in a role that is normal for the age and gender within the social and cultural milieu.
 Figure 44.1 International Classification of Functioning (ICF) model of human functioning and disability. |
Goals of pediatric rehabilitation include minimizing the impairment and maximizing activity and participation in age-appropriate life roles: school, play and recreation, and work. Major objectives include facilitating independent child function in each area of functioning, or domain, that is affected, and also minimizing the burden of disability for the parents and caregivers. Function is promoted in mobility, self-care, communication, cognition, and/or psychosocial domains. Efforts are directed toward achieving maximum independence despite the disorder, primarily through six categories of intervention strategies to help mitigate disability. These include preventing or correcting additional secondary disability; enhancing function in the affected system; enhancing function in systems unaffected; using adaptive equipment to promote function; modifying the social and vocational environment; and using psychological techniques to enhance patient performance and patient/family education. In pediatric rehabilitation, prescriptions for therapy programs, adaptive equipment, orthoses, and prostheses must be appropriate to the age and developmental level of the child and include considerations related to ongoing growth and development.3
By addressing functional impairment, activity restrictions, and participation restrictions, the goal of rehabilitation is to limit the impact of these limitations in physical and psychosocial function so that survivors of childhood cancer are able to optimally participate and function as adults. In studies conducted as part of the Childhood Cancer Survivor Studies (CCSS) and other survivorship studies, there is documentation of the influence of specific childhood cancers and treatments on activity limitations, social role attainment, and health-related quality of life (HRQOL). When compared with survivors who reported no limitations, those with physical performance, executive function, or emotional health deficits are less likely to be employed, married, or have incomes greater than $20,000 a year.4
The interdisciplinary pediatric rehabilitation team evaluating and addressing individualized goals for the child with disability can include one or more of the following specialists: pediatric physiatrist (specialist in physical medicine and rehabilitation), rehabilitation nurse, physical therapist, occupational therapist, speech-language pathologist, psychologist, social worker, therapeutic recreation specialist, prosthetist-orthotist, special educator, and vocational counselor. Care should be coordinated, comprehensive, and family-centered.
Significant functional gains follow rehabilitation of adult patients with cancer. While similar evidence has been minimally evaluated for rehabilitation of children with cancer, extrapolation from adults with cancer and from children with other disabling conditions support the provision of pediatric rehabilitative services to children with cancer.
In the first section of this chapter, the focus is on the common issues of limitations in activity and restrictions in participation that
cross cancer diagnoses involving different organ systems. The second section focuses on specific functional limitations associated with the major specific types of pediatric cancer that result in a significant incidence of disability.
cross cancer diagnoses involving different organ systems. The second section focuses on specific functional limitations associated with the major specific types of pediatric cancer that result in a significant incidence of disability.
TABLE 44.1 Impact of Anticancer Therapies on Function | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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REHABILITATION PROBLEMS
Mobility
Deficits in functional mobility may occur with the generalized deconditioning associated with prolonged or chronic illness and immobility, which is discussed in further detail in the next section of this chapter, as it is such a significant issue in the population of children with cancer. Due either to the tumor or its treatments, central neurologic involvement of the motor strip, long tracts, basal ganglia, cerebellum, or spinal cord, or peripheral neurologic involvement can result in problems in motor function due to weakness or paralysis, spasticity, and/or deficits in balance and coordination, often associated with limited endurance. The physical and occupational therapists work with the child and family to ameliorate specific motor deficits, prevent secondary complications such as contractures, provide training in compensatory strategies, limit the use of abnormal movement patterns, and recommend the use of orthotic and assistive devices as appropriate.
Progression of ambulation retraining may involve the use of gait aids, often beginning with those providing more support, such as a walker, and weaning to crutches and possibly to canes before independence is reestablished. Bracing, most typically with ankle-foot orthoses (AFOs), may be indicated. Design of the AFO is determined by multiple factors, including ankle strength, range of motion, presence of spasticity, and medial-lateral ankle stability. Alternative means of mobility to ambulation may be indicated, temporarily or permanently, for some children with a variety of types of cancer. Some may require a manual wheelchair; however, others may benefit from power wheelchair use for independence in mobility.
As endurance improves, intensity of exercise regimens can be increased. Exercise programs for children in the acute phase of their management may initially focus on passive range of motion to maintain joint flexibility. As the child is able, active participation is progressively increased, with particular activities selected to focus on the child’s specific individualized therapy goals, which are advanced as possible. Participation in sports and recreational activities should be encouraged, whenever possible. Aquatic therapy often allows movement, due to buoyancy and elimination of gravity, not possible out of the water. Again, progression can occur as the child is able, often to the level of competitive sports participation in the long-term.
Immobility
Prolonged bed rest and immobility affect almost every organ system and can negatively impact a child’s functional capacity. Muscle strength and endurance decrease due to the inactivity and reduced force of gravity associated with bed rest. With complete bed rest, a muscle loses 1.0% to 1.5% of its strength per day or 10% to 15% per week.5 Immobilized muscles have also been shown to have a more rapid depletion of glycogen and an increased production of lactic acid during work.6 Muscles immobilized in a shortened position are also at risk for contracture, associated with segmental necrosis, disorganization of myofibrils, and a reduction in the number of sarcomeres. Connective tissue contracts and reorganizes within 1 week of a joint becoming immobile, further increasing the risk for contracture development. Inactivity also results in increased bone resorption that may result in osteoporosis and the risk for pathologic fractures.
Immobilization also has significant effects on the cardiovascular system. Increased sympathetic activity leads to an increased heart rate. Cardiac output, stroke volume, and left ventricular function decline and orthostatic hypotension increases. A decrease in cardiac output combined with a peripheral oxygen utilization deficiency causes a decline in maximal oxygen consumption.7 Blood volume decreases with prolonged bed rest. Plasma volume decreases more than red cell mass resulting in increased blood viscosity.7 Increased blood viscosity combined with immobility places the patient at increased risk for deep venous thrombosis.
Respiratory complications of immobility can be life-threatening. Potential changes include diminished diaphragmatic movement in the supine position, decreased chest excursion, and decreased range of motion of the costovertebral and costochondral joints. These changes can result in a decrease in the vital capacity and functional reserve capacity of 25% to 50%.7 The ventilation-to-perfusion ratio may be altered in dependent areas of the lung, resulting in arteriovenous shunting and reduced oxygenation. Impaired ability to clear secretions can lead to atelectasis and increased risk for pneumonia.
Pressure ulcers are another complication of immobility that can occur when external pressure is greater than capillary pressure for prolonged periods. Poor nutrition, moisture, insensate skin, and shear forces are additional risk factors for skin breakdown. Supine patients are at risk for pressure ulcers over their occiput, sacrum, and heels. Patients lying on their sides are at risk for breakdown over their greater trochanters and patients who sit for prolonged periods are at risk for ulcers over their ischial tuberosities.
Activities of Daily Living
All members of the rehabilitation team, but specifically the occupational therapist, work with the child to increase independence in age-appropriate daily care activities such as eating, grooming, bathing, toileting, and playing, with the use of adaptive equipment as needed. Bladder and bowel dysfunction, in particular, pose common special management challenges and so are discussed in detail. Family education is provided and family members are encouraged to allow the child to function at the highest level of independence at which the child is capable.
Bladder Dysfunction
Various combinations of urinary storage and voiding impairment may occur with tumors along the neural axis from the pons to the cauda equina, due to lower urinary tract dysfunction. Incontinence with lesions above the level of the pons is usually due to disinhibition. Neural pathways that modulate bladder function traverse the length of the spinal cord between the pons and the sacral spinal cord, with events coordinated in the pontine micturition center. Interruption of these pathways result in storage or voiding dysfunction or detrusor-sphincter dyssynergia (DSD) when there is loss of the coordinated function of the detrusor and the external striated urethral sphincter. Lesions at the level of the pons may also impair the coordinated functioning of the lower urinary tract. As seen in spinal cord lesions, manifestations are usually upper motor neuron in nature with involuntary detrusor contractions, nonrelaxation of the external sphincter during detrusor contractions, and subsequent development of bladder wall thickening, trabeculations, and decreased compliance and storage capacity due to detrusor hyperactivity. Fluorourodynamics is the most informative study to evaluate children with neuropathic vesicouretral dysfunction. This study superimposes pressure measurements on the simultaneous fluoroscopic appearance of the bladder and urethra and provides valuable information on the function of the bladder and urethra during filling and voiding. Clean intermittent catheterization is an effective method of bladder emptying and may be used alone or in combination with anticholinergic or alpha-adrenergic medications to protect the upper tracts while achieving satisfactory continence. Fluid intake should be regulated so that only 4 to 5 catheterizations are necessary to not exceed the bladder capacity (age [years] + 2 = ounces).8 If high volumes of fluid are necessary due to the chemotherapy regimen, placement of an indwelling catheter may be necessary in the short term. At 5 to 7 years of age, children with adequate hand function can begin self-catheterization.
Bowel Dysfunction
Constipation, defined as infrequent, excessively hard and dry bowel movements, is a common problem for children with cancer. Decreased rectal filling or emptying may be due to poor intake, dehydration, decreased activity, narcotic analgesics, tumor-related neurologic injury, or neurotoxic chemotherapeutic agents. Vincristine and vinblastine are neurotoxic alkaloids, which commonly disrupt bowel function via their neuropathic effects, including peripheral neuropathy. Nonfunctional afferent and efferent pathways from the sacral cord result in impaired rectal emptying similar to that seen in neurogenic bowel due to spinal cord injury (SCI).
Management includes promoting mobility, providing appropriate positioning for the nonambulatory child, increasing dietary fiber and fluid intake, and minimizing the use of medications that decrease gastrointestinal motility, if possible.
Fluid intake is critical, particularly when strategies to address the constipation include the addition of bulk to the diet. Bulk without adequate hydration increases risk of impaction. If activity is limited or ongoing administration of narcotic or neurotoxic agents is required, a stool softener or mild laxative may be given with adjustment of the dose and frequency to ensure good bowel evacuation at least every other day. The goal of a bowel program is to have the bowel empty regularly and adequately.
The child with a neurogenic bowel may require a formal bowel program with digital stimulation or a suppository, in addition, to stimulate evacuation from below. Use of digital stimulation and suppositories may have to be limited when a child is neutropenic. Alternative bowel programs may need to be utilized during those times. The presence of an anal wink indicates an upper motor neuron lesion and a better prognosis for continence, since sphincter tone is usually adequate to retain the stool. When this reflex is absent, as in lower motor neuron lesions, there may be a flaccid sphincter and inabilities to either expel or retain feces. Constant stool leakage can occur. Routine and consistency are critical to the successful bowel program; therefore, a convenient, relaxed time should be selected to perform the bowel program each day and should vary as little as possible from day to day. Trying to evacuate the bowels 30 minutes after a meal will take advantage of the gastrocolic reflex.
Problems with bowel management may represent a significant source of emotional turmoil for both the child and his or her family, particularly in the older, previously continent child. Successful bowel management enhances the potential of the child to achieve age-appropriate independence and social acceptability.
Communication
Children with cancer may experience communication disorders as a consequence of their primary disease, particularly with primary brain tumors, central nervous system (CNS) metastasis, or as a late effect of cranial irradiation. Depending on the area of the brain affected and the age of the child, communication may be impaired due to deficits in speech, language, cognition, memory, and/or personality. While speech and voice problems are related to motor dysfunction, either due to weakness of the involved structures or incoordination, language skills are more reflective of cognitive functioning (Table 44.2). For this reason, language processing may be further compromised by concomitant impairments in critical cognitive or information-processing skills, such as memory, perception, attention, or organization, as well as behavioral impairment, such as disinhibition, poor self-monitoring, limited frustration tolerance, or poor judgment.
In older children with established language skills, the processing and use of language is usually abruptly disrupted. The pattern of speech and language deficits is dependent on the area of the brain injury. These may be related to receptive and/or expressive language problems or motor dysfunction, including dysarthria with weakness of the oral musculature, apraxia due to motor incoordination, or phonation deficits due to velopharyngeal insufficiency or vocal cord paralysis. The child with severe expressive language deficits and good comprehension may benefit from an augmentative or alternative communication system. Pragmatics may be a problem when there is frontal or right hemisphere involvement. In the very young child, who has not yet fully developed language skills, the pattern of language dysfunction is less predictable than that seen in the older child with a similar lesion. The child may present with a developmental language disorder, secondary either to specific neurologic involvement or as a part of the global developmental delay often seen in children with serious and chronic illness early in life. There is little in the literature on the treatment-related effects or neuroplasticity in this setting and unfortunately, due to the limited former skill acquisition, these children have few compensatory strategies available to them. As language development parallels cognition, in general, factors that affect cognition will have a similar impact on language skills. Following evaluation, communication deficits may be addressed with individual and/or group within a developmental format.
TABLE 44.2 Acquired Communication Impairments | ||||||||||||||||||||
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Thorough speech and language assessment should be completed by the speech pathologist and a therapeutic program planned to address communication deficits in a manner appropriate to the child’s age and medical condition. For the child who has lost the capacity for verbal communication, it is important to provide some form of functional communication as a means of self-expression and to indicate needs. Simple communication boards, electronic augmentative communication devices, keyboards, and sign language are among the available options.
Cognition
Cranial irradiation, as well as both intrathecal and high-dose intravenous methotrexate have long been associated with leukoencephalopathy and learning disability.9 Also, studies have suggested that the administration of high-dose methotrexate potentiates the deleterious effects of cranial irradiation on cognition.10 The impact on cognitive function is an often devastating late effect of cancer
therapy, which significantly impacts QOL, particularly in very young children. The age at the time of treatment is a major factor in the development of cognitive decline following cranial irradiation. Children with leukemia, lymphoma, or brain tumors treated before 4 or 5 years of age are at higher risk for cognitive impairment, in comparison to older children.9 The deleterious effects can have major impact on intellectual and academic performance, social competence, behavior, and vocational potential. Cognitive dysfunction in children who survive brain tumors is covered later in this chapter.
therapy, which significantly impacts QOL, particularly in very young children. The age at the time of treatment is a major factor in the development of cognitive decline following cranial irradiation. Children with leukemia, lymphoma, or brain tumors treated before 4 or 5 years of age are at higher risk for cognitive impairment, in comparison to older children.9 The deleterious effects can have major impact on intellectual and academic performance, social competence, behavior, and vocational potential. Cognitive dysfunction in children who survive brain tumors is covered later in this chapter.
Psychosocial
Issues related to psychological adjustment to chronic illness and disability, family adaptation, and sibling adjustment are critical to the long-term outcomes of children with cancer. It is crucial that comprehensive rehabilitation, in addition to directing efforts to maximize independent function in the domains discussed above also address school reentry and eventual work entry, as well as inclusion with the family and peers in social and recreational activities. These issues are comprehensively covered elsewhere in this text.
SPECIFIC CHILDHOOD CANCERS: REHABILITATION ISSUES
Brain Tumors
Intracranial tumors represent the second most common type of childhood cancer, and most common solid tumor, with peak incidence in early childhood.11 Children with brain tumors experience significant functional deficits related to the primary disease process and also as a consequence of its treatment. Childhood brain tumors represent a heterogeneous group of tumors, which vary in pathologic characteristics, tumor biology, response to therapy, anatomic location, and age at diagnosis. With the advances in diagnostic strategies, neurosurgical techniques, and cooperative therapeutic trials over the past 30 years, more than 60% of children with these neoplasms are now surviving.11 With this improved survival has come increased recognition of the significance of the long-term sequelae of the tumor, its treatment, and the impact of consequent functional deficits on QOL.
Each treatment modality may be associated with both transient and long-term effects, which may have an impact on duration of survival, functional outcome, and QOL. Despite the significantly decreased surgical morbidity due to improvements in surgery, anesthesia, and postoperative care, surgical resections may be associated with significant neurologic morbidity related to the age and preoperative clinical status of the child, type of tumor, location, and extent of resection.
Although the introduction of radiation therapy has significantly improved duration of survival in a number of CNS tumors, it is well recognized that cranial radiation has long-term effects that are progressive and potentially devastating. The extent of the radiation-induced injury is directly related to the dose and volume of CNS irradiated, in addition to the age of the child. Histologic changes in the brain following cranial irradiation may include neuronal dropout, gliosis, and proliferative and sclerosing angiopathy.12 Long-term complications associated with these changes include cognitive deficits, endocrinopathies, vasculopathies, hearing loss, radiation necrosis, and second primary neoplasms.13 Radiation myelitis with spastic paraplegia or tetraplegia may result from spinal cord irradiation. Additionally, radiation to the vertebrae in the young child increases the risk of scoliosis and kyphosis.
The effects of chemotherapy on the developing nervous system are less well understood. Leukoencephalopathy is a late complication associated with both radiation and chemotherapy, particularly methotrexate given intrathecally or in high-dose intravenously. It is characterized clinically by dementia, ataxia, and focal motor deficits, and can progress to coma and death. Other delayed toxic effects of chemotherapy that impact significantly on function include peripheral neuropathies, myopathies, and hearing loss.
Children with brain tumors may experience a wide range of functional deficits related to the effects of the primary lesion or treatment complications. These include motor and sensory deficits, speech and language dysfunction, cognitive impairment, and psycho-emotional disorders. The nature and extent of impairment depends on the age and developmental level of the child at time of diagnosis, the location of the lesion, and the degree of neurologic compromise. With the exception of cognition, there is a paucity of information related to rehabilitation issues in the literature. The basic tenets of neurorehabilitation are heavily impairment-driven, with the goal to maximize function and minimize caregiver and societal burden for all etiologies of acquired brain injuries (ABI).14 Common impairments in persons with brain tumors undergoing rehabilitation include weakness (hemiparesis and general debility), cognitive and visual-perceptive deficits, ataxia, cranial nerve dysfunction, bowel and bladder problems, aphasia, dysphagia, and dysarthria.15,16 Overlapping impairment profiles suggest that the rehabilitation team can approach brain tumor survivors much like more familiar etiologies of ABI.14 Creating a rehabilitation program for the optimization of functional independence necessitates identification of appropriate goals and phases of rehabilitation care as well as clear communication of realistic goals to the patient and family.
Children with brain tumors may require acute inpatient rehabilitation, outpatient rehabilitation, or home-based therapy. Often, children are admitted to the inpatient rehabilitation unit following craniotomy for gross total resection, surgical debulking, or biopsy and sustain impairments and disabilities as a result of the tumor or side effects of treatment. An inpatient pediatric rehabilitation environment assists in optimizing a therapeutic schedule and environment, typically including therapeutic services for 3 or more hours per day. A functional neurologic examination by the team at the time of admission assesses strength, coordination, reflexes, muscle tone, sensation and levels of independence with mobility, activities of daily living, communication, and cognition. The clinician should consider how impairments in multiple areas (musculoskeletal, neurologic, mood, cognitive, visual, and vestibular) collectively affect limitations in activity and participation.14 Impairment-specific functional assessments may assist in providing objective assessments in outlining and developing a plan of care. Currently, there are no objective measurements for functional assessments specific to pediatric brain tumors, so measurements for other ABI are utilized when needed (i.e., Romberg Test, Childhood Orientation and Amnesia Test, Bruininks-Oseretsky Test of Motor Proficiency). Once evaluations are completed, the team formulates and communicates the rehabilitation plan of care based on the goals identified by the team, family, and patient. Integral to this formulation is the identification of appropriate Dietz category and assuring that the goals are realistic and well defined, consider the prognosis, and involve the caregiver.
Rehabilitation strategies utilized in other ABI are also employed in the rehabilitation care of children with impairments and disabilities due to a result of a brain tumor and its treatments. Constraint-induced motor therapy (CIMT), partial weight-supported ambulation, functional electrical stimulation, spasticity management, cognitive and visual and vestibular rehabilitation are all used in the appropriate clinical circumstance.14 For example, a child with a cerebellar astrocytoma may present with vestibular dysfunction, ataxia, dysmetria, and nystagmus and may benefit from incorporating vestibular adaptation and habituation activities as the primary focus. A child with a frontoparietal juvenile pilocytic astrocytoma may present with a dense hemiparesis with evolving hypertonia and spasticity. A trial of a number of therapeutic interventions including CIMT of the unaffected upper extremity and partial weight-supported treadmill training may assist in cortical reintegration. Treatment of spastic muscles with antispasticity medication if generalized spasticity is observed or focal
management with botulinum toxin injections to selected muscles may also assist in functional recovery. In some cases of restorative and more cases of supportive rehabilitation, evaluation of appropriate equipment or devices to obtain or maintain functional independence is necessary. Examples of such equipment might include a walker or wheelchair for mobility, adaptive utensils for activities of daily living, or augmentative communication devices for communication. A thorough evaluation is necessary for identifying appropriate equipment and knowledge of Dietz category is helpful in identification of appropriate equipment needs of both the child and the caregivers. Reevaluation at follow-up visits is also integral in evaluating the current rehabilitation program and optimization of the child’s functional independence.
management with botulinum toxin injections to selected muscles may also assist in functional recovery. In some cases of restorative and more cases of supportive rehabilitation, evaluation of appropriate equipment or devices to obtain or maintain functional independence is necessary. Examples of such equipment might include a walker or wheelchair for mobility, adaptive utensils for activities of daily living, or augmentative communication devices for communication. A thorough evaluation is necessary for identifying appropriate equipment and knowledge of Dietz category is helpful in identification of appropriate equipment needs of both the child and the caregivers. Reevaluation at follow-up visits is also integral in evaluating the current rehabilitation program and optimization of the child’s functional independence.
For the child who is receiving rehabilitation services while undergoing radiation and/or chemotherapy, it is particularly critical to have good communication and cooperation between the rehabilitation and neuro-oncology teams. The rehabilitation team must be aware of the treatment planned and any potential complications. They must be sensitive to problems of pain, nausea, anorexia, constipation, and poor endurance, which may limit the child’s ability to participate fully in the rehabilitation process. When pain is an issue, adequate pain management must be provided, including medication and adjunct strategies and services such as counseling, relaxation techniques, therapeutic modalities, and biofeedback when appropriate. Rehabilitation schedules should be modified appropriately to accommodate the antitumor treatments and allow rest periods. The occupational therapist can address energy conservation strategies and pacing with the family and child. Additionally, the team must be vigilant for subtle signs and symptoms of treatment complications or disease progression and address these with the neuro-oncologist.
Highlighted below are specific areas of dysfunction that present frequent challenges for the child with a brain tumor, with significant potential for impact on QOL.
Physical Performance
Physical disability can have significant impact on educational, vocational, and economic potential. The CCSS recently reported on physical performance limitations and associated participation restrictions in young cancer survivors.
The highest prevalence of physical performance limitations in the CCSS cohort were among survivors of bone (36.9%) and brain tumors (26.6%).17 Treatment with radiation and treatment with a combination of alkylating agents or anthracyclines were risk factors for physical performance limitations and consequent participation restrictions. Survivors treated with radiation were most likely to report limitations in physical performance and participation restrictions in self-care activities, routine activities, school, or work.18
Sensory Deficits
Sensory deficits may occur due to direct involvement of the tumor or due to the antitumor therapy. Visual loss, visual field deficits, gaze palsies, and involuntary ocular movements may all impair vision and result in significant functional impairment. Visual disturbances are quite common in craniopharyngiomas due to compression of the optic chiasm as well as in patients with neurofibromatosis who develop optic gliomas. Thorough ophthalmologic assessment should be completed and visual function monitored as part of the rehabilitation plan. This is particularly important in young children with oculomotor abnormalities who may require treatment to prevent amblyopia. Of note, focal pathology does not always signify focal disease. For example, the abducens nerve has a long free intracranial course and passes in close proximity to bony structures. It may be compromised due to elevation of intracranial pressure, with resultant sixth nerve palsy and diplopia. The occupational therapist can address compensatory strategies for visual deficits, in addition to addressing visual-motor and visual-perceptual deficits. With severe vision impairment, a low vision specialist should be part of the rehabilitation team. Adjunct service providers are usually available through the state agency for the blind, the school district, or special schools for the blind/visually impaired. If visual impairment is severe, referral should always be made to the state commission for the blind for adjunct services and equipment needs.
Hearing loss can result from tumor involvement or as a consequence of radiation or chemotherapy agents such as cisplatin and carboplatin. Cisplatin produces high-frequency sensorineural hearing loss and tinnitus, the latter of which usually subsides. The hearing loss is almost always permanent and is primarily due to injury to the hair cells of the organ of Corti, although damage to the stria vascularis has also been described. Factors associated with a higher risk of cisplatin toxicity include prior or concomitant cranial irradiation, preexisting hearing loss, decreased renal function, concomitant use of other ototoxic drugs, faster infusion rate, higher peak plasma concentration, very young age or older age, higher cumulative dose, in addition to individual susceptibility.19 Previous work had demonstrated that amifostine may ameliorate the risk of severe ototoxicity; however, further investigation has failed to warrant a protective effect in patients receiving treatment for other cancer diagnosis with platinum-based therapies.20,21
With carboplatin, studies have revealed high-frequency hearing loss, but minor or no loss of hair cells. Baseline audiologic evaluation should be provided for all children, with regular reevaluation determined by the type of treatment provided. Hearing assessment should precede initiation of speech and language therapy services. Depending on the deficits, amplification may be warranted in the form of a hearing aid or auditory trainer. For the young child with severe hearing loss, instruction in sign language may be appropriate, in addition to training in oral language skills. For the child who has been exposed to chemotherapeutic agents that are ototoxic, care must be taken to limit further exposure to ototoxic agents, even in the absence of hearing deficits, as the toxic effects may be cumulative.
Impairment of smell and taste are less recognized sensory deficits, but can be seen with tumors which involve the region of the olfactory nerve. These deficits become significant when they affect appetite and therefore nutrition.
Cognitive Impairment
At diagnosis, cognitive impairment is most common with hemispheric and supratentorial midline tumors. Deficits in memory, language acquisition and comprehension, attention, and academic skills vary with age at diagnosis, gender, type and duration of presenting symptoms, tumor extent, and treatment. With improved survival, the impact of cranial irradiation on cognitive function and academic potential has gained increased significance. Radiation therapy is associated with a significant decline in cognitive function that is inversely related to age at diagnosis. A mean IQ loss of 27 points has been demonstrated 2 years following cranial radiation in children under 7 years, while no significant difference was seen in the performance of older children at reevaluation.22 Younger children, particularly those less than 3 years of age, have been found to be more susceptible to the negative cognitive effects of radiation therapy, possibly due to decreased plasticity following global impact to a very young brain, with reported drops in IQ as much as 40 points. Treatment approaches may be adapted, for example, delaying radiation treatment, to ameliorate impact on cognitive function. It is unclear why female gender has also been recognized as a risk factor for neurocognitive deficits but may be related to gender differences in brain development. Deficits have included significant impairments in verbal and performance IQ.23 Problems in attention/concentration, processing speed, working memory, and nondominant hemisphere function, such as visual-motor integration and visual-spatial functions are the most common cognitive deficits among brain tumor survivors.23
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